Fontan to Transplant: Building Teams for Transition.
Dr. Schumacher is the medical director of the pediatric heart transplant program at the University of Michigan Congenital Heart Center at C.S. Mott Children’s Hospital. Throughout his career, Dr. Schumacher’s clinical and research efforts have focused on understanding and improving the outcomes of heart failure and transplantation in children with congenital heart disease with an emphasis on Fontan patients. He is a leader of ACTION’s Fontan subcommittee aimed at improving the quality of care for all Fontan patients needing heart failure treatment and advance cardiac therapies, and he is a member of multiple national working groups aimed at improving outcomes for all single ventricle patients.
The relationship between a single ventricle family and their cardiologist is strong. From my perspective as a cardiologist, each patient requires a tremendous amount of thought and effort, but each patient can also give me with a wonderful sense of accomplishment. Through this single ventricle journey, we cardiologists become attached and even bonded to our families. We go through so many events together – hospital admissions, clinic visits, inter-stage monitoring, nerve-wracking surgeries, challenging recoveries, and joyful discharges. In my experience, the bonds formed during this process are often amazing and powerful. After the Fontan, we get to be there with our families watching children grow up. For a pediatric cardiologist, this is the ultimate reward. The reality is, however, that the path ahead is far from clear. We now know that growing up as a Fontan is accompanied by new challenges for both families and cardiologists. As much as we don’t like to think about it, a large proportion of Fontan patients will eventually need to be seen by a heart failure specialist, and many will need a heart transplant. Single ventricle congenital heart disease is now the leading indication for pediatric heart transplant evaluation. Like it or not, the reality is that sometimes pediatric transplant cardiologists are going to be a part of the Fontan patient’s care team.
Unfortunately, the approach of building a Fontan care team that includes a transplant cardiologist is not widespread. More often, patients and families are “referred for transplant evaluation” and find themselves with a completely new cardiologist who is now overseeing all of their care. Suddenly, the cardiologist (and team) that has seen this patient through all the clinic visits, surgeries, and hospitalizations is no longer “in-charge” and all new people are making the decisions. Some patients even need to transition their care to a completely new center. To make matters even more challenging, all of this change occurs at a time when anxiety about patient health is extremely high. As a pediatric heart failure and transplant specialist, I know from discussions with many patients that this transition can be a jolting and traumatic experience.
Why does this happen? It probably stems from many causes. Given the strong bonds they have, longtime cardiologists understandably don’t want to give up care of their patient so they delay their referral. Similarly, patients and families (appropriately) don’t want to leave their cardiologist, and they may not even understand that heart failure can occur after Fontan or don’t want to acknowledge that possibility. Finally, in our field, there are no formal recommendations for when to refer a Fontan for heart failure consultation. General cardiologists are left to decide on their own without guidance about what an appropriate Fontan heart failure consult is. This is a gap in our field that needs to be filled because a sudden transition in patient care is not the way it has to be. Early consultation from a heart failure/transplant expert would allow us to build Fontan care teams and eliminate the jolting change in providers.
ACTION (Advanced Cardiac Therapies Improving Outcomes Network) is a collaborative group of pediatric heart failure and transplant cardiologists and cardiac surgeons who are working to improve the quality of care that pediatric heart failure patients receive. When ACTION surveyed heart failure providers, a large majority wished that Fontan patients were referred earlier than they typically are. When we explored this finding, there was a clear desire for the patient and family, the primary cardiologist, and the heart failure/transplant cardiologist to be a decision-making care team prior to needing a transplant. Early referral of a Fontan patient for consultation from a heart failure cardiologist would allow the creation of this Fontan advanced care team. The heart failure cardiologist’s perspective and insights, including issues specific to potential future transplant, could be paired with the primary cardiologist and family’s experiences to collaboratively structure medical care plans for the patient. I strongly believe this could improve outcomes for patients. And should the time ever arrive that heart transplant is indicated, the patient and family wouldn’t suddenly be meeting a completely new team – that relationship is already established.
So how do we make this happen? ACTION is building a set of Fontan-patient-specific reasons that should prompt consideration of a referral to a pediatric heart failure specialist. These are based on the experiences and challenges that we have had with Fontan patients undergoing heart transplant. They allow us to meet patients before transplant is urgently needed (or even worse, before a patient becomes too sick for transplant). It will give us time to treat different types of Fontan failure and insure that a patient is as healthy as possible prior to undergoing transplant. It is our hope that by clearly stating these reasons, we will improve the timeliness of patient referrals, improve outcomes, allow the formation of a collaborative care teams, and eliminate the abrupt transfer of care that can be so difficult for both patients and providers. We welcome feedback and insights from patients and families as this effort moves forward. We all share the same goal – the highest possible quality of life for our children.
HLHS Collaborative: September's Action Period Call
After a two month hiatus for centers to regroup with their quality improvement teams, the Action Period calls resumed this week. Discussion revolved around valuable work which continued over the summer months and important preparations for the Fall Learning Session.
The calls began with a review of data (mortaility and daily interstage weight gain) which is driving the current improvement work within the collaborative. Following data discussions, parent travel scholarships were announced to collaborative members, followed by an excellent discussion regarding how centers are funding parents to join them at the Learning Session. A number of centers identified local charities or non-profit groups that assisted in funding; others were able to emphasize to the powers that be the importance of parent involvement and secured funds through their hospital. Happily, more and more hospitals are recognizing the value parents can provide and it is becoming much more commonplace to fund parent partners to attend the Learning Sessions.
Advocate Children’s Hospital uses a combination of the above funding methods and selected parents who demonstrated commitment to their local group. If you're interested in the collaborative and quality improvement work, parents suggest you get involved with your local hospital - either on a parent advisory council, directly with your cardiac center's quality improvement team, or spark a conversation with your child's cardiologist expressing your interest in becoming more involved with NPC-QIC.
After a creative funding discussion, the call shifted toward the Transparency Workgroup. The workgoup recommended to centers that the Collaborative begin sharing collective performance measures on the NPC-QIC website (these measures represent combined data from all the centers in the collaborative and do not represent any one center). The workgroup brainstormed and created an infographic to highlight the Collaborative's progress to date. Some of the data points included in the infographic are:
number of patients in the registry
mortality rate (expressed as survival)
growth rates
readmission rates
parent engagement
who are collaborative members
The Transparency Workgroup is excited to reveal the infographic (once beautified by graphic designers) in the coming weeks. Stay Tuned!
September's Action Period call ended with a Learning Session preview and "homework" for cardiac teams prior to attending in November. We'll just say, the Learning Session will be engaging - for both parents and clinicians - and a lot of good information will be shared and discussed.
If you have the ability to attend the upcoming Learning Session in Cincinnati (November 7-8), we'd love to see you there. To register for the Learning Session as a parent, please visit: http://events.r20.constantcontact.com/register/event?oeidk=a07e9kkxcx92230dff1&llr=kdgqiilab
If you're not able to attend, keep an eye on social media where Sisters by Heart, parents, and clinicians will be updating on the events of each day! Get Twitter updates using #NPCQICLS and Facebook updates at Sisters by Heart and HLHS Parent Advisory Page.
Parent Travel Scholarships - NPC-QIC 2017 Spring Learning Session!
Registration for the NPC-QIC 2017 Spring Learning Session is now open! Sisters by Heart and NPC-QIC want to send YOU to the Spring Learning Session May 18-20th 2017! Together, Sisters by Heart and NPC-QIC are once again offering parent travel scholarships, valued at $500 each, to four parents to attend the learning session in Cincinnati, OH. If you are new to the HLHS world, haven’t heard of NPC-QIC before, or have questions about what NPC-QIC does, we have put together some information for you.
