The Heart-Brain-Body Connection: A Guide for Effective Parent-Led School Advocacy

Dr. Cheryl L. Brosig, PhD

Professor of Pediatrics and the Chief of Pediatric Psychology and Developmental Medicine at the Medical College of Wisconsin.

Kyle Landry

Educational Achievement Partnership Program Manager at Children’s Wisconsin

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When I last posted several weeks ago I alluded to the 5 essential steps the Herma Heart Institute’s Educational Achievement Partnership Program uses to advocate for cardiac patients within the school setting. As a parent it’s likely you speak with your child’s school team on a daily basis. While check-ins and other informal conversations are generally positive and productive, I often hear parents say they just can’t get the message across of how their child’s learning challenges may be related to their medical condition(s). This is where the logic chain comes into play!  

The Heart-Brain-Body Connection

When educating school staff on how pediatric heart disease (as well as other physical and mental health conditions) affect your child’s learning and development, it is important to create a logic chain which links the child’s medical condition(s), to how it affects their physical health, to how it physically impacts brain development, to how it impacts all aspects of neurodevelopment, and finally to how it results in educational challenges observed within the classroom setting. Don’t let that overwhelm you…I’m here to walk you through step-by-step.

Here is when I bust out the logic chain:

• When requesting an IEP/504 Plan evaluation for the first time (initial evaluation)

• When requesting revisions be made to a current IEP/504 Plan (review/revise request)

• During annual health planning or update meetings with school staff (whether they have an IEP/504 Plan or not)

• When educating new teaching or support staff about the child’s medical history (whether they have an IEP/504 Plan or not)

I always make sure I write my logic chain up as a formal letter and save it to a PDF so it cannot be edited. I then email a copy of my letter to all staff working directly with the child on one email thread, and then hand deliver the second copy to a special education teacher, school psychologist, school social worker, or other key staff member my child works with (regular education teacher is appropriate for a child who has never had an IEP/504 Plan before) “for their records.” For some reason receiving it twice shows you mean business, but in a totally approachable way. 

When prepared in advance of an IEP/504 Plan meeting (regardless of an initial or revision meeting) I specifically ask for 10 minutes at the beginning of the meeting to share the letter with the team and answer any questions about how the child’s medical condition directly relates to challenges they may be seeing in the classroom. This step is very important because it sets the foundation for what the rest of the team should be considering when determining qualification or modifications. It’s easy to skim the letter or fail to read it all together, but when a parent comes to a meeting with prepared talking points, everyone listens.

Ok, let’s tackle drafting this letter together!

To start your letter always begin with some sort of brief intro paragraph. I recommend stating your child’s name, acknowledging their complex medical history, identifying concerns in the schools setting, and requesting formal support ensuring that they have access to all of the support services necessary to equitably participate (and thrive!) in an academic setting. Here’s an example you could customize:

I am writing today on behalf of my child/son/daughter, [name]. [Name] was born with a complex congenital heart defect requiring multiple corrective surgeries during his/her first few weeks of life. While [name] is [stable/doing well/etc.] from a cardiac perspective, he/she is at risk for associated neurodevelopmental deficits and differences. Considering [name’s] recent/on-going classroom challenges in [reading/writing/math/executive functioning/attention/behavior/social-emotional/etc.] I would like to request [a formal IEP/504 Plan evaluation/re-evaluation/review and revise meeting/etc.] to ensure he/she has access to all of the support services necessary to equitably thrive in an academic setting. 

Let’s dive in… 

Step 1: Clearly Name and Describe the Medical Conditions(s)

Clearly state your child’s diagnosis/ses and describe what that means in brief layman’s terms. If they have several different diagnoses it’s ok to list them all in bulleted form. 

Here are some examples:

• Artial Septal Defect: a hole in the muscle wall (septum) that typically separates the upper right and left chambers of the heart (atria). As a result, some oxygenated blood from the left atrium flows through the hole in the septum into the right atrium, where it mixes with oxygen-poor blood and increases the total amount of blood that flows toward the lungs. 

• Coarctation of the Aorta: a narrowing or constricting of the aorta, the large vessel that carries the oxygen-rich blood from the heart to the brain and body. When this artery is constricted, the heart has to work much harder to force blood though the narrowing and on to the brain and body, overworking the heart over time.  

• Truncus Arteriosus: a rare defect of the heart in which a single common blood vessel comes out of the heart, instead of the usual two vessels (the main pulmonary artery and aorta), which causes mixed, oxygen-poor blood to be circulated throughout the brain and body. 

• Hypoplastic Left Heart Syndrome (HLHS): a severe congenital heart defect in which the left ventricle is critically underdeveloped at birth, significantly impairing the heart’s ability to pump blood to the brain and body. 

One or two sentence is plenty - how their cardiac anatomy is physically different and how that impacts or changes blood and oxygen circulation. Google is your friend, here. I search diagnoses all the time to write my layman’s definitions. Just beware, with lots of different cardiac diagnoses and lots of different combinations of defects, you need to be sure that what you are describing actually matches your child’s unique anatomy. 

Step 2: Describe the Body and Health Impact

For this section we simply need to make a statement about how the CHD (or other heart diseases or irregularities), as well as any other relevant medical conditions, can impact the body from a physical perspective. I generally have one “bread and butter” sentence that I use and customize as needed to make this point clear. 

Here it is…

Congenital Heart Defects (CHD) and other pediatric heart diseases may affect blood-oxygen circulation in the body, and may cause unique baseline symptoms that may require rest and recovery such as irregular heart rate, breathing disturbances, cyanosis (blue/gray coloring of the mouth, lips, and nail beds), low energy, fatigue, and may cause strength, vitality, and alertness limitations.

If your child experiences additional symptoms as a result of their diagnoses (i.e. headaches, dizziness, stomachaches, body temperature fluctuations, etc.) add those here as well. 

Step 3: Describe the Brain and Developmental Impact

Great! Next, how is the brain potentially impacted? Once again, I will share the statement that works well for the EAPP. And once again, you can choose to use it or customize it in a way that makes most sense for your child. 

Pediatric heart diseases, their treatments, and related complications are known to affect normal blood and oxygen flow, potentially delaying pre- and post- natal brain development. 

Step 4: List Neuropsychological Deficits

Now we take that last sentence one step further…

In fact, significant research studies have suggested that children with heart disease often score lower in hand-eye coordination, fine and gross motor skills, social-emotional functioning, and language development than their typically developing peers. Behavior, attention, immaturity, and learning challenges are also much more common among children with heart disease.

Step 5: Describe Known Educational Challenges and List Challenges Observed

When it comes to the school setting, extensive research has shown lots of common educational challenges. Here are a few common things to consider:

• Educational concerns can arise at different stages of development from preschool though adulthood.

• New concerns may emerge when age level expectations increase in areas of independence, organization, attention, and academic skills.

• Subtle neurodevelopmental deficits affecting multiple domains are extremely common and often result in more substantial academic concerns over time.

• Neuropsychological deficits do not self-correct and these children simply cannot be expected to “catch-up” on their own.

Feel free to do your own additional research too! 

Here is where you braid in your specific concerns for your child. Or better yet, further bulk up this section with the results from any formal neurodevelopmental testing that has been completed. 

This section might look something like this: 

At this time I have concerns related to [name’s]… (list away, but be specific! Share any examples that have been clearly observed in a classroom setting). 

Academic challenges like those [name] is experiencing are very common with pediatric heart patients. These concerns often arise at different times from preschool though adulthood as independence, organization, and skill level demands increase. Subtle neurodevelopmental deficits affecting multiple domains may result in more substantial academic concerns over time as they do not self-correct and these children cannot be expected to simply “catch-up” on their own. 

[Name] received a formal neuropsychological evaluation on [date] at [age]. This assessment included interviews, observations, and completion of both formal and informal tasks, as well as pencil and paper based testing. The completed evaluation report revealed the following diagnoses and impairments… (then list as applicable just as you did with the medical conditions – diagnosis and layman’s description).     

Finally, wrap up your letter by restating the purpose. Don’t forget to sign off with your signature and contact info! If I was writing the letter, my closing paragraph might look like this:

Based on a combination of the known neurodevelopmental challenges related to pediatric heart disease and the challenges observed at home, in the classroom, and in the community, this letter should serve as a formal request for [a/an IEP/504 Plan evaluation/re-evaluation/review and revise meeting/etc.] to evaluate for [new/additional] educational services and/or accommodations. Please contact me at your earliest convenience to complete any necessary paperwork and schedule follow-up.  

Ok, so your letter is done but your work isn’t! 

Remember to send off your letter with a request for 10 minutes at the beginning of any related follow-up meeting to share this information with the team. Review your key talking points ahead of time and practice delivering a clear and concise message: these are my child’s medical conditions, this is how their brain and body are impacted (or at least at risk), and here are my concerns that I would like discussed as a team today. 

Almost done, one last step. I urge you to talk about the importance of school support as a component of follow-up care and advocate for school liaison support within your own hospitals and cardiac centers. If you don’t have the right words to start the conversation, I do. Seriously, share my contact information. While my work is housed within Children’s Wisconsin, my mission is global. Your child matters to me and I am willing to put in the time, effort, and “dirty work” to advocate for the resources and support they deserve. I am also here to celebrate your successes – if these 5 steps transformed the way you were able to advocate for your child or their access to a quality education, I want to hear about it! 

Stay tuned for the very anticipated launch of our new Educational Achievement Partnership Program webpage, loaded with inspiring patient stories and our most tried and true advocacy resources, this Fall!

ATTENTION DEFICIT HYPERACTIVITY DISORDER IN CHILDREN AND YOUTH WITH CONGENITAL HEART DISEASE-PART II

PART II: What to Do If You Think your Child with CHD May Also Have ADHD

David D. Schwartz PhD ABPP
Neuropsychologist, Texas Children’s Hospital
Associate Professor of Pediatrics, Baylor College of Medicine

Katherine Cutitta PhD 
Psychologist, Texas Children’s Hospital
Assistant Professor of Pediatrics, Baylor College of Medicine

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Background

Children with congenital heart disease (CHD) are more likely to develop Attention Deficit Hyperactivity Disorder (ADHD) than their peers. One recent study at Texas Children’s Hospital found that children with CHD (regardless of severity) were more than twice as likely to develop ADHD compared to all other patients. Other studies have shown that roughly 30% of children with more complex CHD have significant attention problems and/or hyperactive/impulsive behavior. In Part I of this Practitioner’s Post, we described the core symptoms of ADHD and placed them in the context of a broader model of self-regulation and self-control, and reviewed the etiology (causes) of ADHD with a focus on children with CHD. In Part II, we explain what to do if you think your child with CHD also has ADHD, with a focus on evidence-based assessment and effective treatment and management strategies.

PART II: What to Do If You Think your Child with CHD May Also Have ADHD

Assessment & Diagnosis

As discussed in Part I of this post, signs and symptoms that your child may have ADHD include attention problems, hyperactive behavior, and/or impulsivity that interferes with their development or daily functioning or causes significant distress. Many people express concern that ADHD is over-diagnosed. Research suggests that, in fact, it is both over-diagnosed and under-diagnosed. Boys with behavior problems and Black children are more likely to be misdiagnosed with ADHD, whereas girls, and children with “quieter” forms of ADHD (such as the inattentive subtype), often go undiagnosed. This is why accurate assessment by a qualified professional is so important.

If your child has a relatively mild form of CHD and has shown otherwise typical development (aside from possible ADHD symptoms), you might first discuss your concerns with your pediatrician. To assess for ADHD, the American Academy of Pediatrics recommends that the healthcare provider take a comprehensive history and gather information about the child’s behavior in different settings, and the best way to do this is through use of standardized behavior rating scales filled out by the child’s parents, teachers, or other caregivers (teens and adults may also complete a self-report form). 

However, if your child has more complex CHD or shows significant developmental delays, a more comprehensive neuropsychological evaluation would be recommended. Specifically, comprehensive evaluation would be recommended for all children and youth with CHD who:

• Required heart surgery in the first few years of life

• Have cyanotic heart disease

• Have a history of mechanical support 

• Have a history of heart failure and/or heart transplantation

• Have other complications or comorbidities (like prematurity, stroke, or seizures)

• Have significant developmental delays or learning problems

• Have a genetic syndrome

Neuropsychological evaluations provide a broad assessment of cognitive and psychological functioning. Your child will be given tests that examine their intellectual functioning, attention, executive functioning, learning, memory, language, and sensorimotor skills, among other areas. The tests typically involve answering questions, solving puzzles, and the like—they do not involve brain imaging, blood work, or other medical tests. 

The reason to seek out a more comprehensive evaluation is that children with complex CHD often have other neurodevelopmental and neurocognitive deficits in addition to ADHD symptoms, and the evaluation can place these difficulties in the context of your child’s medical history. After the evaluation is completed, you will receive a comprehensive report documenting your child’s cognitive strengths and difficulties, as well as extensive recommendations for school, home, and therapeutic intervention if indicated.

To arrange for a neuropsychological evaluation, you can ask your child’s cardiologist for a referral, look into whether your local children’s hospital has providers on staff, or look for a neuropsychologist (or an appropriately-trained pediatric health psychologist if no neuropsychologists are available) in your area.

Intervention & Treatment

Because of their executive deficits, children and youth with ADHD often need additional support from parents, teachers, and other adults to stay organized, remember what they need to do, and complete daily tasks within time expectations. Adult oversight and support is therefore often needed to ensure tasks get done, even for older adolescents. This can go against many parents’ belief that their teen is old enough to take care of these things on their own, but it is important to recognize that performance of these tasks is much more challenging for people with ADHD. Even adults with ADHD may need organizational help from significant others, for example to ensure that they make appointments or that the bills get paid on time.

That said, there are treatments and interventions that can help minimize the impact of these problems on a person’s daily life. Extensive research has shown that there are three treatment approaches that have been shown to be effective for children and youth with ADHD:

• Behavioral therapy (including parent management training)

• Environmental supports and accommodations 

• Medication, especially in combination with behavioral supports

Each of these approaches is discussed in detail below.

Behavioral therapy/behavior management training. Children with ADHD often need a modified parenting approach compared to their peers. They tend to do better with clear rules, predictable consequences, and consistency in parenting across caregivers. They may need help keeping track of time and navigating transitions, and rewards may need to be made more immediate and tangible to better guide their behavior. Behavior management training for parents (and classroom behavior management training for teachers) is a very effective approach that can help families get a better grasp on their child’s behavior and reduce the impact of ADHD symptoms on daily life. Organizational skills training can also be helpful for teens.

For more information on behavioral therapy for ADHD, see here and here.

Environmental supports. ADHD-related school problems are best addressed through development of a comprehensive school plan that targets the behaviors of greatest concern in the classroom. This is typically done through a formal, written plan designed in collaboration with the school. In the U.S., two federal laws govern how children with disabilities like ADHD and CHD receive supports and accommodations in the (public) school environment: Section 504 of the Rehabilitation Act of 1973, and The Individuals with Disabilities Education Act (IDEA). 

• Section 504 is essentially anti-discrimination law designed to decrease barriers to educational opportunity. It applies to anyone with a disability that can affect their access to a free and appropriate public education. If a child qualifies, they will be given a written 504 Plan (parents should receive a copy) that provides classroom accommodations to ensure equal access to educational opportunity. Common accommodations for children with ADHD include preferential seating close to the teacher, extended time for tests and assignments, and organizational assistance. 

• The IDEA governs how states and public agencies provide early intervention, special education, and related services. To qualify for services under IDEA, the child’s disability must affect their educational performance and/or ability to learn and benefit from the general education curriculum. If the child qualifies, the school (in consultation with the family) will develop a written Individualized Education Program (IEP) that specifies learning goals and what the school will do to help student attain them. An IEP may also contain accommodations (such as a behavior intervention plan) similar to those found in a 504 Plan.

Private and parochial schools that do not receive federal funding are not bound by these laws, though many are still happy to work with families to develop an appropriate plan.

504 Plans and IEPs can both be implemented in the general classroom, and do not require the child to be placed in a special setting. By law, the school must place the child in the least restrictive environment possible to ensure adequate education. Of course, some students with more significant developmental or learning disabilities might benefit from part-time or even full-time placement in a resource classroom where they can get more individualized support. 

For more information about school plans for children with ADHD, click here.

Medication. Medication can be life-changing for people with ADHD, reducing the core symptoms and giving them more control over their lives. ADHD medications should not change a person’s personality or make them seem like a “zombie”; if they do, this should be discussed right away with the prescribing physician, who can make changes to the medication or its dose. 

A word about ADHD medication for children with congenital heart disease: Stimulant medications (amphetamine products and methylphenidate) are generally the most effective for treatment of ADHD symptoms, but there has been concern about potential adverse cardiac effects. A recent review article found “no evidence for serious adverse cardiovascular complications in children with known cardiovascular diseases including patients of congenital heart disease who are treated with stimulant medications,” but caution is still required when prescribing these medications for children with CHD. For example, studies suggest that stimulant medications may result in increases in blood pressure and heart rate. Consultation with a cardiologist before starting any ADHD medication is therefore strongly recommended.

For more information about medication for ADHD, click here.

Alternative treatments. Many parents are also interested in alternative approaches to treating ADHD symptoms. In general, there is currently no good scientific evidence that the following approaches work to treat ADHD or executive dysfunction: herbal supplements, homeopathic preparations, neurofeedback, or brain-training programs.

There is some limited evidence that micronutrient supplements such as omega-3 fatty acids might be effective in reducing ADHD symptoms, though more research is needed. Again, consultation with your child’s cardiologist is strongly recommended before starting any supplements to ensure there are no medical contraindications.

