6 Things to do After Being Diagnosed FMF

Familial Mediterranean Fever (FMF) is a recurrent fever, the auto inflammatory condition of the joints, chest and abdomen. It is not contagious but is inherited, usually affecting families from the lands near the Eastern Mediterranean Sea. Patients mainly include persons of Arab, Sephardic Jewish, Turkish or Armenian ancestry. Geneticists estimate that 60 percent of Turks and 75 percent of North African Jews are affected. The cause of FMF is a damaged MEFV autosomal recessive error on gene number 1.

Both parents must carry a copy of the damaged gene in each of the cells in their bodies to have a child with the active disease. The parents don’t show evidence of the disease, but their children have a one-in-four chance of having FMF. Half the rest of the children will be carriers of the gene like their parents, and one child will not have any sign of the damaged gene. Rarely other damaged genes cause FMF. It is rare, but FMF can occur in families of any descent. The disease often first occurs in childhood or adolescence.

Ninety percent of FMF patients are diagnosed by age 20. Rarely, the condition may first be expressed in adulthood and is often less severe. Often, patients have a characteristic rash on the lower legs. The typical incident can last from 48 to 96 hours with an average peak of painful symptoms at about 12 hours. The time lapse between attacks can range from days to many years. Between episodes, an individual usually has no signs, but some children have such frequent attacks that they never fully recover or even grow properly. Several medical specialties help care for FMF patients, including rheumatologists, medical geneticists, immunologists, and nephrologists.

1. Abdominal Pain

Abdominal pain is a classic feature of FMF. The inflammatory process can cause severe damage to many organs and structures in the abdomen. About half the patients have abdominal attacks, and some have many. The pain can be intense and is frequently confused with the passage of gallstones, kidney stones or appendicitis. Many patients undergo unnecessary surgery as a result of misdiagnosis. There is a likely danger of peritonitis during abdominal attacks. It is crucial that FMF patients make sure that their physicians and any new doctors, especially in emergency rooms, are aware of their disease and know how to treat it.

There may be abnormal examinations and medical tests that may lead to an inaccurate diagnosis. To lessen the frequency and severity of abdominal attacks, colchicine, derived from a natural substance also called autumn crocus, or meadow saffron is an anti-inflammatory agent. It is useful in about 90 percent of patients. If colchicine isn’t well tolerated or effective, biologic therapy is helpful as well. Long-term medical treatment can lessen the number and severity of abdominal attacks in Familial Mediterranean Fever. The drugs are not effective during an acute attack but are for long-term prevention. Drug therapy requires frequent follow-ups by a physician.