Health status and quality of life of elderly persons with severe hemophilia born before the advent of modern replacement therapy.
BACKGROUND
More and more people with severe hemophilia reach an old age thanks to an effective treatment. There is no information on the health status and quality of life of elderly people with hemophilia born at a time when replacement therapy was hardly available.


METHODS
Italian patients with severe hemophilia, aged >or=65 years and hence born in 1942 or earlier, were compared with elderly men without bleeding disorders matched for age, sex, geography and social status. The following aspects were evaluated: concomitant illness, orthopedic status, physical functioning and cognitive status. Measurements of generic and disease-specific health-related quality of life were also obtained, together with the presence or absence of depression.


RESULTS
Thirty-nine patients, aged 65-78 years, were investigated; 33 had hemophilia A. All patients had started regular treatment on demand only when they were already 25-30 years of age. Patients were compared with 43 men without hemophilia, aged 65-79 years. More patients with hemophilia had chronic hepatitis B and C, HIV infection and hypertension. On the other hand, their elderly peers without hemophilia were more frequently hypercholesterolemic and had more cardiovascular diseases. Most hemophiliacs had arthropathy and worse values for physical functioning, but their cognitive status was similar to that of elderly non-hemophiliacs. Hemophiliacs reported greater depression and lower health-related quality of life.


CONCLUSIONS
Elderly patients with hemophilia have more co-morbidities and problems in daily living, but similar cognitive status as age-matched non-hemophilic peers. They have more chronic viral infections and hypertension but fewer cardiovascular diseases. These observations should help to optimize health care delivery in this increasing and neglected population of people with hemophilia.
Siboni
S M
SM
Department of Medicine and Medical Specialties, Angelo Bianchi Bonomi Hemophlia and Thrombosis Center, University of Milan, IRCCS Maggiore Hospital, Mangiagalli and Regina Elena Foundation, Milan, Italy.
Mannucci
P M
PM
Gringeri
A
A
Franchini
M
M
Tagliaferri
A
A
Ferretti
M
M
Tradati
F C
FC
Santagostino
E
E
von Mackensen
S
S
D000203:Activities of Daily Living; D000368:Aged; D001779:Blood Coagulation Factors / Q000627:therapeutic use; D006304:Health Status; D006467:Hemophilia A / Q000188:drug therapy / Q000503:physiopathology*; D006801:Humans; D007558:Italy; D008297:Male; D011788:Quality of Life
D001779:Blood Coagulation Factors