Female reproductive health in cystic fibrosis

Hughan, Kara S.; Daley, Tanicia; Rayas, Maria; Kelly, Andrea; Roe, Andrea H.

doi:10.1016/j.jcf.2019.08.024

Cited by 41 publications

(33 citation statements)

References 88 publications

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“…The transition to adult care center is now a key step for patients and their family, and new elements have to be taken into account in disease management, such as employability, desire for married life, and parenthood [ 53 ]. The number of pregnancies and paternities identified in CF patients has therefore progressively increased and one of the challenges is now to assess the safety of CFTR modulator therapies in pregnancy and breastfeeding [ 54 , 55 ]. As a result of the changing epidemiology, a growing number of studies has also been devoted to patients who reach the age of 40 [ 56 , 57 ].…”

Section: The Survival Of Cystic Fibrosis Patientsmentioning

confidence: 99%

The Changing Epidemiology of Cystic Fibrosis: Incidence, Survival and Impact of the CFTR Gene Discovery

Scotet

L’Hostis

Férec

2020

Genes

200

136

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Significant advances in the management of cystic fibrosis (CF) in recent decades have dramatically changed the epidemiology and prognosis of this serious disease, which is no longer an exclusively pediatric disease. This paper aims to review the changes in the incidence and survival of CF and to assess the impact of the discovery of the responsible gene (the CFTR gene) on these changes. The incidence of CF appears to be decreasing in most countries and patient survival, which can be monitored by various indicators, has improved substantially, with an estimated median age of survival of approximately50 years today. Cloning of the CFTR gene 30 years ago and efforts to identify its many mutations have greatly improved the management of CF. Implementation of genetic screening policies has enabled earlier diagnosis (via newborn screening), in addition to prevention within families or in the general population in some areas (via prenatal diagnosis, family testing or population carrier screening). In the past decade, in-depth knowledge of the molecular bases of CF has also enabled the emergence of CFTR modulator therapies which have led to major clinical advances in the treatment of CF. All of these phenomena have contributed to changing the face of CF. The advent of targeted therapies has paved the way for precision medicine and is expected to further improve survival in the coming years.

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Section: The Survival Of Cystic Fibrosis Patientsmentioning

confidence: 99%

The Changing Epidemiology of Cystic Fibrosis: Incidence, Survival and Impact of the CFTR Gene Discovery

Scotet

L’Hostis

Férec

2020

Genes

200

136

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“…Improvements in functional status are evidenced by the fact that 64% of adults with CF are in education or employment 2 . Although women with CF are reported to have higher rates of infertility than the general population, 3,4 increasing rates of pregnancy mean that medical professionals are confronted with issues regarding fertility, family planning and pregnancy.…”

Section: Introductionmentioning

confidence: 99%

The outcome of pregnancy in women with cystic fibrosis: a UK population‐based descriptive study

Ashcroft

Chapman

Mackillop

2020

BJOG

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Objective To estimate the incidence of cystic fibrosis in pregnancy and to explore obstetric and neonatal outcomes. Design A population‐based descriptive study using the methodology of the UK Obstetric Surveillance System (UKOSS). Setting All consultant‐led maternity units in the UK. Population All pregnant women with a diagnosis of cystic fibrosis who booked for antenatal care in a UK obstetric unit between March 2015 and February 2017. Methods Prospective case collection identified using UKOSS monthly notification. Main outcome measures Incidence, maternal morbidity, maternal mortality, gestation at delivery, neonatal mortality, neonatal morbidity. Results We report 71 pregnancies over a 2‐year period. There was one early miscarriage, four terminations and three sets of twins, resulting in the live birth of 69 infants. There were no maternal deaths. One infant died following spontaneous preterm birth at 29 weeks’ gestation. The mean gestation at delivery was 36.2 completed weeks. The mean birthweight centile for gestational age was the 61st centile. We report a positive correlation between both maternal lung function (FEV1) and mean gestation at delivery, and between FEV1 and mean birthweight centile for gestational age. Conclusions Pregnancy outcomes are generally good in women with cystic fibrosis. Successful pregnancy is possible even in those women with FEV1 <60% predicted, although such women have higher chance of preterm delivery and a smaller baby. Tweetable abstract Pregnant women with cystic fibrosis who have poorer lung function at the beginning of pregnancy have a higher risk of having a premature or smaller baby.

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“…Female patients with CF have been shown to have menstrual irregularities often associated with hypothalamic amenorrhea, due to CF-associated factors like undernutrition and chronic stress. 30 The use of hormonal contraceptives could help with these menstrual complaints. Additionally, hormonal contraceptives can help in reducing endogenous sex hormone-driven pulmonary exacerbations.…”

Section: Discussionmentioning

confidence: 99%

“…Besides alleviating provider discomfort with contraception counseling, other common gynecologic issues encountered by this population of patients can be addressed. Female patients with CF have been shown to have menstrual irregularities often associated with hypothalamic amenorrhea, due to CF‐associated factors like undernutrition and chronic stress 30 . The use of hormonal contraceptives could help with these menstrual complaints.…”

Section: Discussionmentioning

confidence: 99%

Reproductive health counseling and contraceptive use in adolescents with cystic fibrosis

Hernandez

Burdick

Adeyemi‐Fowode

2021

Pediatric Pulmonology

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Background: Cystic fibrosis (CF) is a progressive, genetic disease posing reproductive health concerns to affected women, such as high-risk pregnancies and medication interactions leading to contraceptive failure. Reproductive health counseling in this population is of the utmost importance, but barriers to providing it include lack of time, knowledge, and provider discomfort. We sought to evaluate reproductive health counseling and contraceptive use in female adolescent CF patients. Methods: An Institutional Review Board approved retrospective chart review was performed between March 2008 and March 2018. Females 10-21 years old with the diagnosis of CF were reviewed. Descriptive statistics were used. Results: Thirty-three patients met inclusion criteria: 16 non-sexually active and 17 sexually active. Thirteen patients were counseled about pregnancy risks. All sexually active patients used contraception, with the most common being condoms and combined oral contraceptive pills. Six pregnancies occurred, resulting in five live births and one termination.Conclusions: Less than half of patients were counseled about pregnancy and contraception, and most patients chose high failure methods. Female adolescents with CF should be routinely screened for sexual activity, offered long-acting reversible contraception, and counseled on the effects of CF on pregnancy.

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Female reproductive health in cystic fibrosis

Cited by 41 publications

References 88 publications

The Changing Epidemiology of Cystic Fibrosis: Incidence, Survival and Impact of the CFTR Gene Discovery

The Changing Epidemiology of Cystic Fibrosis: Incidence, Survival and Impact of the CFTR Gene Discovery

The outcome of pregnancy in women with cystic fibrosis: a UK population‐based descriptive study

Reproductive health counseling and contraceptive use in adolescents with cystic fibrosis

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