It was summer vacation; I was 13 and in-patient for a "tune up" when my pediatric cystic fibrosis doctor sat me down to have "the talk" as part of the imminent process of transitioning into self-care and adulthood. Although we discussed other embarrassing topics (at least according to a 13-year-old), I most vividly remember his explanation of pregnancy in CF. It included a bit about the importance of a planned pregnancy as a way to ensure that your health was as stable as possible before trying to conceive. His words -- used as my motivation to stay consistent with treatments and make wise decisions -- have stuck with me to this day.
Perhaps this is why when my husband and I were given the thumbs-up from my CF doctor to start trying for our own family, it felt like a small victory 12 years in the making. We were referred to a maternal-fetal medicine (MFM) physician, or high-risk obstetrician-gynecologist (OB-GYN), for a preconception consultation to gather information and receive recommendations as our first steps to parenthood.
Both my CF team and MFM team are located at my absolutely favorite hospital!
The appointment began with a wonderful medical student taking a detailed medical history to identify any risks or issues that the physicians should be aware of. Past surgeries, acute conditions and more chronic issues, like cystic fibrosis-related diabetes (CFRD), were documented for further discussion by the MFM resident who joined us shortly thereafter. The resident was very encouraging and said she believed that I was in a good place health-wise to become pregnant. Yay! She noted that I have good follow-up with my different specialties, consistency with treatments and medications and periods of stability -- all positive habits that they look for in a patient wishing to manage a pregnancy and motherhood with a complex chronic illness. My lung function, on the other hand, was "something they could work with," but I took that comment as a compliment.
Next, the MFM fellow joined us and dove into the risks and what-ifs of pregnancy in CF. Honestly, this was the slightly alarming part of the appointment. We discussed the possibility of exacerbation and lung function decline, the effects of CFRD on both mom and baby (good blood sugar control is key) and the importance of gaining weight and being proactive about nutritional support if needed. The medical jargon and worst-case scenario speeches were nothing I hadn't uncovered in my own research, but hearing them come from the physician's mouth in the cold exam room rattled me a bit. I quickly reminded myself that no two patients, especially CF patients, are the same. Our experiences and outcomes will differ, but it's best to be prepared.
Last, we turned to my long list of medications. To my surprise, most of my daily medications were safe to take while trying to conceive and during pregnancy. However, the high-dose ibuprofen taken to treat and reduce inflammation in my lungs would need to be discontinued during pregnancy. I mentally waved a sad, preemptive goodbye to my favorite medication, and the physician and I vowed to stay in contact to finalize a plan for two other potentially questionable medications.
As the appointment drew to a close, the physicians recommended an echocardiogram (ultrasound of the heart) to rule out the possibility of pulmonary hypertension and explained that this was a pretty standard test requested for patients with lung disease. They told me that a summary of all the information discussed would be shared with my CF doctor, and as a group, we would agree on a plan for moving forward.
My husband and I left the appointment feeling encouraged and reassured that our care rested in good hands, but most of all we felt very hopeful about our future. As all CF patients know, that's the absolute best feeling leaving any appointment.