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A Positive Challenge! Pregnancy in Cystic Fibrosis

Many women with cystic fibrosis (CF) are well enough to consider pregnancy and with the support of their CF and obstetrics team go on to have healthy babies. But how does pregnancy impact their health and how will they be affected after the birth?

One of the most important questions for women with CF is whether pregnancy will have a detrimental effect on their long-term health. The consensus now is that pregnancy does not significantly shorten survival. In a large study published by Gross et al, 680 women with CF who became pregnant were compared with women with CF who did not. It was found that the 10-year survival rate was higher for the women who became pregnant.

However, caution regarding pregnancy is advisable as individual women respond differently. Some women do experience a deterioration in their health during and after pregnancy and the long term prognosis needs consideration.

Women with good pre-pregnancy lung function (FEV1 >70% predicted) tolerated pregnancy very well and had the most successful outcomes and lowest maternal mortality. As lung function declines the risk of poor pregnancy outcome increases, as does maternal mortality.

Pulmonary hypertension and cor pulmonale (the abnormal enlargement of the right side of the heart associated with lung or pulmonary blood vessel disease) are considered absolute contraindications to pregnancy. There are also reports of poor pregnancy outcomes and increased maternal mortality associated with the presence of maternal infection with the organism B. cepacia.

It has also been suggested that women with CF who become pregnant have less severe disease and superior nutritional status than women who do not.

Pregnancy Post Organ Transplant  

Women with CF who have undergone lung or heart-lung transplant are generally advised to wait 2-3 years post-transplant before considering pregnancy to ensure graft stability and to reduce the risk of rejection. There are reports of successful pregnancies after lung transplantation but premature delivery is common and rejection or graft loss is more prevalent than in pregnancy following other organ transplantation.

Pregnancy Counselling

Accurate and unbiased advice and counselling is essential for women with CF who are contemplating pregnancy. A thorough assessment of the severity and rate of progression of the disease is crucial to obtain accurate information about the likely outcome of pregnancy and its effect on maternal health. Thorough discussion about the many challenges of parenthood when coping with a life-limiting disease is imperative. The possibility of maternal death and a partner being left alone to bring up a child is a difficult topic to discuss but should be considered.

CF and Your Baby

If you have CF your baby will be a carrier of the CF gene.  As part of the genetic screening process you will be offered counselling to help understand the results and make the right choice for you and your partner. As an adult with CF, your partner should be tested to see if they carry the CF gene. If they do there is a 50% chance your child would have CF.

Things to check with your CF centre:

  • Talk to your CF specialist about a medication review to make sure they are safe for your baby; some may need changing
  • Ensure weight and lung function are the best they can be to help baby grow
  • Check your blood sugar level
  • Advice regarding a fertility specialist referral if you are having difficulties falling pregnant

It is important to do all your treatments to make sure your baby grows and your lungs keep as healthy as possible to reduce the risk of preterm birth and complications. You need to maintain a regular routine of airway clearance, exercise and inhaled medications to reduce respiratory exacerbations during pregnancy.  Your airway clearance and exercise regimes may need to be adjusted during pregnancy. Regular check-ups with your specialist physiotherapist to make sure you are on track with treatment are very important. Some women may require intravenous (IV) antibiotics prior to conception and expected delivery date to ensure optimal health.

Pre pregnancy an oral glucose tolerance test should be performed to look for evidence of CF related diabetes (CFRD) or impaired glucose tolerance. Women with diabetes should be referred to a specialist in CFRD before conception.

Diabetes during pregnancy is more common with CF. Untreated diabetes may lead to weight loss, fatigue and decline in lung function. High blood sugars can harm your baby; it is therefore necessary to monitor your blood glucose levels during your pregnancy. Testing for diabetes is also important, as the incidence of gestational diabetes is higher in CF and failure to diagnose and treat it may be associated with poorer pregnancy outcome. An oral glucose tolerance test is recommended in the first trimester and at 24-28 weeks of gestation in all women who have normal glucose tolerance prior to pregnancy. Optimising glucose stability will reduce the risks of congenital malformation and pregnancy complications.