Finally, there is evidence that healthy living approaches may help alleviate some ADHD symptoms including executive dysfunction, though their effects are likely small. These generally beneficial approaches include:

• Regular physical activity (keeping in mind any activity limitations your child may have)

• A well-balanced diet of whole, minimally-processed, and nutrient dense foods that is low in added sugars, saturated fats, and sodium 

• Adequate sleep (10-13 hours/night for preschoolers, 9-12 hours/night for grade-school age children, 8-10 hours/night for teens) 

• Practices such as mindfulness and meditation

Summary & Conclusions

Children with CHD have an increased likelihood of having ADHD and executive dysfunction, especially if they have more complex, cyanotic, or palliated disease. Careful evaluation is important to best understand the symptom presentation and the degree to which symptoms might be part of a broader neurodevelopmental disorder. Surveillance of symptoms over time is also crucial, as the picture may change if they experience chronic hypoxemia or heart failure, or undergo cardiac surgery or heart transplant. ADHD interventions are similar for children with and without CHD, and include behavioral therapy and environmental modifications. Medication can also be quite helpful for symptom reduction, but must only be used under the supervision of a cardiologist. When all is said and done, people with ADHD and CHD can be quite successful in their lives, especially when given the appropriate supports.

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Selected References

Berger S. Attention deficit hyperactivity disorder medications in children with heart disease. Curr Opin Pediatr. 2016 Oct;28(5):607-12. doi: 10.1097/MOP.0000000000000388. PMID: 27261563.

Gonzalez VJ, Kimbro RT, Cutitta KE, Shabosky JC, Bilal MF, Penny DJ, Lopez KN. Mental Health Disorders in Children With Congenital Heart Disease. Pediatrics. 2021 Feb;147(2):e20201693. doi: 10.1542/peds.2020-1693. Epub 2021 Jan 4. PMID: 33397689; PMCID: PMC7849200.

Marino BS, Lipkin PH, Newburger JW, Peacock G, Gerdes M, Gaynor JW, Mussatto KA, Uzark K, Goldberg CS, Johnson WH Jr, Li J, Smith SE, Bellinger DC, Mahle WT; American Heart Association Congenital Heart Defects Committee, Council on Cardiovascular Disease in the Young, Council on Cardiovascular Nursing, and Stroke Council. Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation. 2012 Aug 28;126(9):1143-72. doi: 10.1161/CIR.0b013e318265ee8a. Epub 2012 Jul 30. PMID: 22851541.

Shillingford AJ, Glanzman MM, Ittenbach RF, Clancy RR, Gaynor JW, Wernovsky G. Inattention, hyperactivity, and school performance in a population of school-age children with complex congenital heart disease. Pediatrics. 2008 Apr;121(4):e759-67. doi: 10.1542/peds.2007-1066. PMID: 18381503.

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Dr. Katherine Cutitta 

Katherine Cutitta, PhD, is an Assistant Professor at Baylor College of Medicine and serves as the dedicated clinical health psychologist for the Heart Center at Texas Children’s Hospital. She works with families and children with congenital heart disease, as well as adults with congenital heart disease to help improve quality of life and establish heart healthy habits with managing congenital heart disease.

Dr. David Schwartz

Dr. Schwartz is a pediatric neuropsychologist at Texas Children’s Hospital and Associate Professor of Pediatrics at Baylor College of Medicine. He has long worked with children, youth, and young adults with congenital heart disease. Dr. Schwartz currently conducts neuropsychological evaluations of patients seen through the Texas Children’s Hospital Cardiac Developmental Outcomes Program and Heart Transplant Program, and he has completed research on cognitive outcomes in adult survivors of CHD.

ATTENTION DEFICIT HYPERACTIVITY DISORDER IN CHILDREN AND YOUTH WITH CONGENITAL HEART DISEASE- PART I

PART I: What is ADHD and What Does It Look Like in Children with and without CHD

David D. Schwartz PhD ABPP
Neuropsychologist, Texas Children’s Hospital
Associate Professor of Pediatrics, Baylor College of Medicine

Katherine Cutitta PhD 
Psychologist, Texas Children’s Hospital
Assistant Professor of Pediatrics, Baylor College of Medicine

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Background

Children with congenital heart disease (CHD) are more likely to develop Attention Deficit Hyperactivity Disorder (ADHD) than their peers. One recent study at Texas Children’s Hospital found that children with CHD (regardless of severity) were more than twice as likely to develop ADHD compared to all other patients. Other studies have shown that roughly 30% of children with more complex CHD have significant attention problems and/or hyperactive/impulsive behavior. In Part I of this Practitioner’s Post, we describe the core symptoms of ADHD and place them in the context of a broader model of self-regulation and self-control, and review the etiology (causes) of ADHD with a focus on children with CHD. In Part II, we explain what to do if you think your child with CHD also has ADHD, with a focus on evidence-based assessment and effective treatment and management strategies.

PART I: What is ADHD and What Does It Look Like in Children with and without CHD

ADHD is one of the most common neurodevelopmental disorders, affecting roughly 1 in 10 school-age children and adolescents. It is characterized by difficulties with focus and paying attention, hyperactive or restless behavior, and a tendency to act without thinking (impulsivity). People with ADHD often have more difficulty waiting, may struggle to motivate themselves to complete boring or demanding tasks, and can find it challenging to manage their time and stay organized. At the same time, ADHD may be associated with positive characteristics including a high energy level, creativity, and an ability to hyper-focus on tasks perceived to be especially interesting. People with ADHD can be successful in their lives, but to do so they often need support and accommodation.

ADHD is defined based on symptoms—there is no blood test, and it cannot be diagnosed based on a brain scan or other physiological measure. Diagnosis is based on the clinical history (usually taken by a pediatrician or psychologist) and parent and teacher ratings on standard behavioral questionnaires. The clinician will also rule-out alternative explanations for the symptom presentation, and to screen for comorbid conditions such as learning disabilities, behavioral disorders, anxiety, and depressive disorders.

To meet criteria for an ADHD diagnosis, the child has to show symptoms of inattention and/or hyperactive/impulsive behavior for at least 6 months in at least two settings. 

• Children with inattention have problems focusing and paying attention, especially on tasks that they find boring or mentally demanding; they may also get distracted more easily, and have difficulty keeping their mind on one topic. 

• Children with hyperactivity may be overactive and have difficulty staying still; they may act impulsively, without thinking through consequences first; and they may have difficulty with waiting, and choose immediate rewards rather than wait for something potentially better. 

There are three subtypes of ADHD. Children who only show attention problems but are not hyperactive are considered to have the Predominantly Inattentive Presentation of ADHD (which used to be called ADD). Children who are hyperactive without obvious attention problems are considered to have the Predominantly Hyperactive-Impulsive Presentation, and children who have both attention problems and hyperactivity are considered to have the Combined Presentation (this is the most common presentation). It is important to keep in mind that the symptoms by themselves do not warrant a diagnosis of ADHD unless they also cause impairment and have a significant impact on the person’s daily life. 

An additional criterion for diagnosis is that symptoms have to have been present before age 12. However, a kind of secondary or acquired ADHD has been recognized as an outcome of brain injury that can occur at any age. For example, an older adolescent who has a stroke after heart transplant may develop attention problems that meet all of the other criteria for ADHD except for age of onset. While there is some disagreement among professionals about whether a diagnosis of ADHD can be given if symptoms first arose after age 12, many still find a diagnosis useful in these cases, as it can help guide treatment interventions.

For more information about ADHD, click here and here.

ADHD and Executive Functioning

ADHD is associated with deficits in executive functioning, which refers to a set of abilities involved in controlling and regulating your own behavior, either in response to what’s happening in your immediate environment, or in the service of longer-term goals. There are different models of executive functioning, but most include three core abilities:

• Inhibition – stopping an initial response or reaction, which gives you time to think about alternatives. For example, a child may inhibit himself from grabbing a cookie and ask instead, or from blurting out an answer in class and instead raise her hand.

• Working memory – the ability to hold information in mind, often while using it in some way. An example might be figuring out in your head how much change you should get back from a purchase, or weighing different responses to a problem in your mind.

• Shifting (or “mental flexibility”) – the ability to shift attention from one thing to another, change tasks, or  choose an alternative response. Examples include shifting attention from a video game to get washed up for dinner, or changing your approach when something you’re doing isn’t working.

People with ADHD typically show deficits in all three core executive functions. They have difficulty inhibiting behaviors, keeping information in mind without getting distracted, and shifting to a different idea, task, or response (or, alternatively, they may shift too frequently, and have difficulty staying on one topic). As a result, they may also have difficulty with more complex executive functions—such as planning, problem-solving, and decision-making—which rely on these three basic abilities. 

Some researchers have argued that ADHD can be thought of as an executive function deficit disorder, a disorder in self-regulation and self-control. From this point of view, attention problems can be thought of as a deficit in controlling what you pay attention to.

Almost no one has difficulty paying attention to things that are interesting and “capture your attention”—the problem is when you have to make yourself focus on something boring or mentally demanding. A common example is playing video-games.

Parents will often say that their child can’t have ADHD because they can sit and play video-games for hours, but the real problem is that they can’t easily stop playing and shift to a different task—which is why they may have a huge tantrum if made to stop. Similarly, hyperactivity can be thought of as a difficulty with controlling excess movement, and impulsivity with controlling an impulse to say or do something without thinking about it first. 

Difficulty with controlling emotional responses is less often talked about, but may be just as common in ADHD as deficits in attentional and behavioral control. Everyone experiences a surge in emotion when frustrated, excited, or upset, but then a counter-regulatory response usually kicks in to calm us enough to pause and consider how best to respond. Individuals with ADHD often have difficulty with putting the brakes on their emotional reactions. They tend to show poor frustration tolerance, and are more impatient, excitable, and quick to anger. They may also have more difficulty distracting themselves from whatever upset them, and shifting their attention to something else.

Finally, we sometimes also see motivational deficits in children and youth with ADHD. As noted, people with ADHD tend to be more strongly motivated by immediate rewards and have difficulty with deferred gratification, and they find it more difficult to “force themselves” to complete boring or mentally-demanding tasks. This is why using immediate rewards can be especially effective for children with ADHD. It is also why these children may have an especially hard time shifting attention away from video games, which actively engage the reward circuits in the brain. It is important to understand that such motivational deficits are likely brain-based and do not reflect “laziness,” defiance, or a lack of a desire to please others.

How can ADHD affect my child’s daily life?

ADHD can have pervasive effects on a person’s life. School problems are common. Children with ADHD may daydream in class, get distracted easily, and have difficulty staying focused on work (especially mentally-demanding work). Younger children may exhibit disruptive behavior in the classroom (or at home), resulting in a pattern where they are frequently reprimanded and in trouble, which can in turn result in a negative self-image as a “trouble-maker” or “bad kid.” They may also have more difficulty making and keeping friends due to inattention to social cues, immature or overly silly behavior, or difficulty keeping their hands to themselves. 

Adolescents are expected to be responsible for staying on top of their schoolwork, managing their time, and meeting due dates, all of which may be more challenging due to the organizational and executive difficulties that come with ADHD. Teens with ADHD are also more prone to impulsive, risk-taking, and rule-breaking behavior. They are more likely to become parents at an earlier age, have more driving accidents, and may “self medicate” with alcohol or other drugs. Adults with ADHD may have more difficulties sticking with a job and maintaining long-term relationships. 

Children with chronic illnesses like CHD often have to follow medical regimens such as taking medication at a certain time each day, which become their responsibility as they get older. Forgetfulness, disorganization, and inattention can all affect a youth’s ability to stay on top of a medical regimen, making parental oversight especially important.

So what causes ADHD, and how is it linked to CHD?

ADHD is considered a neurodevelopmental disorder, meaning most people are born with it, show symptoms in childhood, and may have delays in their development as a result. ADHD is highly heritable, which means it runs in families due to genes that get passed down from parent to child. If you have ADHD, your child is more likely to have it too. 

Prenatal factors can also play a role in the development of ADHD. For example, fetal exposure to alcohol, nicotine, and other substances increases the risk that the child will have ADHD. In addition, children born prematurely also have a greater risk of ADHD. The basic idea is that, early in life, the developing brain is especially vulnerable to insult and injury, and one of the most common outcomes of early insult is ADHD. 

It is this early vulnerability that places children with CHD at increased risk for ADHD. Congenital heart defects can result in reduced blood flow (ischemia) and reduced oxygen (hypoxemia) to the brain, which can have an effect on how the brain develops prenatally and in early childhood. Children with complex CHD often show immature brain development at birth, with reductions in both cerebral gray and white matter; and these differences are associated with increased risk for neurodevelopmental disability. In fact, children with CHD closely resemble children born prematurely in terms of their neurodevelopmental outcomes.

Cardiac surgeries, especially in the first few years of life, and associated procedures such as being placed on cardiopulmonary bypass or ECMO, can also affect how the brain develops, and add to the risk for ADHD symptoms. Children with cyanotic heart lesions, or with medical co-morbidities, are also at higher risk.

One of the brain regions most vulnerable to early insult are the frontal lobes, which take up about 1/3 of the total surface area of the brain. The largest part of the frontal lobe is the prefrontal cortex, which is the brain region most closely involved in executive functioning. It therefore isn’t surprising that executive dysfunction is among the most common (if not the most common) neurocognitive deficits seen in people with CHD.

Changes in symptoms over time

While most people with ADHD do not “grow out of” the disorder, the symptom presentation can change as children get older. Hyperactive behaviors are most common in younger children, and may moderate over time, especially as the child enters adolescence. Attention problems typically become more evident as children reach grade-school age and expectations for focus and concentration increase. While impulsivity often remains evident throughout development, it may become a different kind of problem in adolescence, as youth begin to have greater freedom from their parents, start to drive, and may be faced with risky choices such as whether to drink alcohol or use other substances, or to engage in unprotected sex. Teens may also begin to have more difficulty with self-esteem, anxiety, and depressed mood. 

Children and youth with CHD may face a different kind of change in ADHD symptoms related to their heart disease. Chronic hypoxemia and heart failure may result in worsening attention over time. Complications of later-occurring heart surgeries, placement of ventricular assist devices, and transplant may also result in a worsening of symptoms. For children and youth with CHD, ADHD symptom surveillance is important, as is periodic re-evaluation every couple of years, or following any new significant cardiac event or surgery.

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Dr. Katherine Cutitta

Katherine Cutitta, PhD, is an Assistant Professor at Baylor College of Medicine and serves as the dedicated clinical health psychologist for the Heart Center at Texas Children’s Hospital. She works with families and children with congenital heart disease, as well as adults with congenital heart disease to help improve quality of life and establish heart healthy habits with managing congenital heart disease.

Dr. David Schwartz

Dr. Schwartz is a pediatric neuropsychologist at Texas Children’s Hospital and Associate Professor of Pediatrics at Baylor College of Medicine. He has long worked with children, youth, and young adults with congenital heart disease. Dr. Schwartz currently conducts neuropsychological evaluations of patients seen through the Texas Children’s Hospital Cardiac Developmental Outcomes Program and Heart Transplant Program, and he has completed research on cognitive outcomes in adult survivors of CHD.

Understanding the ADHD Diagnosis

Mark Beidelman, PsyD 

Pediatric Neuropsychologist at Stanford Children’s Health

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What is ADHD

Attention-deficit/hyperactivity disorder or ADHD is a relatively new diagnosis, but the clusters of symptoms that make up this diagnosis have been identified and categorized under different names dating back to at least 1798 (Crichton 1798). What we now know of as ADHD first appeared in the second edition of the American Psychiatric Association’s Diagnostic and Statistical Manual of Mental Disorders (DSM-II) in 1968. Through the various iterations of DSM-III (1980), DSM-IV (1994), and DSM-5 (2013) there have been subtle changes to the classification of these symptoms as a unified condition. As a professional or as a parent, it’s important to not get overly focused on the diagnostic criteria or the changes over time, but instead to think about what aspects of functioning are impaired in this diagnosis and what steps can be taken to make functional improvements.

 Currently in DSM-5 ADHD is defined as “A persistent pattern of inattention and/or hyperactivity-impulsivity that interferes with functioning or development, as characterized by Inattention and/or Hyperactivity and Impulsivity.”

 Inattention:

• Often fails to give close attention to details or makes careless mistakes in schoolwork, at work, or during other activities

• Often has difficulty sustaining attention in tasks or play activities

• Often does not seem to listen when spoken to directly

• Often does not follow through on instructions and fails to finish schoolwork, chores, or duties in the workplace

• Often has difficulty organizing tasks and activities

• Often avoids, dislikes, or is reluctant to engage in tasks that require sustained mental effort

• Often loses things necessary for tasks or activities

• Is often easily distracted by extraneous stimuli

• Is often forgetful in daily activities

 Hyperactivity and impulsivity:

• Often fidgets with or taps hands or feet or squirms in seat

• Often leaves seat in situations when remaining seated is expected

• Often runs about or climbs in situations where it is inappropriate

• Often unable to play or engage in leisure activities quietly

• Is often “on the go,” acting as if “driven by a motor”

• Often talks excessively

• Often blurts out an answer before a question has been completed

• Often has difficulty waiting his or her turn

• Often interrupts or intrudes on others

 Now, you might be thinking that you have a hard time sustaining attention on tasks, or everyone is reluctant to engage in tasks that require effort…or something like that. This might be true, but the individual symptoms of ADHD are best thought of as part of the grey scale of personal differences, or what makes us who we are. It is only when we see these symptoms clustered in a way that meets a certain severity and frequency threshold and see functional impairments in multiple settings that we consider ADHD.

