Research Matters: The Importance of CHD Surveillance

research matters

For the month of October, PCHA will be focusing on the theme of Research. In the first post of our series, Dr. Matt Oster provides an overview of a recent study that estimates the prevalence of CHD across all age groups in the United States and highlights the importance of surveillance in improving outcomes for CHD across the lifespan.

 

Congenital Heart Defects in the United States: Estimating the Magnitude of the Affected Population in 2010

By Matt Oster, MD, MPH

Congenital heart disease (CHD) is the most common and critical birth defect. Medical research has led to groundbreaking advances in identification and treatment of CHD. While we have learned enough to improve the survival rate to where most babies born with CHD will live to adulthood, there is still so much we don’t know.

Despite how common, critical, and costly CHD is, the understanding of the public health impact of CHD is surprisingly limited. In fact, we cannot accurately answer the basic question of “How many people with CHD are currently living in the U.S.?”

The American Heart Association, in their journal Circulation, recently published a study, Congenital Heart Defects in the United States: Estimating the Magnitude of the Affected Population in 2010, that mapped Canadian CHD statistics onto the U.S. population. The study’s main findings as outlined in the Centers for Disease Control and Prevention (CDC) Key Findings summary included:

  • Approximately 2.4 million people were estimated to be living with a CHD in the United States in 2010. About 1 million of those were children under the age of 18 years and about 1.4 million were adults age 18 years and older.
    • About 12% (289,000 people) were estimated to have a severe CHD.
  • There were slightly more women (1,260,000) than men (1,163,000) living with a CHD in the United States.

However, the authors of the study also draw these additional conclusions –

Our estimates highlight the need for two important efforts:

  • Planning for health services delivery to meet the needs of the growing population of adults with CHD.
  • The development of surveillance data across the lifespan to provide empirical estimates of the prevalence of CHD across all age groups in the US.

First authorized in 2010 by the Congenital Heart Futures Act, the Centers for Disease Control and Prevention and the National Institutes of Health have begun to take steps to address this burden, needing additional resources to continue and expand their efforts. Continued federal investment is necessary to provide rigorous epidemiological and longitudinal public health surveillance and public health research on infants, children, adolescents and adults to better understand CHD at every age, improve outcomes and reduce costs.

Efforts by patient advocacy groups such as the Pediatric Congenital Heart Association are essential to ensure the further development of systems to provide surveillance data to better understand CHD across the lifespan.

Oster_0811_largeDr. Oster is a pediatric cardiologist at Sibley Heart Center Cardiology at Children’s Healthcare of Atlanta. He holds Emory appointments of Assistant Professor of Pediatrics in the School of Medicine and Assistant Professor of Epidemiology in the School of Public Health as well as an appointment as a medical officer at the CDC’s National Center on Birth Defects and Developmental Disabilities. He earned his MD at the University of Pennsylvania School of Medicine and his MPH in epidemiology at Emory University Rollins School of Public Health. He completed residency training in pediatrics at the University of California-San Francisco and fellowship training in pediatric cardiology at Emory University. When not seeing patients, he serves as director of the Children’s Cardiac Outcomes Research Program at Sibley Heart Center. His primary research interests include the epidemiology of CHD and long-term outcomes for patients with CHD.

A Timely Reminder from Texas Children’s Hospital

Dr. Eboni Smith from Texas Children’s writes a timely reminder of monitoring for signs of developmental challenges and seeking out help during this back-to-school season. 

School is now in full swing and the change in seasons is just around the corner. The beginning of a new school year is an exciting time for both parents and children. Days are filled with new schedules, homework and extracurricular activities which can make it very easy to miss the signs that your child is in need of an evaluation or check-up. Continue reading

School Intervention Series: Advocating for a Program

In her final post of a three-part series, Kyle Herma, School Intervention Specialist at Children’s Hospital of Wisconsin, details how to start advocating for a dedicated school liaison at your cardiac center. If you missed the series, you can find her first post here and Complete Resource Guide here.

Advocating for equal accesses to quality education for children with complex health needs is often a difficult process. At times, the numerous boundaries families face seem to make it almost impossible to get appropriate evaluations and support services in place, especially within schools. It is in the untangling of these messy webs of communication and information where I find some of my most fulfilling work. Having a dedicated school liaison position within your cardiac center (usually as part of a multidisciplinary neurodevelopmental follow-up team) is an ideal situation for receiving whole-child focused, comprehensive care; however, there are many ways for parents to step in and be the driving force in centers that have not yet established these types of innovative programs.

