So first things first. Thank you. Although 'Thank You' doesn't really seem to cover the gratitude I feel from the incredible response from the last email. We were in dire need, and to say that people came to our aid is a monumental understatement to the incredible outpouring of emotion, support, prayer, stories, gifts, phone calls, tears, hugs, love, food, understanding and compassion.
The incredible good karma you've created has made me feel more connected and human and loved than I could have dared to believe. You have carried us fully and unconditionally – as Dickens almost said, its been the worst of times but you've made it the best of times. Thank you.
Every touch has moved, inspired, encouraged or calmed me one way or another. I've received several hundred emails and I've got around to replying to about seven, for which I'm very sorry. I hope to get to them all in turn, but please know that every email, visit, phone call and text message has made a difference and has helped us through this really tough time. Thank you.
I know some of you offered up prayers and thought good stuff, and didn't bother with mere mortal communication. Don't worry, all that spiritual energy got through too and I appreciate you mightily beseeching the powers that be. Some prayers went out to the standard white males like Yahweh, Our Lord and L Ron Hubbard. But above and beyond that the Vole has had blessings (the hippies), incantations (the witches) , smudge sticks (the native Americans), crystals (the new agers) and whisky/tequila (the Catholics) sung, poured, rubbed and wafted either over him or ingested nearby.
Some believe in gods. Some in science. Some in leprechauns. Its great to know that whether there is a heaven, or a big rainbow with a pot of gold, or it all just goes dark at the end, you've got this kid covered. He's needed it. Thank you.
So is it working? Well yes. In parts.
The long journey home
And so to the update. When we last left him, our hero aka Oliver Valentine aka The Vole, was stuck at the hospital. Due to a hard birth, and a suspected placental abruption he had nearly bled to death and had suffered "moderate" brain damage to both motor and cognitive parts of his brain. Although Joe Lieberman might not know what 'moderate' means, the docs explained that the damage to his motor center was giving him very stiff body and limb tone which would limit his ability to crawl and probably prevent him from walking. The cognitive damage would be unassessable for a couple of years. It was cold comfort that Bush in the White House was proving half a brain isn't a limiting factor in America in the new millennium.
The most immediate issue was Oliver's eating, or rather lack thereof. The Vole had apparently over-heard that one never be too thin or too rich. Realizing he'd missed the San Francisco real estate boom due to his parents poor timing he decided to focus on his figure and was very coy about sucking down the breast milk Jill lovingly and painfully pumped for him. To bypass this fastidiousness the doctors placed a yellow feeding tube up his nose which pleased Oliver not at all and he dedicated his early coordination efforts to pulling the damn thing out.
The doctors, realizing this was a baby not easily thwarted, removed the tube but darkly warned that Oliver needed to chow down 60cc of milk every three hours or a feeding tube would have to be inserted directly into his little pink tummy. Suitably alarmed Jill and I set to coaxing Oliver to eat. We took shifts and stayed bedside with him 24 hours a day, teasing a tiny bottle past his pouty lips, disapproving gums and uncooperative tongue. At least Jill and I could trade off shifts, grabbing some sleep in hospital. Well I thought, it could always be worse.
And then suddenly it was.
Jill got very sick with a particularly nasty feverish flu, and was promptly banned from the hospital. She was exhausted and now forcibly separated from her child, a horrible position to be in. I was now on double duty at the hospital cycling back home every few hours to pick up breast milk and report on news and bring food to a distraught Jill. It was exhausting, but at least I had the sanctuary of my apartment to crash out in for a few hours now and then. And Oliver was starting to tentatively eat. Well I thought, it could always be worse.
And then suddenly it was.
Post diluvium
My upstairs neighbor, a fabulous guy with dreadful plumbing, lent his apartment to a couple from the UK who left a bath tap running. The first clue something was up was gallons of water suddenly pouring through my bedroom ceiling. My guess is that someone prayed to a Greek deity (as I said, the prayers have been eclectic) and it got routed to Poseidon whose answer to every problem involves water – its really not his fault; polytheocracy breeds domain specialization. If all you have is a hammer, every problem looks like a nail; but we'll get to Thor in a moment. Poseidon's bailiwick is of course all things aquatic, in this case waterfalls of Niagraean proportions. The water poured through light fixtures, out of switches, and down the walls. Paint started peeling off the ceiling and walls in massive clumps and in a little less than an hour my apartment became unlivable with the rotting smell of mould everywhere (yes that's the British spelling of mold, sorry some things just look better with U, plus when it comes to dark dank unlivable conditions, the British know a thing or too about that).
