A Profile in Courage - Wayne and Ruth Zimmerman

COURAGE? You often know it when you see it in action. You definitely feel it. It can be witnessed over time. The attitude is sometimes defined by patience and heart. Webster writes: "facing and dealing with anything recognized as dangerous, difficult, or painful, instead of withdrawing from it, the courage to do what one thinks is right such as in willingness to live by one's convictions." This story began a long time ago as a little six year old girl in northwest Ohio. Bonnie Zimmerman was in first grade with me and I loved her. She seemed vulnerable and in need of friendship support. But the striking brunette was often missing from our class and it troubled me. Finally, one day at home when I expressed strong concern about Bonnie's absence, my mother told me that my classmate had a very bad sickness and there was a mystery as to what it was and what to do about it. This would be for me the second time that a special friend got into my heart. My mom always said that my heart was beating for the "underdog". That first neighborhood friend, Linda, had not only been a close playmate, but ultimately it was Linda's death from a rare stomach cancer that introduced me to the possibility of death at a very young age. To a child that seems impossible, it just should not happen. But it did, and Linda was only 4 years old when she died. So was it going to happen to Bonnie too? My mother said that we would just have to be patient and wait to see. In the meantime, I prayed for Bonnie to get well so she could come back to school.

I never saw Bonnie again. I knew that she was still alive, but too sick to return to school--ever. Later, I would learn that Bonnie's younger sister, Lynn, had also come down with the same physical and neurological symptoms. Lynn was only 8 years old. What was this disease that had no identity for the Zimmermans to date? Where to go for help? Because no local doctors had ever seen such a disruptive syndrome in patients so young, Wayne and Ruth knew that they had to head out to a specialized medical campus. And that is exactly what happened. The parents turned their hopes towards the University Hospital in Columbus, Ohio. That particular hospital was and still is a part of the great Ohio State University campus, and it was there that research was closest to this family for studying rare diseases. Upon arrival, there were the two girls, Bonnie and Lynn, their parents, and two more little daughters/sisters, both of whom were under the age of five. Were their futures also in jeopardy?

The Zimmermans shared the following observations with the medical specialists. According to Wayne and Ruth, Bonnie had developed as a normal infant into toddlerhood. She turned over during developing infancy, crawled into a walking stage, and did all the investigative moves of a normal two and three year old. She ate well, laughed, and played like any other little girl--until the age of five and a half. Then, both father and mother began to notice that one day Bonnie would tie her shoes, open doors, and turn on light switches, but the next day, perhaps she could not tie her shoes, open doors, or turn on light switches. Slowly but surely her muscle abilities were faltering. She became clumsy, fell often, stumbled, and gradually fell into a state of unresponsiveness and non-functioning motor control. Another oddity was Bonnie's repeating over and over an action without being aware of what she was doing. Even the night times were filled with huge and many episodes of grand-mal seizures. Both girls would have them one after the other. Bonnie would have as many as 30 per day. Every moment of every day brought without let up the constant stress, frustration, and heart ache of watching the daughters' deterioration. Ruth shares that if both girls could have just stayed at the same level of development when the disease first manifested its symptoms, the family would have been very happy and accepting. There was no shortage of love and commitment by the parents or with the two other little sisters. But that was not to happen. This disease was going to take over the lives of Bonnie and Lynn as the family looked on, seemingly helpless. And as this tight knit family waited, watched, prayed, and hoped, the University Hospital took blood tests, x-rays, genetic histories, eye exams, and neurological testings. The results were coming in with the available knowledge and experience of the times--the 1940's and 1950's. There was a scarcity of cases to draw, that much was certain. Epilepsy was ruled out. Bonnie and Lynn had symptoms of a much more insidious condition. As soon as the results were shared with Wayne and Ruth, their hearts were crushed, but not dead. Instead of collapsing into an emotional and mental heap from such devastating news, they were determined to give their two oldest daughters, and their two youngest, the best of days in the worst of times. Ruth says that upon their return to their home she had to get "mad". If her girls needed special services that at the time were not available, in fact, not even in existence, then there must be other families dealing with such needs as well. But first, Wayne and Ruth had to absorb what Bonnie and Lynn had. It was Batten's disease according to the specialists, and it was not good.

Bonnie was the first to be diagnosed with the unusual disease. It is not only rare, but fatal. It is an inherited genetic material from parents who are the carriers, but don't have to have the disease. It can take years to show up, even into adulthood when it becomes known as Kuf's disease. Batten's is one of 50 diseases called lysosomal storage disorders where the body cannot get rid of wastes. Genetic mutation causes cells to be thrown out of balance with a build-up of proteins and lipids(fats). Patients suffer progressive neurological impairment which includes seizures, visual impairment/blindness, personality and behavioral changes, dementia, loss of motor skills and the ability to walk, talk, and communicate. There is still no cure at this time. Batten patients can be expected to die early as both children and adults. How common or rare is Batten's disease is hard to confirm, but the data from several countries suggests about 0.5-8 per 100,000 live births. With an average of 1.2 per 100,000 approximately 440,000 people in the U.S. do not have the symptoms but carry the disease-causing mutations in the CLN3 gene. According to the National Human Research Institute, the Juvenile Batten is one of 6,800 RARE diseases affecting 30 million or almost 1 out of every ten Americans. Unbeknown to Wayne and Ruth Zimmerman, they were both carriers of the CLN3 gene. That was astonishing news to them as well as to the medical specialists at the University Hospital in Columbus, Ohio. In fact, as both girls were studied, there would eventually be five medical journals which presented Bonnie and Lynn with their symptoms, the development of their disease, and the new facts that were being learned about the gene CLN3 and how it was transmitted in families. For this family, the Zimmermans, the battle was far from over.