Understanding Neuropsychology

Neuropsychology is the study of the relationship between the brain and behavior. During a neurodevelopmental evaluation a child’s level of cognition and intellectual functioning, emotional and behavioral functioning, and social functioning are assessed. Each assessment will track milestone progress in areas such as: motor skills, play skills, feeding, language development, growth, nutrition, and hearing. The goals of this type of assessment are to identify the child’s ability to function in a group of same-age peers, identify the factors that influence their actions and reactions, determine how levels of functioning  are influenced across different medical treatment/intervention stages, determine the response to or recovery from specific treatments, monitor overall brain development, and provide recommendations for schools in effort to implement appropriate special education services and other learning supports. A neuropsychological evaluation is typically recommended for children between the ages of 6 month to 18 years, who are at high risk for developmental disorders, disabilities, or differences. While the child’s age determines the way they are evaluated, this assessment usually consists of formal pencil-paper testing and interactive completion tasks such as match-making, completing patterns or sequences, and following oral directions. In addition, the neuropsychologist/psychologist will review psychosocial family factors, as well as the child’s developmental and medical history.

The Benefits of a Cardiac Neurodevelopmental Follow-up Program

Children with congenital heart disease are considered high-risk for developmental differences and delays due to many factors related to their medical history, including medication, treatments, and surgical repairs. Fortunately, research also shows that with early identification of these learning delays and appropriate follow-up services put in place, these children can go on to lives long and successful lives.

Neurodevelopmental follow-up programs are designed around a multidisciplinary team of experts who conduct regular, comprehensive assessments of a child’s growth and progress in all areas of functioning and development (also called “neurodevelopment”), and provide families with important information, recommendations, and resources needed to ensure the best possible educational outcomes.  There are several school-age transition points that tend to show an increase of challenges (for example: 3rd grade is a time when children become more independent at school, thus learning difficulties become more evident; the transition from 5th to 6th grade requires a shift in complex problem-solving and organizational skills, and so on). Ongoing neurodevelopmental evaluation is recommended as it is typical for new concerns to arise at different developmental stages.

Once a neurodevelopmental evaluation has been conducted, families will have a better understanding of their child’s overall level of functioning and specific cognitive strengths and weaknesses. Recommendations may be given for academic assistance in terms of accommodation or modification in school or for further psychological or psychiatric therapies/treatments/evaluations. Recommendations may also include planning for transitional service from pediatric to adult care. Most evaluations will also conclude with recommendations for continued skill development at home, ideas for discipline and/or behavior management, and additional resources for support.

Advocating for Neurodevelopmental Follow-up, School Intervention, and Like-programs

I always recommend that my families educate themselves on the developmental milestones of “typically developing” children. I whole-heartedly believe that all children are different and hit “normal” milestones at all different times, but early identification of differences or delays (even if it’s just scheduling an assessment or evaluation) overwhelmingly leads to higher overall academic success rates.

Once you’ve noticed a concern it is important to talk to your child’s primary care provider (general pediatrician) or cardiologist right away. These medical providers will be able to listen to your concerns and help identify action steps (i.e. request an Individualized Education Plan, set up a Neurodvelopmental follow-up, etc). In the event that your cardiac center does not have formal programs in place to assess and assist with neurodevelopmental and educational challenges, there are still ways to seek necessary support. For example, most cardiac programs have a dedicated social worker. A family might request to work with a social worker to express school concerns and connect with existing resources or school support services found within the community. The social worker might be able to schedule a meeting with a hospital-based psychologist or child life specialist who can further assist if your cardiac center does not have these as dedicated cardiac positions.

Parents as Advocates

Parents are often the strongest driving force behind hospital innovation. If your cardiac program does not have access to neurodevelopmental or school support services, you should be asking the question, “why not?” I encourage families to ask their providers, “where do you send your patients for neurodevelopmental follow-up?” (as opposed to the question, “do you offer any neurodevelopmental follow-up?”) and push them to make those hospital-based and community connections to complete their child’s medical team needs. Stay vocal and active in this movement to make neurodevelopmental follow-up and school liaison services part of the expected standard of pediatric healthcare, specifically in the area of cardiology, where this type of comprehensive medical follow-up program is still very new.

Wan tot learn more about the Herma Heart Center’s Neurodevelopmental Follow-up Program? Visit http://www.chw.org/medical-care/herma-heart-center/programs/developmental-follow-up-program/

Want to learn more about the Herma Heart Center’s School Intervention Program? Visit http://www.chw.org/medical-care/herma-heart-center/programs/school-intervention-program/


Kyle Herma

Kyle Herma is the School Intervention Specialist serving the Herma Heart Center at Children’s Hospital of Wisconsin. Kyle has been at Children’s since February 2015 conducting a formal pilot study on school intervention and the impact it has on a child’s overall medical outcome and quality of life. Prior to this position, Kyle was a teacher at Milwaukee College Prep’s 38th street campus. In both roles, Kyle has shown her dedication to serving children who are placed at-risk for school failure and ultimate mission to achieve equal access to quality education for all.

School Intervention Series: A Complete Resource Guide

PCHA welcomes back Kyle Herma, School Intervention Specialist at Children’s Hospital of Wisconsin School for part 2 of her 3 part series about School Intervention. If you missed her first post, you can find it here. Today, Kyle shares a wealth of information for navigating a school’s system without a dedicated school liaison. Her complete resource list is included at the end of this post for easy reference. 

