Cystic fibrosis (CF) and pregnancy
What advice will be given to a cystic fibrosis patient who wants to conceive?
There is no standard advice. In short, she must be in a satisfactory physical shape without any serious complications of cystic fibrosis.
The one important contraindication to pregnancy is if she has developed pulmonary hypertension, where the blood pressure in the arteries in the lungs is abnormally high. Pregnancy in such a situation could cause heart failure, which many necessitate terminating the pregnancy as a life-saving measure.
The physician looking after the woman will carefully assess the effect of the disease, including on her lungs, the gut, pancreas (risk of diabetes) and heart, before giving his or her "blessing" for the planned pregnancy.
What is the advice regarding the prospects of the offspring?
This is a difficult area. One consideration is the inevitably limited life-span of the mother. This needs to be looked at critically by the prospective parents. The second issue is the possibility of the child being affected. The mother will definitely pass on the rogue gene. It may be important to check whether the partner carries the gene. About 5 per cent of the Caucasian (white) population carry the gene, so the chances among them is relatively high. The rate among those of African or Oriental descent is much lower.
A test to check the status of her partner is available but it detects only about 70 per cent of the carriers, and therefore there is still a risk of a carrier being missed.
Can pregnancy worsen the condition of the patient?
This is possible. A careful eye will be kept on the pregnant woman through the course of the pregnancy.
Will physiotherapy and antibiotics continue to be necessary during pregnancy?
Yes, and more so. Penicillin or a form of cephalosporin commonly used to prevent infection are safe.
Also inhalational drugs to help dilate the lungs will almost certainly be required on a regular basis. All these are known to be safe.
For a cystic fibrosis sufferer, is the baby at any kind of risk?
By virtue of the respiratory and nutritional problems cystic fibrosis patients encounter, the risk of growth restriction in the womb is quite considerable. Sometimes it gets so bad that preterm delivery becomes inevitable.
Other maternal complications such as respiratory or heart failure may also prompt preterm delivery.
Is induction of labour any different if the woman is a cystic fibrosis sufferer?
No. Prostaglandin E (Prostin)- which is the most popular induction agent - is safe to use in cystic fibrosis sufferers. Oxytocin (Syntocinon) is also used, when necessary, to augment labour.
What about pain relief in labour?
In cystic fibrosis, there is a slight worry about the effects of opiates such as Morphine or Pethidine, because of their depressive effect on respiration. Because of this, epidural analgesia is strongly encouraged among these patients. This has additional advantages in that it prevents unnecessary exertion in the late stages of labour, something a cystic fibrosis patient can ill-afford. In addition, if a caesarean section becomes necessary, this may be the only form of anaesthesia required, avoiding a general anaesthetic.
An epidural is, by far, the most effective form of pain relief in labour.