The FSH Society

Fascioscapulohumeral (fash-ee-oh-skap-you -lo-hum- er-al) dystrophy, one of the most common types of muscular dystrophy, is more commonly known as FSH or FSHD. It causes progressive weakening and loss of skeletal muscle, presenting initially in the muscles of the face, scapula, and upper arms, though other skeletal muscles can be affected.

FSH is a genetically inherited, autosomal dominant disease which appears to be caused by a mutation on one member of the chromosome 4 pair. A child has a 50% chance of inheriting the disease if a parent is a carrier. Cases of FSH which result from a new mutation are known as sporadic cases. Approximately 20% of sporadic cases are those inherited from a parent who carries the mutation but does not present with the disease, and the rest are the result of a new spontaneous mutation. These sporadic mutations will then be inherited in succeeding generations as an autosomal dominant trait.

Most individuals with FSH will experience the onset of symptoms in their teenage years, though it can present in early infancy or even well into adulthood. One of the most puzzling aspects of the disease is its variability. In different people, weakening of various muscles and muscle groups can occur at unpredictable rates and times. There is also pronounced variability in the severity of the disease, even among members of the same family.

It is estimated that the occurrence of FSH in the general population is one in 20,000. FSH occurs in all racial groups and occurs with equal frequency in both sexes.

The prognosis for those who have FSH is as variable as the disease itself. While it is certain that there will be some loss of skeletal muscle throughout the patient's life, the extent and rapidity of this muscle loss can vary widely from case to case. Some individuals will maintain a fairly normal life, while others will become confined to a wheelchair. The internal muscles, such as the heart, seem spared by the disease, and, though many individuals will have a normal life span, in rare cases FSH can cause respiratory failure.

The FSH Society is a non-profit organization, founded in 1991, which addresses the issues and needs related to Fascioscapulohumeral Dystrophy. According to the FSH Society website,, the goals of the Society are to:

. promote scientific and clinical research through education of the public, governmental bodies, and the medical profession;

. help exchange between researchers and clinicians involved in the diagnosis and study of the cause and treatment of FSH;

. collect and distribute information about FSH, its cause and treatment;

. foster communication among national and international interested parties;

. provide support for those living with FSH by assisting in the organization of support groups and by serving as a referral source for helpful professionals.

Annapolis resident, Doris Olds-Eck, who lost her daughter Karen Lynne Johnsen to respiratory failure from FSH, is hoping to extend her daughter's work as an advocate for those afflicted by the disease by raising $105,000 in 2005. Ms. Johnson was the director of the mid-Atlantic FSH support group and was named Miss Wheelchair Maryland in 1993 and 1994. She was also the former chairman of the Prince George's County Commission on Disabilities and was present at the signing of the Americans with Disabilities Act at the White House in 1990.

Contributions may be made payable to FSH Society Research in the memory of Karen Lynne Johnsen. Send your checks to Carol A. Perez, executive director, FSH Society Inc., 3 Westwood Road, Lexington, MA 02420.


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