The National Pediatric Cardiology Quality Improvement Collaborative’s mission is to improve the care and outcomes for children with cardiovascular disease. NPC-QIC’s current quality improvement project is working to improve survival and quality of life for infants with Hypoplastic left heart syndrome (HLHS). The collaborative is made up of clinicians from fifty-eight (58) centers from across the country and parents of children needing a Norwood (i.e. HLHS).
Here is what some past travel scholarship families have had to say about the event:
"Attending the NPC-QIC Conference was rewarding at multiple levels: First, being in a room of cardiac professionals and parents who are focused and are driven to improve the care of our HLHS kids, and their families, touched my heart and made me further grateful for these dedicated people. I also appreciated hearing that the current Collaborative statistics (which are really good!) still aren't good enough for these professionals. Our kids are fiercely being fought for behind the scenes (as well as on the front lines) and that is incredible to see, first hand. Finally, this group allows their centers to be vulnerable for the sake of improving the HLHS outcomes and that is amazing!
I look forward to be a contributing part of the Collaborative efforts at future conferences and at our center, and hope to share the work being done to encourage and bring hope to newly diagnosed, and current, HLHS families in Colorado and surrounding areas.
Thank you so much Sisters-by-Heart for the opportunity to meet so many amazing people and to continue my efforts to help our center help more HLHS families! It was great to finally meet all of you, too!" - Erica Isakson
"As a Dad of a 3 year old (Roman) with HLHS, I would like to start off by thanking Sisters by Heart for the great opportunity to attend the fall 2014 learning session. Leading up to the learning session I was a little nervous it was going to be a bunch of healthcare professionals speaking in a langue I didn’t understand. Once I got there I realized I could not have been more wrong. The learning session featured individuals from about 50 centers which included doctors, nurses, and parents. They all come together for one common goal - Improving care for patients with HLHS and their families. I am not sure what I enjoyed more either participating in the learning session – or getting to interact with other parents of children with HLHS of all different ages.
During the learning session it was clear that the medical professionals really valued our opinions. I was asked several different questions while I was there ranging from time of diagnosis all the way up to his current care. I know all HLHS parents remember that moment when they find out their child has a life threatening condition. During the learning session we spent hours discussing what could have made the time after that moment better. We heard great examples of things to do, and horrible examples of what to avoid.
I also was excited to meet with parents of other children with HLHS. I was able to meet parents with children of all ages from babies to college students. Hearing their stories provided me great hope. Generally as a parent of HLHS you are always looking so short term; this was a good opportunity to look long term. I thought it was awesome to hear everyone’s stories, especially the ones about the older children.
One last aspect of the conference I wanted to mention was how great it was to see hospitals working together to better care. It would be really easy for the top hospitals to keep all there data in-house and not share it with other hospitals, but that does not happen with this group. All involved are excited and even eager to share their best practices and success stories.
I came away with great comfort knowing there are some many intelligent people who care so greatly about our children, and look forward to being able to attend future learning sessions." - Matt Ulrey Dad of Roman age 3
Parents with a single ventricle child, who required a Norwood or Norwood varient surgery may attend the learning session and/or apply for a travel scholarship. You do not need to be currently involved with your child's interstage clinic, however you are encouraged to reach out to your child's center prior to the learning session.
Sisters by Heart will be accepting travel scholarship entries until April 1st 2017. In order to be considered, please send an email with "Learning Session Scholarship" in the subject field to info@sistersbyheart.org with the following information:
Name
Address
HLHS child's name and DOB
HLHS child's hospital
Have you contacted your child's center to inquire about funding to attend the Learning Session?
A brief paragraph on why you would like to attend
If you are interested in attending and can make arrangements for childcare/time off work, please apply! This is such a wonderful opportunity to share your opinions, answer questions from a “professional” perspective as a parent, and most importantly be involved in helping to improve care for HLHS children.
Parents awarded scholarships will be responsible for making their own travel arrangements. If you have questions regarding the scholarship process, don't hesitate to email us at info@sistersbyheart.org.
If you're already planning on attending the learning session, please make sure to register for the event at NPC-QIC's website
We look forward to seeing you in Cincinnati in May!
On Finding His Tribe
When I was maybe 10 or 11, I read a book about a girl named Dawn, who had cancer. I remember her going to “Cancer Camp” and how much it meant to her to be around her peers, to feel “normal.” For some reason, the idea of that camp stuck with me. I just never knew how close it would hit to home with my own family years later.
My 6-year old son, Bodie, has Hypoplastic Left Heart Syndrome (along with a few other fun surprises). For the past almost 7 years, I have immersed myself in the “Heart Community.” I have made friends with other heart moms, some only virtually through social media, some only in person, and some that started virtually but eventually became in person relationships. In it, found MY tribe. It is full of amazing women with stories like mine, who have children like my son. Women who share my fears, and who talk me off of my anxiety ridden ledge at 2am. We are there for each other through this crazy journey.
I have introduced some of these women to my family, and we do things with the ones who live locally. My son, and his 9 year-old heart healthy sister, get to see other kids like them occasionally. I thought that was enough. I really did.
I was wrong.
MY tribe has been wonderful for me. But it hasn’t quite met the needs of my son and his sister. And I didn’t even realize it, until last weekend, when we went to our first Heart Camp. And then I saw it. The profound connections made between survivors. The look that simply says “I’ve been there. I get it.” The sense of comradery, of feeling “normal” in a world where their scars make them anything but. The overwhelming shared hope of seeing older survivors living amazing, authentic lives in spite of their very serious heart conditions.
Meeting up with a heart family here or there, or once a year at a Heart Picnic is wonderful. There is a very valuable space for that. But, honestly, there is a vast difference between a few hours spent with other heart families – and 72 hours straight spent immersed in the heart culture with tons of families (there were over 60 families at our camp!). It is difficult to even put into words what our weekend at Heart Camp meant to us as a family.
Perhaps the best way to explain it is to describe the most profound moment of the weekend for me. My sweet boy, at the tender age of 6, has just in the past 6 months or so started to become self-conscious about his scars. This summer, he has wanted to keep his rash guard on anytime he is swimming. On Saturday night, the camp was offering all sorts of fun activities, including tattoos and face painting. My son wanted to put a tattoo on his chest. But he wouldn’t take his shirt off. He asked me if we could just go to the bathroom and do it instead, and then started to make up all sorts of excuses for why he didn’t want to get the tattoo after all.
I was at my emotional wits’ end. Despite my reassurances that it was heart camp and he didn’t need to worry about his scars, he wouldn’t budge. I didn’t know how to help him. And then, something extraordinary happened. Our family had been assigned a mentor, an amazing teenager living with heart disease, to shadow us for the weekend. Our mentor was Kenny. When Kenny realized Bodie was struggling, he came over, took his shirt off to show his own scars, and began to talk to Bodie. Before I knew it, we were surrounded by 3 mentors, all teenage boys with their shirts off in the cold evening air, just to show my son that his scars weren’t anything to be ashamed of. And they were reassuring me that they, too, had all gone through it.
Kenny took Bodie a bit away from us to talk to him. I don’t know what their conversation entailed. All I know is that, in a very short period of time, my son had his shirt off and wanted to show me his scars and talk about how brave he was.
I know this is a journey, and this won’t be the last time we talk about my son’s scars. But last weekend was a valuable stepping stone in his journey to acceptance. And for that, I will be forever grateful. That moment would never have happened without heart camp. I love my son, and I support him in every way I can, but, at the end of the day, this is HIS journey, and I don’t know what it’s like to walk in HIS shoes. But his peers at Heart Camp do.