First trimester ultrasound, for accurate dating of the pregnancy, and a second trimester anomaly scan is recommended. Women with pre-existing diabetes may also be offered a foetal echocardiogram. Regular ultrasound assessment of foetal growth and wellbeing in the third trimester is essential.

Nutritional Considerations in Pregnancy

Good nutrition is vital for your health and to store nutrients to help your baby grow. Focus on eating nutrient rich foods and make every mouthful count. The increase in energy requirements while pregnant can make gaining weight a challenge, however weight gain is essential for baby’s growth and development and those who gain adequate weight during pregnancy have better outcomes and bigger, healthier babies. If you have CF and are pregnant you will need to eat a range of foods from the five food groups in three meals and many snacks daily to meet your nutritional needs and those of your baby. Talk to your dietician for advice on increasing energy intake for your pregnancy.

Vitamins and Pregnancy

Taking your vitamins is important to build your stores to have adequate levels for your baby. Your CF team will advise you on additional vitamins needed in pregnancy.  Folate and iron are important for all women planning a pregnancy.

Folate (folic acid) is a B-group vitamin essential for the healthy development of the foetus in early pregnancy, in particular their neural tube. It is recommended you take folate supplements 3 months pre pregnancy to reduce the risk of neural tube defects e.g. spina bifida.

Calcium and vitamin D are important for baby’s bone growth. Pregnant women with CF need more than this because calcium is not always absorbed in the gut. It is important dairy products are stored safely to avoid contamination with bacteria including listeria (a bacteria that can cause infection and serious illness in pregnant women). You need to take your vitamins A, B, D, E and K to build up your stores.

Pregnancy Concerns

Alcohol: No amount of alcohol is safe in pregnancy and therefore should be avoided.

Gastro Intestinal Issues: Hormonal changes and the enlarging uterus often give rise to troublesome gastrointestinal issues in pregnancy. Discuss issues such as morning sickness, reflux and constipation with your CF team as there may be different solutions for each person.

Continence: Pregnancy may weaken the pelvic floor muscles due to the weight of the growing baby and pregnancy hormones which soften the ligaments in the body and the support structures in the pelvic floor. If you have poor bladder control and have urine leakage with coughing, airway clearance, laughing or exercise you will benefit from seeing a women’s health physiotherapist or contacting the National Continence Helpline on 1800 33 0066.

Delivery

An individual delivery management plan should be made with gestational age, disease severity and foetal wellbeing all being taken into account. Elective preterm delivery may be required if maternal health and lung function is declining or if there is evidence of restricted foetal growth. Elective preterm delivery is usually by caesarean section. In the majority of cases, if maternal health is well maintained and the baby is growing normally, vaginal delivery can be anticipated at term. Lung function should be optimised prior to delivery and this may require admission to hospital in the third trimester for intensive physiotherapy and antibiotics prior to induction or caesarean section.

The outlook for people with CF has improved dramatically in recent years. The long-term prognosis in the majority of women is unaffected by pregnancy and, if lung function is maintained, it can be undertaken safely. Women with poor lung function have an increased risk of pregnancy complications and maternal death. Pre-pregnancy counselling and the input from the multidisciplinary team during pregnancy is essential to optimise the outcome for mother and baby

Breastfeeding

Breastfeeding is exhausting and uses up calories so women with CF may find it difficult to breastfeed exclusively. If women are unable to maintain their weight, alternative methods of feeding should be discussed. The safety of maternal medications should also be taken into account when considering the mode of feeding.

Support

It is important to think about the kind of support you will have after the birth of your baby. Do you have a partner, friends or relatives who can help? Those with limited support systems are more likely to have a decline in health after the birth, probably because they do not have as much time to look after their own heath needs.

You might also want to consider how you will be affected by the birth not only physically but on an emotional level. It is hard work caring for a baby and looking after your own health at the same time. Some women with CF have reported worrying about their child and how their health may affect them in the future.

Pregnancy will be an exciting time and at the same time a little daunting. The CF team will be expecting plenty of questions in relation to your health needs.

Useful Resources

Reference

Goddard Julia/Bourke Stephen J. Review Cystic fibrosis and pregnancy 2009.

 

Article from RED Magazine, Edition 2 2016.


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