Knowledge, attitudes and behaviors on reproductive health of patients followed at Hacettepe University adult cystic fibrosis center

Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the CF Transmembrane Regulator (CFTR) gene. Development in current diagnosis and treatment methods, led to incread survival rates and more patients reach adulthood. As population of adult patients increase, the need for reproductive health and genetic counseling services in adulthood has come into question. There is no data from our country about the knowledge and behaviors of patients with cystic fibrosis on reproductive health. In this cross-sectinal thesis, we aimed to investigate knowledge, attitudes and behaviours of adult cystic fibrosis patients about reproductive health. Our study was carried out between April 2021 and October 2022. Patients older than 18 years of age were included. All patients answered the 'Respiratory Functions and Reproductive Health Questionnaire in Cystic Fibrosis Patients'. Investigation of anti-mullerian hormone levels from female patients and scrotal USG and spermiogram examinations from male patients were planned. Pulmonary function tests, imaging studies and other laboratory examinations of the patients were obtained from hospital records. Datas were analyzed using SPSS v.25. Of the 100 patients included in the study; 54 were male and 46 were female (median: 22, IQR: 19-27). A total of 15 (15%) were married, 84 (84%) were single, 1 (1%) divorced. Six (6%) patients had children. Of the 40 (40%) patients who planned to have a child, 34 (85%) were planning to receive genetic counseling services. Although all patients were sexually active, 90 (90%) have not been using any contraception method and the frequency of sexual intercourse in 72 patients was less than once a week. AMH levels were obtained from 38 (82.6%) female patients whereas, only 7 (13%) male patients had spermiogram and 17 (31.5%) male patients had scrotal ultrasound. The mean FEV1 values of the patients were 2.41±0.97 liters (66.97±26.59). Knowledge of patients about infertility did not affect the contraceptive method they applied and the frequency of sexual intercourse (p= 0.408 and 0.18, respectively). It was observed that the source of information did not affect the thoughts about the relationship of the disease with infertility, the method of contraception applied and the frequency ofsexual intercourse (p 0.71, 0.393, 0.662, respectively). The source of information about reproductive health in cystic fibrosis did not affect level of anxiety (p=0.002). Knowing that their level of knowledge about the relationship of their disease with infertility was sufficient also affected their anxiety levels (p=0.007). In conclusion, a significant proportion of patients did not have sufficient knowledge about the effects of the disease on reproductive health, and this situation was also reflected in the attitudes and behaviors of the patients. It was interesting that the majority of male patients did not accept to have spermiogram and scrotal USG examinations. It has been observed that adult patients should be informed about the effects of the disease on reproductive health and should receive genetic counseling.