Outcome of pregnancy in women with cystic fibrosis.

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.     

Guidelines for the management of pregnancy in women with cystic fibrosis

Women with cystic fibrosis (CF) now regularly survive into their reproductive years in good health and wish to have a baby. Many pregnancies have been reported in the literature and it is clear that whilst the outcome for the baby is generally good and some mothers do very well, others find either their CF complicates the pregnancy or is adversely affected by the pregnancy. For some, pregnancy may only become possible after transplantation. Optimal treatment of all aspects of CF needs to be maintained from the preconceptual period until after the baby is born. Clinicians must be prepared to modify their treatment to accommodate the changing physiology during pregnancy and to be aware of changing prescribing before conception, during pregnancy, after birth and during breast feeding. This supplement offers consensus guidelines based on review of the literature and experience of paediatricians, adult and transplant physicians, and nurses, physiotherapists, dietitians, pharmacists and psychologists experienced in CF and anaesthetist and obstetricians with experience of CF pregnancy. It is hoped they will provide practical guidelines helpful to the multidisciplinary CF teams caring for pregnant women with CF.     

Concerns regarding the safety of azithromycin in pregnancy - relevance for women with cystic fibrosis

Chronic oral azithromycin therapy improves clinical outcomes in people with cystic fibrosis (CF), and is recommended for treatment of CF lung disease. Azithromycin is categorized as pregnancy class B. The data for risk of congenital malformations associated with use of azithromycin during pregnancy ranges from no risk to a small increased risk. As with other chronic medications used to treat CF, potential risk to the infant of use of azithromycin during pregnancy must be weighed against the potential risk to the mother of treatment discontinuation. Women with CF considering pregnancy while on chronic azithromycin should be counseled regarding potential risks and benefits.     

Prevalence of urinary incontinence in women with cystic fibrosis.

OBJECTIVE: To determine the prevalence of urinary incontinence (UI) in female patients (aged > or = 15 years) attending a cystic fibrosis (CF) centre, in whom stress UI could be common, as chronic coughing and sputum production are frequent symptoms associated with progressive lung disease in these patients. PATIENTS AND METHODS: An anonymous questionnaire was completed by 176 women with CF (mean age 24.6 years, SD 5.8) during routine assessments as outpatients. RESULTS: In all, 72 patients (41%) were classified as never incontinent; occasional UI was reported in 61 women (35%). Regular UI, occurring twice or more a month for at least two consecutive months in the last year, was reported in 43 patients (24%). Regular UI was associated with increasing age and a lower mean (SD) forced expiratory volume/s (of that predicted) than in women with no urinary symptoms, at 26.9 (6.5) years and 53.5 (23.5)%, and 23.1 (5.4) years and 65.5 (23.2)%, respectively (P < 0.01 and P < 0.05, respectively). All incontinent women recorded stress UI; coughing, laughing and physical activity were associated with UI in 92%, 33% and 21% of the patients, respectively. CONCLUSION: Stress UI is a common symptom in women with CF. As urine loss can be under-reported to the healthcare providers, women should be asked about incontinence as part of their routine follow-up. Pelvic floor muscle exercises are effective in treating stress UI and should be considered for those with CF and regular UI.     

Contraceptive practices in women with cystic fibrosis

There is no literature regarding the contraception practices in female CF patients. A chart review identified 69 women with CF of whom two thirds were using contraception. Eleven different forms of contraception were being used with the oral contraceptive pill the preferred method. Despite theoretical concerns regarding efficacy and toxicity, the choices of contraception are similar to those of the general U.S. population.     

Sexual and reproductive health behaviors and experiences reported by young women with cystic fibrosis

Background

The prevalence of general and disease-specific sexual and reproductive health (SRH) concerns is unknown in the United States (U.S.) female CF population. This study aimed to describe and compare the SRH experiences and behaviors of young women with CF with the general U.S. population.

Sexual and reproductive health care utilization and preferences reported by young women with cystic fibrosis

Background

This study aimed to describe and compare the sexual and reproductive health (SRH) care utilization among young women with cystic fibrosis (CF) with the general United States (U.S.) population.

A novel solution for severe urinary incontinence in women with cystic fibrosis

BACKGROUND: To explore whether Tension-free Vaginal Tape offers a solution for women with cystic fibrosis who suffer from severe stress incontinence. METHODS: Four adults with cystic fibrosis were formally assessed by gynaecological and urological specialists, prior to hospital admission for surgery. RESULTS: The procedure was tolerated well by all patients. In three, leakage ceased completely. The fourth patient experienced considerable improvement in symptoms. CONCLUSIONS: Tension-free Vaginal Tape is a safe, effective and worthwhile solution for stress incontinence in females with cystic fibrosis.     

Effects of liver transplantation on the nutritional status of patients with cystic fibrosis.

The long-term effects of liver transplantation on nutritional status, body composition and pulmonary function in patients with liver disease associated with cystic fibrosis (CF) are poorly defined. We studied 15 patients with CF-associated biliary cirrhosis and severe portal hypertension. Seven underwent liver transplantation (age: 14.8 +/- 6.2 years), and eight were treated conservatively (age: 15.9 +/- 6.7 years). All patients were evaluated at baseline and thereafter yearly for a median duration of 5 years. During follow-up, transplanted patients gained weight and showed a significant increment in body mass index (P < 0.004), whereas patients without transplantation remained stable (P = 0.063). Baseline bone mineral content (dual energy X-ray absorptiometry scan) was lower than normal in all patients (more in transplanted patients) and increased in transplanted patients (P < 0.05), but not in patients without transplantation. In both groups percent body fat did not change, whereas fat free mass increased only in the transplant group (P = 0.06) (P < 0.03 versus nontransplanted patients). Only in transplanted patients' plasma concentrations of vitamin E and A increased (P < 0.05 versus nontransplanted patients). Forced espiratory volume in 1 s and forced vital capacity showed similar deterioration in transplanted and in nontransplanted patients. Liver transplantation is associated with long-term beneficial effects on the nutritional status of CF patients and seems to favor bone mineralization.     

