Intrigue

Today Annie had a hemoglobin transfusion and we finalized our plan for a transplant date.  After collecting all our statistics and having the weigh-in from various sectors, we have decided to go ahead and try for a May 13th date for hospitalizing Annie to begin her transplant process.  Of course our plans are meaningless with this condition and we can only hope that things work out.  Annie's donor has graciously agreed to the new date.

There are protocols to donating bone marrow and both us and the donor are not permitted to know each other until a year after transplant and even then it is a careful process.  Based on stories from friends with donors this person often becomes a significant person in your life.  Tom, Annie and I have all had our DNA typed for match purposes and we had to give our ethnic breakdown.  To our knowledge we were a mutt-mix of Northern European countries.  We also believe Annie's donor is European.

But this is where the intrigue comes in.  Annie inherited one VERY rare allele from Tom.  So rare that there is statistically no chance she'll get a 10/10 donor and our 9/10 is the best we can hope for.  Well this rare allele is only statistically present in the people of Colombia in South America!  Sounds to us like we've unearthed something fishy in Tom's family tree.  You can bet we'll be doing a lot of speculating when we get together with family this Christmas.  Next scheduled appointment is in three weeks.  Thanks to all our support system and best wishes for the holidays.

Wonking out

My head hurts.  We're back in decision-making mode and it's slow going because we each bring up decision-models, statistics, breaking things into percents, grouping vs not grouping....  In the end we'll have made a well thought-out decision but for now it's tedious!  The cause of our decision-fest is that, since Annie's need for transfusions has dropped due to her taking "smurfs," we can consider waiting until May to have her transplant.  Her counts today were 19,000 plt and 8.8 hmg and her next appointment is in two weeks.  She feels good and gets through her days successfully, for now.   On the flip side a potential donor, albeit not a perfect match, has been located and we could start her hospitalization as early as January 2nd.  There are pros and cons to each date and a variety of things to consider, thus our decision summit.  As with any thoughtful decision-process we'll need to fill in gaps by getting more information and we'll need to let it all sink in to see if our decision stands up over some time.  Therefore we'll post more about our timeline once we have things better nailed down.

Losing steam

Annie didn't quite make it to her appointment on the 21st.  We could tell her hemoglobin had dropped very low as she had to sit down after relatively short walks or trips up a staircase.  By the end of the day she didn't have the energy to sit up through dinner.  Thus we took her in for a hemoglobin transfusion yesterday.  Sure enough her hemoglobin was way down at 5.6 (the desired range is 11.5-15.5).  Her platelets were stable at 20,000 (desired range 150,000-350,000).  Although stable it wasn't enough to hold back two, two-hour long bloody noses that occurred since the last update.  We didn't see her doctor since we had to go for an unscheduled transfusion instead of our scheduled appointment.  We'll meet with her doctor next Thursday and update after that.

Stable

Annie's blood test today showed her blood counts being stable from our last visit.  Her hemoglobin was 7.9 last visit and 7.4 this time.  While for a healthy adult this would leave us passed out on the couch, Annie's body has adjusted somewhat to these low counts and she is functioning pretty well with them.  Her platelets were 21,000.  Last visit was odd in our getting a range of values but nonetheless this is a nice number for what we've had for the last year (albeit normal starts at 150,000).  There is no news yet on the donor search as to be expected.  Thus we were able to leave without a transfusion and the doctor didn't think we needed to come back for another two weeks.  Thus, unless Annie feels unwell, we will report again on the 21st.  Go smurfs!

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.

Bad to the bone...marrow

No surprises today, the doctors recommended we begin the process of an unrelated bone marrow transplant for Annie as the ATG seems to have not worked.  She knew this was coming and thus was calm.  Our first job is to select a hospital and transplant team then begin the donor search.  Their hope is to transplant within three months.

There is nothing pleasant about a bone marrow transplant, you wouldn't wish it on your worst enemy.  However we have learned that being miserable about it just makes it worse.  Thus we will continue to squeeze whatever good things we can into the cracks between realities and spin, spin, spin a tale for Annie's benefit.  She has always been happy to wring whatever fun she can out of it all as evidenced by her rendition of "Bad to the Bone."  This is the last of her three-verse parody.

