What is SMA

Information of SMA Disease

Prognosis

Each type of SMA has variability among individual patients. Please keep this in mind when reading this section.easing overall weakness or repeated respiratory infections, the prognosis is poor. Death in the majority of children with Type I S.M.A. usually occurs by 2 years of age.

Type I (Acute)

In the acute type of this disease, the bulbar muscles are often affected, and this may make feeding and swallowing extremely difficult. Breathing is often labored due to reduced strength of the chest muscles, and most breathing can be seen in the abdominal areas, with the chest appearing sunken in.

Because of increasing overall weakness or repeated respiratory infections, the prognosis is poor. Death in the majority of children with Type I S.M.A. usually occurs by 2 years of age.

** IT IS IMPORTANT TO NOTE THE OVERLAP OF TYPE I & II

Type II (Intermediate)

Because of the range of progression seen in patients with Type II, it is hard to tell how fast, if at all, the weakness will progress. Some children may learn to walk with the aid of bracing and may survive into adulthood. However, others, due to weakened chest and respiratory muscles may become increasingly weak with probable respiratory infections such as pneumonia. There are many cases in which the initial progressive weakness may remain the same, or there may be periods of worsening followed by long periods of stability. With such variables, age of death can vary greatly. It can take place as early as 2 years as in Type I or not until adulthood. Although not all children diagnosed with Type II develop respiratory weakness, respiratory failure is usually the cause of death following a bout of pneumonia or other respiratory infection.

Type III Intermediate)

Patients with Type III will again vary greatly. However the prognosis is very good. Often walking will be possible, or the patient will be fully functional for years before assistance is necessary. As with Type I and Type II respiratory infections should be presented and necessary precautions taken.

Type IV (Adult Onset)

There is nothing unusual or distinctive about the current management of the adult forms of Spinal Muscular Atrophy. Proper diagnosis, genetic counseling and appropriate physical therapy remain mainstays.

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