Step by step

Annie had another positive appointment yesterday.  All blood counts are normal except for her hemoglobin and that went up from 10.0 to 10.6.  Normal for that will begin at 12.2.  Her kidney functions have all stayed normal so now we don't need to see the nephrologist any more.  Her visits have been extended to every three weeks and she can get a flu shot.  The rest of her immunizations are scheduled for November.  This is an exciting procedure were a group of nurses surround her and all stick her at once.  She has done it once before and it does seem to be the way to go although it looks absurd.  Annie also gained a pound which is much needed.  She has a full plate of activities and goes merrily through her day which has helped build back her appetite.  We feel like a family of wood-peckers because we "knock on wood" all day long for the miracle of each normal day she gets.  Update in three weeks.

Home run

While it sure seemed like we had a perfect storm of bad luck with Annie's illness and all the dominoes that fell from there, today continued our happy progression of good luck.  First was the news that her kidney ultrasound from today showed all was well there.  Next we learned of continued progress with the blood counts.   Whites and platelets still percolating in the normal range and hemoglobin stepping up again to get to 10.0 (normal starts at 12.2).  Her kidney markers that were causing concern dropped almost by half and put her solidly in the normal range.  Unexpected great news.  And finally we learned that her follow-up engraphment test came back with 100% engraphment.  She is now entirely someone else immunologically speaking...weird, weird thought.  This is not to be taken lightly as many aplastic patients never reach this point and some can unfortunately slip backwards.

Annie chugs steadily through her days without pause and has a nice sprinkling of hair coming in.  Tom takes a picture of her every few days so we can make a time-lapse movie of her hair coming in some day.  Life being what it is there are still things to work on.  She's super skinny but at least didn't lose weight between last visit and today's.  The doctor has noted that the road back to physical strength is a far longer one than the time it took for the body to weaken.  She also has very high ferritin levels due to all the blood transfusions.  However when she was last checked for iron overload a year ago the doctor was shocked by the lack of damage compared to what is usually seen.  Hopefully that will give her a leg up but we'll know better in a few weeks as she'll have a full iron panel done at the next appointment in two weeks.  Finally, she has a few rashes that have popped up.  No one is ringing any alarm bells yet but we're keeping an eye on them.  Still, compared to where we've been, we're feeling nothing but gratitude and awe.

The new kid

Annie made it back for the first day of school and I finally felt I could exhale.  While we have so much to celebrate, it has been hard to get excited because she has been "cured" before only to relapse a year later.  There are many things different about this time however it seemed best to just be thankful for each day.  This event, however, felt like crossing the finish line because it can never be undone.

I doubt there have been as many kids as excited to start school as Annie.  She had her backpack ready for weeks and her uniform set out days early.  Normally poky about getting to bed, she was like a jet engine getting ready for bed.  Similarly she was a child possessed in the morning doing her chores (and everyone else's) to get out as early as possible.

While the excitement will, I'm sure, wear off there are some interesting changes that I'm pretty excited about.  With Annie's years of low hemoglobin it has been hard to know if some of her struggles were her nature or her medical situation.  She would arrive home from school and shut down.  Evenings were straight uphill with us pushing all the way.  Annie's mood would erode as the hours passed and if I tried to help with homework I'd be met with harsh outbursts.  Tonight, however, she came home and plopped herself down at ... her desk.  Not the couch or her sister's room where the endless game of avoid what I have to do would begin.  She happily started her homework and when she had a question, I tentatively offered suggestions to which she said, "that's smart, thanks," and went back to work. All I could do was slowly and quietly back out of the room so as not to risk waking the bear who had apparently gone into hibernation.

So we'll keep our fingers crossed and keep up our last major medical task - watering Annie.  She needs to drink 1750 ml of water a day and that doesn't come naturally.  We set timers and measure out water bottles to stay on track.  The doctors lowered her immunosuppressive dose as well as removed another medication after her appointment Monday.  Hopefully this, combined with her additional water requirements, will help make progress on the kidney front.