Getting heavy

Thursday's blood counts were much of the same, platelets <5000, hmg 8.2, ANC 1300.  We're in a holding pattern where she seems to need a red cell transfusion every two weeks and, while we've staved off the platelet transfusions, the black and blues and minor bleeds are building up.  The other thing that's building up is the evidence that the ATG treatment did not work.  Friday is the day we meet with her doctor and, probably, discuss the path to another transplant.

Before I could post this entry Annie had a "bleeding event" in the form of a four-hour nose bleed.  Thus an unexpected trip to the hospital for platelets and hemoglobin today.

Beads of Courage

While we try to focus on anything but Annie's medical condition when possible, sometimes it denies her the credit she deserves for all she weathers.  Luckily the organization "Beads of Courage" helps acknowledge the special "accomplishments" of kids with serious illnesses.  Like an olympic medal, it provides recognition for all the hours, days and years of pain and sacrifice endured to master mind and body in pursuit of a challenging goal.  The three strands above only document her experiences for two years.  She has four additional years of up to five beads a day for taking medicine, 1-3 beads a week for having blood draw appointments, 1-9 beads an appointment for pokes, another five beads for hospital stays, another four for bone-marrow biopsies, over twenty for transfusions and another six for other medical procedures like MRIs.  

It's a fine line between encouraging Annie to define herself beyond her illness and forgetting to honor all she has overcome.  Thus we'll recommit to stringing Annie's beads and celebrating all that she accomplishes not just the conventional things.  So for today beads for ... 13 pills (so far), one hospital visit, five pokes and one hemoglobin transfusion (hmg 7, plt 6,000).  Next scheduled blood draw in a week.

Gone Fishing

Time to catch up again....  Since Annie needs to stay close to the hospital as we don't know when she'll have a "bleeding event" and need a transfusion, there are no plans to travel this summer.  However, considering being her caregiver is a 24-7 job, a little R&R is in order.  Thus we must split up so as to keep one parent on duty.  Katherine and I took our break last week so, although I'm behind on the updates, I'm caught up on sleep.  Thursday's appointment required hmg as it had dropped into the 6 range.  Platelets were 6,000 but, again, they don't want to transfuse those unless she is having a significant bleeding event.  Her next labs were for Monday and hmg 8.9, plt 6000, ANC 1200.  No transfusion but she did have a nosebleed that evening that took four hours to contain and poor Tom was on his own.  They increased her cyclosporin levels so another poke Friday to check those values.  We've kept her nose to a slow ooze or nothing and her mouth seems quiet at the moment.  She has been feeling pretty good and keeping up her activities for the most part.

In a pinch

Annie did not turn out to have especially low energy or moodiness and, while we were pinching her nose off and on all weekend to control bloody noses, she didn't have the usual signs of extremely low platelets.  Thus we decided to forgo a hospital visit Monday and instead just have her blood drawn and tested locally.  Sure enough her counts were still high enough to wait longer before transfusing.  Hmg was 7.1 and plt 11,000.  While nothing to write home about, the slowed rate of decline is encouraging.  It has been three weeks since she's had a hemoglobin transfusion and we were barely making it a week at the height of her bleeding events.

Our current focus is how to prevent bloody noses since we've had to pinch upwards of an hour in the middle of the night to get them to stop and that's no fun for anyone.  After getting up and down to pinch Saturday night we knew we needed to step up our efforts.  We brainstormed a list of everything we could do to prevent a bloody nose including having someone watch her every waking moment, as she seems to be unconsciously upsetting her nose.  After laying out the entire plan I head off to sit in her bedroom and wait for her to wake and just as I approached her room I heard her blowing her nose in the bathroom.  Noooooooooooooooooooo!  All that pinching the night before and the healing time after was now wasted and we were back at square one needing to pinch and then let the nose heal.  Mind you she has been instructed not to do this kind of thing however it is like telling someone not to itch a bug bite.

So we began nonstop monitoring Sunday to make sure she didn't touch her nose in any way.  We wheeled her humidifier around as she went from room-to-room.  We drilled her on not touching her nose without speaking with us first.  We cleared four hours then six, then, when we weren't expecting it, she came flying across the house running after a friend and being rambunctious.  Not a bad thing, unless you are trying to keep your body quiet to let your nose heal.  So, sure enough, shortly after it started bleeding again.  Humph.  Luckily we contained the bleed with less pinching and it has been only short, infrequent episodes of bleeding since then.  The best moment was last night when around 2 AM she called out, we spring into action, rushed to her room and she calmly asked, "May I blow my nose?"  No blood, just the question.  Regardless of the time, we were happy that she was getting with the program.  I can't help now but wonder how many of those nocturnal bleeds had been absentmindedly provoked.  So we're tiptoeing until Thursday when we go to the hospital in expectation of needing a transfusion.

