Piercing the Darkness: Developments in Optic Nerve Regeneration research

Today we leave the gathering darkness behind and welcome the return of the Light:

Happy Solstice, Beloveds!

Zoe is blind, that we know, and at any other time prior to this point it would be safe to say that she would be blind for life. But with technology and scientific advances happening almost as fast as they can imagine it, there is a chance that Zoe may get to see some day.

I had heard of people traveling to China to receive stem cell therapy for optic nerve regeneration, but not only are the treatments prohibitively expensive (at least $20K per treatment, and they recommend multiple treatments), but the results they have are limited. It all seems a bit too experimental and unregulated to me to even consider considering it.

Closer to home I discovered the Schepens Eye Research Institute online and made use of their "patient liaison", Richard Godfrey, to get educated on what they know about developments in optic nerve regeneration. The following is some text extracted from literature he sent me.

Regenerative Research:

As our knowledge of stem cells has expanded, so has the breadth of our research. A few short years ago, all the focus was on embryonic stem cells and research was limited by the ethical issues and related federal funding ban. Since then, we also have access to progenitor cells, adult stem cells that also have great potential without some of the limiting factors of embryonic stem cells mentioned above. Now, we have discovered that the human body has already existing, dormant stem cells in the central nervous system (CNS) which includes the spinal cord, brain, optic nerve and retina. With both embryonic stem cells and adult stem cells, additional research needs to be done to both develop consistent sources of stem cells and develop a surgical or other stem cell delivery technique. This new approach of activating already existing, dormant stem cells may eliminate the need for these added steps. In addition to knowing that these cells exist, we also now know the molecules that are responsible for that dormancy and we have identified the drug that can activate them. This drug then is likely to become the foundation of a new drug that will both activate and coax these cells to become the kind of cells needed in our goal to restore vision.

In optic nerve regeneration, we know that there are three barriers against regeneration we need to overcome in order to develop a therapy. We already know that the original drug will overcome the first barrier (dormancy) and the second barrier (scar), but the third barrier still must be overcome. This barrier comes from the proteins of the myelin (sheath covering the optic nerve) that send signals preventing regrowth. So the goal is, using the original drug as a foundation, build a new drug to overcome all the barriers safely and reliably.

At the Schepens Eye Research Institute, an affiliate of Harvard Medical School located in Boston, our regenerative center, The Ocular Regeneration Research Center, is made up of 3 separate laboratory teams exploring the great potential of stem cell and gene therapy technologies. The goal of all the labs is the repair/regeneration of the retina and optic nerve. Both are made up of nerve cells, which share the same barriers to regeneration that we are working to overcome. The real strength of Schepens' regenerative research is these 3 labs, 3 research teams, with varied approaches, working collaboratively, are making the idea of vision a reality in coming years. It is a very powerful combination.

Some of the group's individual accomplishments include:

• Dr.Feng Chen's first ever regeneration of the optic nerve in mice

• Dr. Michael Young's years of transplanting brain and retinal stems cells into mice and pigs in preparation for the next step, human retinal damage repair/regeneration

• Dr. Kameran Lashkari's novel, new discovery of adult progenitor cells from the retinas of premature babies that seem to migrate to damaged optic nerve and retina

• Dr. Chen's discovery of the mechanism to reawaken already existing, dormant stem cells in the retina, optic nerve and brain

• Dr. Young's discovery of the molecule in stem cells that is the key to integrating transplanted nerve cells into damaged tissue.

Well, that all sounds promising, eh? But it's not only optic nerve regeneration that might allow Zoe to see in the future. Technology may light up the darkness for her.

The Brainport Device is an amazing doo-dad in development that allows blind people to actually experience vision in the brain via the nerves on the tongue.

Say what?

The article (linked above) can explain the workings of the device better than I can, but remember that it's not really the eyes that see, it's the brain that sees--the eyes just give it the information it needs to experience vision. The Brainport device simply gives the brain the information it needs through different nerves. It's all very heady stuff, but I am just so impressed with the brain that thought of this whole concept in the first place.

For those of you who'd like to see the unit in action, check this video out:



(And, speaking of videos--check the YouTube link for about five recent video uploads of Zoe stuff.)

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On the home front, we're still all doing really well--so nice to be redundant on that subject!

We took Zoe in for a year-post-surgery-check-up with Michael Cunningham (always a pleasure to see him), and he was well satisfied with Zoe's progress. Recent CT scans showed definite signs of bone remodeling in her skull, as well as a gradual evening out of her bumpy-headed appearance. He felt like we shouldn't even consider the idea of a follow up surgery until Zoe's about 5 years old (as opposed to the usual 3 years of age for most craniosynostosis patients); he just felt like she's been through too much already and that another procedure so soon could 'break her".

We're fine with that approach.

Zoe also had a check-in with her transplant team at SCCA with accolades on that front as well. The extensive labs they drew showed that she "has an immune system" and that it is more robust than most patients this soon after transplant. To be sure, we still have to take care not to expose her to sickness as she is immunocompromised compared to the average person, but good news nonetheless!

Zoe is thriving on the whole food purees we are feeding her fresh from the Vitamix through her G tube. Although certain to be filed under "To Much Information", her poops are finally normal for the first time in a year, making it lovely for her not to have to suffer awful, chronic diaper rash (and for us not to have to deal with diarrhea all day long). Although concocted to meet supposedly all of her nutritional needs, the formula she was on for 9 months simply wasn't agreeing with her as much as this whole food mix we're making. Go figure.

Though she's entirely tube fed, Zoe's recently discovered that she absolutely LOVES crackers. Not to eat, but to suck and lick and taste. Her official first word is "Keh-Keh" (cracker) and she asks for them all the time. I mean, incessantly. First thing when she wakes up (at 5am) in the morning, and pretty much all day until she goes to bed. Crackers have supplanted the brief mommy obsession she had--which is both a relief and a "Hey.....! Really...?"


Really.

Jeff and I are good, so good. To be sure we're dealing a bit with cabin fever, but that's a sickness we are happy to manage. I'm back to learning Braille after a long hiatus; Jeff's teaching himself Beatles tunes on the piano. This Christmas is very low-key--no presents, no decorations, no fuss. We will spend time with family both from in and out of town, so the gathering of the Newton-Cormey-Rapp-Nielsen-Bell-Connor clans will be a good (and loud) time.

Zoe will add to it with her screams no doubt--she's been perfecting them and I'm looking forward to showing them off. Oh, and her cuteness, of course.

Abundant Gratitude

Wow. Here it is. The most meaningful Thanksgiving of my life thus far. My heart bursts with gratitude that this hell we have toured, this valley of shadow we've traversed has opened up to such a glorious mountain top.

Our girl lives! She thrives.

I'm grateful, so grateful.

Thank you for sharing this journey with us, for sending comforting and supportive emails and comments, for lifting us up, for visiting, for holding us as we cried, for rallying the troops, for bringing food, for grocery shopping, for sending money, for encouraging me to write when I've been silent---thank you for loving us.

I'm grateful for my rock of a husband who is the strongest man I know. Ever present, ever nurturing. What a lucky, lucky woman I am.

I'm grateful for SCCA, for Seattle Children's Hospital and all of the wonderful people who work there, for transplant technology, for modern medicine, for living in Seattle where we had access to world class health care, for chemotherapy (and I NEVER thought I'd say that), for very, very smart people.

And mostly, I am grateful, so deeply thankful for an anonymous woman who gave of her very genetic substance to an unknown baby girl in the knowledge that she could possibly save a life.

I'm grateful for her social conscience that had her sign up for the registry, for her sense of responsibility that had her step forward when identified as a match, and for her tenacity to follow through with a procedure that wasn't very pleasant--all in the spirit of possibly being able to make a difference.

My heart catches every time I think of it; the magnitude of her gift is so overwhelming.


Yippee! This crazy existence we call Life is such a ride--and it is far from over yet. But at this scenic overlook we've paused at to catch our breath and stretch our legs it's looking pretty damn gorgeous.

E ticket rides don't have nothin' on this.

Not GVHD!

Doctor check-in today gave us the good news that Zoe's rash was not GVHD. Its responsiveness (even though it took several days) to the Benadryl indicates that the rash was likely a delayed reaction to something--some medication probably--having to do with her G Tube surgery.

