HOW THE MAGNITUDE OF CLINICAL SEVERITY AND RECURRENCE RISK AFFECTS REPRODUCTIVE DECISIONS IN ADULT MALES WITH DIFFERENT FORMS OF PROGRESSIVE MUSCULAR-DYSTROPHY

The reproductive history of 177 male patients affected with Becker (BM D) (n=69), limb-girdle (LGMD) (n=54), and facioscapulohumeral (FSHIMD) (n=54) muscular dystrophy (MD) was analysed according to severity of the disease (BMD>LGMD>FSHMD) and magnitude of recurrence risk (RR) (hi gh for FSHMD, intermediate for BMD, and low for LGMD). Additionally, 6 2 male patients were interviewed on psychosocial issues, in order to d isentangle the factors influencing reproductive decisions among patien ts affected with MD. Among male adults, significantly more FSHMD than LGMD or BMD patients were married and had children. Age specific repro ductive outcome was 0.31-0.32 for BMD, 0.51-0.62 for LGMD, and 0.58-1. 02 for FSHMD, reflecting the influence of the disease's severity. High RRs did not significantly diminish reproduction after genetic counsel ling or correlate with less prospective desire for children. Instead, early onset, severity of the disease, and past reproductive history we re found to diminish reproductive outcome after genetic counselling, a nd prospective family planning was also found to be influenced by past reproductive history as well as by emotional/sexual dysfunction with the opposite sex.