The Little Dutch Boy

You would think Annie's body would want to keep its blood on the inside like the rest of us.  I mean she has no platelets, it shouldn't go bleeding all over the place because it cannot be stopped.  However we get one spot plugged (teeth) and the next one seems intent on springing a leak (nose).  We thought we had the nose thing under control.  We humidify her room each night so its like a tropical jungle - dripping with moisture.  She slathers her nose with vaseline to keep it from cracking.  She sets her watch to go off every twenty minutes all day long to remind her to drink.  We go ballistic when she touches her nose, which is hard for her not to do as it gets caked with dry blood.  But since the teeth cleared up the nose has been a real pain.  Back to waking up with blood everywhere.  Thus we're at the hospital for platelets today.  However the efforts with stopping the gum bleeding and the prior nose respite had given her hemoglobin a fighting chance.  Even today we are able to hold out.  Plt at 7000 and hmg 8.2.  Not a bad drop from last Tuesday.  If we could just plug the dyke!  So the plan from here is ... first figure some way to stop the bleeding, then a blood draw and probable hemoglobin transfusion Monday and, hopefully, hold out until next Friday for platelets.  Stretching out the hemoglobin transfusion means an alternately quiet then cranky child until then.  Must practice my positive thinking, "I'm so glad she'll be cranky for the next six days because ... it will help build my tolerance for cranky people."  Hmmm funny how I missed putting that goal on my bucket list!

I am the walrus

Time to catch up....  The dental visit went well as these things go.  Annie was concerned about the pain but decided to forgo the nitric oxide and tough out the tooth removal.  She was pleasantly surprised how easy and low pain it was compared to her other regimes.  Score one for the dentist.  In the mornings her pillow continued to be relatively free of blood stains and she seemed to keep her hemoglobin around better as evidenced by better moods, energy and color in her cheeks.  This observation was confirmed when, on her Tuesday blood draw, she still had hemoglobin of 10.1.  The platelets were 7,000 so we decided to transfuse to protect the hemoglobin.  Luckily that means only a few hours at the hospital versus the seven to nine when she gets both transfusions.  Also that high of hemoglobin allows her to go until next Tuesday before another blood draw.  Since then she has been attending summer camp where she helps younger students as a TA and takes a woodworking and an archery class.  She really enjoys archery as she hasn't been able to participate in most sports due to her need to be kept from getting hit by balls or people as well as her low energy.  So until next Tuesday hopefully the only pokes will be on the bullseye!

CSI-case of the tooth fairy

While I try to keep this blog G-rated, some information requires a bit more of the gritty details.  The bloody show covering Annie and her bed each morning was becoming quite alarming.  It seemed there had to be more than low platelets at play.  Plus she was requiring transfusions more frequently and we had hoped her ATG was going to stretch out the need.  Her nosebleeds seemed under control so the blood had to be coming from her mouth.  It seemed an expert look was in order.  Her lovely dentist was able to see her shortly after my call and ... sure enough, three loose teeth!  After consulting with our doctor-cousin, we learned that yes, the blood loss from the mouth as well as her bruising would cause her hemoglobin to drop more rapidly.  Ah so the mandate to hold off platelet transfusions falls through if the bleeding is causing a need for more hemoglobin transfusions.  Thus off to the hospital last Friday for platelets and an appointment for more today in preparation for tooth extractions tomorrow.  The increased platelet fixes, plus regular use of a clotting medication, has kept Annie and her bed blood-free since last Friday.  Even better, her hemoglobin dropped at half the rate it had been.  She still needed a full, nine-hour day of transfusions today but it sure felt good to know there was a reason behind the increased need for hemoglobin.  We had declared the treatment a failure but this gives a thin thread of hope that maybe progress was being masked by the teeth exodus.  Next appointment on Tuesday.  Until then we'll be readjusting to high-hemoglobin Annie.  This Annie is ravenous and a bit hyper - a stark contrast to the droopy, lack-of-appetite girl of late.

