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By Emily Farmer, Dexter, ME
My sole objective in writing this story is to give courage to everyone preparing and consuming PKU diets. My severely retarded daughter, Michelle (Mickey) Farmer, was born in a small hospital in Maine on Nov. 26, 1956, a time when there was no routine PKU test done for newborns. A year later our second daughter (non-PKU) was born.
As new parents, it took us awhile to recognize that Mickey’s sister was surpassing Mickey in all forms of mental progress. When Mickey was twenty-two months old, we took her to a pediatrician in a city near us. He felt she might be deaf or have cerebral palsy. He made an appointment for us at Children’s Hospital in Boston. It was there that we learned Mickey had PKU. However, in talking with the clinic specialist, we were told that it was too late to place Mickey on a PKU diet. His exact words, which I have in a letter dated December 4, 1961, are these:
"I still would stand on the opinion that a phenylalanine deficient diet would not improve Michelle’s intelligence to the point of being teachable. It might, however, improve her behavior and if you find this sufficiently difficult at home to warrant the unavoidable difficulties in maintaining such a child on a very unnatural diet, it would not be medically wrong to give it a try. I should not personally advise it, but other physicians might well feel differently and it would be reasonable to try if you can find one locally who is prepared and qualified to carry out the close medical supervision necessary."
This idea that the diet probably would not help our daughter was reinforced a number of times—until we believed it. But since subscribing to National PKU News and the PKU Listserv group, I now have a great deal of regret that we did not place Mickey on the diet despite the advice of the physicians. I truly believe that she (and we) would have done considerably better.
Early on, my husband and I and two other couples formed an organization made up of parents of mentally retarded children. Our organization built a school for our small community, funded by federal and county money. One fortunate thing is that Mickey has always been able to attend a day program. The following years were trying though, with many difficult behaviors to contend with. All of this was tolerable until Mickey was about twenty years old. Then, her behavior really got out of hand. We made an appointment with Dr. George Jervis (a specialist in PKU, now deceased) at Letchworth Village in Thiells, New York. He examined Mickey and talked to us about trying to cut down on her phe intake, but not necessarily going on the full diet. I tried the diet, but with little help and not much faith in myself. We also had a doctor living next door who gave me prescriptions for a number of drugs to see if they would help. I hated the effects these drugs had on Mickey. I did not feel any of it was working in the slightest. I was very discouraged.
When Mickey was twenty-three, we placed her in a new facility that opened close to our home, because of her behavior but mostly because there was to be a registered dietitian. They started the PKU diet, but gave her too much phenylalanine and her blood levels stayed between 12 to 20 mg/dl, too high for many positive changes. She did not do well there. It was very close quarters and a restrictive environment.
We were delighted when Mickey had an opportunity to move to a group home. There the cook prepared the menus and had them approved by a dietitian. The meals were an improvement but still not great. Her blood levels ranged from 10 to 12 mg/dl. Her behavior improved somewhat but still she was difficult. (Mickey is nonverbal for the most part and her frustration level is high. She also has autistic tendencies due to PKU.)
In December 1995, Mickey moved to a home with just one other resident and staffed around the clock. We were thrilled. There was no dietitian assigned, though, and the staff was not knowledgeable about the PKU diet. When I got tired of the repetitive menus being offered and Mick’s high blood levels, I spent summer 1996 working up a five-week menu. Now, since my diets (approved by a dietitian) have been used, Mickey’s blood levels have ranged from 3 to 8 mg/dl.
Between the lower blood levels and continuing 2 mg. of Risperdal a day, Mickey has shown very significant improvements. She is a much happier person. She has never been at all sociable, but that aspect has improved a great deal. She actually enjoys a limited number of outings and can indicate, either by signing or verbally saying yes or no, when she does or does not wish to participate. She is much more pleasant to have around. There are still ups and downs, but we are so very pleased with the progress she has made. It has been a lesson in perseverance. I will not give up on trying to make Mickey’s life as comfortable and rewarding as possible until the day I die.
When you get discouraged, just think of Mickey. It should give those of you with early-treated children the courage to continue the every day struggle and realize just how necessary the effort is!