Breakin the Law, Breakin the Law

I'm generally not so much a rule breaker in that rebellious eff-you everyone sucks kind of way, I'm a rule breaker in a "I'm here to meet Hans Clevers, he's giving a talk at noon, "meet the pioneer;" I'm meeting the pioneer" kind of way. So I crashed the largest scientific conference in the world, to meet the biggest name in Cancer, the guy who is going to cure MID. With a baby on my back, a four year old holding my hand, my bewildered spouse, and no official identification or admission, we walked through the gate, past the Pfizer booth, down the cavernous carpeted concrete exhibit hall, and up to the lecture hall with 4 security coats trailing us, worrying about insurance liability, children under the age of 12, admission, identification verification, etc. I am pretty sure a host of angels (or jedis?) stood their ground with us, "these are not the unregistered and underaged children you are looking for." We met Prof Dr Clevers for the 15 minutes between his arrival at the hall and the MC's shepherding him to his seat on the dias. I think this really was the only time I've been star-struck, and the only time I've legitimately stalked someone. HC: How did you find out about my work? Me: I've been following your work since you discovered the intestinal stem cell Lgr5 (3 years ago). I read your Tokyo group's paper on colonic stem cell transplantation. It's amazing that all you have to do is shoot the stem cells up in an enema... HC: and they stay there. It's been eight months already, and they are still there. Me: But how can you get cells into the small intestine, past the ICV? HC: easy; an NG tube that goes all the way through the small intestine with perforations along the way. Me: stunned. It was 15 minutes, and enough hope to last a lifetime. I finally get what groupies are all about. It is connecting to your dream incarnate, to hope and love and a dream come true. We may never meet again. We don't know when they will start clinical trials in children. Those first trials will probably use immunosuppressant protocols like those for surgical whole-organ transplantation. The second generation (if they get there, with funding and longevity) will be the one with self-cells genetically transformed. And who knows when of if THAT will ever happen. But whatever is driving this venture capitalist, MD/PhD, the new Chair of the Dutch Royal Academy of Science, I believe he will press on until this thing is cured. All I can offer is Hope. All I can be is Hope. All I can trust in is Hope. There is no world without end, without Hope. There is no world worth living in. There is no future. There is only Hope. You think that something that is broken cannot be re-broken, especially if it has never been fixed. You can't imagine that something like a broken heart can get more broken, because the pain is unfathomable. And you really can't foresee something as small as a newborn and as full of hope as a baby would be the instigator of that excruciating pain of a re-broken heart; the one that was never fixed. I think the worst year of my life was the first year of my second child's life, because all the wonderful moments were sharply barbed reminders of the exquisite pain and deprivation I didn't know I was missing with my first, affected child. My mind was blown that year. My daughter was beautiful, exuberant, voracious. She rarely cried, she ate with gusto, she smile so hard her dimples popped and her eyes disappeared. I loved her deliriously, I was so full sorrow. Somehow, the resumption of delirium, disorientation, fatigue and pain simply jettisoned my being into outer space. I was catatonic with grief. And then, I began my descent back to earth, the thaw, the flash, the burning ozone refined the sting and suddenly, I was back on our home planet. With a new human, a new job, a new life. I did not expect that. I had heard secondhand that a second child was healing, after having a child with special needs. And it sounded so vague, and veiled. I couldn't have guessed why. I couldn't have guessed that the healing required a break so sharp and searing that my whole being would be blown miles into the sky, into outer space, to freeze and thaw, to orbit and crash, to burn-up in a flame so hot it turned white. But I did, and it did, and I am. Back on earth, grateful for the ride, stunned by the views, happy to be home again. And as always, I write to you because I love you. Because you might be looking for me to tell you that there is Hope. Because this isn't my blog, about my feelings for my feelings, but because it is for you to find, because you are looking to see what will happen when you find out your child or nephew or grandson has MID. We are always here, steadfast. We will always Hope. We don't know what is coming next, we never expected the world to blow up and then get made whole again. There are diseases so rare that prevalence and incidence cannot be estimated (MID), there are unnamed syndromes, there are things that cannot be fixed, but might have work arounds, there are things that aren't fatal but profoundly affect quality of life. There are the affected and those who love them. There are caretakers and those who love THEM. This blog is Bo's blog. It is a light in the darkness for anyone looking. It is hope, it is information, it is armor, it is love. My story is incidental, I am only a supporting player, a narrator. I'm just telling the story of my son, because he can't (well, at least not at the technical level). If you have questions, need help, want recommendations, want to shout out, send us love or give a high-five (or fist-bump if you're from Chicago), never hesitate to do so.