On Saturday morning of our camp, we had the opportunity to attend a Q&A panel with 12 of the teen mentors. Upon being asked what had helped ease their journeys the most, every single one of them recommended Heart Camp. And I don’t doubt it. The benefits are enormous. It was clear from looking at how close all of the teen mentors were that their experiences at Heart Camp had fundamentally shaped their views on their hearts and their futures. They were an exceptionally inspiring and tight knit group of young adults.
We are profoundly lucky in California, since we have 2 heart camps to choose from, Camp Taylor in Northern California (where our family was last weekend) and Camp del Corazon in Southern California (which starts at age 7). With a family camp for younger kids, and options for siblings to attend youth camp alongside the CHDer, Camp Taylor was the perfect choice for our family. And one that was absolutely worth every minute of the 5 hour drive there. Not every state has 2 options within driving distance. But there ARE camps across the United States. We at Sisters by Heart are working on compiling a list – if you know of one, please let us know so we can add it to our list! If you have a CHDer of school age, I could not encourage you enough to see whether there is a heart camp near you. We met one teen mentor who flew from Denver to California for Heart Camp each year. I promise, the experience your child gains will be 100% worth it!
Before heart camp, I had MY tribe. Last month at Heart Camp, our son found HIS tribe. One that he will walk with for the rest of his life. And for that, we are inexplicably grateful. As they so beautifully put it at Camp Taylor, heart camp is where "Kids Meet... Scars Blend... Wonders Happen!!!
NPC-QIC/SBH Response to HLHS Statistics Shared by the CDC
The numbers reported this week by the Centers for Disease Control and Prevention do not represent the current state of outcomes for infants born with HLHS.
Last week the Centers for Disease Control and Prevention (CDC) posted information about a study that has recently been published in the Journal of Pediatrics called Differences in Survival of United States Children with Birth Defects: A Population-Based Study.
Purpose: It is important to understand what question the researchers are attempting to answer when evaluating a research study and its results. The purpose of this particular study, which was performed by a group of researchers on behalf of the National Birth Defects Prevention Network, was to evaluate possible racial or ethnic differences in survival of children born in the United States with various birth defects, ranging from congenital heart defects (CHD) to gastrointestinal defects to neurologic defects. The purpose of this study was not to describe the current state of survival in the United States for any of these birth defects.
Methods: It is also critical to understand the methods that the researchers used to answer their question. These researchers chose to use a birth defect registry that was active in several states: Arizona, Colorado, Florida, Georgia, Illinois, Massachusetts, Michigan, Nebraska, New Jersey, New York, North Carolina and Texas. In fact, some of the states had incomplete information from parts of their state. For example, births from New York City were not included in the New York state information. Importantly for HLHS, the birth defect registry captures all live births, including those infants whose parents elected comfort care only.
All studies have limitations to their methods. In the case of this study, the authors note extensive limitations in their paper. Most published studies have ~1 paragraph describing the limitations; this paper includes 4 extensive paragraphs describing the limitations. Some of these limitations include possible misclassification of birth defects and potential misclassification of deaths. It is important to note again that the purpose of this study was not to describe the current state of survival in the United States for these birth defects. If these authors wanted to answer that question, they would need to use different methods and use more current survival information.
Results and comments: This paper has some important results. The researchers found that children with birth defects born between 1999-2007 to non-Hispanic black and Hispanic mothers have a lower survival rate than those born to mothers of other ethnicities. This was especially true for children born with CHD. We do not know if this is still true in infants born with these birth defects in 2015, but this result should give us pause and cause us to look at our systems of care to make sure we eliminate any ethnic disparities in outcomes in our babies with CHD. Unfortunately, the CDC did not focus on this important finding when posting the results of this study. Instead they focused on the mortality rates of various birth defects noted in this study. In our opinion, the CDC webpage is misleading as it emphasizes the mortality rates noted in this study rather than the results of the purpose of this study, the ethnic differences in mortality rates during that time period (1999-2007). This data does not reflect current mortality rates.
What is the current state of outcomes in infants born with Hypoplastic Left Heart Syndrome? The most accurate and up-to-date information about survival for infants born with HLHS comes from the Society for Thoracic Surgeons (STS) registry, the NPC-QIC registry, and publications that look at longer term survival. The STS tracks survival until hospital discharge for all patients who undergo surgery for congenital heart disease for nearly all centers performing these surgeries in the United States.
For the time period 2010-2013 there were 2177 Norwood procedures performed for HLHS at STS centers and there was an average survival to hospital discharge of 83% among all centers.
For the year 2013 there were 714 Norwood procedures performed in STS centers with a 85% survival rate to discharge.
For information about survival after hospital discharge, in the “interstage” period and beyond, we need to turn to the medical literature since STS only tracks survival to hospital discharge following surgical procedures. There have been several reports describing survival after discharge following the Norwood procedure.
The NPC-QIC registry historically has demonstrated interstage survival of ~90-92% and more recently (since January 2013), interstage survival in centers participating in the NPC-QIC collaborative is approaching 95%.
The Pediatric Heart Network Single Ventricle Reconstruction Trial enrolled infants with single ventricle between 2005 and 2008 and looked at two different surgical approaches to the Norwood procedure. This study enrolled 549 infants (not all of them had HLHS) and the survival rate to 1 year of age was ~73%. It is important to recognize that these patients were operated on and followed at 7 Pediatric Heart Network centers and that this study was started almost 10 years ago.
The National Pediatric Cardiology Quality Improvement Collaborative remains dedicated and committed to improving not only survival but also quality of life for children with HLHS and their families. While we recognize that we have a long way to go in our efforts, we have seen tremendous improvement in outcomes in HLHS in the past decade. The numbers reported by the Centers for Disease Control and Prevention do not represent the current state of outcomes for infants born with HLHS, nor do they reflect the hard work done by caregivers and parents in improving outcomes for our patients.
Jeffrey Anderson, MD, MPH, MBA, Improvement Advisor and Research Committee Chair
Robert Beekman, MD, NPC-QIC Chair
Carole Lannon, MD, MPH, Improvement Design and Implementation Lead
Stacey Lihn, NPC-QIC Parent Lead and Sisters by Heart President
Laura E. Peterson, BSN, SM, Senior Quality Improvement Consultant
You’re the General Manager of Your Team
By Caleb Lihn
This is one of my favorite times of the year, I hate to admit it, but a major reason is because football season is hitting its stride. One of the things that forces me to pay such close attention to football is fantasy football. If you, or a family member, play fantasy football then you know all too well the phenomenon of the fantasy footballer in your house spending an inordinate amount of time on Sunday pouring over scores and statistics. You also probably understand that fantasy football is not particularly realistic of the actual game as it involves drafting several individual players and hoping that, as a collective, they play great games and score you a ton of points. I’ve noticed that some weeks my star players don’t perform very well but, despite that, their real life teams win the game. The reason for this is simple; one player doesn’t make the team or win the game. It takes a complete team performance to deliver a win. Teammates communicating, knowing and executing the plan, reacting to adverse conditions, and giving maximum effort.
Sports is often compared to life. Of course what to compare it to depends on our individual life experiences. As a heart parent, I’ve compared sports to the heart world. I’ve also compared it to war, but that’s a story (or battle) for another day. As heart parents, we’ve all experienced overnight and often lengthy hospital stays. It was during my daughter’s recovery from her second open heart surgery that I realized her care team in many ways is like a football team. From there, I consciously realized something that should be common sense, the outcome is dependent upon the performance of the whole team, not just a single member. While he never coached football, Phil Jackson, coached many basketball teams to NBA championships. He once said “the strength of the team is each individual member…the strength of each member is the team.”