One more thing about diagnosis before we move on. 

There are three “types” of ADHD, which are:

• Predominantly Inattentive Presentation

• Predominantly Hyperactive/Impulsive Presentation

• Combined Presentation

So, ADD is no more! Now ADD is called ADHD, predominantly inattentive presentation, and I suspect ADHD hyperactive/impulsive presentation and ADHD combined presentation explain themselves (i.e., more hyperactive symptoms for hyperactive/impulsive presentation, and a mixture of inattention and hyperactive/impulsive symptoms for combined presentation).

So, if I have explained the content in the above paragraph well enough, we can now understand you could have attention-deficit/hyperactivity disorder, while simultaneously being the least hyperactive person on the planet (so long as you are inattentive enough). Another consideration is that attention is not an all or nothing concept.

A comment I frequently hear from families is something along the lines of, “While we agree our child has difficulty attending to certain things (e.g., school, chores, books), they can attend or even hyper-focus on things they enjoy (e.g., sports, activities, video games), so how could this be ADHD?” To answer this, it is best to think about ADHD as not a pure deficit of attention, but instead as a difficulty regulating attention effectively. Meaning people with ADHD might have difficulty sustaining attention, ignoring impulses, shifting attention/transitioning appropriately, estimating time, organizing, etc.

ADHD, Executive Functions, and Emotions

Continuing this line of thinking from above, some would argue that ADHD is really a deficit of executive functioning. Executive functioning is a term used to describe an array of cognitive skills that include but are not limited to inhibitory control, working memory, cognitive flexibility, reasoning, problem-solving, planning, etc. My simplified definition of executive functioning is coordinating two mental or cognitive skills to solve one task/problem. Given the breadth of content around executive functions and how those executive functions might play out in daily life, let’s just focus on how attention could impact executive functioning and some of the additional consequences of ADHD. 

 ADHD and executive functioning are somewhat of a blurry line, in fact, some people even consider control of attention and impulse control as part of executive functioning. For our purpose on this much larger topic, let’s just say that difficulties with regulating attention and impulses will make it difficult to successfully coordinate multiple cognitive skills in an organized fashion.

 ADHD and emotions are also complex. Emotion regulation difficulties are also common in individuals with ADHD, but not inherently part of the ADHD diagnosis. Symptoms of anxiety and depression have a higher than chance co-occurrence in people with ADHD.

The reasons for these emotional difficulties are likely multifactorial and could even be explained by different mechanisms in the same person. We will list a few relationships between ADHD and emotion regulation, anxiety, and depression. There can be impulsive behaviors that lead to interpersonal, academic, occupational, or relationships difficulties. Poor attention can make people feel lost or behind at school, work, or activities, which can increase anxiety. Difficulties with academic or work functioning can impact esteem/mood and lead to symptoms of depression. This is not an inclusive list, but it is important to realize that ADHD is not just about attention. A better way to think about ADHD might be to realize how these clusters of systems impact the whole person in academic, interpersonal, occupational, and introspective ways.

 The good news is there are successful evidenced based therapies (behavioral therapy, cognitive behavioral therapy, acceptance and commitment therapy, to name a few), behaviors/techniques (mindfulness practice, meditation, exercise, sufficient sleep), and medications (even with individuals with congenital heart disease) that can make lasting changes on attention, executive functioning, and emotional functioning. 

There is a lot more to unpack about all this, including the connection between ADHD and Congenital Heart Defects.  In the next few articles, this series aims to provide a deeper dive into this connection and what therapies and management strategies are available to parents today. 

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Dr. Mark Beidelman

Dr. Beidelman is a pediatric neuropsychologist at Stanford Children’s Health. He has studied neuropsychological functioning through the lifespan, but currently focuses on pediatric populations. He currently splits his time between various clinics that include: Developmental Behavioral Pediatrics, Single Ventricle Program, Cardiovascular Connective Tissue Disorders Program, Spina Bifida Clinic, Stroke Team, and the Center for Interdisciplinary Brain Sciences Research. He also has a pug.

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School Advocacy: The Missing Link in Cardiac Follow-Up Care

Dr. Cheryl L. Brosig, PhD
Professor of Pediatrics and Chief of Pediatric Psychology and Developmental Medicine at the Medical College of Wisconsin.

Kyle Landry
Educational Achievement Partnership Program Manager, Children’s Wisconsin

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The details are different but the story is always the same – they fixed his heart, why is school so hard? 

Children born with congenital heart defects and other pediatric heart diseases are at risk for neurodevelopmental deficits, delays, and differences due to a variety of factors, including prenatal brain injury, fetal cerebrovascular physiology and oxygen delivery, decreased brain maturity, the effect of cardiac surgery on the brain, and the long term effects of lengthy hospitalizations on development. Through years of research, neuropsychologists have learned, and families have witnessed, that children with heart disease may experience limitations in physical and mental strength, vitality, alertness and often score lower in hand-eye coordination, fine and gross motor skills, social-emotional functioning, and language development than their typically developing peers. Behavior, attention, immaturity, and learning challenges are also much more common among children with heart disease.

Though many children with heart disease will experience typical neurodevelopment early on, it isn’t uncommon for new concerns to arise at different stages from preschool through adulthood as independence, organization, and skill level demands increase.

These neurodevelopmental challenges may affect multiple domains and can result in more substantial academic concerns over time as these deficits generally do not self-correct and these children cannot simply “catch-up” on their own.

While schools have established special education programs and supports, educational teams often lack the medical expertise to identify the unique, often subtle, neurodevelopmental needs of cardiac patients. On the other hand, medical providers often focus on aspects of the patient’s physical health, may lack experience in the complex educational system, and do not typically communicate extensively with schools. To further complicate things, unlike heart disease which is diagnosed and treated by medical specialists, neurodevelopmental deficits may be separately identified by medical and school practitioners, using different metrics and qualification criteria, resulting in different follow-up and missed opportunities for early intervention. In fact, a 2014 study led by the Herma Heart Institute’s own Dr. Cheryl Brosig revealed that medical providers recommended academic support for cardiac patients over 3 times more often than schools were actually providing these services.  

Even with the ongoing support of two highly qualified, well-intended teams, both advocating for the student’s best interest, our children continue to fall through the cracks.

Children’s Wisconsin’s Educational Achievement Partnership Program (formerly known as the School Intervention Program) supports Herma Heart Institute patients by providing care coordination services that extend far beyond the walls of the hospital to maximize quality of life. The EAPP places experienced educators specially trained in complex medical conditions and the related impacts of surgeries, treatments, medication, and complications on cognition, physical health, and neurodevelopment at the center of a coordinated care team to ensure clear and consistent communication among families, medical staff, school staff, and community care partners. Through this collaborative partnership, the EAPP removes the burden of translating technical medical information from families and educates school staff on the child’s unique cardiac anatomy and the related heart-brain-body connection. 

While I could talk about the EAPP all day, here’s what I want you to know: the “secret sauce” is that there are no secrets!  

Through this Sisters by Heart blog series you will learn more about ADHD and Executive Functioning challenges related to CHD and how you can help support your child most effectively. As the series wraps up, I’ll be back to share 5 essential steps that the EAPP uses to educate school staff on the unique heart-brain-body connections and give YOU the tools that the EAPP has used to achieved a 97% success rate getting cardiac patients new or expanded health and education plans that allow our patients to thrive in the classroom setting. You won’t want to miss it!

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Dr. Cheryl Brosig

is a Professor of Pediatrics and the Chief of Pediatric Psychology and Developmental Medicine at the Medical College of Wisconsin.  She serves as the Medical Director of the Cardiac Neurodevelopmental Follow Up Program and the Educational Achievement Partnership Program in the Herma Heart Institute at Children’s Wisconsin.  Her clinical and research interests focus on neurodevelopmental and psychosocial outcomes in children and families affected by pediatric heart disease.

Kyle Landry

is the Educational Achievement Partnership Program Manager at Children’s Wisconsin. Since starting the program in 2015, she has served over 500 children with pediatric heart disease by conducting comprehensive school assessments in the clinical environment and helping school staff implement interventions within the school setting. Landry has a bachelor’s degree in Early Childhood Education (2010) and a master’s in Cultural Foundations of Community Engagement and Education (2018) from the University of Wisconsin - Milwaukee. Landry’s previous teaching experience specializing in youth placed at risk for school failure illuminates her belief that every child should have equal access to quality education.

Sisters By Heart Education Series

It took me completely by surprise and hit me like a ton of bricks the day my son was diagnosed with ADHD, Inattentive Type.  I was so confused.  My son had incredible focus and he wasn’t hyper at all.  Turns out, I had no idea what ADHD was.  Actually, I take that back.  I knew a LOT of things about ADHD, and they were all wrong.  So, I didn’t start my journey of educating myself about ADHD from the bottom.  I was below the bottom.  I had to climb out of my hole of complete misunderstanding and even ignorance.  What struck me the hardest was learning how closely it all ties in with Executive Functioning and how closely that ties in with anxiety and depression.  My son, at the tender age of 8, was beginning to experience those effects as well. The more I learned, the more everything started making a lot of sense.  The more I learned, the more I wished I had been better informed and prepared. 

A recent study revealed that children with complex single ventricle CHD have 7 times higher odds of diagnosis or treatment for anxiety and/or depression.  And further, it has been estimated that 50-75% of children with complex CHD are affected by Neurodevelopmental Disabilities – that’s a big fancy catchall word for ADHD, among others.   I learned we aren’t alone. 

In fact, several of us at Sisters by Heart with school aged children, have found ourselves among these numbers. As a result, we are both humbled and excited to launch the first of many continual educational programs featuring practitioner article series and webinars on key challenge areas faced by Single Ventricle patients and their families.  We are grateful to the providers that have partnered with us so far to make this a reality. 

We hope that our first series; “CHD and ADHD: What You Need to Know,” helps you as much as it has helped us. 

Heart Hugs,
Lacie Patterson
Mom to Dylan (HLHS, Age 9)
Director of Fontan Families

Fontan to Transplant: Building Teams for Transition

By: Kurt R. Schumacher, MD MS, Medical Director of the Pediatric Heart Transplant Program at the University of Michigan Congenital Heart Center at C.S. Mott Children's Hospital

Dr. Schumacher is the medical director of the pediatric heart transplant program at the University of Michigan Congenital Heart Center at C.S. Mott Children’s Hospital.  Throughout his career, Dr. Schumacher’s clinical and research efforts have focused on understanding and improving the outcomes of heart failure and transplantation in children with congenital heart disease with an emphasis on Fontan patients.  He is a leader of ACTION’s Fontan subcommittee aimed at improving the quality of care for all Fontan patients needing heart failure treatment and advance cardiac therapies, and he is a member of multiple national working groups aimed at improving outcomes for all single ventricle patients.   

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The relationship between a single ventricle family and their cardiologist is strong.  From my perspective as a cardiologist, each patient requires a tremendous amount of thought and effort, but each patient can also give me with a wonderful sense of accomplishment.  Through this single ventricle journey, we cardiologists become attached and even bonded to our families. We go through so many events together – hospital admissions, clinic visits, inter-stage monitoring, nerve-wracking surgeries, challenging recoveries, and joyful discharges.  In my experience, the bonds formed during this process are often amazing and powerful. After the Fontan, we get to be there with our families watching children grow up. For a pediatric cardiologist, this is the ultimate reward. The reality is, however, that the path ahead is far from clear.  We now know that growing up as a Fontan is accompanied by new challenges for both families and cardiologists. As much as we don’t like to think about it, a large proportion of Fontan patients will eventually need to be seen by a heart failure specialist, and many will need a heart transplant. Single ventricle congenital heart disease is now the leading indication for pediatric heart transplant evaluation.  Like it or not, the reality is that sometimes pediatric transplant cardiologists are going to be a part of the Fontan patient’s care team.

Unfortunately, the approach of building a Fontan care team that includes a transplant cardiologist is not widespread.  More often, patients and families are “referred for transplant evaluation” and find themselves with a completely new cardiologist who is now overseeing all of their care.  Suddenly, the cardiologist (and team) that has seen this patient through all the clinic visits, surgeries, and hospitalizations is no longer “in-charge” and all new people are making the decisions.  Some patients even need to transition their care to a completely new center. To make matters even more challenging, all of this change occurs at a time when anxiety about patient health is extremely high.  As a pediatric heart failure and transplant specialist, I know from discussions with many patients that this transition can be a jolting and traumatic experience.

Why does this happen?  It probably stems from many causes.  Given the strong bonds they have, longtime cardiologists understandably don’t want to give up care of their patient so they delay their referral.  Similarly, patients and families (appropriately) don’t want to leave their cardiologist, and they may not even understand that heart failure can occur after Fontan or don’t want to acknowledge that possibility.  Finally, in our field, there are no formal recommendations for when to refer a Fontan for heart failure consultation.  General cardiologists are left to decide on their own without guidance about what an appropriate Fontan heart failure consult is.  This is a gap in our field that needs to be filled because a sudden transition in patient care is not the way it has to be.  Early consultation from a heart failure/transplant expert would allow us to build Fontan care teams and eliminate the jolting change in providers.

ACTION (Advanced Cardiac Therapies Improving Outcomes Network) is a collaborative group of pediatric heart failure and transplant cardiologists and cardiac surgeons who are working to improve the quality of care that pediatric heart failure patients receive.  When ACTION surveyed heart failure providers, a large majority wished that Fontan patients were referred earlier than they typically are. When we explored this finding, there was a clear desire for the patient and family, the primary cardiologist, and the heart failure/transplant cardiologist to be a decision-making care team prior to needing a transplant.  Early referral of a Fontan patient for consultation from a heart failure cardiologist would allow the creation of this Fontan advanced care team. The heart failure cardiologist’s perspective and insights, including issues specific to potential future transplant, could be paired with the primary cardiologist and family’s experiences to collaboratively structure medical care plans for the patient.  I strongly believe this could improve outcomes for patients. And should the time ever arrive that heart transplant is indicated, the patient and family wouldn’t suddenly be meeting a completely new team – that relationship is already established.

So how do we make this happen?  ACTION is building a set of Fontan-patient-specific reasons that should prompt consideration of a referral to a pediatric heart failure specialist.  These are based on the experiences and challenges that we have had with Fontan patients undergoing heart transplant. They allow us to meet patients before transplant is urgently needed (or even worse, before a patient becomes too sick for transplant).  It will give us time to treat different types of Fontan failure and insure that a patient is as healthy as possible prior to undergoing transplant.   It is our hope that by clearly stating these reasons, we will improve the timeliness of patient referrals, improve outcomes, allow the formation of a collaborative care teams, and eliminate the abrupt transfer of care that can be so difficult for both patients and providers.  We welcome feedback and insights from patients and families as this effort moves forward. We all share the same goal – the highest possible quality of life for our children.

Helping siblings adjust, cope, and thrive

By: Caitlin Meehan, CCLS, Child Life Specialist at Nemours Cardiac Center Nemours/Alfred I. duPont Hospital for Children

Caitlin Meehan, CCLS

Child Life Specialist

Nemours Cardiac Center

Nemours/Alfred I. duPont Hospital for Children

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When families learn that their child has HLHS, a variety of tasks are set in motion. Echocardiograms are scheduled; surgeries are discussed and planned. Parents research outcomes and what to expect for the future, and meet the lineup of staff who will help them along the way.

A diagnosis of HLHS affects the entire family unit, and often overlooked are the siblings of these newly diagnosed patients. What is the best way to describe HLHS and upcoming surgeries to these healthy siblings? How can we help them cope with the hospitalizations and surgeries that are coming? How can parents manage the unique needs of their child with HLHS, while still attending to their other children?

As a Certified Child Life Specialist in a cardiac center, I meet with families prenatally or shortly after birth to address these questions and many others. Parents often wonder how much information they should give to their other children, how to answer questions, and what to expect along the way. By addressing their needs prior to, during, and after hospitalizations, we can assure siblings of HLHS patients can not only cope with this diagnosis, but thrive in spite of it.

Finding the right words

Since an HLHS diagnosis can be shocking for a family, many parents feel like they should hide this from other children, or simply avoid telling them because they do not know what to say. Children tend to know when adults are hiding something from them though, and an absence of information can leave them feeling deceived or confused. They will likely notice subtle differences in routine or parents’ behavior, and eventually have more obvious questions like why their new brother or sister has yet to come home from the hospital. While it is not necessary to give all of the information possible, and it is certainly acceptable to convey uncertainty, it is essential that parents do not pretend as if nothing is wrong. A child who feels lied to may have trouble trusting his or her parent in the future. When speaking to children about their brother or sister’s diagnosis, it is best to use simple, honest, and concrete language to avoid misconceptions. Many attempts to “sugar coat” the conversation can actually lead to confusion and fear in the children hearing this. For example, avoid using words like “sick” as this may lead to additional fears when someone contracts a cold or virus, and this word tends to imply the patient is contagious. I explain that while growing in mommy’s tummy, little brother or sister’s heart did not grow the way yours and mine did and is not working the same. I explain that the heart is a very important part of the body, and he/she will need doctors to fix it before coming home and keep fixing it as he/she grows up. Older kids may worry and should be assured that their brother or sister will not be scared or in pain thanks to very special medicines.  

