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While it has long been appreciated that up to 98% of men with cystic fibrosis (CF) are infertile due to a failure to develop, or early blockage of, the mesonephric ducts and vas deferens,1 the effect of CF on the fertility of women is less clear. Survival is now into the fourth decade of life and, with the largest growth in the CF population seen in the adult age groups,2 it is not surprising that the issues of fertility and reproduction are increasingly raised in adult clinics. Pregnancy is possible and increasing numbers of children are born to women with CF each year.3 Since the first pregnancy in 1960,4 management of all aspects of CF has improved and so the prognosis for mother and child has also improved. This paper reviews the pathophysiology of CF in the female reproductive tract, its effect on fertility and the ability to carry a pregnancy, and the outcome for mother and infant. CF is an autosomal recessive disease arising from abnormalities on the long arm of chromosome 7. Defective production of the cystic fibrosis transmembrane conductance regulator (CFTR) protein5—a complex channel regulating the passage of chloride and, indirectly, sodium and water across luminal cell membranes—results in thickened and desiccated secretions throughout the respiratory and alimentary tracts, sweat ducts, and the reproductive organs. The principal manifestations in adults are due to undernutrition resulting from pancreatic insufficiency and malabsorption, and respiratory failure due to chronic infection, bronchiectasis, and progressive lung destruction. Diabetes mellitus and cirrhosis are secondary manifestations resulting from pancreatic and biliary damage and are increasingly seen in older patients. CFTR is found in large quantities on the cervix6 and, although anatomically normal, histologically the columnar epithelium is distorted by mucus filled balloon or cygnet ring cells. The …

BACKGROUND: There has been increasing concern since 1979 about the emergence of Pseudomonas cepacia (Burkholderia cepacia) in patients with cystic fibrosis in the UK and elsewhere. Colonisation of the sputum has been shown to be associated with increased morbidity and mortality. Evidence suggests person to person transmission and some centres have segregated those colonised with B cepacia from other patients with cystic fibrosis. The outcome of patients colonised by B cepacia has been studied, together with the effects of strict segregation. METHODS: The outcome in 18 patients with sputum colonised by B cepacia was compared with that in age, sex, and severity matched controls with no evidence of B cepacia colonisation by a retrospective case note study. RESULTS: No difference between cases or controls were found in the 24 month period prior to colonisation by B cepacia in lung function, number of days in hospital, or outpatient visits. Colonisation led to an increased rate of loss of lung function and utilisation of hospital services. There was an increase in the numbers of transplants and deaths amongst the cases. Since 1992 there have been only three new cases of B cepacia colonisation and the incidence and prevalence of the organism has fallen dramatically since segregation commenced. CONCLUSIONS: B cepacia appears to be linked to the decline in colonised individuals. There was no evidence that colonisation occurred in patients declining for other reasons. B cepacia colonisation confers a worse prognosis than Pseudomonas aeruginosa alone. Segregation appears to limit the spread of the organism from infected individuals to other patients with cystic fibrosis.

The case history is presented of a patient with acute respiratory failure complicated by nasal obstruction resulting in intolerance of nasal ventilation. Urgent insertion of nasal stents permitted restoration of ventilation with resolution of breathlessness and stabilisation of arterial blood gases.

Following an attempted suicide by drowning in a vat of mineral oil, a previously fit man survived the usually fatal lipoid pneumonia resulting from total immersion after intensive support and prolonged steroid therapy with recovery of chest radiography and pulmonary function at one year.

BACKGROUND--As women with cystic fibrosis are living longer, pregnancy is becoming increasingly common. The combined experience of pregnancies in women with cystic fibrosis from adult centres in the Midlands and North of England has been examined. METHODS--A retrospective study of the case notes of 22 pregnancies in 20 patients with cystic fibrosis examined changes in lung function, body weight, and microbiological status during the course of pregnancy. Duration of pregnancy, birth weight, and maternal survival were amongst other variables studied. The relation between values before pregnancy and important outcome measures were examined. RESULTS--Eighteen of 22 pregnancies were completed producing healthy, non-cystic fibrosis infants (12 female). Mothers lost 13% of FEV1 and 11% of FVC during pregnancy, most of which was regained. Body weight changes were variable, but most mothers gained weight (mean weight gain 5.7 kg). Microbiological status remained unchanged. Six infants were preterm and two were light for dates. Four mothers died up to 3.2 years following delivery. Of the prepregnancy parameters examined, %FEV1 showed the best correlation with maternal weight gain, gestation, birth weight, and maternal survival. CONCLUSIONS--Pregnancy was well tolerated by most mothers with cystic fibrosis although those with moderate to severe lung disease (%FEV1 < 60%) before pregnancy fared worse, producing preterm infants and suffering increased loss of lung function and mortality compared with mildly affected mothers. Prepregnancy %FEV1 appears to be the most useful predictor of important outcome measures in pregnancies in women with cystic fibrosis.