Why I am blogging about Aidan

The PICU attending told us, "Your son is way out there in the stratosphere of medical miracles." This was way later, when we met him during a much less critical hospitalization. He went on to explain that our 4 week old had not only had a cirrhotic liver but also, when he first arrived there, was in the grip of ARDS. 90% of babies with NH die before transplant. 30% of adults with ARDS don't make it, and some of the rest have permanent lung damage. And here was a neonate who weighed about 4 pounds.

At another time a resident told us "they usually don't get over it" -- "IT" referring to the fungal meningitis they diagnosed him with soon after his transplant when he was massively immunesuppressed. Again, this was way after Aidan was over it, on a clinic visit. They took him off anti-rejection meds in order to give him a chance to fight the fungal infection, and of course he had a rejection, which they had figured was better than the alternative. I have no doubt they were right. Then they treated the rejection. Rejection sounds scary to most people, but it is not as scary to doctors as fungal meningitis.

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I have been wondering whether it was the right thing to do to start this blog about Aidan's past. Especially when he is relatively healthy now, and some of his peers are having ongoing crises, and their parents don't know what the outcome will be. Aidan got through the grim gate, though the teeth left their marks, and there may be other gates in the future.

Is it selfish of me, obsessive, depressive, something pathological?

But then, on the other hand:

• I want to be able to remember all this someday, for Aidan's sake and for my own. Sometimes I try to remember details like when Aidan had his last surgery, when he was dealing with that bile duct problem, when he learned to sit. I don't have it written down except in little scraps of journals and calendars here and there. There is so much to remember, and I want to be able to remember.
• I want to be able to get it out of the underside of my mind. When I started writing I felt as if a cloud had lifted. Finally, grabbing those shadows and pulling them out from under that bed. Perhaps this is selfish, but on the other hand, it's also free, unlike a therapy session or an antidepressant. And no one has to read this blog if they don't want to. That's free, too (and if you notice those mixed metaphors, please interpret them as a sophisticated depiction of the post-stress mom's confused state of mind, not just confusion plain and simple : )
  
• Also: I want to have something out there for other parents of NH children or other devastating conditions. In one way, it does not matter if no one ever reads this blog besides me. In another way, I think it does matter that it's out here in the public record, because Aidan's story is unique but it also fits into a wider medical category where there is not that much information or support as yet. Even if no one reads it now, there might come a time when it will be useful to have it out there. Or so I tell myself. There, I feel much better already.
  

I remember one time I had to call the transplant director for some quite innocuous thing. Aidan was doing fine, so I wasn't stressed, right? It was something about getting labs done on me for research into the maternal factor in NH. This physician had fought with Aidan through enough way out there events to fill a medical journal; we had a good working relationship. I had to leave a message on his voice mail. I've left countless messages on voice mail; I can do it at least adequately. This time, I started stammering. I stammered as I left the message, real stammering, as if my teeth were chattering. The totally unexpected stranger who had taken over my voice couldn't seem to figure out how to manage this event, so I kept stammering my way through my stupid little message. I must have sounded drunk or demented (Heey Dr R, y'want to share a beer with Aidan and me??) , and for a long time afterwards I expected to get a call from a kindly medical social worker asking tactfully how things were going. I still wonder what the doctor must have thought and sometimes I wake up cringing, thinking about it. Anyway, he didn't return the call. No wonder. Obvious psychotic issues would probably invalidate the lab results, anyway.

Another liver transplant mom told me she just barely stopped herself from throwing an IV pump out the window once. The thing wouldn't stop beeping and giving her an "occlusion" message. This was her first realization that she was seriously stressed. The stammering was my first realization. A lot of times you think you're coping but you're really just stuffing it under because you can't afford to deal with it right then. That's a legitimate survival tactic, a sort of psychic equivalent of the physical shock that helps you keep your basic systems going, but you can't keep going that way forever. Someday you have to deal with it. That's why our transplant coordinator says that post-crisis is sometimes the most difficult time.

Aidan's iron, and a bit about my quotes

When we first arrived in the university hospital where Aidan was to await his transplant, Dr R introduced himself to us and for the first time we got a diagnosis for our child. We knew his liver was in the last stages of cirrhosis but we did not know anything else.

Dr R, who was the liver transplant director, told us that the diagnosis was Neonatal Hemochromatosis. The basic information is here. At the time, Dr R was very careful to tell us: our baby did not have hereditary hemochromatosis. This is a very different condition; an iron storage disorder caused by some sort of gene mutation.

In Aidan's case, however, he had received his extra iron, which had destroyed his liver in utero, from his mother. Me. I don't have any medical problems, at least not the kinds that affect me, but when I am pregnant, I transmit way too much iron through the placenta. The present research hypothesizes that this is due to an alloimmune response of my body to that of my fetus. This alloimmune response sometimes does not trigger until later pregnancies. Aidan was my 8th pregnancy, though only six came to term. The others were all essentially health and my pregnancy with Aidan was textbook normal for a grand multipara. I suppose I am way out there in the statistical zone since most mothers do not have that many children, and NH is way rarer even than 8 pregnancies.

Kevin and I were told that Aidan would be put on a heavy-duty chelation cocktail while he awaited transplant. No hope of fixing his liver; just hoping to slow the end stage disease process . The thing with livers is that they process proteins which are the building blocks of growth. Aidan was in a Catch-22 -- he needed to grow in order to have the transplant but you need a somewhat functional liver in order to grow.

