CF and the desire to have children

Question

Dear expert team,

I will turn 28 in November, am 1.70m tall, and weigh 45kg. My FEV1 has been stable at 52% for the past 15 years. CF has not been proven genetically but was diagnosed symptomatically. My fiancé is fit as a fiddle except for a thyroid dysfunction. We have good family support at home. I expect to graduate in 2010, and the prospects to get a job in my favourite profession are good.

We are now wondering whether this is a good time to get pregnant. Unfortunately I do not know whether I meet the health requirements for a pregnancy. As for permanent medication, I am taking azithromycin, DNAse, pantoprazole and vitamins as well as NaCl and a combination of fenoterole and ipratropiumbromide.

Unfortunately my clinic is rather anti-pregnancy with CF, and I am therefore a bit reluctant about getting consultations there. Is lung function sufficient to cope with a pregnancy? Which factors do I need to consider beforehand?

Answer

Dear questioner,

If you do want to get pregnant, I would consider this the right time too. Do you have stable, regular cycles? Like many CF patients, you are underweight, which could potentially complicate the goal of a spontaneous pregnancy.

A stable FEV1 of more than 30%, which you have, is one of the basic prerequisites for a pregnancy. If possible, you should do without the azithromycin, even though no effects on a pregnancy have been reported. Instead of pantoprazole, giving ranitidine (drug of choice during pregnancy) would be better, or otherwise omeprazole, if ranitidine is not sufficiently effective.

In case you have further questions, do not hesitate to adress to me again.

Dr. A.-U. Stücker

19.11.2009

19.11.09
Please find extended information on the topic pregnancy and CF in the article from Edenborough et al. 2008, Journal of Cystic Fibrosis 7: S2-32 "Guidelines for the management of pregnancy in women with Cystic Fibrosis".
D. d'Alquen

20.1.2010
As this is an interesting question which is not trivial to answer some additional comments on it are published here:
Before getting pregnant, there are quite a few things that have to been taken into account: one important point would be the carrier-screening of the male partner, in order to predict the risk of the child to suffer also from CF (genetic counseling) as well as psychosocial counseling. Furthermore, it is important to optimize preconceptional health and treatment (e.g. use of drug therapy in pregnancy, optimizing lung function and nutrition etc….).

The question, “can a pregnancy be recommended or not” is even more delicate to answer: it is not easy to find in the Literature “the FEV1 cut-off value” which would speak for or against a potential pregnancy. The decision for or against a pregnancy depends on several aspects of the individual course of the disease and, even if a higher FEV1 level is of advantage, the mentioned FEV1 of 52% is not a contraindication for a pregnancy, as many healthy pregnancies have been reported with such an FEV1 level. However, it is important to discuss this with the CF care team in charge, regarding all the other aspects of interest (such as nutrition, diabetes, microbiological issues and liver disease and psychosocial aspects as well), taking also into account the importance for the quality of life a baby means to a couple who deeply wishes to have a child and so finally to come to a consensus together.


One important paper on the topic is:
Edenborough, F.P., et al., Guidelines for the management of pregnancy in women with Cystic Fibrosis. Journal of Cystic Fibrosis, 2008. S2-S32.
D. d’Alquen