Conceiving and pregnancy with cystic fibrosis: a case report

It is now no longer rare for women with cystic fibrosis (CF) to have children. However, pregnancy with CF does have its risks and should therefore be well thought-out and prepared. A mother with CF shares her experience in the PARI blog.

Do I want a child?

Since I can remember I have been leading a “normal life” despite the fact that I have cystic fibrosis. And I do everything I can to keep it that way – such as doing an inhalation therapy twice a day. When my healthy, younger sister had a child 15 years ago, it was the first time I was confronted with the question of whether I want a child some day. And if so, is that even possible with my sick body? Will my health change because of a pregnancy? And even though I did not have a specific desire to have a child back then, I wanted to know what my options were. Did I have that choice? Could I allow myself to want to have children?

Because I wanted clarity, I just came out with the question to the respiratory specialist I had at the time. And he told me “One child is fine. I would not advise you to have more than that. Every child you have could worsen your lung function and general state of health. Pregnancy is a strain on the body, as is bringing up children. I have four.”

That was when I realised that if I wanted to have a child, I should not wait until I hit 40. The lung function of patients with cystic fibrosis does, after all, tend to worsen and not improve with age. The better the lung function, the better the baseline for a pregnancy.

Women with cystic fibrosis are at a certain risk of needing longer to get pregnant – that’s what I was told. This is why I urged my gynaecologist to refer us to a fertility clinic straight away. I did not want to waste precious years and FEV1 scores to end up finding out that it was not going to happen naturally. After six months of trying without success to conceive, he then referred me to a fertility clinic.

IVF is a time-consuming and in my case sometimes very painful procedure. Collecting the eggs from the ovaries was painful. It is normally done under general anaesthetic, or at least it was at the fertility centre I visited. To protect my lungs, they did not use anaesthetic on me.

Collecting the eggs was not quite as painful as the giving birth, but it came quite close. I was so weakened by it and my circulation was so depleted that I almost ended up being hospitalised. Fortunately, this was something I managed to avoid. After the eggs were collected, I did have to spend three to four days on the sofa though, to recover. You usually get a sick note for one day afterwards, and then you are fine.

At least my body had produced enough eggs that we had enough “material” for four to five attempts. The first attempt ended in an ectopic pregnancy, which meant I had to have vacuum aspiration and a scrape. The second attempt was unsuccessful. The third time we were lucky! I was pregnant! The foetus’s heart was beating and I returned to the fertility clinic under the care of a gynaecologist.

Aside from one nasty infection I caught in my third trimester (it was winter), I was generally well during my pregnancy. Even the infection did not pose any major complications for me or my child, although I did end up having to have intravenous antibiotics.

By that time I was working freelance and less than 40 hours a week. Being freelance meant I could take my time and take breaks when I wanted. That worked out well. There were a few weeks when I was very tired and I needed a lunchtime nap every day.

I also had more doctor’s appointments than I had had before the pregnancy. I was closely monitored by my gynaecologist, pneumologist and diabetologist – I had to go to one or the other doctor every week or two.

There were also tests such as prenatal diagnostics, which raised another worrying question: What do I do if my child is severely disabled? Should I then have the child and put my body through that if my child will maybe die because of a congenital disease before he or she reaches the age of three? Would I be able to bring up a severely handicapped child when I myself have health hurdles to overcome? What kind of life is worth living? Fortunately, we never had to make any difficult decisions like that.

My pregnancy was without complications or problems – well, almost. Because my child was born five weeks too early. There is a certain risk of premature births in pregnant women with CF. But nobody had expected this to happen in my case. There were no warning signs. One day before my waters broke I even went to the gynaecologist at the obstetric clinic.

So I was badly prepared for the birth. I had not packed any drugs or clothes for the hospital. I had not cooked and frozen any meals for when we came back from hospital. Nothing. Oh well... We still managed somehow. I would still, however, advise pregnant women who have CF to make the following preparations before their due date:

• Pack your hospital bag including medications
• Cook and freeze meals: with a newborn there was no time to cook something for myself
• Keep high calorie liquid meals at home
• Get your partner to promise that they will take at least two weeks holiday after the birth (even if the baby comes early)