Archive for: Aileen

We lived in a large house in Kandy, and Aileen and the others were first enrolled in a cooperative school organised by expatriates living in Kandy, then a year of home schooling, a year at the Digana School operated by an International Irrigation Management Institute, Colombo American School, and Maxwell Bahá’í School on Vancouver Island, Canada. From my point of view, such as experience was worth its weight in gold. In the middle of our time in Sri Lanka, the wonderful plan and activities turned into a divorce, unexpectedly and suddenly, and Aileen found herself in the middle.

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 in Aileen’s eyes. 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. In our marriage Carolyn was never able to say “no”, to me and to her children. This was imprinted on the soul of Aileen and she resented the fact that she had to acquiesce to her “father’s regime” and that she could seek refuge with her mother. 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 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.

We found a program for Aileen at the Speech and Hearing Clinic at Mississippi State College for Women in Columbus, Mississippi. This old, well established, and thoroughly Mississippian women’s school in the old south tradition was able to give her personalized attention by highly motivated teachers and staff. And, fortuitously, it has received a grant from the federal government and a new law had been passed requiring schools to accept hearing impaired children into local schools (mainstreaming). When we considered the options to move to North Carolina, the grant that was instrumental in helping Aileen was exhausted, and would not be renewed.

Over and over in my life, whenever confronted with difficulties or crisis, I found that if I kept moving and looking for a solution to whatever the difficulty entailed an answer appeared. Of course I don’t think that 94-142 and the federal grant were cosmically guided resources that God sent me to help my daughter, born to Baha’i pioneers in Haiti. But at the same time that is what happened, and I can cite other instances of this kind of unexpected support and guidance that directly impacted on my life. I don’t believe this only relates to me.

One evening at a Baha’i fireside in Kyiv, we read and studied a prayer about God’s support, tests and difficulties. I asked everyone in the room to think about the most difficult time in their life. A time when they did not have hope and felt helpless and unable to resolve a difficulty. And then I asked them what happened; how they survived the crisis.

Everybody had a story. Everybody has difficulties. And everyone receives support that was unanticipated and unexpected. For me, this is practically the definition of a spiritual test. We are confronted with issues that are by definition overwhelmingly difficult and where we do not have ready answers or solutions. Baha’is teach that these crises are part and parcel of life in the physical plain. I taught a class to pre-youth, when Svitlana’s father died, and I talked about the fact of disease, death and dying. I asked them: “Did God create anything that is bad?” They indicated: “No.”  Then, I went on: “So that means that the physical world animated and sustained by God, which includes death, needs to be understood from this perspective?” So we discussed that death is part of life. It is difficult and a test, but what we call death is simply a transition. And death and free choice is part of the underlying purpose of Creation– to give us a physical classroom to refine and develop our souls. One of my favorite multimedia videos (in Russian) is based on the book “Purpose of the Physical World”, by John Hatcher.

Aileen was a huge test for her parents and we responded in unity, and love. We worked hard for Aileen and found doors to provide her with her needs.

After about a year in Abidjan, we starting noticing that Aileen didn’t always react to people or noise. In the beginning, we could’t quite understand what was happening, because deaf people compensate with other senses, and their behaviour at times will look as if they can hear, even when they can’t. I keep watching and observing, because there was something, but I couldn’t define it. One day, returning home from work, I drove the car close to her and honked the horn. No reaction. First we asked a Bahá’í friend, a doctor, and then we decided it was time to get it check out. There were no adequate facilities in Abidjan, so back again to the US for tests. Aileen is profoundly deaf. We don’t know why it happened, if it is related to GSD or not. In the graphic, you can see that profoundly deaf means she doesn’t begin to hear sounds until they are over 100 decibels, and even above 120 decibels she doesn’t hear some sounds. If you click and open the graphic, you can see that Aileen has hearing above 100 decibels at low frequencies only. High frequencies are off the scale. Ordinary conversation is around 65 decibels. A rock concern is above 110 decibels. We were faced with a second serious difficulty in Aileen’s life, and few resources in Abidjan to help us. I had to recognise that there was no other choice but to return to the United States. I had no desire to return. I loved my work, and serving in Africa. But I could not ignore Aileen’s needs. Carolyn and I understood that caring for her was not going to be easy.

So, after the hospital and diagnosis with glycogen storage disease GSD, we moved to West Africa. I worked for a regional African organisation: “Le Conseil de l’Entente.” Five countries joined together for collaborative development: Ivory Coast, Haute Volta (now Burkina Faso), Togo, Benin, and Niger. I travelled for two years from country to country, a project manager for rural development projects. We had a wonderful life in Abidjan, a very close connection to the Bahá’í community, many friends, many house guests. But this time line is for Aileen. Perhaps it was not very smart to keep pioneering after Aileen’s diagnosis for GSD, but I was happy and confident and believed that we lived in the protection of God. I still think that way, as people commonly say, you never know what our end will be and how.