Alex Tchouchev 3/11/13 Alex Tchouchev 3/11/13

The Cardiac Newborn

Nutrition Series

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

Nutrition and related weight gain are major issues for most of our HLHSers, and for cardiac babies in general. In recognition of Nutrition Month, we have an incredible nutrition series brought to you by Emily LaRose - dietitian at Children's Hospital Los Angeles. Emily's opening post provides a comprehensive look at several nutritional concerns and covers cardiac nutrition from birth to surgery and beyond. Thank you so much Emily for taking the time to provide our families such excellent analysis!

Emily is the dietitian in the Cardiothoracic Intensive Care Unit at Children’s Hospital Los Angeles where she works with the medical team and families to provide nutrition for kiddos before and after cardiac surgery.

Over my nearly 10 years as a registered dietitian, I have worked with children and adults of all ages. After completing the Neonatal Nutrition Fellowship at Texas Children’s Hospital in 2006, I knew that I was destined to focus on working with infants and young kiddos. I began in my current role in 2009 and, while I’ve seen a lot, I continue to learn from patients and families all the time. I am part of a great medical team, and I am in regular contact with colleagues from different hospitals across the country. Together, we can all work to understand how to help cardiac kiddos grow and thrive.

I have met so many wonderful families that serve as ongoing inspiration for the work our team does everyday. I am honored to share some of my professional experience with the Sisters by Heart community; I hope you find it helpful. 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.

Since I will be covering a variety of HLHS nutrition topics over the next couple of weeks, I thought that it would make sense to divide the postings by age: the cardiac newborn, infancy into toddler-hood, growing (and sometimes picky) toddlers, and busy school age kiddos. Happy National Nutrition Month!

The Cardiac Newborn

Usually when babies are admitted to our cardiac intensive care unit, I try to meet with parents to review how we give nutrition to these little ones before and after surgery. With moms who may still be in the birth hospital and dads who may be shuttling back and forth between hospitals and home, it can sometimes take a little while before we’re able to speak but, all the while, we’re getting nutrition into these little ones as best as we can.

In our first visit, I review how we give nutrition to cardiac babies, and I try to make sure that moms have the support they need to get pumping if they’re planning to feed the baby breastmilk when the time comes. Often we talk about the progression of nutrition from IV nutrition to oral feedings and everything that comes in between. I also talk a little about my role as the dietitian on our ICU team since a lot of people aren’t familiar with what a dietitian may do when kiddos aren’t eating.

I thought for this first post I would review some of the nutrition highlights for the cardiac newborn during his or her first hospitalization. A lot of this information comes directly from what I talk to new parents about so that they know what the nutrition plan is… and so that in the midst of everything going on with the baby, they know that we’re doing what we can to help the baby heal and grow.

The Nutrition Plan

Because blood flow to an HLHS baby’s intestines may not be stable or adequate, intravenous (IV) nutrition is often the first, and possibly only, source of nutrition for these little ones before surgery. You may hear this called parenteral nutrition (PN) or total parenteral nutrition (TPN); you may hear the word Intralipid as well. All of these descriptions are talking about the dextrose (sugar/carbohydrate), amino acids (protein), and fat (Intralipid) as well as vitamins, minerals, electrolytes, and other additives that can be given through a vein when feeding into a baby’s tummy isn’t possible.

We can actually get all of the nutrition that a baby needs into a tiny yellow bag and a little white syringe. It’s not a perfect system (we’d rather feed into baby’s tummy when we can), but it’s a lifesaver- literally.

Once deemed safe, many hospitals have feeding protocols that dictate how and when to start feeding into the baby’s tummy (while the IV nutrition continues). Most of the time, feedings are started through a small tube that is placed in the baby’s nose or mouth with a tip that sits either in the stomach or in the small intestine.

With the feeding tube in the stomach, feedings may be started continuously (given 24 hours per day) or as small volume bolus feedings every three hours or so- there is no real consensus on which way is better. With a feeding tube in baby’s intestine, feeds are given continuously. Eventually, these tube feedings are gradually increased (and the IV nutrition decreased) to meet the baby’s energy and protein needs.

Feeding progress is usually very slow because tummy problems can be common with HLHS. Babies may have slowed digestion after surgery or abnormal blood flow to their intestines. They may spit up, have bloody stools, or may be excessively fussy. While some of these problems may be unavoidable, studies have told us that if we have a gradual feeding plan that we stick with, we can avoid some of these symptoms and make faster progress in the long run. If we rush a baby to get more nutrition than he or she is ready for, sometimes we end up taking one step forward followed by many steps back.

So when we start feedings, what will we use?

Amy, Michelle, and Karen gave excellent perspectives on breastmilk and breastfeeding earlier this month. Breastmilk is so amazing and provides so many immune factors, micronutrients, special fats, healthy bacteria, and other benefits to these little ones. It is super easy to digest, and there are a number of studies out there that talk about how breastmilk can help to prevent some of the digestive problems that preemies and HLHS babies are known for. It’s truly customized nutrition that formula will never match!

For some moms, however, providing breastmilk may not be an option. Breast surgery, postnatal complications, certain medical conditions, some medications, and a variety of other factors may make breastfeeding difficult or impossible. Some moms choose not to breastfeed and that is okay, too.

Using donor breastmilk (from one of the nationally recognized milk banks) can be a good option for especially sensitive infants though it is rarely used long term. When breastmilk isn’t an option, there are plenty of formulas available for feeding infants with HLHS or for adding calories and protein to breastmilk (fortifying) when needed.

Some hospitals recommend standard formulas (like Enfamil Premium or Similac Advance) while others use semi-elemental or partially hydrolyzed formulas (like Pregestimil, Nutramigen, or Alimentum) in their feeding protocols. In some cases, even more broken down (elemental) formulas like Elecare or Neocate are needed. When we’re deciding which formula to use, blood flow to the baby’s belly, prematurity (and birth weight), GI problems or surgeries, and other medical conditions are all things we think about.

Are there cases where breastmilk or one of these formulas can’t be used?

As Michelle and Karen mentioned, chylothorax (or chylous effusion) can add an extra challenge when we’re talking about feeding babies with HLHS. Chylothorax can actually happen as a result of any surgery around the heart, and treatment almost always includes diet modification. The primary challenge is to find a way to get the long chain fat out of the baby’s diet while still getting him enough calories to grow. Unfortunately, long chain fat is the primary fat in breastmilk and infant formula.

IV nutrition is an option and may be used in some cases though, as I’ve mentioned, we try to feed into a baby’s tummy when we can. Skimmed breastmilk may be an option if enough fat can be removed; other formulas and special types of fat can then be added to the milk so that the infant has what she needs to grow.

If skimmed breastmilk isn’t an option, special formulas (like Enfaport, Monogen, and many others) can be used for oral or tube feedings until the drainage stops. Treatment usually continues for another two to 10 weeks until the cardiologist is confident that the area where the leak occurred is fully healed.

When can babies start to take feedings by mouth?

As babies recover, their tube feedings will increase and the breathing tube will come out. Once a baby is fully awake and his or her breathing is stable, we will start to think about letting the baby try some feedings by mouth- usually from a bottle.

Occupational therapists and speech language pathologists may evaluate if the baby is ready, able, and willing to take some nutrition by mouth. They will come up with treatment plans to help the baby progress to oral feeding as long as the baby is safe to do so. They observe babies as they eat and work with families to figure out how a baby is responding to feedings- is he sweating a lot, is her breathing rate increasing, is he working too hard, is she able to suck-swallow-breathe safely?

If a baby has a weak cry or is coughing or gagging with his feedings, swallow studies may be ordered to make sure that the breastmilk or formula isn’t going down the airway (aspiration). In some cases, positional feeding techniques are used to prevent aspiration. Sometimes feedings may be thickened (using baby cereal or other thickeners) if the baby is aspirating with thin liquids. For some infants, aspiration makes feeding by mouth unsafe and long-term tube feedings are needed.

With practice, the hope is that the baby will begin to take more and more nutrition by mouth relying less on the feeding tube. For some babies, eating causes a lot of stress so they may be limited to oral feeding a couple of times per day or for a small amount of time per feeding until they get stronger.

Once the team is confident that the baby is able to eat enough to stay hydrated, we will start talking about taking the feeding tube out. Some babies learn to take all of their feedings by mouth within a couple of weeks and others take much longer. Unfortunately, we don’t have great ways to predict who will eat well and who will not. When a baby can’t eat enough by mouth, but is otherwise ready for discharge, the team may recommend that he go home with full or partial tube feedings.

So how much nutrition do cardiac babies really need?

We have all sorts of ways that we can estimate how much nutrition someone may need- from babies and toddlers to older kids and adults. There are standard calorie levels for term babies without medical conditions as well as standards for premature babies. All babies are different and what works for one may not work for another. Ultimately, for HLHS babies, we rely on four things to give us hints about what a baby’s nutrition needs may be: research, previous experience, healing, and growth.

Research tells us that right after cardiac surgery babies have reduced calorie needs. The physical stress that their little bodies go through causes their metabolism to hibernate (sort of) for a week or so. After they recover, their nutrition needs increase, and, if they’ve been on bypass, they increase a lot.

Most research suggests that infants with HLHS often require upwards of 10 to 30% more than other term infants though there are infants who grow well on far less than we’d expect (and some who require even more). Energy needs depend upon how well the baby is able to eat, if he or she is able to absorb the nutrition their being given, how hard he or she is working to breathe, any other medical conditions, and much more.

We think of breastmilk as having about 20 calories per ounce on average though there can be a lot of variability. Standard formula dilution, with a few exceptions, is also 20 calories per ounce. For our HLHS babies, we usually find that breastmilk and formula needs to be mixed to at least 24 calories per ounce for babies to grow- but not always. Sometimes we will increase calories even further to 27 or even 30 calories per ounce to get babies to grow.