Hoo-ray!

Also in the Good News Department, Zoe's liver enzymes were normal for the first time since her transplant.

Yay!

She fell asleep on the couch tonight at 5pm, which means she'll likely be up at 3am.

Boo!

But I'm not the one who is on Zoe duty tomorrow morning--

Yipee! :-)




Look, Ma-- No Tube!

Rash?

Haven't posted an update about the rash because I don't really know what to say about it. It's diminished some--actually more than some, but she still scratches herself quite a bit. We'll know more on Wednesday when we go in for her regularly scheduled check-up.

Other than that, Zoe's doing well and pretty well recovered from the surgery. Tonight was a two barfer, so the bedding and jammies had to be changed twice, but in general it seems like maybe she's barfing less than with the NG tube.

I learned that Vita-Mix will sell discounted demo models (lightly used motor, new pitcher, full seven year warranty) to people with medical conditions that would benefit from the use of their blenders. G Tubes definitely apply.

Keep forgetting to take pictures of her without all the nasty tape on her face now that the NG tube's gone--will post some as soon as I coordinate memory with camera and kid. It's a challenging proposition as my brain just doesn't seem to want to do higher functioning these days.

In related news....what was I saying?

Back home

Zoe was discharged yesterday from the hospital--everything seemed to be back to normal with her.

This morning I arose at 5am to find an itchy red rash covering much of Zoe's torso.

Could this be it? The appearance of the dreaded GVHD?
(cue the villain entry theme)
Dum, da-dum, dum!

A visit to the doc today for labs and a skin scan. Manley (today's attending) thought that the skin rash did look similar to GVHD, but wanted to rule out a delayed reaction to the antibiotics administered during the G Tube placement surgery. He had us dose her with Benadryl with the intent of seeing if any improvement might happen. Marked improvement would mean the rash was likely not GVHD based.

So, there was some improvement over the next few hours, but some rash still remains. We check in with Manley tomorrow to find out whether her tacrolimus (immunosuppresent/anti-GVHD drug) levels are therapeutic and to discuss the state of her rash.

It's quite possible that, if it is GVHD, it was triggered by the inflammation response she had to the surgery. The fever she had was the first one she's gotten since her transplant. It very well may have gotten her new T cells hot and bothered and on a tear to attack. This is how GVHD can get going--and why they want us to keep Zoe from getting sick for at least the first year after transplant; her new T cells, when activated by inflammation or viruses, can get confused as to what is the enemy and decide that Zoe (or her skin, or liver, or GI tract, or any and all of it) must be attacked.

It must not be a common reaction to G Tube placement surgery, or else I'm sure they would have mentioned it.

If they determine it to be GVHD then Zoe would be put on a course of prednisone for treatment.

Ugh. Steroids. "Roid" rage. Weeee! Here we go again

--or, maybe not. We may not know for certain for a few more days.....but I tend to like to prepare myself.

By the way...

Finally posted a recent video of Zoe--check the YouTube link for a shot of Cute!

G Tube Placement

Zoe went in on Monday for her G Tube placement surgery. The procedure took only 15 minutes, but recovery is still ongoing.

We were only supposed to stay one night in the hospital with her, but due to her stronger-than-usual reaction she's there for a second night tonight.

She spiked a fever and had bad enough pain that oxycodone and Tylenol did not cut it for her; only morphine seemed to do anything. Her gut function appeared to just halt for a good 24 hours after the procedure in reaction, which made getting her back to her formula feeds impossible for longer than expected.

This all said, she's doing well.

Imaging showed that her G Tube was placed correctly and that the inside of her stomach looks pink and healthy. Tonight is my night to stay home--and Jeff just called to tell me that our energetic and happy girl has made a reappearance. Guts finally seem to be moving, meaning they can go forward with getting her back on her feed schedule.

Had a little feeling that it wasn't going to be quite as smooth as they said it would be. Zoe's just living up to her namesake and continuing to keep us in the here and now.

Blessed tyke.

General Update

Wow. Lookie at that--no posts for two months! As I've mentioned before, no posts undoubtedly mean good news maintains in ZENland. Of course there's plenty to talk about, but the longer I go without posting about it, the more I have to write, and then the less I'm motivated to blog it all out. Enough of you have sent me interweb pant kicks, so I guess I have to put down those bon-bons, roll off the couch, and get to work...

A month ago we took Zoe in for her 6 month check-up post-shunt placement for a CT scan to see how her brain was doing. Every CT image since her transplant has shown an alarmingly large right ventricle--even up to 3 months after the shunt was placed.

This time the CT revealed a very slimmed-down right ventricle to the relief of all. It got me wondering about the brain and developmental movement as it relates to Zoe's experience.

As you know, Zoe is quite delayed in her gross motor skills ability. At 21 months of age she is not yet crawling when most of her peers are accomplished runners, climbers and tumblers. When placed on the floor, for the longest time she would only lie mostly still, on her back, with her movements limited to kicking.

In the month leading up to the CT scan it seemed that all of the sudden Zoe was motivated to do more than just lie and kick. She started to get up on hands and knees for moments at a time, push her self backwards with her hands while on her stomach, and scoot herself forward with her legs while on her back.

With the news of her ventricle shrinking down to almost normal size I wondered if it correlated with Zoe's increased motor activity. The study of developmental movement in babies has shown connection between certain physical movements and the stimulation of brain development in infants. Did Zoe's increased activity help to heal her brain, or did the shrinking of her ventricle help promote her gross motor skills? Hard to say, but it's an interesting question.

As such, she's still just on the cusp of crawling. It's clear that she's muscularly not as strong as her compatriots, as she can only sustain weight on her arms in the crawl position for a few seconds at a time. She keeps trying though. Maybe by her second birthday she'll finally be a crawler. Then I suppose we really will have to baby-proof our house for the first time. Such a novelty!

Plans are in place to remove Zoe's NG tube and place a G tube directly through the abdominal wall into the stomach. We had a satisfying experience when Zoe had to go in for a torso X-Ray in the pre-G tube placement exam. The doc who came in to read her films looked a bit confused and asked, "What is your daughter's condition??" Jeff replied, "Osteopetrosis." The doctor said with further confusion, "But her bones look fine!" A light came on and he continued, "Ah! But she had a bone marrow transplant!"

Guess we know for certain now that it worked :-)

So the G tube gets placed this next Monday, October 26th. It will be a minimally invasive surgery that will require Zoe to stay only one night in the hospital. She'll overnight in the oncology unit where she resided during her BMT -- a precaution taken due to the fact that she is still immunocompromised. Apparently Seattle Children's is currently crawling with kids with H1N1 already, so I'm happy for the immunity-friendly accomodations. Also, it will feel like old home week getting to see all of our favorite nurses again (and yes, even some of the overly-perky ones).

Despite her delays, Zoe is doing her best to be a properly mischievous almost two year old. She's teething currently, so finds it endlessly entertaining to try and use my fingers as teething tools. When she manages to catch me unawares and bite me HARD, my yelp of pain only makes her chortle delightedly and try even harder to lead my fingers to her mouth. I've told her many times that she should bite her own fingers; she's game, but not quite as entertained by that prospect.

She's a dedicated escape artist; we've started to call her Zodini. She takes her right arm out of every outfit we put her in and shoots it out of the neck hole so that she sports the currently fashionable one shouldered look. Any pants that we put on her promptly come off (even the ones we put on her under the onsies eventually get taken off), and those that she can't take off soon have both of her legs in one pant leg, leaving her flopping her conjoined limbs like a little mermaid.

She would have us play her special Zoe music over the stereo all day long if we could stand it. I am chagrined to say that she's as opinionated about music as I am--anything that is not "her" music is marked by wails of protest. Introducing new songs to her is a process --but I'm determined because I have just about had it up to here with "Baby Beluga".

Zoe's been on a jaunt for the last couple months where she's decided that 3- 3:30 AM is a perfect time for getting up. Believe me, we've tried letting her stay in her crib, but an hour of her kicking the headboard and bellowing loudly just on the other side of the wall from us convinces us eventually that there's no going back to sleep. So, we've gotten into a routine of taking turns doing the early shift (the one who has to work the coming day gets to sleep in). On my mornings I take her to the living room, put her in her little room with plenty of toys, turn on her music, and doze fitfully on the couch. I think that's pretty much what Jeff does on his mornings too, but I've never gotten up to check.