Going back to school after a diagnosis or hospitalization can be scary for everyone! Families might worry about how their child will transition, schools might worry about how they will care for the child, and children might worry about what to say and how to act around friends they haven’t seen in a while (just to name a few examples). Each and every one of these concerns is valid and understandable. So, how do we swing that pendulum of emotion from feeling anxious to feeling prepared? Continue reading

School Intervention Series: Making a Difference

Continuing our September Back-to-School theme, we are excited to introduce guest writer Kyle Herma, School Intervention Specialist at Children’s Hospital of Wisconsin. Kyle is blogging a 3-part series for PCHA beginning with an introduction on what she does and why. Over the next couple weeks, Kyle will share tips, tricks and recommendations for navigating a school’s system without a dedicated school liaison (and how to start advocating for one in your cardiac center) and provide a collection of resources on how to ensure your child is getting all of the services and supports required to have equal access to a quality education.

Twenty nine years ago my sister was born with Hypoplastic Left Heart Syndrome (HLHS), a congenital heart defect that left a chamber of her heart severely underdeveloped at birth. This was at a time when medical technology and surgical repairs options were very limited for a complex baby like my sister. While she ended up losing her battle with HLHS, her short time on Earth ultimately began a battle much greater – the fight to eradicate congenital heart defects completely and in the process, improve medical outcomes and quality of life for those currently affected. Today, the Herma Heart Center (HHC) at Children’s Hospital of Wisconsin is known for having the best published survival rates of HLHS in the world. However, a top recognition like this is not achieved without constant work towards excellence and innovation in all areas of care.

Herma Heart Center

How does this relate to PCHA’s “Back-to-School” theme this month? It does on so many levels! Two years ago I was a kindergarten teacher, loving every minute of every day guiding 4 and 5-year-old kids as they discovered their world. I worked in the inner city of Milwaukee at a low-income Charter School – all of my students considered “at-risk” due to a variety of different statistics. Every day I sought to plan lessons that not only were rich in academic content and student engagement, but also focused on building a classroom culture of strong future leaders and community advocates. While I had always been involved with the Herma Heart Center on various levels because of my family’s deep connection, I was a teacher. I loved being a teacher.

In 2015, I got a call. The Cardiac Neurodevelopmental Follow-Up Program, one of the HHC’s leading whole-child focused programs, was expanding and looking to hire a School Intervention Specialist after they noticed a very high need for multifaceted school intervention in students with complex health needs – specifically in the area of pediatric cardiac neurodevelopment. The job requirements outlined a liaison-type service, with the goal of working to ensure clear and consistent communication between the medical staff, the family, and the child’s school at all times. I began researching far and wide. I wanted to learn everything I could about how CHD affects a child’s neurodevelopmental functioning and what type of supports schools have in place to modify for or accommodate these children. All of my searches came up empty! There was nothing. While significant literary research supported that children with complex health needs and chronic illness are at a greater risk of reduced student engagement, higher disruptive behavior, lower academic achievement, an increased exposure to bullying (among many other well-documented negative educational outcomes), structured programs for school re-entry and intervention are rare. In this moment I knew that I needed to step in to fill a role much larger than a classroom teacher.

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The School Intervention Program officially “went live” in February 2015. I began with a very focused population of heart transplant patients that pinpointed those re-integrating into school post-transplant, but also offered intervention services to all of our heart transplant patients no matter how many years post-transplant they were. The response was huge. My patient population quickly grew to all heart transplant patients (including those wait-listed for transplant) and several patients with advanced heart failure who were anticipating a future transplant. My pilot study served 55 cardiac patients, ages 3 (preschool) to 24 (college). The schools’ concerns that were addressed included: attendance and absence support, special education support, attention and behavior plans, and documentation/medical record communication – just to name a few of the big categories. Of those 55 patients, 57% have exited the program with their school concern fully resolved, 36% still receive ongoing school intervention but are making great progress towards their school goals, and 7% transitioned to different medical centers where school intervention could no longer be followed. I’ve witnessed one of my high school student graduate with his class just 5 months post-transplant, I’ve heard from a school nurse that she could not believe a student is finally getting to live a “normal” life without any medical interventions needed during the school day, and I’ve helped a mom send her 7 year old to school for the first time because she finally felt confident the school could handle his needs.

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You may be thinking, “That’s great, but my child did not have a transplant. This doesn’t help us.” I am hear to tell you that it does! By doing a formal pilot study on a small population of patients and proving there is an urgent need for formalized school support, I am establishing both attractive outcomes data and the sustainability of this type of position. I frequently share these outcomes with colleagues, speak about my work at a director and leadership level, and even present at international conferences just so people can see how this seemingly non-medical work is directly related to improved medical outcomes. And guess what? People ARE listening.

Herma_Kyle

I am excited to be sharing with you over the next couple weeks to help bridge the very different worlds of medicine and education to ensure that children with complex health needs, specifically CHD, are not falling though the gaps. Here’s to a great school year!


