Frequently Asked Questions about Bodie's Heart

There are a number of questions I get asked frequently about Bodie and his heart condition. So I thought I'd ask a FAQ section to the blog, where I can put the questions with my answers. Hope it helps! And if you have other questions you'd like answered, please let me know!

What is Bodie's heart condition called? 
It's called Hypoplastic Left Heart Syndrome (HLHS), which is a class of heart defects where certain structures on the left side of the heart do not form or form incorrectly. In Bodie's case, none of the structures on the left side of his heart formed at all. The diagnosis is incompatible with life, but life expectancy can be prolonged with a series of staged open-heart surgeries, of which Bodie has had 3 (the Norwood, Glenn and the Fontan) as well two additional "bonus" surgeries (to receive a pacemaker and a shunt replacement between how Norwood and Fontan, and a pacemaker replacement 4 days after his Fontan).

Does Bodie have any other issues besides HLHS?
In addition to HLHS, Bodie has CPVT (Catecholaminergic polymorphic ventricular tachycardia) - how's that for a mouthful?  CPVT is an adrenaline induced heart arrhythmia. We say it's in addition to HLHS because CPVT very, very, very rarely accompanies HLHS (as in, there's a less than a 1 in a million shot, and we only believe it because we've proven it through genetic testing). He inherited CPVT from his father, and it has caused significant issues for them both, including cardiac arrest at the age of 41 for his father. It is exceptionally rare for one individual to have both things - we say Bodie won the "crappy lottery." :-)

When did you find out about Bodie's heart condition?
We found out something was wrong with his heart when I was 17 weeks pregnant (see here for the full story) and received our official diagnosis of HLHS 6 weeks later (see here).

Why didn't you just terminate the pregnancy when you found out about his heart defect?
To put it simply, termination wasn't an option for us. We are believers in Jesus Christ and believe passionately that it is God's authority, and his authority alone, to make the decision to begin and end a life. As parents, our calling is to love our children and to sanctify them at every opportunity, and that includes the time before birth.

Why put him through the surgeries? Why not just do a heart transplant at birth?
2 reasons: (1) availability and (2) the fact that transplants are not a cure-all. (1) Availability. Newborn hearts are hard to come by. The top causes of death in infants are Congenital Heart Defects and Sudden Infant Death Syndrome. If a child died from a CHD, the heart obviously wouldn't be suitable for transplant for another child. And children who pass from SIDS are often found too late for viable organ donation. So, many children who do have to go the transplant route end up passing away before a heart becomes available. (2) Not a cure-all. A transplant is really just trading one set of problems for another. You always have to worry about rejection - of course the first year is the scariest for that (since most rejection occurs in the first year), but it can happen at any time. Kids have to be on anti-rejection drugs for life. Antirejection drugs are scary, scary immunosuppressants that wreak havoc on the entire body, not just the part you're trying to get not to reject the heart. And did you know a heart transplant doesn't last forever? So, once you get that heart, you're on a ticking clock until it stops working and you have to go back on the list for a 2nd transplant, which are much riskier and harder to come by than the first time. So, while transplant gives your child the incredible opportunity to have a fully-functioning heart, it's kinda just trading one set of problems for another. BUT this is not to say that we wouldn't have welcomed the transplant route, if it had been the best course for Bodie up front. And we know it is highly likely we will have to go the transplant route at some point. I do have to say that we know HLHSers who have had transplants instead of the palliative surgeries (for a multitude of reasons) and are doing quite well now. But because the palliative surgeries appeared to be a viable option for Bodie, we opted for that route instead.

Do all kids with HLHS have pacemakers?
Nope - in fact, the majority who do have them do not get them until much later, years post-Fontan. The number of open-heart surgeries these kids must endure can create a lot of scar tissue on the heart muscle itself, which can lead to arrhythmias. That's the #1 reason these kids need pacemakers later down the road. Bodie's case was a little different. He needed his due to bradycardia (abnormally low resting heartrate) and tachycardia (episodes of very high heartrate), both caused by the CPVT he inherited from his father's side of the family.

Does Bodie live a "normal" life?
Absolutely. We cannot say this often enough or loudly enough. We were told when we received his diagnosis that he wouldn't have as much energy as his peers. We call that a "false promise" in our household, because we literally cannot keep up with this kid! He is in a perpetual state of motion. He goes to preschool 3 full days a week, where he participates in both soccer and gymnastics, both of which he loves dearly. He is gearing up for Kindergarten in the Fall. In his spare time, he dresses up like a SWAT team member, is obsessed with Teenage Mutant Ninja Turtles and paramedics, and can be found with a light saver at just about any moment. He is incredibly verbal and is testing above average in both cognitive development and communication skills. We are beyond blessed at how "normal" his life is.

