Field Manual: The Heartland Amandafesto

A Heartland Amandafesto

It is important that all members and supporters of the Heartland know the top issues affecting us today. I have outlined them below with remediation tactics you can do from your own computers and mailboxes.

Problem One: Issues with Continuous Care.

  1. Across the nation, teens and young adults with CHD are dropping out of care.
  2. When they finally get sick enough to seek out care, they are often misdirected to adult onset cardiologists with no expertise in congenital heart disease.
  3. There are not enough adult congenital heart specialists practicing and they’re often isolated to the largest population centers, making them difficult for patients to see.
  4. Many teens and adults with CHD are lacking medical coverage and have trouble navigating the system to get adequate care in a timely manner.
  5. Many medical records, especially for adults with CHD have been lost over the years.
  6. There is no national tracking of these records or an active or comprehensive national registry for children who have had open-heart surgery.
  7. Often when a patient seeks acute care several years after surgery, specialists are starting from square one just getting a sense of their anatomy and how to treat them.

Reasons:  Many patients/parents either were expressly told or bought into the idea that they were “cured” by surgery and are uneducated about long term complications and the need for follow up care, or they are in denial about the same issues. Before the 1980s we never saw large numbers of children surviving into adulthood with CHD, and for the first time in history there are more adults than children with CHD and that number is growing each year.   Solutions:  Parents need to have the expectation that, even after successful surgery, a heart defect is a chronic and life-long condition that needs routine follow-up care to prevent critical complications later in life. Society needs to recognize how prevalent CHD is and support electronic medical records and best practices to keep and share information across time and geographies in the interest of the patient and the patient’s quality of life. Medical schools need to establish more Adult CHD career paths for physicians to increase the number of practitioners to help the growing number of adults with CHD. Patients and parents need to be more effective advocates and partners to bring these needs to light in the public and take responsibility for them at home.

Problem Two:  Research and information is lacking

  1. Much of what is available about CHDs and available to parents is dated and inadequate to enable intelligent questions, facilitate meaningful dialogs, or solicit informed consent.
  2. CHD is the single most common defect, kills more babies globally than any other disease, and yet it is underfunded compared to other ailments. Specifically, according the Children’s Heart Foundation:In the private sector, (the American Heart Association) only 1% of every dollar received at the major private funder of cardiovascular research goes to any Pediatric cardiac research.Likewise in 2007, the National Institutes of Health’s NHLBI (National Heart, Lung and Blood Institute) allocated less than 3% of every dollar invested in research to any Pediatric cardiovascular research.
  3. The research done has been very successful but hasn’t gone far enough. It’s truly a mixed blessing because without the progress made in the twentieth century so many children would not be alive as adults today. Yet, now those lives are compromised. Tens of thousands who would have died ten or twenty years ago are now saved every year, but what does their future hold? Imagine how the absence of an answer to that question rocks the world of an adolescent who is envisioning his future. Yes, as a mother I’m grateful you saved Liam’s life, but doctors, researchers, society, and myself included, owe Liam and the millions like him a more stable future with more information.*Transplant is not an option. There are simply not enough hearts for transplant need with all the kids who are currently living with half a heart or other severe forms of CHD. Transplant is only another form of palliation, and for kids with hypoplastic hearts, their bodies don’t want a full heart at age twenty-five because their bodies are used to a different system. They want to keep working with a half heart, as compromised as that is. We need artificial hearts for the newborns and artificial half-hearts or assist devices for the half-hearted who are here before the artificial heart arrives. We need stem cell research to prevent organ rejection today and to prevent artificial heart rejection in the future. There are a million good ideas and possibilities out there, and we need to make them happen because what’s here today won’t save Liam or the thousands upon thousands like him tomorrow. This is urgent. This is not negotiable.

Reasons:  Every disease needs money. That’s just the way it is. I believe that there are four specific elements that have limited funding for CHD (as compared to diseases like autism or breast cancer) and they are lack of awareness in the public, lack of knowledge in the general medical community and nonprofit sector, excessive denial on the part of parents of and patients with CHD, and missed opportunities on the part of CHD researchers.      

