Monday, October 21, 2013

Hey there, hospital, it's been a while, so I guess we should visit :(

It never gets easier. I would have just as soon skipped the blog, but realized that if I was crying like a baby at some youtube clips linked on FB, then in the spirit of full disclosure, I should 'fess up.

Spoiler: Bo is doing great and his last day of antibiotics is tomorrow.

But emotionally, we are all a little out of practice, and still a bit on edge.

The school year started without the drama of a line infection, like last year. But unlike last year, hospital policy had changed. So (on the Monday before last) when he started acting a bit grumpier than usual, and complained of pain (which he NEVER does), I immediately assumed the worst. Hooray for catastrophic thinking. Being too casual caused us to miss severe dehydration in 2010 (we thought it was maladjustment to the baby) but not last fall (the new GI thought we were overreacting; it was a line infection- we were clearly correct). So even though his temperature was not very high (it never went above 100'F last fall), I knew it was a very real possibility.

The home health nurse drove in to draw blood cultures, which were run at our local hospital. All the doctors were looped in that night. The next morning (last Tuesday) when he spiked a 102'F temperature, we were off running. The cultures had already started growing, and the machine was awoken. We started packing and a room was prepared while the little one was in her morning preschool. I had to drive into work that afternoon to send a package via FedEx, as well as run a meeting the following morning. JVC spent those 2 days with both kids in-patient. His mom came to provide relief (AMEN). And friends, DVDs (via childlife), art therapy and gifts helped to break up the rest of the week. Bo was home by Friday night, and we started planning for his scheduled clinic visit across the state less than a week later.

The weekend and the early part of the week back from the hospital was an attempt at normalizing, but then we were packed and away from home for another 5 days. And even though the antibiotics schedule was blessedly humane (one dose every 8 hours), all the time away from home was wearing on all of us. This was especially telling when the little one, who only experienced extra treats and company declared on Saturday that she wanted to go home.

The nephrology team is exceptional. However, during a spell this summer, the new senior fellow was left in charge of Bo's case, as the chief was traveling to Asia as a keynote speaker and the pharmacist was on vacation. Typically, they are required to comment on the laboratory testing performed monthly on Bo's blood sample to ensure his medicine is balanced to meet his health needs. When the fellow (presumably overwhelmed and/or shirking) did not call the GI team with comments, I called him myself. It was not a pleasant exchange. I do not tolerate sub-par care for my child. Thankfully, we had an uneventful clinic visit. The chief continues to provide exceptional care. And I'm pretty sure the senior fellow will not soon forget this lesson.

This is the 2nd line infection he's had while at home, ever (aka central line associated blood stream infections CLABSI or catheter-related blood stream infections CRBSI).

Tuesday, August 6, 2013

midsummer's night dream

It seems that we've arrived on the shores of Valhalla after we finally gave up looking. Bo has made it through the hottest part of the summer without requiring hospitalization, and Ahn hasn't fallen on her head. So I call that a win.

I've still got a lot to learn in my current position (so I'm still not bored = good), and I'm still trying to decide how to best upend the applecart (for my midlife crisis). An obnoxious car seems too obvious (and too tacky), and really, not enough pain would be inflicted with this terminal project. We'll see.

Bo starts full-days of school in a few weeks, and Ahn will start preschool. I'm up way too late. And our old cars need more work than I'm ready to admit to anyone but my mechanic.

Nite, all!

Sunday, July 21, 2013

Microvillous Inclusion Disease Network

We had our first annual meeting at the Oley Conference on the Cape this year. A third of the North American families met as a subcommittee (read: grillin and chillin), which is ironic considering none of our MID kids actually eat. These evenings gave me a shot in the arm, and a conviction that these kids may have challenges, but that doesn't mean they are sick.

I love Oley. Just seeing the teenagers slouching around and being teenagers with their backpacks and curly tubing, infusing through all their drama, makes me tear up. As I follow the papers coming out and the new clinical trials being planned for regenerative medicine for the small intestines and gene therapy for single gene mutations, I know there are legions of men and women working to make our kids lives safer. And I truly believe this is something we will see in our lifetime.

