Spinocerebellar degeneration

SPINOCEREBELLAR DEGENERATION

            Many people know about “One Liter of Tears” and have followed that film. This is a real story about a 15-year-old Japanese girl named Ikeuchi Aya who suffered an incurable disease, but lived life to the fullest until her death at 25. The story is based on the diary Aya kept writing until she could no longer hold a pen. At her age fifteen, unusual things started happening to Aya. She started falling down often and walked in a strange way. She also always dropped food from her chopsticks, could not estimate the distance of the objects in front of her, could not pour water into a cup without spilling it, and occasionally wobbled while walking. Her mother Shioka, took Aya to see the doctor, and he informed Shioka that Aya had Spinocerebellar degeneration. What happened to Aya, may be, make people wonder and emerge some questions.

What is Spinocerebellar degeneration disease?

Spinocerebellar degeneration is a disease where the sufferers get deterioration in their cerebellum and spinal cord function and more and more time these two organs will get worse and can damage. The words Spinocerebellar degeneration derive from spino meaning spinal cord, cerebellar meaning cerebellum, and degeneration meaning decrease. It is also usually called Spinocerebellar ataxia because the sufferers will lose their balance, as the result of the deterioration in their cerebellum which role as the centre of human balance and coordinating the muscles movements.

What does cause the Spinocerebellar degeneration disease?

Spinocerebellar degeneration is a hereditary disease caused by the genetic disorder called autosomal dominant, in which the affected person inherits a normal gene from one parent and a faulty gene from the other parent. In addition, this disease also can be caused by the gene mutation; it is the change of the genetic material chemical structure causing the change of individual character.

How does that disease attack our body?

The symptoms of Spinocerebellar degeneration have been happening since the sufferers at the age of fifteen, and then, what happened to the sufferers. Firstly, the signals from their cerebellum will not be able to get to their muscles correctly. It first begins with falling and slipping oftenly. Then, they will have difficulty in walking and lifting heavy objects. They will not be able to control their fingers very well as fine muscle movement is adversely affected. In addition, they also will not able to respond the reflect movements, such as hold up their body with their hands when fallen down, as their spinal cords which role as the control of reflect movement degenerate in functions, hence it can not deliver their message well. Over time, talking, eating and breathing will be difficult. They will have to use a wheelchair and eventually bedridden. Eventually, they will never wake up.

Is there any medication or treatment to cure it?

There is no known cure for Spinocerebellar ataxia. Treatments are generally limited to softening symptoms, not the disease itself. The condition can be irreversible. The treatment of incoordination or ataxia, then mostly involves the use of adaptive devices to allow the ataxic individual to maintain as much independence as possible. Such devices may include a cane, crutches, walker, or wheelchair for those with impaired gait; devices to assist with writing, feeding, and self care if hand and arm coordination are impaired; and communication devices for those with impaired speech. The supports from the people around are also needed to give the life spirits for the sufferers. However, hopefully, our future generations are able to find the cure of this disease as the development of their knowledge and ability and the technology.