Cystic fibrosis and pregnancy

Cystic fibrosis is a medical condition caused by a defective, inherited gene. Due to the malfunctioning of this gene, a an individual will have thick and sticky secretions of the sweat, saliva, and digestive juices. Severe lung damage and nutritional deficits occur which makes CF a life threatening disorder. Symptoms are skin saltiness, failure to grow, greasy, foul smelling bowel movements, rectal prolapse, coughing, wheezing, and clubbing of the fingertips.

For the pregnant woman with cystic fibrosis, the disease becomes more challenging which requires special care. Many women are infertile due to the changes in the fallopian tubes from cystic fibrosis. When women are able to become pregnant early prenatal care is essential to deliver a healthy, normal infant.

Planning ahead, prior to becoming pregnant, is important to the birth of a healthy infant. Women must carefully plan their nutrition and exercise. Weight should be within normal range for height, good nutrition should be practiced and lung capacity should be around 40% to 50%. The woman\’s partner should also undergo screening prior to pregnancy to determine his carrier status for for the disease.

Nutrition during any pregnancy should be above average, but this is especially true in the woman with cystic fibrosis. A good caloric intake to maintain nutrition and increase weight gain should be included. If this is not accomplished a pregnant woman with cystic fibrosis may need additional care at the hospital. She would receive a long-term IV fluid regimen during her pregnancy. In the last 3 months of pregnancy it is not uncommon for the pregnant cystic fibrosis patient to be admitted for specialized care and monitoring.

Any sign of respiratory distress is a red alert signal. It should be treated immediately with antibiotic therapy. The cystic fibrosis patient needs to receive adequate oxygen flow as soon as possible, especially now that she is pregnant. Medications used to treat cystic fibrosis are safe for the baby and the woman is strongly encouraged to continue them. Breastfeeding is also advised for women who suffer from cystic fibrosis because it is the best nutrition for the infant and will not cause problems for the woman.

The life expectancy for a cystic fibrosis patient ranges between 30 and 40 years old. It is a difficult decision to make when determining whether a cystic fibrosis patient would want to become pregnant knowing this fact.

New advancements and greater technology are enabling patients with life threatening illness, including cystic fibrosis patients, to live a longer life than what they knew previously. Lung transplants can be done to lengthen the life of a CF patient and have been known to do so with an approximate 5-year additional prolonged life expectancy. However, not every one will qualify for this procedure.

If you are pregnant and discover that you are pregnant or are planning on becoming pregnant see your health care provider as soon as possible in order to give your baby and yourself the best possible chance in regards to healthy living.

RESOURCES

March of Dimes: Carrier Screening for cystic Fibrosis

Current Opinion in Pulmonary Medicine: Pregnancy in Cystic Fibrosis

Journal of the American Medical Association: Pregnancy in Cystic Fibrosis

Clinical Obstetrics and Gynecology: Cystic Fibrosis in Pregnancy