In addition to some of the formulas I’d mentioned, hind milk feedings, skimmed fat (from mom’s breastmilk), other protein, fat, and carbohydrate additives may be used to change the nutrition profile or increase the calories in breastmilk or formula.

Ultimately, the baby will tell us how much nutrition he or she needs to grow and thrive. All babies are a little different and, like medicine, nutrition isn’t an exact science.

What is normal growth for an HLHS baby?

Weight loss just after birth is normal for all babies. Usually, up to about 10% is expected with the goal of baby regaining his or her birth weight by two weeks of age. But what is normal for a baby who has had surgery or who has relied on nutrition support? It’s tough to say and can vary greatly.

Often, when we’re talking about weight gain for a newborn baby, we’re looking for about 20 to 30 grams per day (or about five to seven ounces per week) after the first two weeks of life. We’re also expecting newborns to grow in length by 1.5 to 2.5 centimeters (1/2 to 1 inch) per month. Again, these are general goals for all newborn babies; more weight growth may be normal for some babies, less may be normal for others, it depends.

Another consideration is to look at how proportionate a baby is using weight-for-length growth standards (charts). If a baby is gaining weight well but is not growing longer, he may appear chubbier than other babies his age. If a baby is gaining weight normally but is growing in length faster than most babies, she may appear thin. Despite the average standards we use, gaining 15 grams a day may be normal for some babies while gaining 40 grams a day may be ideal for others.

If an infant is getting enough nutrition, in theory these growth goals should be possible. Unfortunately, that still doesn’t mean that growth is easy or the same for all babies. Getting enough nutrition to show good growth before it’s time for another surgery is vital, and I know that HLHS babies (and parents) work really hard for every ounce.

Things to think about

1. If you want to breastfeed, start pumping early on to establish your milk supply and continue pumping 8-10 times each day. Babies usually eat every 2 to 3 hours so pumping on a similar schedule will remind your body to keep making milk even if you baby isn’t feeding just yet. Breastmilk can be frozen for about 6 months in a regular freezer but make sure to date it so you are sure to use the older milk first.
2. Right after birth through about a week or two after surgery, a cardiac baby’s weights often do crazy things. We see weight loss in the beginning, a lot of weight gain with fluids, weight loss again… it’s a rollercoaster! A couple of weeks after surgery is usually a good time to start looking at what your baby’s weight is doing. He or she may not be gaining weight by leaps and bounds, but the average trend should be positive.

3. Be patient. Even if everything goes perfectly, it takes awhile for an HLHS baby to get to full feedings (by tube or by mouth); if we rush the process, we often have to backtrack. Keep in mind that when babies are born, it takes time for them to learn to eat. HLHS babies have some extra challenges including higher nutrition needs, different breathing requirements, a heart that’s working with fewer parts, digestive problems, recent surgery, etc.

4. A weight gain goal of 20 to 30 grams per day is a guideline but may not be the right amount of weight gain for all babies. Looking at weight for length on growth charts can be a really helpful way to make sure that a baby is gaining weight proportionately over time. Your pediatrician (or dietitian) has these growth charts if you want to see them.

5. All babies are different and it’s always okay to ask questions! If you haven’t met a dietitian and would like to- just ask! If you have questions about your baby’s nutrition or other medical conditions- just ask! We’re all here to help.

Alex Tchouchev 3/7/13 Alex Tchouchev 3/7/13

Breastfeeding the HLHS Baby - the Practitioner Perspective

Nutrition Series

Michelle Steltzer, CPNP-AC, Cardiovascular Program Nurse Practitioner

On Monday, one our Sisters by Heart moms, Amy, shared the parent perspective on the question of whether it’s possible to breastfeed an HLHS baby. Today, we are fortunate enough to have two seasoned practitioners from Boston Children’s Hospital provide their perspective on breastfeeding an HLHS baby!

Michelle Steltzer, CPNP-AC
Cardiovascular Program Nurse Practitioner
Michelle is currently working on publishing a case study on a breastfeeding HLHSer who transitioned to fully breastfeeding during the interstage period with excellent growth.

It is an honor to share my perspective with Sisters by Heart and I truly appreciate all the work that you do each day to reach out, connect, and support families with HLHS. As mentioned, the congenital heart disease population is near and dear to my heart having worked many years as a bedside nurse in the ICU and in 1999 as a cardiology nurse practitioner in the ICU/ward at Children's Hospital of Wisconsin/Medical College of Wisconsin (1993-2007). I transitioned to a cardiovascular surgery nurse practitioner on the ward at Boston Children’s Hospital 5 years ago. Through the years, I have written a lot about nutrition, spoken at national and local conferences, and gained much experiential practical knowledge at the bedside with amazing insight provided by great patients and families like you folks struggling with feeding, growth, and nutrition. I am happy to share my perspective with you and trust you find it useful.

On a daily basis, I am inspired by the dedication and passion of so many families that ensure the benefits of breast milk are available for their infant via breast feeding, expressed breast milk by bottle, and/ or other tube feedings. As a mother of two children that breastfed both infants until going back to work, it is no simple request for any parent. Breastfeeding is a lot of hard work and requires much dedication and support by the entire family to ensure success. Currently, as Amy mentioned, I am working on writing up a case study that will give a detailed description of one mom’s successful transition to breastfeeding during the interstage period. To give you perspective, I walked to the patient’s bedside following morning rounds report by my NP colleagues after not seeing this particular infant since the initial newborn surgical hospital stay. Upon seeing the infant, I promptly walked out of the room thinking to myself, “This patient cannot possibly be the patient advertised during sign out by my peers that recently had a Glenn surgery. I must be in the wrong patient room.” Needless to say, I was completely wrong in my presumption and will share more details in a journal publication in the future about this practice changing experience.

One key to success, particularly in the CHD population, is reaching out and truly engaging all members involved in your infant’s care team that is rallying for successful growth and nutrition. This includes: first and foremost the family, pediatrician, referring and primary cardiologist, hospital teams (prenatally, postnatally in CICU, floor wards, and follow-up clinic visits), early intervention, speech/feeding therapy, and cardiac developmental centers available at your institution/region. These team players (from nursing, lactation, nutrition, MD, therapists, etc) along with family dedication are essential to ongoing success with breastfeeding for this high risk and fragile population - particularly during the interstage period. This period is often described as a very isolating experience for the family, particularly for a dedicated breastfeeding mother. Thoughtful discussions with mothers and families throughout all care opportunities with health care providers are critical to ensure the best outcome for the infant and family.

As Amy mentioned, breastfeeding and oral feeding in general is a lot of work and a "stress test,” particularly for infants with HLHS. Realizing this at baseline is important because there can be additional complicating morbidity factors that may impact the stress of feeding. Some of these concerns are: poor ventricular function, valve regurgitation, breathing fast due to too much blood flow or other respiratory concerns, vocal cord injury, oral aversion, reflux, other non-cardiac anomalies, and other morbidity factors that may contribute to an infant's failure to thrive and grow. Having said that, these factors should not limit the decision to utilize breast milk and all its beneficial immune properties.

Discussions with health care providers about breastfeeding and utilizing your precious breast milk is essential. It is also important to note, that there are some special cases such as chylous effusion (milky appearing fluid accumulating or draining around the lungs), milk protein allergy, or guiac positive (bloody) stools that may require alternative supports for nutrition. In these instances, a conversation about alternative options and adjustment in the breast milk diet may need to occur in the short term period.

Practical 5 Pearls of Wisdom to Share with Moms Interested in Breastfeeding
1. Discuss prenatally and postnatally your thoughts on breastfeeding. On a daily basis bring up your desire to encourage breastfeeding and connect with all your lactation resources early.

2. Keep your breast milk supply up drinking proper fluids and frequent pumping at least every 3 hours. If concerned, please reach out to your lactation consultant early. This way you will have plenty of that nutrient rich milk with all its beneficial immune properties when your baby is ready. *Please see below our lactation consultant expert from Boston Children’s Hospital, Karen Sussman-Karten’s input for other pearls of wisdom on breast milk supply and “lactoengineering.”

3. Practice patience and persistence. Never miss an opportunity to practice. Try to breastfeed or even just work on non-nutritive suck if your team agrees during the inpatient stay (preoperatively if possible) and outpatient. In some cases, you may need to be persistent in your requests to try breastfeeding, but don't be shy in speaking up for your baby. Once your child is on bolus feedings, there are at least 8 feeds at least in a day and thus 8 times to practice daily with the health care team and ultimately the primary feeders of the infant after discharge.

4. Advocate and utilize the resources available to you in the home surveillance monitoring program (saturations, heart rate, daily weights, and logging the number, volume, and length of feeds-particularly if breastfeeding).

It continues to be an issue among some institutions that insurances will not cover scales. Advocate early with your primary team, ask for scales that weigh to at least 10grams (there are many on the market), and if possible consider scales that weigh to <10 grams.
Communicate honestly with your providers about how the breastfeeding and growth is progressing. It is important to share with your providers if you are noticing fatigue, sweating or diaphoresis during feedings because these may be a sign that your baby is working too hard to feed to adequately grow and thrive.
Avoid feeding (by bottle or breast) longer than 20-30 minutes to minimize losing more calories during the feeding process than actually gaining. There are some weeks where you may need to add more bottle feeds due to your family circumstances or or infant’s illness. Make sure to pay particularly close attention to your baby’s progress with your home scale. These periods may warrant more detailed intake and should not be thought of as a failure to breastfeed successfully.
Remember the nature of shunt dependent blood flow in HLHS heightens your infant’s vulnerability during the interstage period, and this is the primary reason why home surveillance monitoring was introduced over a decade ago. In some cases, your infant may require to be seen by your local providers (cardiology or pediatrician) or visit the emergency room for reassessment of plan of care. Some instances may require readmission for closer monitoring of feeding and cardiovascular status. This is by no means a failure on your part, but the “nature of the disease process of HLHS.”