Faith, hope and love.

Saturday, June 22, 2013


It came as no surprise to me that Bo's last day of kindergarten would be special. An award-winning journalist sat in the front row, recording hours of audio footage. She walked home with us and spent another hour talking to both Bo and me (see/hear the link above).

What surprised me, strangely, was realizing how many of my friends, family and colleagues tuned into the naturally running public radio programming. And the commensurate outpouring of acknowledgement was a tremendous boost. I'm pretty sure my heart grew three sizes too big.

As we prepare to join the big, annual family reunion for a family that no one hopes to join, I'm excited for Bo. I'm deliriously happy that he is old enough to see and meet and make friends with other people with the same medical technology and the same (or higher functioning, in the case of adults and older teens) level of understanding. Not that his condition or his brilliance will ever be normalized, but that he feels this community wrapping its arms around him, and us, in a way that our dear and loving typical community cannot.

I get misty looking at Bo, not just because he is an incredible guy in his most absolute essence, but because his BEING is a result of the love from all these communities, intimate and professional, typical and affected, local and international. He is running and singing, inventing and drawing, reading and sleeping peacefully because the many gestures of many hands seen and unseen are working around the clock for him.

I'm with the band.

Wednesday, June 12, 2013

The Natural Order

Things in Bo's world are right. They follow a natural order. You turn 6, get lots of LEGOs, finish kindergarten and have your baby sister idolize you.

For us, all that is expected from a 6 year old are all things that we consider novel (for a first child) and miraculous (for a child saddled with Microvillous Inclusion disease). But we've had a proper Spring (as opposed to Texas-style scorchers), a hospital-free school year and a successfully scaled-back birthday party.

Lucky for Ahn, she is so intent on doing everything that Bo does that even though we only read to her a fraction of the time we read to Bo at that age, she loves and craves reading, too. Everything he likes and does, she likes and does: reading, building LEGOs, trucks, and science. She is so convinced of his pure awesomeness that when we had to do a dressing change after their bath last night, she insisted she get a dressing, too.

Since we had an extra from the kit (which has adhesive that Bo is allergic to), I pretended to clean a spot on her chest and applied the Tegaderm. At first, I tried to distract her, then remove her from the room, but she was so insistant that she too get a dressing change. It was the only way to keep her from disturbing the sterile field around Bo. When both kids were done, and freshly dressed, Ahnnie's only complaint was that she wanted me to "paint" her belly, too. We only have so many betadine swabs, that due to the iodine, stain the skin yellow. It was hard convincing her that skipping that step was ok. It was even harder convincing her that we were not, in fact, going to "hook-up" fluids to her invisible central line. There was the briefest moment of grief; I silently sent up a prayer to all our friends who lost siblings to chronic illnesses, and prayed that our children would never know that day.

Tomorrow, Bo will go to his surgeon's office to have his line repaired. Please pray that it is as smooth and uneventful a procedure as we know it can be (it's literally a cut-paste job). And send a small prayer that neither child freaks out, and that both cooperate with JVC (he will have our home health nurse to help with Bo, but that means there will be that many more people crowded into the exam room).

Tuesday, May 28, 2013

Who We Are Not

We are not to be pitied. We are not freaks. Along the same lines, we don't have mo'better superpowers, we were simply given the privilege of using them with laserlike focus sooner than our peer-set. We were totally NOT prepared for this (might add that I passed for a pretty good facsimile of a Chinese-American meangirl, high grades, competitive, and with a whiff of shallow/sheltered).

More importantly, we do not take anything for granted (basic healthcare, complicated healthcare (like REALLY complicated), family dinners [yes, every day], modern appliances because potty training makes these things really count, decent wages, interesting work, kind co-workers, unkind co-workers (including the very weird- yes! I appreciate you!), beautiful seasons, sweet blooming cherry trees, the hardware store with everything in it, the 24-hour donut shop that is SO good it's even in Zagats (yes, it's in Kalamazoo!).