AA & MDSIF

Aplastic Anemia got a little less unknown recently with the public announcement by ABC correspondent Robin Roberts that she has MDS - another bone marrow failure condition.  Research and advocacy for both conditions are supported by the Aplastic Anemia & MDS International Foundation.  We have attended several of their conferences and have been impressed by their active support of providing research grants.  Their website is http://www.aamds.org/  

Katherine's 8th grade class donated some of the money they raised over nine years to support this foundation.  This is a clip of Katherine introducing the foundation and later of Annie accepting their donation on behalf of the foundation.

Annie's results from Thursday's blood test came in Friday at plt 9, hmg 9.1, anc 530.  We had hoped to go until next week before a transfusion.  Unfortunately Annie has had trouble with bleeding in her mouth and last night it became clear she'd need a transfusion today.  She and Tom went to the ER this morning.  I am now convinced that part of the ER doctrine is to discourage anyone from going to the ER.  In our experience everything there takes ten times longer.  Thus although they went at 8:00 this morning and were immediately checked in, they are still waiting for a transfusion.  She has been admitted to the main hospital so this could be stretched into the evening.  They required her to have another CBC and her hmg has dropped to 7.1 so they'll transfuse both.

Now we know if we have any hope of getting through this intact we have to look for silver linings in everything.  So this one's is we're glad to take care of this now so Annie won't have to miss any of her week-long robotics training next week.  She has signed up to be part of the school's first robotics team and had been concerned about missing the training for transfusions next week.  Thus hopefully I won't have to write again before a week from Monday.  As always many thanks for the thoughts and prayers.

Waiting for the GPS to restart

Playing catch up again.  Thursday's appointment revealed Annie needed both platelets and hemoglobin but there wasn't time for both.  Thus she got platelets on Thursday, the needle was kept in but bandaged, and she got hemoglobin Friday.  Next blood test Thursday.

The basic conclusion from the doctor's appointment was that we haven't seen the desired results from her treatment yet but, with the medical team's vacation schedules, it will be the beginning of August before making conclusions.  So, if there is no last minute progress, that means planning for another transplant.  Since, over the years, we've burned through most of the emotions surrounding all this, we'll attempt to take whatever comes in stride.  Until then it's blood tests, transfusions and appreciation for what's going right.

Learning to dance in the rain

We've been a bit mixed around between graduation activities and coordinating Annie's appointments but I finally have information to post.  Her counts Monday were plt 9000, hmg 10.1, ANC 890 and CSA 251.  Her "bleeding events" are becoming problematic so we have a platelet transfusion planned for Friday and a doctor's appointment tomorrow.

Being graduation season I am reminded of a student's yearbook quotation which has helped us a lot.  It is, "Life is not about waiting for the storm to pass, but about learning to dance in the rain."  This is one of the things Annie's condition has taught all of us - that you can't wait for everything to be "right" to find things to be happy about.  I think this has helped Annie stay positive in spite of the challenges.  That's her above doing a tap dance to "Singing in the Rain" with her friend in the school talent show.

Thus, although we're still waiting for some sign things are improving, we're all choosing to be happy anyways.  If we learn anything telling at our appointment this week I'll post, otherwise we'll wait until her next blood test, early next week.

Thanks and congratulations to all our graduates!

The leopard look

Gratefully Annie made it through to Tuesday without any emergency visits to the hospital. However her body is more than ready for a transfusion.  Due to her low platelets she is sporting a leopard look these days from all her bruises and petechiae.  It's not just Annie that looks like a leopard, it's also her bed.  While bloody noses throughout the day get managed with pinching and tissues, at night they drift over sheets, blankets, pillows and skin.  Even after six years I still haven't completely desensitized to walking into my daughter's bedroom some mornings to face something akin to the scene of a massacre.  A leopard cub on leopard print sheets.

Today's blood counts are plt < 5,000, hmg 6.2, we'll get ANC and CSA later.  Thus we're settled in for a full day of platelet and hemoglobin transfusions.  Assuming no upsets my next report will be Monday.

Zip-a-Dee-Doo-Dah

Well she made it through the Girl Scout camping extravaganza which included seven runs on the zip line!  She is on the left in the video above.  Luckily no bloody noses and, while she had a few extra bruises, they were no worse than what she manages to get even when we keep her home. She did have to follow her own program for a few activities such as skip hikes and stay outside the mine excavation (one of her greatest threats is a fungal infection).  Today's blood counts also showed that enough of last Wednesday's transfusion is sticking around to hopefully avoid a transfusion this week.  Hmg 9.2, plt 11,000, ANC 760 and CSA 240.  Hopefully we can skip the second blood draw this week.  So, if all goes well, I'll post after next Tuesday's draw (as Monday is Memorial Day).

