A Day in a CF Patient's Life: Clinic Visit
My daughter, Margo, recently had her quarterly CF clinic appointment, so I am going to take you through our typical routine.
One thing I’ve found in talking with other CF families is that no two clinics operate exactly the same, so I’m always interested in learning what others do in their quarterly visits. This will also give readers who are unfamiliar with cystic fibrosis clinic visits a small glimpse into what Margo does every three months.
The journey to clinic
We live about 45 minutes from Children’s Hospital of Philadelphia (CHOP), and considering typical traffic and construction on the way and in the city, we try to leave at least 90 minutes before we need to be there. This time around, I’m especially glad we did as we encountered both of these things arriving.
My husband and I lived in Philly for 11 years and Margo was born there, so we always welcome the opportunity to get to come back to our favorite place.
In the waiting room
It’s sometimes hard to remember that even before COVID, CF clinics have always had policies in place to maintain safe distancing between patients. The waiting room can be hard for Margo. The receptionist specifies where we need to sit in the waiting room in order to keep a safe space from other patients with CF.
While our area typically has a TV showing cartoons, another part of the very large waiting zone has a submarine play structure that Margo always wants to play on. It creates frustration and often tears, so it’s typically something we try to manage expectations about beforehand.
Once we are in the exam room, Margo has her vitals checked, height and weight taken, and her throat swabbed for any bacteria.
We typically hold our breath when she is on the scale until we see the number, and then we are either delighted, satisfied, or instantly worried, depending on whether her weight is stable or if she’s gained or lost. Gaining and keeping on weight is challenging for CF patients, and Margo is no exception.
Typically, her throat swab is surprisingly uneventful. Margo doesn’t like having it done, but she does it like a champ and we are always so impressed by this. I think as long as she’s not having a shot, anything else is strictly uncomfortable and easy to deal with.
Pulmonary function test
Margo has started doing pulmonary function tests (PFT), which measure both the capacity of the lungs and how well the air flows in and out of the lungs. Eventually, these tests will be a big deal for Margo, as they measure the progression of CF.
I know many adult CF patients that dread these tests because if the numbers are too low, it could mean there is a lung infection or other underlying issue that must be addressed with medication, treatment, or hospitalization. However, Margo is still very much in practice mode.
The screen shows a birthday cake or a dandelion and has to use her forceful breath to blow the candles or blow the fluff off, and the machine then measures her breath.
Meet with the CF care team
After the PFT comes the actual clinic appointment where we meet with her care team. We either meet with the pulmonologist and a nurse or a nurse practitioner on the team. Additionally, we typically see the nutritionist, pharmacist, respiratory therapist, and social worker.
Pre-COVID, the whole team would usually gather in the exam room and we would have a discussion, but with current restrictions, there are only two team members in the room at a time, so it’s often a parade of people coming in. Anyone who enters the room always wears a gown, mask, and gloves, even before the pandemic.
We discuss Margo’s growth chart, treatment plan, medications, digestion, calorie intake, and whether we’ve noticed anything out of the ordinary.
No question too small
We usually have a list of questions to ask the team, which can range from big issues to small questions— this visit, we asked about whether it’s safe for Margo to play in a sandbox at a public park and about what their recommendation is for flying right now. Usually my husband and I are both in attendance, so one parent focuses on keeping Margo entertained while the other speaks with the care team.
From there, the doctor or nurse practitioner listens to Margo’s lungs, and checks her ears, nose, mouth, and stomach. Once that is completed, we are finally finished and say our goodbyes. We will be back in just three months.
However, my least favorite part starts after the appointment- we have to wait about one week to find out if Margo cultured any bacteria throat swab. If she did, we have a conversation with the clinic to determine next steps, and if necessary, start an inhaled antibiotic to combat it. However, I try my best to take it one step at a time and not dwell on what the results might be.
Unwinding after the visit
Clinic days are long and often tedious, particularly for Margo, so I love getting to make them as special as possible when we are able to. We always treat Margo to lunch in Philly afterwards and let her choose the restaurant. Several times it’s been Shake Shack for milkshakes and burgers, but this week she chose pizza. Fortunately there are a ton of outdoor dining options in the city so we had a great lunch safely outside.
If our schedule allows, we also try to do a fun activity of Margo’s choice— she chose the carousel and playground at Franklin Square, and the time before she chose seeing the Liberty Bell.
If all goes well, Margo crashes hard on the way home from Philly. It usually takes me a day to completely bounce back after clinic, both mentally and physically. But I'm also grateful to get to spend the day with Margo and celebrating all the hard work she puts in to keeping herself healthy.
Have you (or a loved one) been experiencing any negative side effects from Trikafta?