Whatever you say doctor...

We didn't make it to this week's appointment unscathed.  Last Thursday night we had a replay of what I described in the last entry.  Thus another night of transfusions in the ER.  Routine appointment Monday of this week and then an appointment with an ENT yesterday.

Turned out to be a long wait.  After an hour and a half we still hadn't seen the doctor (in the end we never did see the doctor we were scheduled to see) and then a different doctor came in - a young, handsome, male doctor sporting a Justin Bieber hair cut.  Well let's just say a spoonful of sugar does still help the medicine go down because Miss Annie was quite the attentive patient from that point on.  A nose spray that evoked blood curling screams from Annie when done in the ER now came with a scream that instead sounded like her singing a note in a song.  She allowed him to do that spray three different times.  God help the woman in the ER who did it, she would have been torn to shreds had she tried it again.

Nothing troublesome was found in her nose but we did learn some new hygiene things to try and keep her blood vessels from cracking.  Annie has been following the doctor's instructions to a T.  We're always happy to be hopeful that things will get better so that's what we'll do.  Next appointment a week from tomorrow.

Scary Movie

Warning, this is kind of a gross entry.  I'd say our lives can get to about 80% normal and we can even forget for a while what's coming but then we get a shocker of a reminder.  The most recent came last Thursday night.  I went to bed early only to be awoken by Tom at 12:30 informing me that he'd been up with Annie for three hours dealing with a bloody nose.  No problem, I was rested and could take over.  However an hour later (here's the gross part) Annie proceeded to cough up, in one big batch, over two cups of clotted blood.  Uh huh gross - like the stuff you figure must be fake in the movies.  Apparently she'd been swallowing blood for those four hours and her stomach was none too thrilled.  So it was off to the ER for Tom and Annie where they remained until returning home at 6:30 in the morning after a platelet and blood transfusion.  This was the second emergency hospital visit for this kind of episode and what's really unfortunate is that her blood counts did not require a transfusion otherwise.

At Monday's appointment we discussed this fact with her doctor who suggested we meet with an ENT to see if there was anything else we could do (we already use nose spray, tons of liquids, humidifiers, vaseline...).  That appointment is coming up on the 29th.  We also learned there is a new donor who is an 11/12 match.  Again we'll never get a perfect match but this is better in a few ways.  We are still waiting to hear if we are a go for May.  In the meantime, despite the drama, Annie popped back to her old self and has continued on with her activities.

In calling up this blog to update by mistake I typed in the address of an old blog that I had forgotten.  It chronicles her relapse and our Odyssey from 2007-2009.  I'm not going to read it, living it was enough, but for the record it is http://annietupdates.blogspot.com.

Next appointment still getting scheduled but probably a week from Monday.

Update

We didn't quite make it to today's appointment but, again, we chose to look at the good things this made possible.  First, the back story.  Upon picking Annie up from the last day of school before vacation she tripped over a tree root and landed on various body parts, including her face.  What a way to start what we were hoping was a few weeks of R and R.  While we didn't have to take her to the hospital, we did get the pleasure of seeing her with half a face of black and blues for the holidays.  Additionally this meant her blood counts would drop faster as her body lost blood to the bruising.  In spite of this inauspicious start things stayed pretty calm until last Sunday afternoon.  It gets pretty dry this time of year and this ups the odds of Annie getting a bloody nose.  Lately we've had the bad luck of her veins cracking so high up in her nose that pinching doesn't work.  She got one of these Sunday and things escalated to the point where we had to take her to the ER.  Luckily her new hospital was not crowded and she was in and out with both a platelet and hemoglobin transfusion in record-breaking time.  This also meant we were able to put off today's appointment until 1/13.  It all feels like we're on borrowed time as the original plan was to begin the transplant process yesterday.  I'm really glad we were able to put it off a bit longer as this would have been our most depressing Christmas ever knowing what was awaiting us the day after New Year's.  Next update hopefully after the 1/13 appointment.

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!