Helping siblings cope with separation

One of the biggest concerns parents have is the potential separation from the non-hospitalized child. Often this child is, by necessity, left at home and cared for by other family members. It can be difficult for them to be away from parents and in unfamiliar settings. Parents can help by reassuring them that they are loved, they are safe, and they have done nothing wrong. Young children think very egocentrically and for this reason, they tend to think their thoughts or actions have an effect on how the world functions. Siblings may feel like they caused their brother or sister’s heart condition or that their parents are sad or scared because of something they did. In my interactions with siblings of new babies with HLHS and other congenital heart defects, I have heard a variety of misconceptions. One little girl thought her brother had a heart defect because she hugged her mom too tight while pregnant. Another boy thought it was because he had a cold and was around his pregnant mom. When we inform kids of what is going on we are also informing them of what is not, and that they, and no one, is to blame for this diagnosis.

Another way to help the non-hospitalized child is by helping them feel connected to their sibling and parents while they are away. For various reasons, visiting might be unfeasible or limited, so using technology and different activities can help bridge the distance. Routine phone calls or video chats such as before school or at bedtime may provide relief during what are typically difficult transitions. Social stories and homemade picture books use pictures to illustrate information such as a sequence of events, what is going to happen, and why. These can help younger children or those with special needs know where parents are, and more importantly, that they will come back. Siblings, especially those who are older, love taking on a caring role, so involving them in care and offering opportunities for control may help them cope. If the hospitalization is planned, brothers or sisters can help pack, can select favorite blankets or toys, or even decorations for the room so a little piece of them is in the hospital with the family. Likewise, having a keepsake from a parent or the hospitalized child can help provide comfort and help siblings cope with separation. When siblings are able to visit, I offer opportunities to make family portraits with hand/footprints, take family pictures, or provide scrapbooking items so siblings have something to take home. This is also as a way to document this special time.

Preparing siblings to visit the hospital

The topic parent ask me about most often are siblings visiting an intensive care unit or hospital in general. Parents do not want to scare or upset children by exposing them to breathing tubes, central lines, ventilators, etc. My advice is to follow the lead of your child when considering a visit. Do you have a fearful, anxious child?  Do new situations or surroundings tend to frighten or overwhelm him/her? Or, is this child a little calmer and logical, adapting well to change? Is he or she persistent in asking to visit? Ask yourself, are the fears of bringing in the other child more related to your own fears, or what this child has expressed? Most children’s hospitals have personnel and resources to help prepare siblings for hospital visits, as well as educate, support, and occupy them once they are there. The medical team can put you in contact with a Child Life Specialist or Social Worker who is trained specifically to help your other children understand, know what to expect, and feel comfortable in this far-from-normal environment. As you know, children have very creative imaginations and for this reason, the fear of the unknown is likely more intense than a specific machine or tube. In my experience, most siblings are picturing circumstances far worse than reality, and when properly prepared for even a brief visit, we can put their minds at ease. This is certainly not the case across the board, and you know best if a visit is too much for your child. However, know that there are many resources to help facilitate this, and often it can bring many benefits to the non-hospitalized child.  

Attending to the needs of siblings

Both in the hospital and back at home, it can be difficult to divide your time and energy among your children, especially when your child with HLHS requires so much attention. It is common for healthy siblings to exhibit attention seeking behaviors as they navigate these waters. Regression, jealousy, sleep troubles, and difficulty with separation are normal reactions and should subside with time on their own. If over time, this escalates to serious behaviors such as violence, excessive anger, or depression, a visit to a mental health care professional can offer more focused treatment. Formal and informal support groups for siblings and families can be helpful for some children, as they offer a space to safely share feelings and connect them with peers in similar circumstances. When possible, provide dedicated one-on-one time to the healthy sibling. This may not be easy for many families depending on circumstances, but even brief check ins where this child is the focus will have great benefits. This can be as simple as a special dinner or a walk in the park. The goal of these is not to distract your other children or cheer them up, but rather to make them feel special and allow them to express feelings, worries, and have their concerns validated if need be.

HLHS affects the entire family; this is unavoidable. However, with age appropriate explanations, inclusive activities, and special attention, siblings can grow into resilient and well-adjusted individuals. In fact, children whose siblings have a chronic condition such as HLHS have the opportunity to show more independence and maturity, and even become more compassionate and accepting teenagers and adults.  

*Parents have granted written permission for use of all photos.

Practitioner's Perspective: Family Centered Care

by Michael V. DiMaria, M.D.
Children's Hospital Colorado

Family centered rounds have been introduced in many children’s hospitals for several reasons, but one of the primary motives is that families like to be involved in the health care decisions of their children. Family centered rounds are also a manifestation of a change in the culture of medicine, which has occurred relatively quickly. The way we as physicians practice medicine has shifted from what was a more paternalistic model to a cooperative one; this is addressed very explicitly in medical school curricula: we are in the era of ‘patient autonomy In pediatrics this translates into ‘family autonomy.’ The consequence of the rapid change is that both health care provider and patients can be unfamiliar with the process, and unsure of how to make best use of this opportunity.

To understand what this means for families, let’s start by talking about what to expect on morning rounds. First thing in the morning, after the incoming team of providers has gotten up to speed on developments from the overnight shift, the group gathers at each patient’s bedside in turn. The goal will be to review the events of the last day, discuss how the patient is doing, and come up with the plan for the next day. This is an important time for everyone on the medical team and the family to get on the same page. Often times, especially at a teaching hospital, a large crowd will be present. In order to make things flow more quickly and easily, the attending may ask that any questions or concerns be raised at the end of the presentation; it is difficult for the team to stay focused and efficient if there are frequent side conversations.

Next, the players: the patient’s nurse will be at the bedside, advocating for the family, bringing up unresolved issues, and clarifying his role in the plan. A resident or fellow will ‘present’ the patient to the group, meaning that she will be responsible for knowing all the details about the patient and summarizing them efficiently to the group. To briefly explain how she came to be in this position, after graduating from medical school and receiving an M.D., she did a 1 year internship, then began a 2-5 year residency; after residency, she may do an additional 3-4 years of training, called a ‘fellowship’ to sub-specialize. After finishing all of her training, a doctor becomes an ‘attending physician’ – the person in charge of the team. The attending will be listening, teaching, and guiding the trainee through the process of assembling the data and figuring out what to do next. Various other people may be present, including pharmacists, nutritionists, nursing leadership, physical therapists, etc.

As alluded to above, rounds will consist of a brief description of the day’s events, a review of the physical exam, then a bunch of numbers (lab values, fluid intake and output, ventilator settings, etc.). At the end of all of this comes the assessment and plan. It goes without saying that during the assessment the presenter verbalizes their impression of how the patient is doing. The plan is usually presented in parts, according to ‘organ systems’ (all of the respiratory issues are grouped together, as are all of the cardiovascular problems, etc). This will sound something like, “From a respiratory standpoint, I think we can try to decrease the oxygen from 2 to 0.5 liters per minute. From a nutritional standpoint, I think that Sam can try to eat a solid diet today…” This will continue until all of the active medical issues have been addressed.

When patients and care providers first encounter family centered rounds, some may find it uncomfortable to have conversations about sensitive topics in the presence of a large group. Of course, there should always be the option for the family to have a more private conversation with only the essential members of the team, should there be concerns about privacy. From the resident’s perspective, my colleagues and I were initially very nervous about presenting a patient history and physical in front of the patient’s family as a resident. Aside from being a little shy, I think I was nervous that I would get something wrong and that the family would correct me in front of the boss. I quickly got over this fear, when it became obvious that the common goal was historical accuracy.

There are several points during the process when the family’s input will be especially helpful. It goes without saying that nobody knows a child better than the primary caregivers, and so who better to be the definitive source of historical information? Because team members may come and go twice a day (at the end of a 12 hour shift), none will have the perspective of the family, who will have been with the patient continuously. Being able to give the team an idea of the overall trend of the patient’s symptoms can be very helpful. Similarly, it can be difficult for people who don’t know the child to tell what sorts of behaviors are personality-related and which are indicators that things are not quite right.

The goal of this process is to get the patient home, feeling well, and able to participate in all of the things that go into being a healthy, happy kid. More often than not, this means that the family will need to continue some interventions in the home. So, part of having a child in the hospital is learning about the disease and the treatments. Being an informed caretaker will better enable the family members to identify when problems occur at home, and what to do about them. Some measure of our success as care providers is whether or not the family feels comfortable once they are discharged. Our hope is that the shared knowledge and unified goals of families and care providers results in a close working relationship that develops in this setting of family centered care.Thank you, Dr. DiMaria, for lending your insights and expertise to our HLHS families!

Thank you, Dr. DiMaria, for lending your insights and expertise to our HLHS families!

U.S. News & World Report Rankings Explained

By: Dr. Jeffrey Anderson, Pediatric Cardiologist at Cincinnati Children's Hospital Medical Center (CCHMC)

Dr. Anderson has been a pediatric cardiologist in the Heart Institute at Cincinnati Children's Hospital Medical Center (CCHMC) since 2009.  He is a graduate of the University of Utah School of Medicine and recently completed a Masters in Business Administration at the Ross School of Business at the University of Michigan. Dr. Anderson completed his pediatrics residency at the University of North Carolina Children’s Hospital before coming to CCHMC, where he finished his training in Pediatric Cardiology and advanced training in Pediatric Electrophysiology. He currently leads quality improvement and value efforts within the Heart Institute as co-director of the Heart Institute Safety, Quality and Value program.  Nationally, Dr. Anderson holds several leadership positions including roles with the National Pediatric Cardiology Quality Improvement Collaborative and the American College of Cardiology.

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Each spring the U.S. News & World Report releases their rankings of the best medical centers and clinical programs in the United States.  The purpose of these rankings, as noted by the U.S. News & World Report is to “identify the best medical centers in various specialties for the most difficult patients”.  Because the methods used by the U.S. News & World Report are complex, understanding and interpreting the rankings can be difficult for patients and families.  This post is meant to help families understand some of the detail that goes into these rankings and give some guidance to interpreting them as you make decisions about your child’s care.

History of the U.S. News & World Report rankings

The following is taken directly from the 2015-2016 US News & World Methodology Report (http://www.usnews.com/pubfiles/2014_BCH_methodology_report.pdf).  It is important to note that the U.S. News & World Report ranking for Pediatrics initially was entirely based on a reputational survey of physicians.  Between 2007 and 2015 the reliance on reputation has decreased and the report has been more and more heavily based on outcomes, providing evidence-based processes of care, and structural (e.g. provider certification, presence of  specific programs) elements.  In 2015, the Pediatric subspecialty rankings still included a reputational survey, but this survey was only weighted at 16.7% of the total score.  While the U.S. News & World rankings are still not perfect, each year additional outcomes measures have been added and the overall methodology continues to improve.

In 1990, U.S. News & World Report began publishing what was then called America’s Best Hospitals. The intent was to identify the best medical centers in various specialties for the most difficult patients − those whose illnesses pose unusual challenges because of underlying conditions, procedure difficulty or other medical issues that add significant risk.

The 2014-15 rankings mark the 25th year of annual publication. The focus on identifying top sources of care for the most difficult patients remains the same.

Pediatrics was among the original specialties in which hospitals were ranked, but until 2007 the pediatric rankings were based entirely on a reputational survey of physicians. Still, hard data to inform the rankings remained absent. Such data are critical because young patients present special challenges. Their small size relative to adults complicates every facet of care, from intubation to drug dosages; they are more vulnerable to infection; they depend on adults to manage and administer their medications, and they are treated for congenital diseases such as spina bifida and cystic fibrosis.

In the absence of databases for pediatrics comparable to MedPAR for Medicare recipients, U.S. News resolved to collect data directly from children’s hospitals. The first rankings that incorporated such data were published in 2007. Those rankings listed the top 30 children’s centers only in General Pediatrics. Data collection was subsequently broadened and deepened, and Best Children’s Hospitals now ranks the top 50 centers in 10 specialties: Cancer, Cardiology & Heart Surgery, Diabetes & Endocrinology, Gastroenterology & GI Surgery, Neonatology, Nephrology, Neurology & Neurosurgery, Orthopedics, Pulmonology and Urology.

The survey uses the framework developed in the 1960’s by Avedis Donabedian, conceptually defining the quality of healthcare using a structure, process and outcome framework. This framework is at the heart of many healthcare quality improvement initiatives and is based on the interrelationship of three key elements:

1. Structure: Indirect quality-of-care measures related to a physical setting and resources.  Examples include staffing ratios, certifications, and patient volume.

2. Process: Measures that evaluate the method or process by which care is delivered, including both technical and interpersonal components. Examples include providing evidence-based care such as microalbumin screening for children with diabetes, and timely care delivery such as the timing of antibiotic administration for immunocompromised children with fever. Also included in this section is reputation with pediatric specialists.

3. Outcomes: Outcome elements describe valued results related to lengthening life, relieving pain, reducing disabilities, and  optimal performance on disease-specific measures. Examples include bloodstream infections, survival after organ transplantation, and survival after complex heart surgeries.

Where does the information come from that determines the U.S. News & World Report rankings for Cardiology and who decides what questions to ask?

Information that is used to determine U.S. News & World Report rankings comes from two sources: 1) a survey that is completed and submitted by participating hospitals s and 2) a reputational survey that is sent to pediatric cardiologists and pediatric cardiac surgeon.   Each year, programs are asked to submit data that are used to determine the rankings

• Outcomes at the program (33% of the score)

  • Surgical mortality for various levels of complexity (STAT 1-5).*

  • Survival to first birthday after Norwood or hybrid Stage I palliation.*

  • One and three year survival after heart transplantation.

  • Prevention of ICU infections and pressure ulcers.

• Processes at the program (16.7% of the score)

  • Does the program’s hospital use appropriate infection prevention measures?

  • Does the program formally monitor for surgical site infections?

  • Does the program have a single ventricle interstage monitoring program?

  • Does the program refer complex congenital heart patients for neurodevelopmental evaluation and intervention?

  • Does the program participate in known safety control measures?

  • Does the program participate in national registry and improvement collaboratives (NPC-QIC, IMPACT, STS, PHN)?

• Structure of the program (33% of the score)

  • What is the surgical volume of the program for various levels of complex surgery (STAT 2-5)?*

  • What is the catheter procedural volume of the program?

  • Does the program have a cardiology and cardiac surgery fellowship?

  • Does the program have a cardiac transplant program?

  • Does the program have full-time subspecialists available (cardiac intensivists, cardiac anesthesiologists, adult congenital heart specialists?

  • Does the program have adequate nursing coverage?

  • Is the program committed to quality improvement and clinical research?

  • Is the program committed to engaging families?

* It is important to note that the surgical volume and outcome measures cover a four year period and lag for one year in the U.S. News & World Report rankings.  For example, in the 2015 report surgical data from calendar year 2010-2013 was used to determine surgical volume and outcomes.

• Reputation of the program (16.7%)

  • Each year a survey is sent to practicing pediatric cardiologists and pediatric cardiac surgeons.  This survey asks participants to list 10 U.S. hospitals that they believe “provide the best care in Pediatric Cardiology for patients who have the most challenging conditions or who need particularly difficult procedures”.

  • The average of the previous four years of responses is used to score this part of the ranking.  For example, in the 2015 survey the responses from 2012-2015 were used to determine the reputation score.

A question raised over the years has been the absence of patient and family experience scores as part of the rankings. This is obviously an important component of quality with patient/family responses providing a well-rounded and different perspective from which to inform improvement. Nationally standardized patient experience tools do exist for Pediatrics. Despite this, the tools are not broadly implemented across all reporting hospitals. Because of this variation, the ability to use these scores as part of a ranking methodology is not possible. Currently, hospitals are free to select the tool and method that best meets their informational and improvement needs. As long as this situation remains and hospitals are not required to report using a specific instrument, the use of patient and family experience performance data will not work for the rankings.

Continuous Survey Improvement

Each year, The U.S. News & World Report organizes a committee of pediatric specialists who review survey content and measures and make recommendations for improvement. This process helps ensure cross-hospital representation and involves content experts in the deliberations around measure selection and definitions. Although core outcomes that are nationally reported elsewhere (i.e. STAT survival) remain on the survey from year to year, changes that occur through this process can make year to year comparisons somewhat difficult. The makeup of the committee determining these questions not released by U.S. News & World Report.  Each year, clinical programs are asked for input to improve the questions and that input may or may not be used to make changes the following year.

So how do I interpret and use these rankings?

First, there are problems with any system that attempts to compare clinical programs and rank them, the U.S. News & World Report is no exception.  However, the U.S. News & World Report has made efforts over the last several years to place more of a focus on outcomes than on reputation.   In addition, it is important that we have some way for parents and families to make informed decisions about programs and currently, the U.S. News & World Report provides a platform for this comparison. 

One of the challenges with the U.S. News & World Report is the fact that much of the data, especially data about processes and structure of the programs, are self-reported.  While the U.S. News & World Report does have the right to audit programs to determine the validity of their data, this is not a common occurrence.  Another consideration is the lag associated with surgical volume and outcome data.  Because the rankings use data from a four-year period ending the year prior, changes and improvement in the surgical program will not be reflected through the data for quite some time.

Despite the challenges described, the U.S. News & World Report rankings is a great way to begin the conversation about quality. After all, getting started is often the most difficult step. From that point, continued momentum for improvement around common measures that matter to patients and families can follow.  If nationally standardized quality measures for a specific specialty or condition exist, then it is important that these measures be reflected in the survey. Viewed from this perspective, the survey becomes a way to both reinforce and make visible,  quality measures that truly matter and make a difference in the lives’ of patients and families.  Above all, probably the most important thing when using the U.S. News & World Report to make decisions about your child’s care, is that it serves as an important way to begin and continue the conversation about quality. From there, discussions with your cardiologist or cardiac surgeon about the structure and outcomes of the program at their center can continue, and then segue to a transparent conversation about program outcomes and efforts in place to continually improve.