I have no doubt that Coach Jackson applied his comments to sports, however, the very same could be said for your child’s care team or heart center. Think about it, everyone in your child’s center has their position and assignment, just like a football team.
There may be some debate about who is the quarterback, but it’s routinely stated that the most difficult position in sports is the quarterback position. If you’re an NFL fan, then you also know that without a good quarterback, your team stands little chance of success. Similarly, no heart center can function without their own quarterback, the cardio thoracic surgeon. A job also known to be one of the most difficult specialties in medicine. A team may seem more attractive or flashy if it has a good quarterback, but keep in mind the quarterback can’t do it alone. It takes a team to win.
Every team needs a head coach, someone to see the big picture and make sure the plan is implemented. In the heart world, the head coach is your child’s cardiologist.
The offensive lineman are the unsung heroes in the trenches, using their muscle to make the quarterback look good. The offensive lineman of the heart center are the intensivists.
The interventional cardiologists are the wide receivers on the team. They both require good hands, finesse, and taking precise routes.
In football, special teams are charged with helping the team maintain good field position. The special teamers in the heart center are the nurses. Nurses are tasked with maintaining our kids after a surgery, catheter, or unexpected setback. Special teams are a key component of any football team, just as the nurses are an integral part of the care team.
Quarterbacks need to be in sync with their center, the one responsible for snapping them the ball. They have to have a rhythm and excellent communication to execute a clean exchange of the football. The heart center’s quarterback, the surgeon, similarly relies on the anesthesiologist, requiring the same rhythm and communication for the best chance at success.
Most football teams have a nutritionist. The nutritionist is the running back of the heart center. They are given the play and told to carry the ball through a tight space. Their position is fully dependent on timing and capitalizing on the right window of opportunity.
Most importantly, every team needs a general manager – someone who decides who makes the team roster, takes in all the information, including recommendations from all members of the team, and has final say in ultimate decisions. The general manager of your child’s team is you. So next time you are in the huddle (i.e. interviewing care teams, in rounds, at the bedside) make sure you harness your inner GM and speak up, ask questions, and share concerns.
When deciding on the team that cares for your child, remember “the strength of the team is each individual member.” Choose a well balanced team, that is impressive at all positions, works together, communicates well, reacts favorably to adverse conditions, and gives maximum effort.
Assembling your team can be overwhelming, particularly for a family new to the heart community, however, like any other general manager, you need to learn to lean on your scouts. In this world, your scouts are the parents and families who’ve traveled this road before you, who’ve already assembled their team and taken on the opponent. Use your scouts, don’t hesitate to reach out to heart families via social media and support groups. By reviewing their game film you can hopefully improve the outcome of your child’s journey.
Caleb is father to 7-year-old Emerson and 5-year-old Zoe, who was diagnosed in utero with hypoplastic left heart syndrome. An in utero diagnosis provided early learning, including the importance of cardiac experience when treating and caring for complex CHDs. A second opinion led Caleb and his wife to the Children’s Hospital of Philadelphia where Zoe underwent her three-staged palliation.
As you may have guessed, Caleb is also a fan of football and addicted to fantasy football (are four teams too many?). In his spare time, Caleb is an attorney in Phoenix, Arizona.
Hope for Superheroes: Fontan care packages!
Four years ago, Sisters by Heart was born out of a mission to provide support and hope to newly diagnosed HLHS families. We are well known for our signature Norwood care packages, which include all the essentials a family needs to support a newborn child in the hospital recovering from open-heart surgery – and most importantly, HOPE! We have loved supporting newly diagnosed families as they have begun their journeys and joined our heart family.
But we wanted to do more, to branch out, to provide even more support to the families we love and serve. And so the “Fontan care package” idea was born. Our Board has been hard at work over the past year deciding on the perfect items to include in a care package for a toddler or preschooler recovering from open-heart surgery. We wanted to include items to empower a young child and his or her family, and to pay homage to just how strong these kids are, and just how far they’ve come. And, of course, to give our customary dose of HOPE!
We’re thrilled to announce today is our official launch date for our Fontan superhero care packages!
Before we launched, we needed someone to test out our care package, to make sure it was just right. We were thrilled to have that recipient be this cutie, Kate.
Kate has had a particularly rough go of it, including an ecmo run and a Glenn takedown. She likes to give her medical team a run for their money and it is no small feat that she has arrived at the Fontan. Her family received a Norwood care package when her mother was still pregnant with Kate. And her amazing mama, Erica, is well known and well loved by our board. So there was no question that she was our perfect first recipient!
Our Fontan superhero care packages include a super-Fontan cape and mask, a coloring book specially designed just for SBH and crayons, a hospital gown, silicone bracelets for mom, dad and HLHSer, stickers for siblings and, from time to time, a few extra goodies! And it’s all wrapped up in a cool Fontan superhero drawstring backpack!
Sisters by Heart supports full transparency in outcomes data among hospitals that treat HLHS
Sisters by Heart’s mission is to support, empower, and educate families, particularly newly diagnosed, with Hypoplastic Left Heart Syndrome (HLHS). HLHS is one of the most complex congenital heart defects requiring at least three open heart surgeries, the first to be performed within days of birth. It is essential that HLHS families have accurate, accessible information and data to empower them in making the best decision for their family, including where to seek treatment for their child. To that end, Sisters by Heart supports full transparency in outcomes data among hospitals that treat HLHS and other complex congenital heart defects (CCHD).
The recent story by CNN, “Secret Deaths: CNN finds high surgical death rate for children at a Florida hospital” [http://www.cnn.com/2015/…/01/health/st-marys-medical-center/], was incredibly distressing. Sisters by Heart believes that medical institutions treating HLHS should be fully transparent in their treatment and outcomes data, including but not limited to: the number of HLHS children treated annually; number of Norwood, Glenn, and Fontan surgeries performed annually; survival rates; complications (number and types); and length of stay. In the absence of transparency, families cannot make informed decisions and children can – and do – die unnecessarily because hospitals with bad outcomes are allowed to continue treating these complex, medically fragile children.
A follow up story by CNN two days ago indicated that the federal agency Centers for Medicaid and Medicare Services (CMS) is investigating St. Mary’s Hospital, as the infants’ treatment in question were all covered by Medicaid. While Sisters by Heart is gratified to hear that CMS is looking closely at St. Mary’s, we also know this is not an isolated incident. There are hospitals operating on HLHS and other complex congenital heart defects that should not be. It is particularly unjust to think that our most vulnerable children are being treated at institutions not equipped to treat them, while infants with private insurance have better options available to them.
It should be noted that there are institutions currently making their data publicly available. [See CNN’s list here: http://www.cnn.com/…/2015/06/health/pediatric-heart-surgery/]. However, it is essential that there is consistency in the types of data released and the way that data is measured in order for the data to be truly meaningful for families. And it is our firm belief that every institution that treats HLHS and CCHD children should make their data publicly available.
While voluntary transparency by some institutions is promising, Sisters by Heart believes it may move too slowly and that too many babies will die unnecessarily in the interim. It is our firm belief that not all hospitals currently treating HLHS infants and those with other complex congenital heart defects should continue to treat these children. We call on all pediatric heart programs to publicly release their outcomes data, and to support the empowerment of parents and families to make decisions based on objective, factual information. Sisters by Heart stands committed to ensuring that data transparency happens quickly – whether through voluntary or regulatory action processes.
A Tea Just for You: Heart Mom Comfort Tea!
