Smurfing USA

Although our waves aren't nearly like those people in the East are struggling with, we had a bit of a ride today.

After another week without emergency bleeding events we went to the hospital today for Annie's blood test.  Her hemoglobin was 7.9 - just on the edge of transfusing, so we held off.  However her platelets were reported at 68,000!  I had to ask the nurse to repeat this to me three times.  Annie hasn't made that many platelets since getting aplastic anemia (except after her first transplant).  I had to conclude that the "smurfs" were doing their thing.

However, ten minutes later, we were told the lab reported a change and they counted 15,000 - 45,000 with some "large platelets."  This was all very unusual to me and I've read over 300 CBCs.  Apparently this was also rare for the nurse since she didn't know how to explain it.  To be fair, Annie is the first one on Eltrombopag at either of the hospitals we've been to so no one really knows what to expect.

After doing some research, it seems "large platelets" are immature ones that the bone marrow releases early when the body has extremely low levels of platelets.  Why this makes counting so imprecise I don't know.  But we're just grateful for her body to produce anything - especially when it keeps her from needing a platelet transfusion and keeps us from being awaken in the middle of the night.

We received a status report on the donor search.  Luckily the hospital was able to get a grant to conduct a search for a match.  We are told not to expect to hear anything for a month.  After being explained the process I must say I was filled with amazing pride for humanity.  To think there is this web of people working in concert to try to save the life of one little girl is amazing.  That there are people all over the globe making themselves available to save another person - that they don't know - really makes it feel like a small, connected world.

Next appointment scheduled for 11/8 - as always no news is good news.

Super-sib

This picture is a month old but, since things have been quiet (by our standards), it seemed a good time to praise Annie's unsung hero - her sister Katherine.   When we started this journey, almost seven years ago, we were given good advice about the impact a child's illness has on a sibling.  Katherine was only seven-years-old but all along she has been a trooper. When Annie's treatment required her to wear a hat to school every day, Katherine did too. When we adopted a vegan diet to support Annie's health needs, Katherine did too.  When we had to leave our house and belongings, Katherine rolled with the changes. She spent vacations with other families and relatives, she moved to her grandparents' house when she got sick, and she went home from school with a different family every day.  She has spent countless hours entertaining her sister in hospital rooms and at blood draws.  

Far from resenting Annie, Katherine has loved her and tried to protect her like no one else.  When she'd hear an ambulance go by school, she'd excuse herself from class to make sure it wasn't for Annie.  She has done the lion's share of kid chores and had more than her fair share of "the short end of the stick" but she has also enjoyed many blessings and opportunities.  

There is an organization called "Super Sibs" whose mission is to give the siblings some of the spotlight usually focused on the sick kid. Over the years they have sent her a steady supply of notes, poems, trophies, medals and kudos. I'm often surprised just how much these gestures have meant to Katherine. Certainly the close relationship between the girls is another silver-lining of this journey.

Annie had transfusions for platelets and red cells yesterday-over nine hours at the hospital. The donor search has not yet begun as we're still in the insurance ... phase, but things seem to be moving along.  In the mean time, every two weeks, we continue to raise the dose of her "smurfs" (eltrombopag) and lower the dose of her "bunnies" (cyclosporine).  Will report after our next doctor appointment on the 29th if nothing else comes up.

The Honeymoon Phase

This was the calmest week we've had in months.  The only time we really had to deal with medical issues was Annie's hospital appointment Thursday and she found that to be pretty, well, fun-ish.  Since it was mid-day, the hospital was kind of quiet.  The sun was shining off the fountain out front and, again, Annie found the whole thing lovely.  Going to the relatively empty transfusion area she was greeted by a flock of nurses who remembered her from five years ago.  All that fawning feels good.  They gave her a present from the toy room.

Then it was time for the poke and Annie dreamily said, "Wow, I didn't feel a thing." (Since it was the arm that was blue for five weeks from the blood infiltration, we had left it alone so I'm guessing her veins had healed nicely.)  They wrapped her arm/IV in a tape color she hadn't seen before. "The blue matches your eyes," said nurse Ginger.  Then, while waiting for the blood test results, we walked to the Farmer's Market (!) they have on the hospital grounds.  Annie selected some peaches and we settled in for lunch on a walkway with beautiful views of the mountains.

When I called for the test results we learned she didn't need a hemoglobin transfusion and that the doctor would see us early (huh, we used to wait upwards of four hour...not kidding).  After the uneventful check-up it was off for the platelet transfusion (remember this hospital has a set value where they transfuse versus waiting for a bleeding event).  They combined the platelets with IV fluid so the rate was 200 versus 80 and the transfusion was done in only 20 minutes.

