Spinal Muscular Atrophy (SMA) Type 3

SMA Type 3, also known as Mild Spinal Muscular Atrophy and Kugelberg-Welander Disease, is usually diagnosed between 18 months and early adolescence. This is the mildest form of child onset SMA and prognosis is very good with people living a typically normal life span, however, weakness gets progressively worse over time. They can stand and walk at the beginning but usually with difficulty. Balance and climbing stairs can both be problems, and getting from sitting to standing may prove difficult. They are usually wheelchair bound in time.

Tongue fasciculations are rarely seen in SMA Type 3 patients but a fine tremor may be seen in outstretched fingers. Respiratory complications and problems with swallowing are also rare.

Scoliosis (curvature of the spine) occurs in some children, this is due to a weakness in the muscles supporting the bones of the spinal column. An operation can be done to straighten the spine and prevent further deterioration. They should be regularly monitored so problems can be picked up and dealt with early on. There will become a time when the decision has to be made to use a wheelchair.

Respiratory infections should be dealt with quickly. SMA Type 3 patients may tire very easily and excess weight will add to their problems. A dietician can help give advice and support as a sensible diet is very important as unnecessary weight puts strain on the muscles, which makes moving more difficult. Physiotherapy is very important as regular exercise helps to maintain muscle strength. There are some neuromuscular centres across the UK that have specially trained physiotherapists and occupational therapists.