Twelve years ago, on Father’s Day, I got a call from the hospital with my new heart. In honor of Father’s Day and my anniversary, I am sharing a letter my dad wrote to another parent with a child in need of a heart transplant.
Teenage Heart Transplant – A Father’s View
Val was diagnosed with Restrictive Cardio-Myopathy (RCM) at age 9. She was listed at age 14 and transplanted at age 15. She’s 24 now.
Symptoms/Diagnosis. As she said, we saw symptoms as young as age 5 when she couldn’t keep up at soccer and was the slowest girl in running laps. She would also say, “I can feel my heart” at times, like on walks. The pediatrician finally caught a murmur and referred her to Children’s at age 9. At Children’s, not everyone could pick up the murmur; it apparently was slight. It’s entirely likely that a pediatrician with less sensitive ears would have delayed diagnosis for possibly years.
How to Know When. The best indication we had of her decline and how close to needing a transplant was the stress test. Each test has a number. As her condition worsened, the numbers came down. I plotted it all on a graph and asked her cardiologist at what point a transplant is needed. As I recall he said between 12 and 15 is when a transplant is needed. Her numbers started in the 20’s (I think 30-40 is normal) and came down with almost each test. (They leveled out once or even went up, giving us some hope, but went down the next test.) Sure enough, just as the number hit 15, her cardiologist listed her. (If I can find it in electronic form, I can send you Val’s graph.)
Shortly before Val was transplanted, she had periods of no energy. She was still Priority 2. One time I had to go to school when her friends called from play practice. Val could not get herself up from the floor (it started because she was laughing too much). But she was in the play for the real show. As she said, she could not make it between classes on time. She was just about to get to the point of not being able to do normal activities, and we were planning on home-schooling her that Fall, when we got the call in June. That was one full year after being listed.
She was lucky: she got transplanted while still Priority 2. Her cardiologist was talking VAD if she got to Priority 1 with no donor available.
The Transplant. The surgery itself is apparently “just another” open heart surgery. You just have to get over not wanting your daughter getting needles and tubes stuck into her. She and you will get a little more used to that as she gets catheters in the months before-hand. But at the transplant itself, I finally counted up all the tubes going into Val. I recall there being approximately 10. I measured post-op progress by how many had been removed.
As far as worrying about something going wrong in the surgery, all I can say is (1) the statistics are really good, (2) Children’s is as good as it gets, (3) pray (or smoke or knit or whatever you do), and (4) there’s not much you can do about it anyway.
It’s “just” heart surgery, but you hate to see your kid go through it. But there are other parents and kids who have already gone through it multiple times before the transplant. Val was lucky in the sense that RCM was the “only” thing wrong with her.
While I think Children’s is about as good as it gets, it is still a hospital. There is no one really responsible for complete patient care. Each doctor or nurse has a specialty role. They don’t coordinate that well. Sometimes doctors will contradict other doctors or even their own previous orders. And they don’t always write stuff down for nurses; they just leave verbal messages or orders. I recommend you stick around your child and listen to what the doctors and nurses are saying and what’s happening with your child and what she’s saying. And you be your child’s champion. You might have to ask hard questions or even refuse to go along with something. Don’t assume anything. There are a million people coming into your child’s hospital room, each doing things or asking things. And they apparently don’t talk to each other or compare notes. (The assigned nurse in the IC is an exception. She will know just about everything going on with your child and is usually right there.)
It turns out your child has one doctor truly in charge at a time. In surgery and then in the IC it is the surgeon. After that (in the normal hospital room) it is the cardiologist, again. Everyone else is less necessary. If you need to, appeal to that doctor. (There was a time with Val when the surgeon and her cardiologist were not seeing totally eye-to-eye on something, and it came close to a turf battle. It almost came to “she’s my patient until she leaves the IC”; it didn’t, but there was some serious glowering. I can’t remember the issue, but I think I was more on the surgeon’s side.)
Recovery. Val had a fairly unique post-surgery problem. They left her heart sack (pericardium) in, which was standard practice. I later understood that they remove it routinely with all adult open-heart surgeries, but leave it in for children. They remove it on adults because about 2% of the time it fills up with fluid and restricts pumping action. Apparently, it almost never happens with kids (at least infants). Well, that happened to Val. It also happened with one other teen transplant before Val. I’ll play doctor here, but I think teens are more like adults in this regard – it’ll happen about 2% of the time. (There’s not that much data on teens; it’s mostly adults and infants.)
In Val’s case, she ultimately had to go in for a second surgery – not open heart, but open chest. And in the meantime, there were scary moments when her heart was being “choked” and we had no idea why. (Emergency room, the biggest needle you ever saw, near loss of consciousness – bad stuff.) Then we had to drain it occasionally (tube in the chest), which I did at home for a while before the second surgery. It was this second surgery (about 2 months after the first) that made her scar more visible; the first one was close to invisible, or at least no keloid.
You might want to talk to the doctors about removing the pericardium during the transplant. For all I know, they do that routinely now for teens just like they do for adults.
If that little pericardium thing had not happened, the recovery would have been great. She came home within a week of the transplant. Her cheeks were pink. She had endurance and felt fine (until the fluid built up). And after her second surgery, all was well (mostly, see below).
Rejection and Medicines. You’ll come to know the names and dosages of more medicines than you’d think you’re capable of. They slowly take her off some meds and reduce dosages. At the beginning, I think there were something like 7 or even 9 pill types. After about a year, it was down to two, and has been ever since.
They started Val out on Cyclosporine I believe, which was their standard practice. It had some side-effects, but they switched her to Prograf (FK) after we asked some questions. She and we liked that a lot better. You might want to ask about that. For all I know, FK is now the standard.
Val’s rejection was usually mild (I think the number assigned was 0 or 1). One time the expert who looks through the microscope was on vacation or something, and they used a back-up person. That guy said the rejection was high and they called Val in for extra steroids to be taken intravenously, etc. It’s not terrible, but nothing you want to happen either. In this case it turned out the biopsy was misread. There really wasn’t rejection (or it was low). Apparently, the examination of the biopsy is more art than science, or takes lots of practice and experience.
You can expect some “low” rejection numbers. They actually try to adjust doses so that they give you just enough to keep rejection away. As they calibrate that over the months, it’s not unusual to see a rejection number above zero. If numbers get high, they give her more steroids or something. After a year it should all settle out. At least that was all pretty copacetic in Val’s case (after the one false alarm).
The Out-Years. Once Val recovered from the surgery itself (some sore chest, etc.), which took weeks to a month or two, she was normal by all counts. She had energy and pink cheeks right after the transplant. By anything I can tell, she is normal. It is usual for transplant patients to have higher-than-normal pulse rates. I think Val’s is about 90 to 100, even at rest. She has a little hand trembling (where you hold your hand steady and see if it moves), which I think is a side-effect of one of the meds. (Nothing bad. Probably couldn’t be a rifle marksman.) Other than that, pure normal. You’d have to ask Val about the scar, but she seems OK with it.
She finished high-school, then college. She played sports as much as she wanted. Etc. Nine years out. All tests since that first year have been good. Biopsies and catheters never go away completely, but they do them less often as time goes on. (Now its like a cath every three years.)
Summary. I probably scared you too much. But things come one at a time and you’ll find you can handle them. Your kid will do better than you do. And some people have it worse. You’ll see that, too, at Children’s.