Happy Juice

Annie made it through her biopsy without trouble.  She has had seven of these over the years and it is always interesting to hear what she has to say after they give her the "happy juice" but before she falls asleep.  I've tried asking her things like, "where did you hide your sister's diary," but she just giggles.  Last biopsy she sleepily told Tom he had four eyes.  Today was a case of truth is stranger than fiction.  When the doctor asked her what she did on her vacation she said, "I swam in the pool, went to the beach and was peed on by a tiger."  While the doctor thought this was very silly it was, in fact, true.  We did visit a caged tiger that was agitated and pacing and, unbeknownst to me, Annie was growling at it.  (She later explained she was trying to talk to it.)  The tiger turned, lifted it's tail and sprayed her head to foot.  While it's a great story now she was fairly traumatized at the time.  We did conclude however that it had to be good luck as how many people get peed on by a tiger and live to tell about it?  Next doctor's appointment April 22nd.

Photo Shoot

For the most part Annie sailed through her round of tests today.  She'd say her least favorite part was having to drink 1000 ml of water with iodine for the above CT scan.  Although there was some bribery involved it was soooo much easier getting her to do it than when she was four-years-old.  She did top off the day's schedule with a platelet transfusion as her level wasn't high enough for tomorrow's bone marrow biopsy.  Guess I got a bit too smug as, no sooner had I texted Tom that we were wrapping up a successful afternoon, a bloody nose started.  Luckily she was simultaneously getting platelets so it didn't amount to much.   Biopsy at 10 in the morning...no food and drink until after.  Will report afterwards.

Countdown

For the most part no news was good news these past few weeks.  Over four weeks Annie had only two  bloody nose crises but we didn't need to go to the ER for either.  She also only had one full day of transfusions.  If she keeps to using a saline spray every 30 minutes her nose seems not to dry out and crack.  We even managed a nice vacation and Annie celebrated her 12th birthday.

Now we switch to medical mode and all plans start to revolve around her care.  This Thursday she'll have an IV and blood testing, a complete ventilatory study, NM renal w/ GFR, X-Ray chest PA and lateral, CT oral contrast, X-Ray for bone age, CT neck, chest, abd and pelf, CT brain, CT sinus and echo/EKG.  Then Friday she gets a bone marrow biopsy.  April 22nd she has measurements for her total body irradiation, doctor's appointment and maybe transfusions.  May 2nd a day of transfusions, May 3rd surgery to install a port and May 5th she gets admitted.  That's a line-up we'll surely be taking one day at a time and trying not to think about any more than necessary.  Will update after this week's procedures.

Optimism Bias

It's one thing to try and convince an adult they have optimism bias but trying to convince an 11-year-old is, well, optimism bias.  We're currently struggling with bloody nose relapses.  After two or so weeks with the new protocol it was tough to convince Annie she needed to apply gel three times a day and spray her nose throughout the day and keep up the incessant water consumption.  Things got lax, the air got dry and the bloody nose came back.  We got it under control again but then after a smooth period the push back resumed.  "Mom, my nose is fine.  I don't need the spray.  I can tell."  Uh, huh....another bloody nose.  "Mom I should always listen to you.  I promise I won't fight you."  Great but then a few days later....  We're waiting out another bloody nose now and weighing when to get the next transfusion.

Her test schedule has been made and it's quite an itinerary.  On April 11th she has 9:30 labs and IV, 10:00 Pulmonary function test, 11:00 GFR, 12:00 Chest X-ray, 1:30 CT prep, 1:45 Bone age X-ray, 2:30 CT scan, 3:30 EKG & Echocardiogram, next day 10:00 bone marrow test.  Can't wait.

Another Tour of Duty

Annie's donor has graciously agreed to our May transplant date so now the countdown officially begins.   Annie will be admitted Sunday, May 5th with the first of the what I'm calling the "twin towers of doom" to begin on Monday, May 6th. This first "tower" involves the process of killing off her bone marrow with chemotherapy and radiation in preparation for replacing it with the donor's marrow.  As with her other hospitalizations, it seems like heading off to war except that it's a child going and we can only provide company and bear witness.  For the four weeks prior to her admission she will go through a barrage of tests to check for any problems that may be lurking in her body and further complicate the procedure. When not attending to these matters we'll be trying to give Annie as normal a life as possible.  She had a bleeding event last Friday but we were able to stop it after a couple hours and didn't have to take her to the ER.  No bloody noses since then.  Her next transfusion is scheduled for Thursday.

A place for everything and everything in its place.

Annie's blood stayed put for once!  Since her visit with the ENT doctor she has followed his recommendations and there has not been one drop of blood.  Even on good stretches we had spots so this is quite remarkable.  Her counts were pretty low on Thursday (14,000 plt, 8.5 hmg) but we put off the transfusions until Monday (the fewer the better).  She is getting a bit cranky with the low hemoglobin but, being the weekend, she can relax.  We're about three months out from her transplant so it has appeared on our horizon and future plans are being shaped accordingly.  Next appointment on the 28th.

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.