On my honor I will try ...

These days it's all about getting Annie to her Girl Scout trip.  Her health prevented her from attending her class trip to Joshua Tree but she was adamant that she was not going to miss the annual Girl Scout trip.  We planned tomorrow's dental cleaning just so that she'd have to have a platelet transfusion right before the trip as her high point of last year was riding the zip line.  This is no easy feat for a body with low platelets nor a nervous mother.  However these are the things Annie fights for, the things that get her through the rough spots and we try not to let fear or reasonable risk take that from her.  We thought the hemoglobin from last week would still be with her but no go.  Today's counts were hmg 7.6, plt 8,000, ANC 780 and CSA 192.  We got the medical team behind giving her a red cell transfusion in addition to the platelets so she had the best chance possible of successfully making it through her scouting trip.  Thus the short hospital visit we had planned turned in to a full day but now she's tanked.  Here's to hoping that my next posting will be after Monday's blood draw and it will include a picture of her on the zip line - a true miracle for a kid with aplastic anemia.

Bottoms up

Annie's appointment today was pretty uneventful and, for us, uneventful is good.  Still nothing to conclude about the treatment so far and her chemistry panel from Monday was fine except for her BUN was high and this means she needs to drink more water.

Making sure Annie drinks enough water has been a major part of our lives for the past six years.  Being on high doses of cyclosporine puts you at risk for renal failure and drinking a lot of water is the only protection.  Annie is part camel and would prefer not to drink all day so this adds to the fun of keeping her hydrated.  For a while I used to go to her school every lunch and snack to make sure she washed her hands, drank water and stayed out-of-trouble.  Luckily she has taken on some of this responsibility as she has gotten older however we still have to stay on her throughout the day.  At home we set a timer to go off every 15 minutes to remind her to have a swig of water.  At school it's hit or miss.  We try to set up systems, leave notes, and hassle/scare/bribe her in to compliance.  The alternative is unacceptable so I guess it's time to expand our efforts.  Next time you see Annie maybe ask to see her cool water bottle and if she'll show you how it works :)

Her CSA was 229 so we're off the hook for a blood test until next week.  However a side-effect of an oral rinse she has to use is stained teeth.  This has really begun to bug Annie as her friends notice the discoloration so its time for some stain removal.  Dental appointments have to be timed to coincide with a platelet transfusion and antibiotics.  Thus next Wednesday she'll have a blood test, antibiotic dose and platelet transfusion followed by a dental visit Thursday.

So until next Wednesday we'll hope for lots of hydration and keeping Annie healthy and out of trouble.

Playing chicken

We finally had to swerve this morning in this sicko game of chicken we play.  Annie's symptoms from low platelets (bleeding gums, bruising, petechiae...) became so untenable that we had to bring her to the hospital for a transfusion this morning.  Her plt are the lowest recorded value <5000, hmg 7, ANC 540 and we don't know the CSA yet.  Thus she is getting both platelet and hemoglobin transfusions today.  The refuel two weeks ago gave her a pretty good run, and us a bit of a breather, so hopefully that system will work again.  We have a meeting with her medical team Thursday and will report again then.  Thanks for the continued prayers!

Still floating

We've had relative calm since last Tuesday, phew!  Her CSA level was just out of range so they agreed to let us skip Thursday's blood draw so we could give her veins a break.  This is nice not only from a logistics standpoint but also because it relieves us from the unpleasant experience of waiting for the nurse's call giving us her blood counts.  Also there doesn't seem to be much enthusiasm about inserting a line at this point so giving her veins a break was pretty important.  Today's blood draw showed she is still floating down from last week's big refuel but doesn't need another transfusion yet.  Her numbers were hmg 9, plt 9,000, ANC 360 and CSA 332.  We were given the option of waiting until next Monday to test again and we gladly accepted.  Thus we'll hope her body makes it until then, hope, hope, hope....

Grrrr

Although we got all the transfusions in yesterday the level of her cyclosporine was too high (414 and we're trying for between 200-300) so back for a blood draw today and probably Thursday!  Luckily the phlebotomist found a vein on the first poke this morning.  Hopefully our luck and those veins will hold out.  Still researching the internal port options as there are notable disadvantages to each option.