After beating a hasty retreat I called the landlord, who sent around Leo, a lanky 50 year old problem fixer with a moisture meter and a sledgehammer. Leo assured me it was fixable. 'Leave it with me' he said. 'You focus on the baby' I biked off to the hospital with the latest supply of warm breast milk turning my place over to Leo and his extensive experience. "Help yourself to the beer in the fridge" I said. Well I thought, it could always be worse.
And then suddenly it was.
When I got back to the apartment Leo had punched huge holes in the ceilings and walls of the different rooms ostensibly to assess damage and start the drying process. But Leo was more enthusiastic than prudent with his sledgehammer; Thor had decided to make an appearance after all. Leo/Thor (anag Hole Rot, go figure) neglected to put sheets over any of the furnishings or even mask the holes he was making, he had just stood in the middle of my apartment, a beer in one hand swinging his hammer with the other. Now most of my 1930s apartment was covered in a lung clogging dust that looked suspiciously like asbestos. Elsewhere the asbestos/dust had mixed with the brown mildewy water to form a rancid cement that adhered to musical instruments, bedding, furniture, clothes. "it's the worst case of damage I've ever seen in a non-carpeted place" he said nodding at the damage while helping himself to another beer from my fridge, "I've been doing this for 25 years". I couldn't help but reflect that in 25 years Leo/Thor must have destroyed more people's apartments than Hurricane Katrina. But all I said was "I think you've effectively fireproofed this place with all this asbestos". Before I could stop him he said "well it could always be worse" Right at that moment the wall that held the bracket that held my beautiful, mountain bike, gave way and wall, bracket and bike crashed to the floor. "Cheers" I said, and grabbed a beer.
Home again home again
Back at the hospital things were looking up. Even though Oliver was not chowing the full amount every three hours the doctors decided that he was essentially a well baby and should go home. Jill was now recovered sufficiently so we went together to the hospital to pick up Oliver. I had a nagging feeling that they had made a terrible mistake putting us in charge of a baby, shouldn't we know more? All they showed us was a video explaining baby CPR in which the first confusing step was to call out to your child "baby baby, are you OK?" But it was never explained what was considered a satisfactory response from a two week old baby.
So after two long weeks we finally walked out the hospital with our kid. Much to my amazement no alarms went off and no one chased after us demanding we return The Vole to a place where there were professionals. What sort of irresponsible behavior was this? Did they truly let everyone just waltz home with their baby armed with nothing more than a receiving blanket, diapers and rash cream. It was clearly madness bordering on negligence to put a baby in our charge but with any old lunatic allowed to raise a kid it did start to explain the messed up state of the world. Hopefully we could better than Britney.
We went back to Jill's apartment as they had now moved in a dozen fans and dehumidifiers into my apartment – the noise and moisture making my place feel like the main runway at Miami airport in July. I checked messages, and there were still none from the hospital demanding our return. "Well I'm glad that's over" I said "We won't be going back there for a while". I'm almost glad I didn't know how wrong I was going to be – otherwise I would have just broken down and cried right there.
The next few days were perfect. Jill and Oliver and I all curled up, not really sleeping, not really waking with a stream of visitors bringing well wishes and food and gifts for the boy. Day three at home we realized we were getting a little cabin fever, so I hoisted Oliver over my shoulder, a position he still seems to favor, and we headed for our local noodle house. Oliver had spent most of his life in a hospital so hearing the clatter of the kitchen calmed him – reminded him of the preceding two weeks chaos. To this day he is happier being up and out and about in places where there is noise and activity.
Beyond the Pale
At the end of the first week out, Oliver's third on the planet, we took him to the pediatrician for a standard check up. Our doctor wasn't there, so we got the stand-in med student, Tai. "He's kind of pale" we said. Looking at me Tai said "It looks like he got the Irish side of the family tree". "Can we get a blood count?" we asked. I asked this not only because he seemed Goth white, but also because his blood counts at the hospital had been kind of low. "No, we'll do that in a couple of weeks" she said. It seemed wrong, like quite a bit of the contradictory medical advice we had received to date, but once again we demurred. Once again we were wrong to.
