Broken Heart

It wasn't eight months ago that my husband and I were talking about how lucky we have been with our daughter, Ally.

Don't get me wrong - I'm not changing that position now, but it seems as if I should've knocked on some wood when the conversation happened. At four years old, I could count on one hand the number of sick visits Ally has taken to the doctor. She didn't have her first one until seventeen months old when she suddenly developed an ear infection, and even after that very first sick child visit, there were very few more. I personally felt, and her pediatrician agreed with me, that this could very easily be attributed to extended breast feeding (she was over 2 before we stopped) and also that I was blessed enough to stay home with her and take her to work with me rather than having to drop her off at the Germ Festival (aka daycare) every day.

What sparked this conversation between Al and me is that at age 3 1/2, Ally started crossing her right eye, and at first we thought she was doing it on purpose. Months went by and she didn't stop, in fact it started getting more and more noticeable and frequent. At one of her rare sick visits to Dr. Ellis, we asked about it, and he sent us to see an ophthalmologist. I admit to taking it harder than I should have when we were told she needed glasses. No big deal, in retrospect, but my previously Picture Of Health daughter suddenly needed something she wouldn't understand. After patching her "good" eye for three months, her eyes are now about even with a +4.50 prescription lens. She loves her glasses... they're purple.

A month after her follow up appointment with the ophthalmologist, we returned to Dr. Ellis for her four-year-old checkup. Totally routine... she's healthy and happy and (finally!) potty trained. Until he notices a heart murmur.

"It's probably nothing," He said, and before we had her shoes back on, we had an appointment with a pediatric cardiologist for an echo.

Somehow, when a doctor tells you "it's probably nothing" but he's referring to an imperfection in your baby's vital organ, it's not so easy, for me at least, to believe that it's probably nothing. It's not something I'm proud of, this tendency to fear the worst, but there it is, my greatest weakness. Where Ally is concerned, it has always been nothing, or almost nothing, for over four years, but still I worried.

Four days later we sat in a pediatric cardiologist's office while the doctor sent her out of the room and told us to stay put.

"It's not nothing," He said.

Hypertrophic Cardiomyopathy is a congenital heart defect in which the heart muscle grows too thick and can't efficiently pump blood. In Ally specifically, there is significant blockage of the left ventricle, and her heart must work too hard to get her blood where it needs to go.

Obviously neither of us knew anything about this disease, but we knew enough by the time we walked out of there to be devastated. Her entire life will be affected, she can't participate in sports or strenuous exercise of any kind. It doesn't get better, it does get worse. She will need surgery. Most alarming, we were informed, is that when you hear of otherwise perfectly healthy kids falling out on a sports field with sudden death, it is usually this disease. A silent and very serious killer that often exhibits no symptoms before sudden death occurs. We are lucky to have found out about it.

The doctor sent us on our way and told us to come back in six months, or call him if she started showing symptoms.

That lasted for about twelve hours, at which point we decided that with UAB and world-renowned heart doctors a hundred miles away, it was beyond stupid not to get a second opinion.

My sister-in-law's mother, Robbie, is a cardiac nurse at UAB, so the next day I spoke with her, asked her who to see, and a week later we were in Dr. Lau's office.

It's not what you know, it's who you know, they say.

Dr. Lau did another echo, and an EKG, and put Ally on a Holter monitor for a 24 hour period. In a case such as hers, he said, he needed to consult with his colleagues. She's very, very young to have such a severe manifestation of this disease. It typically does not show up until puberty, so the fact that she has it so bad now is unsettling. At the same time, performing open heart surgery on a four year old is nothing to go into lightly, either.

Dr. Lau promised he would call me once the results of the Holter were in. When they were, he did, and it wasn't good news. They felt that we should seriously consider surgery sooner rather than later.

It's a sickening feeling that doesn't really go away. Every time I think about it, about what that entails, I lose a little bit of nerve, get a little queasy, a little shaky. That's my baby they're talking about cutting open. Something essential to her survival that they want to operate on.

I needed reassurance from someone who could be both informed and objective, and after a phone call to Ally's pediatrician, Dr. Ellis, we made up our mind to get a third opinion, from a specialist in this condition, a decision which had the full support of every doctor we spoke to. HCM is rare enough that specialists are relatively hard to find, but with family in Ohio we decided the best place to go would be the Cleveland Clinic, to see Dr. Zahka.

Dr. Zahka requested extensive bloodwork...bloodwork arrived in advance, the MRI disk rode to Ohio with us.

Dr. Zahka answered a lot of questions for us, reiterating what the others had said about her case being severe. Surgery would be necessary within a year, most likely, and unfortunately a year does not reduce the level of complexity with this surgery. Ten years would make a difference, but there's no way she'll go that long without surgery. He assures us he will review the MRI and the echo with other doctors in Cleveland, and will call us once he's done so.

Five days later, Dr. Zahka is telling me that it needs to be within six months now. There's no benefit to waiting, and even though he'd originally been of the opinion that an ICD (implantable cardioverter defibrillator) wasn't a good idea, he'd changed his mind about that, too.

Knowing that so many brilliant minds, both in Birmingham and Cleveland, have been talking, thinking, consulting about Ally is both comforting - under the circumstances - and upsetting, because the circumstances warrant so much attention. There's no doubt about it, her little heart is not in good shape. It's bad enough that while they do not usually want to perform such a serious surgery on such a small child, in her case not doing surgery is the riskier option.

It's heavy stuff. Hard for a parent, considering either path, knowing the risks involved.

My sweet, tender hearted, stubborn, smart, funny, silly girl. If I could trade places with her, I would. In a heartbeat.