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Kyle Herma is the School Intervention Specialist serving the Herma Heart Center at Children’s Hospital of Wisconsin. Kyle has been at Children’s since February 2015 conducting a formal pilot study on school intervention and the impact it has on a child’s overall medical outcome and quality of life. Prior to this position, Kyle was a teacher at Milwaukee College Prep’s 38th street campus. In both roles, Kyle has shown her dedication to serving children who are placed at-risk for school failure and ultimate mission to achieve equal access to quality education for all.

Back-to-School Q&A Panel

This September, PCHA is celebrating the start of the 2016-2017 school year with our Back-to-School series. For our Q&A Panel this month, we interviewed three people to gain a greater perspective on how CHD affects kids differently during this exciting time of year.

Congratulations on the start of a new school year! Please introduce yourself. What grade will you or your child be entering?

Frances: My name is Frances and I volunteer as the blog coordinator for PCHA. My husband and I live in California and have a confidant and outgoing 3-year old daughter who was born with severe mitral valve prolapse and a VSD. She had a very successful open heart surgery at 8 months old. She’ll be starting a couple mornings of preschool this year.

Margaret: Hi! My name is Margaret and I’m a heart mom to an awesome 8-year old heart hero named Kieran who will be starting 2nd grade. We’ve been through Birth to 3, as well as the IEP and 504 Plan process. I am also a parent adviser to our local hospital’s School Intervention Program.

Jack: Hello, my name is Jack Radandt and I was born April 15th, 2001, with Hypoplastic Left Heart Syndrome (HLHS). I had three open heart surgeries by the age of three, Norwood, Glenn, and Fontan. I lived a pretty normal life after my Fontan surgery. I was able to attend school, and even keep up with the other kids my age. At age eleven I experienced heart failure. I went to Children’s Hospital of Wisconsin in Milwaukee and was placed on the transplant list in October of 2012. I then needed to be on a device to bridge me to transplant. In December of 2012, I had surgery for a device called Heartware Ventricular Assist Device or (HVAD). I was the first single ventricle child in the United States to have the device and second in the world. I was also the first single ventricle child in the world to go home on this device. I had the HVAD for five months, until I received a heart transplant on May 20th, 2013.

What are you or is your child looking forward to most this school year?

Jack: I am looking forward to all the speaking events that I was able to get this school year.

Margaret: Kieran says, “gym!”

Frances: Making new friends. She has the ability to make a friend wherever we go, even when we run errands!

Do you notify your or your child’s school or teacher about your or your child’s heart condition? If so, how do you go about doing so?

Margaret: We definitely do. I’ve learned that teachers and school staff really appreciate being informed. Not every CHD student will need an IEP or a 504 plan, but we have both. Each teacher gets documentation about his HLHS, health, and classroom needs. Each year, I’ve met with school staff before school starts to make sure we’re all on the same page and to answer any questions. He has an excellent team at school that is communicative and proactive. We don’t just think about the regular classroom teacher — it is important to have a plan in place so that the school nurse, office staff, lunch and recess supervisors, gym teacher, substitute teachers, and any other school professional who might work with Kieran be informed of his health plan.

Jack: I do notify my school’s faculty, staff, and students of my condition, and I am very open about my scar and surgeries.

Frances: When filling out her general medical information for the preschool, we noted her cardiologist in addition to her pediatrician. She also has a medical device identification card for her annuloplasty ring in case any emergency arises affecting her heart. While our daughter has zero restrictions and no known issues otherwise, we included a copy of this card for the preschool and let the director know about it as a precaution.

Do you or your child have any limitations or require medication during school? If so, how do you handle this?

Margaret: Kieran has HLHS, and his cardiologist has requested that he stay indoors when it is below freezing. On these days, he gets to pick a classmate to stay inside and play board games with. He also has an adaptive PhyEd teacher working with him in gym class. He has a water bottle with him all day to prevent dehydration. For fire drills, he has instructions in his health plan to be allowed to wear a coat outside if it’s cold out. There are certain things we take on a case-by-case basis, such as field trips and walking trips.

Frances: Our daughter doesn’t have any limitations or medications, though the future is uncertain. Her heart will need to be monitored more closely during puberty as her device may be affected during this exponential growth period.

Jack: I don’t have to deal with this because I take my medication right before I go to school and right before I go to bed.

What reaction do you get from the staff if you notify them? Do you feel this affects how the teacher and/or staff interacts with you or your child?

Frances: They thanked me for sharing the information. They haven’t mentioned it otherwise, and from what I’ve seen do not give her any special treatment.

Margaret: We’re fortunate to have a neighborhood school that fosters a very positive learning environment for everyone, and is innovative about classroom adaptations. When we’ve notified them, they’ve responded very positively with a can-do attitude. Last year at our back-to-school meeting, not only did Kieran’s new teacher attended, but the office staff, principal, school nurse, and almost everyone involved with his IEP. Shedding light on the HLHS and the secondary challenges we’ve faced helps them understand how to meet Kieran’s needs better, as well as helps them understand why he (and we as parents) sometimes act the way we do.