Fill 'r Up

Although Annie kept it together for the weekend she was really running on empty.  Today's hemoglobin was the lowest we've seen (although that's just because they transfused before this point at the other hospital).  The stats are hmg 4.6, plt 7,000 and ANC 240.  So today she's having a major refueling - a bag and a half of red and, hopefully, a bag of platelets.  I say hopefully because she is having trouble tolerating the transfusions and they have to slow the rate to the lowest speed.  This ward closes at 6 p.m. so if she can't get it all in she has to come back tomorrow.  All these pokes have become a problem and she's running out of viable veins.  So again we're looking into a more permanent line.  It's hard to know which type, if at all, since we are still midstream in this procedure and different types are better for different situations.  We'll be gathering information and weighing options over the next few days.  In the meantime we hope to increase her transfusion rate and get her finished today.  We're also hoping to wait until next Monday for labs since her medication levels seem to be in range and, with today's refueling, she should make it a week.  So I'll post if we learn anything new or make any important decisions otherwise no news means she fueled up today and is keeping up her routine until next Monday.

Flip flop

So normally it's us trying to convince the medical team Annie should get a transfusion but today that was flip-flopped.  We got a call in the morning saying Annie should come in for a transfusion as her hemoglobin was down to 5.4 (her platelets were 34,000 and ANC 370).  We couldn't believe it was so low as she had made it through three days of school without complaint and was like her old self last night dancing around the house and singing - something we haven't seen in a couple months.  We were certain her hemoglobin had gone up; why we thought we should feel certain about anything considering our track record is beyond me.  Any how we explained our logic to the nurse and we all agreed to make a transfusion appointment for Monday and have her blood draw at the hospital so she has only one poke.  Thus we'll keep a close eye on her this weekend and hopefully not need to make any hospital visits until then.

Iron Overload

Couldn't pass up the opportunity to add a little more eye-candy to this blog but Annie does have "iron overload." One side-effect of multiple hemoglobin transfusions is an over-accumulation of iron in the liver.  So last night Annie had a previously scheduled MRI appointment.  Thus she spent 50 minutes in the noisy "doughnut" watching the movie Tinkerbell.  A pretty ho-hum hospital visit by our standards, thank goodness.  Her blood draw this morning was also uneventful however they won't have the lab results ready until some time tomorrow.  Thus all quiet on the western front for the moment.

ER

It's just because it has been 24-hours and I am so grateful to have had dinner and be in my house that I can  consider writing about last night.  Now maybe if George Clooney had been our doctor things would be different but....

6:30 p.m. Sunday night, putting dinner on the table and Annie starts crying in the other room.  It's the "bleeding event" we've been dreading.  We alternated pinching her nose for 30 minutes but it kept bleeding, really badly.  So we called the hospital ER and arrived by 7:30.  Although we got a bed pretty quickly it wasn't until 9:30 that she got her platelet transfusion.  Three hours of bleeding and pinching.  Three pokes.  Six hours since anyone had eaten.  Nightmare.  Finally home at 10:30 p.m.  No dinner.  Everyone wiped out.   Blood counts were plt 8,000, ANC 490, hmg 7.1.  Really nothing nice to say except I'm glad it's over.  Next post Thursday, I hope!

Chaos theory

Annie ended up having a red cell transfusion yesterday and, when not scheduled, these take a long time.  Including driving time, it was a nine-hour process.  Since we've been in this phase for a while now some patterns are emerging.

Based on research with cancer patients (who also often need lots of transfusions) transfusing platelets, unless the patient is uncontrollably bleeding, actually impedes the body's ability to produce platelets on its own.  Thus this medical team is comfortable letting her platelet level go to "zero." This is new ground for us and taking a bit of an adjustment.  Similarly there are risks to receiving red blood cell transfusions, as well as the body getting lazy about making cells itself, so the team wants to wait until she feels crummy to give those.

The inherent problem with this plan is that it means we go along until the next traumatic event happens.  Then chaos erupts with a bleeding or pained child, last minute changes of plan, fighting traffic, trying to find a nurse and bed at the hospital....  When I presented this reality to the doctor he basically acknowledged that it's just how it has to be.  Nonetheless the rest of the appointment was summed up by, "it's too early to draw any conclusions."  Annie did get weekly transfusions for two months the last time she had ATG so we're going to be patient and wait until the end of May to start evaluating whether this treatment was effective.  So until then we'll keep tip-toeing through this kind of haunted-house, always on alert for when the next scary event will send us scurrying.