The doctors at the university center were honest with us. They told us the statistics -- grim--and that this would be a battle. A marathon, was the exact term. Other parents may feel differently about this, but for us it was refreshing. We didn't want to be patted on the hand. We'd done that at our home hospital and then when reality hits, you are blindsided. We wanted a team who would face reality and commit themselves to doing their utmost for our child, and this was what the team emphasized.

We were also told that any future child would have >80% chance of having the same condition. At that time the only treatment options were the wryly named "chelation cocktail" and eventual transplant.

My present blog title, though I may change it, came from a poem by Robert Browning. I thought it evoked the strength of the disease and Aidan's victory, with a few scars where the iron gate's teeth caught him on the way out. It looks a bit grim up there, though, and I don't want this to be a grim blog, so I may change it.

The other quotes:

Of what use were wings to a man fast bound in chains of iron?
Afflictions are but the shadows of God's wings.
"...to have faith is to have wings."

I put the first one up there as a rhetorical question. The answer is that wings were of great use to Aidan. Wings are of course to be understood figuratively as the immaterial things that bear us up through the worst times. Things like the commitment of a medical team, the prayers and support of friends, the hope and love of relatives, his own courage and joy during his struggle. They were stronger than his "iron chains" (shameless puns here; thanks, Dad!)

But it looked too despairing by itself, so I put up the others.

About Waiting

Moreena wrote about finding that her daughter is not on the transplant recipient list, three months after she had thought she had been placed. Status: 7, meaning inactive.

This brought me back so clearly to an evening in middle to late September 1999. I was in the NICU, holding Aidan. He had finally reached the weight where he could be a possible candidate for transplant; the minimum was 5 kilos, or about 11 pounds. He was three months old. He had a pik line into his scalp (slightly more permanent version of an IV) , which was constantly oozing a bit of blood because of his coagulation dysfunction; he wore a bunch of monitor attachments; he had an oxygen cannula in his nose because his distended belly (from peritoneal fluid due to liver failure, sort of like the large bellies of famine victims) compressed his lungs too much for him to be able to take a deep breath. His hands and feet bore the stigmata of hundreds of little pricks from IVs and daily blood draws, many of them very bruised, again because of his prolonged coagulation times due to end-stage liver failure. Even today, many of his littler veins are stopped up or rerouted because of the scarring, so every blood draw is a bit of a reverse roulette. ... jab that needle and hope for the best, and sometimes repeat again and again.

AH -- I'll take a deep breath now. It's hard to bring myself back to those days, and there is a lot of medical terminology there. Basically, the kid was covered with tubes and wires, his little arms and legs were wrinkled and thin, and he was a deep, warm golden from the liver disease. Even his eyes -- golden and brown, looking up at me thoughtfully, with a hint of introspective melancholy. He was at risk for a major hemorrhage at any moment, and he could hardly breathe. He had to struggle through every day, and he had struggled through about 100 of them so far. Everyone knew he was getting to the end, if he didn't get that transplant.

His belly was like a big golden melon. You see a petite woman pregnant with twins and you get a very rough idea. A very deeply tanned pregnant woman, perhaps. It was hard to hold him so he would be comfortable. If I shifted him the wrong way he'd give a small groan and his oxygen SATS would drop. His heartrate would periodically go above 200 during these days. Then the monitor would ding. So it would be like this: me humming, rocking, looking into his deep soft eyes. Ding! a musical, soft note, like an airplane announcement. I hum some more, exchange a couple of words with a nurse. Dinnngg! Above 200 again. This was happening all day, because Aidan's liver failure was generalizing to his other organs, and he was in the beginning stages of heart failure. They couldn't do anything about it; the monitor was just there as a sort of herald, a reminder to keep a lookout. Sometimes if the dinging continued for too long, the nurse would push the mute button. Then I'd watch the screen, with fascination, until it dropped back down to the 170's or whatever was his norm back then.

My husband was to be the living donor. He had gotten all the tests and he had the same type blood and was in great health. The surgery would happen any day, now that Aidan was up to the magic 5 kg. We had a graph that we used to mark down his daily weight. Oh, we were involved parents. I spent several hours every day at the NICU unless I had a cold, and then Kevin would go instead. I was drinking up this time with my baby. Kevin (my husband) would keep an eye on our other five kids. It took the nurses about ten minutes to unhook him, swaddle him, put him in my lap, and rearrange all his tubes, so I made sure I got to the bathroom BEFORE because basically, that was it. If I was going to put him back down in his isolette, it was going to be a while before I could get him in my arms again, because that was a major undertaking for everyone.

And that was all I wanted; just to hold him. There were so many days in his short life when I hadn't even been able to do that. It was like all my mom instincts were focused into a pinhole -- just get my baby in my arms, wrapped in immaculately clean, warm- from- the- warming- oven hospital flannels. Stay with him all day. Just hold him. Forget going to the bathroom or getting a snack. Just hold him and hold him. Then go home (to our little temporary city apartment) and try to dredge up some mothering instincts for the rest of the kids.

This one will have to continue in another post. Anyway, here is Aidan during that time.

Starting Somewhere

This will not be easy.

Even thinking about those days makes me a bit disoriented. I've written about it before:

Why June is a Weird Month
Gift of Life
When the Time Train Derails

"When life gropes

Feebly for the path where fell
Light last on the evening slopes,

One friend in that path shall be,

To secure my step from wrong;

One to count night day for me,

Patient through the watches long,

Serving most with none to see."

Robert Browning

I dedicate this blog to all Aidan's friends, on earth and in heaven, and in the hospital, which seems to be an in-between place that partakes a bit of both.