I had a quick business trip the following week to New Jersey. I got on the plane on Tuesday, and when I got off 6 hours later my voicemail was filled with messages from Jill. She and Oliver were back in the ER. Jill had decided to take O in to the pediatrician to check on some goop that was in his eye that didn't seem to be clearing. Our main doctor, the fantastic Emily, took one look at him and ignoring the minor eye problem ordered immediate blood work while Jill waited. Oliver's red counts were so low the lab didn't believe it and redid the tests; technically Oliver should have been in a near coma. The shocking test results were confirmed, and Jill and Oliver were rushed back to the Intensive Care Nursery for an emergency infusion. I joined them the next day.
It turns out that Oliver suffers from a very rare, but very profound anemia. The major suspect, still not proven, is Diamond Blackfan Anemia, named after the two doctors that uncovered it. It is genetic, RPS-19 is implicated (whatever that means). Its rare about 50 babies a year get it, there are about 1000 people worldwide with the disease. There is no known cure. Oliver does not make red blood cells or rather he makes them but somehow they are on an early death cycle, so none make it into the blood stream. As such, to stay alive, Oliver needs to be transfused every three weeks or so. Each transfusion takes a day as they do a specialized match to make sure there are no blood reactions, and then they have to pump the red cells in slowly so as not to overstress his heart.
Whose line is it anyway?
About two months into his life Oliver had a very hard day when the doctors could not find a vein that they could use for the transfusion. A combination of a healthily fat baby, narrow blood starved veins, and Olivers small size meant that he had a miserable day where a series of half a dozen doctors each stuck four or five needles into him trying to hit a vein. Finally we begged the doctors to put a permanent intravenous line into Oliver to stop the incessant puncturing which was wildly distressing him, and us. The doctors placed a Broviac line which is a central line that has an entry point into the middle of his chest and runs up into the major veins in his neck. The good news is that the Broviac means an end to the heel sticks that they used every week to draw blood from his feet. Transfusions are also now painless. The bad news is that The Vole is no longer water proof, as he has a hole in his chest, so can not take an immersive bath, joining a long illustrious line of famously stinky Irish men (Dedalus claimed he took a bath once a month, whether he needed to or not, Bob Geldoff isn't much better). It's a shame as we found early on The Vole really likes thrashing around in water.
The biggest challenge is the dressing around the chest hole needs changing once a week. Friends know that I am more enthusiastic than precise when eating, and with Oliver the apple hasn't fallen far from the tree: as the applesauce can attest. Trying to keep a site sterile when its constantly being covered in food and saliva (Oliver's not mine, I hasten to add) is hard. When the site gets irritated Oliver always figures out how to get hold of the line and give it frustrated pull, but so far the line has held up amazingly well. The wound dressing is complex with at least half a dozen components to make it work, but Jill has figured it out and can now dress the site better than most of the nurses that come by to check on him.
There have been a few issues with the line nearly getting infected which have landed us back in hospital, one of which grounded us for a week of emergency antibiotic infusions. But generally we are getting into the swing of flushing his line once a day and changing the dressing weekly; although both our apartments look like a scene out of MASH as there are boxes of syringes, medicine vials, ointments and wound care patches everwhere.
The video montage bit where we go from week four to month seven
And so the months have been roaring by, he is now seven months old. And Oliver has been growing well, he's now over 15 and half pounds. The motor deficiencies that we were sternly warned about manifest themselves in a slight shake in his hands, but he is one strong and determined kid. The physical therapists who work with him repeatedly shake their head when they look at his progress and then at the medical charts. "It doesn't seem possible", they say admiringly, watching as he grunts and groans onto his knees and does a good imitation of starting to crawl. "If anything he's somewhat advanced, we need to slow him down". He may yet be a bit gimpy in his gait, but it seems almost assured that he will find a way to propel himself around. Its fortunate that he has a will of iron, even if he has relentlessly non-ferrous blood production.
We had an early on interaction with the neurologist who asked if we had noticed anything wrong with Oliver.
We said, "there's the anemia."