Zoe's starting to show signs that she's cycling out of that monstrous phase--she's been waking up a little later: 5am two days ago, 6am yesterday, 4am today (my day to get up--dang!).

As for Jeff and I, I have to say that we are doing and feeling so much better. It's been quite a radical shift, actually. We've been exercising vigorously on an average of 5 days a week for the last 4 months. In addition to that we took a yoga/meditation class about a month ago that taught us a practice we are doing twice a day now. The class was just what we needed to help lift us out of our rut of feeling victimized by what we've been through with Zoe, and has given us a practice which helps us to center ourselves on a regular basis. I highly recommend the class to those of you drawn to this sort of thing--it's offered around the world in different cities, is not religion-based --and will inject some serious juice in your life.

We are getting ready to hunker down for the upcoming cold and flu season. My cousin Caren just called me today to tell me that 15% of her son's school is out with what looks like H1N1. It's not a virus that is looking as dangerous as they feared, but it's seriously unfriendly to young children, especially immunocompromised ones. This may be a long, lonely winter of Netflix and internet surfing for our little family as we try to avoid any of us getting sick.

Wish us luck.

Anniversary of sorts

Today I realized that it's been exactly one year since Zoe's diagnosis. A year ago, these very minutes I was writing my first blog post, reeling from the news we had just been handed hours before.

What a crazy, crazy year it's been since then.

And~

Zoe is here, she's alive, and she's thriving.

I had no sense of this outcome, no mother's intuition about how it would turn out. In the months after her diagnosis, but before her transplant, I would look at her sometimes and know that she was going to make it. But just as often I would look at her, heart aching, seeing the funeral with all her favorite music playing. Those visions just knocked me flat.

It was quite a head space to occupy for a good while there--we've only really became convinced of her sticking around with us for awhile yet in the last few months.

Jeff and I have grown and changed and stretched and broken and grown some more, but mostly we still feel like we're treading water (however, with less anxiety and thrashing about). I wish I could say that I've grown into a more spiritually advanced person, a more patient person, or a more gracious person for the experience, but mostly I just feel more tired and more easily irritated. I hope this won't always be the case though. I think that as Zoe progresses (and sleeps longer!) I may finally get back to my old, less irritated self.

Zoe continues to be the powerhouse of vitality and regeneration that she is. She had her Hickman line taken out on the 11th as scheduled, and it's been pretty smooth sailing since then. She's had a couple pokes for blood draws and only fussed a bit.

She seems so close to crawling finally--she's rolling from front to back, back to front, scooting on her back, reaching beyond her immediate circle of comfortable arm's reach. She finally seems motivated to explore beyond her immediate vicinity. As such, she's finally graduated from being able to lie on the couch without supervision, to having to be placed on the floor so that she won't roll off; a milestone most babies achieve by the 4th or 5th month. So, she's close to 20 months, but all good things..., right?

She's been strong enough for all of this physical work for a couple months now, but she didn't seem interested in moving beyond the comfort of lying on her back all day. I had started to describe her as "not gross motor skills delayed, but motivationally delayed". Motivation is finally kicking in in spades.

Yesterday we took her in for her check up with Dr. Carpenter at the SCCA. All was pleasure at her progress. There had been a concern arising last week as her lab reports had shown that one of her liver enzyme levels had shot up to quite high levels (not good). Only two things can make a post transplant's liver enzymes shoot up: adverse drug effects, or GVHD.

Her pediatrician had us hold one of her drugs for the week to see if this week's lab reports improved. They did, significantly (yay!)--but Carpenter said it could have been a coincidence, "hard to say." He didn't want to start any taper of her steroids or immunosuppression drugs even though she's been doing so well. His reasoning is that she had an unrelated donor's peripheral blood stem cell transplant--and the risk of GVHD is very significant with such.

Best not to rush to rock the boat.

The plan is to wait until Zoe stabilizes from the placement of her G-tube. G-tube? Newest development.

Currently Zoe has an NG-tube (nasal gastric) that goes through her nose, down her esophagus into her stomach for the purpose of feeding her and administering meds. A G-tube is simply a gastric tube that is surgically inserted through her abdominal wall directly to her stomach. A cap keeps it closed while not in use and the whole thing hides under her clothing. Without the NG tube there is no longer a risk to her pulling it out and aspirating the formula, there is no need for tape to be perpetually on her face, she no longer has a visible statement to all who look at her that something is "wrong" with her, and she will no longer have a tube dangling down her throat. This last part is possibly the best perk: without a tube in her throat she may feel more inclined to actually start swallowing food again.

It means surgery though, and another appliance sticking out of her, when we had just gotten rid of the central line. It does bring a greater risk of infection as well. However, the risk is not as strong as the Hickman risk was, and we did pretty well with that. I talked to a couple people who said that the G-Tube is great--one of them a mother of a small child who had one put in after a long time with an NG-tube. She said it was a God-send.

So, once the G-tube is placed and Zoe seems to be stable and business as usual after that, THEN Carpenter said he will look at tapering Zoe's meds.

Something out there is determined that I will learn patience. Eventually.

Zoe on the Main Line

Big exciting news in the ZEN universe is that Zoe's hickman line will be removed on August 11th.

I've been itching for it to come out; its usefulness has been waning with the growing gaps between blood draws. And when I think on it too much, I cringe with the thought that these dangling tubes Zoe constantly play with tap into a vein that goes directly to her heart.

I thought it was Dr. Carpenter's call to determine its removal date, but Zoe's pediatrician said that it was within her jurisdiction as well, and agreed that its removal would be a good thing since its benefits no longer outweighed the risks.

Zoe could have gotten it removed sooner than August 11th, but lines are only removed on Tuesdays--and only two per Tuesday at that. So, she's got the first available slot, which puts her on August 11th.

When her Hickman goes away, so will the daily line flushes, the twice daily blood thinner injections, the weekly dressing changes, and the line protection protocol prior to bath time (the inconvenience of which has unfortunately rendered Zoe rarely bathed).

Hot diggity dog.
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Zoe has started to try and imitate words said to her. It's a particularly interesting process since she can't watch us and see how we are forming the words with our mouths.

Her ability to imitate relies on her understanding how sounds we make relate to all of the various babbling sounds she makes on a regular basis. A plastic "pitcher" handed to her went through the rapid name evolution of "dada" to "dida" to "geetcha". It all happened through a quick succession of my stating the word and Zoe attempting to say it more correctly with each effort.

I remember as a child not hearing certain words in detail. I would call a signal a "sigunal" and not be able to understand why my mother kept telling me I was saying it wrong; I couldn't hear the difference between the two pronunciations. I think about this and imagine that Zoe has more of a limit to what she can do verbally since she can't see my mouth form the words. Mama is "Nana"--and no amount of prolonging the "m" gets her to understand that there is a difference between the two words.

Humorously enough, "nana" is also how she says "nose".

Yep, that's me--Nana with the nana. My nose follows me everywhere...
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This evening was quite the unique experience for us: we went to Seattle Children's Hospital as visitors for the very first time.

Yesterday afternoon I got a call from an old high school friend, Ryan, that I had reconnected with at the 10 year reunion. Ryan lives with his wife and 8 week old baby girl in Kalispell, Montana. The call yesterday was hurried and bewildered; Ryan's wife and daughter were to be flown ASAP to Seattle Children's as they had just ascertained that Ava, his daughter, had a rather serious heart defect that needed to be fixed right away.

Ryan called me within hours of the diagnosis and I was transported to that place, last August 26th, when our lives as parents got pushed over the precipice--just as theirs were in free fall at that very moment.

His voice, his shock and the sound of a psyche in overdrive were all too familiar. They were coming here--and by god, did we know how to support them!

All of the love and care that you all have shown us have been waiting for opportunities to be paid forward, and walking up to the Giraffe entrance with dinner for our friends felt like a sacred honor that you all have passed on through us.

Thank you.

P.Y.T.

