Sunday, October 17, 2010

Half a Miracle

The unit that I am working on is a day procedure unit. I'm sure I've mentioned this before. Basically we function as a souped up doctor's office where kids (ages 0-18) come for procedures, treatments, IV medications, blood work and/or to be seen by the service they are covered under in the hospital (GI, Neurology, Rheumatology, Immunology, etc. etc.)

It covers a lot of areas of medicine I'm completely unfamiliar with. I could tell you all about premature baby physiology, how to care for a pre-op gastroschisis patient or how to set up for a nurse to insert a PICC line, but none of that is useful where I'm working. At times, it's frustrating to be so out of my element. On the other hand, I'm learning and seeing new things.

One of the more interesting patient populations that come to our unit is those who have had, or who might need, transplants. I think the general population has the idea that once someone receives a transplant then *poof* you are cured, and can get back to being 'healthy'. In reality, transplant patients have just traded one fatal condition for another condition that must be managed for the rest of their lives. Don't get me wrong, these patients are very grateful and happy about their new organs. One nurse I work with told me transplant patients often consider themselves to have two 'birthdays'. Their actual one, and the day they got their transplant.

But things don't always work out with the new organ. Sometimes, even with good matching and all the drug therapy available, the newly transplanted organs fail to work in the 'host' body and get rejected. I met one 8 month old baby the other day who has had not one, but TWO liver transplants. The first organ failed within a week and she ended up getting another organ which 'took'. She is really cute and looks almost completely normal. Probably most amazingly of all, I met a family the other day whose son has received a bowel, liver AND kidney transplant. That's most of his major organs. It kind of boggles the mind.

However, the follow up care for these patients is no joke. These patients will be medicated daily for life with anti-rejection drugs. In our unit we also often give these kids an IV infusion of immunoglobulin which helps to block the patient's own immune response to the new organ. All this immune suppressing can of course be a issue with the patient trying to fight off the pesky flus, colds and other bugs that our bodies deal with on a daily basis. It can also have other major consequences, such as the patient developing cancer. That's right, cancer. Cancer is an overgrowth of abnormal cells within the body, and the immune system plays a role in preventing their growth. Suppress the immune system and you've just upped your cancer risk. We have kids in the hospital who have died, not from the disease that was originally killing them that required them to get a new organ...but from the cancer that they got post-transplant from taking all the immune suppressing drugs that were keeping them from rejecting that lovely new organ.

Seems completely unfair doesn't it?

I've always been somewhat interested in transplant patients, not because I think they are medically interesting (although they are)...but because someday I'm afraid I'll need to be one. Although my heart is coping well, and has been since my 'major' surgery at the age of 6...I'm always afraid one day it will stop doing what I ask of it. Now my parents, my family, even my doctors have never really wanted to play the 'what if' game with me when it comes to my heart. I've always felt they have stuck with the opinion of 'you're doing really well right now...why worry about it?' (HAHAHAHA...have you MET ME??!! I am a WORLD CLASS worrier.)

Still. I do worry. Every day. It's always at the back of my mind. One time I tried to explain it to my husband. I told him it's like this:

Every day when I get off the subway at my stop there are two large flights of stairs that I have to climb. Probably about 50 stairs in total. Every time I'm at the bottom and look up, the thought goes through my head what if this is the time that I can't make it to the top without stopping to catch my breath? I think it every time. It's not even a clear thought anymore after all these years. It's just a single small stab of dread. And then I climb the stairs and continue on with my day. But I know, if the day comes when I cannot climb those stairs without stopping, then it's the beginning of the end for my heart. And what comes after, I do not know.

This all sounds incredibly ominous...but that's what it is to live with an organ that is not 'perfect'. I cannot escape that fact.

This all brings me to the real point of my story.

Last week, I was looking after a little girl who needed IV antibiotics for a fairly minor infection. Something any kid could end up with...but this girl wasn't a regular kid. She has, what probably I, and most cardiologist would agree, is the 'worst' kind of heart condition. Hypoplastic Left Heart Syndrome (HLHS). It is basically the mirror image of what my heart defect is. I am missing the right ventricle of the heart...she is missing the left. The left ventricle being 'more important' because it's the pump that circulates blood to your body, while the right is responsible for pumping the blood to your lungs. Surgeons have constructed my heart so that the blood is pumped all the way around my body AND to my lungs by only my single left ventricle. This, obviously for me works well. For a person who has to rely on the inherently weaker right ventricle to do the same job,'s a less than completely satisfactory situation.