Parents as Partners…. The long journey to true transparency.

By: Bambi Alexander-Banys, Nurse Practitioner

Bambi Alexander-Banys worked in pediatric psychiatric emergency and the neonatal intensive care unit before graduating with her Masters of Science in Nursing from Boston College.  Commited to education as well as practice, Ms. Alexander-Banys began teaching in 2001 and maintained an adjunct faculty position for over ten years. She provided outpatient care to disadvantaged youth and owned her own concierge practice in Silicon Valley before joining the inpatient cardiology team at Lucile Packard Children's Hospital (LPCH) at Stanford where, in addition to providing inpatient clinical care, she manages the single ventricle Interstage Home Monitoring Program.

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‘Parents as Partners’ is a smart phrase uttered by a wise and insightful parent following our transparency workgroup retreat at the recent National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) Learning Session.  At first glance I knew that was exactly the right phrase to describe what we are striving for in our efforts to improve transparency.  Just as in other kinds of partnerships, each individual enters the relationship with a slightly different history, varied communication style, and sometimes, even trust issues from prior failed partnerships.  So, regardless of everyone’s shared goal, and strong desire to achieve that goal, progress can be slow and it always requires a lot of work.

I say this as a means not of telling others to be patient and ‘keep on keeping on’, but rather to remind myself.  You see, I am not, by nature, a patient person.  I’m more of an action kind of gal; see a problem, identify the solution, implement it, move on.  So, when the solution seems so obvious – share openly without reservation or hesitation – it is hard for me to work through a slow and methodical process.  But just like I cannot move an individual patient, family, or even my own children or husband, to a decision or through a process faster than they are ready for given their own life experiences, perspectives and communication styles, I cannot bulldoze fifty plus surgical centers and all of their associated components through this process.  What seems obvious and easy to me may seem terrifying to someone else.  That terror is very likely grounded in something legitimate for that individual, thus the need for continued patience and understanding.

How does one, especially one who is impatient like me, help move others forward?  While I can’t push anyone else, I can show it is safe and hopefully in the process help them inch their own way closer.  That was the goal of the Transparency Pilot at the NPC-QIC Learning Session and why it was essential to me that my center not only participate but especially that we embrace the opportunity to show that it is OK to talk about the things you aren’t great at.  So, there I stood, the first to speak, in front of over 200 hundred people talking about a process we struggle with and why we’ve not been successful in efforts to record the requisite data at clinic visits.  I didn’t feel compelled to qualify it by explaining all the things we do well, or counter it with the outcomes we are proud of.  I just put the data out there, acknowledged we don’t do that piece well, with language that was intentionally highlighting this as a ‘negative’ disclosure.  It was painless.

Even though most of what was shared in the rest of the session reflected well on the centers and thus was ‘low risk’, the pilot was, in my mind, a huge success.  I suspect people cared little about the data itself and whether it was ‘good’ or ‘bad’.  Breaking the ice and putting it out there, without any negative response or discomfort, is what really mattered.  It was the first step in building trust and bringing a diverse group of partners closer together.  It was also the easiest step.

As we move further down the path, asking more centers to share and to share data that is more likely to make people uncomfortable, we will necessarily need to continue to build a framework that supports and encourages a sense of safety.  Much like business partners delineate terms in an actual contract, not because they assume the worst of one another or else they would not be partners, but because you cannot always predict the future or assume shared opinions on managing unforeseen variables, we might need to establish more a formal structure and some agreements initially.  More steps.  More patience.

If all of this patience is a challenge for me and other clinicians, I cannot imagine how difficult it is for parents who justifiably argue that this is information that they are entitled to and NEED in order to advocate for their families and better support their centers.  Should it be this difficult?  No.  Should it take this long?  No.  Should it require this much patience and persistence?  No.  Yet, it is and it does.  We don’t live in our ideal world, we are still making it.  And if we are to be successful in this undertaking, in building this partnership, we must be patient with the process and each other.  I promise to keep trying and I hope you will too

Photo from the Spring 2015 NPCQICLS (left to right): Holly Bauser-Heaton (Postdoctoral Medical Fellow, Cardiology), Jennie Briend (Parent), Gail Wright (Clinical Associate Professor, Pediatrics - Cardiology), Bambi Alexander-Bayns (Nurse Pracitioner)

A Collaborative and Holistic Approach for Children with Heart Disease

By: The Heart Program & Brain Institue at Nicklaus Childrens Hospital

For over 25 years, Dr. Gil Wernovsky has focused on optimizing long-term functional and developmental outcomes for children with congenital heart disease. He is truly a pioneer and ongoing leader in this area. 

Gil’s career has exemplified excellence in multiple domains including: pediatric cardiology, cardiac intensive care, and administration. Currently he holds the position of Medical Director of Patient- and Family-Centered Care at Miami Children’s Hospital. In addition to this innovative position, Dr. Wernovsky continues to provide attending services in the cardiac intensive care unit, hold outpatient clinics, serve on numerous scientific advisory boards, and provide mentorship to students, fellows and junior faculty. 

Dr. Wernovsky has done extensive research on the long-term outcomes of congenital heart disease, particularly transposition of the great arteries and single ventricle heart disease. He has published over 250 peer-reviewed manuscripts, editorials and chapters, in addition to editing 8 books. 

Dr. Wernovsky is a long-time supporter of Sisters by Heart and our mission to support and empower HLHS families. We greatly admire his dedication not just to the CHD population, but to their families as well. 

Information from the NeuroCardiac and Development Program at Nicklaus Children's Hospital, Miami, Fl
Presented jointly by The Heart Program & Brain Institue

www.nicklauschildrens.org

Gil Wernovsky, MD - Medical Director, Patient- and Family- Centered Care, The Heart Program at Nicklaus Children’s Hospital

Nancy Dobrolet, MD - Co-Director, NeuroCardiac and Development Program, The Heart Program at Nicklaus Children’s Hospital

Ann Hyslop, MD - Co-Director, NeuroCardiac and Development Program, The Brain Institute at Nicklaus Children’s Hospital

Anne Schmelzer, BA, BSN, RN - Nurse Coordinator, NeuroCardiac Development Program, The Heart Program at Nicklaus Children’s Hospital

Background

Children with complex congenital heart defects (CHD) are now surviving neonatal and infant surgery with a frequency thought to be impossible only a generation ago.  While advances in medicine and surgery have allowed the ability to “mend” children born with CHD, the increasing number of survivors has created a growing population of children in our primary and secondary school systems.

In the United States, over 35,000 infants are born each year with CHD, and more than a third of these infants will undergo surgical interventions in the first year of life.  It is estimated that there are more than one million adults now living in the US with a variety of congenital heart defects.  This figure includes over 150,000 adults living with “complex CHD (cCHD)” - CHD severe enough to require surgery in the first months of life.  Due to improved survival rates, there are even larger numbers of school-age children with cCHD.  As initial survival has increased significantly, greater attention has been directed toward understanding the long-term behavioral challenges, academic performance and functional outcomes of this growing group of children and young adults.

Much of the information I will summarize below is very new; unknown to many doctors, nurses and families 5-15 years ago.  Many centers are actively pursuing research in this area, which can be broken down into four main areas:

1. What are the types of difficulties that children with cCHD have, and how frequently do they occur?

2. What are the causes of these difficulties?

3. What are the best treatments for these problems?

4. How can we decrease the frequency of these problems in the future?

What are the types of difficulties that children with complex CHD have, and how frequently do they occur?

As a group, children with cCHD have a higher likelihood of academic, behavioral, coordination and social problems compared to children without CHD.  This does not mean that all children with cCHD have these difficulties (in fact, approximately ½ have normal development in every respect), but the number of children is significantly higher than that seen in the general population. These problems seem to be more prevalent in children with cCHD compared to less severe forms of CHD that either don't require surgery, can be repaired during a heart catheterization (for example, an atrial septal defect or patent ductus arteriosus), or don't require surgery until later childhood.

In infancy, problems that are more prevalent in babies with cCHD include feeding difficulties (perhaps in half of all children requiring neonatal heart surgery) and delays in reaching some motor milestones such as rolling over, crawling or walking.  Most newborns achieve full feedings by mouth shortly after discharge from the hospital, but some require supplemental feedings through a feeding tube into later infancy and beyond.  While delays in motor skills are extremely common, most milestones are only delayed by a few months. Many hospitals now recommend speech and physical therapy consultations after newborn heart surgery for evaluation and treatment recommendations.

In preschool children with cCHD, there is a growing recognition of delays in certain elements of speech and language. To greatly oversimplify, speech and language can be broken down into two components: receptive language (the child hears and understands words), and expressive language (getting the words out with proper grammar and pronunciation).  In most children with cCHD, receptive language is normal: if you say “point to the apple”, the child will point to the apple.  However, expressive language is delayed: when you point to an apple and say “what is that?”, even though the child knows it's an apple, he or she can't seem to “find the word”.

In addition to delays in expressive language, some preschool children with cCHD (probably about 1/4) have ongoing difficulties with motor skills, including large motor (clumsiness) and fine motor (drawing, cutting) delays.  In most children, these problems improve by the time they enter school.

As children with cCHD enter primary and secondary school in larger numbers, there is a growing recognition of a combination of challenges that may combine to cause academic and social problems.  Attention deficit/hyperactivity disorder (ADHD) is a term that may be over applied in our current society, and is an extremely complicated combination of learning and behavioral interactions.  Again to greatly oversimplify, the main components are impulsiveness, easy distractibility and hyperactivity.  As we look more carefully at children with cCHD, various aspects of ADHD occur in perhaps one third to one half of children, which is perhaps two to three times higher than that seen in the general population.  It is not clear if continued frustrations with speech and fine motor (handwriting) control are related to the higher incidence of ADHD—in other words, which is the chicken and which is the egg?

Two related issues are now also being seen in larger numbers of school age children with cCHD: problems with visual-motor integration and executive planning.  Visual-motor integration is the ability to coordinate thoughts and images into action.  In adults, for example, driving a car represents highly complex visual motor integration. In school-age children, however, learning handwriting represents a particular challenge: seeing handwriting on the board, knowing that it's a particular letter, and getting the hand to make the letter can be very frustrating to an otherwise bright child.  As you can see, handwriting is a common theme, and many parents have told us that their child's handwriting is “one of the worst in the class”.  Executive planning is a feature that allows us to plan out a course of action.  For example, first I will get dressed, then I will go eat breakfast, then I will pack my bag, and then head off to school.  As the number of tasks increases, it becomes increasingly difficult to coordinate and/or remember.  Some children with cCHD have a particular problem in this area, but the exact frequency of this problem is currently unknown.  Finally, there are no studies as of yet to determine whether ‘higher order’ intellectual functions (such as complex mathematics or learning a second language), or artistic talents (such as painting or music) will be affected in our children with cCHD.

What are the causes of these problems?

Although it is tempting to point at “one” feature as the cause of the problems I've outlined above, that would be a terrible oversimplification. We are only now beginning to recognize that the effects of cCHD on the brain of children with CHD are multiple, and cumulative over the early years of development.

Following conception, the preliminary portions of the brain form at exactly the same time that the heart forms, in the first trimester.  In most cases, whatever caused the CHD has left its mark on the heart by the end of the first trimester; in most cases cCHD is essentially determined by the end of the 8th week of gestation.  In contrast, the brain continues to develop and mature throughout pregnancy.  We are just beginning to learn that the abnormal circulation caused by cCHD in the fetus is responsible for brain abnormalities present at birth.  It is now recognized that congenital heart disease and “congenital brain disease” go together in many children.  However, it is impossible to determine which is the chicken and which is the egg.  Does the same factor or factors that cause CHD also cause brain abnormalities as well?  Is the brain “wired” the same way in children with CHD compared to normal?  Does the abnormal fetal circulation put an abnormal fetal brain at greater risk?  These are questions that are only now being investigated, as technologies such as fetal and newborn magnetic resonance imaging (MRI) become more routinely available.  Recent studies suggest that the brain is either structurally or functionally abnormal at birth in up to 25% of newborns with cCHD.  The brain of many children appears ‘immature’, several studies have shown that the brain of a full term infant with cCHD has the complexity (or “maturity”) of the brain of a 35-36 week gestation infant without cCHD.  Importantly, the incidence of brain abnormalities at birth in children with less complex forms of CHD is considerably less than described for cCHD.

In cCHD, so many things happen so quickly to the newborn, that it is impossible to sort out the relative contribution of many of them to long-term outcomes.  These factors may include, but are not limited to:

• a low oxygen level after birth

• the effects of the heart lung machine (cardiopulmonary bypass) during surgery

• the cumulative effects of anestetics and sedatives

• the effects of abnormal function of the heart before and/or after surgery

• seizures and/or stroke that may occur before, during or after surgery

• nutritional deficiencies

• the combined effects of a long stay in the intensive care unit and hospital

• the combined effects of acute and post-traumatic stress on the family, with secondary effects to the child

In past research, much attention was paid to the details of the operation.  How long was the operation, how low was the regulated body temperature during surgery (hypothermia), did the circulation need to be stopped completely (hypothermic circulatory arrest) during the most delicate part of surgery?  While these factors are certainly important to the brain and later development, we are beginning to realize that many other factors, both before and after surgery, play an even larger role.  Recent work suggests that the effects of surgery, the heart lung machine and anesthesia contribute only a small amount to later development, explaining less than 10% of the variability in performance that we see in our children.

Nonetheless, the amount of time a patient spends on the heart-lung machine during the operation is a research topic of considerable interest, in particular, the time spent in "hypothermic circulatory arrest".  While it is beyond the scope of this summary to go into all of the details, the majority of studies have shown little to no effects for short periods of cardiopulmonary bypass and/or circulatory arrest.

There is general agreement that shorter periods of both cardiopulmonary bypass and hypothermic circulatory arrest are preferable to longer periods, exactly how long and in what combination continues to be a subject of research and controversy.

Importantly, newer recent studies are showing that longer overall stays in the intensive care unit and hospital as a newborn are associated with worse school performance years later.  This most likely reflects the cumulative effects of the CHD and its treatment.  Research is very active in this area, as is research in the long-term effects of acute stress and longer-term post-traumatic stress of the parents on later development in the child.  For many parents of school age children with cCHD, the early infancy months may seem like a blur, just getting by day-to-day.  The conscious (or sub-conscious) fear of a child’s upcoming surgery, an unexpected medical problem or an unknown life-expectancy may play subtle roles in child rearing, limit setting, behavioral control, promoting independence and goal-setting, with secondary effects on later school performance and/or social development in some children.

What are the best treatments for these problems?

Unfortunately, the research into the treatment of the problems I've outlined above is still in its infancy.  It has only been in recent years that the increased incidence of hyperactivity, school and behavioral difficulties has been recognized.  Prior to that time, there were simply not enough children old enough who survived with different forms of cCHD for this to be fully recognized.  Increasingly, physicians who care for young children with CHD suggest formal developmental testing in infancy and the preschool years to identify potential problems and institute therapies such as speech therapy, occupational and physical therapy, and to recommend changes in learning environments that facilitate learning in children with different strengths and challenges.  The American Heart Association has recently published guidelines in this area.

In our opinion, there is insufficient data at the current time to comment on the safety or potential benefits of the psychotropic drugs (for example, stimulant medications for ADHD, anti-depressants, etc.) in children with cCHD.  Many of the medications currently available for children with structurally normal hearts slightly increase the risk of rhythm problems; and children with CHD are possibly at increased risk for rhythm disturbances when using these medications.  It must be emphasized that no large study has determined how safe (or dangerous) these drugs are in children with CHD, or even if they work the same way as in children without CHD.  The decision to use medications to deal with behavioral issues or ADHD must be individualized to the child, balancing the unknown risks of these medications in children with CHD against the lifelong implications of academic and social difficulties.  Close follow-up, planning and surveillance are warranted when beginning any new medication.

Finally, it must be recognized that the early stress of the diagnosis and treatment may cause psychological stress for years or longer - both in the child and the caregivers. One of the goals of our program is to validate these feelings, to recognize the incredible stress a diagnosis of cCHD has on an entire family unit, and to strongly recommend counseling and evaluation by trained professionals in this area.  This is a very new concept and is being met with some resistance, almost as though asking for mental health help is a sign of “weakness” or somehow admitting to ineffective parenting skills. Sadly, there continues to be a stigma associated with mental health issues such as anxiety, depression, bipolar disorders etc, for which early treatment (medical and therapy) have been shown over and over to improve results.  Parenting a child with cCHD is tough.  Very tough.  It affects the marriage, the siblings, the grandparents, and everyone involved. We now ask many families to strongly consider professional help as soon as the diagnosis is made, and to continue along with the ongoing medical and psychological issues faced by the entire family.  This model of preventative counseling for PTSD, pioneered preventatively by members of the military, is solid, and it works.  Importantly, we strongly believe that this type of mental health approach for the family will have major benefits over the long term for the child with cCHD.

How can we decrease the frequency of these problems in the future?

In my opinion, the outlook for our children with cCHD remains quite optimistic.  There are certainly continued challenges in improving overall quality of life.  Not only are patients surviving longer compared to one or two decades ago, but serial studies show improvement in many areas of functioning when compared with older children who were repaired in the past.

It must be emphasized that it is quite difficult to apply the results (standardized test results) of our current school-aged children (who were diagnosed and treated in the 1990's) to our babies born in 2010-2015.  This is due to significant improvements in the last two decades—including prenatal diagnosis, post-operative care, and a better understanding of cardiopulmonary bypass.  A number of additional factors will ultimately contribute to the academic success of our children, including parent and teacher expectations. It is highly likely that babies born today will have better outcomes than those born just a generation before.

