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By Jane V. Perr, MD; Psychiatric Director, AHRC Nassau County Chapter, New York
I was asked to see our client, Henry (age 48), for an emergency evaluation in February 1999 due to escalating and unpredictable aggression, and because he was not responsive to a variety of drugs. I was spurred to try to find out whether his PKU might be implicated in the symptoms he was having due to his non-PKU sister’s questioning and the suspicions I had myself. I was very fortunate to find the PKU News Web site. It confirmed our suspicion and the importance of diet for the adult with PKU, even for Henry who had never been on diet.
Henry started having seizures at three months and had slow development. When his PKU was finally diagnosed at age 8, he was started on medication for "anxiety." He was placed in special education, and when he was too old for school, he came to our day program. He had a history of eczema, agitation, anxiety, and impulsive behavior, but seemed to be well maintained on medication. The treating psychiatrist attributed an initial increase in these symptoms in 1996 and early 1997 to his move to one of our residences. His medication was increased with an improvement in "target" behaviors. However, beginning in November 1997, he began having much more frequent unprovoked assaultive behavior. He also had increased verbal aggression, self-injurious behavior (hitting himself in the face, pulling his hair and picking his skin), hyperactivity, muscle tension, property destruction, and confusion. He had episodes of screaming, increased self-talk, and an inappropriate and labile affect that looked psychotic. Throughout 1998, the treating psychiatrist tried various medications unsuccessfully. Henry’s sleep and appetite were at times "off" and in the months prior to starting the diet, he had some unintentional weight loss.
Henry’s diet was designed by Terry Goldberg, nutritionist at the metabolic clinic at North Shore Univ. Hospital in Manhasset, NY and implemented by Darci Weisbrott, head of nutrition at our agency. His initial phe level was 26.6 mg/dl. Fortunately, Henry took to the diet very easily, calling the medical food, his "happy drink." Staff at our facility are very committed to the diet.
It took about six weeks to see behavior improvements. Since beginning the diet in February 1999, he has had only two major aggressive episodes. (One may have been precipitated by his not receiving his medical food.) As his phe level decreased, his level of anxiety lessened while his alertness, frustration tolerance and sociability increased. His sleep has improved as well as his appetite, leading to a desired weight gain. When his level is at its lowest (5 mg/dl), he is dramatically calmer and happier.
Henry’s sister, Eileen (age 44), diagnosed at age 5 and only mildly retarded, has since benefited from the diet. Her behavioral symptoms were much milder than Henry’s. She was verbally aggressive at times, however, and before starting the diet, this behavior had increased. Her initial phe level was 27 mg/dl. She responded very well to the diet, with a subsequent reduction of her phe level to 7.6 mg/dl three months later. At such low phe levels, she is less anxious and has much-improved behavior.
Another of our PKU residents, Felicia, who is profoundly mentally retarded, also was recently started on the diet. Target behaviors have included pacing, irritability, poor sleep, hair pulling, arm biting, head banging, and aggressiveness. When her phe level is low, she is less agitated with better sleep and less self-injurious behavior.
Henry, his sister Eileen, and Felicia each have shown clear improvements in their general well-being since starting the diet. Even with devoted staff and the guidance of a PKU nutritionist, maintaining the diet has been difficult—but the value of continuing it seems very clear.