My turn

Bo was struggling with extreme thirst. We were worried that he was getting dehydrated. But, his labs turned out perfect. It was excruciating telling him that we would not give him any more water. For every sip he took, he spit up twice that. The risk of dehydration was getting very real. I cried, he cried. We were all very distressed. The pediatricians were nervous about changing anything. The GI was nervous because of his history of kidney stones. But finally, they agreed to let us administer a bolus of saline in the middle of the day. They took out 200cc from his TPN and shifted that volume into the day time bolus. And bingo, the thirst is no longer an issue, or it was back to normal. He is growing taller. I had to let out the hems for several pairs of pants. And it amazed me. I've been in bed all day with the stomach bug that Ahn had. Bo somehow didn't get it (thank God!), but the rest of us three were laid out. Although I have some time to update, I'm feeling so crummy myself that this is really as much as I can do.

Loving the Gemini

They are both Gemini's. They both love life. Everyone they meet is a friend. They find the person looking back at themselves in the mirror delightful. They are Gemini's, I realized with a startle, because they each have a duality. The living, breathing child that is warm to the touch, and a delight to see. And then there is their other. The child who almost was not. And so, when I see them, hold them, feed them, scold them, pick up and put down for bed/time outs/car seat adjustments/high chairs, I remember this. I often wonder if I'm simply morbid, or a grief monger. But preparing them (me?) for the future, the unknown, the likely disappointments, grief, loss and pain to come requires arming them with joy, compassion and love today. That means giving them structure, assuring they have enough sleep, trying to acclimate their palates to good/whole foods. Because one day, when their twin looms, or mine, or that of someone they love, I want them to be prepared. I want to be prepared. On the one hand, US culture and society wants us/me to pretend and strive for eternal youth. But my child is young, and has a much higher probability of death than most of us. On the other hand, Bo is fairly stable, and I certainly don't want to point out the shadow of his twin, looming over my head. And that is my reality. It is real. Death. His. Very real. More real than mine. And then, there's Life. Very much alive. Tantrum-ing. Reading. Hamming it up for the baby's giggles. While every day may be one day closer to the twin. I also know that every day is a victory. Every day is a gift.

Merry Christmas and Happy Hannukah! Since the last update, Bo's line has required several applications of alteplace (TpA), and finally an ethanol lock (0.5cc 70% ethanol instilled for 4 hours, and subsequently drawn out) to clear what appeared to be an occlusion caused by his lipid. My theory is that the fibrin (gunky protein that is part of the clotting factors in your blood) formed a hairy network in and around the end of his line, and that the lipids stuck to them causing a 2-ingredient clot, requiring two anti-clotting procedures. Somewhere in there, his line broke (due to the occlusion and repeatedly built up pressure) and the surgeon was able to splice a new end neatly, and without much ado. Baby Ahn is toddling and pointing. She continues to appear typical, including her language (resistant, as she prefers sign language, pointing, and general brattiness). She has started to use her words with prompting, started potty training, and reading. I still don't understand why Omegaven hasn't been approved by the FDA. The company that makes it actually has taken some leadership of this, but apparently, not significantly more than before. There have been days when I wondered what I would be doing if Bo was not here. I wonder when and what I should say to Bo when he asks about his line. He asked me when Ahn was getting hers. I explained that she was not going to get one. I had to choke back tears during this exchange. It made me wonder what I should say to Ahn, when things do not go as planned for Bo. I speculate about all the ways they will become broken, and all the ways I can prevent that from happening. In some ways, it is a relief to think that we will bear the suffering of the world long after he has gone, and he won't ever have to face pain and death without me. But obviously, there is little comfort in any of those scenarios. So for today, I'll pursue all those things imagined in the "after" meditation, so Bo can see, in THIS life, how much his endurance has inspired me. PS I don't know why, but none of my paragraph breaks are showing up and I'm too lazy to troubleshoot this right now.