Just in time for Mother's Day, Sisters by Heart is proud to announce a new partnership! The Herb Stop, located in Pine, Arizona, (owned and operated by a heart mom/daughter duo - Leilah and Natalie) developed Heart Mom Comfort Tea especially for Sisters by Heart and heart moms everywhere!
Heart Mom Comfort Tea's proprietary herbal blend was hand-selected by herbalists Leilah and Natalie, to bring comfort and calming to heart moms. The Tea comes with a message of hope and comfort for all heart moms (and dads) - and, at the same time, supports newly diagnosed HLHS families, as proceeds from sales will benefit Sisters by Heart.
For the month of May, The Herb Stop is selling Heart Mom Comfort Tea with an adorable heart-shaped filter for only $17.95!
To sweeten the deal even more, Herb Stop is offering a gift set to one lucky winner which will include a jar of Heart Mom Comfort Tea, a Heart-Shaped Filter, Walnut Scrub, Herbal Bath Bags and a bottle of Lavender Oil. Everyone who places an order in May gets a chance to win!
After May, the Tea will sell for $15.95, with the filter sold separately. Make sure to grab yours now to get Heart Mom Comfort Tea and the heart filter for you or a special heart mom in your life!
Read more about Herb Stop owners, Leilah and Natalie's journey with congenital heart defects, and why our CHD community has inspired them!
by Leilah Brietler (Founder of the Herb Stop)
When my daughter Natalie was born, she was definitely ready to join the world. Labor was quick. We could not avoid going through a few red lights as we made our way to the hospital. We checked in at 3 a.m. At 6:35 Natalie made her arrival, without complications and, surprisingly, without that usual first baby cry. With great joy I held my daughter. Our eyes met and in that moment knew we were meant to share life’s journey. That sacred moment was brief. A nurse soon entered the room. She swiftly took Natalie from my arms and, without explanation as to what was happening, my beautiful daughter was placed in intensive care. Suddenly, all alone, I was left to wonder what was wrong. My precious daughter looked beautiful and perfect, why was she taken from me? I knew there must be a mistake. I waited. After a few, very long, hours of not knowing, a pediatrician entered the room and I learned of Natalie’s heart conditions.
Our first appointment with our pediatrician was intense. Natalie was placed in a tightly fitting, tube-like machine with her arms above her head. She screamed profusely, almost turning blue. This experience was extremely traumatizing both for Natalie and for me. I felt alone and without support. My husband was working and unable to be with me. Our two-year old son was home with a babysitter. The nurse took an exhausted Natalie out of this tube-like machine and handed her to me to dress in preparation for meeting with our doctor. The attending pediatric cardiologist entered the room. He sat down and drew a picture showing Natalie’s numerous heart conditions. In the next few, though to me they felt endless, minutes, he explained in detail the conditions that affected Natalie’s heart. It all ended with a prognosis of doom and gloom. Because her survival was at stake, she would require weekly monitoring at the hospital. Her heart condition was considered so severe that it carried the likelihood of open-heart surgery at a young age.
I was devastated. In the cold and sterile environment of the hospital I sat in shock. How could this brave, perfect looking little girl have such life threatening problems? Why was there nobody there to comfort me, to console me, to empathize, or to offer hope?
After many sleepless nights crying and worrying, I prayed and pleaded for help. As an herbalist I sought connection through the use of herbs. There I found both answers and hope. I raised Natalie with daily herbal support. I also taught her always to keep love in her heart and to be a joyful person. The heart, after all, is the center of love.
Today Natalie is 33 year old. She is a vegan, a weightlifter, a popular herbalist and a successful businesswoman. Every day it is my joy to work by her side. As a team Natalie and I own and operate the Herb Stop in Pine, AZ. Natalie is monitored every six months by two cardiologists. Surgery is still not in the picture. She is without symptoms and lives life to the fullest knowing that her heart is delicate and needs daily support.
I feel close to the “Sisters by Heart” because I believe in the way they reach out in support of one another so that other women do not have to suffer alone and without answers as I did. Natalie also shares this tie to “Sisters by Heart.” On some level I am sure she remembers the traumatic experiences she had to endure upon her arrival into this world. All these brave young mothers are truly our sisters by heart.
Parent Travel Scholarships - NPC-QIC 2015 Spring Learning Session!
Registration for the NPC-QIC 2015 Spring Learning Session is now open! Sisters by Heart and NPC-QIC want to send YOU to the Spring Learning Session May 1-2, 2015! Together, Sisters by Heart and NPC-QIC are once again offering parent travel scholarships, valued at $500 each, to four parents to attend the learning session in Cincinnati, OH. If you are new to the HLHS world, haven’t heard of NPC-QIC before, or have questions about what NPC-QIC does, we have put together some information for you.
The National Pediatric Cardiology Quality Improvement Collaborative’s mission is to improve the care and outcomes for children with cardiovascular disease. NPC-QIC’s current quality improvement project is working to improve survival and quality of life for infants with Hypoplastic left heart syndrome (HLHS). The collaborative is made up of clinicians from fifty-eight (58) centers from across the country and parents of children needing a Norwood (i.e. HLHS).
Last November, HLHS parents Erica Isakson (Colorado) and Matt Ulrey (Florida) attended Learning Session 10 for the first time after being awarded a parent travel scholarship. Their testimonials speak volumes to the work NPC-QIC is doing to improve the lives of children born with HLHS and their partnership with HLHS parents:
"Attending the NPC-QIC Conference was rewarding at multiple levels: First, being in a room of cardiac professionals and parents who are focused and are driven to improve the care of our HLHS kids, and their families, touched my heart and made me further grateful for these dedicated people. I also appreciated hearing that the current Collaborative statistics (which are really good!) still aren't good enough for these professionals. Our kids are fiercely being fought for behind the scenes (as well as on the front lines) and that is incredible to see, first hand. Finally, this group allows their centers to be vulnerable for the sake of improving the HLHS outcomes and that is amazing!
I look forward to be a contributing part of the Collaborative efforts at future conferences and at our center, and hope to share the work being done to encourage and bring hope to newly diagnosed, and current, HLHS families in Colorado and surrounding areas.
Thank you so much Sisters-by-Heart for the opportunity to meet so many amazing people and to continue my efforts to help our center help more HLHS families! It was great to finally meet all of you, too!" - Erica Isakson
"As a Dad of a 3 year old (Roman) with HLHS, I would like to start off by thanking Sisters by Heart for the great opportunity to attend the fall 2014 learning session. Leading up to the learning session I was a little nervous it was going to be a bunch of healthcare professionals speaking in a langue I didn’t understand. Once I got there I realized I could not have been more wrong. The learning session featured individuals from about 50 centers which included doctors, nurses, and parents. They all come together for one common goal - Improving care for patients with HLHS and their families. I am not sure what I enjoyed more either participating in the learning session – or getting to interact with other parents of children with HLHS of all different ages.
During the learning session it was clear that the medical professionals really valued our opinions. I was asked several different questions while I was there ranging from time of diagnosis all the way up to his current care. I know all HLHS parents remember that moment when they find out their child has a life threatening condition. During the learning session we spent hours discussing what could have made the time after that moment better. We heard great examples of things to do, and horrible examples of what to avoid.
I also was excited to meet with parents of other children with HLHS. I was able to meet parents with children of all ages from babies to college students. Hearing their stories provided me great hope. Generally as a parent of HLHS you are always looking so short term; this was a good opportunity to look long term. I thought it was awesome to hear everyone’s stories, especially the ones about the older children.