Next appointment in two weeks (no news is good news until then).  While I know this is the calm before the storm, we're soaking up every drop of it!

The path ahead

The week began with a meeting at the hospital where Annie had her first BMT.  It was unbelievable (and comforting) to see how excited Annie was to be back there.  Exiting the freeway she exclaimed, "I remember this!"  Entering the property she rejoiced, "Every hospital should be like this, see the beautiful gardens!"  As we drove to the drop-off area, "Look - all the old people!"  As it turns out Annie finds much greater comfort from the calm nature and pace of a suburban hospital mostly filled with older patients than the relative chaos of a city hospital that caters to children.

We had a thorough meeting regarding the plans for Annie's treatment.  In a nutshell there are three parts to a BMT.  First the pre-regimen.  This begins with multiple full-body tests to assess for any other issues that could create complications.  Next comes the job of killing her existing immune system.  This will be the first of the two nasty bookends.  Obviously there is nothing pleasant about living through a part of you dying.  This task will be accomplished with a combination of total body irradiation (TBI), cytoxan and fludarabine.  One conciliation is that she is in the most experienced hands we can give her and everything possible will be done to minimize the unpleasantness.  She will be in isolation from this point on as the process will render her body utterly defenseless against infection.

Next comes the transplant, a surprisingly simple process of dripping donated stem cells in fluid through an IV line for a short period of time.  Then there is a three-week period of anxious waiting to see if the donated stem cells successfully repopulate her body.  If that hurdle is cleared the second bookend commences.  This is the war of graph versus host disease (GVHD).  This we didn't really have to deal with before as she received her own stem cells we had stored from her cord blood.  GVHD can range from minor to acute.  Factors affecting this are the dynamics of the donor cells, how well her system was destroyed and how her body responds to the graph.  The path is measured from Day 0 at transplant to Day 100 with hoped for milestones in between.  There is no one description of Day 100, it is a convention.  For each individual, life post transplant is unique and really not an ending. You work with your transplant for the rest of your life to varying degrees.

While that is the path, we don't start down it until a donor is identified and, hopefully, willing to become part of the journey.  This will be a big part of our story for the month ahead.  In the mean time, the week was gratefully pretty-routine with no acute bloody noses or ER visits.  Of course routine for us doesn't mean uneventful.  There was the small matter of Annie tripping over her backpack on Thursday and ending up on crutches.  Luckily we dodged a bullet with that one and she was back on her feet the next day.  As you can imagine we work very hard these days on keeping an even keel no matter what life throws at us.

Sprung

As usual things didn't go quite so smoothly but not too bad considering.  Saturday night the chills and fever returned.  At 11 p.m. she was at 103 degrees F.  After some Tylenol and an intense fluid IV (think bathroom break every two hours all night with Mom wheeling the IV cart behind) the temperature subsided not to return.  The bacteria cultures were negative thus just a virus she picked up. Seems the flu got to town before the kids got to the flu shots.  (Flu shots were our first stop after leaving the hospital. Yes, the doctor said she should go immediately to the drug store even having been sick.  So if she has the ok to go to the corner drug store for a flu shot I'd say its a good idea for most everyone.)  She was discharged Sunday morning and just as they were about to remove the IV... she got a bloody nose!  Really truth is stranger than fiction. So we spent the next hour managing that and debating about a platelet transfusion before leaving.  So they needed to draw blood and check her numbers and our 11 a.m. discharge stretched to after 3 p.m.  The counts came back in the gray zone so the hospital didn't want to transfuse.  Thus we had to decide to fight the decision or not.

We've had to make so many complicated decisions about pretty weighty issues that we've studied up on decision making.  The most important thing we learned is that we all should study up on it.  Turns out the cause of a lot of mistakes and hardship is due to not being informed in even rudimentary decision making techniques.  Above is a weighted decision matrix we used to decide about last Tuesday's visit to the hospital for a platelet transfusion.  I can't emphasize enough how great these things are.  So many times I've learned that I was about to make the wrong decision because I was caught up in the emotion of one facet of a decision but when I broke down all the elements and weighted them that a much better choice was obvious.  For yesterday's decision we used a pro/con list and, again, it was clear that our emotions were pulling us one way but the facts clearly pointed in another direction.  Thus we took her home without the transfusion.

She's been doing great since although we kept her home from school just in case.  Today we have our first meeting with our probable BMT team so I'm sure more decision matrices are in our future.  Will post once we've sorted through our options.

Rained out

Never a dull moment at my house.  By Monday night it was clear that every part of Annie, except her nose, was determined to bleed and things were getting worse.  Thus off for a platelet transfusion Tuesday morning.  Not fun but there was something special around the bend to keep the spirits up.  Tom and the girls had a "last hurrah before transplant" overnight at Disneyland planned for the weekend.

