Epilogue

 So, maybe waiting over two years to update the blog is a little extreme, but there you have it. Truthfully, I could have happily continued not updating the blog since most who know us and who are concerned with Zoe’s welfare know that she continues to thrive.  But, occasionally I find that strangers who stumble upon the blog looking to compare notes with their own process of ushering a child, often a child suffering the from the same genetic condition, through a transplant are curious to know what has happened since the last post.

Bottom line: Zoe is doing very well.  She’s a vibrant 4.5 year old with a huge personality. We’re all rather well recovered from that chapter in our lives, but the effects of it will always live with us.  Our focus has gone from managing a child with a life threatening condition, to learning how to usher a child with a disability of blindness, and some other less severe physical disabilities, through life as a well functioning individual.  As such, our lives encompass both the typical experiences of “normal” families (park play times, childish goofiness, tantrums) to the not so typical (the constant education of strangers about Zoe’s blindness, special services, Braille lessons).  We won’t ever really feel like we’re walking a typical path with Zoe, but where we are is so much more in that realm of “normal” than where we’ve been with her. 

That’s the short report.

But, if you want the longer story of how we got from there to where we are now…

 So, the med taper continued until the end of the Summer of 2010 when she was taken off of everything.  Things went along smoothly until sometime December that Fall when it suddenly dawned on me one morning while I was lounging with her that I hadn’t seen her clap with flat hands in quite awhile.

An odd thing to suddenly focus on, but my mind went there, and attempts to get Zoe to straighten her hands and fingers out fully were met with screams of pain.  Her hands were rigidly held in a cupped position. 

It was easy to miss since most people’s hands at rest are naturally cupped, but now that I was noticing it, I was perplexed at how I could have missed it.  Zoe’s hands were almost completely lacking in movement beyond the most basic and limited clutching function.  Further examination by Jeff revealed extremely rigid wrists, hands, feet and ankles, which was strange given the fact that Zoe was still really hypotonic in the rest of her body.

Holy crap.

As any terrified parent would do, I took to the internet with search words such as “joint contractures”, “hypotonicity”, “pediatric”—and I was regaled with all sorts of references to muscular dystrophy. (A reminder to not do medical research for your kid on the web when you’re freaked out—it’ll only mess you up.)

In a panic I contacted Dr. Manley and Dr. Carpenter.  Within days they saw her and confirmed the presence of GVHD.  According to Carpenter, muscle contractures such as hers were a rare form of GVHD, and the fact it was presenting in a child was even more rare, but here we were.

So, back on the immunosuppressants again!   This time, instead of tacrolimus she was put on very low dose of methotrexate.  We were told that while it’s used as a chemotherapy drug in high doses to combat cancer, in low doses it’s effective at treating autoimmune disorders.

To physically combat the contractures she was fitted with braces to wear on her hands at night, and ideally at times throughout the day.  That went over like a lead balloon, as they say.  She’d wake up screaming in the night until we took the things off, and when we tried to put them on during waking hours she’d scream just as hard.  Eventually she’d let us put them on her for novelty’s sake, but she’d want them off pretty quickly.  Made sense since, being blind, her hands were her eyes.  It seemed a cruel twist of fate for the GVHD to mess with her main vehicles of perception.  The braces didn’t last very long.

The contractures in her ankles inhibited her forward progress in gross motor skills development as well.  At almost 3 years of age Zoe could still not walk on her own, stand up by herself without the aid of something to hold onto, nor sit or squat down on the floor without aid.  Stiff ankles made her unsteady, and her blindness seemed to promote a lack of confidence in navigating spaces around her.

Some things were mercifully progressing: she did finally learn to eat by mouth just prior to discovering the GVHD. 

Up until that point, as you might recall, she was an entirely G-tube fed girl.  We made her whole food diet up from scratch every morning in the Vita-mix and fed it to her in measured doses throughout the day.  Every once in awhile we’d try to offer her something to taste, but she’d vehemently refuse it.  She was perfectly happy to be a tube-fed gal.

Earlier that year, my chiropractor mentioned that he had heard from another patient about a program at Seattle Children’s that was specifically designed to put tube-fed kids through an intensive training to get them back on oral feeds.  Apparently this wasn’t an advertised program since it was in high demand. 

