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Werdnig-Hoffmann disease
There is no treatment for the progressive weakness caused by the disease. Supportive care is important. Attention must be paid to the respiratory system because affected people have difficulty protecting themselves from choking. Breathing complications are common.
Physical therapy is important to prevent contractions of muscles and tendons and abnormal curvature of the spine (scoliosis). Bracing may be necessary.
The lifespan in SMA type I is seldom longer than 2 - 3 years. Survival time with type II is longer, but the disease kills most of those affected while they are still children. Children with type III disease may survive into early adulthood. However, people with all forms of the disease have worsening weakness and debility.
Call your health care provider if your child:
Breathing difficulty can rapidly become an emergency condition.
Kliegman RM, Behrman RE, Jenson HB, Stanton BF. The hip. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Elsevier; 2007:chap 606.
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