Batten Disease brought out best in my mother

To the Editor:

As some of you know, my oldest sister — Lindley Joy Matthews — passed away on Jan. 26.
For those that attended the funeral, I personally want to recognize my mother — Diane (Lindley) Whitmer — who is also deceased. She was able to work independently, some at home and arranged her schedule accordingly, so she could care for Lindley in her home. She was an extraordinary mother/person and she gave Lindley so much love and the best care possible.
If my mother would have lived long enough, she would have taken care of Lindley at home until her death. Lindley well surpassed the expected age expectancy for her disease — about 10 years — and I think the biggest reason for this was the special diet that my mother formulated for Lindley.
My mother started writing a book about Lindley’s life, and we hope to finish it someday so we can share it with others.
I also wanted to give some information about the disease Lindley had for those that may not know about it. Lindley had Batten Disease, the most common form of a group of disorders called Neuronal Ceroid Lipofuscinoses (or NCLs). It is a fatal, inherited disorder of the nervous system that begins in childhood. Early symptoms of this disorder usually appear between the ages of 5 and 10, when parents or physicians may notice that a previously normal child has begun to develop vision problems or seizures.
In some cases, the early signs are subtle, taking the form of personality and behavior changes, slow learning, clumsiness, or stumbling. Over time, affected children suffer mental impairment, worsening seizures, and progressive loss of sight and motor skills. Eventually, children with Batten disease become blind, bedridden, and physically and mentally incapacitated, requiring 24-hour care. Batten disease is often fatal by the late teens or twenties.
Childhood NCLs are autosomal recessive disorders; that is, they occur only when a child inherits two copies of the defective gene, one from each parent. When both parents carry one defective gene, each of their children faces a one in four chance of developing NCL. At the same time, each child also faces a one in two chance of inheriting just one copy of the defective gene. Individuals who have only one defective gene are known as carriers, meaning they do not develop the disease, but they can pass the gene on to their own children.
My parents and their families were both from around the Norborne area. It was a very tragic destiny that one of their children had Batten Disease, but with that knowledge I was tested and found that I am a carrier. This informed me to have my husband tested, because if he would also have been a carrier then we would have made the decision to not have children due to the possibility of them having Batten Disease.
It was painful enough for me to see my sibling endure the life that was dealt to her, but I can’t imagine all the pain and emotions my parents felt as they watched their child deteriorate. There is a foundation called “Beyond Batten Disease Foundation” that was founded to raise awareness and money to accelerate research to find a cure for Batten disease. They are also in the process of developing an easy and inexpensive blood test to detect the gene mutations for Batten disease, and hundreds of other rare conditions like it, that claim the lives of thousands of children each year. This test should be available sometime this year. If you go to their website: you can sign up to be one of the first to know when it’s available.
You can also visit to learn more about Batten Disease or to donate money for research.
In conclusion, I would like to thank Lindley for being such a sweet big sister and for teaching me so much about life. Many people take life for granted, or having healthy children for granted etc., but my sister opened my eyes and taught me to be grateful for every day. She was a sweet soul who was here for just a short while and she will never be forgotten.
I am glad she can be with my mother now and that she will never have to suffer again. As my mother said, “Life is so short, and all that truly matters is love and how we share it with others.”

– Danika Linville, Norborne

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