I'll keep updating approximately every two weeks as we move through doctor's appointments this fall.  At some point I hope to move to a different blog where people can get an email if there is an update posted.  This is because there is still a significant part of this story to tell.  Who is the mystery man with stem cells that produce improved homework behavior?  We of course want to share all those details with Annie's super support team but we have to wait two years!  So I'll figure that out before we hopefully can move on to an un-blogworthy routine.

Day 100!

     This is Tom writing. Today is Day 100, a significant milestone in a Bone Marrow Transplant. It represents two different things. First, like any milestone it is a chance to reflect on the past (e.g. "I just turned 40 and still have my hair... good."). For us, this reflection is almost 100% positive. Prior to the transplant, we had "the consultation", a meeting with our doctor where she laid out all of the incredibly scary things that could happen. Essentially none of them did happen. Yes, there were hurdles along the way (hurdles which now seem like bumps). However, given what could have gone wrong, we're incredibly blessed that things went so well. Second, Day 100 represents a change in the rules for the future. Gone are the masks (though we still do have to be careful around sick people). Gone or much relaxed are many of the rules Annie has been living under. To celebrate we went out to a favorite restaurant to celebrate (see the picture above). Soon you'll see Annie at many of her favorite haunts.
     Our we completely out of the woods yet? No. Annie will still be on fairly heavy-duty medication for a full year. She'll still have many rules to follow (such as staying out of the sun). Plus, no new puppies for us for a while. Plus, there's still some chances of bad things happening, though those chances diminish every day. However, at this milestone we are definitely cheering!

10 bottles of beer on the wall...

Day 90: 10 days to go.  Annie's summer of seclusion is about to come to an end and today her doctor gave the go-ahead for her to at least begin school on August 28th - a mere six days after Day 100.  The doctor stressed that this is not the norm and that very few of the pediatric patients will be resuming school on time.  Annie's relatively smooth recovery so far is thanks in great extent to all those who came before her and the medical professionals that meticulously refined their craft from each patient's experience.  We know some of those patients and we are indebted to their struggles which were studied and learned from to help give kids like Annie a smoother ride.

Today was Annie's first needle poke in three months - now that her Hickman has been removed.  She handled it like a champ and, for the first time in seven years, did not sit on my lap or hold my hand to have it done. Annie's white and platelet counts stayed in the normal range and her hemoglobin took a step up to 9.4 from 9.0.  This is a happy development and hopefully means she won't need any more transfusions.   Her creatinine level however is elevated so she will add another specialist to her arsenal and is scheduled to see a nephrologist in a couple weeks to tend to her kidneys.  Until then we'll stay even more vigilant about keeping up her water consumption.  Already a timer goes off every 30 minutes for her to drink 2 oz of water all day long but we'll redouble our efforts and hope for improvement by our next appointment in two weeks.

We'll plan to post on Day 100 when we hope to take Annie out into the big world to celebrate.

Rolling Along

Day 86: Still hanging out at home without event, well except that Annie now rolls everywhere.  Next doctor's appointment Monday, will update after that.

Free to be

Day 73:  Annie had her Hickman removed yesterday.  It was a big psychological boost as it's hard to feel like you're getting better when you still have tubes sticking out of your body.  Additionally it will free her up for carefree showers and the possibility of swimming.  Thankfully it will free us up from the daily care the tubes required.

Her blood counts continue to be positive; platelets and whites in the normal range and hemoglobin holding at 9.0.  While this is still below the normal range of 11.5-15.5, 9.0 doesn't make her too lethargic or moody (she has been as low as 5.0 but she was very unpleasant to be around).  Her latest project to stay engaged inside our home has been the construction of a Lego village.  Here she is with today's creation - a cruise ship.  So while her body may have been stuck at home, her mind has wondered free and wild.  Next appointment in two weeks so we'll post then.

