High Fear of Disease Occurrence, Low Quality of Life Associated Multiple Endocrine Neoplasia Type 1

Patients with multiple endocrine neoplasia type 1 (MEN1) suffer from a fear of disease occurrence that is associated with a lower quality of life, according to a study recently published in The Journal of Clinical Endocrinology & Metabolism.

Researchers led by Gerlof D. Valk, MD, PhD, of the University Medical Center Utrecht in the Netherlands, point out that MEN1 is characterized by a lifetime risk of developing primary hyperparathyroidism (pHPT) of almost 100%, a life time risk of developing duodenopancreatic neuroendocrine tumors (dpNETs) of more than 80%, and a risk of developing pituitary tumors (PIT) of 70%. The average life expectancy in the Dutch MEN population is 73, which is 10 years shorter than the average Dutch population, and since MEN1 develops at a young age, these patients must be intensively monitored.

Fear of cancer occurrence and quality of life have been studied in other hereditary cancer syndromes like Von Hippel Lindau disease, but few studies have addressed these issues in MEN1 patients. “Up to now, it is unclear if having MEN1 leads to psychological distress because of fear of disease occurrence (FDO), thereby potentially affecting quality of life,” the authors write.

The researchers sent the Cancer Worry Scale questionnaire (with a score of 14 or higher reflecting a high FDO), the SF-36 Health Related Quality of Life questionnaire, and questions on sociodemographic and medical history to 285 eligible MEN1 patients in the Netherlands, with 227 completing the questionnaires. The mean age of the cohort was 47 years old.

Overall, the patients experienced a FDO of 15.1, with 58% reporting a score 14 or higher, and adjusting for age and gender, the FDO scores were negatively associated to almost all SF-36 subscales, the authors write. What’s more is that patients had higher FDO scores for their family members than themselves. “MEN1 is a diagnosis that often affects multiple family members, and therefore the high FDO for patients’ family members, requires that regular follow up visits should include addressing worries about relatives with MEN1 related problems and psychosocial support should be provided when needed,” the authors write.

Based on these findings, the authors conclude: “The majority of MEN1 patients have fear of disease occurrence for themselves and even more for their relatives. This psychological distress is associated with a lower health related quality of life. Therefore, in the medical care for MEN1, emphasis should also be placed on fear of disease occurrence and quality of life.”

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