5. Be practical with your expectations. Don't expect your baby to fully breastfeed by the time of first discharge following stage 1 palliation (whether it is Norwood with BT shunt, Sano with right ventricular to pulmonary artery homograft, or Hybrid). Lastly, please remember that you are not alone, take care of yourself, and never give up!

Karen Sussman-Karten, RN, IBCLC
Karen is a Lactation Consultant in the Lactation Support Program at Boston Children’s Hospital and is currently the Board Chair of the Mother’s Milk Bank of New England.

Amy and Michelle have shared so much valuable wisdom about breastfeeding and providing breastmilk for CHD babies. I have been a lactation consultant at Boston Children’s Hospital since 2006 and I have a few more thoughts for you.

Having adequate milk production is the foundation for success. Without it your options are limited. Since your baby will probably not be able to breastfeed in the first few days, expressing milk is essential to establish milk production. It’s important to start as soon after delivery as possible. Pumping frequently, every 2-4 hours is recommended. You may be able to collect more milk with manual expression in those early days. “Hands on pumping,” massaging and compressing while using a breast pump, can also result in collecting more milk.

Think of your early breastfeeding attempts as practice sessions. Don’t expect your baby to drink large volumes at the breast initially. Try not to get discouraged; practicing will lead to improvement over time and the skin to skin contact can help with milk production and breastfeeding.

Once your baby seems to be drinking at the breast, doing a pre and post feed weight to measure intake can be helpful, particularly in the hospital when you have access to use a very accurate scale for pre and post weights. As mentioned by Amy, our parent expert, most baby scales intended for daily weight checks are not sensitive enough to weigh less than 10 grams. Of course purchasing an accurate scale can be costly. You may want to consider renting one like the Medela baby weigh scale for home based on your infant’s progression with breastfeeding. It’s very important to keep your baby in the same clothing and diaper for the pre and post weight. Changing the diaper or weighing your baby naked will not give accurate results.

“Lactoengineering” refers to ways of altering the breast milk or breastfeeding to meet your CHD baby’s specific needs. Since getting the most calories is a major concern, there are some techniques you can use to increase caloric intake. The caloric content of your breast milk can be tested using a creamatocrit machine or milk analyzer. Some hospitals have this equipment. The assumption is that breast milk has 20 calories per ounce but when tested it is often higher. This becomes important if your infant’s EBM is being fortified above 20 calories per ounce, potentially up to 26 or 28 or more calories per ounce. Some infants do not tolerate the additives for a higher calorie diet so it can be very helpful to know the general caloric density of your breast milk as a baseline. Sometimes the additives can be reduced or eliminated depending on the results. Beware, the fat content of your milk varies throughout the day so testing once is like a snapshot of the calories per ounce.

Another interesting phenomenon is that the fat content of your milk increases toward the end of a nursing or pumping session. If you make more milk than your baby drinks, you can use more of the fat rich “hindmilk” to increase the calories. You can pump for the first 3 minutes and freeze that “foremilk” for later use. Then either nurse your baby or pump the remainder of the “hindmilk” and use only that.

If your baby has a chylous effusion, they cannot tolerate the fat portion (long chain fatty acids) in breast milk. It is possible to separate the fat from your milk and use the “skimmed” breastmilk. This can be done with a centrifuge machine or with other methods of allowing the milk to separate and removing the cream layer. This “skimmed” breast milk will need to have other oils and nutrients added to make up for the calories lost by removing the cream. This allows your baby to continue receiving your milk rather than a special formula while they have a chylous effusion.

Thank you so much, Michelle and Karen for your helpful insights into breastfeeding a child with a complex congenital heart defect like HLHS!

Stay tuned for next week’s post in our nutrition series, “The Cardiac Newborn!”

Alex Tchouchev 3/7/13 Alex Tchouchev 3/7/13

Breastfeeding the HLHS Baby - the Practitioner Perspective

Nutrition Series

By: Michelle Steltzer, CPNP-AC, Cardiovascular Program Nurse Practitioner

It continues to be an issue among some institutions that insurances will not cover scales. Advocate early with your primary team, ask for scales that weigh to at least 10grams (there are many on the market), and if possible consider scales that weigh to <10 grams.
Communicate honestly with your providers about how the breastfeeding and growth is progressing. It is important to share with your providers if you are noticing fatigue, sweating or diaphoresis during feedings because these may be a sign that your baby is working too hard to feed to adequately grow and thrive.
Avoid feeding (by bottle or breast) longer than 20-30 minutes to minimize losing more calories during the feeding process than actually gaining. There are some weeks where you may need to add more bottle feeds due to your family circumstances or or infant’s illness. Make sure to pay particularly close attention to your baby’s progress with your home scale. These periods may warrant more detailed intake and should not be thought of as a failure to breastfeed successfully.
Remember the nature of shunt dependent blood flow in HLHS heightens your infant’s vulnerability during the interstage period, and this is the primary reason why home surveillance monitoring was introduced over a decade ago. In some cases, your infant may require to be seen by your local providers (cardiology or pediatrician) or visit the emergency room for reassessment of plan of care. Some instances may require readmission for closer monitoring of feeding and cardiovascular status. This is by no means a failure on your part, but the “nature of the disease process of HLHS.”

Thank you so much, Michelle and Karen for your helpful insights into breastfeeding a child with a complex congenital heart defect like HLHS!

Stay tuned for next week’s post in our nutrition series, “The Cardiac Newborn!”

Alex Tchouchev 3/4/13 Alex Tchouchev 3/4/13

Breastfeeding the HLHS Baby - the Parent Perspective

Nutrition Series

By: Amy Bennet, Parent

March is Nutrition month! In honor of Nutrition month, we’ve created a special blog series on nutrition. We’re kicking off the month with a post on breastfeeding, written by one of our own Sisters by Heart moms, Amy, mom to Bodie. We will follow it with a series on nutrition issues faced by our HLHSers, all provided by Emily LaRose, Dietitian at Children’s Hospital of Los Angeles. Without further adieu, Happy Nutrition Month – and happy breastfeeding!

“Will I be able to breastfeed my baby?” This question gets asked SO often, and the answer is not terribly straightforward. I wanted to take a few moments to answer this question from the perspective of a mom who’s been there and wondered the exact same thing.

I should mention that I’m a huge fan of breastfeeding. I breastfed my heart-healthy (now 5-year old) daughter until she self-weaned right around her 2nd birthday. So, breastfeeding is important to me. It was something I desperately wanted to do with my HLHSer, but, for a myriad of reasons, was unable to. I ultimately pumped for him for 10 months and had enough breast milk to last him until 15 months of age. For most HLHSers, breastfeeding, at least exclusively, simply isn’t feasible. There are a lot of factors working against breastfeeding our special kiddos. The rare mom who is able to exclusively breastfeed is a bit like an exotic animal observed in the wild, while the rest of us stare and whisper in awe. BUT it is possible for some mothers, and partially breastfeeding is possible for many more. If breastfeeding is important to you, by all means, try to make it work! Here are some things to keep in mind:

Monitoring
Many hospitals follow an interstage monitoring program, which requires parents to track specific data points (i.e. oxygen saturations, heart rate, weight, intake). Thus far, monitoring programs are reducing interstage mortality. Feedings are tracked closely interstage, since even slowing down slightly during feeds raises a red flag for practitioners. The ability to monitor the exact amount of “ins” for your baby is crucial – an easy task when armed with a bottle, but obviously more difficult with breastfeeding. For this reason, some hospitals discourage breastfeeding. Some doctors are uncomfortable with how difficult it is to measure feeds done via breastfeeding. But it’s not impossible! You can overcome this obstacle if your insurance approves a scale, by purchasing a baby scale, or renting one yourself weighing your baby before and after each feed to calculate the exact amount of breast milk taken. It’s more challenging, but definitely not impossible. There are some scales that weigh to smaller increments, but are also much more costly. Finding one that weighs to 10 grams is often reasonable enough for most families to track the daily weights.

Weight gain and cardiac function
This is probably the biggest obstacle to exclusively breastfeeding an HLHS baby. One of your primary goals interstage is to grow your HLHSer big enough to be ready for his or her second stage surgery, either the Glenn or the Hemi-Fontan. Many interstage HLHSers struggle with growth because their hearts are working so hard. It’s very common for interstage babies to receive supplemented formula or breast milk for higher caloric concentration. In my case, I pumped, and my son was not able to gain weight on the calories in my breast milk alone, so my pumped milk was fortified with a high calorie formula. If your baby cannot gain weight on the calories in breast milk alone and fortification is necessary, exclusive breastfeeding simply may not be a possibility, at least until after the second stage surgery when growth is typically less of an issue. But, that doesn’t mean you can’t breastfeed at all. I do know a number of HLHS mothers who’ve breastfed a few times a day and fed with fortified formula for the remainder of their feeds. If this is the only thing stopping you, definitely check with your cardiologist to see whether this might be a possibility.

Related to weight gain, it takes a lot of work to eat, whether by breast or bottle. For some babies, particularly those whose cardiac function is depressed, it can be really hard to gain enough weight on oral feeding, even if it’s supplemented, because their hearts are simply working too hard. For these babies, tube feeds are necessary to allow the baby to grow without having to work for it. But again, that doesn’t mean you can’t pump breast milk and tube feed your baby, so that they can still get the immune boosting benefits of breast milk.