In the next month, Bo will turn 6 and graduate from kindergarten, Ahn will turn 3, we will all go to Oley and he will find kids his age and ability also on TPN, my human study will have some sort of data that will determine where the project is going, we might have some better idea what to do about Bo's stalled out growth, and we will have an entire summer to enjoy the succulent, humid, bursting summer together.

I remain more committed than ever to an unapologetically messy house. The clothes are crumpled in laundry baskets (at least they're clean), the piles of books threaten to decapitate toes (people love to read, yay!), library books are long overdue (I'm underwriting their 2013 budget). But it's OK! Why? Because we're doing more important things everyday: we have dinner together, we read books to each other for bedtime, we walk to see the gnomes (they live in a giant knot in the tree around the corner), we inspect the ants/roly-poly's/millipedes in the yard, we talk about simple machines (ramps and pulleys are 2 current favorites), we sing songs (both real and imagined), we are copycats (well, Ahn has to do everything that Bo does), we cuddle, we throw, we run, we dance, we ring-around-the-rosie, we red light/green light, we play the pee-nano (piano), we play the dumps (the drums), we eat superman ice cream, we do homework, we pick fruit from the farmers market, we pick plants from the garden center, we visit our friends before they have surgery, we watch videos of Weird Al, we take our shoes off, we help others put shoes on, we fill our backpacks and we empty mommy's purse.

May each season be uneventful, may each day's disappointment and joy be woven into a shimmering fabric that punctuates our memories with both the beauty and brutality of a life fully lived, may the complexity of our experiences be a barrier to our hearts' door closing to any in need, may our friends always be warmed by the hearth of our true soul's compassion, may we always find the courage to turn to another for the strength we may not have in the moment, may our dearest one's companions remind us who we once were, what we dreamed and who we are becoming, may we continue to hold ourselves and one another accountable for cherishing each other into a better tomorrow, today.

Sunday, April 28, 2013

I regret being well-rested, said no mother, ever

And yet, here I am. Surfing, listening to the shushing of the cycling baby monitor, up waaay past my bedtime. There are 6 loads of laundry waiting to be folded (the sitter folded a grand total of ONE), and all sorts of administrative things I could be doing (last year's taxes, oh... and the year before's), besides the most pressing of all (sleep).

Although Bo has stopped growing (no one knows why, but we are going to find out, dammit!), as in not a centimeter in many months, and the constant IV drug shortages are nerve-wracking, the truth is, the kid is full of vinegar (as in, up at 6:30am, jumping on my bed and demanding we "watch my jig!"). And the other truth is, while all that may be contributing to my poor sleep hygiene, I would most likely be ding-ding/dong-dong late into the night, anyway. Also, the drug of biggest concern has returned to the market just in time (BIG sigh). Although, who knows which one will be in shortage next?

We had our first glorious first real day of spring. After a breakfast of donuts and coffee (NOT for them! they had milk), we spent the entire morning outside, goofing around on the lawn, cleaning up the lawn and doing a little tidying up. We ducked inside for a quick lunch, and maintenance of the marathon laundry (see above) event for me, while the kids had an hour of quiet time. We went BACK out to the playground, got a hurried dinner and shuffled my fresh-smelling littles into their fluffy beds.

My best friend from my last office stopped by, my guy is driving home from a great gig, and I even got to drop off the plastic bag recycling at the store (in my attempt to be doing enough). And the nerdiest thing that has filled me with glee is discovering that the service spits out a report of ALL the prescription meds for each of us in whatever timeframe I request from ALL the pharmacies we use, so I never have to chase BPA-filled receipts to file my FSA claim forms, ever again. The close runner-up of nerd-alert? I just realized that the bottomless pit of cardboard boxes from Bo's medical supplies can be used to contain all our yardwaste for the city's monthly brush pick-up! Laziness score + recycling avoidance (the time and effort required to breakdown boxes for recycling is almost insulting) + freecycle avoidance + lawnwaste/kraftbag frugality savings $$!!