Dr. Cunningham confirmed the existence of this sought-after program and referred Zoe.  In November for two weeks we took Zoe Monday through Friday to the Occupational Therapy department to work with Karen Quinn-Shea on eating skills.  Basically, the strategy was to reduce her tube feeds steadily and significantly enough for her to start to experience hunger.  And, while we did that she worked with us and Zoe for two meals and a snack time each day to introduce her to foods.  Basically, the idea was to keep her playing with applesauce, peanut butter, and crackers, and cajoling her to taste them constantly enough until she started to make the connection between consuming food and the satiation of hunger. 

It was an arduous and exhausting process.  Each morning we got there at 7:45 to get Zoe weighed in and then settled in for three eating sessions that were spaced out to take about 1 to 1.5 hours each session.  There wasn’t enough time between each session to really go anywhere, so we usually hung out in the play room at the hospital, and spent nap time in the chapel (quietest place I could find there).  Our daily sessions ended at 2pm, but we were expected to continued the routine at home for another snack time and dinner, all the while following the prescribed taper on her tube feeds.

By the end of the two weeks, Zoe was down from five tube feedings a day to only one, plus boluses of water throughout the day.  She ate the rest of her food by mouth, but it was difficult to watch her diet go from a richly balanced and supremely healthy whole food concoction we made for her daily in the Vitamix, to one that consisted of crackers, peanut butter, and maybe a little applesauce.  And now, instead of her meals taking about a minute to squirt into her G-tube, we were now spending over an hour five times a day trying to get her to eat.  I’m sure I don’t have much sympathy from anyone who’s been the parent of a young fussy eater, but this was weird new territory for us.

For the next year Zoe only ate food on crackers.  We expanded her diet to include squash, ricotta and bananas.  All tube feeds and water boluses were eventually stopped.  At the age of 3.5, a year ago, Zoe finally learned how to eat with a spoon.  Her diet is still limited compared to other kids her age, but she eats healthy Indian food that we make for her and eschews anything sweet, so I’m not really concerned about it.  She’s been completely off of food and water boluses through the G tube for a year now, and in April of this year—three months after we used the G tube for the last time for meds—she had it removed.

Zoe is still delayed in her gross motor skills abilities.  She’s coming along, but she only just a year ago—at 3.5 years of age—learned how to walk unassisted.  In addition to the feeding program, we were lucky enough to get PT for her through Seattle Children’s, and after a few weeks she finally felt confident enough to walk without assistance.  Months later we took advantage of PT yet again to teach Zoe how to stand up and sit down without holding onto anything.

I would say that at 4.5 she is still not a very confident walker.  Around the house she is most comfortable stepping out, but outside and in unfamiliar places she really insists on holding someone’s hand. 

She also does not have very good stamina.  She doesn’t run around like other kids do, as though they have limitless reserves; she prefers to sit on the couch or move within a rather limited and low key scope.  It’s hard to say whether it’s due to lowered energy from her medical history, or whether it’s from a self perpetuating cycle of lack of movement resulting in lack of stamina for movement.  We really are working on getting her to move more and more, but it’s somewhat of an excruciating endeavor.  We try to force her to walk longer and longer distances, but once she decides she’s done walking, it becomes an exercise in trying to get her not to scream so loudly that the surrounding neighborhood thinks I’m attempting to murder her.  She’s got a strong will, that one.

But!  I don’t want to paint a picture of a languid, listless girl, far from it.  She sings and talks non-stop, she plays her keyboard and makes up music and songs.  She loves to dance, and her new obsession is doing a conga line with Daddy or Mama around the living room (so, really, if I were willing to dance a conga line for hours on end, I might actually get her to exercise without any whining.)

Okay, back to the GVHD.

Zoe was on the methotrexate for just over year.  During that entire time, and for six weeks beyond it she was also on a broad spectrum antibiotic called Bactrim to protect her immunosuppressed system from getting pneumonia.  Her hands and feet gradually loosened up a bit, and when Carpenter was completely satisfied that the GVHD was not progressing and was in fact retreating, he took her off the drugs at the beginning of 2012.  So far no return of GVHD.

