Reproductive and sexual health in adolescents with cystic fibrosis

Needs research—and skilled communication by health professionals

Advances in therapeutic technologies have created a new generation of adolescents with chronic disease and disability. Once uniformly considered a fatal disease of infancy and childhood, cystic fibrosis is now considered a life limiting disease of adults, and median survival is at least to the fourth decade in most developed countries. In this changing context, it is timely to reflect on the significance of sexual and reproductive health in young people with chronic disease and the challenge this presents to their health care professionals.

Infertility in men with cystic fibrosis was first reported almost three decades ago, as survival through adolescence began to increase.1 2 3 We now know that almost all males with the disease are infertile due to abnormal development of the mesonephric portion of the reproductive tract. Recent reproductive developments have focused on the aetiology of male infertility4 5 and new therapeutic technologies such as microepididymal sperm aspiration.6 7 However, there are still large gaps in our understanding of male infertility. For example, we do not know when is the best time to discuss reproductive health with patients, what is the ideal content of such discussions, or the impact that infertility may have on their sexuality. The few published studies simply show that adolescent males have a strikingly poor understanding of the effect of cystic fibrosis on their reproductive health.8 9

Women with cystic fibrosis fare even worse in terms of the lack of even the most basic information about the effect of the disease on their fertility. But the increasing numbers of pregnancies in women with cystic fibrosis is forcing a reassessment of the long held notion that there are abnormalities in the female genital tract that are specific to cystic fibrosis and which cause infertility, a belief based on the results of a single study of three women.10 The risk of pregnancy is also now being reassessed using epidemiologically sound studies rather than descriptive reports. While it is true that, until recently, few centres had enough adult women with cystic fibrosis to be able to perform controlled studies, there is less excuse for our failure to recognise common problems such as the high risk of vulvovaginal candidiasis in women taking long term antibiotics.11 Inadequate integration of primary and specialty health care for these young women, a problem generic to people with chronic illness,12 is only part of the explanation.

Reproductive health issues are often poorly communicated or even ignored when dealing with young people,13 14 especially in those with a chronic disease like cystic fibrosis15 where the attention and expertise of health professionals is focused on stabilising the disease and on survival. Like other health care professionals,16 physicians specialising in cystic fibrosis describe embarrassment when talking about reproductive health issues,17 which is hardly surprising given their lack of training in this area. Such training is as pertinent for specialists (in both paediatric and adult domains) as it is for primary care practitioners. While physicians may think that involving general practitioners or multidisciplinary teams will make it more likely that reproductive health is discussed (as if the ability to discuss these issues arises spontaneously), there is no evidence to support this belief.

All adolescents find aspects of sexuality confusing, embarrassing, or worrying. But those with chronic disease face additional stresses that may affect their psychosexual health. These may include questionable fertility in survivors of cancer; the difficulties of negotiating intimate relationships in patients with significant body scars or physical anomalies such as an ileostomy, those who must perform intermittent catheterisation, and those with cerebral palsy or who are regular wheelchair users; and difficulties informing a partner of future infertility for those with Turner's syndrome and cystic fibrosis.

Adolescents and young adults with chronic disease and disability deserve health care professionals who are knowledgeable and skilled in generic adolescent health care. They deserve more extensive research about their sexual and reproductive health needs and to have health practitioners with strong communication skills. Care of the disease is absolutely necessary but of itself it is not sufficient if we wish to achieve success in terms of quality as well as length of life.