More Women with Cystic Fibrosis Are Becoming Mothers

With new treatments on the horizon everyday for people with Cystic Fibrosis (CF) more and more women with this disease was making the decision to become mothers.

Cystic Fibrosis is a progressive, often fatal genetic illness that affects primarily the respiratory and digestive systems. Patients often have frequent troubles with lung infections and low weight gain from malabsorption of the nutrients in their food. CF was once referred to as a childhood illness, as very few infants diagnosed lived past early childhood. However, with a median life expectancy near forty years old and treatments that are improving quality of life everyday for people with this disease, many women with CF have now reaching their child bearing years and deciding to have children.

While it was once the case that if a woman with CF reached her child bearing years, the probability was that her health by that time would not be adequate enough to sustain a pregnancy. Many CF women today are finding that this is no longer the case. Most girls with CF are reaching their child bearing years and so the idea of motherhood is now a common topic of conversation. In fact, many CF centers are now preparing guidelines for CF women choosing to look into pregnancy and becoming mothers to help with their CF care both during and after gestation.

One of the biggest factors that doctors are concerned with for CF mothers-to-be is the condition their lungs are in prior to pregnancy. Studies, such as a recent publication in the Journal of Cystic Fibrosis have shown that while mothers with mild to moderate lung involvement suffer no ill consequences from pregnancy, mothers with poorer lung function often decline during pregnancy. Lung function for those with Cystic Fibrosis is tested with an easy test known as a Pulmonary Function Test (PFT). This test measures what the lungs are able to expel during a short period of time and can look at what both the larger and smaller airways are capable of. As a CF patient's lung function declines, so do the numbers on their PFT test. Ideally, doctors prefer to see patients PFTs to be at least 50% of their predicted ability to even think about pregnancy, and of course find that the higher the percentage on the test, the better the outcome for the mother during pregnancy.

CF patients are often on many medications to help control their CF, and while many are safe for pregnancy, some are not. CF patients and doctors have to work together to find the right combination of medications that will work to keep both mother and baby safe. For this reason, women with CF who are entertaining thoughts of motherhood should speak with their doctors before becoming pregnant, so that they are able to help formulate a plan with their physicians for their care during the pregnancy.

Another concern for CF mothers-to-be is adequate weight gain. In a healthy pregnancy it is usually suggested that the mother gain around 25 lbs. Weight gain can sometimes be difficult for the CF patient due to their body's difficulty with digesting foods. The CF mother-to-be should plan on increasing her caloric intake significantly during pregnancy; she may want to add supplemental shakes into her diet as well. Some women find eating small quantities of high calorie, high fat foods frequently throughout the day to be helpful.

If lung function is stable and weight gain adequate, most women with CF go on to have healthy, safe, and uneventful pregnancies. Delivery generally poses little risk to the CF mother, and most deliver vaginally unless an outside force, often unrelated to CF (breech position, labor not progressing, etc.) intercedes.

Due to the thicker nature of mucous in women with CF, some have a hard time being able to conceive. This is because the mucous in the cervix sometimes prevents sperm from being able to swim through. Some CF women conceive successful by introducing a mucous thinner into their repertoire, such as guaifenesin, found in over-the-counter cough medications (women trying this should be sure that they only use guaifenesin, as other medications can actually dry up secretions). Taking this supplement before trying to conceive can sometimes thin the cervical mucous enough that the CF woman is able to conceive naturally. Others find they must use medical intervention o conceive. Due to the fact that having Cystic Fibrosis can place an added toll on the body on top of pregnancy, some doctors are hesitant to introduce any medical conception techniques that might result in multiple births. However, many CF women have reported successful pregnancies after both medicated and unmedicated intra-uterine insertions (where doctors use a small catheter to place the man's sperm directly into the woman's uterus - most often aided with the help of follicle stimulating medications such as clomid, but can be done without) as well as in-vitro fertilization techniques. There are reports of successful pregnancies and births of multiples in women with CF as well.

After birth care is as important for women with CF as pregnancy care was. For some women, now caring for an infant places an added stress into their daily lives as well as an impediment in their daily treatment routines. It is imperative that the CF mother has adequate support so that her treatments, and thus overall health, do not suffer as a result of caring for her child and not herself.

At one time there was a misconception that CF women would not be able to breastfeed: that their bodies would not be able to sustain feeding an infant and that their milk stores were inadequate. This has proven to be untrue. If the CF woman wants to breastfeed, she should not be discouraged form doing so. She will still have to maintain a high caloric intake while nursing, but she can rest assured that her milk is nutritionally sound for her infant (also documented by studies gathered in the Journal of Cystic Fibrosis). As long as the CF mother doesn't lose an unsafe amount of weight from nursing, nursing does not harm her body. Further, most medications for CF have been found to be safe for nursing. It is highly suggested that the nursing CF mother get a copy of the book Medications and Mother's Milk by Thomas Hale, PhD to keep on hand so that she can be on top of the latest lactation research. There is still a raging ethical debate for women with CF who want to be mothers. The debate is two fold. First, inevitably, the CF mother will pass down to her child the gene that carries CF. If she does not have her partner genetically tested prior to becoming pregnant they also run the risk of having a child with CF. Though a carrier of the CF gene does not have CF, that person does carry on the gene that causes CF and could affect future generations. Second, with a median life expectancy of still not quite forty years old (36.5), many mothers face dying while their children are still quite young. While some with CF still die quite young, there are other who are living into their fifties, sixties and even seventies. Many women who are now thinking about becoming mothers have to base their decisions on these criteria, as well as taking into account the medical advancements that are coming down the pipeline. Emotions can get heated in CF communities on this topic, but most women with CF who have children or hope to have children find that they are willing to work even harder to maintain their health because they plan to be around for a long time. And they have good reason to be.

Works Cited:
Science Direct. 2008. Journal of Cystic Fibrosis. Vol.7, Supplement 1. January 20, 2008. S2-S32
http://www.sciencedirect.com>