The best way to improve the outcomes for future generations is a continued partnership between patients, parents, researchers, nurses and physicians.  It is crucial that physicians, parents and patients continue to advocate at the government level for research monies directed at the cause and treatment of heart disease in children as well as its secondary effects on brain development and quality of life.  Philanthropic contributions play a significant role in start-up funds for research as well.  Finally, if families and children are willing, voluntary participation in clinical research studies remains the cornerstone of the process.

Summary of Current Findings, 2015

• In the absence of an associated structural brain abnormality or genetic syndrome, cognitive function (IQ, “intelligence”) is typically within the normal range for most children with CHD

• Parental IQ and socioeconomic status are most strongly associated with IQ in CHD patients, rather than the type of CHD or its management

• Behavior problems, especially impulse control and attention difficulties, are prevalent

• Deficits in visual-motor integration and language skills are common

• Executive functioning—the ability to plan and perform complex tasks—is impaired in some children

• Some identified risk factors for academic and behavioral difficulties include: complex CHD requiring multiple operations, a long hospital stay, and multiple unplanned interventions.  Health-related quality of life is also affected by the number of medications necessary and number of doctor visits per year.

• The association between open heart surgery and postoperative care with later cognitive, language, or behavior difficulties continues to be an area of investigation

• Treatment strategies continue to be “in evolution”, but include usual medical therapies for ADHD (under the supervision of the cardiologist), behavioral therapies, home exercise and physical therapy programs and encouraging daily physical activity and participation in group activities

• Psychosocial support and mental health consultation for families in the early postoperative period have been shown to be beneficial to both the parents and the infant

Selected references:

Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management a scientific statement from the American Heart Association. Marino BS and colleagues. Circulation 2012. http://circ.ahajournals.org/content/126/9/1143.full

Risk and Prevalence of Developmental Delay in Young Children With Congenital Heart Disease.  Mussatto KA and colleagues.  Pediatrics 2014 http://pediatrics.aappublications.org/content/133/3/e570.full

Current insights regarding neurological and developmental abnormalities in children and young adults with complex congenital cardiac disease.  Wernovsky G. Cardiology in the Young 2006 https://www.researchgate.net/profile/Gil_Wernovsky/publication/7371218_Current_insights_regarding_neurological_and_developmental_abnormalities_in_children_and_young_adults_with_complex_congenital_cardiac_disease/links/0046352b0ca2fd32c4000000.pdf

Best Practices in Managing Transition to Adulthood for Adolescents With Congenital Heart Disease: The Transition Process and Medical and Psychosocial Issues - A Scientific Statement From the American Heart Association. Sable C and colleagues.  Circulation 2011. http://circ.ahajournals.org/content/123/13/1454.full

Parenting children with hypoplastic left heart syndrome: Finding a balance.  Lee A and Rempel GR. Journal for Specialists in Pediatric Nursing  Volume 16, Issue 3, pages 179–189, July 2011.

Practitioner's Perspective: Family Centered Care

by Michael V. DiMaria, M.D.

Children's Hospital Colorado

Family centered rounds have been introduced in many children’s hospitals for several reasons, but one of the primary motives is that families like to be involved in the health care decisions of their children. Family centered rounds are also a manifestation of a change in the culture of medicine, which has occurred relatively quickly. The way we as physicians practice medicine has shifted from what was a more paternalistic model to a cooperative one; this is addressed very explicitly in medical school curricula: we are in the era of ‘patient autonomy In pediatrics this translates into ‘family autonomy.’ The consequence of the rapid change is that both health care provider and patients can be unfamiliar with the process, and unsure of how to make best use of this opportunity.

To understand what this means for families, let’s start by talking about what to expect on morning rounds. First thing in the morning, after the incoming team of providers has gotten up to speed on developments from the overnight shift, the group gathers at each patient’s bedside in turn. The goal will be to review the events of the last day, discuss how the patient is doing, and come up with the plan for the next day. This is an important time for everyone on the medical team and the family to get on the same page. Often times, especially at a teaching hospital, a large crowd will be present. In order to make things flow more quickly and easily, the attending may ask that any questions or concerns be raised at the end of the presentation; it is difficult for the team to stay focused and efficient if there are frequent side conversations.

Next, the players: the patient’s nurse will be at the bedside, advocating for the family, bringing up unresolved issues, and clarifying his role in the plan. A resident or fellow will ‘present’ the patient to the group, meaning that she will be responsible for knowing all the details about the patient and summarizing them efficiently to the group. To briefly explain how she came to be in this position, after graduating from medical school and receiving an M.D., she did a 1 year internship, then began a 2-5 year residency; after residency, she may do an additional 3-4 years of training, called a ‘fellowship’ to sub-specialize. After finishing all of her training, a doctor becomes an ‘attending physician’ – the person in charge of the team. The attending will be listening, teaching, and guiding the trainee through the process of assembling the data and figuring out what to do next. Various other people may be present, including pharmacists, nutritionists, nursing leadership, physical therapists, etc.

As alluded to above, rounds will consist of a brief description of the day’s events, a review of the physical exam, then a bunch of numbers (lab values, fluid intake and output, ventilator settings, etc.). At the end of all of this comes the assessment and plan. It goes without saying that during the assessment the presenter verbalizes their impression of how the patient is doing. The plan is usually presented in parts, according to ‘organ systems’ (all of the respiratory issues are grouped together, as are all of the cardiovascular problems, etc). This will sound something like, “From a respiratory standpoint, I think we can try to decrease the oxygen from 2 to 0.5 liters per minute. From a nutritional standpoint, I think that Sam can try to eat a solid diet today…” This will continue until all of the active medical issues have been addressed.

When patients and care providers first encounter family centered rounds, some may find it uncomfortable to have conversations about sensitive topics in the presence of a large group. Of course, there should always be the option for the family to have a more private conversation with only the essential members of the team, should there be concerns about privacy. From the resident’s perspective, my colleagues and I were initially very nervous about presenting a patient history and physical in front of the patient’s family as a resident. Aside from being a little shy, I think I was nervous that I would get something wrong and that the family would correct me in front of the boss. I quickly got over this fear, when it became obvious that the common goal was historical accuracy.

There are several points during the process when the family’s input will be especially helpful. It goes without saying that nobody knows a child better than the primary caregivers, and so who better to be the definitive source of historical information? Because team members may come and go twice a day (at the end of a 12 hour shift), none will have the perspective of the family, who will have been with the patient continuously. Being able to give the team an idea of the overall trend of the patient’s symptoms can be very helpful. Similarly, it can be difficult for people who don’t know the child to tell what sorts of behaviors are personality-related and which are indicators that things are not quite right.

The goal of this process is to get the patient home, feeling well, and able to participate in all of the things that go into being a healthy, happy kid. More often than not, this means that the family will need to continue some interventions in the home. So, part of having a child in the hospital is learning about the disease and the treatments. Being an informed caretaker will better enable the family members to identify when problems occur at home, and what to do about them. Some measure of our success as care providers is whether or not the family feels comfortable once they are discharged. Our hope is that the shared knowledge and unified goals of families and care providers results in a close working relationship that develops in this setting of family centered care.

Preparing Your Child for the Fontan

By: Sherry Polise, CCLS, Child Life Specialist III - The Cardiac Center
The Children’s Hospital of Philadelphia

“Helping Your Child Prepare for their Fontan Surgery”

The Fontan Surgery is stressful and overwhelming for parents for many reasons, but one of the most difficult aspects is because of your child’s age.  Most children entering into this procedure are between 2 and 5 years old so they are just beginning to understand their body and how it works, but they are also extremely limited in the amount of complex information they can process.  Parents often feel overwhelmed at the thought of talking to their 3-year-old about open heart surgery when they themselves get confused and worried when thinking about the topic.  For these kids there are still plenty of things you can do before surgery that can help your child feel comfortable, have a better understanding of what is happening, and help set the stage for  the most positive experience possible  while at the hospital.
Before surgery, there are many things you can do to help support your child’s understanding of what is to come.

-Be honest and talk about what’s happening: First and foremost, it is important to be honest -    even with young children - about what is happening while using age-appropriate language.  A simple way of explaining heart surgery to a child even as young as 2 or 3 might be “remember when we went to the doctor and took the special pictures of your heart? He saw a little boo-boo that needs be fixed and that’s called surgery”.  There are some 3 year olds who might hear that and ask a ton of questions right away and some who might look puzzled and immediately want to go back to playing.  This range of reactions is okay and normal; the most important thing is that you are using consistent, honest, and age-appropriate language so that your child feels empowered to ask questions if they have them.

-Use simple concrete language:  Kids at this age can have wild imaginations; sometimes that imagination can help them have a lot of fun, but it can also make them envision really scary things too.  When taking about the hospital, try to avoid analogies and metaphors like “having a zipper” or “fixing your engine” as these can evoke confusing and scary imagery for young children.  Using familiar terms like “boo-boo” or “owie” helps your child understand what you are talking about without letting their imaginations run wild.

-Be aware of timing:  One common question that parents often ask is “when is the right time to tell my child about surgery?”  For most children in this age range, we recommend telling them no more than 1 to 3 days prior to the procedure.  This allows time for them to ask questions and process the information but not too much time to sit and worry about it or even forget the information completely.

-Ask to speak with a child life specialist:  All pediatric hospitals employ child life specialists who are trained to help explain medical procedures to children of all ages.  You can ask to speak to one about how best to meet your child’s needs.  Often a child life specialist can use a doll or book to help prepare your child for the hospital and the things they will see on their body after surgery.  Often times, they can arrange a pre-op tour with you and your child to give them an opportunity to see the hospital and ask any questions beforehand.

-Focus on some of the positive aspects of the hospital: It is important to tell your child the truth about things that are happening but make sure you emphasize the positive and the things that are important to them.  Remind that them you will be with them at the hospital and that there are toys, books, and movies at the hospital too.  Most children’s hospitals have a playroom as well.  These things are comforting and reassuring to young children.

-Read and engage: Another thing you can do prior to surgery and just in general is to incorporate story books about the hospital or going to the doctor into your normal reading routine.  Reading about your child’s favorite character going for a check-up or visiting the hospital can help normalize these things for them and provide an opportunity for dialogue about these experiences.

-Use medical play:  For children at this age, they are also learning about and processing their world through play.  Medical play can be extremely helpful for children in giving them an opportunity to “be the doctor” instead of the patient.  You can help your child by providing them a play doctor’s kit and a doll and simply being there as a reflective listener during the play.  You can also listen to your child while they are being the doctor and clear up any misconceptions you overhear.

-Help them to take charge: You can also help your child prepare for surgery by helping them have some control over the situation.  As you well know as the parent of a preschooler, a sense of independence and control is very important for them.  You can foster that sense of independence by allowing your child to help pack their bag for the hospital and allowing them to include the things that are important to them (i.e. a favorite DVD, favorite sippy cup, pajamas, etc.).  Siblings can help too!

-It is okay to not know:  If your child asks you something about the hospital that you don’t know or aren’t sure how to answer in a way that isn’t too scary, you can be honest and say “that’s a great question, I’m not sure but let’s write it down and we can ask when we go to the hospital”.  This can give you an opportunity to think about your response but also assures your child that you will get them the answer.

You can also find more information and helpful tips on preparing your child for surgery here.

Thank you Sherry for sharing your insights and advice on preparing for the Fontan!  We are truly grateful for your expertise.

Preparing Your Child for the Fontan

Sherry Polise, CCLS
Child Life Specialist III
The Cardiac Center
The Children’s Hospital of Philadelphia

Helping Your Child Prepare for their Fontan Surgery

The Fontan Surgery is stressful and overwhelming for parents for many reasons, but one of the most difficult aspects is because of your child’s age.  Most children entering into this procedure are between 2 and 5 years old so they are just beginning to understand their body and how it works, but they are also extremely limited in the amount of complex information they can process.  Parents often feel overwhelmed at the thought of talking to their 3-year-old about open heart surgery when they themselves get confused and worried when thinking about the topic.  For these kids there are still plenty of things you can do before surgery that can help your child feel comfortable, have a better understanding of what is happening, and help set the stage for  the most positive experience possible  while at the hospital.

Before surgery, there are many things you can do to help support your child’s understanding of what is to come.

• Be honest and talk about what’s happening: First and foremost, it is important to be honest -    even with young children - about what is happening while using age-appropriate language.  A simple way of explaining heart surgery to a child even as young as 2 or 3 might be “remember when we went to the doctor and took the special pictures of your heart? He saw a little boo-boo that needs be fixed and that’s called surgery”.  There are some 3 year olds who might hear that and ask a ton of questions right away and some who might look puzzled and immediately want to go back to playing.  This range of reactions is okay and normal; the most important thing is that you are using consistent, honest, and age-appropriate language so that your child feels empowered to ask questions if they have them.

• Use simple concrete language:  Kids at this age can have wild imaginations; sometimes that imagination can help them have a lot of fun, but it can also make them envision really scary things too.  When taking about the hospital, try to avoid analogies and metaphors like “having a zipper” or “fixing your engine” as these can evoke confusing and scary imagery for young children.  Using familiar terms like “boo-boo” or “owie” helps your child understand what you are talking about without letting their imaginations run wild.

• Be aware of timing:  One common question that parents often ask is “when is the right time to tell my child about surgery?”  For most children in this age range, we recommend telling them no more than 1 to 3 days prior to the procedure.  This allows time for them to ask questions and process the information but not too much time to sit and worry about it or even forget the information completely.

• Ask to speak with a child life specialist:  All pediatric hospitals employ child life specialists who are trained to help explain medical procedures to children of all ages.  You can ask to speak to one about how best to meet your child’s needs.  Often a child life specialist can use a doll or book to help prepare your child for the hospital and the things they will see on their body after surgery.  Often times, they can arrange a pre-op tour with you and your child to give them an opportunity to see the hospital and ask any questions beforehand.

• Focus on some of the positive aspects of the hospital: It is important to tell your child the truth about things that are happening but make sure you emphasize the positive and the things that are important to them.  Remind that them you will be with them at the hospital and that there are toys, books, and movies at the hospital too.  Most children’s hospitals have a playroom as well.  These things are comforting and reassuring to young children.

• Read and engage: Another thing you can do prior to surgery and just in general is to incorporate story books about the hospital or going to the doctor into your normal reading routine.  Reading about your child’s favorite character going for a check-up or visiting the hospital can help normalize these things for them and provide an opportunity for dialogue about these experiences.

• Use medical play:  For children at this age, they are also learning about and processing their world through play.  Medical play can be extremely helpful for children in giving them an opportunity to “be the doctor” instead of the patient.  You can help your child by providing them a play doctor’s kit and a doll and simply being there as a reflective listener during the play.  You can also listen to your child while they are being the doctor and clear up any misconceptions you overhear.

• Help them to take charge: You can also help your child prepare for surgery by helping them have some control over the situation.  As you well know as the parent of a preschooler, a sense of independence and control is very important for them.  You can foster that sense of independence by allowing your child to help pack their bag for the hospital and allowing them to include the things that are important to them (i.e. a favorite DVD, favorite sippy cup, pajamas, etc.).  Siblings can help too!

• It is okay to not know:  If your child asks you something about the hospital that you don’t know or aren’t sure how to answer in a way that isn’t too scary, you can be honest and say “that’s a great question, I’m not sure but let’s write it down and we can ask when we go to the hospital”.  This can give you an opportunity to think about your response but also assures your child that you will get them the answer.

Thank you Sherry for sharing your insights and advice on preparing for the Fontan!  We are truly grateful for your expertise.

Practitioner's Perspective: Pain Management and Withdrawal

By:  Dr. Moffett, Pharm.D. , Texas Children's Hospital

If you've been indoctrinated into the Congenital Heart Defect community, one important lesson learned is that all CHD babies and children are different.  As pediatric cardiac surgeon, Dr. Redmond Burke stated, "The babies' hearts are like snowflakes, they are never the same." 

One size definitely does not fit all.  A particular surgery may be ideal for one HLHS infant, but not for another.  The same can be said about medication administration and pain management. 

The vast majority of HLHS infants and children undergoing palliation require opiate pain medications (i.e. Fentanyl, Morphine, etc.) following the immediate post-operative period.  For various reasons, pain medications may be prolonged causing an infant to become opiate dependent or "hooked." 

It is certainly accurate to state that not all HLHS patients become opiate dependent or suffer from opiate withdrawal following open heart surgery.   As stated, each child's care and needs vary.

Recently, with growing concern and questions surrounding opiate dependency from our families, we reached out to Dr. Moffett, Pharm.D. at Texas Children's Hospital, to lend us insights regarding opiate withdrawal and methadone use in the congenital heart patient.

Methadone Use for Opiate Withdrawal

The most frequently asked questions I have received from parents are regarding ‘withdrawal’ and methadone therapy.  There can be a big stigma surrounding the drug methadone, even though it can be very beneficial for children with congenital heart disease who have been in the intensive care unit for long periods of time.

Below, I’ve summarized some of the most common questions I’ve received from parents regarding opiate withdrawal, methadone use, and their child.  As always, every child is unique, and the answers to the questions below provide general information.  Contact your child’s physician or the clinical pharmacist if you have any questions regarding their medications.

What is opiate withdrawal?

Children who experience opiate withdrawal have often received medications in the intensive care unit, such as morphine or fentanyl, to manage their pain.  These medications are in a class of drugs known as opiates.  When patients receive these medications in high doses or for long periods of time, they can become ‘tolerant’ to these medications.