Jack: Some of the faculty and staff that find out about my condition feel very uncomfortable about the whole situation.

How open are you or is your child about CHD with peers at school? How does this affect your or your child’s relationships?

Jack: I am very open about my condition. This is my life and I’m not ashamed of it at all.

Frances: Since she’s still very young, our daughter doesn’t make a point to either hide or reveal it. While I want her to be proud of her scar, I also want to allow her to talk about CHD on her own terms whether that’s mentioning it to close friends or being a vocal advocate. She is a naturally confidant and extroverted individual, so it doesn’t seem to bother her when someone points it out or asks. For now we focus on making sure she knows her scar is something good and how to respond in situations. I don’t make any consideration with clothing when it comes to her scar, choosing her outfits based on the weather and her own personal preferences.

Margaret: Kieran is very sensitive about his heart condition. We don’t actively keep it a secret, but we don’t actively volunteer information about it to his peers, either. He feels very strongly that he is a “normal” child and wants to be seen that way. Everyone is different, and I know many heart parents who believe it’s important for their child to be CHD advocates, but I feel it’s important for now for him to feel comfortable at school and have it be a “safe space” for him to feel normal. He sometimes does participate in CHD activities with me outside of school, but he doesn’t understand why they’re important. He sees his “heart friends” as regular friends. Many heart kids don’t truly know the gravity of some of their heart defects until they are much older. A cause that affects him much more, and has for years, is hunger and his desire to see everyone in the world have enough food to eat. I think that’s wonderful. We should all be able to focus on areas of need that spark our sense of fairness.

What is your or your child’s favorite subject or activity?

Frances: She loves the arts – dancing, painting and music.

Jack: My favorite subject in school is biology.

Margaret: Definitely gym. He also likes math and music.

What, if any, concerns do you or your child have in regards to CHD for the school year?

Margaret: My biggest concern is that somewhere, at some point, there might be a breakdown in coordination at school. I worry most when there is a substitute teacher in the classroom, because I’m not informed of it, and I have no idea if they’ve read his 504 plan and understand it. Luckily, he comes into contact with many staff throughout the school day, and I think they all do a good job keeping an eye on him and all the students. Sometimes, because he wants to be seen as “normal” in front of his peers, he’s not as assertive as he should be. I also worry about rough play during recess. This has been a problem at times throughout the past school years, where he will be tackled or otherwise roughed up during normal play, which has resulted in some bruising because he is on blood thinners.

Frances: You would have no idea our daughter has CHD besides her scar, so my only concern is her peers setting her apart in a negative way because of it. Her preschool focuses very much on emotional competence and socialization in a play based setting, and what we liked most about it when touring was how respectful every child was to each other regardless of their differences.

Jack: I am in a very small school so I really have no concerns besides being ill.

If you or your child has a high sensitivity to illness due to CHD, how do you or your child combat this at school?

Frances: While she doesn’t have a high sensitivity, her pediatrician still errs on the side of caution by making sure she gets the first flu vaccination that comes in for the season which we are thankful for. We also chose a preschool with a smaller class size and a strong emphasis on cleanliness.

Jack: I am sick a lot so missing school is always a big concern.

Margaret: The school nurse, or sometimes the classroom teacher, is really great about informing us about illness at school. They will email us personally if a lot of kids are out sick, or with certain communicable illnesses requiring all parents at school to be notified, the school nurse sends home flyers. We are most concerned about things like strep and seasonal flu. If it’s an outbreak the classroom, we would most likely keep him home until it had passed. We use hand sanitizer, get a flu shot, and try to get enough sleep and eat healthy. We emphasize to Kieran the importance of good hygiene. His school has a great custodian and they are good about keeping the classroom and school wiped down.

What is your biggest hope for yourself or your child this school year?

Jack: To remain on honor role and avoid illnesses.

Margaret: That he will make more friends and feel more included socially. Not only is he an only child, but like many CHD kids, especially those with critical heart defects, he is a bit behind for his age socially. Add to that the fact that he easily tires during playground games and has to take breaks during physical activities, he sometimes feels frustrated that he can’t keep up with other kids, especially most of the boys. Of course, I also hope he has a great learning experience this year and finds areas of learning he really loves.

Frances: I hope she will be able to make new friendships and start a solid foundation of a love for learning!

What area(s) is your child most successful at school?

Frances: Since day one, my husband and I have never experienced separation issues with her. She’s very adaptable, confidant and according to her preschool teacher, not at all afraid to ask questions.

Margaret: He a wonderful singer, and is also very creative when it comes to visual art. Last year, he did after school Spanish and book club, which was good for him. He is successful at reading, although he pretends to think it’s “boring.”

Jack: Science class over any other classes.