I have been told by my former NICU veteran colleagues that if I had been born in the same year that I was, (1982) with HLHS...the doctors would have had a completely different plan for me. Their plan would have been no plan at all. They would have handed me back to my parents and said "take her home and enjoy her for as long as she lasts". There was no saving HLHS patients back in 1982.

So, to see this little girl running down the hallway the other day was pretty amazing. She was not however, completely 'well' looking. Her lips were blue-ish and her fingers were clubbed (a deformity of the fingers often associated with low oxygen saturation). For pictures see here. She has been admitted to hospital for issues in the past, and I'm sure she will be again.

She was however, smart, cute, funny and in school. She was as totally 'normal' as you could get while missing half a heart.

This (along with pretty much everything else these days), makes me think of Aidan. Before I got pregnant, I knew that due my own heart defect, my child has a slightly higher risk of having a heart defect too. The generally population averages about 1-2% of having a child with a heart defect. I, apparently have a 3-5%. The defect my child may have would not necessarily be the same as mine.

I remember thinking...okay, I could deal with that...just as long as it's not HLHS. Honestly, I'm not sure I would have continued with a pregnancy if it meant having a child with HLHS. Not because I wouldn't want to care for a child with that defect, or would be afraid of losing them due to it (which of course I would be)...but because I would not want them to have to cope with seeing that set of stairs and knowing they will not be able to climb it without stopping.

But seeing that little girl the other day made me think. She is happy, laughing, playing, goes to school, is loved by her family...but one day may face her heart wearing out, and a transplant, and maybe rejection of the organ or possible cancer related to the drugs...and the constant knowledge and fear that she will not make it to old age, or to having kids, or to graduating university, or to have a boyfriend (or girlfriend), and may never live away from home.

But my son, with his perfect heart, died. And she, with her damaged heart, runs down the hallway yelling 'Daddy, Mommy, watch me!'

It's like watching half a miracle.

Have you, or someone you are close to, ever coped with a 'life limiting' disease? How has it shaped your perceptions? Does modern medicine ever feel to you like 'half a miracle', where some are saved, but at what cost?


  1. My son was diagnosed with HLHS at 28 weeks gestation and died at 29 weeks gestation. That little girl is going to have the toughest life possible and each day she lives is like a month for her, sometimes a year. Just like with HRHS, every single second matters. I am very sorry for the loss of your son. I wish things were different for you.

  2. You're so right that not all transplant stories have a happy ending. My dear uncle (my dad's sister's husband) died at 52 after not just one but two liver transplants that didn't take. I don't think any of us thought it wouldn't work. The last time I saw him, he was just waiting for the call & was so upbeat & excited to get on with it. He never left the hospital.

    For all the descriptions I read that it's a "rare heart condition," dh & I met a lot of bereaved parents through our support group whose child had had HLHS. Obviously, these were the babies that didn't make it. Some parents terminated the pregnancy after thinking about the what they would have to put their child through & the quality of life he or she might have -- some went through the pregnancy but the baby didn't make it to the first operation -- some had the operation & the baby died later. Hard to know what I would do in the same situation. It's a choice no parent should have to make.

  3. Wow, I really liked this post. Obviously I don't like all the problems that people have, but I found it interesting. As you know, I volunteered at Sick Kids on the organ transplant floor for a few years. I saw kids live and I grew close to kids who died. It was heartbreaking to watch them suffer, but it felt so good when they went home after transplant. Of course, I knew that they could easily be back. I knew that the organs could be rejected and they would have to go through it all again.

    At the church I go to, there was a little boy who had 3 liver transplants. He was perfectly healthy until he was 9 months old and then they discovered that his liver was failing. One transplant lasted a year or so. Then he had another one. That one failed right away and he had his third and last transplant on his 3rd birthday, Oct 3. He died the same day. He had all his transplants at McMaster Children's Hospital. In spite of all the pain he experienced, he was a very happy little boy. His struggle and his passing changed everyone who knew him. He is actually buried in the same place that Jacob is. When I was choosing a spot in the garden for his ashes, I put them near Sam's so that he would be near another little boy.

    I hope you never get out of breath when climbing those stairs.