How does Bodie differ from a "typical" child his age?
The biggest difference between Bodie and a typical 5-year old is in his endurance. Although he runs around and can keep up with kids his own age, he still tires easily. We believe it is due in part to having low oxygen saturations for so long that his body never developed the endurance of a typical kid his age Although his oxygen saturations are now typically around 95% and he doesn't get out of breath like he used to, he still lags behind his peers a bit in endurance. Because he only has half a heart, his body has always had to work harder to keep up. But he doesn't let it stop him at all. His cardiologist has told us not to limit his activities, that he will learn to "self-limit" and slow down when he needs to. We haven't seen that yet! Ha!

But he looks so healthy! Is he really as sick as you say?
Thank you - and YES. The challenge about cardiac kids is that they can look totally fine on the outside, but be ticking time bombs on the inside. It's very scary to parent a child like that. We see his cardiologist every 3 months to always have a good handle on his heart function, which gives us considerable peace of mind. We let Bodie live life as much as possible, including letting him run around, play in the dirt and be a kid. But we do try to limit his exposure to visibly really sick kids (especially those with the flu, which can be pretty detrimental to a kid like Bodie, and would likely land him in the hospital) and do watch him carefully in really hot or cold weather as it's hard for his body to regulate and he can easily become dehydrated. So, we let Bodie "self-limit," and when he gets older, we will respect those limits, including letting him sit out of activities if he decides it's just too hard for him. It's not about pushing him and making him tougher in the process as it would be with a typical child. It's about respecting how serious his heart defect is and not pushing his body into cardiac arrest.

How many medications does Bodie take and how hard is it to administer them?
Right now, Bodie takes medicine 2 times a day - with breakfast and with dinner.  In total, he currently takes 6 medications: Baby Aspirin (to reduce the risk of clotting), Enalapril (to help preserve long-term ventricular function), Sildenafil (to help with pulmonary hypertension, developed in utero as a result of his heart defect), Singulair (for his asthma - maybe from the heart defect, maybe just from living in Los Angeles, who knows?) and Flecanide and Nadalol (both to control his arrhythmias). It sounds overwhelming to have to administer 6 different medications to your child, but honestly, it becomes so much a part of life that it's very easy. It's no more difficult than, say, administering Tylenol or Motrin to a healthy child. All but one of his meds are in pill form and our little rockstar throws them all back with a sip of water. It's actually pretty amazing. Easy peasy.

Will he always be on medication?
Very likely. We are currently weaning his Silendafil down and hope to be completely off of that in the next year or so. And we expect him to outgrow his asthma at some point an lose the Singuilar. But he will always be on Baby Aspirin due to the risk of clotting for these kiddos. Beyond that, it will be up to Bodie and his cardiologist. Our hope is that he will outgrow his arythmias and the need for the Flecanide and Nadalol - but given the family history, that's not terribly likely to happen. It is highly likely that, in order for him to come off his anti-arythmia drugs, he will need an additional surgery to cut the nerve that sends adrenaline to the heart. That is on the horizon for sometime in the next few years. Once he's past that surgery, his long-term meds may include Lasix (to reduce workload on the heart) and Enalapril.

What is his long-term prognosis?
The short answer? We don't know. The surgeries to treat HLHS have only been done for about the past 25 years, and major advances in medicine have been made in the past 10 years in this area. Survival statistics used to be less than 5% when they started the palliative surgeries. They're now 60-75% at 5-years at most major institutions, which is amazing progress in a relatively short time frame. When they started these surgeries, the assumption was that all of these kids would need transplants at some point in the future, since the right ventricle is eventually going to get tired of pumping to the entire body. So the goal was simply to get them as far as possible with their native hearts before going down the transplant route. But, to their surprise, many of these kids are making it to their teens, some even into their 20's, and have not yet needed transplants. So, who knows? Our hope is that, by the time Bodie needs a transplant, they'll have figured out a better way to do it. That's why we're so passionate about backing funding for CHD research.

6 comments:

  1. Your blog is beautiful as is your family! Glad to see that your son is doing so well! Another heart warrior out there!

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  2. Body is a cute boy.

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  3. This blog is absolutely wonderful. I am working with a family whose 13 month old was diagnosed before birth with Hypo Plastic Left Heart syndrome and he is facing another surgery in a couple weeks. I was wondering if you could e-mail (vsjarvis59@gmail.com) me some resources to pass on to the family to help with outside expenses such as gas, food, lodging, and other bills. Anything would be grateful.
    Thanks,
    Vickie Jarvis

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  4. Thank you for sharing Bodie's story! Our grandson is facing heart surgery soon to correct a complete AV malformation. He is 20 months and his name is Bennett!!💚

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  5. My husband and I were recently given the unforunate diagnosis of hlhs for our little one at our 17 week ultrasound. So many emotions and thoughts are running through our minds. Our life is forever changed. I, of course, have been doing lots of research online (not always a good thing), and came across your blog. You truly are an inspiration and have changed my outlook. You are so positive and give me hope. Thank you!!

    Eva

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    1. Hi Eva! I'm so glad you found my blog. If you haven't already, please reach out to Sisters by Heart (www.sistersbyheart.org) - it's the best place for comprehensive support and resources for newly diagnosed HLHS families. It is a scary diagnosis, but there is SO MUCH HOPE! <3

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