Friday was a focused push to get weekend homework done so all that was left was fun.  Friday at dinner-time (what is it about dinner-time and disaster for us) Annie came down with heavy chills.  We have a sheet of paper with a list of symptoms that require an immediate hospital visit/call and this is one of them.  Temperature? 102+ and yup that's also on the list.  Thus another trip to the ER although this time we knew we'd be admitted.  Arriving at the ER around 8 p.m. means getting a room by, oh, 3 a.m.  but what do you expect, Disneyland?

But of course that's what the girls did expect, so this has been a big disappointment.  However it's also - yet another - chance "to make lemonade out of lemons."  Frankly we're pretty tired of lemonade around here but it's the best we have.  Katherine is in fact currently trying to use the bed-controls to simulate an amusement park ride.  They tried making Halloween masks although we forgot adhesive and they won't give us scissors (?) so we could only muster up hospital tape, thus they looked fairly absurd (see the 3/17 entry for other fabulous creations with hospital tape).  Luckily Annie's fever is under control and, if it stays that way, she can go home tomorrow.  Her hemoglobin was back down to 7.4 so she's getting a blood transfusion now.  If we're lucky maybe we could avoid a hospital visit during the week although I really try not to get my mind set on anything these days.

Head, shoulders, knees and toes...

I can't help but think about this children's song when looking at Annie.  It seems the trouble spot rotates from one place to another.  The "smurfs" continue to keep the bloody noses under control.  She'll get a little drip but it stops.  No four-hour nose bleeds or night-alarms for over a week!  Also she has had enough energy to get through school, after school activities and homework while staying in a good mood.  That too is a welcome change.  However we just seem to move on to the next territory.  

We happily changed out her blood spotted sheets hoping the new set would stay clean but no, a patchwork of spots greeted us in the morning.  Turns out she's cutting molars!  And although the blue arm is starting to fade, being back at school and having more energy has led to black and blues all over her legs.  Also she still needed a transfusion Friday (hmg 7.5, plt 6,000) and she has run out of arm veins so they moved on to her hands.  The first poke failed so both hands now have bruises.  

Annie, however, is nonplused by any of it.  She got Emily's pajamas and could care less about the reason.  She is full speed ahead for the next medical event as long as it means more Emily loot.  It actually brings tremendous comfort that she has something to balance out any negative feelings she might have about the future.  

We have our first meeting planned with the BMT team a week from Monday.

Smurfs

Annie's new pills (eltrombopag) arrived by overnight delivery Friday.  We gathered round with great fanfare as the bottle was opened for her first dose.  What would they look like?  Much to her delight they were smurf-blue.  We have silly names for every nasty thing she has to do so why not add smurfs to the menagerie?  Her only concern was about any side-effects she might have but so far so good.  She has reason to worry as bothersome hair growth has been one side-effect of her other medication.

We, again, play the role of guinea pig.  This is new stuff.  It only came into our life when, a few weeks ago, Tom's google alerts had an article about the drug being approved.  It sounds like she's the first user at her hospital.  The success rate in trials was 45%.  From what we've read, we're looking to see if anything happens in a couple weeks.  The doctor plans to start the dose low and reevaluate at the two-week mark.

Annie was already blue so we can't blame the drug for that.  Her hemoglobin transfusion from a week ago came out of her vein and the blood pumped into her tissues.  Thus her right arm is bluish-black from the blood collected under the skin.  She was asked to cantor the first school mass but she suggested she might scare off the new kindergarteners when raising her hand to invite the congregation to sing.  Other than that she seems to have made it through the first week of school with only one interruption for a blood draw.

Yesterday afternoon she was upset about chores and started to cry (normal kid stuff).  Usually crying starts her nose running and we begin a nose-bleed cycle.  She started dabbing her nose (she dabs most of the day and I find little tissue balls with blood spots all around the house) but ...  nothing red.  Then she did the forbidden, she blew her nose.  The poor kid has been prohibited from blowing her nose for months as it sets off bloody nose episodes that usually result in emergency room ordeals.  However this time, nothing.  I might as well have seen my kid score the winning touchdown.  How was this possible?  She blew her nose and no blood came out.

About an hour later she banged her mouth on her water bottle and cut her lip.  This is the kind of event that ends up ruining our weekends.  Blood will come out unabated, swallowing and stomachaches ensue and, again, off to the emergency room.  She sucked on some ice and by dinner, no more bleeding.  Not to be thwarted, a couple hours later after brushing her teeth, her gums started to bleed.  We were out walking and didn't know but when we returned to say good night her gums had a proper patch of dried blood at the spot.