How low can she go?

Well I wouldn't say we're having a limbo party but Annie has been enjoying her stay-cation.  Although she has been in a good mood, has not been sick or had headaches and is not actively bleeding, her counts were basically down again today... plt 6000, ANC 270, hmg 5.7.  She is also checked for the level of immunosuppressive medicine in her system with a test called CSA.  They want that between 200 and 300 and it has been around 150.  Thus they increased her dosage and it was at 311 today.  Since she has no active symptoms we'll pass on tomorrow's transfusion appointment however she still will meet with her doctor in the afternoon.  Will post what we learn tomorrow.

Thus I'll sign off to the beat of Chubby Checker's Limbo Rock..."Every limbo boy and girl, all around the limbo world, Gonna do the limbo rock, All around the limbo clock, Jack be limbo, Jack be quick...."

Stay-cation

Somebody forgot to pray... Sunday morning Annie woke up sick.  First a headache, back to sleep.  Then a stomach-ache, back to sleep.  Then threw-up and ... well by this time we must have had a critical mass of people praying at Easter mass as then she felt, great.  In fact she spent the rest of the day munching and playing with her cousins.  Roller coaster.

Today's labs were back down.  Plt 8,000, ANC 450, Hmg 5.6. However, since the medical team doesn't want to transfuse unless something bad is happening we plan to try and stretch it out until Thursday.  Annie is having a stay-cation at home and seems quite happy to eat, listen to books on tape and recline. Thus about the most low-risk days we could conjure up.

No news is good news and will report Thursday's results.

An ominous cloud

Two nights ago Tom became feverish and since then has been in the throws of a flu.  While we all get our flu shots first thing, there are many strains, and things still sneak through.  He has been sequestered away from all of us and I look like someone out of the movie "Contagion" when I enter his room.  I wear a mask, rush in, drop off food, leave and disinfect myself.  The problem is the worry of whether we got infected before we knew he was sick.  Thus lots of extra healthy habits and visualizing killing off any virus before it multiplies.  So it's mostly learning to cope with the specter of the rest of us, especially Annie, getting sick.  We were told five days would be the incubation period so if we can make it to Easter hopefully we can breathe a sigh of relief.

Annie is spending the day getting her red blood cell transfusion.  We are in an area we've never been in before.  It is called "Fast Track."  Sounded promising to me until I discovered it was just the child-friendly name for Urgent Care.  So much for the drive-thru transfusion I was imagining!

Well the roller-coaster twisted again and we were just handed her lab results.  Her hemoglobin went up a bit to 6.6 so they want to not transfuse.  ANC also up to 540.  Plt down to 31.  Also tapering off prednisone...bye-bye puffy cheeks.  Thus we're going home.  Called to tell Tom and his fever has broken.  So just the specter remains until we learn more after Monday's labs.  Hopefully we'll have nothing to report until then.  No more flus developing would be a great prayer - thanks!

Buddha Baby

I know you think this is a picture of a Buddha statue on the left and Annie on the right but it's an old picture and now Annie actually looks more like the statue than the girl.  And that's thanks to prednisone.  All the munching and puffing has temporarily physically changed Annie and all the recent experiences continue to mature her emotionally.

Today's blood test showed another drop in counts.  Hemoglobin down to 5.8, ANC to 400 and platelets to 45,000 however that number is due to her platelet transfusion on Friday.  Thus she has a red blood cell transfusion scheduled for Thursday.

While all this sounds unpleasant to us, she retains a joyful attitude and is genuinely happy in spite of it all.  Will report in after her blood test/transfusion Thursday.

If not one thing, another

As part of the roller coaster ride it seems some blood line is always causing a problem, but it rotates through as to which one.  Reds and whites and platelets - oh my!

Earlier in the week it was the ANC (part of the white count) that dropped and meant we sprang into home isolation mode.  This last blood test the ANC went back up to 540 (cleared for school) but the hemoglobin (reds) drifted down to 8.8 and the platelets are in the lowest, <5000, category.  Unfortunately this has led to two days of prolonged bloody noses (over 90 minutes each).  Today's required her coming home from school.  The medical team decided to transfuse so that is where Annie is at now.  Next blood test Monday.

Round and round and round we go where we stop nobody knows!

