Saturday, July 10, 2010

Taming the Black Beast

Le Bete Noire: the stuff of comics, myth and legend.  The Black Beast.

The late scleroderma researcher Dr. Carwile Leroy published an editorial in 1991 entitled "Pulmonary Hypertension: the bete noire of the diffuse connective tissue diseases."  He noted that while interstitial lung disease and renal crisis in scleroderma had some encouraging treatment options, pulmonary hypertension (PAH), "an especially destructive type of vascular involvement," remained unsolved.  PAH has emerged as the most severe complication and leading cause of death in scleroderma.

Treatment options have improved since this publication less than 20 years ago.  The primary change is bosentan, which is a well-tolerated oral agent used for PAH.

In my case, I have been on maximal dose of bosentan for over 2 years, so the rapidly rising pulmonary pressures cannot be improved through this medication.

My hope is that the stem cell transplant will stabilize the pulmonary pressures--along with the interstitial lung disease and worsening skin tightness--and allow me to continue a relatively active life.

I leave tomorrow morning for Chicago.   I will be admitted to the hospital early Monday for overnight chemotherapy, which is the first stage in the harvest process.  I will be on a new set of medications and get frequent lab draws over the next two weeks.  Late next week, probably on the 22nd of July, I will undergo stem cell harvest.

The goal: the only bete noire in my life will be a dark chocolate cake, flourless and rich... and my only problem with it will be the calories!  :)

4 comments:

  1. Holly, you will do beautifully. This is a good thing. All our love and best wishes, and please keep us posted if you can! We think of you always. Mary

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  2. Love you dearly, Holly. I know you will Slay the Black Beast and will return to rule the Land of Court! Curtis

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  3. Holly, you have the prayers and good wishes of so many family members and friends behind you. I know your positive, strong spirit will see you though this next hurdle. Thanks again for keeping us aware of what is happening with you through your blog.
    Love and hugs,
    Elaine

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  4. Love you to pieces, Holly. If love will beat the Black Beast, you shall win the battle.

    Chemo Sabe

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