History - 1998
n February 18, 1998 I received a letter from Dr Patterson at the Mayo Clinic stating that Jolynne did indeed have Niemann-Pick disease Type C.
Jolynne had a very difficult time eating. She would hold food in her mouth and would not (or could not) swallow it. At first we could chop her food and she could eat it with few complications. After awhile, though, we had to puree her food.
Her medications were also becoming more difficult to administer. Most of her medications were in tablet form and had to be ground up. Jolynne was always a picky eater and anything that tasted bad she didn't eat. It was very tricky getting the medicine mixed in well enough for her to eat it.
On March 27, Jolynne and I went to see Grease at the movie theatre. Since it was only the two of us, we talked Jolynne into wearing Depends. She very much hated wearing these but it would have been difficult for me to take her to the bathroom in a public facility. Once there, we had no problems and Jolynne soon forgot that she had them on. She enjoyed the movie very much (remembrance).
Eventually, Jolynne ceased to eat much at all. On April 15, Dr Skelton inserted her second PEG tube (she was somewhat upset to learn that Dr Collipp had removed the first one). This was very much a relief for all of us. We no longer had to coax Jolynne into swallowing and we could give her the medication that she needed and be sure that she got it all. We fed her Fibresource HN using bolus feeding.
On May 5, 1998 Jolynne, her parents, Kayleigh and I went to Attaché Show Choir.
Jo was still able to travel in her wheelchair and liked very much to get out and go places. Every day, at least once, we would drive Jolynne around so that she could just look out of the car at things. During the day, while I was at work, her mother would take her to the mall and other places periodically.
On May 15, Jolynne started vomiting. We called and got the on-call doctor (Dr Thiel) who suggested that we give her Pepto-Bismol. This did not help and we went to St Dominic hospital. By providence, Dr Fredericks was in the emergency room when we got there and she put Jolynne in ICU. Several tests were done but nothing was found. It was suggested that she just had a virus. One of the ICU nurses said that any time the doctors can't find something on the tests, they blame a virus.
On May 31 Jolynne started having a very difficult time breathing. She had short breaths and it sounded as though her tongue would not let the air back out of her throat. We went back to St Dominic hospital. The breathing was so bad that Dr Fredericks attempted to place a tube down her throat. She was unable to and told us that her vocal cords were frozen and would not let the tube past. She suggested that she get a tracheostomy and on June 1 Dr ??? performed the operation. We came home on June 22.
I was not fully aware of the changes that were to occur because of the trach. Dr Fredericks informed us that she would, from now on, be confined to a bed. She attempted to get a Kin-Air but we had to settle for an air mattress from Kendall. The night before June 22, I moved the den into our daughter's toy room. We were asked by St Dominic to choose from a list of suppliers who we wanted. I chose McNamed since they offered respiratory therapy and we got our equipment from them. This decision was entirely uninformed and the Social Worker at the hospital was not too much help. McNamed sent a respiratory therapist and another person to set up the equipment. The airbed was set up and along with that we had an oxygen machine, a humidifier, a suction pump (for suctioning the trach if necessary) and a nebulizer (for breathing treatments).
Jolynne's aunt found a rubber shampoo basin called EZ-Shampoo and a bag that could hold water and hang from the ceiling from Home Care Products. This was very helpful in shampooing Jolynne's hair in the bed.
On June 29, we were on our way back to St Dominic hospital for a 105.5° temperature. We came home July 1 after the temperature subsided and there seemed to be little worry of infection.
Her temperature went to 106.2° on July 24 and we made another trip to St Dominic. Each time her temperature would climb we would call 911 and we took an ambulance to the hospital.
Initially I had trusted (and I use the term lightly) our insurance company to pay for the ambulance trips and they did pay for each one. However, just in case, American Medical Response (AMR) offered a $49.00 pre-paid plan for unlimited calls for one year. I signed up for this since it was little money compared to the full charge. The ambulance did get reimbursed for the balance for each trip anyway.
This time we came home August 8.
On October 9, we went to the hospital for fever again. Dr Flowers determine that there was an infection in the port and they removed it on the 14th. We came home on the 20th. Jolynne was placed on intravenous Cipro (an antibiotic) until 29th.
On December 10, I decided not to treat Jolynne for a severe lung infection. I had talked with Dr Fredericks about this and she told me that it had taken two very powerful antibiotics in the lab to kill the infection. The drugs could have possibly caused liver and kidney problems. Jolynne's parents had told me that they would support whatever decision I made. Since Jolynne's quality of life was in a much more rapid decline, we started giving her 5mg of Morphine at bedtime to help with her pain and inability to sleep.