Solutions:

  1. Awareness: The public has no idea that CHD is as common as autism or that at least half of children diagnosed with CHD will have at least one open-heart surgery to survive it. Yet, when you talk to individuals they can always come up with at least one person they know who has congenital heart disease, but they know it as a hole or some other descriptor. We need to re-brand the collection of defects to improve awareness.
  2. Knowledge: When I’ve spoken with employees of the American Heart Association, they often do not know that Congenital Heart Disease is different from the adult onset cardiovascular disease they routinely encounter in their volunteers and constituents. If they don’t understand, we’re already in trouble. Likewise I’ve encountered OB-GYNs and pediatricians in my own experiences who don’t know a quarter of what I know about CHD, yet it is the most common birth defect. More shockingly, many mothers are urged and even goaded into terminating babies like Liam by general practitioners or obstetricians who attended medical school fifteen or twenty years ago and have learned nothing about CHD since. The world has changed, and other medical professionals need to get with the program so that CHD research becomes the priority it needs to be.
  3. Denial: Denial is so dangerous. Aside from the acute danger on an individual level, this endemic denial that anything is wrong or that it’s someone else’s problem has an enormous impact on mobilizing public support for CHD research. Historically, some cardiologists have been collaborators in this lie, telling parents their kids were “fixed,” and free from follow-up care or failing to provide sufficient detail about the child’s surgeries for future reference. Maybe they believed that the kids were fixed and wouldn’t need long term care, but that hasn’t borne out across the one million adults living with CHD. It’s a bit too easy a few years out from major surgery to want to buy into this idea that it’s in the past, but CHD will come back in some form for many patients. Maybe a fraction of the families will be the lucky ones with no complications down the line, but that’s big gamble to take with the futures of so many on the promise of a fantasy. The Heartland needs to wake up and get out there, especially those families who aren’t in the hospitals, who have gotten through the really hard times. We owe it to the ones who are struggling, the ones who haven’t made it and our own kids’ whose future’s we’ve bought on credit, hoping the research will be there. It’s our responsibility to make sure it gets delivered.
  4. Doctors, doctors, doctors: PhDs and MDs alike. Guess what? You have an untapped goldmine of interested supporters who are itching to help you get your research funded. Look at the autism and breast cancer movements – we’re just as passionate as them. It’s time you harness our horsepower. What we need is leadership and direction. Our cause is true, let’s work together.

Problem Three: Bad or no screening at all. When it comes to CHD kids, the following areas have been historically lacking:

  1. Insufficient screening for infants and children with CHD at birth.  Recently the Department Health and Human Services finally advised pulse ox screenings for all newborns. Ideally, this will prevent thousands of CHD deaths in the coming years because low oxygen is a huge indicator of congenital heart defects. A low score prompts follow up screening not just for acute cases like Liam’s but for kids whose defects might not cause damage for years, but by the time they’re detected might be irreversible. This is a good thing, but not every state has agreed to do so. Maybe in the coming years this problem will be solved.
  2. Insufficient screening for CHD before birth. I’ve heard time and time again from mothers who had a twenty-week ultrasound and their children’s hypoplastic ventricle was still missed. This is partially because with certain version of this type of defect it gets proportionally smaller as the pregnancy progresses, but in some cases it’s just inadequate screening. I’m more concerned with the women, who like me, are denied a mid-pregnancy ultrasound because of an initial screening earlier in the pregnancy. It should not be an either/or thing; every pregnant woman should be given a full screening of her fetus’ vital organs prior to birth. To withhold this screening is to increase the risk of death for kids who are missed.
  3. Inadequate sports physicals and pediatric screening. Every time I hear about a kid who dies on an athletic field or roller coaster, I think CHD, and I’ve yet to be proven wrong. Autopsies bear it out time and again. Often this is cardiomyopathy or some other birth defect with conduction that causes irregular beat. Sometimes it enlargement of the heart from a structural defect that then causes conduction issues, but I’ve yet to follow a news story like this and find some other cause for premature death.
  4. SStrong-arming parents into late term terminations.  No parent should be made to feel guilty for not having a termination, especially by someone who doesn’t know anything about the status and possibilities of modern pediatric cardiology.   This is wrong, and while I respect all parents’ rights to choose what is best for them and their families, they should never make such a huge decision without all of the most current and relevant information.

Reasons:  Money. It costs money to screen kids for oxygen levels (about $10 whole dollars) and screen fetus (about $500, thought that’s typically covered it’s the repeat that’s the problem) and EKGs and training (about $100) for sports screenings at general practitioners and pediatricians. As a parent whose child has nearly died and whose friends include many who’ve lost children, this cost per screening seems low compared to the eight thousand infant lives lost each year to CHD or the routine news stories about that basketball player, track star, football hero, or gymnast who dropped dead after a game.   Solutions:  Screening and education. Simple screening. It may not catch every case, but we give mammograms, prostate exams, and colonoscopies as a matter of routine to our older citizens to preserve their golden years. Do our youngest citizens deserve less when they have so much more living ahead of them?  Education is as simple as requiring a couple of hours of pediatric cardiology training every few years for those doctors and midwives treating pregnant women and newborn babies as well as pediatricians and general practitioners giving children sports physicals.      