He said, "well other than the anemia."
"There's the limb stiffness", we ventured
He said, "other than the anemia and the limb stiffness."
"Well there's the reflux; he throws up quite a bit of food."
He said "Other than the anemia, the limb stiffness and the reflux, anything else wrong?"
"Well there's the tongue that seems to curl a bit, we're worried about speech and eating"
"OK other than the anemia, the limb stiffness, the reflux, the tongue curl, anything else?"
"Oh well other than that he's a perfect specimen!" we said somewhat exasperated.
Then he said ""Well its just that I think Oliver might be blind"
"Argghgghghghghghgh!!!!!!!!!!!"
Like much else we've been told by the doctors this turned out to be wrong. When he chooses to Oliver focuses in on people with a laser like intensity. He can coordinate picking up objects and most importantly, at least to him, putting them in his mouth. Cognitively its still hard to assess his level of processing, but he his babbling in a concerted manner, shows interest in his surroundings, loves his toys, is crazy about dogs and has a wonderful funny laugh that will melt your heart.
If you met this baby in the street you would be struck with what a beautiful, incredibly strong and alert boy he is, you would not guess at his history. You might also think, hey what's this baby doing in the street, is this one of Britney's kids?
Jill spends almost every waking moment time managing his trips to be transfused, participating in his physical therapy work, working with the neurologists, doing medical research on his conditions and running him to the pediatrician and the assorted specialists who are involved. Oliver is beyond a full-time job and Jill has taken it on cheerfully and is a fierce devoted advocate for her son. He owes a lot of his progress to her unflagging enthusiasm, faith and skill.
And my neighbor made a very generous donation that helped get the apartment painted and back to its former shape. Sadly that paint has once again peeled, and there are some ongoing remnants of the flood, but my place is now more livable for me than for mould, which wasn't the case for quite a long time.
Check Please
Between the emergency room visits, the transfusions, the birthing - Oliver is now a million dollar baby. And it is almost entirely picked up by Californias Medicaid program, Medi-Cal. We pay for some additional physical therapy work that we believe is helpful, but the state has picked up the tab for the MRIs, the blood, the ongoing PT work. We would be beyond bankrupt without this support. I keep waiting for the other shoe to drop and to get some astronomic bill in the post, so I make sure the car is stocked with a tank full of gas, a trunk full of diapers and a map to Mexico that avoids the major freeways. But so far so good – the Feds have not come knocking. I've been paying taxes for what seems like forever, and I'm generally peeved that I'm simply helping buy bombs for the military or paying for ineffective road repairs. Now I realize that a little of the money goes to helping the surprising number of babies that are in need. So I don't feel so bad about paying taxes any more. I want to especially thank those of you who are American tax payers for doing your part in keeping Oliver alive. Without your help, we couldn't do this – I hope that thought helps a bit come April.
To Infinity. And Beyond!
So whats next? The first thing is to confirm that this anemia is Diamond Blackfan. Oliver had a bone marrow biopsy a couple of weeks ago. Apparently the results are not due back for six months. I tried to point out that on CSI they can do genetic testing in less than an hour, including six commercial breaks and they always get their man. The hospital was not impressed. Apparently isolating the genes is time consuming and expensive. And given the rarity of the disease, lots of people all over the country and even the world want to do their own bit of double helix dicing and splicing on our boy. So six months it is.
Meanwhile Oliver will get transfused every three weeks or so until he is one year old. At that point they will give him small doses of steroids – although no one is quite sure how the drugs work on this disease. If you saw how strong this kid already is and have watched The Hulk you'll know that that giving him steroids seems to be asking for trouble. There's a 50/50 chance they'll work, and about a ten percent chance he'll get huge and green and run through San Francisco and have to be brought down by helicopters. If the steroids fail and he ducks the helicopters, we'll go back to transfusions. One day we may consider a bone marrow transplant, although transplants are seen as very risky/expensive/painful so are currently being down played as an option.
So what is Oliver's prognosis? Unfortunately not great. Life expectancy for DBA kids is somewhere between twenty to forty years old. It turns out that all those transfusions are hard on the body. The very iron you need turns out to slowly kill your organs when you get it transfused, which if you can't resist puns and play fast and loose with definitions (yeah I'm talking to you Alanis Morrisette), might be described as iron-ic. Other risk factors include increased incidence of leukemia and cancer as the mixing of the bloods mess up your chemistry and to add insult to injury the disease also precludes chemo from being an option for cancer treatment.
