Betsy Alyn Taft was born on July 28, 1988. She was welcomed home by her Mom (Debra) and Dad (William), and two older sisters: Jennifer (8) and Jessica (17 months). We were all very excited to have Betsy as a new member of the family. She weighed 7 lbs. 13 oz. and was 19 inches long. She was the healthiest of all three children (at birth).
Betsy was bright, alert, and started sleeping through the night before she was six weeks old. She grew rapidly and seemed interested in absorbing all the details of life around her.
We were not aware Betsy was sick until at the age of 4 1/2 months she began having trouble with ear infections. Shortly afterward, she became colicky and cried continuously. We made several trips to the Dr.'s office, but the answer was always the same. She just doesn't want to eat because her ears are infected and it hurts to swallow. The Dr.'s just didn't believe she acted colicky at the 'old' age of 5 months (usually infant colic is subsiding at this age, not beginning).
The Dr.'s didn't seem interested in finding out what her problem was until she began to lose weight (1/2 lb. in 2 days). She was admitted to the hospital for failure to thrive. Miraculously, the Dr.'s determined she had a problem making it difficult for food to pass out of her stomach. The pressure needed to force the food through also created reflux into her esophagus, causing heartburn.
During her hospital stay, we noticed a new problem. Betsy was unable to grasp objects with her hands, and unable to hold her head up, skills she had already mastered. Her Pediatrician finally consulted with a Neurologist, but even then he (the pediatrician) said, 'She is probably just developmentally delayed, she'll be fine by kindergarten. The Neurologist was not as optimistic. He came to see her at 7:30 p.m. in her hospital room, and within 10 minutes decided he needed to perform a spinal tap. We weren't sure why he needed a spinal tap, but it didn't sound like a good thing.
The next week was hectic to say the least. The lab drew so much blood, we thought she wouldn't have any left. Not to mention the other tests: the EEG, BAER, ultrasound of the brain, and the MRI. We tried to get the Dr.'s to tell us what they thought the problem was, but they just said, "I don't know. The lab results were poor, and the BAER was slightly abnormal." After much prodding, the Neurologist finally said, "I think she has a progressive degenerative disease." We didn't know what he meant, but it sounded like he was saying our daughter was going to die.
Betsy was finally released from the hospital and her illness remained a mystery. She was still very irritable and cried almost all the time. She didn't want to eat, and the Dr.'s wouldn't help. They had her on several medicines for her stomach, but wouldn't give us anything to calm her down. They said, "We don't know what is wrong with her, and we don't want to mask the symptoms." Many nights we paced the floor with our screaming daughter in our arms, frequently crying ourselves because nothing we did seemed to help.
Finally the call came, We think we know what is wrong with your daughter, they said. Since we both worked, we drove separately to the Dr.'s office. He was very kind and explained that our daughter was suffering from a genetic disease, Krabbe's Disease. He told us there was nothing they could do, but at least with this disease she will eventually become unaware of the pain she is in. The Dr. told us we could care for her at home or place her in a nursing home.
We left the Dr.'s office devastated. How do you deal with suddenly finding out your daughter is going to die' We were barely able to hold the tears in until we left his office. On the way home, the road signs blurred, and it was difficult just to drive down the road. We finally made it home, safe and sound, just emotionally drained. We talked it over and decided we wanted to care for Betsy at home. No question about it, she was our daughter and we would take care of her.
Betsy was referred to Edmarc Hospice for Children. At the time, it was one of only 3 strictly pediatric hospices in the country. The nurse came to meet with us and explained the program. Betsy was still able to drink from a bottle, and we thought it would be awhile before we would need the nurse's help. We were wrong.
One week after admission to Edmarc, Betsy was unable to drink from a bottle for all her feedings. The nurse came and taught us how to place a tube through her nose into her stomach (a procedure called gavage feeding). We began feeding her through the tube every other feeding. By this time, Betsy was no longer sleeping through the night. We took turns each night, providing the care she needed. Not long after the tube feedings started, we had to start suctioning her. Betsy was no longer able to swallow all her secretions.
Six weeks after she was discharged from the hospital, Betsy began having seizures. The Dr. couldn't believe she was having seizures so soon, because her EEG was normal in the hospital. She was placed on Klonopin along with the slew of other medicines she was taking. The Klonopin worked for a short time, but pretty soon the seizures started again, despite the Klonopin. More seizure medications were added, first Phenobarbital and then Zarontin. This seemed to control the seizures for awhile, but she was still very irritable.
The only things that helped, were warm baths, and sitting in a quiet room with soothing music playing. When Betsy cried too much (or too long) she would vomit, so we started giving her chloral hydrate to help her rest. This worked well, but it seemed she was sleeping most of the time, and crying the rest. We don't know how we made it through those difficult months. We were finally able to move her bed into the family room where we could all visit her. The noise and chaos of a toddler (Jessica) no longer seemed to bother Betsy. Every day Jessica would kiss her little sister on the head and say 'Oh Baby, it's okay.
While attending the national conference for the United Leukodystrophy Foundation, we were able to talk to a Dr. about Betsy. He had worked with a lot of children with leukodystrophy, and told us he thought Depakene would work better for her seizures and be less sedating than the other medications she was taking. We discussed this with her Neurologist and he agreed to try it. Betsy was started on the Depakene and weaned off the other medicines for seizures. She was finally awake, and not crying, or having seizures. Her disease had progressed so far by this time, that she was unable to move, couldn't swallow, and required frequent suctioning.
Betsy's first birthday came and we had a huge celebration. Among those attending were aunts, uncles, grandparents, friends of the family, and several other children with various forms of leukodystrophy (Betsy was the only one with Krabbe's Disease). The Dr. told us he didn't expect her to live to be a year old, but he was wrong.
As the months progressed, new problems came to light. One problem was sinus infections related to the tube she constantly had in one side of her nose. The tube stayed in for 3 weeks at a time, and it seemed she constantly had nasal drainage. Betsy's gastroenterologist recommended a tube be placed surgically so she would no longer need the tube in her nose. She'll be more comfortable, he said. Because of all her stomach problems, the tube was placed in her jejunum, a portion of the small intestine. We were not aware of all the problems this new tube would create. (Betsy's Neurologist was totally against this procedure, he felt her needs could be met with the tube in her nose).
The first problem seemed to be getting out of the hospital. There always seemed to be a new reason to keep her. We finally made it home, and more problems followed. The tube drained bile, the bile burned her skin, and now instead of refluxing formula up into her esophagus, she was refluxing acid.
Betsy's condition continued to deteriorate. She began swelling, she wasn't tolerating her feedings and she was unable to control her body temperature. If it was hot, her temp was high. In a cold room, her temp would not even register on the thermometer. Finally, the day came when Betsy had suffered enough. She had trouble breathing and the Edmarc nurse spent all day with us. Eventually, the nurse left to go home, but we knew she would be back. It was time to let Betsy go. Mom cradled Betsy in her arms, kissed her, and told her she loved her and it was okay to go. Betsy died peacefully that night, at home with her parents, sister and grandparents at her side. Betsy died on November 2, 1989. She was 15 months old.
How could any family live through a terrible ordeal like this? We made it through with the help of family, friends, co-workers and the staff of Edmarc Hospice for Children. The community rallied around us, strangers prayed for us and we supported each other as best we could. No one can make it through a tragedy like this on his or her own, and they shouldn't have to. If you know someone dealing with a crisis, let him or her know you care. It can mean all the difference.