Thanksgiving every day!

Bo used to be such a light sleeper that we were prisoners in our own home. I did not put laundry away for literally years. A squeak of the floor, the rattle of a dresser drawer handle, the light in the hallway; all of could lead to a blood-curling scream that might take an hour to quell. He was not only a light sleeper, but an ANGRY waker. It strikes me as funny tonight, as I am banging around, the baby sleeping peacefully several feet away with nothing between us but a wooden door, Bo getting ready for bed, humming the Star Wars theme song, loudly; how easy it is to give up the niceties you imagine as non-negotiable. And how nice it is to have the luxury of those niceties back. Speaking of which, there has been some drama in the transition to new job, and specifically with Bo's line. Yes, his line broke this morning. No, he's not in the hospital. Yes it was scary and sad. No, Bo did not cry (although Ahn did, because in their haste to get it fixed, they forgot to bring babyfood- so she was MAD). Yes, my new insurance covers all the medical stuff as my old. BUT no, the nursing coverage is a mere fraction of that. Yes, we are all nervous for the transition. No we have no idea how this is really gonna work. Yes, I still giggle at work, because I still like it that well. No, my cubicle is not any less dreary (although the live plants are a treat that I have not seen in YEARS, so I guess that makes up for the threadbare carpet). Yes, for most people the amount and quality of care Bo has had is unimaginably amazing. So no, I'm not looking a gift horse in the mouth, just sayin that we got used to the gift. But also, yes, I'm finding the gift of enjoying my job has increased my energy level by a billion, so I'm a lot more helpful with the littles. So, he's not dead, yet. YAY us. BUT, these things still send waves of terrifying panic over all of us. And the fallout can weirdly last a way longer time than I expect, because in the immediacy of the situation, we all just do what has to be done in as calm a manner as possible. And when every thing has calmed down, when you feel like your life is out of immediate danger? That's when you fall apart. So, just so you know. You know?

Shana Tov!

Orientation at the new job. First exam of the semester. Nerves. My cubicle is smaller and drab. It's somewhat depressing to see in its bare state. That is not why I am there. And as long as I can keep that thought at the top of my head, I can enjoy the blessing that it is. My sister posted a gauntlet she laid down, to scare yourself, pushing past your comfort zone, once a month. She is doing it! I am doing it! It is scary!!!

ch-ch-ch-ch-cherry BOMB

So I dropped the Bomb on Tuesday and packed it all in today, Friday. I get a week off (paid), that's good. I spent my whole industrial career there, so now I feel very confused, that's bad. We are covered by my old employers' insurance until my new one kicks in, that's good. It's through COBRA, that's not all bad, but I have no idea how much that will cost, so that's a little bad. I don't have to move or make big changes that will traumatize the kids or Bo's medical state, that's good. It requires a commute, that's bad. I have no gaps in employment, that's really really really good. I'm feeling very insecure from all this change, that's bad. Leaving was gonna happen, and I would be facing a change no matter what, so on the balance, this is all good. This kind of seismic change is terrifying considering the responsibilities I have towards Bo's health and wellness, and being this freaked-out can only be bad. But hopefully it's short-lived, and that's good. On the balance: it's all good. God is good.