Thank you all and best wishes for the new school year! Be sure to check back throughout September for our guest writers covering relatable Back-To-School topics and be on the lookout for your children being featured in a blog post. Share your first day of school photos at advocacy@conqueringchd.org by Friday Sept. 9th to be included!

Songs of Love for the Heart

“When words fail, music speaks.”

Once said by Hans Christian Andersen, best remembered for his fairy tales like The Little Mermaid and The Snow Queen that inspired Frozen, this renown Danish writer understood the importance of music.

With any serious child illness, there are times when words fail us. PCHA was asked to try out a product that steps in for your child in these moments. It’s called Songs of Love, a nonprofit foundation dedicated to providing personalized uplifting songs, free of charge, for children and teens currently facing tough medical, physical or emotional challenges. At this early stage in our 3 year old daughter’s journey with CHD, we want to instill the fact that her scar from open heart surgery is something to be celebrated. It is a beautiful reminder that she is with us, happy and healthy. So we requested a song for her.

The process was very simple. We went online and filled out the form. We waited five weeks and received an email that it was complete and on it’s way in the mail (and to note, I appreciated that they realize to a young child, physically receiving this gift gives it greater anticipation and awe versus opening an email, though you can request a digital file be sent). And then we waited.

As my husband and I are both lifelong musicians, we weren’t expecting the next masterpiece or top 40 song, but we also weren’t expecting her reaction when we played her personalized song. When filling out the form, I had put in more than enough special interests, hobbies and favorite things to give the musician something to work with, and I also was very specific in that she loves a heavy beat she can dance to, pop or rock, noting that she really loved the song Immortals (which we discovered while watching Disney’s Big Hero 6). So when we popped the CD in and it began to play, we just had to smile at the instant burst of excitement and energy as she twirled, danced and bopped around for her entire 3 minute song. And then we knew we would be singing it for weeks, when she told us to replay it three more times in a row.

Words sometimes fail us, especially in trying to explain to young children about a complex condition, and in our case, how to embrace a lifelong journey with CHD full of the unknowns that await. Songs of Love is a fun resource that PCHA members find fitting for younger children. While we cannot fully explain to our daughter just how miraculous her repaired heart is, and how her scar is a beautiful reminder of that, Songs of Love helps her understand this in a small way as someone wrote a special song, just for her, in celebration of her heart.

To request a free, personalized song and for more information, visit Songs of Love.


Frances volunteers as the blog coordinator for PCHA. She graduated from USC and previously worked in film, marketing, and software consulting. Frances resides in California with her husband and daughter – who was born with severe mitral valve prolapse and a VSD and underwent open heart surgery at 8 months old.

Transparency Blog Series Recap

For the month of July, PCHA recognized the importance of transparency. Our goal is to inform, educate, and empower patients and families to actively participate in data-driven, shared-decision making. The fight to conquer CHD is strengthened by the involvement of all of those who share our mission. It takes a community coming together around education, research and awareness to make an impact. Volunteer, join our advocacy network today, and connect with us on Facebook! In case you missed a post, below is the full Transparency Blog series:

Passion and Progress: A Provider’s Perspective

A Life Raft: A Parent’s Experience using the Guided Questions Tool

A 2nd Opinion: Finding the Right Care

A Lesson in Learning To Be a Better Patient

The Importance of Information to Support Shared-Decision Making

The Importance of Information to Support Shared-Decision Making

The Pediatric Congenital Heart Association firmly believes that patient-engaged care leads to improved patient experience and outcomes. In this tragic story, one family believes that they did not have the information needed to properly understand the expectations for their child. We encourage patients and providers to work closely together to find a balance between hope and expectations to maximize the experience. While recommendations that follow are the opinion of this family, we do believe they should be included as part of a broader discussion.

Our daughter Sadie was diagnosed with Tetralogy of Fallot (TOF) in July of 2014 shortly after our 20 week ultrasound. We were not only shocked and terrified, but we also could not find anyone we knew to talk about this with. Congenital heart defects (CHDs) were a mystery to our friends and family. Even our OB had limited information regarding Sadie’s diagnosis. We immediately began researching to find out as much as we could about Sadie’s condition, including potential surgical options, outcomes, potential complications and information regarding quality of life. We quickly learned what most parents with children with CHDs already knew – that this information is not only widely unavailable, but extremely difficult to understand.

My husband and I considered our family fortunate to live close to a reputable children’s hospital with a high-ranking pediatric heart center. We also felt lucky that Sadie’s defect was diagnosed early – it would give our doctors several months to prepare us to care for a child with a CHD. We assumed our doctors would empower us with all the information we needed to become an integral part of Sadie’s care team. We now feel those assumptions were incorrect and that we were ill-prepared to effectively care for our daughter.

