History - 1997
n 1996 we had decided to move back to Mississippi so that we could be closer to Jolynne's parents. Should the "psychoses" happen on a regular basis (as they appeared to do) then her mother would be better able to help Jolynne.
At this time I thought that Jolynne had mild cerebral palsy (which three different neurologists had diagnosed her with) which accounted for her abnormal gait and stumbling. I also thought that she had some form of mental illness which occurred on a cyclical basis (see 1992 History).
I knew, however much I denied it to myself, that her condition was getting worse. I had talked to Dr Levy about this and that is how we arranged the last visit to the neurologist (Dr ???). Jolynne had maintained that there was nothing wrong and that she just had a bad gyro (a high school joke).
After we moved to Mississippi, Jolynne was due for a gynecological exam. Her mother took her to Dr Ball for the pap smear. While she was there, they had several other tests run for possible endocrine imbalances etc. None of these tests turned up anything abnormal except for the pap smear which showed an irregular growth. This was later removed with no complications and was not related to the disease as far as we know.
Jolynne's mother had heard about a very good neurologist in Jackson and wanted to get her opinion on Jolynne's condition. Keep in mind that three neurologists had seen Jolynne in Oklahoma City and all, independently, came to the conclusion that she had mild cerebral palsy. Jolynne and her mother visited Dr Fredericks who examined her and immediately said that her conditions (including the mental conditions) were definitely neurological in origin and not psychological. To my knowledge, she based this observation on Jolynne's gaze, gait, and feet. Jolynne had trouble looking down without moving her head. This is known as vertical gaze paresis. She had problems with her gait in that she would walk with her legs slightly further apart than "normal" thus causing a "waddle". She also has very high arches and, when her feet are propped up with no pressure on them, her toes bow upwards and curl back. Dr Fredericks did not know at this time what the condition was but based on these (and most probably other) things that Jolynne had a neurological disease.
Dr Fredericks suggested that we check into a hospital for three or four days to examine Jolynne thoroughly. We checked in to St Dominic Hospital in Jackson on February 25. Several tests were performed including an MRI and a spinal tap. A neuro-psychological test was given to Jolynne by Dr Manning. All tests showed sub-standard results (click to view). We were released on February 28.
On March 7 we started occupational and physical therapy at the Mississippi Methodist Rehabilitation Center as an outpatient. These therapies lasted off and on until August 8.
Dr Fredericks also consulted with Dr Subramony at the University Medical Center in Jackson. Jolynne went to see him on March 25. From this consultation Dr Fredericks believed that Jolynne might have Whipple's Disease, a disease that usually effects the intestines but can sometimes effect the brain. We scheduled an appointment on April 9 to have an intestine biopsy done. Dr Skelton performed the biopsy.
The biopsy was sent to Athena Diagnostics and showed some "blue things" that seemed to indicate to Dr Fredericks that the disease was indeed Whipple's Disease. This disease is curable with intense antibiotic treatments. Since the disease was in the brain this called for antibiotics that could penetrate the barrier to the brain. Intravenous antibiotics were needed and this had to be done in the hospital. We were scheduled once again to go to St Dominic Hospital for this on June 10 and were released on June 24. During this stay it was noticed that, when Jolynne walked she sometimes had a shortness of breath. Dr Stone was asked to look at her to determine if there may be a heart problem. There was none.
After being released from the hospital Jolynne was placed in bactrim, a strong sulfur based antibiotic that could be taken by mouth. She had an allergic reaction to this and went for a couple of weeks without any antibiotics.
Dr Fredericks decided that intravenous penicillin could be given and we were going to do this at home. A PICC line was inserted at St Dominic Hospital on July 29 and IV drugs from IVONIX were shipped to our house. A pump was attached to the PICC line and dosages were administered on a timed basis. During these treatments Jolynne started to have much more difficulty walking. She had fallen several times and we took her to River Oaks Hospital to have an x-ray done on her knee. The x-rays showed that there were no broken bones. On August 8, she could not walk at all. In addition, Jolynne's jaws clenched and she could not open her mouth. We went to St Dominic Hospital's emergency room and called Dr Fredericks. We were checked into a room in the Neurology Wing.
This is the beginning of a heightened quest for a diagnosis since, it now was apparent that the disease was not Whipple's Disease. Another MRI was done and this time several anomalies were noted. This is the first time such abnormalities showed up on the MRI. Jolynne had had two in Oklahoma City and two previous ones in Jackson all being "normal". Several drugs were administered: an antibiotic, dilantin, and coumadin. Jolynne progressed to the point that she was only just breathing. Dr Skelton placed a PEG tube into her stomach to allow her to get nourishment.
