Tenley’s Story

When Tenley was 13-months-old her parents started noticing she wasn’t meeting her milestones. They brought Tenley to Gillette Children’s Specialty Healthcare for a MRI.  The results showed a small amount of damage to the white matter in her brain. On September 15, she was diagnosed under the umbrella of cerebral palsy. Tenley continued to show regression so the family returned to Gillette Children’s for another MRI.  The second MRI showed a significant increase in the amount of damage to her white matter. On November 11, 2011 at 18-months-old Tenley was diagnosed with Leukodystrophy, a terminal brain disease. She has been given a short life expectancy that the family has chosen not to reveal in order to help them cherish each second instead of watching the clock.

Click here for Tenley’s CaringBridge page.

What is Leukodystrophy?

Leukodystrophy refers to progressive degeneration of the white matter of the brain due to imperfect growth or development of the myelin sheath, the fatty covering that acts as an insulator around nerve fiber. Myelin, which lends its color to the white matter of the brain, is a complex substance made up of at least ten different chemicals. The leukodystrophies are a group of disorders that are caused by genetic defects in how myelin produces or metabolizes these chemicals. Each of the leukodystrophies is the result of a defect in the gene that controls one (and only one) of the chemicals. Specific leukodystrophies include metachromatic leukodystrophy, Krabbé disease, adrenoleukodystrophy, Pelizaeus-Merzbacher disease, Canavan disease, Childhood Ataxia with Central Nervous System Hypomyelination or CACH (also known as Vanishing White Matter Disease), Alexander disease, Refsum disease, and cerebrotendinous xanthomatosis. The most common symptom of a leukodystrophy disease is a gradual decline in an infant or child who previously appeared well. Progressive loss may appear in body tone, movements, gait, speech, ability to eat, vision, hearing, and behavior. There is often a slowdown in mental and physical development. Symptoms vary according to the specific type of leukodystrophy, and may be difficult to recognize in the early stages of the disease.

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