Thursday, January 1, 2015

Why I Chose To Stay On Kalydeco While Pregnant

I frequently get e-mails from women with cystic fibrosis who have gotten pregnant (expectedly or unexpectedly) after starting Kalydeco and are desperate for information and advice about the safety of Kalydeco during pregnancy and breastfeeding. Apparently I'm one of the few Kalydeco mamas out there blogging about the experience, because they all seem to find their way to my blog! (Ironically enough, most of them are from the UK, and literally every single one has told me that she is probably the first UK woman to ever be pregnant on Kalydeco, which is obviously not true. UK cysters, clearly we need to teach your doctors a thing or two!)

Because I get this question so frequently, I figured it was high time that I have post detailing my Kalydeco conception experience and why I chose to stay on a drug with zero human data for pregnancy.

To those who are finding this post by searching about Kalydeco pregnancy, I do so understand the anxiety you're feeling. I got pregnant only a few months after the drug was given FDA approval, and at the time I knew of only one other woman in the world who had gotten pregnant on Kalydeco—and she was only two or three months further along than I was. (Later, I learned that there were one or two women who also got pregnant right around the same time I did, but we didn't know about them until after I'd delivered Kate.) Not only was my pregnancy a (blessed) surprise, but I went into my decision-making process without any anecdotal evidence whatsoever. These days, in addition to the rodent study data that helped me make my choice, there's a wealth of evidence-by-mouth that Kalydeco is fairly safe in both pregnancy and nursing. I can think of at least half a dozen CF mamas who have stayed on Kalydeco through all or most of their pregnancies and now have healthy, happy babies—and many of us have successfully nursed them too (I'm still nursing Kate once daily at 20 months). If you're pregnant or trying to get pregnant and you're on Kalydeco, I HIGHLY recommend joining the Facebook group Cystic Fibrosis Mummies. Not only are there hundreds of women in there who have successfully achieved motherhood in one fashion or another while living with CF, but there are lots of Kalydeco mamas who are happy to share their personal experiences. It's a "secret" Facebook group, but if you're interested in joining drop me a line or message either of the creators, whose information can be found here.

Before I launch into my story, here is a big fat disclaimer: All of this information pertains only to Kalydeco (ivacaftor). I have no experience with any of the combination drugs, including Orkambi (ivacaftor+lumacaftor), and can't vouch for its safety in pregnancy. And, of course, I'm not a doctor or pharmaceutical researcher, so please take this only as my own experience and not medical advice! However, I have done a post specifically about what I know concerning pregnancy and Orkambi because it's a question I get asked so often. You can find that here.

How Kalydeco Got Me Pregnant

Well, okay, my husband helped. ;) However, I've never in my life had anything that resembled a normal 28-day menstrual cycle; ever since I was a teenager my cycles have been extremely long and irregular, and they often stopped completely for months at a time when I got sick (like that time I didn't have a period for nine months and then my hospital pregnancy test was "inconclusive"before I'd ever had sex). I had always suspected that this would spell the doom of my hopes for normal fertility, and I was correct. Before getting pregnant with Kate, we tried for a little over a year and a half with nothing to show for it except the doctor telling me my hormone levels were so low that I was nearly menopausal (at 23!), and that there was no explicable reason for it. Although we could never find any medical evidence for my infertility, and CF specialists still insist that CF does not greatly impair female infertility, it was fairly clear to Mahon and I and all of the doctors who were treating us that my fertility problems were due to the stress that living with CF places on my body.

A few weeks after I started Kalydeco, I suddenly noticed a host of weird PMS symptoms that I'd never had before—and after four years of marriage and a year and a half of infertility (during which I charted my temperature and other fertility signs), I was fairly sure I'd had every symptom in the book. Although I did have a period that month (a very early miscarriage), I had been pregnant, and all of those same symptoms came immediately back the next cycle—my pregnancy with Kate. It was fairly clear that Kalydeco had improved my hormonal balance to the point where I could conceive.