If a child has become tolerant to opiate medications, and they are suddenly stopped, a child can experience ‘withdrawal’ from these medications.

What are the signs and symptoms of opiate withdrawal?

Not every child will experience the same signs and symptoms, but common signs and symptoms include:  irritability, diarrhea, stuffy nose, nausea or vomiting, poor eating, high blood pressure, or high heart rate.
 

How is opiate withdrawal prevented?

Typically, if a child has been receiving high doses of opiate medications for long periods of time, slowly reducing the dose of the medication or reducing the number of times per day that the medication is taken will prevent opiate withdrawal.  This may occur over days or weeks, and can be done in the hospital or after discharge from the hospital.

Different medications can be used to prevent opiate withdrawal, and methadone is a very common medication used to prevent opiate withdrawal in children with congenital heart surgery.

Why is methadone used for opiate withdrawal?

Methadone is long acting, similar to morphine or fentanyl, and can be taken orally.  Therefore, methadone can be used to prevent withdrawal in patients after they have been discharged from the hospital.  Since methadone is long acting, the dose can be slowly reduced and the methadone will be slowly eliminated from the body.  This helps to prevent the signs and symptoms of withdrawal.
 

Is there anything special I need to do to get methadone from my pharmacy?

Your child’s doctor may have to fill out a special prescription for you to take to the pharmacy to get methadone.  Additionally, some pharmacies may not carry methadone, so it is a good idea to make arrangements prior to being discharged from the hospital so that your child doesn’t miss any doses.

I’ve heard about people using methadone, and they are usually addicted to drugs.  Is my child addicted and that’s why he/she is on methadone?

No, in this situation, children are not ‘addicted’ and they don’t get a ‘high’ from the methadone.  The dose of methadone is carefully chosen so that patients don’t experience the signs and symptoms of withdrawal, but also don’t feel any other effects.  The methadone is helping the child feel as normal as possible.

What things should I watch for while my child is on methadone?

You should contact your child’s physician if they begin to experience any of the signs and symptoms of withdrawal as mentioned above.  Additionally, if the dose of methadone is too high, your child might be drowsy or sleeping, or appear sluggish or tired.  As always, if there is a change in your child that you are not comfortable with, or have questions about, call your child’s physician.

In conclusion, these questions represent what I have been most commonly asked by parents regarding opiate withdrawal and methadone use for their child.  There are always other questions that can come up in your child’s care, so, do not hesitate to ask if something is unclear.
 

Brady S Moffett, PharmD, MPH

Pain Management and Withdrawal

By: Brady S Moffett, PharmD, MPH

If you've been indoctrinated into the Congenital Heart Defect community, one important lesson learned is that all CHD babies and children are different.  As pediatric cardiac surgeon, Dr. Redmond Burke stated, "The babies' hearts are like snowflakes, they are never the same." 

One size definitely does not fit all.  A particular surgery may be ideal for one HLHS infant, but not for another.  The same can be said about medication administration and pain management. 

The vast majority of HLHS infants and children undergoing palliation require opiate pain medications (i.e. Fentanyl, Morphine, etc.) following the immediate post-operative period.  For various reasons, pain medications may be prolonged causing an infant to become opiate dependent or "hooked." 

It is certainly accurate to state that not all HLHS patients become opiate dependent or suffer from opiate withdrawal following open heart surgery.   As stated, each child's care and needs vary.

Recently, with growing concern and questions surrounding opiate dependency from our families, we reached out to Dr. Moffett, Pharm.D. at Texas Children's Hospital, to lend us insights regarding opiate withdrawal and methadone use in the congenital heart patient.
 

Methadone Use for Opiate Withdrawal

by Brady Moffett, PharmD/MPH

The most frequently asked questions I have received from parents are regarding ‘withdrawal’ and methadone therapy.  There can be a big stigma surrounding the drug methadone, even though it can be very beneficial for children with congenital heart disease who have been in the intensive care unit for long periods of time.
 

Below, I’ve summarized some of the most common questions I’ve received from parents regarding opiate withdrawal, methadone use, and their child.  As always, every child is unique, and the answers to the questions below provide general information.  Contact your child’s physician or the clinical pharmacist if you have any questions regarding their medications.

What is opiate withdrawal?
 

Children who experience opiate withdrawal have often received medications in the intensive care unit, such as morphine or fentanyl, to manage their pain.  These medications are in a class of drugs known as opiates.  When patients receive these medications in high doses or for long periods of time, they can become ‘tolerant’ to these medications.

If a child has become tolerant to opiate medications, and they are suddenly stopped, a child can experience ‘withdrawal’ from these medications.  

What are the signs and symptoms of opiate withdrawal?

Not every child will experience the same signs and symptoms, but common signs and symptoms include:  irritability, diarrhea, stuffy nose, nausea or vomiting, poor eating, high blood pressure, or high heart rate.
 

How is opiate withdrawal prevented?

Typically, if a child has been receiving high doses of opiate medications for long periods of time, slowly reducing the dose of the medication or reducing the number of times per day that the medication is taken will prevent opiate withdrawal.  This may occur over days or weeks, and can be done in the hospital or after discharge from the hospital.
 

Different medications can be used to prevent opiate withdrawal, and methadone is a very common medication used to prevent opiate withdrawal in children with congenital heart surgery.

Why is methadone used for opiate withdrawal?

Methadone is long acting, similar to morphine or fentanyl, and can be taken orally.  Therefore, methadone can be used to prevent withdrawal in patients after they have been discharged from the hospital.  Since methadone is long acting, the dose can be slowly reduced and the methadone will be slowly eliminated from the body.  This helps to prevent the signs and symptoms of withdrawal.
 

Is there anything special I need to do to get methadone from my pharmacy?

Your child’s doctor may have to fill out a special prescription for you to take to the pharmacy to get methadone.  Additionally, some pharmacies may not carry methadone, so it is a good idea to make arrangements prior to being discharged from the hospital so that your child doesn’t miss any doses. 
 

I’ve heard about people using methadone, and they are usually addicted to drugs.  Is my child addicted and that’s why he/she is on methadone?

No, in this situation, children are not ‘addicted’ and they don’t get a ‘high’ from the methadone.  The dose of methadone is carefully chosen so that patients don’t experience the signs and symptoms of withdrawal, but also don’t feel any other effects.  The methadone is helping the child feel as normal as possible.
 

What things should I watch for while my child is on methadone?

You should contact your child’s physician if they begin to experience any of the signs and symptoms of withdrawal as mentioned above.  Additionally, if the dose of methadone is too high, your child might be drowsy or sleeping, or appear sluggish or tired.  As always, if there is a change in your child that you are not comfortable with, or have questions about, call your child’s physician.

In conclusion, these questions represent what I have been most commonly asked by parents regarding opiate withdrawal and methadone use for their child.  There are always other questions that can come up in your child’s care, so, do not hesitate to ask if something is unclear.
 

Brady S Moffett, PharmD, MPH

Thank you, Dr. Moffett, for lending your time and expertise to our HLHS families. 

Busy Kiddos and Busy Families

Nutrition Series

By: Emily LaRose, MS, RD, CNSC, CSP, Dietitian at Children's Hospital of Los Angeles

It’s the final week of our National Nutrition Month series!  I have really enjoyed working on these posts as a resource for all of the HLHS families out there.  In this last post, I am going to review some of the feeding guidelines used after the Fontan operation, and I am going to cover some nutrition tips for older kiddos.  As promised, I’m also including a couple of recipes for the whole family to enjoy!  The postings I make on this site are my own personal opinions and do not reflect the opinions or views of Children’s Hospital Los Angeles.

Busy Kiddos and Busy Families

Before I get into talking about family nutrition, I wanted to comment on another topic that is specific to our HLHS kiddos: nutrition after the Fontan.  I’ve shared some of what we expect and how we provide nutrition for neonates, infants, and toddlers including some centers’ goal weight for the Fontan.  After the Fontan, however, some centers have different ways that they may modify a kiddo’s nutritional plan.

Immediately following this third surgical palliation, some hospitals restrict sodium, others restrict fat, most restrict fluids, and some centers do a combination of all of these to prevent some of the post-op complications (including chylothorax) and to facilitate recovery.  These restrictions are often temporary, but they can be very important.

At CHLA, we restrict fluids while kiddos are in the hospital after the Fontan.  We also put all of our kiddos on a low fat diet for the first six weeks (or so) after their operation.  The thought is that by restricting fat, we’re trying to minimize the likelihood of a kiddo developing a chylous effusion (where fat leaks into the space around the lungs) that can sometimes happen because of surgery and pressure changes in the chest.

With the low fat diet, we usually have to acknowledge that kiddos may lose a little bit of weight but, because it’s such a short amount of time, weight loss shouldn’t be too significant.  We spend time with families helping to problem solve meals so they have the confidence they need to make low fat foods at home.  Again, this modification is usually temporary so once the cardiologist gives a family a thumbs up to resume a normal diet, there aren’t usually any lasting restrictions.

For kiddos on tube feedings, different formulas may be used after the Fontan as well.  Tube feeding changes are very individual and can be addressed by your child’s dietitian or medical team.

Once a kiddo is back on his regular diet, we encourage a variety of foods including fruits and veggies, healthy fats, and lean proteins.  Some kids do have higher than average nutrition needs so strategies for weight gain can be used but, for the most part, kiddos with HLHS post-Fontan should be following the same general nutrition guidelines as the rest of us.

What about a heart healthy diet?

The idea of a heart healthy diet comes up a lot for our kiddos with heart defects.  When we think of a traditional ‘heart healthy’ diet, we think of adults with heart disease risk factors, but having a diet focused on whole foods and healthy fats can be healthy for all of us.

In the years following the Fontan, our goal remains to keep kids healthy and growing.  When we think about what a heart healthy diet looks like, a few things come to my mind:

• After age two or so, kids don’t need the saturated fats from whole milk anymore so at that point many kids can transition to lower fat or fat free milk.

• Healthy fats can come from fish, olive oil, nuts, seeds, avocados, and other non-animal sources.  It’s a great idea to get some mixed fats into your family’s diet… and, no, fried foods are not a good source of healthy fats even if a vegetable fat is used.

• Don’t overdo it on cheeses and animal fats.  Sure, cheese, eggs, and meats can be healthy because they provide protein, vitamins, and minerals, but eating too much of these foods is not good for anyone.

• Stay away from heavily processed foods.  Processed foods often have a lot of sodium, may have trans fats, and usually don’t offer a whole lot of true nutritional benefit even if the label suggests otherwise.

• Eat fresh fruits and veggies!  It’s not new or surprising, but providing your family (and kiddos) with a colorful variety of fresh fruits and veggies will provide them with more than just vitamin C and fiber.  Plants have a variety of antioxidants that we’re still learning about and most research has said that taking a supplement is not as beneficial as eating food.

We’re busy… what can we do to keep the whole family eating well?

Even with the best of intentions, all of our nutrition and diet goals can be quickly derailed in our busy day-to-day lives.  Home cooked meals become fast food burgers and frozen lasagnas for a lot of families.  Many parents report that they just don’t have time to cook , don’t know how to cook, or feel that cooking at home is too expensive.

Nutritional benefits aside, there have been a number of studies that have proven that home cooking is usually much more affordable than eating fast food or frozen meals.  While I’m a believer that almost anything can exist in a well-rounded diet, I also believe that we should make an effort to eat well most of the time.  So what can busy parents do to prevent frequent trips through the drive through?

• Plan ahead and shop with a list: Planning a weekly menu can help cut back on grocery shopping time, save money, and prevent food waste.  While you’re at the store, it’s also helpful to focus most of your shopping on the perimeter of the store where there are fewer processed foods.

• Cook ahead:  If you have a few hours once a week to devote to cooking ahead, your food plan can be made much easier.  In our house, we spend Sunday afternoons making pasta sauce, roasting veggies, and making meals like beans, lasagna, or enchiladas that can be frozen or refrigerated and reheated for another night during the week when there’s less time to cook.  

• Get kids involved in cooking: Even young kids can help knead dough, add ingredients to a bowl, or sprinkle salt on a dish.  Kitchen tasks can change as kiddos get older and can handle more responsibility.  Including kids in cooking makes often them more open to trying new foods that they helped prepare!

• Let kids pick out a fruit or veggie at the store: For some parents, bringing kiddos to the grocery store or farmers market and allowing them to pick a new fruit or veggie is a good way for the whole family to try something different.  Again, encouraging (and allowing) kids to get involved can be a great way to get them excited about food, eating, and cooking.

What about recipes?

Historically, recipes have come from cookbooks, magazines, friends, and family members, but in the age of the internet, there are even more resources available.  The key is finding a few that provide recipes that match your cooking skill level and that you can trust for reliability.

Cookbooks: The Essential Vegetarian by Diana Shaw (now out of print) and How to Cook Everything (by Mark Bittman) are a couple of my favorites.  I always figure that it’s easy to make a vegetarian meal non-vegetarian… and Mr. Bittman’s book really does include almost everything.

Websites: I find it helpful to use magazine sites like Bon Appetit, Cooking Light, and Saveur all of whom have kitchens where recipes are well tested prior to publication.  I also like the ‘Photograzing’ section of the Serious Eats website (www.seriouseats.com) for finding inspiration.  While the pictures provide links to other websites and blogs that may or may not contain recipes, I find it to be like window shopping- I may not buy what’s in the window, but it may inspire me in some other way.

I grew up cooking Southern food alongside my parents, grandparents, aunts, and uncles so, like many others, I have a collection of family recipes that I like to refer to.  I also went to culinary school (and have my B.S. in Culinary Arts Nutrition).  Because of my cooking family and education background, I cook by inspiration and by feel more than by recipe (but that’s not how I started!).

When I bake I rely on recipes, and I try to keep note what works and what doesn’t.  The Joy of Baking website (www.joyofbaking.com by Stephanie Jaworski) is a great go-to site for me.  I especially love that all of the recipes have weight measures since when I bake cakes and things, I use a scale.

As promised, I wanted to include a couple of recipes for the whole family so here goes.  To give a little bit of background, I have been making various kinds of ‘pockets’ for a few years.  I really like that they can be frozen for a quick lunch or dinner on the go.  It’s also really easy to make different sizes for your young kiddos (just like an empanada, calzone, or other savory stuffed pastry).  I usually fill them with veggies, but you could add meat, chicken, or just about anything else.

Sweet Potato Pockets - With an Indian Flare

For those with kiddos on a low fat diet, I would suggest this first recipe since it can be made with fat free yogurt and fat free milk.  Because this is an essentially fat-free dough, it can be a little chewy, but I like it.  You can substitute some of the flour for whole wheat if you’d like and, of course, the fillings can be changed to whatever you like.  Kids can help mash, mix, and fill these little pastry pockets… though you may end up with a mess.  Have fun and feel free to get really creative!

Makes ~10 pockets

Ingredients:
For the pastry dough:
2 ½ cups flour (plus more for rolling out the dough)
½ cup non-fat plain yogurt
½ cup non-fat milk
½ teaspoon salt

For the filling:
1 pound of sweet potatoes, peeled and steamed/roasted
½ cup wilted spinach, cooked or frozen and drained
½ cup peas, cooked or frozen
½ teaspoon salt
Pepper to taste
¼ cup fresh cilantro, chopped (optional)
½ teaspoon of mild curry powder (optional)
2 tablespoons of pepper jelly (optional)

Preparation:

1. Preheat the oven to 400 degrees.Line a baking tray with parchment paper or a silpat baking sheet.

2. To prepare the dough, combine flour, yogurt, milk, and salt in a mixing bowl.  Gently mix with a fork until it comes together to make a ball of dough.  Cover with plastic wrap and allow the dough to rest in the refrigerator.

3. Prepare the filling by mashing the sweet potatoes with the back of a fork then folding in spinach, peas, salt, and cilantro.  You can add black pepper, curry powder, and pepper jelly if you’d like.  Taste the filling and adjust the seasoning as desired.

4. Remove the dough from the refrigerator and divide into about 10 pieces (or more if you’re making smaller pockets).  Press or roll each piece of dough into a six inch circle.  Add about ¼ cup of filling to each round of dough.  Fold the dough in half (into a half moon shape) and fold the edges in or press the edges with a fork to seal them.

5. Bake the pockets for 20 to 25 minutes or until lightly browned.

A few notes: You could swap out the sweet potatoes for butternut squash or even roasted cauliflower.  I do usually add all cooked items to the pockets so that the filling is soft and so that they don’t leak a lot of liquid while they cook.  One of our favorites is to make this dough and fill it with wilted spinach, feta cheese, onions, and dill for a little Greek influence.  If you’re not following a low fat diet, you can brush the outside with a little olive oil to help the pockets brown in the oven.

Chicken and Black Bean Pockets - Tex-Mex Style

For those who aren’t on a low fat diet, I love the following dough recipe for savory pastries.  It does have butter in it so there is some saturated fat but when you break it down into servings, the amount of butter per person actually isn’t too much.  I also love the flavor of the olive oil in the crust (and it’s even better with some pepper added to it if your family likes).  These are fun to make with kids since there is plenty they can do to help out.  And you really can fill these with anything!