Swallowing
Another thing that can sometimes play a major role in whether or not you’ll be able to feed is whether your baby has any swallowing issues. In my son’s case, his left vocal cord was paralyzed during his Norwood (not an uncommon complication following open heart surgery where work is done on the aorta, as the nerve controlling the vocal cords run alongside the aorta – due to the extensive amount of aortic reconstruction during the Norwood, this complication is seen). Vocal cords are what protect the baby’s airway. For a baby with vocal cord paresis, the paralyzed vocal cord lacks the ability to adequately protect the airway and, depending on the severity of the paralysis, oral feeds may need to be thickened using a thickening agent or avoided altogether. In either case, although breastfeeding is not safe for the baby until either the affected cord heals or the remaining cord compensates, you can still pump breast milk and feed it to your baby via bottle or feeding tube.

Feeding Difficulties
Many of our HLHSers have difficulties with feeding in general. Often, these difficulties come in the form of oral aversions, or difficulty coordinating sucking, swallowing and breathing simultaneously. There are many factors that can cause this. For babies at institutions that do not allow oral feeds prior to the Norwood, sometimes, by the time the baby has had surgery, been extubated and finally given a chance to take a bottle or breastfeed, the newborn’s sucking reflex is gone and they quite literally do not know what to do with a bottle or breast. Or they can suck, but can’t quite coordinate their swallowing and breathing effectively. For others, particularly those on the ventilator for prolonged periods of time, they may associate things being in their mouth with discomfort and pain, and oral feeds are quite challenging. In both of these cases, breastfeeding is unlikely; however, there are a few things you can do to try to work through this and enable your baby to take a bottle of pumped breast milk.

In Sisters by Heart care packages, we include newborn pacifiers, and encourage parents to use them with their newborn prior to surgery and until the baby is permitted to attempt oral feeds. While it will not guarantee a baby will retain that sucking reflex, it can help keep the baby in touch with that sucking reflex. You can also ask your medical team to intubate through the nose, rather than the mouth, so that the baby can take a pacifier even while intubated. (Some, but not all, institutions can comply with the request, depending on your child’s situation.) And, definitely, seek out occupational therapy, lactation specialists and feeding therapist as soon as possible at your surgical center because they are often very practical at providing a lot of strategies to encourage oral feeding and breastfeeding.

As you can see, the question of whether or not you’ll be able to breastfeed your HLHSer, or any complex CHDer for that matter, can be a loaded one, dependent on a lot of factors. Yes, the odds are stacked against you being able to exclusively breastfeed, but that doesn’t mean it can’t happen; many mothers are able to pump and alternate breastfeeds with bottle feeds. If this is something that’s important to you, then push for it. Let your medical team know upfront that you intend to breastfeed (I told my entire medical team while I was still pregnant, in fact!).

And if breastfeeding simply isn’t an option for any of the reasons mentioned above, pumping is almost always an option – so, to those of you moms who fall into this category, I say pump, pump and pump away!!! The benefits of breast milk are huge, regardless of the source: breast, bottle or feeding tube. It’s not easy to pump exclusively, but take it from someone who’s been there, in your shoes – it is absolutely worth it! Do what you can to keep your supply up and pump as long as you can – whether it’s 1 day, 1 week or 1 year, your CHDer will receive benefits!

Many hospitals have resources dedicated to lactation support for cardiac patients, so make sure to ask! For instance, Children’s Hospital of Philadelphia provides a lot of great information on breastfeeding a cardiac patient here!

Another hospital currently focusing on lactation support for cardiac patients is Boston Children’s Hospital. Stay tuned for Thursday’s post, where two practitioners from BCH share their input on this important topic!

Thank you, Amy, for sharing your insights with us!

Alex Tchouchev 2/20/13 Alex Tchouchev 2/20/13

Practitioner's Perspective: We Can't Make It All Better

Darci Walker, PsyD, Founder of Core Parenting

Parenting an HLHS child is, well, different. HLHS brings an entirely different set of parenting challenges and struggles. Sisters by Heart is grateful to Dr. Darci Walker who helps explain the struggles we may face, from a psychologist's perspective. Thank you, Dr. Walker, for lending your most insightful thoughts to our HLHS families.

This child. This parent. This moment.

I remember the moment I stepped outside of the hospital for the first time with my firstborn son. I remember a feeling of sheer terror. I looked at my husband, and he looked at me, and we looked at this little thing in between us, and one of us actually said, “Really? They are just going to let us walk out of here with him? What the hell do we do now?”

The enormity of the future weighed so heavily in that moment. My job seemed impossible. I was unprepared and unqualified and unsuspecting. I needed to protect him. Keep him safe. Keep him healthy. Make sure that the world is safe and secure and he has all the goodness he deserves.

I knew, in that moment, that I was completely incapable of actually ensuring that his road would be completely smooth and that nothing bad would ever happen to him. And that realization hit me like a ton of bricks.

But, I was lucky. He was healthy and I was able to seek comfort in the fact that I could worry about impending doom another day. Right then, he was okay. I could breathe and let myself believe that I had more control than I really did. I would have some time to figure out what parenting is really all about. I could wrap myself in a delusion of control. Like the myth that my very kiss could make an owie disappear. And, somehow, that myth helped me through the early stages of parenthood.

Of course eventually, for me as for all parents, the myth would shatter, and the true depth and challenge of what parenting really is would become clear. For many parents it’s the first time your child gets hurt. Or bleeds. Or has an incredibly high fever. Or gets lost. Or gets bullied by someone. This is when some parents really figure it out, when the real truth of parenting sinks in. The powerlessness of it all. The truth that this little human, that came out of our bodies, is really on their own path. We are not in control of their lives and we can’t protect them from all things. No, our job is not to kiss away the owie. Our job is to be with them, no matter how painful the owie. Parenting is about walking impossible paths and tolerating impossible pain and supporting our kids through impossible limits. It’s not about making it go away. Because we can’t. It’s not about making bad things not happen. Because we can’t always do that either. It’s about being by their side. This child. This parent. This moment.

It’s in these moments of utter pain that we realize that all we can do is do the best we can to stay beside them and love them and give them every ounce we have, but in the end, every ounce won’t really be enough to eliminate all pain and suffering. And while this is really true for all children from day one, many of us don’t have to learn the agony of that realization for some time.

But, other parents have to hit the ground running. Parents who have babies born prematurely or who have babies with medical issues, like children born with hypoplastic left heart syndrome, never have a chance to feel this calm before the storm. Parents who have babies that move from the delivery room to the NICU faster than a blink of an eye never have a chance to wrap themselves in a false sense of control and comfort that they can make everything all right.

Right from the start, these families are forced to watch from the backseat as their little person travels on their own path, surrounded by doctors and machines and tubes and acute needs. Right from the start these parents have to live with the truth that they can’t make it all better. That their kiss doesn’t really make the owie go away. And that realization is heart wrenching. There is so much anxiety, fear, depression.

These little people have a tremendously difficult road ahead of them. Their families have enormous pain to endure. And yet, there remains: This child. This parent. This moment.

If we can let go of our myths about what parenting means. If we can let go of our fears about the future. Just for a moment. Then we can realize that what this child, what any child, needs in this moment is a parent who can tolerate the pain and be there with them. By their side. Walking this path, this painfully difficult path. Holding them, rocking them, touching them, fighting for them, singing to them, smiling at them, crying with them, reading to them. That is parenting. This is parenting. This moment of connection and support and touch is parenting.

I worked with a family whose infant son died hours after birth. While they knew that their son would die soon, they knew they had a very important role in his life. They held him, they rocked him, they sang to him. They talked to him and whispered in his ear and cried with him as he died. This is parenting. This child. This parent. This moment.

I can’t claim to understand the enormity of having a sick child. But I do know the difficulty of letting go of my expectations, my feelings that I have somehow failed my child, my feelings of frustration with a world that is unfair and harsh and cruel. I do know the pain of watching a child struggle with something that I can’t un-do. And I do know that sometimes, when all I want to do is crawl into my bed and hide from it all, what I need to do instead is just go be there with my child.

And I also know how amazing it feels when I can truly be there in the moment for my child. This child. This parent. This moment.

My child and me. Now. Touch, hold, connect, support.

That is parenting. I do know that the feeling of connection and strength and beauty that comes out of it is the most important thing in both our lives. Because while the kiss can’t make the owie go away, the connection in that kiss can give the child the support he needs to know that he can take the next step. What a gift. Parenting in its most pure and true sense.

Dr. Darci Walker, is a mom to two boys and Clinical Psychologist with experience working with families, individuals and children in a variety of settings. In 2010 she co-founded Core Parenting in Portland, Oregon and specializes in working with parents and the variety of transitions that occur during this amazing stage of life. From postpartum depression to relationship difficulties, career changes to identity reformation, Dr. Walker is interested in the multiple layers of challenges and joys that parenting brings and loves working with both men and women as they negotiate through these processes, highlighting her belief that thriving parents lead to thriving children! Read more at Dr. Walker's Blog and on Facebook.

Alex Tchouchev 10/22/12 Alex Tchouchev 10/22/12

A Practitioner's Perspective: Choosing a Pediatrician/Vaccinating your HLHSer

By: Jennifer Melnychuk, MD ("Dr. Jen"), All Star Pediatrics Exton, PA

To introduce myself, I am a pediatrician in private practice in the Philadelphia suburbs. I have been in private practice for over six years. Prior to that, I completed my residency training at the Children’s Hospital of Philadelphia. My experience with children with congenital heart disease has come from both caring for patients in the hospital as well as in the out-patient setting.

Awaiting the arrival of a new baby is such an exciting and scary time for any family but even more so for parents of children with heart disease. Not only do you have to research a pediatrician for your newborn, but you have to select a cardiologist, cardiac surgeon, birth hospital, etc. It only made sense that the Sisters by Heart website wanted to offer expecting parents some advice when selecting a pediatrician and I was so pleased when they asked me to weigh in.