Why I Chose To Stay On Kalydeco During The Pregnancy

Obviously, as soon as I had that positive pregnancy test in hand, the first thought on everyone's mind was whether or not I would stop Kalydeco or remain on it through the pregnancy. As I mentioned above, at that time I had only heard of one woman in the world to stay on Kalydeco during pregnancy, and since she was only a few months along her story didn't even help us much, since there was no telling whether Kalydeco was going to have an effect on her baby yet. My doctors and I had discussed the possibility of a Kalydeco pregnancy when I started the drug, but because we had been infertile for quite some time already and weren't going to be able to start fertility treatments for a few months after I began Kalydeco, we were sure that was a "far in the future" kind of concern and hoped that at that point there might be more information available.

The day that I got my positive test, I called my doctor's office with the news and also spent a lot of that afternoon combing through all the literature on Kalydeco I could find. The only real information available was from the package insert, which touched briefly on the pregnancy and lactation studies they had done with rodents while Kalydeco was in early stages. (It's listed under section 8.1-8.2 in the package insert.) Although Kalydeco was found both to cross the placenta and be excreted in the milk of lactating rodents given Kalydeco, no evidence of teratogenicity (toxicity causing birth defects or other issues with a fetus) was found in the animals studied even at 6-12 times the human dose. Although rodent studies are not perfect and there are many documented cases of humans reacting very differently to drugs than studied rodents, it's still comforting to know that there was no teratogenic effect even at enormous doses—and the rodent trial results were enough to get an FDA Category B labeling in the United States, which is a fairly difficult pregnancy class labeling to get. (Most CF medications are Category C.) All of this information did help me feel a little bit calmer about the possibility of remaining on Kalydeco.

My doctor is extremely conservative when it comes to medication and pregnancy and was very uncomfortable with the idea of me staying on Kalydeco while pregnant, but he was equally uncomfortable with the idea of me going off it. There is a growing body of belief—both in the medical community and the CF community—that going off Kalydeco once you're on it can lead to very fast declines and sometimes permanent lung damage that doesn't reverse even after you resume taking it. We both felt that the early stages of pregnancy were not a good time to risk acute respiratory distress and lung damage. Additionally, because it was so clear that the Kalydeco had altered my hormone levels to make it possible for me to conceive in the first place, my doctor and I were both very worried that stopping the Kalydeco would lead to miscarriage as my hormone levels dropped again.

And lest the above paragraph make it sound like my doctor generally preferred the idea of me staying on Kalydeco to the idea of me going off it, let me clarify. He didn't like either option! ;) We had a long phone conversation about all of the potential problems with either choice. Ultimately, he said that he felt he couldn't even really advise me about what choice to make because they both seemed like such bad options, but he said that he would support me in whichever choice I made. We also discussed potentially going off Kalydeco just for the first trimester (when the risk of birth defects is greatest) and then resuming it, but agreed that that would still carry the risks of serious lung illness and miscarriage.

I spent the rest of the day talking to my husband and doing some serious soul-searching and praying about my options. Ultimately, we both felt peaceful about me staying on Kalydeco, feeling that the risks were much greater to both me and the baby if I was to go off of it.

The Happy Ending

As you can probably tell if you've read my blog for awhile, my pregnancy went very well (despite a host of strange non-CF-related complications) and Kate has always been a normal, healthy baby. She has never appeared to have any negative effects from Kalydeco, despite the fact that I stayed on it through my whole pregnancy and have continued taking it while breastfeeding, which I'm still doing. I also have several friends who have had similarly uneventful experiences with Kalydeco during pregnancy and nursing. In addition, I completely attribute the fact that my lungs were very healthy throughout my pregnancy to Kalydeco, since I was enormously more stable than I had ever been before in my adult life. I am almost certain that had I chosen to stop Kalydeco I would have had a very rough pregnancy in terms of pulmonary health.

I hope that this information is helpful to anyone who might be wrestling with this decision right now! I'd be happy to answer more questions via e-mail (my e-mail is on my blog sidebar), and happy to add any prospective or expecting CF moms to the Facebook group I mentioned above.

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