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.