According to Zoe's oncologist at SCCA, "she's ahead of the curve" as far as how well she's doing at this point past her transplant. She still seems to have no sign of chronic GVHD showing up--especially remarkable given that she had a peripheral blood stem cell transplant (which result in the highest rate of GVHD occurrence).

Our appointment--a monthly check-in--was three Tuesdays ago (see, my timeliness is definitely slipping) at SCCA.

It was so good to see the jaw drop of the PA Natalie when she walked in and saw Zoe for the first time since halfway through Zoe's hospital stay. Last Natalie had seen was Zoe with a distended abdomen, a refractory platelet problem, acute skin GVHD, and a swollen head. To say that Zoe was an altogether new baby in Natalie's eyes is an understatement; watching Natalie's face full of wonder as she gave a boisterous Zoe her exam was potent medicine for Jeff and I.

I guess we can really believe that we have a Wonder Baby.

As well as she is doing, Dr. Carpenter (the oncologist) declared that he didn't want to shake anything up by changing her meds. He did say that she's well enough to stretch her next check-in out to two months; he said he'd see about the appropriateness of backing her off the steroids then.

According to Dr. Carpenter, the steroids are at a minimal dose as it is. A good indicator of this is the fact that Zoe does not really have that steroidal puffiness that people get when they are on a larger dose. Certainly she is chunky, probably chunkier than she would be if she weren't on the steroids, but she doesn't have the jowls and the abdomen of people on higher doses.

That was good to hear.

We've been slowly introducing Zoe's gut to the concept of "bolus feeds" which is where we give her a "large" dose (70ml) of her formula and give her a break from her continuous feeds for an hour and a half afterward. The idea is to eventually get her able to take 120mls of formula four times throughout the day. At the point that this is possible, Zoe will not be needing to be on a continuous feed drip 24hrs a day.

Definitely a worthy thing to work towards, but until we get there this new regimen has us fiddling with either meds or a formula bolus every two hours from 8 am to 8pm (with another med push at midnight).

I'm used to it by now. On my days with Zoe I don't get out much since going anywhere means prepping for at least an hour (and whoops, gotta get another med push in--and, there goes her diaper!).

Jeff and I are nursing machines.

And hopefully soon we will be well toned (let alone honed) nursing machines. Jeff ordered a workout DVD series, advertised on late night infomercials, known as P90X. We have been doing push ups and chin ups and crazy-difficult exercises for the last two weeks that have made us extremely sore.

We figure, we're already exhausted--why not be exhausted for physical reasons? I don't think I've ever exercised at this intensity in my lifetime--but talk to me in a few more weeks; two weeks ain't long enough to brag about...

Here are a couple pics of Zoe working with her PT Sarah--who is helping Zoe become comfortable with putting weight on her feet.




Check YouTube in the next couple days to see some videos of Zoe working with her PT and life skills educator...

1.5 Birthday!

Short post--I have been working on a much longer one for what's been waaaaay too long. But my eyelids are slipping down on my efforts once again, so I wanted to get a quick one in to say:

Zoe's 18 months today!

What a love and joy she is --and such a little ham. Tonight I went into her room 20 minutes after I had put her down for bed to do some fiddling with her feed pump. She was lying quietly with eyes closed, blankie held next to her face, and breathing slowly and steadily.

Without warning she whipped the blankie down, opened her eyes and yelled, "Baaaaaahhh!" with a big grin on her face. It surprised me so much that I started to laugh hysterically (I know, I know--you're never supposed to laugh at behavior you don't want to encourage...). She was quite pleased with herself--and I was immensely impressed with this obvious comedic genius we are raising.

I uploaded a couple more videos to the YouTube channel--one of Zoe playing a piano duet with me, and the other of Zoe showing Jeff the proper way to follow an exercise video.

These two pics are of Zoe in her walker--we're trying to help her understand that standing is "fun"! She's not really buying it...

Day ????

My "weekly" posts are being stretched further apart with each posting now. I believe I have hit the two week mark for the first time in awhile. I've gone long enough that I've lost count of the days post transplant.

It's definitely a sign of good things: with less trauma and excitement I feel that I have less to report.

Zoe just thrives, that's the gist of it all.

She's massively delayed for her age, but for HER, for Zoe--she is amazing. She's a master at rolling over from front to back. She's an accomplished sitter, and she's slowly learning that supporting her body weight using her feet and legs while being held upright can be a fun thing. Zoe jabbers and laughs all day long--interspersed with healthy amounts of whining of course--filling our house with happy baby sounds.

We are blessed.

That isn't to say that we are not still suffering from too much sleep deprivation, nursing fatigue, doctor and therapy appointment overload, and accompanying mood challenges, because we are.

But, I'll take it all because it's all part of what has allowed us much more time with this amazing little girl.




This last photo is a picture of Zoe during one of her sessions with the speech therapist whose other specialty is helping kids learn how to eat. Although the photo makes it look promising with the scooper of food in her mouth, Zoe still refuses to have much to do with food.

Check YouTube for eating therapy vids...

Day 135: The Dark Side of the Coin

Zoe is doing great--just some issue with a diaper rash from hell and some accompanying diarrhea (aren't you glad you asked?). I placed her on her tummy today with her bottom bare to give it an airing--eventually I glanced over to find her on her back. That's the second time in a little over a week she's rolled over on her own. At seventeen months old it's not very precocious for her age, but for Zoe it's perfect--and we couldn't be more thrilled.
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I've been dragging my feet in writing about this next bit, but here it is:

Remember the UK family I once mentioned who has a girl, Alishba, just a few months older than Zoe with osteopetrosis as well? I don't know if I mentioned that within days of her diagnosis almost a year ago her mother found out that she was pregnant again.

That child, another girl, was diagnosed in utero with OP as well. Simrah was born March 10th this year, and started her transplant proceedings a month and a half later.

Simrah contracted an influenza virus a few weeks ago during her transplant process, was on a ventilator for several days, and so tragically and unfortunately was not able to handle the stress of it all and passed away on May 25th.

Her family, understandably, is in shock and grieving. I think of her mother and can barely hold the comprehension of her pain in losing a child so soon after giving birth it.

There it is. Right there. The other side of this crazy coin toss that we have experienced.

I am in communication with Alishba and Simrah's twin aunts, and to them I sent this email that better expresses what I can't seem to access now while I write of this:

Oh Loves,

I'm just sick for you. There is no getting around the pain you all have to go through.

I don't know if this brings any comfort at all, but when we were on the other side of the transplant process, being told that Zoe had a 50% chance of survival, we had a couple talks with a Zen Buddhist monk who counseled us.

He told us that this whole experience was about Life and Death. That there was no softening that fact.

Life.
Death.

He said that Zoe was still so close to her state of Being before Life, that if she had to go, it would be a familiar and comforting place for her. It would not be a scary Unknown--for her.

Does not really make it easier for us--for you, the family that is left grieving Simrah's retreat back to the Beyond she so recently emerged from. It's not fair, it is horribly painful and beyond ghastly and tragic to think of the path leading up to her departure.

It is a shock to see how horribly wrong this process can go. It does reduce me to tears whenever I think of your little angel--and it's nothing compared to what you all are going through. I know it brings home how easily I could have lost Zoe myself.

I am so grateful that Alishba and Zoe are both doing so well--what a healing salve of a girl your niece must be to you all! Just as I smother my girl in hugs and kisses --smother that little one of yours with the same!

I wish for you two love and comfort and healing for the pain of your loss of little Simrah. I don't know that I believe in a God, but I do believe in the primordial soup of Love that is the base of all Being--and know that Simrah is cradled tight in that Love that was, and is, her Birthright.

As it is for all of us.

xxoo Kellie

If any of you feel as though you would like to send your words or thoughts of comfort to their family through Shaheena, one of Simrah's aunts, you can contact her through her YouTube channel--and see videos of Simrah and Alishba as well.

Day +127: Dot Matrix

This week saw us going to the SCCA for the first time in 3 weeks. We met with Dr. Carpenter who wanted to check-in with Zoe's prognosis before he recommended tweaking her steroid and anti-GVHD medicine dosing.

