Baseline Cystic fibrosis disease severity has an adverse impact on pregnancy and infant outcomes, but does not impact disease progression

Cohen‐Cymberknoh, Malena; Reiss, B. Gindi; Reiter, Joel; Lechtzin, Noah; Melo, Joel; Pérez, Gema; Blau, Hannah; Mussaffi, Huda; Levine, Hagit; Bentur, Lea; Gur, Michal; Livnat, Galit; Miranda, Javier; Polverino, Eva; Blasi, Francesco; Aliberti, Stefano; Aviram, Micha; Golan‐Tripto, Inbal; Picard, Elie; Novoselsky, Michal; Amsalem, Hagai; Celnikier, Drorith Hochner; Kerem, Eitan; Shteinberg, Michal

doi:10.1016/j.jcf.2020.09.002

Cited by 26 publications

(22 citation statements)

References 24 publications

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“…For women with CF, pregnancy carries added health risks, including higher rates of pneumonia, renal failure, and rarely death, with a mortality rate of 1% [6] . Severe pulmonary disease (FEV1 < 50%), pulmonary hypertension, Burkholderia Cepacia infection, and exocrine pancreatic insufficiency have been shown to be important predictors of poor maternal outcomes [7] , [8] , [9] , [10] . During pregnancy, women with CF experience increased frequency of pulmonary exacerbations, hospitalizations and illness-related visits [11] , [12] .…”

Section: Special Considerations For Pregnancy In Women With Cfmentioning

confidence: 99%

“…Appropriate weight gain can be difficult to achieve, and women may require oral supplements or, in more severe circumstances, enteral feeds [12] , [13] . However, despite these challenges, women with CF who tolerate pregnancy do not experience deterioration of CF disease afterwards [10] . When compared to never-pregnant women with CF matched by age and disease status, pregnancy does not impact 10-year survival rates [14] or subsequent progression of pulmonary disease [10] , [11] , [12] , [15] , [16] .…”

Section: Special Considerations For Pregnancy In Women With Cfmentioning

confidence: 99%

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Gestational and pregestational diabetes in pregnant women with cystic fibrosis

Oxman

Roe

Ullal

et al. 2022

Journal of Clinical & Translational Endocrinology

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Highlights Women living with cystic fibrosis (CF) are increasingly pursuing pregnancy. Gestational and pregestational diabetes are highly prevalent in women with CF. Diabetes impacts fetal outcomes, and good glycemic control reduces risk of pregnancy complications. Diabetes during pregnancy in women with CF requires intensive endocrine and nutrition care, ideally incorporated into the CF multidisciplinary team.

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Section: Special Considerations For Pregnancy In Women With Cfmentioning

confidence: 99%

Section: Special Considerations For Pregnancy In Women With Cfmentioning

confidence: 99%

Gestational and pregestational diabetes in pregnant women with cystic fibrosis

Oxman

Roe

Ullal

et al. 2022

Journal of Clinical & Translational Endocrinology

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“…Unfortunately, the woman died of respiratory failure less than 2 months after the birth of her 34-week-old infant; it was felt that her pregnancy substantially accelerated her disease progression, and consequently, caution was advised for women with CF considering pregnancy [ 36 ]. More modern data continues to suggest that for women with moderate to severe CF lung disease, there is increased risk of premature delivery of neonates who also have a higher incidence of complications [ 37 , 38 , 39 , 40 ]. Babies born to mothers with CF also have a higher rate of congenital anomalies compared to the incidence in women without CF [ 38 ].…”

Section: Pregnancymentioning

confidence: 99%

“…Another risk factor for maternal and infant complications, diabetes, occurs in approximately 30% of adults with CF [ 3 , 41 ]. Importantly for women with CF, investigators have shown that in spite of some decline in health during pregnancy, with our current management, women with CF do not experience accelerated disease progression [ 40 , 42 ]. The impact of CFTR modulators on the health of the pregnant mother and her infant is not yet known.…”

Section: Pregnancymentioning

confidence: 99%

Fertility, Pregnancy and Lactation Considerations for Women with CF in the CFTR Modulator Era

Jain

Taylor‐Cousar

2021

JPM

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Cystic fibrosis (CF) is an autosomal recessive genetic disorder impacting approximately 80,000 people of all races and ethnicities world-wide. CF is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene which encodes a protein of the same name. Protein dysfunction results in abnormal chloride and bicarbonate transport in mucus membranes, including those in the respiratory, gastrointestinal and reproductive tracts. Abnormal anion transport causes viscous secretions at the site of involvement. The majority of people with CF succumb to respiratory failure following recurrent cycles of infection and inflammation in the airways. Historically, providers treated the signs and symptoms of CF, but since 2012, have been able to impact the basic defect for the subset of people with CF who have mutations that respond to the new class of drugs, CFTR protein modulators. With the improved health and longevity afforded by CFTR modulators, more women are interested in parenthood and are becoming pregnant. Furthermore, this class of drugs likely increases fertility in women with CF. However, the safety of CFTR modulators in pregnancy and lactation is only beginning to be established. We summarize available data on the impact of CFTR modulators on fertility, pregnancy and lactation in women with CF.

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“…8 Pregnancies are also on the increase that require a close interdisciplinary working relationship with obstetric teams; complex decision-making frequently is required to minimize the risk of complications to the mother and child. 9 Cancer is an important new challenge in CF that could stifle further progress in survival, even though paradoxically it's likely to be a result of living longer in the first place. A recent metanalysis of GI cancer showed that the risk might be at least 10 times greater in CF than the general population.…”

mentioning

confidence: 99%

Introducing the Adult Cystic Fibrosis Series

Simmonds

2021

Chest

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Cystic fibrosis (CF) has come a long way since its first description in 1938 by the pathologist Dorothy Andersen. 1 What was then a fatal condition in childhood with a >90% mortality rate in the first year of life has now been transformed into a disease across the age spectrum for which survival well into adulthood is expected in most countries. Treatments have progressed dramatically from basic, but important, supportive and symptomatic interventions to state-of-the-art highly effective targeted therapies. 2 The cloning of the CF gene (CF transmembrane conductance regulator [CFTR]) in 1989 was a watershed moment in our knowledge, but it has only been relatively recently that these targeted treatments '"CFTR modulators" have been realized. Although they will likely bring further demographic shifts, for a long time now, the proportion of adults to children has been increasing significantly, 3 with most countries, particularly in the developed world, reporting an adult preponderance. In some regions, such as Scandinavia, this is over 60% ($18 years old). 4 Median predicted survival in the United States is currently 47.4 years, with predictions that new therapies will likely increase this much further. 5 This progress should be applauded, of course, but as with most successes, the momentum should not stop,

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Baseline Cystic fibrosis disease severity has an adverse impact on pregnancy and infant outcomes, but does not impact disease progression

Cited by 26 publications

References 24 publications

Gestational and pregestational diabetes in pregnant women with cystic fibrosis

Gestational and pregestational diabetes in pregnant women with cystic fibrosis

Fertility, Pregnancy and Lactation Considerations for Women with CF in the CFTR Modulator Era

Introducing the Adult Cystic Fibrosis Series

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