Quality of life and functional outcome for individuals who underwent very early colectomy for familial adenomatous polyposis.

BACKGROUND: The major manifestation of familial adenomatous polyposis is colorectal adenomas, which, if untreated, lead to colorectal cancer. The impact of IPAA on quality of life in adolescents with familial adenomatous polyposis is favorable. There is a group of children who develop polyps at a younger age requiring earlier colectomy. Little is known about this very young subgroup in relation to bowel function or quality of life.

OBJECTIVE: The aim of this study was to investigate the outcome in patients with familial adenomatous polyposis who had colectomy at ≤14 years.

DESIGN: A cross-sectional quantitative survey was designed to assess outcome. Standardized validated instruments included bowel/psychosocial functioning and quality of life.

RESULTS: Among 1337 patients with familial adenomatous polyposis from 409 kindreds, 4% (n = 59) of patients underwent colectomy at ≤14 years of age. Response rate was 84% (n = 32). The mean age at colectomy was 12 years (SD 2), with a current mean age of 24 years (SD 8.5). Fifty-seven percent of patients reported continence. Of the 43% reporting daytime or nighttime incontinence, the majority are <18 years (86%). Younger participants (currently less than 18 years of age) report more restrictions. Mental health is significantly lower among participants with incontinence. They report higher depression and anxiety symptoms, higher levels of intrusion and avoidance, and inferior mental health. The percentage of those worrying about risk of cancer is significantly higher in the younger group (71% vs 24%). Most patients (n = 24, 75%) have had surveillance endoscopy within the past 2 years.

LIMITATIONS: This study is limited by study generalizability, selection bias, and small sample size.

CONCLUSIONS: Twelve years after colectomy more than half of the patients have favorable bowel function. The rate of incontinence is high, especially among younger patients who have had a shorter time since surgery. Patients with incontinence reported lower psychosocial functioning, are very concerned about their cancer risk, and experience greater distress. This subgroup would benefit from added psychological interventions to enhance coping with familial adenomatous polyposis and surgery.