Back in ACK (Day +779)
We are back in Nantucket (ACK, is the airport designator; like DFW or LAX). We flew up yesterday.
We were back at the Mayo about a week ago for Jackie's monthly checkup. Her blood counts are all within normal ranges. There is no sign whatsoever of CLL. That is the good news. As I have done all along I've shared the good news with the bad.
The bad news is that her GvHD continues to progress. It has turned into scleroderma. You can learn more about the disease here. In a nutshell it is a disease with no cure. The disease causes problems with the connective tissues within the skin due to excess collagen production. The skin thickens and feels like plastic. The sweat glands and hair follicles are choked off.
In Jackie's case the change has occurred very rapidly. The process began some months ago as the GvHD attacked her arms first, then her legs. The scleroderma is rapidly advancing and is beginning to attack her upper legs and thighs as well as her back. It is extremely painful. It is limiting her mobility. She has difficulty tying her shoes, walking, and climbing stairs. Wearing clothes is painful. She says it "feels like she is wearing a suit of armor and if she bends her arms or legs they will burst open". There is also a great deal of itching. She slathers herself in various creams and ointments including hydrocortisone cream; all with little or no benefit. She soldiers on but it is not a pleasant time for her. Due to the over prescribing of pain medications the doctors are hesitant to supply here with something to relieve her pain. I'm certain if they had to live one hour in her condition their views would be quite different! On Monday we will contact her doctor and beg for pain meds for relief. Tonight she took another one of the precious few tablets she has left.
The doctors have no idea of what will happen with regard to this problem. There is just too little data. They are not very optimistic about an improvement in the condition. It is possible that Jackie's hybrid immune system will eventually adjust and things will improve or return to a more normal state. It is also possible the scleroderma may progress further; only time will tell. I have to admit this is one possibility that I (we) never considered when we began the transplant. I suppose it beats the alternative of dealing with CLL. Almost certainly Jackie would not be alive today had the stem cell transplant not been performed. Hopefully this is just another bump in a very long road. The doctors tell us that over time GvHD has a tendency to subside and improve. Hopefully this is true of side effects such as scleroderma as well.
I assume that anyone still following this blog will be familiar with PC and Chaya Venkrat's CLLtopics site. Chaya's husband PC has recently undergone a double cord blood stem cell transplant. PC's progress is being blogged as "Harvey's Journal". It will provide some insight into the similarities and differences between a mini-allo transplant (like Jackie's procedure) and PC's double cord blood transplant.
Bruce and Jackie