One last aspect of the conference I wanted to mention was how great it was to see hospitals working together to better care. It would be really easy for the top hospitals to keep all there data in-house and not share it with other hospitals, but that does not happen with this group. All involved are excited and even eager to share their best practices and success stories.
I came away with great comfort knowing there are some many intelligent people who care so greatly about our children, and look forward to being able to attend future learning sessions." - Matt Ulrey Dad of Roman age 3
Scholarship Awardees - Erica, Scott, and Carmen
Parents with a single ventricle child, who required a Norwood or Norwood varient surgery may attend the learning session and/or apply for a travel scholarship. You do not need to be currently involved with your child's interstage clinic, however you are encouraged to reach out to your child's center prior to the learning session.
Sisters by Heart will be accepting travel scholarship entries until March 16, 2015. In order to be considered, please send an email with "Learning Session Scholarship" in the subject field to info@sistersbyheart.org with the following information:
Name
Address
HLHS child's name and DOB
HLHS child's hospital
Have you contacted your child's center to inquire about funding to attend the Learning Session?
A brief paragraph on why you would like to attend
If you are interested in attending and can make arrangements for childcare/time off work, please apply! This is such a wonderful opportunity to share your opinions, answer questions from a “professional” perspective as a parent, and most importantly be involved in helping to improve care for HLHS children.
Awarded scholarships will be announced via email by March 23, 2015. Parents awarded scholarships will be responsible for making their own travel arrangements. If you have questions regarding the scholarship process, don't hesitate to email us at info@sistersbyheart.org.
Parent Dinner, Cincinnati 2014
If you're already planning on attending the learning session, please make sure to register for the event at http://events.r20.constantcontact.com/register/event?oeidk=a07eaftn5jz6f...
In order to receive the discounted room rate for the conference, please use this link to make hotel arrangements: http://events.r20.constantcontact.com/register/event?oeidk=a07eak0c1qi1cab2988&llr=kdgqiilab
Sisters by Heart Board Members, Cincinnati 2014
We look forward to seeing you in Cincinnati in May!
HLHS Collaborative: January's Action Period Call
As promised, and in conjunction with HLHS heart dad, Trent Neely, the following is a summary from January's NPC-QIC Action Period Call:
January's call continued some robust conversations regarding Parent Engagement and Social Media. The discussion began with some changes in the data used to measure if centers are engaging and supporting parents - adding the key component of “Support." While it remains important to engage parents in the care of their children, the support that parents receive on their journey is vital as well. A number of centers commented on the importance of education and preparation, and how to avoid overwhelming parents with too much information.
The discussion then shifted to the use of social media and how parents and centers are using it to connect and communicate. They highlighted the connection that the NPC-QIC has made with Sisters by Heart and how they are working together to share information with both hospitals and parents. With the current divide between caregivers and parents in social media, this is a valuable bridge that is being built to provide a better online resource. Both organizations are utilizing Facebook and Twitter as social media outlets and encourage parents to join or follow them there. There was also a conversation about individual centers utilizing these resourses as well. Click the hyperlinks if you'd like to follow NPC-QIC on Twitter or Facebook. Sisters by Heart also has a Twitter and Facebook page if you've not located it yet.
The current project that the NPC-QIC is working on is care of HLHS patients through the interstage period. This work has been ongoing for years and will continue. They announced today that work will begin on Phase 2 and will center on Neonatal care. The focus will be on the hospitalization from birth through the Norwood surgery and recovery.
The NPC-QIC Learning Session will be held again in Cincinnati, Ohio on April 25th and 26th. Parents interested in attending should email jcchdqi@cchmc.org. Parents can also connect with their cardiac center's collaborative team for more information.
As a follow up to January's Call, feel free to comment on this blog post and share your experiences regarding how your cardiac care team engaged you, if they are active on social media outlets, and any advice you'd like to leave for the collaborative in this regard. Many cardiologists and centers follow our blog, so share away!
As always, thank you Trent Neely, for creating a summary of the monthly action calls for all parents to see!
Fundraising with Heart: Tink & Key
Today, on the second day of Heart Awareness Month, Sisters by Heart is thrilled to share our partnership with Tink & Key to promote CHD awareness!
If you haven't discovered Tink & Key, you're missing out! Tink, owned by a fellow heart mom, sells adorable tees and jeans for kids and adults. They're probably best known for their "Zipper Club Member" tees.
Need we say more?
Even if you don't have a child with a heart defect, there are several adorable items suited for all family members (even mom and dad)!
NPC-QIC Action Period Call Update
Thanks again to Trent Neely (dad to Avery, HLHS), for January's call summary. If you're not a member of NPC-QIC's HLHS Parent Advisory Facebook Page, please check it out. It's a great place for updates and a simple avenue to engage in the HLHS collaborative. For those new to the HLHS community, the National Pediatric Cardiology Quality Improvement Collaborative holds monthly calls to discuss ongoing work and action items to continue tackling HLHS and improving the treatment and outcomes for our children.
January 2015 Call Update
After a winter holiday break, the Action Period calls resumed with a flurry of activity! The call began with an announcement of three new centers to the Collaborative, bringing the total to 58 centers. Welcome UF Health Shands Children’s Hospital, Golisano Children's Hospital, and University of Minnesota Masonic Children's Hospital.
A report of research followed, with a study conducted through the NPC-QIC database, examined the timing of Stage 2 palliation. There is wide variety in the timing of the second surgery between centers and this study looked to evaluate any risks or benefits to an early or late timing of Stage 2. Of the data analyzed, there was a variation in timing that ranged from 3 ½ to 7 months with the early group having Stage 2 prior to about 5 months. There were some interesting findings that I’m sure will help centers think about the optimal timing of surgery and what shorter or longer wait times for the Glenn might translate to long-term. This research is currently being prepared for publication so look for it in an upcoming Research Explained from the NPC-QIC. An abstract is available here: http://circ.ahajournals.org/conte…/…/Suppl_2/A12605.abstract
An update on a new project to develop a Book of Hope through Empowered by Kids was then provided. This book will include stories from parents, patients, nurses, a cardiologist, and a surgeon to help newly diagnosed parents through their journey. Deadlines for submissions are January 31, 2015, so get your submissions in ASAP! If you need further information, please read our recent blog post on the HLHS Book of Hope.
There was then a brief discussion of funding opportunities that the Collaborative is pursuing to help further the current work as well as the planned Phase 2 project. Phase 2 is moving forward and building upon Phase 1 which was limited to just the Interstage period. Phase 2 will focus on diagnosis through the first year of life and will include additional data about neuro-development and family quality of life. Phase 2 will also incorporate parent reported data collection as well.
There is a lot of new and exciting developments for the Collaborative; it's energizing to see the ongoing work for HLHS children and families, and in turn, improving the care and treatment for all critical congenital heart defects.
Fundraising with Heart: The Baby Blue Sound Collective
Last month, Sisters by Heart was honored to attend Baby Blue Sound Collective's performance in Miami, FL. The Baby Blue Sound Collective is a musical ensemble composed of pediatric heart surgeons, cardiologists, nurses, and other CHD care providers from around the country. The BBSC currently performs at many of the pediatric cardiology and cardiac surgery medical conferences throughout the USA and Europe: the personnel of the band is different for each performance, and primarily depends upon who is on the course faculty at the particular meeting. The songs and genres vary, but include classical, jazz, pop, musical theater, blues and rock.
We were blown away by the talent of this incredible group of clinicians! The BBSC encouraged those in attendance to make donations throughout the night - and through their generosity, Sisters by Heart raised over $600! We are so grateful to Dr. Gil Wernovsky and the entire BBSC crew for their continued dedication in supporting CHD families.