So, a red-letter day, full of sun, fresh air, and newly discovered efficiencies. Corny jokes, and made-up song lyrics to nursery rhymes. Goldfish crackers and secret gnomes (there's a small village in a huge knot in the tree around the corner from the house, which Ahn demands to "sthee gnomz?!" like 8 times a day). Lush green grass, crisp evenings, hot tea. The next 8 months always make up for the last 2 (because January and February are beloved because of their winteryness; whereas March and April are ALWAYS that way, and scorned for it). Maybe will put up a tree swing!

Maybe I'll read my bookclub book. Night, all!

Tuesday, April 16, 2013

Waiting to see what my Senators are REALLY made of

Just sent both senators modifications of the below. If you want to help (or stop my incessant whining), you can write your senators, too. Tell them that my whining keeps you up at night. That me and my kid are (or are like) family. That we matter. And that a sucky painful and slow death will just make everything suckier.

I know people are freaking out about the bombings. But see, that wasn't preventable. IV drug shortages? Those are kinda preventable. And what a lot of folks don't realize is that LOTS of people will be impacted when the well truly runs dry (even, for instance, all those injured in the Boston attack; tiny babies in the NICU, their families; anyone who gets in a car-crash and needs IV supplementation; cancer patients who need the same; you see where this is going).

So you have something to crib from, this is what I wrote. When I'm feeling ultra-creative, I will try and post a letter you can just copy and paste and not have to edit one bit. Until then, let this be your muse:

Dear Senator:

I left a voicemail on your office phone a few weeks ago regarding my dismay over the IV drug shortages. Your office hands were kind enough to remit a letter acknowledging receipt of said message, and declared you would need further details.

Here they are: 
I live in xxxxxxxxxxx with my family of 4. I am the primary wage-earner, and a native Michigander. I have 2 advanced degrees and choose to live in Michigan. 

My son has an ultra-rare (there are fewer than a dozen living in the world) disease (microvillous inclusion disease) whereby he absorbs nothing through his gastrointestinal tract. He is alive by receiving all his fluid and nutrition requirements intravenously. His infusion company has been heroic in their efforts to meet his nutritional needs through many of the shortages, until now. 

The current IV phosphate shortages have almost stymied us. There are only 2 products with this as their salt: potassium phosphate (also known as "K-phos") and sodium phosphate. There are only 2 FDA-approved manufacturers of this product (as many other companies made the business decision in the '90's up till now, to exit this business for not generating any profit): American Regent (Luitpold), and Hospira.

Apparently Hospira's enormous 483 compelled them to fire their CEO and hire one who promised to fix it. They poured $125MM into it (probably a bunch of charlatan "consultants"), and got reinspected by FDA. They received a scathing additional 483, the new hotshot CEO was replaced, and we are still in critical shortage situation.

When I say critical, what I mean is, my son already has bones that are not as dense as they should be. He is FIVE years old. He should not be getting osteoperosis. But without phosphorous, the calcium has nothing to hang onto, and then the bones fall apart. His bones will start dissolving, little by little. He will start to get fractures. Many, small hairline fractures. His growth will stop. He will be in chronic and unquenchable pain. He will be in a wheelchair. At some point, if this goes on for long enough, this bone disease will progress beyond fixing, even if this product comes back online to full volume of supply.

I need your help. I don't know what you can do. Maybe help us source from Europe? Maybe help these companies get compliant? Maybe send a few bucks to FDA to get these places reinspected sooner? I really am at a loss, which is saying a lot, considering I trained as a mircrobiologist academically and spent my first 3 years in industry working to get a big pharmaceutical company out of its sterile injectables' 483.

To see how far we have come with my child's health only to face this long, dark tunnel of uncertainty lined with threats of suffering and pain seems excruciatingly cruel. UMich doctors told us he would not live to see his 1st birthday without a multimillion dollar multi-visceral organ transplant. And that without that, he definitely would not live to see 5. He has never been transplanted. He has not died, yet. His 6th birthday is this coming June. He goes to the public school around the corner. He reads at a 6th grade level. He loves to do robot dances. And antagonize his 2 year old sister. He loves to find science books at the library. And play board games with his nurses.

Please help my son. 

Respectfully yours,

PS I have voted at every election, no matter where I was living, and always voted for you at every opportunity. I'm only slightly pandering (you can see why), but honestly, it's because I believe that you are truly putting Michigan's best interests first.