Makes ~10 pockets

Ingredients:
For the pastry dough:
1 cup flour (plus more for rolling out the dough)
1 cup cornmeal
5 tablespoons of butter, very cold
¼ cup olive oil plus extra for brushing
1 teaspoon salt
Cold water

For the filling:
1 cup cooked chicken (ground, shredded, or diced chicken is fine)
½ cup black beans, cooked or canned
½ cup corn, frozen or roasted
½ cup salsa
¼ cup fresh cilantro, chopped
Chopped onions (optional)
Shredded cheese (optional)

Preparation:

1. Preheat the oven to 400 degrees.  Line a baking tray with parchment paper or a silpat baking sheet.

2. To prepare the dough, combine the flour, cornmeal, and salt in a mixing bowl.  Cut the butter into small pieces and blend into the flour mixture until it looks like a coarse meal.  Gently stir in olive oil.  Gradually add cold water until the dough comes together enough to make a ball.  Cover the dough and allow it to rest in the refrigerator for at least 30 minutes.

3. Prepare the filling by mixing all of the ingredients together in a mixing bowl.  Taste the filling and adjust the seasoning as desired.

4. Remove the dough from the refrigerator and divide into about 10 pieces (or more if you’re making smaller pockets).  Press or roll each piece into a six inch circle.  

5. Add about ¼ cup of filling to each round of dough.  Top the filling with cheese if you’d like.  Fold the dough in half (into a half moon shape) and press the edges with a fork to seal them.

6. Brush the tops of the pockets with a little olive oil.  Bake for 20 to 25 minutes or until lightly browned.

A few notes: These are easy to make vegetarian with added veggies or more beans (and without chicken).  You could also swap out the chicken for another kind of meat if you’d prefer.  This is also a great way to use leftover chicken if you have some in the fridge!  I’ve also been known to use this dough to make a larger vegetable pie rolling it out to fit a 9” spring form pan and filling it with all sorts of things.

I have really enjoyed writing these posts for Sisters by Heart; I am honored to have been included in your National Nutrition Month blog series.  I hope that the information I have provided has clarified how we think about nutrition for kiddos with HLHS as they grow.  All kiddos are different so, as I’ve mentioned, if you have questions or need help with your little one’s nutrition, let your medical team know.  I wish you all the best, and Happy Cooking!

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Thank you so much, Emily, for all of your fantastic nutritional advice! All of us here at SBH, and the families we serve, have so appreciated your insights!

The Growing (and Sometimes Picky) Toddler

Nutrition Series

By: Emily LaRose, MS, RD, CNSC, CSP, Dietitian at Children's Hospital of Los Angeles

It’s week four of the National Nutrition Month series!  Emily LaRose, nutritionist at Children's Hospital Los Angeles, is back again, providing valuable insights into how to navigate the nutritional needs of your cardiac toddler. Thank you, Emily for partnering with Sisters by Heart to share your knowledge and advice with our families!

In this post, I am going to provide some resources and tips to address some of the challenges we face in trying to feed toddlers.  We all know that feeding a toddler can be tough!  The postings I make on this site are my own personal opinions and do not reflect the opinions or views of Children’s Hospital Los Angeles.
 Growing (and Sometimes Picky) ToddlersFeeding a toddler can be a challenge.  As kids grow, they develop all sorts of preferences that can change without notice.  One day broccoli looks like ‘little trees’ that are fun to eat… and the next day there could be meltdowns about the mere presence of broccoli on a plate.  So what is a parent to do?

In short, it is best to stay calm.  One of the most important things to keep in mind about feeding a toddler is that eating shouldn’t be a battle.  Sometimes toddlers exert the little control they have over their ‘world’ at the dinner table.  It’s part of learning and development.  In theory, eating is the one thing that a toddler can decide not to do… and there’s not much you can do about it (in the very short term).

There are a number of feeding experts around though Ellyn Satter (http://www.ellynsatter.com/) is widely known for her realistic and practical approach to feeding little ones.  She has a great website that’s full of tips for parents of young and picky kiddos, and she has a book called Child of Mine that should be required reading for anyone feeding young kids.  I love Ms. Satter’s approach to feeding young kiddos, and I find myself referring to her work often.

When I speak with families, I usually focus on the following as a way to get started:
 

• Make family meals a priority.  Your kiddo is going to learn what and how to eat by watching you so eating together (without distractions like T.V. or cell phones) is really important.  If you want your kiddo to eat her veggies, you need to eat your veggies… that’s how she’ll learn.

• If you want your kiddo to grow up eating a variety of foods, you have to feed him a variety of foods.  Your child isn’t going to like everything you give him but, by continuing to offer him a variety of foods, he will be more likely to try new things.  Putting some of each of the foods offered at a meal on your toddler’s plate, even if he doesn’t eat them all, is a good way to start.

• Be very careful about saying that your kiddo likes or dislikes certain foods.  If your toddler hears you say that she doesn’t like spinach, she may not even really know what spinach is but it’s very possible that she will grow up thinking she doesn’t like it.

• Make a meal and snack schedule.  If your little one doesn’t eat much for breakfast, it’s not a great idea to chase him around the house with cereal or other snacks all morning just to get him to eat something.  The best thing you can do it wait until the next meal or snack before you try to feed him something else.  

• Kids’ tastes change really quickly.  Just because your little one doesn’t like something today doesn’t mean she won’t like it two days from now.  It’s also important to keep in mind that kids may have to see a new food 10+ times before they’re even willing to try it… and, even then, they may still spit it out a few times before they’re willing to swallow it.

What if my kiddo isn’t gaining enough weight?
Weight gain normally slows after a kiddo turns one.  Normal weight gain for kids between one and two years of age is only about 8 grams per day (which is about a pound every month and a half!).  Between two and six years of age, weight gain slows down a little more to about 6 grams per day (which is about a pound every two and a half months!).

For kids who have grown well, parents usually find there is less and less pressure on how much weight a kiddo is gaining as long as they’re on a good path.  Many times, 15 kilograms (about 33 pounds) is used as a goal weight for the Fontan with many kids reaching this goal sometime between the ages of two and five- but there are plenty of exceptions.

Having the pressure of a weight goal can make feeding feel like uphill battle for some families.  Poor heart function, oral motor problems, early feelings of fullness, and other medical problems can make weight gain difficult.  With lower weight gain norms, we have a great opportunity for catch-up growth in kids who have had a hard time gaining weight.

All kids grow differently so it’s important to know what is and is not normal for your child.  I’m a believer that we can get all kids to grow if we get them enough nutrition, but what works for one may not work for another.  Some kids need extra fat, others benefit from more protein and/or carbohydrates.

It’s always okay to ask to meet with a dietitian if you have concerns about your kiddo’s growth or intake.  Some doctors will provide you with a referral, and some insurance companies may cover visit costs.  Dietitians can help you figure out how to get your little one enough of the right foods to get him growing.  Sometimes it takes a little bit of trial and error but, with patience, things will get better.

What about supplement drinks?
Doctors may recommend nutritional supplements to help your kiddo gain weight better.  There are plenty to choose from- some with different forms of proteins, carbohydrates, and fats.

The most common supplements for kids who don’t have digestive problems are Pediasure (Abbott) and Boost Kid Essentials (Nestle) but there are a lot of others.  Many are milk based so they may not work for kids with allergies; most are lactose-free and should be okay for kids with lactose intolerance.  These products have more calories that ‘regular’ cows’ milk because of added sugars and fats, but they have added vitamins and minerals, too.

If you’d prefer, you could try making your own supplement drinks since there isn’t anything truly magical about many of the commercial products.  Of course, it’s a good idea to check with your kiddo’s pediatrician (or dietitian) first just to get an okay.

I’ve included a couple of sample recipes, but I wouldn’t use either as a kiddo’s only source of nutrition.  Either one could be added to his or her diet as a ‘boost.’  There are a lot of resources out there with recipes, but these are a couple that I have had success with.

If you were to blend the following foods you would have a drink with a few more calories and about the same amount of protein as a bottle of Pediasure (248 calories vs. 240 calories both with ~7g protein).
 

• 6 ounces of whole milk 

• ½ of a ripe banana 

• 2 teaspoons of honey

• 1 teaspoon of flaxseed oil

For another example, you could blend the following items for a drink with a few more calories and about the same amount of protein as a bottle of Boost Kid Essentials 1.5 (366 calories vs. 360 calories both with ~10g protein).
 

• 6 ounces of whole milk 

• ¼ cup of cooked/mashed sweet potatoes

• 1 tablespoon of honey

• 1 tablespoon of almond butter

• 1 teaspoon of flaxseed oil 

Since these recipes are small, you may need to make larger batches to accommodate your blender or food processor; you can always refrigerate any extra for later.  As another option, you could use baby foods that mix into milk really easily.  The options are endless so feel free to get creative; adding cinnamon, nut butters, chocolate powder, and berries are all good options- but don’t stop there!

If you were going to use almond milk, rice milk, or another type of dairy milk substitute, the recipe would not provide as many calories or as much protein as the ones I’ve included here.  Using nut butters, cooked eggs, tofu, avocado, and other additives can help to fill in the gaps- your dietitian can help you figure out your best options.

*** If your kiddo is on tube feedings, always discuss any proposed changes with your child’s doctor or dietitian before you make any changes.  The recipes I’ve included are not intended as tube feeding formula replacements.

What if my kid is on tube feedings?
For kiddos who are on tube feedings, we make adjustments for growth a little bit differently.  Sometimes we will try new formulas to see if we can get a kiddo growing with a different mixture of fats or by giving different proteins.  Sometimes we’ll change the timing of feedings or give some feedings overnight while the kiddo is sleeping.  Sometimes we can add foods to tube feedings to help with weight gain, too.

Does my child need to take a multivitamin?
With a normal and varied diet, most kids probably don’t need to be on a special multivitamin, but it’s always a good idea to ask your child’s pediatrician about what may be best for your kiddo.  If needed, most physicians will suggest a once-a-day chewable vitamin or something similar.

I’m partial to vitamin products without a lot of sugar or extra filler ingredients.  I don’t usually recommend gummy vitamins for a few reasons (though we get asked often).
 

• Gummy vitamins can be a choking hazard because young children tend not to chew them very well.  

• They tend to stick in kiddos’ teeth and can cause dental problems.  Since we worry a lot about the mouth being a possible place where infections can start (especially for cardiac kiddos), we try not to encourage anything that can cause decay and other problems.

• Dosage recommendations for gummy vitamins are usually between two and six per day (depending upon the brand) and, truly, no one needs to eat candy as a daily supplement.

• They usually are not complete sources of vitamins and minerals.  Many of the powdered or chewable vitamins have better amounts of all of the micronutrients we’re looking for.

As the package says, always keep vitamins out of reach of small children; overdoses can be fatal.
 

The Cardiac Infant

Nutrition Series

By: Emily LaRose, MS, RD, CNSC, CSP, Dietitian at Children's Hospital of Los Angeles

We're continuing on with our Nutrition Series this week, with the help of resident expert Emily LaRose, dietitian at Children's Hospital Los Angeles. Today, Emily discusses the nutritional concerns of the cardiac infant into early toddlerhood. Thank you, Emily for continuing to share your knowledge and advice with our families!

I’m back for week two of the National Nutrition Month series!  In this post, I’m very excited to write about what happens for baby’s nutrition once he is discharged home from the hospital the first time.  This post will take us through starting solids and transitioning to cow’s milk.  The postings I make on this site are my own personal opinions and do not reflect the opinions or views of Children’s Hospital Los Angeles.

As I’d mentioned in my last post, nutrition and growth are both big parts of the interstage period.  While we can establish a nutrition plan in the hospital, our plans may change once a baby has had some time at home so we’re always ready to make adjustments.  Once babies are home, we rely on parents to tell us what is and isn’t working.

We focus a lot on growth and weight gain in the interstage period because babies who grow well tend to stay healthier, do better with their future surgeries, and have better overall development than those who haven’t grown as well.  A good nutritional foundation helps with the body’s response to surgery and with healing afterward. Good nutrition also helps with immune function and brain development.

After the Glenn, weight gain and growth are generally less of a concern for a few reasons:
• Babies tend to gain weight a little more easily after then Glenn than in the interstage period.
• The blood flow to the baby’s belly is usually more stable so digestive problems are usually less of an issue.
• There isn’t as much focus on rapid growth because there usually isn’t another surgery planned for the near future (since the Fontan is usually many months away).

After the Glenn, we are still trying to get babies to grow (so we do usually continue with high calorie breastmilk or formula) but, in a lot of ways, post-Glenn nutrition becomes more like ‘typical’ baby nutrition.  Babies progress toward taking solid foods, develop regular eating schedules, and eventually start taking cow’s milk just like other babies.

So going forward how much weight gain should we expect?
As I’d mentioned before, usually we say that weight gain of 20 to 30 grams per day (five to seven ounces per week) is normal for babies for the first three months or so.  But, after the first few months, growth goals for the remainder of the first year are very individual for kiddos with HLHS.

There are standard growth goals, but if a baby gets a slower start, he may go through periods of faster growth (which we call ‘catch-up growth’).  If a baby gets sick or spends some extra time in the hospital, she may grow a little more slowly for awhile before her weight gain picks up.  Again, there is no set way that all kiddos grow, but sometimes averages can help us figure out how things are going.

For growth goals, the following will give you an idea of what would be average, but keep in mind that your kiddo may be growing on his or her own curve.
• 3 to 6 months: average weight gain may slow down a little to an average of about 18 grams per day (about four to six ounces per week).  Length gains should be about 0.5 centimeters per week (or about an inch every five weeks).
• 6 to 12 months: weight gain slows down a little more to an average of about 12 grams per day (about three ounces per week).  Length gains also slow down a little more to about 0.3 centimeters per week (or about an inch every seven or eight weeks).

Another good rule of thumb for weight gain is that a baby should double her birth weight by six months and triple it by one year.  So if your little one was born at seven pounds, she should be about 14 pounds at six months, and about 21 pounds at her first birthday- if everything goes exactly according to plan.

When can I start giving my baby solids?
People ask about when to start babies on solids all the time… and, unfortunately, there are a lot of different answers.  The American Academy of Pediatrics recommends solely breastfeeding for the first six months of age though multiple other sources suggest that between four and six months is a good age to start solid foods.  Giving solid foods is not recommended for infants less than four months of age.

For an HLHS kiddo, cardiologists will often suggest that families wait to start a baby on solids after her Glenn but that isn’t always the rule.  If you’re thinking that your little one may be ready to try some solid foods, talking to your baby’s cardiologist and pediatrician is a good idea.

The way the solids are introduced to an HLHS infant is just like a non-cardiac baby.  Basically, we rely on the baby to tell us when he’s ready and we go from there.  For more information, this website (http://www.healthychildren.org/English/ages-stages/baby/feeding-nutrition/Pages/default.aspx) was created by the American Academy of Pediatrics and is a great resource.  They have covered everything from how to know if your baby is ready for solids, what kinds of foods are good to start with, how much to give, and much more.  They have even included a sample menu that could be helpful for older babies.

A few things to remember:
• If your baby turns her head away and refuses to eat, don’t force her.  It’s okay to wait to try solid foods another day.
• Your baby will learn to eat by watching other people eat.  Include your baby in family meals and feed him when other people are eating.
• Babies are messy eaters, but that’s how they learn to eat.  It is normal for meals to end up on the baby’s clothes, bib, and face.
• Your baby isn’t going to like all of the foods that you offer her but tastes change quickly so it’s okay to repeat a food a few days later.  You may be surprised with what happens!
• We always want to make sure our cardiac babies are safe so if you notice that your baby is coughing, gagging, or choking with his feedings, check in with his medical team to make sure that you are okay to continue.
• If your baby has a feeding tube, he may be able to have some solids added to his feeding plan as well.  Once he is at the right age and developmental level for solids, ask your dietitian, cardiologist, and/or pediatrician if this may be an option for your kiddo.

What about food allergies?
Allergies are an important subject since food allergies have become more common in recent years.  No one knows what is leading to so many more cases of food allergy, but everything from household cleaners and antibiotics to environmental changes and genetically modified foods has been blamed.  At this point, we just don’t know (and, in my opinion, there are probably many factors at play).

While the current American Academy of Pediatrics feeding guidelines don’t specifically address when to introduce allergenic foods to babies, some of the most current academic papers (including one published earlier this year in the Journal of Allergy & Clinical Immunology: In Practice) on prevention of food allergies suggest starting highly allergenic foods like soy, wheat, shellfish, and even tree nuts early on.

Recommendations are always changing so, again, this is something you will want to talk to your kiddo’s pediatrician about.

How do I transition my baby from a bottle-feeding schedule to a meal schedule?
As your baby grows, solid foods will play a larger and larger role in his nutrition plan; the goal remains to get the baby to grow as normally as possible.  It’s important to remember that all babies and kiddos are different so allowing them to develop at their own pace is important.

Some babies wean themselves off of the bottle and transition to meals all by themselves.  They can be so excited about meals that the bottles fall away and sippy cups of milk fill in where some of the bottles once were.  Other kiddos demand a more gradual transition.

If you’re having trouble, I often suggest that parents write out the baby’s schedule (of meals and bottles).  At around one year, she will probably need four or five meals per day (of solids) to keep her energy levels up and to keep her growing normally.  If she is taking four or five bottles per day, but only taking solids two times per day, replacing once bottle at a time with a meal or snack is a good place to start if your medical team is okay with it.

A few other things that may help include the following:
• When you can, eat with her so that she knows that it is mealtime.
• It can help to offer solids first and then formula or milk so that she’s not too full for her ‘big girl’ meals.
• You can make a schedule change every few days (give or take) to get her onto her grown up schedule.
• We try to wean babies off of the bottle between 12 to 14 months so encouraging them to use a cup earlier on really helps (if they’re ready).

We see many picky eaters in the next stage of development.  When kiddos aren’t growing well or when parents worry about whether or not kids are getting enough nutrition in, the question of supplmental drinks often arises.  Stay tuned for my next post which focuses on feeding our picky cardiac toddlers.