Paget's disease of bone: The skeletal distribution, complications and quality of life as perceived by patients

ContextPaget's disease of bone (PDB) is a focal disorder of bone metabolism with overgrowth of affected bone resulting in the skeletal complications of this disease.ObjectiveThis study examines what patients know about the skeletal distribution of their PDB, and correlates this with their reports of complications and quality of life.DesignThe New England Registry for PDB (NRPD) is a voluntary registry with a questionnaire linked to a radiographic database. Data were collected by mail beginning in 2001.SettingAmbulatory population.PatientsAny patient with PDB living in New England was eligible to enroll; 285 elected to participate, mean age 73.2 years.Main outcome measuresPatients were asked what bones were affected by PDB, and whether they suffered complications from PDB. Radiographic studies were sought to corroborate their responses. An SF-12 was administered.ResultsCompared to the general population, they reported substantially lower levels of physical health (Physical Component Score (PCS) mean = 40), and slightly better mental health (Mental Component Score (MCS) mean = 52). There were more instances of agreement on disease presence and fewer instances on disagreement (p = 0.001). Radiographic studies supported the presence of a complication from PDB when deformity, fracture and joint replacement had occurred, but were less correlative when headache or hearing loss was reported.ConclusionsMost patients with PDB are aware of the skeletal distribution of their disease; there is a reasonable correlate between complications ascribed to PDB and the presence of PDB on the radiograph except when headache or hearing loss is reported.