Problem Four: Poor Communication Across Disciplines

  1. What do pediatric gastroenterologists, neurologists, pulmnolists, nephrologists, orthopedists, and cardiologists have in common? Kids with CHD, and yet many parents of these kids with CHD   have to juggle these specialties and moderate communication across disciplines. That is not in the mommy job description in any parenting handbook I’ve read. How do we know when these defects and complications are related when they’re always treated in isolation?
  2. We have poor and limited resources and counseling options to deal with the challenges we face. A great deal can be leveraged from grief counseling, but we need more specific health care for both caregivers raising children with chronic life-threatening disease and adolescents and adults living with these birth defects long past childhood.
  3. We need better communications between cardiology and OB/GYNs, midwives, and general practitioners. What happened to me should never, ever happen again. Any midwife or any person treating pregnant women should know exactly who to contact for the best congenital heart care.
  4. We need better communication in cardiology departments for long-term care. They’ve got the crises covered, but we need to have more comparative statistics over the long term, not just at one institution, but across institutions for maximum information sharing.

Reasons:  Everyone is busy, and each discipline has plenty of its own kind to treat. Yet, we routinely see kids with CHD afflicted by multiple defects.   Solutions:  As a society we need to recognize that CHD is common and it’s not going away. We need to make this big disease a priority in training and communications across disciplines. It’s not fair for parents or adolescents or adults with CHD to be the go-betweens in such complex and difficult information exchanges. It’s not fair to save so many lives and leave them lost for support in establishing their quality of life.   All children’s hospitals treating CHD need to have outreach programs in place with to maintain contact with those who are delivering the babies whose lives they will save. Information should be readily at hand for referrals and assistance. No mother should ever have to find her own pediatric cardiologist the way I did. Almost every one of these problems could be solved with better communication at a relatively low price. It’s as much about doing more, it’s about doing it better.   Problem Five:  CHD REALLY is a disability – sorry folks it just is!

  1. Many parents don’t file an IEP or a 504 form for their kids when they start school. Then if there is a stroke, infection, long hospitalization or other issue their kids are not protected and families have to fight school districts after fighting for their kids’ lives.
  2. Until 2011, kids with single ventricles had to fight for social security coverage. This problem has thankfully been resolved, but sadly, there were a select few very healthy hypoplasts who resented that legislation because they didn’t want to be considered disabled.

Reasons:  If you spent thirty minutes with Liam without knowing about him, without reading this book, you would not believe me when I tell you he is disabled. But he is disabled. He only has half a heart and his respiratory system and liver function are compromised if not yet impaired. They are both at risk along with his heart for long term failure. He doesn’t have the stamina of other kids and he has orthopedic defects on top of that. If he can get an office job as an adult, then he’ll probably be fine to work 30-40 hours a week, maybe. Manual labor is highly unlikely for someone with Liam’s anatomy. Still, many parents of and patients with CHD would like to ignore this fact. It’s one thing to be in denial for yourself, but when you withhold or fight remediation for your less fortunate peers, that’s just narcissism.   Solutions:  Documentation is crucial. The disabilities of children with severe or moderate but unmonitored CHD escalate with age. Liam is getting a pacemaker sooner rather than later. If it happens during the school year, I have his 504 in place to protect him so he will get his homework and adequate time to recover from surgery and do his work. I’m also protected because Liam is usually covered with deep black bruises on any bony part of his body that might bump anything at all. This is from aspirin therapy, and that is noted in his 504 plan. The school and the school district is protected because Liam’s condition is a point of record and the institutions can plan accordingly. Doctors can help by providing details and helping with preformatted descriptions of the impacts of the disease and limitations for each patient.   The goal is not to limit Liam or kids like him, but to protect them from being limited or victimized because of their preexisting medical conditions. We don’t want to focus on what they can’t do, but we need to be realistic that there are limitations. We can’t wish away CHD and hiding from the disability leaves the disabled more open to being excluded from social protections put in place to protect everyone.   For more information about 504 plans and special needs trusts see my web site at http://www.amandaroseadams.com.

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