The unexpected and rapid move from Starkville to Cary, North Carolina was made with 2 children. We initially rented a home, and then a condo near the center of Cary, and went about the work of setting up for Aileen her deaf support services and medical backstopping at Duke University. We arrived in time for the start of school in September 1982. Aileen was 6 years old, Nabil 4.
When we arrived in North Carolina Aileen’s auditory training was well underway, and we had understood the path to go forward. Negotiations were undertaken with the school system and Aileen was mainstreamed. On the other hand, her GSD was not under control. We started a program at the Duke University Medical School, under the direction of a Chinese doctor, Y. T. Chen. Two years later Dr. Chen published “Cornstarch therapy in type I glycogen-storage disease,” based on the work involving Aileen as a patient. I call this work the first GSD miracle. It changed our lives for the better. Who would have guessed that due to this research, not only was the condition regulated more effectively, not only did the seizures begin to diminish and eventually to cease, but it was the simplest of procedures. It required no expensive medical “food” that was expensive and, when in Africa and Mississippi, we had to organize its purchase and shipment to our home. It required eventually no N-G tube and infusion pump every night. And we didn’t have to monitor Aileen and watch her eat the required amount of food at the required time.
With GSD issues, I was always the bad guy. It is not in Carolyn’s nature to be openly tough with the children, but she was well aware of the consequences if the schedule was not followed— both immediate and long term. This was imprinted on the soul of Aileen and she resented the fact that she had to acquiesce to her “father’s regime.” This resentment was nurtured by the divorce, and turned into a rebellion against me, and against the values of her parents that extended into her adult life.
In 1984, uncooked cornstarch was found to be the most effective therapy for maintaining blood glucose concentrations in the desirable range. Research demonstrated that cornstarch therapy only prevents hypoglycemia for a median time of 4.25 h in children. Initially, all children must awaken in the middle of the night for therapy, and delayed administration of the therapy can be associated with the development of hypoglycemia, seizures, and neurologic injury. Even as adults, almost all patients still require therapy every 4-6 h, and overnight therapy is required in >90% of patients to achieve optimal metabolic control.
“The constant anxiety about avoiding hypoglycemia and the necessity to interrupt sleep to receive therapy 1 to 3 times per night is deeply detrimental for these patients and their families. The effort is exhausting, and the resulting fatigue affects the work and quality of life of the patients and their families. In addition, exhaustion from waking up every night eventually leads to delayed administration of a cornstarch dose, which puts children with GSD Ia at extreme risk from hypoglycemia.”
A few years later, in 1987 Dr. Chen, published “Prolongation of normoglycemia in patients with type I glycogen storage disease,” again partially based on work with Aileen. We have much to thank for the inspiration of Dr. Chen. In this research, long-term effects of cornstarch therapy on biochemical values and physical growth in children with type I glycogen storage disease (GSD I) were compared to those of children receiving continuous nocturnal nasogastric glucose feedings (CNG). Blood glucose, lactate, cholesterol and triglyceride levels were not significantly different between the two methods of treatment. All patients maintained linear growth rates normal for their age. The data confirmed that cornstarch is a simple, effective and safe therapy for GSD I. Interestingly enough, by 2010 Duke was undertaking research, where “Gene Therapy Could Save Kids From a Lifetime of Eating Cornstarch.” If I met the author, I would remind him how wonderful this research affected the lives of the children, and parents.
There is a post-script to this GSD discussion and Dr. Chen. When Lua was born, Dr. Chen diagnosed her with GSD Type-I, the same as Aileen. Another post-script, the miracle of corn starch opened the door to our return to the field of Baha’i pioneering.
Assistant Professor, Extension Seed
- Between children, work, and Baha’i activities, our life was full
- District Teaching Committee
- National Convention
- Baha’i Club
- Cary Community
- Some consulting, full professors did not approve
- Desktop computers
- Dr Chen and the first miracle
- Lua born and GSD and vasectomy
- Another miracle with Lua and Dr Chen
- Life got easier, and we were ready to return to international pioneering
House in Garner we bought after a couple of years in Cary. Eventually taken over by Carolyn after the divorce which she sold.