He and the transplant team were very pleased with Zoe's progress, and are still not able to say whether Zoe is technically dealing with mild "chronic" GVHD or simply taking her time in managing her mild "acute" GVHD. Regardless of semantics, he declared that he wanted her to hold steady at current medication levels for another month and then see her again. If all continues to go well during that time he will then consider tapering her meds.

Meanwhile, we are to keep a look out for new signs of GVHD. Apparently making it to Day +100 clear of the stuff doesn't mean that she's off the hook.

Well, whatever.

I'm so beyond sweating all that. She's doing well, and I'm choosing to believe that she will keep heading towards health.
----------------------------------------------------

I've been learning Braille and am actually enjoying it!

I've discovered another wonder of Zoe's name just in the learning of this new modality. It takes a bit of explaining to have you fully enjoy the discovery, but here it goes:

Braille creates numbers and letters by using a 6 dot formation such as that found on a basic six dot domino. The dots in a cell are numbered:

1 4
2 5
3 6

The letters of the Braille alphabet are grouped in three rows:

a b c d e f g h i j
k l m n o p q r s t
u v x y z

w

"W" sits by itself as there is no such letter in the French alphabet (the mother tongue of Louis Braille).

In the first row each Braille letter has a specific formation that only utilizes one of dots 1,2,4 & 5 --the top four dots.

Every letter in the second row is essentially the same as the letter directly above it in the first row, except that a dot in position 3 has been added.

Every letter in the third row is essentially the same as the letter directly above it in the second row, except that a dot in position 6 has been added.

So, for example the letter "a" is represented by a dot in position 1. "k", the letter that is situated below it in the second row is made by dot 1, plus dot 3. "u" is made up of dots 1 and 3 plus 6.

Going on, the letter "b" is made up of dots 1 and 2. "l" below it is made up of dots 1 and 2 plus 3; "v" below that is made up of dots 1,2,3 plus 6.

And going further again: "c" = 1,4 ; "m" = 1,4,3; "x"= 1,4,3,6

Everyone following me?

(Usually the numbered dots describing each formation are written in numerical order, but I wrote them in the fashion I did so that you can see the progression of how each letter builds on the one above it.)

So, if you look at the formation of letters of the alphabet the way I wrote them above, and mentally draw columns that group the letters vertically, you will see which letters are derivatives of the ones above it. Get to the letter "z" and you will see that it is derived from the letter "o" above it which was, in turn, derived from the letter "e" above that!

Pretty crazy, huh?

(For those who want to know how to write "zoe" in Braille: "z"=1,3,5,6; "o"=1,3,5; "e"=1,5)

Here's a quick reference sheet that I received as part of my learning materials from the Hadley School for the Blind. It doesn't show up quite as well in the blog version of the scan, but it gives you a little more of an idea of how it all works:

Day +121

Four months and a day past transplant. I just re-watched the video we made on transplant day--and it took me right back to that gaping sensation of the Enormous Unknown.

Makes me want to cry just seeing and feeling that again.

She done good. We all done good. Every single one of us --that means you!

The Zoe-meister keeps thriving and is slowly gaining the ground other kids her age have mastered by now. She sits up well and is close to being able to roll over. She is being very resistant to eating food by mouth though. She went through a period where she was willing to try anything offered to her; now she complains when anything is put in her mouth.

Many things are two steps forward and one step back with her. I keep myself focused on the long term and know, absolutely, that one day she will eat normally, as well as walk and talk and do all the things any other kid does (with her own adaptations, of course). I look at other kids her age and younger quizzically, because they are simply different species to me.

In my world Zoe is the norm.

She is starting to teeth again, after almost a year since her last two came in. The advancement of her osteopetrosis and the resulting overgrowth of bone had locked her teeth into her jaws, not allowing them to progress in the normal fashion. Her new osteoclasts have obviously been diligently at work and have remodeled her bone enough to allow teeth to begin peeking through. Three new beauties are flashing pearly through her pink gums--along with the accompanying crankiness. Poor girl will probably be teething triple time now!

Also, her hair is finally starting to grow back. We call her our little Chia Baby.





A couple new vids added to the YouTube link. It's all about getting the girl to laugh...

Day +113: Million Dollar Baby

$1,014,771.28

That's what the Explanation of Benefits that came in the mail yesterday said Seattle Children's billed our insurance for Zoe's transplant and related services.

Regence negotiated the fees down to $906,199.58--and paid the whopping sum of $248,057.29.

$658,142.29.

That's what the EOB said we owe. (Hello, heart--would you mind beating again?)

But.

We are given to understand that we will be okay--we have been assured up and down that Medicaid will pay for anything not covered by the insurance, that this will be the case through the end of the year.

Jeff called financial services at Children's just to be reassured of this after that gem came in the mail. Gary from the financial department (a very sweet man with the most stunning comb-over I have ever witnessed. Seriously. It stunned me when I saw it.) calmly reaffirmed that everything will be taken care of.

Again, I find myself being grateful for not being an "overly" successful couple in our business in the last couple years. Not that we've been floundering by any means, but I'm thankful that we didn't make one dollar more than the cut-off for aid.

The margin between making too much to qualify for financial aid--and making enough to actually afford over $650K of medical bills is astronomically wide. Anecdotally I know of a local family who found themselves within this margin--and it is crushing them. AND, they still have a chronically sick kid to have to manage as well.

What is wrong with our country? This medical monetary hemoraging has got to be fixed. Maybe pluck a billion or two from TARP...
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This week we saw Zoe's new pediatrician for the first time. Blythe Thompson works at the Hemo-Oncology department at Seattle Children's and is very familiar with post transplant children. We liked her.

Mainly things have been continuing to hold steady with Miss Z--with little tweakings here and there: her NG tube had to be pulled out an inch and a half as an X-ray showed it was going beyond her stomach and into the intestine (not good); her topical steroid for stomach GVHD got increased back to its original, larger dose (here comes Crazy Baby!) as her stools were not improving.

We're moving forward with PT and eating help for Zoe. I've posted a new vid on YouTube of Zoe relishing a cracker--the likes of which we haven't seen since. It was a brief burst of interest in food, and now she's back to resisting attempts to put edibles into her mouth.

Honestly, it seems easier to just fill up her formula bag at this point and set the feed pump going than wrestling food into her mouth.

I mean really, what mother wouldn't agree with me on that?

Day +105

Day +100 passed last rainy Saturday, with little fanfare. It's so good to be this far on the other side, to realize that it's been a solid 6 or 8 weeks since I started to allow myself to future-trip with my girl again.

I've been thinking about what it will be like to raise a blind child--a girl in particular. The awesomeness of that journey freezes me up a bit when I think of it; so many things to figure out.

How do you teach a child with no verbal memory of sight what a bird is? How it flies? What colors are? The enormity of the ocean? Stars and space?

Sex education??!

That one stymies me. Pictures are pretty helpful for that topic. Without that option...how...exactly?

I just signed up for an Intro to Braille correspondence course offered for free by the Hadley School for family members of blind people. I figured I had better get started learning it now. Zoe will outstrip me regardless, but I don't want to be completely unprepared. My understanding is that sighted people actually learn Braille by visually reading it. Apparently it's a bit late for us to develop the needed tactile sensitivity to read it by touch.

We watched an amazing documentary on YouTube about a 14 year old blind boy who taught himself how to navigate through his world by using echo location. It's a truly inspiring piece done in five parts that you can watch by clicking on these links:

Part 1
Part 2
Part 3
Part 4
Part 5

I particularly was inspired by the mother's attitude, which was obviously a huge support and benefit for him.

I'm taking it in, this learning about being a mother to a special needs little girl. Some of it comes naturally, but I'm seeing that most of it is plain old going to be constantly-in-my-face opportunities for "personal growth experiences."

Already started really. And how.

Day +97

We're closing in on that 100 day bench mark. SCCA and I are at odds on what day it actually is--I say Day +97, they say Day +96. I like my number better.

Today we had the big "exit conference" with the current attending, the BMT team nurse and fellow to go over Zoe's history to date and to outline a plan for the future.

So far they consider her to be doing remarkably well, especially given her particular disorder and particularly considering that she had a peripheral blood stem cell transplant. As I had mentioned before, osteopetrosis has a high rate of graft failure, and pbsc transplants have a high rate of GVHD problems. So far the graft is holding strong, and her GVHD problems seem to be limited and responsive to treatment.