I wouldn’t say that her hands and feet are back to normal.  Her ankles can articulate a little more than a year ago, but they are still quite stiff.  Fortunately walking doesn’t really require full range of movement in the ankles.  If we ask her to stand on her tippy toes, she can only raise her heels about an inch or so off the ground.

The hands can straighten out completely now and feel loose in finger extension, but she really can’t close her hands into a fist hardly at all.  The fingers do not articulate at all at the joint that joins them with the hand, and her wrists do not flex forward, nor really do they extend backwards significantly.  When she wants to put her hand down to support her weight, the form is very much akin to a knuckle-walking primate.

And, as bad as this may sound, it’s so much better than it was!  We have been in the path of blessings in many ways throughout this process, and no less so in the treatment of this contracture issue.  Months ago we were made aware of an amazingly talented Rolfer (a person that does Rolfing, a specialized type of deep tissue massage) in town that does nerve release and mobilization.  When we contacted him about working on Zoe, he offered to work on her for free based on her age and situation.  The ongoing work he’s done on her has made all the difference in her mobility.  And, Zoe loves to see him.  She protested vociferously the first couple sessions, but now she receives it like a trooper and even looks forward to it.  Without his work so generously given she would not have nearly the range of motion recovered that she does.

We take her to a park most days to work on gross motor and play skills with her.  I’m trying to teach her how to “pump” on the swing, which is a challenge on a couple fronts:  her limited hands make it difficult to hold the chain tightly enough to support her weight, and it’s actually quite difficult to describe to a young blind kid the mechanics of pumping!  I obviously can’t model it for her, so I’m left with description and touching.  When the swing is moving in a limited fashion I can put her legs in the proper form, but incorporating the necessary leaning into the mix is challenging.  Instructions to “lean backwards while kicking and traveling forward” translates into some interesting moves on her part that are not really close to accomplishing what I’m describing, but she gets definite props for the gusto-filled attempts.

I left the blog in a bit of a cliffhanger state as far as what I learned from Zoe’s donor Elizabeth.  As I stated in the previous post, we established contact and exchanged several emails filling in the gaps for each other.

You may remember from a much older post that we had hoped for bone marrow in Zoe’s transplant, but were told that the donor was only willing to give stem cells.  I believe I indicated in that post that the doctors and donation coordinator on our side even reiterated to coordinators on the donor side that we really preferred bone marrow over stem cells, only to be told yet again that “absolutely not—the donor is only willing to give stem cells.”  It was a difficult experience at that time, but as you may also recall, stem cells ended up being a more perfect transplant source than marrow due its faster engraftment time.

When Elizabeth and I began emailing, I sent her the link to my blog along with a heads up about that particular topic and that, while I was voicing frustration at the time in the blog, I really was very grateful that things went exactly as they did.

Elizabeth’s response was very interesting: she said that her donation center told her that stem cell donation was the only option.  She told me, and I absolutely believe her, that she absolutely would have been happy to donate bone marrow as she was so thrilled to be called up to donate to someone in need.  The donation center she lived near was not set up for surgery.  She could have donated at a hospital near by, but was not told that this was an option.  Based on what she told me and what I told her, we both came to the unhappy conclusion that possibly her donation center was motivated by a drive for money and the need to keep her as a patient more than they were motivated by the desire to do the right thing.

It was a shock to us all to learn this bigger picture, and for awhile there we thought to take that donation center to task, but as everything had turned out so well, we dropped it.  The battle had already long been won; no need to seek out another war.

It was just so lovely to learn more about this amazing woman whose cells saved Zoe’s life.  I got permission from her to quote from an email she sent me, because I just love this story she told:

I lost my mom to cancer almost fourteen years ago.  (It was nothing to do with blood or heredity, so please don't worry.  It was adenocarcinoma of the lung caused by radon in the house and exacerbated by smoking in her teens through mid-thirties).  She was a huge part of my life and we were blessed to be able to share an incredible relationship.

Until she died, I thought little of spirituality.  I was twenty-seven, so really I thought little about death, loss, karma, etc.  But, after losing her, my eyes were open to possibility and I came to know in my heart that there is so much more to life than we can see.  