Roller coaster ride

One thing I dislike about keeping people informed of Annie's status is having to include others in this sicko roller coaster ride from which we can't seem to escape.  It seems we are barely able to catch our breath from one plunge before we shoot to a crest and then plummet again.  We try sooooo hard to figure out how not to get swept up in the vagrancy of it all but we've yet to unlock the secret to accomplishing that.

So, if you haven't guessed, we got the blood counts late today and they were down.  Not that this isn't exactly what is expected.  It will take months of volatile blood counts before we have a sense if things are working as we'd hoped.  It's just that her ANC took a nose dive from 2000 to 240 in four days. Now there is the possibility of lab error and white counts bounce around a lot but it still sets off a whole new set of logistics.  We have to switch to home isolation mode and virtual school.

Her other counts, for those who track this, are hmg 8.8 and platelets 8,000.  Her medical team wants to avoid transfusing so we'll hope for the best and see how her body responds.  The next blood test is Thursday and I'll update when we get those results - which sometimes takes a day.

I've had a number of people have trouble setting up an account so they get an email when there is a new posting.  If you would like me to set this up for you I can do it on my side.  Just email me or leave a comment and I will do so.  This way there will be no need to check for updates, they will automatically be sent to you.

Below the surface

Side effects of prednisone include moodiness and puffiness both of which we all got to enjoy this weekend.  However prednisone also hides the "serum sickness" lurking just below the surface.  So as we attempted to ease off the prednisone Annie's personal Loch Ness monster broke through the surface in the form of a hives-like looking rash on her face and body and some discomfort.  She also had the pleasure of blood blisters in her mouth.  By this morning things were getting worse.  She was always going to have to go to school late as her blood draw was scheduled for this morning but with the rash she just wanted to hide at home.  However by mid-morning the rash had faded and, since she was otherwise feeling well, we encouraged her to go to school.  

All day no matter what I was doing I worried in the background.  Then at 10:48 she called from school.  The rash was back.  Her classmates were worried and pointed it out to her.  The doctor said as long as she didn't have a fever she didn't need to go to the hospital and she felt fine so I encouraged her to stay.  All day I pictured her with a raw, red rash across her body and face.  I imagined how challenging it must have been to have people staring at her all day.  I drove myself crazy.  

I play this game to help me cope where I say, "I'm so glad...(something bad that happened)."  So I said, "I'm so glad she is experiencing what it's like to be disfigured, it will give her greater empathy for others with similar challenges, she'll really learn to value the good things hidden beneath the surface."  Crazy, right, but it helps.  Any how the moment came when I picked her up at school.  Anxiously I waited and then she bound into the room with a big smile on her face and her skin as smooth as porcelain.  For the rest of the day I've had a big smile on my face.

Ignorance is bliss and her blood counts won't be available until tomorrow.  So for tonight, seeing is deceiving, and I can pretend, for a little while, that all is good on the surface and below.

Absolute Neutrophil Count

First I must say I am shocked that someone would make a painting of an absolute neutrophil count chart but there this was on the internet.  I can, however, understand that someone would find beauty in their neutrophil count making a linear ascent to over 1000.  It is Annie's ANC that determines if she may leave the house or not.  If she may be in places where we don't know the health status of every person.  It is what kept her out of school for four months the last time she had immunosuppressive therapy.  So we were happy to learn from today's blood count that her ANC had gone from 540 to over 2000.  Anything over 500 means she can go to school.  Below that it's shut in.  This is quite a jump since Tuesday however she had a tickle in her throat this morning and the body ups production in response.  Whatever the case her doctor seemed pleased that her bone marrow got busy doing anything.  

Here hemoglobin and platelets stayed pretty steady at 9.4 and 22,000 respectively.  Since she has been transfused for both within the last week there is no way to know yet if any of that is due to her ability to produce and retain cells.

Most of the doctor's appointment revolved around tweaking medication doses to get her CSA (level of immunosuppressive medicine) up to the desired range while starting to wean her off the prednisone and blood pressure medications slowly so as not to trigger the serum sickness they are meant to prevent.  Since no red flags were raised during the meeting we were relieved.  Thus we'll try and keep her healthy and out of trouble, while enjoying as much of her normal routine as possible, while we wait and see if her body can produce adequate numbers of blood cells without destroying them.  This process doesn't happen over night but instead over months.  Her twice weekly blood tests will give us a glimpse into her progress.  Thus I will update Monday evening once we know her lab results from that day.  We are, as always, grateful for the support that has gotten us this far!