When the going gets tough, the tough get philosophical
There are a few reasons to be cheerful here. First and most importantly the medical community have been very wrong on this boy several times before, so the racing certainty is that this prognosis is wrong too. Second, the technology is getting better every day and who knows what they will be able to cure in twenty years, twenty years ago it seems they were practically using leeches. And third with such a small sample of people with this disease (the DBA registry is 420 kids) no one really knows much about it - the chance of being an outlier is pretty good. Of course that could break either way, but this kid is pretty determined so I'd bet he'll be on the right side of the bell curve.
But having said that I don't want define Oliver's happiness and success in life on him beating the not inconsiderable odds. We all know it's the quality of the life not the length of it that makes it worth living. Sometimes we forget, but I'm lucky to have Oliver there to remind me about what's important. I hope that in whatever time Oliver has with us he will pack a lot of living in.
People are of course the most important element in any life and I've been extremely lucky to find amazing and exceptional people. Because you are getting this overly long note you are one of those exceptional people and a friend of mine and so in Oliver's life. (nb and if you didn't get this note either directly or in the first tranche, please don't take umbrage, you are still in the gang. My address book is not quite as complete as it could be, and my systems aren't set up for large email sends - my email admin ability is no reflection on friendship. This is also to say, if you know someone who didn't get this note and you know should have, feel free to pass the message along). My exceptional friends are the largest gift I have for The Vole. Thank you for being part of that treasure. Some of you I see regularly, some of you I haven't seen for a long while but I hope that our paths will cross again and you will have a chance to say hi, and visit with him – even if it's for a little while. Your presence in his life, even those who are remote, gives Oliver a very rich life. I also hope that I can encourage Oliver to live fearlessly and to follow whatever dreams he may have. It is always important to appreciate each day, quadrupley so, when you have, perhaps, four times fewer of them than most of us. I'm hoping I can provide a life for him where he will have the opportunity to travel a little and see this wondrous and diverse world that we live in. I know that some of you have donated to a fund for him to help him grasp whatever opportunities might come his way. Thank you for enabling his life this way. All you need is love. But a little cash certainly helps, I think John if not Yoko would agree.
All of that is of course the future, and unwritten. For right now Jill and I have our hands full with our beautiful, willful, unique child. Of course neither of us anticipated the severity of issues he would have, but even with that we feel blessed. He's a cool kid, a blast to be around, a little dynamo of seemingly endless energy that needs almost no sleep to fully recharge. Every day he does something that surprises us, amazes us, delights us or scares the bejesus out of us. And we wouldn't trade that for the world. He is family and loved fiercely and absolutely.
Run on the Bank
I would ask that if you would like to help, that at some point, if you are able, you give a pint of blood to your local blood bank. Oliver would be dead by now if not for the selfless blood donations of others. These angels we cannot thank directly because we don't know who they are, but they make possible each day with our son.
We are taxing the system mightily as we continue to consume a considerable amount of blood. I would love to know that we were helping replace the blood we take so that other parents can also have the opportunity to watch their children grow, so that others aren't deprived by our urgent ongoing incessant need. I have to confess, the benefit of blood drives always seemed abstract to me. I knew they were a good thing to do, but where does the blood go, is it really that useful? When you meet Oliver you will see the benefit is both immediate and vital.
So in brief…
A picture is worth a thousand words, at this point we are nearly five pictures in – thank you for your patience. If you would like to keep up with Oliver in a more concise manner and see what is going on in his life, please check in at www.flickr.com/photos/georgecor
And so that's it for now. You know as much as we do. Hug your loved ones. And hug yourself too. Its easy to take for granted this thing called life. When you see how precarious it can be you realize how precious it is. You are precious and loved and your presence here is nothing short of a miracle. Celebrate that. I know I do.
Much love from all of us here.
George, Jill and of course Oliver Valentine.
Ps late breaking news, through a generous gift OliverValentine now has his own domain at www.OliverValentine.com. Right now there is just a link to Flickr, but we'll link other info into that as we get it.
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