While you will be spending a lot of time with the cardiology staff, your general pediatrician will also play an important role in your child’s care. Your pediatrician should be familiar with complex congenital heart disease from diagnosis to post-op care to long-term follow-up. You should ask where your pediatrician trained, as certain medical centers specialize in cardiac care. He or she should be board-certified in pediatrics which means they trained at an accredited pediatrics program, passed the board exam and maintain educational requirements and quality improvement projects. In a busy pediatric office, your pediatrician should allow extra time for your child’s visit, including special attention that should be paid to growth and development. You will have lots of questions (and you should never be afraid to ask a question!) and you want to make sure your doctor has the time to answer them all. Your doctor should also stay updated on your child’s specialty care and have a contact through the medical center to remain informed.

Finally, you should ask how you can contact your pediatrician as concerns arise both when the office is open and after-hours. Your pediatrician should respond to your questions the same day and you should be familiar with the other providers (and they should be familiar with your child’s care) in the practice for when your pediatrician is not in the office. You should have easy access to a physician after-hours as well for emergencies.

The rest of the staff including the front desk staff, the nurses and the billers all play a role in your child’s care as well. The front desk staff should be aware that your child has complex needs - they may schedule appointments when the office is less busy or have your child brought back to an exam room immediately so your child is exposed to less illness in the waiting room.

The nursing staff who are answering questions on the phones during office hours should also know that your child has heart disease. For example, the nurses may give different advice about a cough or a fever for a patient with complex medical needs. The nurses should be knowledgeable about your child’s medications as they will often be refilling them. Your child likely will need many referrals for specialty care which the nurses or a referral coordinator will help you with.

The billing staff will be crucial to help you navigate insurance issues as they arise. Certain states provide supplemental insurance for children with complex health needs and your pediatrician’s office should be familiar with those options and accept those insurances.

My final thoughts involve the importance of vaccination for all children, but especially those with chronic medical conditions. Children with heart disease are particularly vulnerable to complications of many illnesses and we need to protect them as much as possible.

Illnesses that attack your child’s lungs require special attention for children with heart disease. While influenza (“the flu”) may just cause fevers, body aches, and a lingering cough in some children, a child with heart disease could end up in the intensive care unit from the same illness. Your pediatrician’s office should have a system in place to prioritize your child (and his or her siblings) to receive the flu vaccine as soon as it becomes available.

Respiratory Syncytial Virus (or RSV) can also be devastating to certain groups of children like those with heart disease or infants that were born premature. Unfortunately, no vaccine provides long-term immunity against this illness. Children who are at highest risk are recommended to receive monthly injections of antibodies to protect against this illness during the peak season – typically from October to early Spring. Again, your pediatrician’s office should coordinate this for you. Please make sure you have a contact person in the office to assist in insurance coverage for this very expensive immunization and to assist in setting up your monthly visits to receive the injection.

You have probably heard that pertussis (or whooping cough) has been making a comeback recently. This past year has seen a significant rise in the number of cases due to a number of factors including groups of families who chose not to immunize their children and adults losing the protection of the vaccines they received as children. Please make sure your pediatrician is up-to-date on the latest recommendations on immunizations from the American Academy of Pediatrics, as changes can come up.

Please also insure that any person that has frequent contact with your child also has received his or her vaccines – like the flu and whooping cough (Tdap or DTap) – including you, grandparents, daycare providers and even the staff that works at your pediatrician’s office. It is also wise to ask about your pediatrician’s office policy on vaccination. Recently, more offices are asking families that chose not to vaccinate their children to find another provider. This is primarily to protect other children in the office. While no vaccine provides 100% protection, you want to assure that the other patients in the waiting room are fully vaccinated so diseases like the flu, whooping cough, and measles are not spread by visiting your doctor.

While there is so much to think about, always remember your child is a blessing and will provide you with countless joy. Good luck!

Jennifer Melnychuk, MD ("Dr. Jen")
All Star Pediatrics

Thank you Dr. Jen for sharing your experience in treating complex cardiac patients and offering your insights into caring for them. We, at Sisters by Heart, greatly appreciate your time and contribution to educating our HLHS families on the importance of vaccinations and guidance on choosing the right Pediatrician for their child. We know our families will be equally as appreciative for your time and expertise!

Helpful Tip: Most Pediatric offices will allow expecting parents to set up an appointment with one of their Pediatricians to give them an opportunity to ask questions and find out more about the practice. Here is a list of questions from the JCCHD Parent Page (suggested by HLHS parents) you can ask when meeting with a Pediatrician. As Dr. Jen mentioned above, your child's Pediatrician is an integral part of his or her care team - it's important that the docs and office staff accommodate your needs, especially during interstage. If they are not willing to do so, you may want to keep looking!

Alex Tchouchev 9/20/12 Alex Tchouchev 9/20/12

Practitioner’s Post: The Promise of Stem Cells

By: Sunjay Kaushal, M.D., Ph.D., Director of Pediatric Cardiac Surgery at the University of Maryland Medical Center

Dr. Sunjay Kaushal is a cardiothoracic surgeon with a passion for reconstructing the hearts of those battling critical congenital heart disease. During reconstruction, Dr. Kaushal also looks at the pediatric heart with an innovative eye. In talking to Dr. Kaushal, it’s clear that he’s dedicated not only to reconstructing the hearts of our babes, but also in finding ways to improve their lives for many years to come.

Thank you, Dr. Kaushal, for taking time out of your busy days to share your research and trials with Sisters by Heart and our families. There is so much hope in medical advancements and your continued work.

A few years ago, Dr. Kaushal began studying the positive outcomes with stem cell therapies, in the adult population, following damage to the heart after ischemic events (i.e. heart attacks.) He asked himself a simple question: If stem cells are assisting in the regenerative process for adults, can we use similar therapies for children? The short answer, he found, is yes.

Over the past year-plus, Dr. Kaushal preliminarily proved his theory – that infant heart tissue containing specific stem cells has the ability to: 1) survive outside of the body, 2) grow and multiply (yes, like Gremlins) and, 3) assist in recovery and strengthen heart function when injected into the heart. How did he prove this theory, you ask? He did it by testing harvested human heart tissue from 150 HLHS and CCHD patients and injecting them into animal models. Armed with quantitative information and data, he presented to the FDA for approval of a new clinical trial which is slated to begin soon at University of Maryland Medical Center.

Essentially, the clinical trial looks like this:

During the Norwood operation, heart tissue taken from the right atrium, which would routinely be discarded post cannulation, is instead banked for future use on that same patient. The harvested heart tissue is then grown to duplicate the patient’s own stem cells for reintroduction to the patient’s heart. Once expanded, the cells are placed back into the heart during the Glenn/hemi-Fontan operation via injection into the coronary arteries just prior to coming off bypass. The infusion of stem cells takes less than 4-5 minutes to complete.

The purpose of Dr. Kaushal’s trial is to determine how effective these specific stem cells are in regenerating heart tissue and strengthening the heart muscle/function in single ventricle patients. Obviously, a major risk factor with HLHS patients is heart failure. The theory is: inject the heart with its own, younger stem cells and the heart will regain a large percentage of function. So, is this possible? Dr. Kaushal believes so. He believes that based on the adult studies performed to date, the trial is both safe and effective. In the adult-based study, heart function improved by 12%. For those of you who are familiar with the term “ejection fraction,” the EF improved by 12%. That is a staggering improvement. Not only did the study show effectiveness, but it illustrated that the stem cells continued to work over a year’s time. At 4 months, the heart function/EF improved by 4%, at 8 months, 8% and over a year’s time an overall improvement of 12%. Dr. Kaushal cautions that we must remember that this is a Phase I study, which only examines safety issues. Resident stem cells will need to be evaluated for efficacy in a larger Phase II study that will be appropriately powered to eventually determine their efficacy.

Even more exciting… when Dr. Kaushal compared his preliminary results in children to that of an adult heart, he’s finding that the infant cell models outperform the adult models “3 to 1.” Therefore, the younger the stem cell, the more potent and effective it is in repair and regeneration.

Why does it work?

Researchers aren’t exactly sure. Dr. Kaushal will attempt to answer that question during his clinical trials with HLHS patients. The two thoughts include: 1) the new stem cells are secreting good proteins and therefore are improving function and/or 2) the new stem cells assist in preventing stress to the older cells. Either way, it’s promising news for parents of children with HLHS or any CCHD.

Imagine if surgeons and physicians can inject stem cells into the hearts of our children and dramatically improve heart function… We know we got the chills when thinking of the possibilities…

Dr. Kaushal’s trial is awaiting hospital approval. He’s also investigating the possibility of securing funding from both the public and private sectors so that parents can enroll their children free of charge.

How will we know if the clinical trial works?

Dr. Kaushal’s proposed clinical trial will enlist 30 HLHS patients and follow them over a year’s time, post-stem cell infusion at Stage 2 palliation. He will conduct a baseline MRI to measure function the morning of Stage 2. After Stage 2 and the injection/infusion of stem cells, he’ll perform repeat echocardiograms and MRIs at 6 and 12 months post-op to compare the heart’s performance and regeneration. No other tests are needed. Dr. Kaushal pointed out that the right heart (i.e. right ventricle) is “not normal” and very hard to assess on echo and therefore, the MRIs are needed to gain an accurate measurement.

Can my older HLHS child or HLHS adult benefit?

Yes. Plain and simple. Dr. Kaushal’s hope is that, once approved, his trial will illustrate and prove the theory that stem cell therapy is safe and effective for single ventricle infants and children. If that is the case, if a child or adult with HLHS, “down the road” experiences dysfunction, they can receive stem cell injections from banked/frozen cells. The frozen stem cells, after thawing for 2 months, are just as potent as they were before freezing.

What do we know now?

Data for stem cell therapy in infants and children living with HLHS is very scarce. Dr. Sano (who developed the Sano shunt for a modification to the Norwood) is conducting similar research in Japan but is using a different type of stem cell (still from heart tissue.) Dr. Sano is injecting cells into heart muscle at one month of age, shortly after the Norwoodprocedure.