Last week they added a new steroid to the mix to treat her mild stomach GVHD (bringing her med pushes to five times a day--woo hoo). A few days into the new routine I noticed that Zoe had a new edge to her personality. Her happy highs were very high--and her lows were maniacal-Jack-Nicholson-at-the-door-with-a-bloody-hatchet types of rage-fests.

I started to wonder who had swapped my sweet daughter with a crazy pixie.

Then I realized, ah yes--'roid rage again. And though the team declared up and down that the new steroid didn't generally have the side effect of personality changes, they backed off her dose a bit. Within a few days she was back to the girl we knew.

We are getting Zoe set up with the Boyer Clinic to help her move forward with gross and fine motor skills. Last week their vision educator, a lovely woman named Mary Ellen, came to assess her. She works with kids of all vision levels and said that unless the child doesn't have eyes, she never assumes that they have absolutely no vision. She told us that she knows two blind adults who use "echolocation" to move around without a cane. She said that one is so proficient at it that he rides his bike around the city. Yee-oww!

Yesterday the PT, Sarah, that we will be working with came to assess Zoe. The tests Sarah did with her described Zoe as having the gross motor skills of a 4 month old, and the fine motor skills of a 9 month old.

Zoe's got the smarts though. The main challenge is to figure out how to motivate her to WANT to reach the developmental milestones. She's spent so much time on her back that she prefers to be in that position. When I sit her up she'll humor me for a bit--and then throw herself backward, or scoot herself down--whatever will get her back on her back. She'll then grin in a self satisfied way and kick her feet enthusiastically.
--------------------------------------------------------------------------

I was contacted by a lactation consultant that I corresponded with prior to the transplant. She wanted to know what I had done regarding the whole breast feeding issue with Zoe, and I felt a bit chagrined and defensive to have to respond back to her that I didn't, in fact, breast feed Zoe as I had intended to do during her transplant. I had good reasons (as detailed in earlier posts on the subject) backing me up--and listed them off to her.

Her response surprised me: she said that I probably now had more knowledge on the subject than many people and that, if I were to write an article for the Journal of Human Lactation on my experience and findings, it would most certainly get published. Not a scientific article by any means, but one that outlines the exploration process I went through and the answers that influenced my outcome on the matter.

The idea is certainly ego stroking. But, I would actually have to get my brain working to do it.

Am mulling over it, enjoying the prospect, and...procrastinating.

Day +89

The endoscopy last week showed a beautiful GI tract--no visible signs of gut GVHD. Although the procedure wasn't a major one, it took about 5 hours from our front door and back again.

Last week was long and pushed the limits for us in terms of endurance. Clinic visit Monday at SCCA, ophthalmology appointment Tuesday at Children's (never simple), endoscopy Thursday at Children's, clinic visit Friday at SCCA, blood draw Saturday at SCCA. Not to mention fitting in work as well--and all those other things like pushing meds four times a day, IV hydration, keeping her feed pump going, and attempting not to go crazy.

So, I'm complaining. Yeah. So?

I've come to terms with the fact that I will never be featured as one of those people that bear up under pressure angelically or with envious grace. Those people can kiss my ass.

Anyway.

Although Zoe's gut looked visibly clear, the biopsies they took during her endoscopy showed some *mild* stomach GVHD that they termed as "sub-clinical". Accordingly they started her on a topical steroid that will treat it. The idea is that if the inflammation is kept in check then the graft and her body will eventually learn to live in harmony. It's a fine-tuned balance between keeping the GVHD at bay and not compromising her health too much (steroids=not so great).

Also, I can't remeber if I mentioned this, but during her shunt placement surgery they also took a skin biopsy to check for skin GVHD. The biopsy came back positive, but it wasn't clear if it was from the hyper-acute GVHD she had during the transplant, or if it reflected a current flare. As she is still on a prednisone taper schedule that suppresses GVHD (and her immune system), it's not possible to know how much GVHD is currently an issue.

Tommorrow is another SCCA clinic appointment (extra-long due to an oral care visit), Thursday we meet with a vision educator from the Boyer Clinic (early intervention program) and have a hearing test and neurosurgery appointment, and Friday is another SCCA clinic day.

Wednesday we are supposed to go to a "long term care" class, but we told them flat-out that we aren't going and to "not bother rescheduling us". The majority of their classes are aimed at adults going through cancer care regimens. We just don't have the time or patience to sit through something like that and cobble together information that might work for us.

So anyway, this week is a "light" week.

Next week is our extra long "exit" consultation with the SCCA people. Then, at Day +100, we will be set loose. For out-of-town patients Day +100 is when they are cleared to go back to where they came from and have their own doctor take over long term care.

We have a line on a pediatrician that works with the SCCA folks and operates out of Seattle Children's. I think we are going to be persuing that option.

A lot of Jeff's family was in from out-of-town this last weekend as Jeff's brother Martin had an opening for a major installation at the Museum of Glass in Tacoma. One of Jeff's cousins who came, Robin, is a professional organizer who spent three hours at our house today helping me make a plan for carving some space and clarity out of the chaos that is in our house.

It's definitely a virgo thing to be so nurtured by such an event. We virgos seem to externalize our inner need for order and serenity and often find ourselves organizing under the sink when our minds need to de-clutter.

I've got bags of stuff by the front door that I'm booting out of here. My definition of junk has widened while identification of "important things" has narrowed.

Thank you, Robin.

And Zoe.

Day +84: New YouTube videos

It's been a while, but I've finally got more videos for you--a total Zoe onslaught! Just click on that YouTube link at the right and you'll be more than sated...

Tomorrow (Thursday) Zoe goes in for an endoscopy to see if she has any gut GVHD. I guess they figured that one procedure this last week under anesthesia wasn't quite enough.

Her head looks sliced and diced with her latest incision and hardware addition (giving her a big bump that I'm not entirely happy with). She's undergone a fair amount of pain, but is now starting to sound like her usual chipper self.

Her head is looking a bit strange now that it is being properly drained. Well, as it was pretty strange looking prior to now, I guess I should say it has taken on a new quality of strangeness: it's looking deflated.

She has dips and sink holes where fluid had previously been bulging out. The picture below shows a little of this --look for the bone ridge sticking up towards the top of her head from this perspective.


It's not all that dramatic looking in the picture, but her new head gave me the heebie jeebies for the first couple days. Now it's just incorporated into my sense of how she looks.

SCCA is starting to get things in place for our "exit conference"--Day +100 is when they set us loose and has us taking Zoe to her pediatrician for ongoing check-in's. We need to do a little investigating to see if we might need to find someone new who has experience with post -transplant children (not quite a common specialty), or if we will stick with her own pediatrician who has been great at looking into things she isn't familiar with.

We are getting Zoe set up with an early intervention program that will help her move forward with gross and fine motor skills. In my mind we can't get them started with her fast enough as she is so often bored from not being able to do much to entertain herself. She has the active mind of a 15 month old, but motor abilities of a 3 month old. She can't see things around her, nor move towards things that she might feel.

An ophthalmology appointment she had yesterday confirmed that she is blind as a naked mole rat. Vision that I observed she had in the hospital, that of being able to register light changes, was likely done-in by this last experience of hydrocephaly (fluid build-up in the brain). It wasn't surprising to hear that she is blind, but there was a part of me that was hoping for a miraculous vision recovery. The doc did say that well, given her young age, there is a slim chance that some recovery might happen, but I really got the sense that he didn't think it was likely.

Last year at Easter I was asked what one word represented what I wanted to welcome in for the year that followed. With my young 3 month old baby just beginning to manifest vision problems and other inexplicable issues, I stated "Miracles".

We have gotten some of those. That a stem cell transplant even works is a pretty damn big miracle in my book.

This year my Easter wish is for "Transcendence."

On so many levels...

Day + 80: Home!

This morning the Neurosurgery Team came by and checked on Zoe. Everything looked good and they said she was clear to go as far as they where concerned. So, after adjusting her pain medication and watching her vital signs for any complications, we got the OK from the SCCA Team and left around 6 PM.

