Aplastic Anemia Countdown – To Transplant I Go!

It’s getting to be crunch time.  The fact is, I cannot continue to be transfused indefinitely.  Blood and platelet transfusions for AAers are meant to be temporary measures that are only in place until they are no longer necessary and the immune system has recovered.  They are not meant to be the crutch that keeps us alive for the rest of our lives.  The immune system must be able to function on its own, and regular blood cycles must be able to sustain and maintain the body without medical intervention.  My body is just not doing it.

Aplastic Anemia Blog - Travolta

The Boy in the Plastic Bubble with dreamy 1970s John Travolta

Why can’t I be transfused indefinitely?  Well, remember the The Boy in the Plastic Bubble?  The TV movie from 1976 starring John Travolta?  That movie was based on the true story of Ted DeVita, who also suffered from Severe Aplastic Anemia.  He died at the age of 18 – not from his Aplastic Anemia, but iron overload from frequent transfusions (transfusional hemosiderosis).

I have been aware for some time that my CBC levels and blood production have plateaued.  I have been functioning at more or less the same level for nearly three months, with no significant increases in cell production and retention.  If the ATG and drug therapy were working, by now we would be seeing increasing blood levels and a reduction in the number of transfusions.  Obviously, this has not happened.

From an emotional standpoint, the waiting period from the administration of my ATG to this point has been agonizing.  For me, each CBC is fraught with tension and anxiety as I hope and pray the results will show improvement from the last test.  Lately, all my hopes seem to be dashed.  I was told shortly after my diagnosis that ATG takes a minimum of three months to work, and sometimes five to six.  I am about to enter my sixth month.

A week ago, I expressed my unhappiness with my progress to my physician and he agreed my situation was not promising.  We decided if there was no significant progress by August 1st, I would be put on the stem cell/bone marrow transplant track.  A few days later, after thinking through my situation, I asked my doctor if there was any rational reason why we should wait even until August 1st – a seemingly arbitrary date.  I have donors and I’m ready.  So, it looks like I’m moving forward with a transplant.

My Dear Readers, DO NOT DISTRESS for me!  I am very happy with this course.  In fact, if I had my druthers, I would never have had ATG to begin with and would only have gone to transplant.  Let me explain why.  ATG is not a perfect therapy.  As I explained many moons ago in my second ever blog post, the drug course only works to some degree in around 50% of Aplastic Anemia cases.  Often it only partially works, and the blood counts never quite return to normal.  On the good side, the “new normal” may mean a life free from transfusions.  However, the relapse rate is relatively high and patients live their lives with a permanently compromised immune system.  Therefore, they have higher rates of cancer, as well as other illnesses since they do not have a system that is working efficiently to destroy outside toxins and cell mutations.  In contrast, a stem cell transplant (SCT) – otherwise known as a bone marrow transplant – offers the chance of complete recovery.  That’s why the medical community considers it a cure.  Also, the success/cure rate with a transplant is considerably higher.

So, why not just send everyone with Aplastic Anemia to transplant?  Well, it is a very intense, invasive therapy that can result in devastating side effects in some patients.  However, I am a great candidate for transplant, despite not having a matched donation from my sister.  The success rate of transplants is much higher for patients in my age bracket.  I am not in the youngest section, but since I am only 30 I am still in the 21-30 age range.  Also, the matching process for unrelated donors has improved ten-fold in just the last eight years or so.  My Bone Marrow/Stem Cell Transplant Coordinator at Cedar’s-Sinai has already located great full matches.  At one time, they only matched six proteins.  Now they match 10.  In addition, the transplant committee searches for DNA compatibility.  These advancements greatly reduce the risk of Graft vs. Host disease (GvH), which is a relatively common complication with transplant patients.  Another odd in my favor with transplant is that I am in relatively good health with no underlying diseases causing my AA.  My organs are strong and healthy, and all my other internal processes are functioning normally and in some cases optimally.  Finally, we are almost certain of the etiology of the disease in my case.  By knowing to what I was exposed (sulfa), I will be able to take steps to avoid taking the drug again in the future and my relapse rate will be reduced almost to nothing.

So what does this mean?  Right now I am in a holding pattern until the transplant is scheduled.  Since the donors have been located, my Transplant Coordinator and the entire Transplant Department will meet to decide who is the most compatible donor and what course of preparative regimen I will require.  In order to prep the body for transplant, the remaining stem cells in my bone marrow must be destroyed.  My few white blood cells must be killed, and all dormant diseases and immunities must be wiped out of my system.  All my antibodies from vaccines and past illnesses (measles, mumps, chicken pox, etc.) will be killed and I will have the immune system of a fragile baby.  This is achieved through a combination of re-administration of ATG and chemotherapy.  I may also have radiation, but that has yet to be determined.  I am really hoping not to have the radiation, as it may destroy my chances of having children naturally.  More on that at a later date.

Prior to my preparative regimen (Hell Week) and actual transplant, I will undergo a series of tests, including a psychological evaluation to determine whether or not I am healthy enough to undergo the SCT.  My donor will also undergo testing, although less rigorous.  The donor has to be administered a general anesthetic to extract their bone marrow, so the actual transplant and Hell Week must be coordinated according to their schedule.  I will have five days of the chemo/ATG, one day of rest, and (deep voice) ON THE SEVENTH DAY, I will receive the stem cells from the donor’s marrow.  From then on, it’s a waiting game for the stem cells to engraft in my marrow and begin populating my blood stream with new, healthy cells.

Aplastic Anemia Blog - Gift

Please register to be a Bone Marrow Donor!

Bone marrow donation is an amazing gift.  It requires sacrifice on the part of the donor and is not something that my fellow recipients and I take lightly.  The donor must be completely anesthetized and their bone marrow is extracted through dozens of tiny holes in the pelvis.  Once the extraction is complete, the marrow is couriered to the recipient from wherever they are in the world – similarly to organ donation.  The marrow replaces itself completely within 4 to 6 weeks, but donors can expect to have a sore pelvis for 2 to 3 weeks.

I cannot begin to express my gratitude to the millions of people on the Be the Match donor registry, and to the person who will ultimately give me this gift.  Through their sacrifice and willingness to undergo discomfort and inconvenience, they will save my life.  Most people find it hard to find the time to drive friends to the airport, let alone donate part of their body to a complete stranger.  I am moved beyond words by whoever they are, and hope to someday thank them in person.  The rules are very strict about contact between donor and recipient, but one year post transplant, I will be able to meet them or at least speak to them.   My hope is that after I am fully recovered, I will be able to join the Be the Match registry and perhaps someday pay it forward to someone else in need.*

*Update – I have learned that transplant survivors are not eligible to donate bone marrow or stem cells.  While this is disappointing, I still hope to pay it forward in the way of volunteering for the Be the Match Foundation.

1 Comment (+add yours?)

  1. Dawnzo
    Jul 17, 2011 @ 21:57:56

    Best of luck to you, Katherine. Thank you for yet another clear description of a complicated subject that we are all now very concerned with. Sorry I missed you last week when I was at DWA! Hopefully I will see you soon. Wishing you the best on this next step. ❤


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