Yesterday there was some buzz around the CF community about a new study being done on fertility in women with Cystic Fibrosis. I am so excited that this area is receiving more research attention! It wasn’t until the last 10-20 years that so many women with CF were living long enough to sustain pregnancies, so research on women with CF conceiving and carrying pregnancies is really hard to come by. There’s even less known about why women with CF often have issues with infertility. So, even though this will be a small study, I’m happy that it’s even being done.
In fact, an exciting thing about it is that it’s focusing on how women are planning (or not planning) pregnancies while using the new CFTR-modulating drugs Orkambi and Kalydeco. For those of you unfamiliar with these drugs, they are the very first medications that successfully target the root issue that causes CF: the defective proteins at the cellular level. Everything else on the market just treats the symptoms. Though they’re not a cure, these drugs are a breakthrough and the start of a very important line of biomedical research that could lead to a cure. Patients that take them have fewer infections and, generally, an improvement in lung function – though the effects are more dramatic in some patients than others. That may not sound like much, but it’s actually huge progress. This is a progressive disease in which lung function gets worse over time and infections can kill, so slowing, stopping, or even reversing that trend is a Big Deal.
Another somewhat unintended effect of these drugs is an improvement in fertility. The thick mucus CF causes doesn’t just affect patients’ lungs and digestive tracts – it can also cause blockages in the reproductive system, preventing sperm from being able to reach the egg. When the thick mucus is reduced or eliminated by the medication, suddenly women who were infertile for years can become pregnant – either on purpose or not! The drug can also interfere with hormonal birth control, making it less effective.
I haven’t written about Orkambi on this blog yet, though at one point I wrote a long draft post about it (and just couldn’t seem to find the right words to say how I felt). Orkambi just went on the market this past summer after years of research and development, which I followed very closely. I have the genetic mutation that Orkambi treats and I was waiting eagerly for years for its release. Unfortunately, because it’s such a new drug, its effects on pregnant women and fetuses are unknown, so my doctor told me I could not take it if I am trying to conceive. As a result, I’ve never started taking it.
To be honest, it’s kind of a painful subject for me. After so many years of waiting, this potentially life-changing drug is finally here – but I can’t have it. The timing is exactly wrong. I probably won’t be able to start it for at least a year, after my (theoretical) baby is conceived, born, and finished breastfeeding. It really hurts when I think about it. It’s a sacrifice I’m willing to make, but it’s also a big part of the reason we’re accelerating our attempt to get pregnant by starting IVF. The longer I wait to get pregnant, the longer it will be before I can start Orkambi.