Zoe is fast asleep in her bed as I type. We are soon to follow.

Details to come.

Thanks for your love, support, and prayers

Zendada

Day +79: Good Li'l Shunting

Zoe is back in our room at the SCCA unit in Seattle Children's after her surgery this late afternoon.

The neurosurgeon, Browd, said that everything went as well as could be expected. Three things singular to her situation presented themselves:

1. The tube running from her brain down to her abdominal cavity could not be placed in the usual route medial to her breast tissue as her central line was in the way. He placed it to the outside of her right breast tissue instead (and assures me that it won't cause a problem once she starts to develop).
2. Due to her enlarged liver, he had to make a wider than usual incision by her naval in order to guide the abdominal portion of the tube into place.
3. He said that closing her incisions up was not the experience they usually have, as her skin was of a poor quality "like an 80 year-old's". (hmm, have heard that description before--but back in November it was from her other surgeon in reference to her dura-mater! What is it with Zoe and her 80 year-old status?)

But, nothing really worrisome--the real concern now according to him is whether any infection will present itself in the next few days and weeks. Keep those antiseptic thoughts wafting her way!

Zoe did have to be rather doped up tonight to keep her from endlessly scratching from the itchiness caused by the morphine they gave her. She half pulled out her NG tube from all her face rubbing when I had my back turned briefly, so now she has on arm cuffs that keep them from bending (and reaching her face).

As far as the skin being of poor quality, they tell us it could be due to skin GVHD. SCCA had Browd do a skin biopsy for them to analyze as a part of her Day +80 work up that they do for all transplant patients. From that we should know more about what's going on with her skin on Monday.

Good Friday! Let's have an Even Better Saturday!

Day +78

Just the facts, Ma'am:

Zoe goes in for surgery tomorrow to have a shunt put into her head to enable her brain to drain the cerebral spinal fluid it's obviously not able to deal with on its own. We check into the hospital at 11:30am, her surgery is at 3:30pm.

They don't know why her brain is doing this, but likely it's due to fall-out from the surgery and possibly the effects of the chemo from her transplant pre-conditioning.

The surgery will take approximately an hour. The neurosurgeon will install the tube that will go from the ventricles in her brain, stretching down to her abdominal cavity where the extra fluid will be diverted and absorbed. There will be a programmable valve installed that will be controlled via a special magnet.

Zoe will likely have the shunt for the rest of her life.

A different neurosurgeon (Browd) than the one (Avellino) who worked on her last November will be Zoe's doctor. Avellino has taken a position at the UW Medical center and is no longer treating children. Cunningham assures us that Browd is very good at what he does.

Risk of infection for this procedure stands at 5% of all of the shunt installations they do due to the fact that hardware placement is involved. Anytime hardware is introduced into the body the risk of infection increases.

As Zoe is immunocompromised she will be checking into the SCCA unit prior to tomorrow's surgery and will be returned to the unit after the procedure.

The hospital stay for normal kids usually is a day past the surgery, but as Zoe is "special", we know this may not be the case for her.

This will be the fourth time we will be handing her over to anesthesiologists for a surgical procedure, and it hasn't gotten any easier. While we are necessarily becoming inured to this mind tilting medical saga, the underlying terror of potentially having our hearts ripped out is pretty much still a constant.

Zoe seems to enjoy marking her milestones in accordance to certain auspicious days: Her birthday fell on "Epiphany Day" in the Catholic religion that celebrates the arrival of the Magi to visit the baby Jesus. Her skull surgery date of November 6th was presided over by the patron saint Winnoc who had cured a blind man. Her transplant date was the day before the historical swearing in of our first African American President, and her shunt will be going in on Good Friday. If you follow that sort of thing. I find it all interesting, but don't give it much portentive weight.

Oof, tired--off to bed...

Day +76: Here We Go Again

It was determined today that Zoe needs to have surgery, ASAP, to install a shunt in her skull.

Last Friday we took her in for a consult with Dr. Michael Cunningham in the Craniofacial clinic to have a check-in about her progressively weird head shape and talk about where we go from here.

Yes, he said that her bumpy head was due to surgical suture blow-out during her high fevers, but that he wasn't concerned at that point about needing to act quickly based on how well she was doing and by looking at the most recent CT results from 7 weeks ago.

Her right ventricle looked rather large, but it wasn't clear as to whether it was large due to internal pressure pushing it out (not good), or a lack of external pressure keeping her brain contained (not as urgent of an issue). Apparently brains will expand to fill the space provided, and as there was a large amount of space created by the surgical sutures releasing, it was conceivable that her right ventricle grew as a result of brain expansion into the large bulge on the right side of her skull.

If the ventricle had grown due to internally produced pressure from undrained cerebral spinal fluid, a shunt would be needed to release the pressure. However, if the ventricle was determined to be large from the lack of external pressure, then a shunt would be the wrong path to take as it would cause the brain to deflate from unneeded drainage.

If I confuse, it's only because I care (and still suffer from brain mush due to sleep deprivation).

The CT scan also showed that the area of damaged brain tissue in the right temporal side of the brain was still quite visible--it was a dark mass on the screen. While it looked alarming to me, it wasn't much of a concern to Cunningham. She was young and full of tissue "plasticity" that would compensate for the damaged area, he said. Her state of being, the fact that she was doing so well, was the largest indicator of the overall health of her brain. That, he said, was a more important indicator than what we saw in the scans on the computer screen.

The meeting was left with Cunningham saying that he would review the CT's with Dr's Hopper and Avellino and that they would all confer on the best next step for Zoe.

This morning we awoke to find Zoe's soft spot on her head visibly larger (haven't we been down this path before?). The groove of trusting more and more in Zoe's smoother sailing from here on out got gouged out by the old familiar terror: What the hell was going on with her??!

I snapped some photos of her head,

sent them to Cunningham and then paged him. He got her in for a CT scan by 12:30, and by 2:30 we heard from him that her right ventricle was indeed larger than the last scan had shown, and that the neurosurgeon on call said that she would need a shunt.

I don't know many of the particulars about what this means (How long will she have to have a shunt? How serious is the surgery? What is projected recovery? How do things change with an immunocompromised patient?), but tomorrow we will likely meet with neurosurgery and learn more.

Stay tuned.

Day +70

Wow, a week since the last post. I definitely have much less time to write these days.

We are gradually getting into a groove with all of Zoe's medical needs, but it is a constant and demanding schedule.

Twice in the last week we've awoken early to find Zoe in the middle of a chilly lake of formula due to the feeding tube disconnecting from her feed pump. The first time we were all really pissed off about it, the second time it was more of an annoyance (and Zoe, the one swimming in the mess, was remarkably sanguine about it all).

She is simply remarkable in all of this--so seemingly unscathed emotionally by her experience. She's just sweet and happy most of the time (except when she's not).

In contrast, her parents aren't quite there yet themselves.

We take Zoe to twice weekly clinic appointments at SCCA on Tuesdays and Thursdays for blood draws and check ups. Last Friday's appointment came after a terrible night's sleep, and I was in a foul, foul place about all of the work that stretched unceasingly ahead.

I admit, I may have been rude.

(Madame PA: This is what I know for certain-- when I am crabby about all this, telling me that 'at least I have it easier than a single parent in my position' is not going to make me snap out of my self pity. It will only make me bite your head off. The only proper response is to cluck and say, 'I'm sorry you're having a hard time; you will get through this.')

But, being back at home and having some normalcy again is definitely great--and the work load gets easier with familiarity. I figure that probably the amount of work we have with her is not more than a parent with two children, and people obviously survive that just fine.

Working (massaging) on a fuller schedule now that we're back at home has been good too--it's great to focus on other people's issues other than our own. And massage is such a reciprocal career in that it's usually a mutually healing experience.

On that note, if you're in the area and need a massage, feel free to contact us. Between Jeff and I, we are providers for the major insurance carriers of Aetna, Regence (Blue Shield), Premera, Uniform Medical, L&I, and soon United Healthcare. We have years of experience in both the relaxation and medical massage arenas.

In letting us support your physical health, it's another great way that you can support us.