We generally kind of dread night time as there is a good chance of being awoken by a screaming, bloody kid.  Nonetheless we head into each night hoping we'll be spared.  Usually we get one or two nights catch up.  Both Friday and Saturday nights have been quiet and no blood spots on the pillow in the morning.

We are grateful for any break we get and avoiding all he possible problems of the past 48 hours is a gift.  We're just following the mantra of so many, one that a friend recently invoked, "I am really into a day at a time."

Brave Emily

Annie has many happy, normal-ish parts to her days.  Then again she has some really tough parts.  While there is sorrowfully little we can do for her medically there is much we can do to enhance her emotional and spiritual journey.  One such thing is ... consumerism (always good to help the economy).

When we "evacuated" our home upon learning her body was contaminated with the pesticide chlordane we had to leave a lot of things behind.  One such thing for Annie was her American Girl doll and accessories.  Annie's pleasure in imaginative play gave her hours of fun with her doll.  That experience was interrupted but we figured she'd grown beyond dolls and never replaced it.  Maybe it's regression in times of stress, maybe we expect kids to grow up too fast, maybe it's therapy and maybe some people just love imagining but Annie is still feeling keen about having a doll.  What a gift.

During her first BMT (bone marrow transplant) she had a baby doll that she looked after and it gave her control in a situation that offers little in that direction.  Getting another doll could do the same.  Thus to congratulate her on having a good attitude about yet another transplant we took her to the American Girl store and let her pick out a new doll.  She had two weeks notice and gained much pleasure from researching the purchase.  Ultimately she settled on the one that had been left behind, Brave Emily.  Emily is a British girl circa 1944 who comes to America.  There are books about her life and lots to buy.  This is the business model in full bloom.  There are many dolls with life stories and stuff to purchase.  My thought is even if Annie looses interest in the doll-play the adult pleasure of collecting will kick in and keep the engine running.

The accessories and doll clothes are like crack for kids.  New stuff coming out all the time... pets, furniture, outfits for every event and historical period, sports equipment, instruments ... on and on.  All displayed in cases with pictures of the fun to be had upon ownership.  While this might usually get on my nerves it's a dream for my situation.  Our deal is when Annie goes through something tough we have a little stash with some AG swag that she then receives.  So far it has had its desired effect as she has expectantly asked me, "so when is my next procedure?"

So although she had a tough time with her hemoglobin transfusion on Thursday she gets to look forward to getting Emily a "pet" next Saturday.  Whatever it takes.

We've cleared the dosage issue for the Eltrombopag but are now waiting on insurance approval.  We're also almost done receiving all the necessary records to interview transplant teams.  Final issue is that while insurance covers the transplant it doesn't cover the pricey matter of the search (can't do one without the other) but one hospital has a grant for this so always much to sort through.

Annie will start school and keep up a 6th grader routine as much as and for as long as possible.

Gimmicks

Rarely a dull week around here.  When Annie had her emergency visit to the hospital last week for a bloody nose an ENT doctor was called in to help.  He made the suggestion of using tongue depressors bound together to help with nose pinching.  We thought this sounded great.  I modeled the contraption shown above for a steady stream of nurses and doctors who had never seen this before.  It seemed we'd found something that could be of help in the future.  And then Monday night rolled around.  Just as we were sitting down for dinner Annie sprung a bloody nose.  On went the sticks.  Next thing I know Tom's grabbing a bowl into which she started spitting blood.  This bloody nose seemed to be dripping down her throat and into her mouth.  It really looked horrifying and we debated between 911 and rushing to the hospital.  Hospital won out so off went Tom and Annie.  Again after hours of pinching and waiting they finally got a platelet transfusion and came home after midnight.  There was a desire to also give her hemoglobin but that required admitting her to the hospital and they'd had enough for one night.  I now wonder, were the sticks the problem?  We'll be going back to pinching and then maybe segue to the sticks to experiment next time.

We still had to go back Wednesday for the hemoglobin transfusion.  After the hours it took to wait, register, wait, triage, wait, get poked, wait ... it was learned that her hemoglobin had gone up and she wouldn't need a transfusion. Yeah. Again we witnessed the pattern of curbing her bleeding with platelets resulting in keeping her hemoglobin longer.  Catch 22 - they don't want to transfuse platelets but that means we need to transfuse hemoglobin more often.  Nonetheless we have a break, at least in terms of the schedule, until next Wednesday.

Meanwhile we're making appointments to meet with transplant teams and trying to get her a prescription to a medicine called Eltrombopag.  Tom read about it clearing clinical trials. It's for in people who have trouble making platelets.  The doctor said Annie could try it but we're on hold waiting for them to figure out the proper dosing...wait...wait...wait.