Friday, April 12, 2013

It's a Nailbiter

For most of my youth and young adult life I felt like a girl waiting for a cause. With the energy and drive of ten men, I could have been the next Buddha, given the right initiative. Sadly, I was a bit, um, emotionally underdeveloped, and fortunately for me, I was privileged enough to have very little troubling my mind (thank you  to the Women's Rights and Civil Rights activists who made my laconic teenage years possible).

Right now, the IV drug shortages are threatening Bo's health. If we cannot get enough IV phoshphates and if the oral pills (which technically shouldn't work, but appear to be helping, but whose helping is nearly impossible to quantify because he doesn't absorb like a typical kid...) they want to admit him to the hospital. Theoretically, they would maintain a supply when infusion companies have run out. Except that the shortages are so severe, our hospital has also run out.

This is a big deal because his bones are already not as dense as they should be. Missing phosphorous would turn his underdense bones into brittle twigs. It would suck and be very painful.

So write your senator. Because no matter what the infusion companies, pharmacies or even doctors do, it's the voting citizen that the senators' lackeys are counting when they are deciding on legislation that needs more teeth/enforcement.

Other than that, just another day in paradise (40 degrees, grey and rainy with a high of 39 tomorrow).

Friday, March 8, 2013

Singing Off Key

My mother did not sing to me very often, that I recall. What I do remember was the one lovely lullabye in her childhood tongue, sung, certainly wobbly with emotion, and a bit off-key. I tend to sing it a bit the same way; my register doesn't reach as high as my throat used to be able to reach. And each slightly sharp-ish note makes me a little maudlin, and in-love.

I love the feeling of music coming from ME. Resonating through my chest, my daughters skull and into the very fiber of her being. Shakey notes and all. It reminds me of singing lullabyes, all manner of nursery rhymes and made up verses to Old MacDonald to Bo, in an effort to coax him to sleep.

This invariably brings more tears. But I am so happy. Because now, he pretty much goes to sleep and stays asleep until morning. And he's just sleeping, and not in THE BIG SLEEP.

In fact, he is so stable, that instead of burning my adrenals out wondering if my kid will still be alive in the morning, I sleep, too! And then I wake up stupid early. Because I actually can convince myself to do so. And I work my tail off at work. And one day a week, I work for free at the hospital for half a day. And Thursdays, as long and grueling as they are, can only in the smallest way, show the nurses and doctors who saved Bo's life that their ho-hum long shift jobs are so much more than that to me. That they are magical beings whose every touch were the difference, literally between life and death.

And I wonder if they know that there was another person whose life they saved during those long summer days in 2007? That this Lazarus had laid down for so many years that it seemed permanent. But one day, after a long time, the faintest flutter became, after many more days and weeks, an eruption of life being lived.

And today, I came home to help Bo with homework. I took the hair ties off of a stuffed caterpillar's antenae. I listened patiently to my favorite scientist delight in the surprise of his own brilliance. I sang about rainbows to my daughter and I read about germs to my son. And after the house grew silent in its contentment, I dutifully popped open my laptop to finish some work that the VP wanted.

It has been a very long week. Full of hard work, discipline, tedious chores, bad manners, tax preparations (which are not complete, still!) and laundry. But it is an exhaustion that I am so grateful for.

Tuesday, January 1, 2013

2013 Year of the Black Water Snake

The dark waters and stealthy energy of the snake are upon us. I cannot wait for the surprises this year will bring, I can only offer this: a long and patient welcome. With the planning and organizing that years of formal education and corporate employment has trained into instinct, I will bring my best preparedness to the table.

And I'm pretty sure that once seated, it becomes clear that we are not at table. But maybe a zeppelin in outer space. Or in a castle of unicorns. Nonetheless, the preparations will serve me well, I'm sure, in the least expected dimension.

There are some years that I wasn't sad to say goodbye to, or were so entrenched in mental-emotional fog that I remember little. 2012 was a beautiful year for me: I got my brain back. We will remember it fondly. And 2013 will be its own exciting adventure.