With proper management, careful monitoring, and working closely with your care team and obstetric team, many people with cystic fibrosis are able to carry a child without significantly impacting their long-term health. At the same time, however, the unique demands that pregnancy places on the body can have serious implications for people with CF.
The progressive decline in lung function and impaired nutrition associated with CF can pose several challenges, including a higher risk for health complications and an increased treatment burden. For this reason, women with CF who plan their pregnancies for a time when they are in the best health possible tend to have the most successful outcomes.
Am I Healthy Enough for Pregnancy?
Several factors help determine how well a woman with CF will handle pregnancy. These factors are:
- Lung function: Ideally, a person with CF should have a forced expiratory volume (FEV1) of 50% or more before becoming pregnant. People with lower FEV1 values can also get pregnant, but their risk of complications may be higher — affecting both your health and the health of your newborn.
- B. cepacia: Some studies have shown that infection with some forms of Burkholderia cepacia can lead to more problems during pregnancy. If you have B. cepacia and are interested in becoming pregnant, talk to your CF care team to learn more about the possible implications for both your and your baby's health.
- Nutrition: Before getting pregnant, it is important that your nutritional status is good enough to support both you and your baby throughout your pregnancy, given potential morning sickness or changes in appetite. This means having a body mass index (BMI) of at least 22 kilograms per square meter (kg/m2). If your BMI is below this, improving your nutritional status before you get pregnant would benefit you and your baby, as low BMI in pregnancy is associated with problems including poor fetal growth and premature delivery.
- Cystic fibrosis-related diabetes: If you have cystic fibrosis-related diabetes (CFRD), monitoring and controlling your blood glucose prior to getting pregnant will be key. Diabetes has been shown to increase your risk of experiencing problems during pregnancy, so it is important to discuss your CFRD with your care team. If you are taking insulin to control your diabetes, your insulin requirements may change once you are pregnant.
- Cirrhosis (liver disease): CF can be associated with severe liver disease, such as cirrhosis. Liver disease is also a potential complication of pregnancy with CF. For this reason, if you have liver disease, you should discuss the implications of getting pregnant with your CF care team.
- Post-transplant or immunocompromised for any reason: Pregnancy after a transplant is possible, but carries much greater risk as some immunosuppressants can cause birth defects, and other health related problems may emerge. Talking to your transplant team before getting pregnant is very important. Learning about it can help you understand the risks and health implications.
- Chronic kidney disease: If you have chronic kidney disease, you are at higher risk for complications such as preeclampsia, premature delivery, and caesarean delivery. People with chronic kidney disease who are considering becoming pregnant should discuss the risks with their CF care team as well as a kidney specialist.
- Psychosocial stressors: Situations in your life that can harm your health while pregnant include food insecurity, financial uncertainty, not having adequate health insurance or social support, and experiencing domestic abuse. You can contact Cystic Fibrosis Foundation Compass, a personalized service that can help you with insurance, financial, legal, and other issues, at 844-COMPASS (844-266-7277).
- Anxiety: Anxiety is a normal emotion that comes and goes in response to fears or worries about changes in health, work, relationships, or money. A person is said to have an anxiety disorder if the anxiety does not go away, gets worse over time, and prevents a person from participating in daily activities.
- Depression: Unlike ordinary sadness, depression can last for a long time if not treated. It can make people feel hopeless and lose interest in things they normally would enjoy.
- Advanced CF lung disease: Advanced CF lung disease (ACFLD) can increase risk for people who are pregnant as well as newborns, including premature delivery, caesarean delivery, and low birthweight. People with ACFLD who are considering getting pregnant should talk with their care team.
- CFTR modulator therapy: Talk to your CF care team about important considerations if you are taking a CFTR modulator and are planning to get pregnant.
Potential Complications of Pregnancy With CF
The majority of CF pregnancies result in live births and there is no clear increased risk of fetal death or birth defects. But this does not mean getting pregnant comes without risks if you have CF.