Additionally, researchers in Germany have successfully used bone marrow cells in HLHS children. They’ve injected 10 CCHDers (not all HLHS) with autologous bone marrow stem cells and are seeing dramatic improvements in function. In fact, one child – an HLHSer in heart failure and listed for transplant – was injected with bone marrow cells while on the transplant list. The parents were willing to do anything to avoid transplant. And another lesson in asking many, many questions comes into play. They talked to doctors about their options and were told about the experimental bone marrow cell infusions. The parents agreed to the infusions and were ecstatic at its results. The child’s heart function improved significantly and subsequently, the child was removed from the transplant list. Based on his conversations with German researchers, Dr. Kaushal is gearing up for a similar study here in the United States using bone marrow cells for treating HLHS and dilated cardiomyopathy patients with depressed function.

What if I didn’t bank my child’s cord blood/tissue?

Don’t panic. The body is full of stem cells that can be harvested. Dr. Kaushal emphasized that there are no current treatments for HLHS, or any CCHDs, utilizing umbilical cord blood stem cells, although there are current studies ongoing at Duke University regarding the use of umbilical cord blood therapy for HLHS patients. Dr. Kaushal recommends, if you have the resources to bank cord blood/tissue, go ahead and bank. Medicine evolves and you never know what options will be on the table 5, 10, 15 years from now.

Dr. Kaushal and his team hold much enthusiasm and cautious optimism about their new clinical trial which, once approved to begin enrolling patients, could change the landscape of medical treatment for HLHS infants, children and even adults.

Trust us when we tell you, Dr. Kaushal, we’re ALL cheering you on and sending all the positive energy we can muster. Our children’s lives depend on it.

Watch Dr. Kaushal’s discussion on his clinical trial: http://www.umm.edu/media/video/kaushal-circ-study.htm

Sunjay Kaushal, M.D., Ph.D. is the Director of Pediatric Cardiac Surgery at the University of Maryland Medical Center. His special interests include pediatric CT surgery, neonatal heart surgery, fontan conversion, valve repair, adults with CHD and research with a focus on pediatric patients with HLHS and Cardiomyopathy. Learn more about Dr. Kaushal or contact him at: http://www.umm.edu/doctors/sunjay__kaushal.html#ixzz278rjvxNM

Alex Tchouchev 5/6/12 Alex Tchouchev 5/6/12

Practitioner's Post: Stem Cell Research for the HLHS Patient

If you've been following us on Facebook or Twitter, you've likely seen our posts and tweets about Dr. Timothy Nelson and the HLHS Research Program at the Mayo Clinic in Rochester, MN. The information Dr. Nelson and his researchers shared with Sisters by Heart is extremely fascinating and provides great hope (and a serious sense of urgency) for all of us affected by Hypoplastic Left Heart Syndrome. Thank you Dr. Nelson and the program's research coordinator, Julia, for sharing the latest developments in regenerative medicine for the HLHS patient.

By: Timothy Nelson, MD, PhD, program director of the Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome at Mayo Clinic

I’m Timothy Nelson, MD, PhD, program director of the Todd and Karen Wanek Family Program for Hypoplastic Left Heart Syndrome at Mayo Clinic. The goal of our research program is to develop innovative clinical trials that offer the latest advances in cell-based therapy that could be used to treat people with HLHS.

Upon completing the three stages of surgery, HLHS is ultimately a disease of the right ventricle that becomes too weak to be able to sustain normal cardiac function. Fortunately, surgical procedures over the last two decades have lead to dramatic improvement in the care of children with HLHS, but many questions remain unanswered.

Through our research, we hope to better understand the causes of HLHS, to develop a system for predicting right ventricle performance, and to determine whether or not the patient’s own stem cells could potentially be an effective treatment. Understanding whether stem cells are dysfunctional in patients with HLHS or whether they are normal will be very important to help determine whether the use of stem cells or regenerative medicine could be an appropriate therapeutic strategy. Who is the right person for regenerative therapies? What are the right types of stem cells? When is the right time for this potential therapy? To answer these questions, we are focusing on research studies that involve families affected by HLHS.

Our program is focused on the next generation of therapies that could be used to improve the function and long-term performance of the right ventricle – one of the potential therapies being regenerative medicine through the use of stem cells. Regenerative medicine is the process of regenerating human tissues with new building blocks to heal or replace damaged or diseased tissue. This would be similar to planting a seed in order to grow a flower. Stem cell therapies aim to transplant “stem cells” into damaged hearts with the goal of growing new heart muscle.

Bioengineered stem cells start with the collection of tissue, such as discarded skin from a surgical procedure, and are then converted into stem cells that can acquire a new ability to grow into heart muscle. These stem cells – and ultimately the bioengineered heart muscle derived from these cells – originate from the patient’s own body. These cells can then be studied in the laboratory to further understand the molecular causes of HLHS, and hopefully provide a powerful tool to better customize innovative solutions for people with HLHS.

To see video regarding the bioengineering of stem cells, watch this fascinating video; the video depicts how Dr. Nelson is creating bioengineered tissue for HLHS research.

Dr. Nelson's team shared with us the video clip of the beating cardiac tissue he's created in the lab.

Regenerative medicine strategies and stem cell-based therapeutics have been applied to heart disease in adult patients over the last 8 years. This experience allows us to consider the possibility of applying similar technology to patients with HLHS and other congenital heart disease. Although this is not yet available, the promising vision of this regenerative approach becoming a reality requires significant scientific and preclinical research studies to be the focus of today. We are fully dependent on the participation of families affected by HLHS in order to generate the data that is required to make the new discoveries and innovations for the clinical practice of tomorrow.

Although we don't yet know that stem cells will be used in the care of patients with HLHS, this type of research will be paramount for the scientific community to be able to make an informed decision as to the safety and efficacy of such hopeful therapeutic strategies. Ultimately, we hope our research will result in the ability to delay, or prevent, the need for heart transplants in people with this congenital heart condition. More information about the ongoing research for HLHS can be found at:

http://mayoresearch.mayo.edu/mayo/research/hypoplastic-left-heart-syndrome/

If you have specific questions regarding Dr. Nelson and his team's research, feel free to contact them at: hlhs@mayo.edu

Alex Tchouchev 2/27/12 Alex Tchouchev 2/27/12

The Practitioner's Perspective: Dental Care and Congenital Heart Defects

By: Michelle M. Kelman, DDS

Pediatric Dentist, Cedars-Sinai Medical Center, Department of Surgery, Dentistry, UCLA School of Dentistry, Pediatrics

“Go brush your teeth.”

It’s a phrase that most children feel they hear too often. There is so much to do at the start and then the end of the day that all too often, tooth brushing is overlooked. Studies have shown that children with congenital cardiac disease (CCD) have poor oral health compared with healthy children. As you may already know, children with congenital heart disease (CHD) are at risk for bacterial endocarditis, an infection of the tissue that lines the heart and blood vessels. It is important for your child’s health to keep the oral cavity healthy to prevent infection that may begin in the mouth.

Some important facts to consider:

Studies have shown that children with CCD suffer poor oral health, compared with heart healthy children. This is most likely related to a combination of abnormal dental formation associated with the cardiac condition and dental neglect.
Children with CCD are more likely to have abnormal tooth formation including developmental enamel defects such as weakness in enamel, are more prone to malocclusion that includes crowding of teeth, and are more prone to harmful gum disease, compared with heart healthy children.
The chronic intake of sweetened medications is associated with increased cavities in children with CCD.
Children with CHD are prone to develop infective endocarditis from bacteremia not only induced by dental procedures but also from chronic poor oral health. Untreated dental decay may develop quickly into pulp infections which are associated with bacteremia, endocarditis, and even brain abscesses in children with CCD.
In regards to dental treatment, severely affected children may have reduced tolerance to the stress induced by dental treatment. Complications of CCD such as hematological, respiratory and immunological problems, and drug interaction with chronic medications must all be considered for children with CCD.

Unfortunately, a significant number of children with CCD do not receive optimal professional dental care until the child already has a mouth full of cavities. The goal is to prevent this. Here are some oral health guidelines to follow:

Home Oral Care:

Start taking care of your children’s gums before the first tooth erupts. Wipe down the gums after every meal, especially after medications.
Help your children with their toothbrushing. Children under the age of eight do not have the manual dexterity skills to brush on their own.
Teeth should be brushed for two minutes at least twice a day. As most medications are very sugary, it is recommended that your child brushes after medication is given.
Flossing: Children’s Flossers are recommended because of their ease of use. Because of tooth crowding, food/ sugary medications get stuck in between the teeth. It’s essential to floss the teeth to get the food out from in between them and from the gum line.
Tooth Wipes: It is recommended that you carry tooth wipes with you for “on the go” situations. It is recommended to wipe your child’s teeth following medication.
Diet: Avoid excessive sugar intake and “sticky” foods such as hard candies & even food that you feel is "healthy" like raisins (still "sticky"!) As soothing as sticky candy may be, a scoop of sorbet is much better for the teeth than a jolly rancher.

Seek out a knowledgeable dentist:

Your child should be seen by a pediatric dentist by the age of one. Make sure the dentist you have chosen has experience treating children with CCD. Please arrange for your cardiologist to send over a complete medical history and recommendations for treatment prior to your child’s first visit. Most children with CCD do not need premedication with antibiotics. The pediatric dentist and your cardiologist are familiar with the latest guidelines and will determine if premedication is needed.
Your pediatric dentist will form a preventative treatment plan that may include more frequent cleanings.
Your dentist may prescribe topical fluoride therapy and fluoride supplementation.
As the molars develop in the mouth, your pediatric dentist may recommend sealant placement on all molars with deep grooves and fissures. This will help prevent the entrapment of food/ sugar in these grooves and thus assist in preventing the formation of cavities.
If your child already has cavities that need to be filled, your pediatric dentist will coordinate with your cardiologist a treatment plan that will place the least amount of stress on your child. This may include treatment under General Anesthesia with an anesthesiologist that specializes in high risk pediatric patients.