Sharing HOPE: An exciting opportunity
Hello Sisters by Heart Family! SBH is invited to participate in a hugely impactful project with Empowered By Kids.
Sisters by Heart was founded from a deep desire to spread Hope to newly diagnosed families. How would you have reacted to a "Book of Hope" containing stories of children and families living with an HLHS diagnosis at the time of your diagnosis? Sisters by Heart feels strongly that sharing your hope and inspiration with families who are walking our journey can be a soul-shifting event for the newly diagnosed. The "Book of Hope" will be provided in Sisters by Heart care packages and given to care centers across the country at no cost for distribution to newly diagnosed families.
We'd love to capture a few dozen incredible stories to include in the HLHS "Book of Hope." To participate, please prepare a write up (250 word maximum) outlining your journey with HLHS. Your story should reflect words of inspiration that you needed at diagnosis - reflecting that it is indeed not fair, but you will get through this and there are brighter days ahead. You can also supply a photo or artwork for the book.
No demographic data will be revealed in the Book of Hope. Your story will include you/your child's first name or patient name (for teen/adults). Patient ages will be included.
Empowered by Kids has worked with two other pediatric rare disease communities to create their Books of Hope, which can be viewed here:
NPC-QIC 2014 Fall Learning Session!
Registration for the NPC-QIC 2014 Fall Learning Session is now open! Sisters by Heart and NPC-QIC want to send YOU to the Fall Learning Session November 7-8, 2014! Together, Sisters by Heart and NPC-QIC are offering parent travel scholarships, valued at $500 each, to three parents to attend the learning session in Cincinnati, OH. If you are new to the HLHS world, haven’t heard of NPC-QIC before, or have questions about what NPC-QIC does, we have put together a little information for you. The National Pediatric Cardiology Quality Improvement Collaborative’s mission is to improve the care and outcomes for children with cardiovascular disease. NPC-QIC’s current quality improvement project is working to improve survival and quality of life for infants with Hypoplastic left heart syndrome (HLHS) during the "interstage" period between discharge from their initial open heart surgery- Stage 1 Norwood - and admission for a Stage 2 biredirectional Glenn procedure. Fifty-five (55) centers from across the country have come together with parents to improve the care for HLHS babies.
Ask any parent who has attended a NPC-QIC learning session about their experience and their reaction will be the same; it is life changing. Natasha Sawyer, Sisters by Heart board member, shared her experience on her personal blog after attending her first learning session in January of 2013:
“The conference was more than I could have ever imagined it would be. I'm not sure what I expected, but it definitely wasn't this. Teams consisting of cardiologists, nurse practitioners, nurses, dieticians, therapists, social workers, and parents filled a large conference room and the excitement began. Over 100 people were in attendance, including 15 parents, representing centers from across the country. From the moment I walked in, I felt welcomed. I felt important. Doctors and team members expressed how thrilled they were to see so many parents in attendance and how crucial we are to the success of the collaborative.
Before the conference, I did not fully comprehend how amazing pediatric cardiologists and their team members are. Sure, I knew it took a special kind of person to work with babies and children with life-threatening heart defects, but until this weekend, I did not see it as more than just their "job". Please bare with me for a few minutes, I am still on an "emotional high" from the past two days and I am desperately trying not to jump up and down and shout for joy while I try my best to describe what I witnessed.. The members of the NPC-QIC have more passion for saving Hypoplastic Left Heart Syndrome babies than you can even begin to imagine. They are not satisfied with their current programs. They are not satisfied with the percentage of babies surviving from the first to second surgery, even though just 30 years ago these babies were being sent home to die. They are spending so much time and energy going above and beyond what is expected of them to change the future of children born like my son. They are changing the quality of life for these kids- kids and families they have yet to meet and have no tie to! I used to think Cardiologists were keeping their ideas to themselves, not wanting to share their "trade secrets" with anyone else. This weekend, I saw how open every team was to sharing their "secrets" with their colleagues. I watched cardiologists and team members from different centers take notes as another shared what is working for their center.
This weekend, I saw not only passion for caring for HLHS kids, but a fire to continue to make advancements and improvements for these children. I am so humbled by this experience, and feel forever indebted to these physicians and care team members who are helping our children continue to defy the odds. I cannot wait to share everything I have learned with my team's collaborative coordinator.” Natasha Sawyer, HLHS Mom, http://www.ramblingsofaheartmomma.blogspot.com/2013/01/npc-qic-2013-wint...
Parents with a single ventricle child, who required a Norwood or Norwood varient surgery may attend the learning session and/or apply for a travel scholarship. You do not need to be currently involved with your child's interstage clinic.
Sisters by Heart will be accepting travel scholarship entries until September 1, 2014. In order to be considered, please send an email with "Learning Session Scholarship" in the subject field to info@sistersbyheart.org with the following information:
Name
Address
HLHS child's name and DOB
HLHS child's hospital
A brief paragraph on why you would like to attend
If you are interested in attending and can make arrangements for childcare/time off work, please apply! This is such a wonderful opportunity to share your opinions, answer questions from a “professional” perspective as a parent, and most importantly be involved in helping to improve care for HLHS children.
Awarded scholarships will be announced via email by September 8, 2014.
Please make sure to register for the event using the following link if you will be attending the Fall Learning Session! http://events.r20.constantcontact.com/register/event?oeidk=a07e9kkxcx922...
We look forward to seeing you in Cincinnati in November!
Using Crisis to Create: The Asher James Foundation
In June, Sisters by Heart President, Stacey Lihn, presented at the Mended Little Hearts' National CHD Symposium. Following, she was able to meet and talk with HLHS parents across the country. Jen DeBouver is one of many parents that inspired Stacey, and we've asked Jen to share her journey and advocacy efforts - to educate parents on the risks of blood clots in our CHD community and how you might be able to help.
When I was pregnant with my son, my second child, I thought nothing could go wrong – not this time. I had just lost my daughter Olivia at 27 weeks. She no longer had a heartbeat the doctor told us. When it came around 26 weeks of pregnancy with Asher, I was a nervous wreck. My husband and I were at a 26 week check-up when the ultrasound tech left to get the doctor. I couldn’t believe something was happening again around the same time as Olivia. When the doctor returned, he told us that he wanted us to get a second opinion on Asher’s heart because he saw something but wanted to make sure.
We had an echo done and found out that Asher had Aortic Stenosis. The doctor gave us two options – wait until he was born in which his heart defect would develop into HLHS or have a fetal intervention. As a parent we wanted to do the best possible thing for our baby so we decided to do the fetal intervention. A week later we flew from Chicago to Houston to have the fetal intervention.
When we got to Texas Children’s, it was a full day of testing to see if we were a candidate for the fetal intervention. This is when we learned that in a week, Asher’s heart had gotten worse. He had developed coarctation of the arch and had leaks in his mitral and tricuspid valve. The cardiologist started crying and told us that our baby had no idea that he was sick. We met with the entire team and one of the doctors told us that without the fetal intervention, Asher wouldn’t make it to birth and that it was a Hail Mary that he would make it through the procedure. Asher was born on September 10, 2012, at 36 weeks – 9 weeks after the fetal intervention. After we had a very successful fetal intervention, we started calling Asher our little Fighter. Hours after being born, he had to have a cath procedure to re-inflate the balloon that was inserted during the fetal intervention. At 11 days old, he underwent his first surgery to fix his aortic arch.