Effects of upper respiratory tract infections in patients with cystic fibrosis.

BACKGROUND: The polymerase chain reaction has improved the detection of picornaviruses and rhinoviruses and our understanding of their role in reversible airways disease. The effects of colds on lower respiratory morbidity and bacterial colonisation in cystic fibrosis remain uncertain. METHODS: Children with cystic fibrosis were evaluated regularly in the clinic and the parents notified the investigators when their child developed a cold. Nasopharyngeal specimens were collected at the start of the infection for polymerase chain reaction, bacteriology was also undertaken and again three weeks later, and pulmonary function was measured in children aged > or = 6 years at four day intervals for three weeks. The effects of colds on rate of progression of cystic fibrosis were assessed by pulmonary function, Shwachman scores, and radiology. RESULTS: Thirty eight children suffered 147 colds over 17 months. Picornaviruses were detected in 51 (43%) of 119 nasopharyngeal specimens, and 21 of the 51 were further identified as rhinoviruses. Pulmonary dysfunction was similar following picornavirus and non-picornavirus infections; the mean change from baseline in forced expiratory volume in one second (FEV1) was -16.5% and -10.3% at 1-4 days and 21-24 days, respectively, after onset of a cold. Children who experienced more colds than average had evidence of disease progression with reduction in Shwachman score, increasing Chrispin-Norman score, and greater deterioration in FEV1 per annum. Ten of 12 new bacterial infections were associated with a cold. CONCLUSIONS: Picornavirus and non-picornavirus colds are associated with pulmonary function abnormalities and disease progression in patients with cystic fibrosis, and predispose to secondary bacterial infection and colonisation.     

Parenthood impacts short-term health outcomes in people with cystic fibrosis

BackgroundPeople with cystic fibrosis (CF) are living longer, healthier lives. A growing number are considering parenthood. There is a substantial knowledge gap regarding the health impacts of parenthood on people with CF.MethodsUsing data from the United Kingdom CF registry from 2015 to 2019, we evaluated the impact of having a child on percent predicted forced expiratory volume in 1 second (ppFEV1), body mass index (BMI), and pulmonary exacerbations via multivariable longitudinal data analysis adjusting for age and sex in the year before a person with CF became a parent to the first year following parenthood. We examined whether changes from pre- to post-parenthood differed by sex or CF transmembrane conductance regulator (CFTR) modulator use.ResultsAmong 296 people with CF who became parents, we found a significant decrease in ppFEV1, (adjusted mean difference -3.19, CI: -4.31, -2.07; p<0.01) and BMI (adjusted mean difference -0.28, CI: -0.51, -0.05; p<0.02) and a significant increase in pulmonary exacerbations (adjusted rate ratio 1.3, CI: 1.13, 1.49; p<0.01) in the year following the birth of a child compared to the year prior. Further analysis showed that CFTR modulator use mitigated parenthood impacts on ppFEV1, but not on BMI and pulmonary exacerbations. Females experienced significantly worse impacts on BMI from pre- to post-parenthood compared to males.ConclusionsParenthood adversely impacts short-term health outcomes for people with CF and CFTR modulator use mitigates such effects. To better understand the impact of the widespread use of highly effective CFTR modulators, longer-term, prospective parenthood studies are needed.     

Successful pregnancy and delivery in a young woman with cystic fibrosis and gestational diabetes.

We describe a 23-year-old Delta F508 homozygote cystic fibrosis primigravida. At the onset of gestation, she had mild to moderate pulmonary involvement, exocrine pancreatic insufficiency, focal biliary cirrhosis, satisfactory nutritional status and normal fasting and post-prandial glucose blood levels. At 29 weeks, she developed polyhydramnion and gestational diabetes. At 37 weeks, she was delivered of a live 2,980 g boy by caesarean section under epidural anaesthesia. Insulin was subsequently discontinued and her pulmonary function improved spontaneously. Neither maternal nor neonatal health problems were observed during the 3-month follow-up.     

Pregnancy outcome in women with cystic fibrosis and poor pulmonary function

BackgroundTo investigate how poor pre-gestational pulmonary function influenced pregnancy outcome and clinical status evolution in women with cystic fibrosis.MethodsPregnancies in women without lung transplantation with a first delivery reported to the French cystic fibrosis registry between 2000 and 2012 were identified. Pregnancy outcomes and clinical trends (body mass index – BMI, and pulmonary function) over a 4-year follow-up in women with poor pre-gestational pulmonary function, defined as forced expiratory volume (FEV₁) ≤ 50%, were compared to those in women with FEV₁ ˃ 50%.ResultsA total of 149 women had a first delivery and 36 (24.2%) of these had pre-gestational FEV₁ ≤ 50%. There was no significant difference in age or frequency of assisted conception between the 2 groups. The rate of cesarean section was significantly higher in women with FEV₁ ≤ 50% (43.7% vs. 21.1%, p = .01). The frequency of preterm birth did not differ significantly between the two groups, but median infant birthweight was significantly lower in women with FEV₁ ≤ 50% (2705 g; range: 650–3700 vs. 3044 g; range: 1590–3860, p = .003). Despite significantly lower FEV₁ and BMI the year before pregnancy for women with poor pulmonary function, the decline in these parameters during the study period did not differ significantly between the two groups.ConclusionPoor pre-gestational pulmonary function in women with cystic fibrosis was associated with a higher rate of cesarean section and a clinically significant impact on fetal growth, but was not associated with more important pulmonary and nutritional decline over the study period.