Through what seemed like coincidence, I met mediums who told me about my life and my mom's journey in rich detail that could not be fabricated. In all these cases, with at least three different mediums, a female spirit, a soul described as "special", "beautiful", "unique", "angelic" kept coming into their readings about my life.  

Now at this time, I had my two older boys and I wanted another child.  So, of course, I just KNEW that my third was going to be this special soul.  I had no doubt!  So when my amniocentesis said that I was going to be the mother of three boys, I doubted the results.  My husband, not as spiritual as me at the time, laughed and said, "I can't believe you doubt the doctors and trust the psychic!!!"  Ok, he had a point....

So then I raged at the medium.  "How can you lie?" I asked one. She said, "I am sorry if you are disappointed, but I still see a special female spirit.  Maybe you will have a fourth child."  When I assured her that was not an option.  She said, "Weirder things have happened".

Then, as I would meet other mediums, this "angelic", "special" spirit would continue to appear in readings.  I knew I was thrilled with my family.  I knew I was done having children.  Who was this spirit? Who was she to me?

Well, in the fall of 2008 when I was told I was a "perfect" match for a baby.  I looked and Daren and said, "it's the angel from the readings" (he rolled his eyes).  When we went to the meeting and they told me she was a girl, Daren stopped rolling his eyes.  He also knew that, no matter what, I was giving whatever I could give to this child.  Surgery, bone marrow, anything!  (I still would, by the way....)

Now, being the impatient person that I am, I could not wait a year to find out how "baby girl" was doing.  So, even though I no longer need to go to mediums to feel my mom's presence in the world and in my life, I went to my friend Galit.  She did not know about the donation.  Now, Galit is gifted but she is also newer at being open to her gift.  Unlike someone experienced who understands all of the symbols she is given, Galit sometimes doesn't understand the more abstract.  I went to her house for a reading simply to hear about Zoe and her progress.  

She asked me, "Which one of your sisters has a baby girl?"

 

None.  

"Who is this baby that you are connected to in your family?"

No baby girl.  

She kept insisting that someone close to me, in my life, had a baby or was "getting" a baby and this baby would be connected to me. This went on for two hours.  This baby girl image kept popping into our conversation.  I had no idea what she was talking about (in hindsight this seems daft) and frankly I was annoyed...

I said..."Galit, how is the baby doing, the one who received my stem cells?"

Galit just started to laugh...she kept seeing a baby girl whom I was connected to "like an adoption".  She had no idea what it meant or how to describe it.  

How can one describe a connection like we share?  

Then, she told me the news: "It will be a difficult year, but she is going to be fine!"  What a wonderful relief!

"Will I know her?" I asked.  

And then she said what you have solidified, "She will be a part of your life."  

Kellie, I couldn't be happier or more honored to be even a little part of Zoe's journey.  And, I have been told by many that it will be an incredible life.  She is special...but you know that already!

I really do know it.  It’s interesting to watch how people respond to Zoe who meet her for the first time.  She interacts with the world around her differently, and I think it causes people to step out of whatever auto-pilot they’ve developed around interactions with children, or even with any sort of typical person.  A friend with a bit of a psychic gift herself once told me that Zoe would help people learn to "really see”.  And, watching how people interact with her, how they become truly present and joyful in her presence, I can see how this might be true.

So, I think that’s about it for now.  I may update this blog again, but for now it’s at a better stopping place than where I left it a couple years ago.

Just a blurb about where Jeff and I are currently: we’re doing great.  We’ve continued with our daily yoga and meditation practice we learned three years ago that was so instrumental in pulling us out of our PTSD from the experience with Zoe.  It’s given both of us more of an experience of flexibility and resilience in every aspect of our lives. As such, I’ve actually been working on a career change in the last year and a half by taking math and science prerequisites for a Nurse Practitioner program I hope to be accepted into next June.  I’m also having fun learning to play the ukelele :-) 

When not doing all the wonderful things he does to support me in my schooling endeavors, Jeff has been keeping his mind and creativity active by becoming an impressively good blues and jazz piano player for someone who started in middle age, and by pursuing the crafting of the perfect ultralight alcohol burning camp stove. What a husband and daddy.  How did I get so lucky?

So, Life goes on.

What a crazy ride.