It was determined that every avenue was exhausted to find a cause except for a brain biopsy. Dr Azordegan performed the biopsy with a CT scan guided procedure since the area of the brain was in a delicate place. He said that the procedure went well and only about three drops of blood were spilt. This meant that a minimum amount of brain damage had occurred. The biopsy samples were to be sent to Johns Hopkins but it was decided later to send them to the Mayo Clinic. The results did not show anything definitive.
After awhile Jolynne started to improve and she was moved to the Transitionary Care Unit (TCU) at St Dominic Hospital on September 2. When the biopsy results were returned, Dr Fredericks said that she was at a loss and that the only thing she could suggest was that we go to the Mayo Clinic. Arrangements were made to fly up to Rochester Minnesota. When the team arrived to transport Jolynne to the airplane on September 30, Dr Fredericks stopped them. Jolynne had developed a blood clot in her right leg. It started just below her pelvis and extended down to just above the knee. On October 6 we were transferred back to the Neurology Wing and waited there until we could be transported to the Mayo Clinic. We were delayed until October 7.
On October 7, we arrived in Rochester and were admitted to St Mary's Hospital, the inpatient section of the Mayo Clinic. We were greeted by the resident that was our main contact at the hospital, Dr Rogers. Later that day a team of seven doctors visited headed by Dr Dodick. I gave then some history about Jolynne and her symptoms and they reviewed the medical records we had brought from St Dominic Hospital. They did some physical examinations and left to discuss their course of action.
One of the doctors from the team performed a physical examination of Jolynne's breasts to determine if there were signs of breast cancer. No lumps were found. Another spinal tap was performed and a bone marrow biopsy was taken. The bone marrow biopsy showed that there were sea-blue hystiocytes present. I was told that this could indicate sea-blue histiocytosis but that other symptoms that Jolynne exhibited did not indicate this. A skin biopsy was ordered. A meeting with Dr Patterson at the Mayo Clinic was arranged. Dr Patterson made a preliminary diagnosis of Niemann-Pick Disease Type C. He stated that this was a very rare genetic disease in which the cells could not metabolize cholesterol. Over time, the cholesterol would build up in the cells and eventually destroy them. He could not be certain until the skin biopsy came back since there is not yet a definitive test for Niemann-Pick Disease Type C.
During our stay at St Mary's Hospital, Dr Dodick was either called away or had other engagements and Dr Matsamoto took over for him.
After the skin biopsy was taken we were informed that it would be about three weeks before the fibroblasts were grown and that all of the tests that they could perform were done. Jolynne, by this time, had gotten markedly better. She was now able to talk and to move about well in the bed. She spoke best when talking on the phone. She would get very agitated and she would try to scoot out of the bed. We would ride her around in a wheelchair to allow her to see more sites than the four walls of her room. Arrangements were made to transport her back to Mississippi to the Mississippi Methodist Rehabilitation Center (MMRC).
We were given her dismissal summary from St Mary's and a discharge summary from Physical/Occupational Therapy and released.
We arrived at MMRC on October 24, 1997 and were informed that Dr Collipp was to be our rehabilitation doctor. While at MMRC, Jolynne went through some rigorous physical, occupational and speech therapy. Almost immediatly after ariving, the doctor removed her catheter and we were forced to help her up and down into the bathroom. This was done because this was a rehabilitation hospital and the doctor's felt that it was necessary to get each patient back self sufficient as soon as possible. At night we would attempt to place diapers (called by the brand name Depends to keep from embarrassing the patients) on Jo but she hated them and would rip them to shreds.
She continued to improve and with the improvements came increased agitation. As I said before, Jolynne never liked to sit still. MMRC is quite a bit smaller than St Mary's in Rochester so going for walks in a wheelchair presented a larger problem.
MMRC did have a deck off of the 3rd floor that we could wheel around on. We were sitting on this deck while a high school football game was being played across the street. The band was playing and Jolynne thoroughly enjoyed it. She clapped (although not in time) to the music.
MMRC had an intercom system that announced all calls to each patient's room. When a nurse or doctor was paged, Jolynne thought that she was at a fast food drive through. She would order a hamburger, french-fries and a coke each time it went off. She would also think that the doctors were taking orders when she saw them in the halls.
During this time Jolynne had a swallowing test done and the doctor decided that there were to be no restrictions on Jolynne's eating. We did have to place thickener into her drinks though. He removed the PEG tube.
MMRC had a McDonald's on the second floor. Her parents and I were not too sure about going all the way into eating so soon. She and I did go to McDonald's a couple of times and she was aware enough to understand that she could only have french-fries and a coke.
We were released from MMRC on November 12, 1997 when we returned home.