Back in the saddle

Day 59:  It has been a relatively calm few weeks.  Annie's platelet and white counts continue to be in the normal range.  Apparently her hemoglobin count could take up to a year to reach its full potential and even then it may not be as robust as the other two lines.  Currently it is at 9.0.  However after seven years of low platelets and all the restrictions that came with that (no bike riding...), Annie is thrilled to be cruising the neighborhood on her bike, at dusk (she has to stay out of the sun for a year).  Of course we discovered that her bike was too small, and she has to wear a mask, but no matter, it is pure joy for her.  She also seems to happily move through her days at home with a variety of visitors coming to play games, share hobbies and help her catch up on school work.  She gets tired of her bald look but luckily doesn't dwell. There are adjustments we make between doctor visits in response to any imbalances in her chemistry panels (sodium too low, potassium too high...) and while we seem to be out of the acute GVHD period there are still some things we're watching in terms of chronic GVHD.  She even had her first bloody nose in months but it stopped after a short while like most people's.  After bike riding, her next big goal is to get her Hickman out so she can swim, move more freely, and not require daily and weekly care plus special wrapping to take a shower.  While this means she'll need to go back to being poked for blood tests, she's so used to it that it's an easy trade off for her.  The doctor gave the OK to schedule this surgery and hopefully it will happen in the next couple weeks.  So I'll aim to update then, July 25th.  As usual we are grateful for all the prayers, support and good thoughts that have helped us all get to this point as well as we could hope.

Patience

Day 38: Another good appointment.  Her platelets passed 150,000 which puts her in the normal range for the first time in seven years.  Hemoglobin went down to 10.0 but we're hoping it will rebound at some point before requiring a transfusion.  Whites are holding pretty steady (they bounce around for everyone).  Since Annie will be immunosuppressed for the next year, even though her counts may get strong she is still at greater risk for infections and disease so she has to be extra careful in that arena.

Her kidney function markers improved enough that we can try taking her off the IV pole again Sunday night.  Even better, most of her other markers are stable enough to enable her to only have to go to the hospital for blood draws only once per week.  She has also been able to go off a few medications so we are down to three plus a magnesium supplement.

As the medical tasks become less acute Annie has begun to think about life beyond health milestones.  She wants her prior body strength back, now.  She would like to have her long hair back, now.  She would like to go out to shops, restaurants, classes and play, now.  Luckily these moments pass quickly as we try to keep her busy so she doesn't dwell.

Our next goal is to get her Hickman port removed.  Usually this happens after Day 100 but her doctor was willing to revisit the topic in a month.  Since we are hoping nothing eventful happens before then I will not plan to post again until Thursday, July 11th.

Report Card

Day 34: It's report card season but grades weren't ready when we had our appointment today.  We learned her blood counts are still progressing well but they didn't have the engraftment results from the biopsy.  They did however have information about cellularity.  Back in April when she had her last bone marrow biopsy her bone marrow had 15% cellularity.  That means it was 15% full of stem cells making blood cells and 85% empty, thus why it's called aplastic anemia (empty of blood).  Her biopsy from last Thursday revealed that she now has 80% cellularity.  That was excellent news for this point in the process.  What remained to be known was what % of those cells were her donor's DNA and what % her old, flawed DNA.  We were told we'd hear by Wednesday.  Late afternoon, however, we received an email of her engraftment report and it showed that her bone marrow is now 99.66% her donor's DNA and 0.34% her DNA.  This too was excellent news for day 34 and the expectation is that it will eventually go to 100%.

Since the news came after today's appointment we'll have to wait until Thursday's appointment to better understand how this impacts things from here.  We still have issues to deal with and she was put back on her IV pole for nights today as her kidney numbers went up again.  So there are still hurdles to cross but the engraftment report was the biggest and now we just want to be careful to not mess things up and we're grateful for all the hard work, prayers, help and good luck so far.  We'll report after Thursday afternoon's appointment but it's definitely a cause for celebration in the mean time.