The most common complication of pregnancy with CF is preterm — or premature — delivery, which, in one study, was associated with low pre-pregnancy lung function.1 Diabetes was also reported to be more common in people who delivered prematurely.
Overall, adequate nutrition, well-controlled blood sugars, and a reasonable pre-pregnancy FEV1 (60% or more of predicted) are associated with better outcomes.
In addition to premature delivery, some of the most common complications associated with pregnancy and CF include:
- Exacerbations: Research suggests that some pregnant people with CF may experience more exacerbations, so it is important that you continue your CF respiratory therapies throughout pregnancy to stay as healthy as possible. Your CF care team and obstetrician will want to discuss whether any of your treatments should be stopped or changed during pregnancy — especially if you require antibiotics.
- Nutritional deficiency: Because your nutritional demands will change during pregnancy and put you at risk for inadequate weight gain or weight loss, most people with CF will need to increase their caloric intake, drink supplements, and focus on maintaining and gaining weight while pregnant. Many people with CF need to take nutritional supplements during pregnancy, so it is important to pay close attention to your nutritional status and work closely with your CF dietitian or another member of your care team.
- Gestational diabetes: Pregnant people with CF have been found to have a higher risk of developing gestational diabetes (diabetes that develops during pregnancy) than those in the general population. Many people with CF already have impaired glucose tolerance. Because the hormonal changes that accompany normal pregnancy often cause a degree of impaired glucose tolerance as well, people with CF are more likely to develop gestational diabetes than the average pregnant woman. For this reason, your care team should consistently screen for diabetes throughout your pregnancy.
- Constipation: Although it is common for all people to experience constipation during pregnancy, having CF puts you at a higher risk. To prevent constipation, you can take steps, such as drinking more water, adding fiber to your diet, and eating more fruits and vegetables. In addition, your care team may recommend the use of stool softeners or laxatives while you are pregnant. Contact your CF care team if you notice a decrease in the number or amount of normal stools.
- Vitamin A (retinol) toxicity: Because high levels of vitamin A — especially within the first three months of pregnancy — can be associated with developmental issues for your baby, it is important that your care team carefully monitors your vitamin A levels when managing your pregnancy. If you are taking vitamin A supplements, your obstetrician will likely have you stop taking them during your pregnancy. However, it is important that you do not stop taking your other CF-related vitamins without discussing it with your care team and obstetrician first.
- Hypertension: Pregnant people with CF have a higher risk of hypertension — high blood pressure — than pregnant people without CF. For this reason, your care team and obstetrician should closely monitor your blood pressure both before and during your pregnancy.
“Despite the fact that all pregnancies for people with CF will be considered ‘high risk,’ it doesn’t mean that your pregnancy will not be amazing. It just means that you have to keep a close eye on yourself, be very aware of your body and your health, and take extra great care of yourself and your baby.” Lindsey Tipsword, adult with CF, from the CF Community Blog.
Dealing With the Unexpected
Although planning your pregnancy is ideal, life does not always go according to plan. If you experience an unexpected pregnancy, your first step should be to contact your CF care team. Your care team will work with you to assess the possible impact that being pregnant could have on your health and help develop a care plan that makes sense for your situation.
The decision about how to proceed in the face of an unplanned pregnancy will depend on the trimester, as well as many other factors. For example, some people with CF may feel it would be difficult to care for both themselves and a child with CF, so getting your partner genetically tested could provide helpful information as you anticipate the impact on your health.
In addition, if you have advanced lung disease, you may need to consider your options. Only you can decide what is right for you, and discussing both the physical and emotional implications of your pregnancy with your CF care team can help inform that decision.
1 Jain R, Kazmerski TM, Zuckerwise LC, et al. Pregnancy in Cystic Fibrosis: Review of the literature and expert recommendations. J Cyst Fibros. 2022 May;21(3):387-395. doi: 10.1016/j.jcf.2021.07.019. Epub 2021 Aug 26.