Day +64: Fun with Home Care

Monday was the momentous discharge day--and I was too darn busy to properly dignify it with a posting here. I am just going to have to get comfortable with always being two steps behind in this next year--because DANG, this home care regimen is pretty much a full time job!

But, I get ahead of myself.

I was the one who got to spend the last night at the hospital--fitting as I was there for the first night as well. The little stinker must have heard me say to Jeff, as he was leaving for the night, that it would no doubt be a much better night than the first sleepless one.

It was, but just barely.

Zoe decided that 1am was a perfect time for playing and jabbering. The lovely nurse offered to stroll her around the unit--and had her out for 2 hours before Z tired of socializing with the night nurses and showed signs of wanting to nod off again. Then she was up every hour from then, and I was reminded of the days when she was a newborn.

Monday was marked by a flurry of activity aimed towards getting us out of the hospital at a decent time. And, even though everything went very smoothly, we didn't get out of there until 2:30pm.

The home care regimen has been a shock to the system in terms of how much work it involves. Zoe needs medications administered at four different times a day, the earliest dose coming at 8am, the latest being given at midnight. Makes for figuring out an adequate sleeping schedule rather challenging.

We hook her up to IV fluids every night (nerve wracking in that we are accessing her central line and therefore increasing her risk of infection) and have to maintain and administer feeds through a pump that pushes formula through her NG tube at a set rate. Feeds have to be turned off 30 prior to administering meds and then turned on again 30 minutes after meds (admittedly we don't wait 30 minutes after the midnight feeding).

Also, we have to flush her central line once a day and change the dressing on the central line once a week (which is a big improvement from when people had to change the dressing daily). In a couple weeks we will have to start back up with the twice daily blood thinner injections to make certain she doesn't clot around her central line.

Twice a week Zoe needs to go to clinic appointments at the SCCA for a few hours worth of blood draws, check ups, and consultations with the attending doctor, the nutritionist and the PA.

All we know is that between work, home chores, baby and baby's medical schedule we are seriously running most of the day--and not sleeping enough at night. They tell us this will get better with time as her medical needs start to taper, but that it IS a seriously overwhelming experience for all parents who leave the hospital with their newly transplanted kid.

Here are some pics of all of her paraphernalia:

From top to bottom: gloves and alcohol wipes, medications, central line flushing supplies, dressing change supplies, tubing, IV pump

In the refrigerator: IV bags, meds

Supplies for the blood thinner injections that we will have to start doing again twice a day in a couple weeks (once her diaper rash heals)

Supplies in bedroom

Zoe in bed hooked up to her IV pump and feed pump (both in the black and red bag at the upper right hand corner of the picture)

It's quite a lot--but it still beats living at the hospital where all the stuff was done for us (but the worrying). Well, off to bed, perchance to sleep (but no guarantees)...

Day +62: HOME!

Just a quick note to say we are indeed home, we are surviving it so far--and we have a truck-load (not my original description) of stuff to do for Zoe's home care.

Will post a more thorough documenting soon--

but I have got. to. get. some. SLEEP!

Midnight and morning medication come altogether too fast...

Oh, and Zoe is fabulous.

Day +59: Ten weeks!

Wow, I stop posting every night, and the next thing I know it's been days since I last updated.

The MRI of Zoe's gall bladder revealed no change: still full of sludge, the walls still thickened. A consult with the GI docs indicated no procedures needed; the general consensus is that the resumption of feedings will eventually help her gall bladder clear itself out.

Word is Zoe gets to be discharged this coming Monday. This time I'm thinking there is a strong likelihood that it will actually happen.

Jeff and I were trained today on how to operate her feed pump that pushes formula through her NG tube. Tomorrow we will get trained in how to hook her up to her IV nutrition as she is not fully off of it yet.

The amount of stuff she is being sent home with, from a mountain of medications to be administered three times a day, to all of the medical paraphernalia required for her support--wow.

I'm just hoping that I won't start to secretly want to be back at the hospital when we really get to experience being 100% in charge of her daily maintenance. I hear it's very overwhelming at first, but that every week gets easier as medications and clinic visits gradually begin to taper.

I ran into our favorite nurse Kelly last night before tucking into bed at the hospital. Turns out she had to race her daughter (the one who had gone through BMT and is now around Day +180) to the hospital due to sudden and violent symptoms that indicated what turned out to be an infection. She's on antibiotics and doing okay now, but it was a grim reminder that immunosuppression is serious and dangerous--and that being discharged from the original hospital stay does not mean that the transplant process is over.

The coming year will be about vigilance and stamina for us. We've finished the steep hill climb portion of the marathon, now comes the long haul.

I'm feeling good about it though. Real good.

Day +55

We still are inching forward toward discharge. Tomorrow's ultrasound will tell us more, but there is a question as to what is going on with Zoe's gall bladder. Her liver enzymes keep spiking, so tomorrow they will see if any improvement has been made.

In the grand scheme of things it's not really a huge thing, but it is part of what's keeping her from being discharged.

The other issue holding her back is getting her GI tract to handle appropriate feed levels for her size. She's still on a fair amount of IV nutrition as her gut doesn't seem to like having too much food in it. It could all be related to the gall bladder issue--again, tomorrow's ultrasound will (hopefully) tell us more.

Aside from these two sticking points, Zoe is just a little spunky chunk of fun--and mischievousness.

I am having to learn the lesson every parent eventually learns: Do Not Laugh at Behavior You Don't Want Repeated!

Oy.

She started pulling off her diaper tab (you know, the one that keeps it shut), and I thought she was so funny and brilliant for her dexterity and skill--and perhaps reacted in a not entirely appropriate way (or at least not in a way that had future vision).

Consequently she now has this horribly magnificent way of unlatching her diaper directly before she poops and has a kicking frenzy.

I'm loving the endless laundry service now, but when we go home this new talent of hers will be a problem.
-------------------------------------------

Jeff got his turn at being the patient today: he had to have a root canal this morning. Poor guy! I just have to tell myself that if it had to happen during this process, it's a very good thing it didn't need to happen a month ago. Always something to be grateful for!

And speaking of gratitude, here's an email I just got today from Diana, the organizer of Sunday night's fundraiser, sent out after the event:

Greetings everyone -

I wanted very badly to express much more tonight than I was able and thought that maybe I could finish off in an after party email.

First, I am so very grateful to Deborah Music and Joe Whinney from Theo Chocolate for all of the amazing effort that they put into tonight's benefit. They provided the venue, the tours and the chocolate samples at no cost so that more of our money raised could directly benefit Jeff, Kellie and Zoe.

Kelly and Manny Loya (representing Altered State of Wine) donated wine for the occasion.

Tony Stevens and Trevor Larkin donated their time and talent so that we could listen to live music.

Kal Austin provided the food and drink.

Henry, Skiah and Manny made posters and helped to organize and clean.

Countless other people brought wine, printed pictures, collected the donations and spread the word and without this concerted effort the benefit would not have been such a great success.

Many of the people attending tonight have never met Jeff and Kellie before. This is a perfect reminder that we need not know everyone to be a community of people helping one another. I am quite overwhelmed at the generosity displayed here tonight.

Between us all we raised over $2500...

For those who attended, for those who did not and still donated and for those who have sent well wishes - I thank you all deeply.

- Diana.

And how!

Blessings have a way of persisting in this process. We are deeply grateful.

Day +53: Choco-Love

I am blissfully buzzing on love and chocolate from tonight's fundraiser for Zoe at Theo Chocolate in Fremont, Seattle.

Our friend Diana, her son Skiah and a couple other friends of his organized an amazing party at our local chocolatier to raise money for us to help get us through this experience with Zoe. Many people showed up for the event, half of which we didn't even recognize.

Wow. It just blew us away. I kept bursting out bawling tonight.

The factory gave a couple tours during the event. We learned that chocolate contains phenylethylamine (which speeds up the flow of information between nerve cells), dopamine (the feel-good chemical) and norepinephrine (which stimulates the production of adrenaline and makes our heart race fast) — all the same chemicals responsible for that energized and euphoric feeling that new lovers experience.

All I know is that I'm warm all over from a love vibe that I don't think is entirely or even mostly chocolate-induced.

Gratitude. Grace. Community.

Zoe's got her village, and it is circled tight. Thank you, and namaste.