Asher rocked every procedure and was doing amazing. But at 4 weeks old all that changed. One day Asher looked amazing and then the next he started looking red and puffy and continued getting puffier from there. After 2 days of being swollen, we found out that blood clots had developed in his jugular veins and arms, restricting blood flow from his head to his heart. The hematologist tried different medications on him, some seemed to work and some didn’t. She told us that they were using scaled down adult medications because there aren’t any medications designed for children. She also told us that there weren’t any foundations supporting blood clots in children.
Asher lived for 6 weeks before he lost his battle to these blood clots on October 23, 2012. After everything that his hematologist told us, I knew that I wanted to continue Asher’s fight by starting a foundation in his memory for children’s blood clots. So a few months later, my husband and I started the Asher James Congenital Heart Disease & Thrombosis Foundation. We wanted to let others know that their children were at risk for clots when hospitalized because we had no idea this could happen until it happened to us.
This year we held our first Children’s Blood Clot Awareness Day at Ann & Robert H. Lurie Children’s Hospital of Chicago to inform families and staff of blood clot awareness. We spoke on Capitol Hill to both Congress and the Senate on children’s blood clots and shared Asher’s story.
In the Fall, we are holding our first blood clot awareness walk. We know that Asher is still with us and is making a difference in children’s lives and we continue his fight through awareness, and one day, research in children’s blood clots. Find out more about our foundation at www.asherjamesfoundation.org.
Thank you, Jennifer, for sharing Asher's story and your courageous fight to raise awareness and answers for both the CHD community and all children at risk for thrombosis. Feel free to "like" the Asher James Foundation on Facebook at https://www.facebook.com/asherjamesfoundation and show Jen your support by commenting on the Foundation's page and/or sharing your story about how thrombosis/blood clots affected your CHD journey.
CHD Kids and School Special Services
Summer is almost over and many HLHS children are headed to school - for the first time or as returning students. Trent Hamilton, HLHS dad and Middle School Principal, generously shared his wealth of information with Sisters by Heart on educational benefits and services for CHD children in the public school system. Thank you, Trent, for sharing this information with our families!
Several people in our CHD support group have recently had questions about getting special services for the kids who are starting school this year. Specifically, we have been discussing the differences between Special Education services through the Individuals with Disabilities Education Act (IDEA) and Section 504 of the Rehabilitation Act of 1973 (Section 504), and which would be appropriate for their CHD child. As a school principal, I deal with both Section 504 and IDEA students on a daily basis. I hope that the following blog will help you make the right decisions to provide assistance and protection to your CHD child as they start the new school year.
Basically, the two laws are similar in that they both provide educational benefits to kids with disabilities, but they differ in their eligibility requirements and the types of benefits that are provided. The requirements for eligibility under IDEA are stricter than those under Section 504, but IDEA provides more benefits than does Section 504. Essentially, all students under IDEA are also protected under Section 504, but not all students under Section 504 are protected by IDEA. It is easier to place a student under Section 504 because it has less procedural criteria, but these students are generally provided less assistance and monitoring by the school. IDEA status is more difficult to get because there are more specific criteria and it has much stricter regulation, but it does provide more types and degrees of assistance and special services to the student. Section 504 protects people for their lifespan in many areas, including school, employment, and transportation, while IDEA only addresses the special education of students from ages 3 to 21.
Students are eligible for IDEA if they have a specific impairment or condition that adversely affects their educational performance. These impairments/conditions can include, but are not limited to: autism, learning disabilities, speech/hearing impairments, emotional disturbance, or other health impairments, but the condition MUST adversely affect their education. Students are eligible for Section 504 if they have a condition that substantially limits a major life activity, even if the condition does not affect their education. Major life activities include: walking, seeing, speaking, breathing, learning, and working. But Section 504 conditions do NOT have to have an educational affect.
IDEA requires that students be placed in the most appropriate learning environment for them individually. This can include regular or special education classes. It also requires that each student has an individualized education program (IEP), which provides specific modifications and accommodations to help the student succeed academically. Section 504 generally places students in a setting that is comparable to that of students who are not disabled. It also provides some accommodations, but these are usually used in the regular educational setting.
So, which type of services is right for your child? This depends on the level that your child’s CHD has affected them and their development. If your child’s CHD caused them to have any type of developmental or learning disability, then IDEA is probably the right program. If your child’s CHD slows them down physically, but has not affected their learning or development, then Section 504 is probably best. There are obviously exceptions to these, so be sure to consult your child’s school if you aren’t sure which is right for you.
You might be wondering why you need any type of service at all if your child doesn’t have any learning or developmental issues. As a principal and a CHD parent, I highly recommend that you pursue some sort of protection for your child at school. While your child may not need accommodations or special services in order to succeed academically, they may need some of the other protections that are provided under Section 504. One of these protections involves attendance. Many states require that students attend 90% of the available school days and, even if an absence is for approved medical reasons, a student can be retained for excessive absences. If a student has protection under Section 504, special accommodations can be made in the event of an extended illness or hospitalization. Also, your child may not experience severe educational deficits early in their school years, but if they do later in life, having a 504 plan can make it easier to get accommodations or even move to IDEA protection. Section 504 also makes it easier to get certain exemptions from activities, like running, lifting weights, etc. In addition to all of this, the Section 504 plan follows your child through school as a record and every new teacher is required to review it during the school year. This means that each new teacher will be notified of your child’s condition and know what your child needs.
I hope that this information helps you in making the right educational decisions for your CHD child. If you still have questions, I encourage you to speak to your school’s counselor or special education diagnostician. Again, your CHD child does not have to have either one of these services, but I assure you that my son (HLHS) will, at least, have a Section 504 plan.
Trent Hamilton
Heart Dad & Middle School Principal
HLHS Collaborative: June's Action Period Call
The Action Period call started as usual, with the typical data review. For those who have allowed your center to share data with the Collaborative, it is collected for research and quality improvement. To date there are 1304 patients in the registry. Despite the typical data review, the focus was on the mortality rate because decreasing mortality is one of the primary goals. Prior to the inception of the Collaborative in 2008, interstage mortality rates in the medical literature were around 10-15%. The NPCQIC mortality rate at times seemed to hover around 10%. However, when looking at all the data, it shows that the mortality from 2008 to 2014 is 8.1%. Then came the big news, when looking at a more recent period, from January 2013 to May 2014 the Collaborative interstage mortality rate was 6%! This was the first time that the Collaborative was able to statistically show a reduction in mortality. The thought is that by working together, the involved centers were able to improve care processes that impact care. There was excitement and celebration, but also a humbleness that 6% is still not good enough. This is only the mortality rate for infants from Norwood discharge through the interstage period to Glenn surgery. Moving forward there will be a continued focus on this interstage time period but also working with clinicians who care for these infants during this earlier time frame to find additional ways to decrease mortality. It is always encouraging to see practitioners get excited about accomplishments and then get right back to work to find more ways to improve, especially when it directly impacts the care of your own child.
After the big news the focus shifted to a second main goal of the Collaborative, caloric intake. The discussion revolved around how centers document caloric intake and weight gain for all infants they see. Representatives from Advocate Children’s, Primary Children’s, and LeBonheur Children’s all shared ways that they track caloric intake and weight gain as well as who is responsible for doing so. Advocate Children’s tracks it like other vital signs like blood pressure and heart rate to give an example of the importance this has.
The call ended with a number of announcements. Nemours A.I. DuPont Hospital for Children in Wilmington, Delaware is the latest addition to the Collaborative bringing the total number of centers to 55. The fall Learning Session will be on November 7 & 8 in Cincinnati. The final announcement was that the Parent Page on the NPC-QIC website is under construction to help improve the resources available for parents. After that all signed off to get back to improving care with a heartfelt thanks from all of the parents!