During the 40's and 50's there were no special schools for the physically disabled or the mentally challenged in northwest Ohio. In fact, there were no insurance companies which would insure for such a condition as Batten's. The entire medical costs would lie in the hands of the Zimmermans. They needed help and someone would need to step in and it would be the Zimmermans themselves. Ruth got really upset about the situation and began to talk to anyone and everyone who would listen. As a feature writer for a local newspaper, Ruth was given permission to write about her family and this rare disease not only to educate the public, but to promote the idea that a special needs school should be considered for children with such disabilities by the state and by the public. Wayne would relate that, "There was no one to help. There were regular schools to send our daughters to when they were feeling good. They could have attended the public school, but when they were ill, they would miss so much. That wasn't good for them or the school."

As it turned out, others were listening. Families who had children with special educational disabilities such as mental disadvantages and physical handicaps were contacted by the Zimmermans through the Fulton County Crippled Children's organization. The message was loud and clear: all these children needed a school for themselves. The task would be monumental however because no one had any idea of whom to turn to, who to contact, how to set up such a school, or who to hire as teachers. No one had any idea at all. Nothing. But the Zimmermans did not quit. They made many trips to the state capital, Columbus, to ask questions. to make appointments with an agency, to talk with people "in the know", and to knock on many doors for further guidance. What seemed to be a snail's pace actually began to come together. With guidelines having been determined for organizing and maintaining a school for the area's special needs students, Wayne and Ruth, along with other parents who joined in on this huge task/mission, searched for a place and a teacher. A teacher? One teacher? Yes, because that is what could be afforded from out of the parents' pockets. So, each family set aside monthly the amount of $30.00 to pay for the teacher's salary. In a local church the first class began with 7 students ranging in ages 8-17. Lynn would be able to attend for 6 months only, and Bonnie not at all. Over the years while still without a permanent location, another church and local organization would provide school space. However, as Wayne shared early on, "We all knew that we needed a building of our own for those who needed help. Some of the families went to the banks and borrowed money for the construction of a school. We all chipped in with our labor and after a few months the Happi-Time School for Retarded Children was built here in town." The school brought children from far and wide throughout the area. Families were stepping out of the dark and into the light. This new school and new concept brought hope where once there had been nothing. And as Wayne continued, "Before we had a bus to haul the children to school, quite often the parents would bring their child to school and then congregate here at our home for the morning since school was but half a day." After this special place was up and running, finally the state of Ohio and other counties began to pay attention. Effort became law, and the directors of the separate counties' Public Assistance Program began to seek ways to help alleviate the burdens, many being financial, that these parents had taken on by themselves. The school successively did pass out of the hands of the parents and into the responsibility of state and county ventures in the 1960's.

Meanwhile, as Bonnie and Lynn were continuing to spiral downward into the depths of the disease, Wayne and Ruth realized that they could not keep their daughters at home any longer. The couple still had two younger girls to love and care for as well as try to attend to the huge needs of Bonnie and Lynn. At this time there were no nursing or care facilities which would be able to take in the 24/7 needs of this situation, so the Zimmermans again looked toward Columbus, Ohio, for help and guidance. True to their commitment in caring for Bonnie and Lynn, the Zimmermans found Emerich Hospital in Columbus which would accept and care for the girls. Ruth explained that because neither Bonnie nor Lynn had recognized them the last two years at home, and because the home surroundings were of no matter or any concern to the girls any longer, "It made it easier to make such a decision. We would never have left them if either had recognized us. They simply no longer did. We went once a month to visit them but never knew us for a period of six years, and that was hard to deal with. Bonnie and Lynn lived to be 15 years old respectively.

While Wayne was still living, he and Ruth realized that much good had come to them and others through the grief of losing Bonnie and Lynn. Their girls had been treated with love, kindness, and respect by the doctors and the hospitals as these medical staffs were studying, documenting, and learning more about this rare and devastating disease. As a result, the medical world in Columbus, Ohio, came to know in a very personal way what eventually the global medical community would come to benefit in also. The AMA (American Medical Association) and JAMA (Journal of American Medical Association) would follow and inscribe meticulously this progressive condition which would enable other countries to grow in knowledge of and contribute to the growing knowledge universally. Enabling other families, both carriers of the CLN3 gene and the patients with the disease to contend with the symptoms much earlier in its various stages, meant quicker diagnosis and treatment- possibly slowing the disease down and giving a better quality of life to the patients. Perhaps the greatest legacy is the establishment of special needs schools and classes for the disabled throughout Ohio as well as the rest of the country. It is quite often through personal tragedy that progress for the betterment of others comes about with time, patience and focus. The goal ultimately became clear to Wayne and Ruth, but the efforts personally and publicly had to continue until the mission was completed. And that was and continues to be the courageous gift of the Zimmermans given to others. Well done, Friends, well done!

PS--Wayne Zimmerman died in 2003. His widow, Ruth, is a vibrant and energetic 93 year old mother, and grandmother who maintains in meticulous fashion one of the area's most beautiful and historic of homes--a place visited by Wild Bill Hickcock during the very early 1900's while performing with Annie Oakley and the Barnum and Bailey Circus. Her bright blue eyes sparkle as she leads me up the 19 steps to the second floor where American history comes alive with the displays of exquisite artifacts from the Far East, rare books on the American western plains Indians, oil paintings of the American west, and a tiny, but original photograph of Tom Thumb! It was a privileged room filled with the joy of life and learning--just like Ruth's personal life journey. Carol Paz at npazcar@aol.com

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