Before Sadie was born we had multiple appointments with Sadie’s care team, including multiple cardiologists and support providers. Every doctor we met with was extremely optimistic regarding prognosis, surgical outcome and quality of life. The two-stage repair was explained as well as long-term outcomes that included decreasing frequency of follow-up appointments with the expectation of a third surgery in her teen years. Over the course of these appointments, our doctors focused on making sure we understood the anatomy and physiology of Sadie’s defect, how she would be cared for in the hospital after she was born, and the specifics of her surgery.  Although we were upfront about wanting as much information as possible so that we could be prepared for every scenario; there was little discussion regarding risks, complications or adverse outcomes. We were told with great confidence that Sadie would have an excellent quality of life, equal to or at least extremely close to that of a non-CHD child.

However, every step of the way there were unexpected findings. A week before delivery Sadie was diagnosed with pulmonary atresia (a more severe form of TOF). Only after her death were we told that this significantly increased her mortality rate. Then, the evening before her surgery, we were presented with a new surgical strategy that we were hearing about for the first time.

We felt we could not effectively take part in the decision making process regarding our daughter’s care when we were being presented with new information on an as needed basis. Putting us at an even bigger disadvantage was the fact that we were trying to understand and process this new information at an extremely emotionally charged time.

Shortly after her discharge home, we took Sadie to the ER. She was experiencing several complications from her condition and was admitted back into the cardiac care unit. Most of these complications had not been previously discussed with us as possible outcomes yet were quickly dismissed with phrases like “these things sometimes happen” and “this isn’t abnormal to see in a child with her condition.”  These responses made us increasingly anxious, fearful and confused. As the disconnect between what we had been prepared for and what was happening grew during this second hospital stay, we increasingly felt that our doctors hadn’t been truthful with us regarding Sadie’s condition, and, in turn, had not realistically prepared us to care for our daughter. We began to question our doctors, Sadie’s future, and our own ability to care for our daughter. Less than two days after she was discharged, Sadie suddenly stopped breathing at home and died at the nearby hospital.

We learned a lot during our 23 days with Sadie. We will never dispute that our doctors only wanted the best possible outcome for our daughter. We are grateful that people with their knowledge and skill exist to give children like Sadie a chance at the best possible life. But there are many areas where our doctors and medical team could have done a better job to ensure Sadie got the best possible care.

We depended on our doctors to prepare us for ALL realistic outcomes. Overstating the positives and understating the potential risks and complications robbed us of our ability to make informed decisions regarding our daughter’s care. Perhaps our doctors were trying to protect us by omitting scary and complicated outcomes or to shield an expectant mother of a sick child from what they perceive as unnecessary stress. I don’t know. What I do know is that the lack of information made us feel like helpless bystanders instead of informed parents who were part of a team of people making decisions together in the best interest of a sick child.

Our lives will never be the same after experiencing Sadie’s life and death. We can never go back to re-advocate for our daughter and we will never be able to change the outcome. We have spent a lot of time replaying the events of Sadie’s life and death. In doing so, we have been able to identify several things that we wish our doctors had considered, as we would have been better prepared to care and advocate for our daughter.

Timing of information sharing. Parents deserve as much information as possible as soon as possible for several reasons.

  • Start building trust between doctors and parents through honesty and transparency.
  • Providing comprehensive information early and often allows families to absorb different information over time as stress levels go up and down.
  • Parents need the full spectrum of information to be able to plan and prepare to care for a child with a CHD.

What information to share. It would be helpful for medical teams to come up with standard information for each diagnosis to share with parents. Such as:

  • Hospital outcome data that includes number of patients, surgical cases, and both surgical and long-term mortality rates.
  • Benefits, risks and recovery (in and out of the hospital) for each planned surgery, as well as unplanned but possible alternate surgeries.
  • Additional treatment approaches necessary including common medications and their side effects.
  • Potential complications that children with this defect may experience.
  • Possible associated disorders.
  • Long-term outcomes and challenges children with this defect may face such as physical or neurological challenges.
  • The full spectrum of what caring for a child with this defect looks like, not only during the hospital stay, but at home and on a long-term basis.

How to effectively communicate information. Given the complexity of the information, how it is communicated is just as important as when and what. The more resources and open lines of communication that parents have, the more supported and connected they will feel.

  • Find the balance between benefit and risk, between optimism that all will go according to plan and concern regarding complications that may arise.
  • Use multiple modalities – verbal, video, images and writing. A written reference can be very helpful and could eliminate miscommunication and provide clarification for parents.
  • If possible, it would be helpful for medical teams to assign one point of contact to make sure all the agreed-upon standard information has been communicated to parents, especially when one specific doctor is not assigned. At the very least, doctors should let parents know who to follow up with and how to reach them should they have questions or concerns at any time.

We wanted, expected and deserved to be presented with all the information needed to be able to advocate for our child. Many complications and outcomes may be out of the doctor’s control, but providing parents with ALL the relevant information at the earliest possible time is well within it.


