An Answer — it is treatable and reversible!
From: “Smith, Dianne” Dianne.Smith@mpls.frb.org
Subject: [JennysHope] Update #30 An Answer — it is treatable and reversible!
Date: Wed, 4 Apr 2001 21:09:56 -0500
Jenny has been through so much since the last update. Still her strength and will to fight are unsurpassed.
We have been seeing a new Neurologist at the University of Minnesota. Last Tuesday we met Dr. Charnas for the first time and he gave Jenny a thorough examination. As his exam progressed, it was clear he was very concerned about Jenny’s condition. The things that were concerning him were Jenny’s lack of sensation in her fingers and toes and her total body weakness. He did not think that this problem was related to the progression of alpha-mannosidosis, for which we were extremely thankful. He had us come back the next day, and we spent the entire day in various labs and examine rooms. They did a spinal tap, another EMG and connectivity test and lots of blood work. The results from all of the tests are back and Jenny has an auto-immune process attacking her nerves. The best treatment is to do plasmapheresis or plasma exchange.
The whole process will begin tomorrow when Jenny will get a new temporary Hickmann catheter in her left jugular vein. It is special because it will handle the volume and has appropriate ports for the machine they will use to accomplish the plasmapheresis. Then on Friday they will hook Jenny’s one port to a machine that will extract her blood, remove her plasma, add new plasma and re-transfuse Jenny with this new blood into the other port. (Something is causing Jenny’s spinal fluid to have a protein count of 90 and normal is below 50 and having the plasma replaced should resolve that.) Anyway, each treatment will take about 2-3 hours. Jenny will need to have this done 5 times over the next two weeks. It could take another 3 weeks after that till we see progress because the high protein causes nerve inflammation and it takes substantial time for that to subside. We are so thankful that this is treatable and reversible, some of these neurological disorders are not . Our prayers are answered once again.
We have seen mild improvements in Jenny’s movement, she can pull herself onto her side in bed with the help of the handrails, she can push her arms a little when I dress her and today she seemed to hold more of her body weight when Steve was helping her into the car. These are all encouraging signs that her body will recover, without the treatment – though it might take 10 years till she could walk again!
Jenny also saw the Retinologist this week and got more good news. Dr. Steuer said her left eye is now 20/30 (better than he ever imagined it would get to). He has now indicated that he feels it is possible that Jenny’s left eye might get to 20/20 someday. Her right eye is still blind, the CMV virus is still inactive and Jenny is off the foscarnet (the IV treatment for CMV) for 2 weeks now. He indicated that most of the patients that he sees with CMV in their retinas have Aids and since their immune systems don’t recover, they get other problems when the eye tries to fight the scar tissue in the damaged retinas — he does not see that in Jenny’s right eye. The human body is so amazing. We are very pleased about that as well.
All in all, this has been a long, difficult bone marrow transplant. We have tried our best to stay strong and positive for Jenny’s sake. As long as Jenny can have a long, healthy rest of her life, this past two years will be well worth it and probably some day they will just be a faded memory.
Thank you so much for your continued support, especially your prayers and encouragement. We couldn’t do this alone.
Love,
The Smiths
Steve, Dianne, Jenny and Jason