To conclude, the time to start taking care of your child’s teeth is before the first tooth erupts. Brushing and flossing properly at home, and having regular dental visits, from a very early age, is essential.

Michelle M. Kelman, DDS
Pediatric Dentist
Cedars-Sinai Medical Center
Department of Surgery, Dentistry
UCLA School of Dentistry, Pediatrics

For more information on the American Heart Association guidelines for antibiotics prior to dental care:
http://www.ada.org/2157.aspx#heart
http://www.ada.org/sections/professionalResources/pdfs/patient_75.pdf

A special note from Sisters by Heart:

In addition to February being Congenital Heart Defect Awareness Month, it is National Children's Dental Health Month. All information here is for the purpose of helping you care for your child with a Congenital Heart Defect (and your healthy heart children, too!) However, please consult with your dentist regarding personalized care and a treatment plan for your child. This is very good general advice, but does not take the place of seeing a dentist. If you are not currently implementing these practices in your child's oral hygiene, it is a great time to start. No need to panic if you have some room for improvement in your home care and also if you have not had your child see a dentist yet. You may get recommendations/referrals from your cardiologist, pediatrician, and/or your adult/general dentist. No matter the age of your child, it's a great time to seek out a dentist who can work well with your child, your cardiologist, and you! ~Sara Hale, Sisters by Heart Mom/DDS

Alex Tchouchev 10/9/11 Alex Tchouchev 10/9/11

The Practitioner's Perspective: The Genetic Link

By: Paul Grossfeld, M.D., Associate Clinical Professor at UCSD School of Medicine and Attending Pediatric Cardiologist at Rady Children’s Hospital in San Diego

We’re excited to bring you the following post from Paul Grossfeld, M.D., Associate Clinical Professor at UCSD School of Medicine and Attending Pediatric Cardiologist at Rady Children’s Hospital in San Diego. Dr. Grossfeld spent many hours caring for one of our own Sisters by Heart warriors, Travis DiCarlo, and subsequently spent time discussing his research with Travis’ parents, Roger and Nicole. Dr. Grossfeld’s research lab is working towards identifying the specific genetic causes of congenital heart defects in order to improve therapies and ultimately lead to prevention. Dr. Grossfeld’s lab is 50% funded by the Children’s Heart Foundation with the remaining funds coming from private fundraising efforts. It is our hope that funding for promising research, such as Dr. Grossfeld’s, improves drastically to increase awareness for CHD and the countless lives they effect.

Thank you so much Dr. Grossfeld for sharing your research with us!

Congenital heart defects are the most common type of birth defect, affecting almost 1% of all newborn infants. Hypoplastic left heart syndrome (HLHS) accounts for a small percentage of all heart defects, but remains one of the most common causes of death in infants with congenital heart disease. Although most cases of HLHS and other heart defects are due to a genetic cause, a specific disease-causing gene mutation is only known in about 10% of patients. Clearly, identifying the specific genetic causes of congenital heart defects will improve therapies and ultimately lead to prevention.

We have embarked on a multidisciplinary approach involving human genetics and genetically engineered animal models to identify genes for congenital heart defects, including HLHS. The first approach has focused on a rare chromosomal disorder called Jacobsen syndrome (11q-), caused by deletions in the 11th human chromosome. These patients have the highest frequency of HLHS of any known human genetic disorder, as well as many of the most common congenital heart defects that occur in the general population. The second approach entails studying other chromosomal disorders associated with congenital heart disease. Specifically, with recent technological advances, patients with congenital heart defects can be analyzed to identify microscopic losses or gains of chromosomal material that contain genes that cause congenital heart defects.

We have recently identified several new chromosomal disorders that likely harbor previously unidentified disease-causing genes for congenital heart defects. Lastly, with the amazing advances in DNA sequencing technologies, it is now feasible to perform direct DNA sequencing on the entire genome (i.e., of all ~30,000 genes) to identify a putative disease-causing mutation. Taken together, these three approaches are enabling us to identify numerous new “candidate disease-causing genes.”

Due to obvious limitations in human genetic studies, we have utilized genetically engineered animal models to study the function of candidate genes in heart development, and to establish causality for specific heart defects. Towards that end, we have demonstrated that deletion of a single gene in the mouse that is deleted in Jacobsen syndrome causes heart defects, including a hypoplastic left ventricle and ventricular septal defects. We are also using simpler organisms including zebrafish and chicks to understand further the functions of these genes. These studies allow us to identify the specific cellular lineages in cardiac development, which could lead to new strategies for the prevention of congenital heart defects.

We are also embarking on a recently developed technology that allows human skin cells to be “reprogrammed” into developing heart cells. As described above, specific lineages in which the causative gene is known to function can then be studied to characterize the mechanism by which absence of the gene causes heart defects, so-called “disease in a dish”. This extraordinary technology will eventually lead to the identification of putative drugs that could potentially preserve the function of these cellular lineages during heart development, thereby preventing some types of congenital heart defects.

Click here for a list of Dr. Grossfeld’s publications.

To support Dr. Grossfeld’s research, please mail checks to:
UC San Diego
Health Sciences Development
200 West Arbor Dr. #8982
San Diego, CA 92103-8982
Attention: Shawna Fallon

Payment can be made to: “UC Regents #42364”

To make a donation over the phone, please call 619-543-3499.

94% of all donations go DIRECTLY to support Dr. Grossfeld’s research!!!

Alex Tchouchev 10/9/11 Alex Tchouchev 10/9/11

Practitioner's Post: Grief and HLHS

By: Dr. Kenneth J. Doka, Professor and a Senior Consultant to the Hospice Foundation of America

All of us at Sisters by Heart experienced a significant sense of loss at the time of our child's diagnosis of Hypoplastic Left Heart Syndrome, and continue to grieve throughout our journeys with HLHS. While we don't always identify our feelings as grief, that is what we experience.

Grief: deep and poignant distress caused by or as if by bereavement; also: a cause of such suffering

To help identify some of the feelings associated with grief, this chart identifies the journey from "'normal' functioning" to the "return to meaningful life."

With grief playing such a large role in all HLHS journeys, we reached out to one of the nation's leading experts on grief, Dr. Kenneth J. Doka. Dr. Doka is a Professor and a Senior Consultant to the Hospice Foundation of America. He's written countless books and articles on grief and offering new perspectives on grieving.

Dr. Doka, extremely touched by our HLHS community and all that we endure, took the time to explain what happens once we, as parents, receive a diagnosis of HLHS for our infants.

Thank you, Dr. Doka, for providing the following Practioner's Post regarding anticipatory mourning.

Kenneth Doka, Ph.D.

Anticipatory Mourning

Feelings of grief that develop before, rather than after, a loss.

If we have ever heard of anticipatory grief or anticipatory mourning, we are likely to think of it as the grief that we experience when we expect or anticipate a loss such as the death of our child. In truth, the concept is much broader than that. One of my colleagues, Dr. Therese A. Rando, the author of a book on anticipatory mourning once called the term a “useful misnomer.” What Dr. Rando meant is that grieving an anticipated death is only part of what the concept means. As she redefines it, the term actually refers to all the losses that we experience as part of an illness whether or not the illness is, in fact, fatal.

These losses can be extensive in HLHS. It begins with the identification of the defect. We never expect our child to be born with a life-threatening illness. From the very moment of diagnosis, our world is turned upside down as our new born baby begins a seemingly never-ending series of surgeries. We mourn the loss of normalcy – the expectation of the child’s milestones here becomes fears for survivorship.

We may experience other losses as well. While rates are declining, many children born with HLHS experience developmental delays, neurological difficulties, learning disabilities, and chronic health problems. As the child ages and becomes aware of their condition, they too may grieve the condition.

All of these are losses too that need to be grieved.

This grief is a normal and natural response to the losses that parents, grandparents, siblings – all who love the child – will experience. It is natural too that the older child will grieve this condition as well.

Acknowledging the legitimacy of that grief is an important first step. We need not look at our grief as a betrayal or lack of hope. It is simply a response to loss. Once we acknowledge our grief, we can examine how we are coping with our losses. Gaining information, getting support, and joining with other parents are often helpful steps as we face a reality we never expected to or should have to face.

Kenneth J. Doka, PhD

Professor, The Graduate School, The College of New Rochelle
Senior Consultant, the Hospice Foundation of America

For additional information on Dr. Doka's work, please visit his site and publications list at http://www.drkendoka.com/.

We, at Sisters by Heart, want you to know that grieving is an ever-evolving process. Some days are certainly better than others. Just as Dr. Doka suggests, Sisters by Heart is here to help you: gain information, find support and connect with others to help make this journey a bit less strenuous. You are not alone in this journey!

For additional information on Dr. Doka's work, please visit his site and publications list at http://www.drkendoka.com/.

Alex Tchouchev 9/11/11 Alex Tchouchev 9/11/11

The Practitioner's Perspective: How to Choose a Congenital Heart Center

We're so excited to start a new series of guest blog postings from various practitioners who deal with our special kids on a regular basis. First up is Anthony Rossi, MD, the Director of the Cardiac Intensive Care Program at Miami Children's Hospital! He provides a unique perspective on how to go about choosing the right Congenital Heart Center for your child. Thank you so much Dr. Rossi for providing our inaugural practitioner's post!

