Progress In Leaps and Bounds!

I have just re-read my last update and I cannot believe how much has changed since then. This past 9 weeks have been so incredible. If I hadn’t been present to witness Jenny’s transformation, I’m not sure I would believe it – it is nothing short of a miracle.

Throughout June + July, Jenny continued to have physical therapy 3 times a week. The therapist was always impressed with Jenny’s diligence and progress. Since the pain in her hip subsided, she was standing for longer periods and with the support of mountain boots for her ankles, she began taking steps with the walker. By the middle of June, Jenny attempted the stairs. The first time, she went up 4 stairs with the therapist’s assistance, the next day only 3, then the next time 7, and the next time, she just said to the therapist, “I’m going all the way up!’ And she did, she was so exhausted and exhilarated when she got to the top; she had to crawl the rest of the way to her room. It was the first time she saw her room in over 4 months! Her excitement and joy were overwhelming. From that day forward, nothing could stop Jenny from mastering those stairs.

She was on the therapist’s case constantly. By July 4, she was able to go up the stairs without a break and with only Steve’s hand for balance. Coming down needed more foot control, so at first she would just scoot down on her butt. We would have the walker or the wheelchair at the bottom of the stairs to accommodate her once she was down. As always Jenny would attempt more stairs each time she came down, now she just holds Steve’s hand for balance and she walks all the way down without a rest. It is such a beautiful sight to see!

While all of this mobility was going on, we saw Jenny’s hands and arms regain strength and control as well. Each day she would attempt to do more things for herself. Now she can dress herself entirely, even the mountain boots! Jenny can walk (using the edge of her bed and walls for balance) from her bed to her bathroom, she can brush her own teeth and brush her hair. She can close her blinds in her room when she is ready to go to sleep and she can turn her bedroom lights on and off when needed.
Today for the first time, when Jenny needed to go to the bathroom during the day, she walked to her bathroom and managed to take care of everything without a problem. You want to talk about thrilled parents! These steps are so significant and each day is filled with something new. I can’t tell you the happiness that Steve, Jenny and I share each and every day.

Jenny had a fever on July 20, we spent 4 hours in the emergency room at FUMC. It turns out that the Hickmann was infected, so out it came on the 21st. We think that 25 and a half months with a Hickmann catheter might be a world’s record. We also got rid of the hospital bed at the end of
July, it was clear that Jenny wanted to sleep in her own bed – in her room. We leave the walker and wheelchair out in the garage for backup purposes only. They’re only needed for long trips (like to Target or the grocery store). If we’re just going out to eat, or to a friend’s house, we just give Jenny a hand to hold for balance and she does all of the walking. No problem.

Jenny’s donor, Todd Bobeda, and his wife Ryann, came to visit us on July 29. It was a whirl-wind of activity and Jenny had little trouble keeping up to the rest of us. Her stamina is truly rebounding. It was such good timing for our visit with Todd and Ryann because they could see Jenny at her best, getting around, being chatty and happy. The whole time they were with us, it just felt so “right” to be with them. We had an Open House on Tuesday evening, July 31, so that family and friends could meet Todd and express their gratitude to him. Jenny’s Uncle Dan made a speech to thank Todd, and then another thank you to Dr. Davies. Dr. Davies then turned everyone’s attention to Jenny. The real trooper, the brave, strong and courageous young lady that did it all. It was without a doubt, a moment/an evening I’ll never forget as long as I live.

Here is a summarization of Jenny’s counts: her platelets are at 105,000 (normal is 150,000 to 450,000). Jenny hasn’t had a platelet transfusion since October 28, 2000. Her white cells are at 4200. Her hemoglobin (red cells) is hovering around 9.0. Jenny’s last blood transfusion was July 6. Dr. Davies has done an analysis of Jenny’s blood and it is about to “mature” and that means that her new blood and immune system will soon be taking care of all of her body’s needs without any more transfusions.

I hope that it is not too soon to say that it appears that the transplant is done, Jenny’s body has recovered completely. The new marrow has done it’s all important job of increasing Jenny’s alpha-mannosidase level to the normal range. And the progression of the horrible disease, alpha-mannosidosis, has been stopped. All of this is so amazing.

Where would we be without the team of doctors that treated Jenny? Dr. Ensor Transfeldt, the Orthopedic Surgeon that did the two spine surgeries (posterior and anterior/posterior spine fusions) in 1995 and 1996. Dr. Eric Steuer, the Neuro-Retinologist that got us through the CMV virus in Jenny’s eyes. Her left eye is 20/30 and the right is 10/200. We still hope for more improvement on the right eye. Dr. Larry Charnas, the Pediatric Neurologist that spoke to Heads of Neurology Departments at all of the major universities in the country to determine what was going on with Jenny’s nerves and how to treat it. Plasmapheresis was clearly the correct treatment for this obscure condition. And, most importantly, Dr. Stella Davies, the smartest, the hardest-working and most dedicated Bone Marrow Transplant doctor ever. No doubt, Jenny’s disease and the bone marrow
transplant treatment have been major medical challenges. Thanks to the dedication and diligence of these four doctors we have our dear daughter with us for the rest of our lives.

Now that we are where we are, I will have less updates. I will still keep you informed if anything changes, but I expect it would only be every 3 – 4 months. In the meantime, no news is good news.

Thank you for your continued support and encouragement. It kept us going through the difficult times. Keep us in your thoughts and prayers and we will do the same for all of you.

Love,
The Smiths
Steve, Dianne, Jenny and Jason