Krista's Story

 In 1965 while living in Toronto, Ontario, I gave birth to my third daughter, Krista. My other daughters, who were 2 and 4 years old, were born without PKU. Krista's development was slow. When we insisted upon some answers, we found ourselves by her side during a one-month hospital stay and finally received word of her diagnosis. At the age of nine months, her diagnosis was PKU. We had never heard of the condition. To our knowledge, there was no history of PKU in our families. From the age of nine months until five years of age, Krista was on the low protein diet with formula. The doctors felt her brain had "developed adequately" at age five and thought she could safely be taken off the diet (a common practice in those days).

 At age three, Krista was in a two-year nursery school program. After that, she went into a special program for mentally and physically disabled children which was available through the public school system. Then, after much consideration, we enrolled Krista at Shepherd's Home & School in Union Grove, Wisconsin. She was only seven years old, but Shepherd's offered compassionate Christian care for the mentally disabled. We were told that Krista would benefit greatly from the school program they had to offer. She learned to read and write, and learned excellent social skills and spiritual values. Her behavior was hyperactive, but never self-abusive. Now, twenty-six years later, she still enjoys being a resident of Shepherd's and has made many special friendships. She works in the laundry room and is involved in many activities. We had always considered her healthy and happy. However, about two years ago, we had cause for great concern.

Suddenly, Krista started to lose weight and seemed very nervous. She began biting her tongue and lip. Her behavior was quite agitated at times. The nurse at Shepherds alerted me to these significant changes. After taking her repeatedly to the doctor, a dentist and an oral surgeon, I was still left without answers. Why was she behaving like this and how could we help her? It was then that I recalled my oldest daughter's conversation with a doctor from Mayo Clinic in Minnesota. The doctor suggested that Krista might benefit from a phe-restricted diet. I was unaware at that time how important diet was for Krista, even at the age of 31.

Krista's blood phe level was tested and found to be over 30 mg/dl (1800 micromol/L). Since a safe level is now thought to be about 2-6 mg/dl (120-360 micromol/L), I knew something needed to be done. My concerns for her were great. How would she adjust to the diet after being on a normal diet for 25 years? I couldn’t' even remember what the diet involved. We were starting from "square one" again!

My first phone call was to the Public Health Department. After many other telephone calls, waiting expectantly for returned calls, explanations and referrals, I was referred to the dietitian at the PKU clinic at the University of South Florida, Tampa. She told me about the Winter 1997 National PKU News article entitled, Off-Diet Young Adults with PKU: Lives in Danger. I read about some of the horrible experiences other people had as a result of not being on the diet. Increasingly, I realized the urgency of Krista returning to the formula and diet. Although I realized that Krista's returning to the diet would not alter the consequences of her late diagnosis, I was devastated to think of the damage to her mind and body that might continue to occur if she did not go back on the diet soon.

Dr. Malone at the PKU clinic in Tampa was the first one to see Krista. Before he would even suggest the diet for her, he and the dietitian (Pilar Goldstein) called Shepherds Home in Wisconsin to ask the Food Service Director if he would be willing to manage the diet for Krista. I was thrilled when he graciously obliged. The next hurdle was to see if Krista would accept the new diet and formula, and to see her levels stabilize before she returned to Shepherds. I spent many sleepless nights struggling to create a diet, with only a small amount of phe, that would satisfy her needs. I was determined to do it! I remember when the dietitian gave us a loaf of low protein bread. When the loaf was gone, I panicked. What would I do without bread? Thankfully, help was on its way. Kindly, Tampa parent Marsha Magol offered to bake a loaf of bread for Krista and helped us through that difficult time. Now that I am more familiar with the low protein foods, I bake Krista's bread whenever she is home.

What a challenge! I worked hard to meet the daily requirements for the diet and to keep Krista happy as well. After just one week on the diet, Krista's level went down to 2 mg/dl (120 micromol/L). We were thrilled with such wonderful results in a short time!

When she was off the diet, Krista loved to eat. At the top of her "favorites" list were hamburgers and French fries. She was in the habit of eating all the high phe foods that were not good for her. But the transition has been incredible. She has accepted the diet very well, even at the age of 32 years. Her self-abusive behavior has completely stopped. She is feeling "great" (her own word). We have noticed that her attention span seems longer and her thinking is much clearer. Her ability to follow directions also has improved. It is thrilling to say that her general well being is so much better after returning to the diet. We are incredibly grateful to see so many remarkable changes in Krista. She's a changed person and remains a happy young lady–and a joy to her family!

Doris Barber
E-mail: Barberdori@aol.com