Erin Schuster is the mother of three little ones, two sons and one daughter. Her daughter, Sadie, was born with multiple congenital heart defects and died from complications from her defects at 23 days old. Since Sadie’s death, Erin has been working to raise CHD awareness and funding for CHD research. She is also interested in raising awareness and educating others regarding infant loss and the life-long grief journey experienced by bereaved parents. Erin strongly believes in the Pediatric Congenital Heart Association’s goal to empower CHD parents with the information necessary to become effective advocates for their children. For more about Sadie’s story, please visit www.rememberingsadie.org

A Lesson in Learning To Be a Better Patient

As CHD is a lifelong condition, an important aspect of transparency is knowledge of a complete medical history. PCHA welcomes Meghan Chell, a CHD survivor, who speaks about her recent challenges in finding the right care as an adult CHD patient. 

If you don’t learn your medical history, you’re doomed to repeat it.

Three years ago, my life as a mostly-healthy person fell apart around me. Sure, I was born with coarctation of the aorta and had three surgeries as a child, but for over 20 years I’d been incident free.  I rode my bike, did yoga, hiked, swam, basically did whatever I wanted, and every year grew more distant from my congenital condition. I went to my annual visits, and every now and then had to explain the source of my sternotomy scar, but acted as though my struggles with CHD were behind me.

Until I was suddenly reminded that they weren’t.

I was 33 years old, recently engaged, and working as a wastewater and remediation engineer in Portland. A typical fair-weather cyclist, I started biking to work again once the “frozen mix” of winter turned into the random rains of spring. I was having trouble with uphill stretches of my commute but simply wrote it off as loss of stamina due to winter laziness. When I had an offer of interesting but strenuous field work at a remote mining site, I scheduled my annual visit to get my cardiologist’s seal of approval before committing.

It started off as a typical visit: an echocardiogram to be followed by an appointment with my cardiologist. He’d overseen my care since I was in recovery from my last coarctation repair in 1992. We had discussed transferring my care to another doctor, but decided that it was best to stay with the doctor who was most familiar with my case. He was in California where my family had moved right after my heart surgery, so I used the travel as a chance to see friends and family in the area.

I knew something was wrong before the echo was halfway through. The technician was warm and gregarious, so the two of us typically gab throughout the test. But at some point he became quiet and focused. I waited for an hour while my doctor was reportedly reviewing my files before I finally learned what happened. My 21-year old coarctation repair, an ascending to descending aortic conduit, had clotted and was no longer providing a bypass for blood flow to my lower body. This explained my fatigue and headaches with exercise – my lower body had insufficient blood flow, while my upper body was hypertensive. He proposed a quick and easy solution – a stent to widen the narrow portion of my aorta, which could be performed with a catheter, and I could be back to work within a week. First more tests to confirm – an MRI, a fit test, and blood work. Then we’d schedule for the procedure in Southern California.

Fast-forward three months. I’ve had all my tests and dealt with the fragmented, back and forth communication with my doctor through his receptionist. Once the diagnosis is finally confirmed, I travel to Los Angeles for the cath procedure. While in recovery and barely conscious, the surgeon then tells me that they didn’t place the stent. Why? Apparently there was no need! My aorta was only slightly narrowed and shouldn’t cause a restriction. So the aorta wasn’t the problem! I protest as much as I can – the tests, the conduit being clotted, my symptoms. He shows me a grainy photo from the cath procedure and points to measurements that I can’t see through the fog of anesthesia. He says we’ll discuss this further when I’m more awake, but he doesn’t reappear that day. Eight hours later I’m driven to my father’s house, where I promptly have a good cry and fall asleep.

When I got home to Portland, I put my recovery time and mounting frustrating to good use, investigating local cardiologists for more engaged care. I found a clinic at Oregon Health and Science University, a mere 3 miles away, which had multiple adult congenital specialists. I also requested records from my California cardiologist to familiarize myself with the details of my 30-year medical history, many of which I only barely recollect. When they arrived, I was shocked to find that there were only records through 2008. I realized that when my doctor was supposedly reviewing my files, he was in actuality probably trying to locate the other 16 years of them! I also learned that my coarctation is mainly due to a hypoplastic, inflexible portion aorta, presumably due to scarring from my original repairs in 1984. Though it looks fairly normal at rest, it doesn’t expand as it should during exercise. Had my doctor known my medical history, he would have known that a stent was not a viable option and never would have given me the false hope of a quick and easy fix.

In retrospect, though I do hold my doctor accountable for being unfamiliar with my history when he recommended this unsuccessful procedure, I realized I needed to be a better patient. I had lived in a dream world for over two decades, and hadn’t kept up to date with my care or what was best for my condition. Had I been seeing an adult specialist, they would have been monitoring for this failure, and could have prolonged the usefulness of my conduit. It’s a heavy weight to bear, but we survivors and family members of congenital heart disease need to remember that we have to be the most knowledgeable about our specific conditions and cases. As such, we need to always be active members in our treatment which sometimes means questioning an easy solution when we know it isn’t the right one.


Meghann ChellMeghann is a congenital heart disease survivor, born with coarctation of the aorta and a bicuspid aortic valve. She lives in Portland, Oregon and works as an environmental engineer. Her focus in CHD activism includes patient engagement, transparency, and medical records availability.