By: Anthony Rossi, MD, Director of the Cardiac Intensive Care Program at Miami Children's Hospital

First, I am a practicing pediatric cardiologist at Miami Children’s Hospital. I am currently directing the cardiac intensive care program here. I’ve directed a cardiac intensive care program since 1989. I suspect I have directed a cardiac intensive care program longer than just about anyone who’s currently doing it. At best, I can speak from experience. At worse, it just makes me old! What I say is my opinion and my opinion only. However, what I will suggest to all of you is what I would suggest to any family member that might be in the same position as you. There might be some physicians out there that disagree, Or more likely, I’ll say things that they believe but won’t say publicly. So just think of me as your personal, inside source. Feel free to email me if you have a question at Anthony.rossi@mch.com. I’m more than willing to tell you who I think is the best at what we do.

If you are reading this, you are unlucky enough to be the parent of a child born or soon to be born with congenital heart disease. Or you just might have someone close to you in that unfortunate position. Here’s my message, don’t despair, don’t give up, it can and usually does get much better. And you have the potential to really influence the outcome in a positive way.

It was probably just a short time ago when you felt that you didn’t have a care in the world, and everything in life was as great as it could ever be. Perhaps you were pregnant, with marvelous expectations of the new life you were about to deliver to the world. A child who would be strong and smart and kind. A child who would go on to do great things.

The devastating news that your child has congenital heart disease can come in many ways. Perhaps your child had already been born and was apparently well in the newborn nursery. Occasionally your baby has been whisked away to some other hospital before you had a chance to kiss him/her goodbye. Sometimes it’s just a routine trip to the pediatrician for a “well-baby visit”. And then your whole world came tumbling down. Congenital heart disease can present in a number of ways, none of them are anything but horrific for a family. Even lesions that your cardiologist insists aren’t a big problem to fix mean years of unplanned visits to the office at best and “simple” heart surgery at worse. Simple heart surgery? It’s catastrophic to learn that any child needs to have any type of heart surgery, and there’s nothing simple about any of this, at least to a parent.

When I was in college (a Jesuit school at that) I learned the definition of despair was to be put in a position where you feel like you have no influence on the outcome. I am certain that this is exactly how every parent feels after learning their child was born with heart disease. I mean we were all taught, count the toes, count the fingers, all there so all good, right? Wrong! Who told us we needed to count the heart chambers?

But today, the news for children with congenital heart disease is better than it has ever been and the advances are occurring at a breath taking pace. The unfortunate part of this is that it’s often up to the parents to make sure that their child is getting the very best and most up to date care. Today, there are about 120 centers in the United States that perform congenital heart surgery and most will do it very, very well. In 1988, I was lucky enough to be in Philadelphia and if you had a child with HLHS, it was about the only place in the world where surgery for that problem was even offered. Babies came from around the world in hopes of saving their lives. In 2011, there are dozens of centers in the United States who have results that are now superior to the results we had in Philadelphia by Dr. Norwood himself. That’s amazing news. Back then, we could not be sure that the majority of these children who survived their surgeries would not grow up and become “cardiac cripples.” Every time a child comes back years after his/her surgery and looks great we take it for granted they are supposed to look like that. Twenty years ago we had no idea. As cardiologists all we could do was hope. We’ve now learned that these babies can grow up and lead very productive lives. They are in every way the blessing we believed they would be.

So when you’ve learned your child has congenital heart disease, what do you do? First, take a deep breath. Get the details. Listen. The more information you have, the better the chance you’ll make an informed decision. I will make a sports analogy here; major league baseball teams all have amazing athletes. They are the best of the best in the world. But all major league teams are not the same. It’s just human nature, we are all not created equally and some of us are better than others. Yes, weak teams will win games occasionally, but not as often as The Yankees, Red Sox or Phillies. Not all heart centers are created equally either. If you want to increase your odds of winning, go with the best.

When your baby is born with congenital heart disease

Sometimes things seem quite emergent and decisions need to be made rapidly. In actuality, there aren’t many problems (even serious ones like HLHS) where you don’t have the opportunity to take your time to do your homework and make the right decision. Usually most babies with heart disease are stable if medicines like prostaglandin E1 are used. There are some rare occasions where cardiologists need to act fast, but this is only in a minority of cases.

OK, first question to your doctors, how many of these cases do you see, and how many of these procedures have they done here? How many of these procedures has this particular cardiologist or surgeon done themselves? What are the outcomes for your doctor in this program for this problem? Don’t let them skirt the issue. Demand answers. Even in the very best centers, there will be the physicians with less experience, like those out of training. If you want to increase your baby’s odds, you need to ask these questions. Are there people here with more experience? Are there centers with more experience? Some metropolitan areas may have a number of congenital heart centers. They are NOT all created equally. You can be at a great medical center, but congenital heart surgery may be one of their weaker specialties. A great center might be down the street or perhaps an hour away. You won’t know unless you ask. I worked in New York City for almost a decade; there were about 7 centers performing congenital heart surgery. You could have, by chance, ended up in any one of these. But I can tell you that your child’s likelihood of having a successful outcome was greatly enhanced if you were in one of the top 3.

If you have no other data available, then the total volume of congenital heart surgery for a center and for a surgeon, and the number of these particular types of cases they have done, are probably the best indicators of excellence you can have. If you don’t have time for more extensive research, most of the time the volume data will lead you in the right direction. In all human endeavors, human factors play a huge role in surgical success - experience counts. Are there a number of surgeries a center needs to do to be great? Some reports suggest 300 cases per year. The Society of Thoracic Surgery considers programs that perform 200 open heart cases a year to be large. In general you want to be there, especially if the problem is either rare or complex. So ask, is this the largest program in the region? Now there are some great congenital heart surgical centers that perform smaller number surgeries than the numbers I’ve presented. I just personally believe the odds favor the bigger ones. One final thought, I suppose you can be too large. McDonald's makes the most burgers in the world, but probably not the best. Be sure you don’t feel like you’re in a factory or your baby is just a number (or your child is not just a referred to as a disease-“the hypoplast in bed two”).

Ok, so we established that experience counts (that coming from an old guy)! What else matters? The overall quality of the hospital or medical center is important. You want to know that everything matters in that center. From the quality of the surgeon to the center’s obsession with cleanliness. Ok, quick check. Look at the floors. Are they as clean as you like? If not, I suspect that hospital is slacking in other areas as well. Great hospitals are sparkling clean. I promise. A great center will have great sub-specialty doctors. Great centers have leaders in their field. If you Google your doctors, do scholarly articles come up? Not all great medical centers have great congenital heart programs, but it is impossible to have a great congenital heart program in any center that isn’t great.

How about the congenital heart program. Do they have all the pieces? All programs will have echo, but do they have dedicated echo physicians who have trained in noninvasive imaging? How about cardiac catheterization? How about electrophysiology or MRI? Do they have a dedicated cardiac ICU program? Will your child be cared for by physicians and nurses who care for asthmatics and diabetics and patients with pneumonia or will he/she be cared for by specialists in cardiac ICU?

Of course, so much of this comes down to who your surgeon is. I guess it’s the single most important factor. How do you know he’s the right one? Experience counts, what are his numbers? How many years has he done these operations? Where did he train? Was it one of the best congenital heart centers (say top 20 US News and World Report)? Most importantly, when you meet him, do you trust him? Does he look you in the eye? Does he have time for you? Is he annoyed when you ask questions? Is he annoyed if you ask for a second opinion (all great physicians are never put off by asking for a second opinion-it’s a big red flag if they are, they probably lack confidence).

OK, after 20 years of this, I could go on for ever. So I’ll summarize.

Do your homework. There are great sites available that should help steer you in the right direction. Two of these include:

http://health.usnews.com/best-hospitals/pediatric-rankings/cardiology-and-heart-surgery

http://www.youtube.com/user/Redmond111

But don’t believe everything you read on the internet. There’s nothing that prevents people from stretching the truth a bit on the internet.

Ask questions. Lots. If the explanation doesn’t make sense ask again. If you still don’t understand, it’s not you, it’s the person explaining it to you. Red flag!
If you have no other data, go to a center with more experience, one that does more surgery. Ask if there is one close or a center of excellence for your baby’s particular problem in the US.
Make sure you are in a comprehensive congenital heart center that has all the specialties covered, and make sure they have more that one of each (what happens if you need a cath and the cath guy is on vacation)?
Dedicated cardiac ICU, dedicated cath lab. Insist.
Know that you have all the rights. It is your child, not the hospital’s. You can advocate getting your child transferred to another center if you wish.

So that’s it for now. Good luck to all of you. You’re already on the right track. That’s how you found this web page. Continue to follow your instincts; they will lead you in the right direction. Oh yeah, ASK A LOT OF QUESTIONS.

Anthony Rossi, MD

Director, Cardiac Intensive Care Program

Miami Children's Hospital

The Cardiac Newborn

Nutrition Series

The Cardiac Newborn

The Nutrition Plan

So when we start feedings, what will we use?

Are there cases where breastmilk or one of these formulas can’t be used?

When can babies start to take feedings by mouth?

So how much nutrition do cardiac babies really need?

What is normal growth for an HLHS baby?

Things to think about

Breastfeeding the HLHS Baby - the Practitioner Perspective

Nutrition Series

Breastfeeding the HLHS Baby - the Practitioner Perspective

Nutrition Series

Breastfeeding the HLHS Baby - the Parent Perspective

Nutrition Series

Practitioner's Perspective: We Can't Make It All Better

A Practitioner's Perspective: Choosing a Pediatrician/Vaccinating your HLHSer

Practitioner’s Post: The Promise of Stem Cells

Practitioner's Post: Stem Cell Research for the HLHS Patient

The Practitioner's Perspective: Dental Care and Congenital Heart Defects

The Practitioner's Perspective: The Genetic Link

Practitioner's Post: Grief and HLHS

The Practitioner's Perspective: How to Choose a Congenital Heart Center

When your baby is born with congenital heart disease

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