Madeline is supposed to visit her care team at the CF clinic every three months. This is standard care for people with cystic fibrosis and is a critical part of making sure that her health stays on track. One of these four visits each year is known as an "annual visit" and includes additional testing: an oral glucose tolerance test, basic labs to check her vitamin levels, and a chest x-ray. Last summer we took some pictures throughout the day so we could capture what a typical annual visit is like for Madeline these days.
When a clinic day comes along, my kind father-in-law usually comes up to watch the little ones so I can just focus on Madeline for the day. I'll let a baby tag along for the first year or so and it's no big deal, but there comes a day when it's too distracting. Last month Evan proved that he is now far too disruptive, so I'm officially benching him for future visits. Anyway, we drive to the Children's Hospital of Philadelphia (CHOP), which is 1-1.5 hours away depending on traffic, for Madeline's clinic visits. We are gone all day long, and it can feel like a really long day! It's nice to spend some time together, though, and we always have a good time hanging out.
An annual visit begins with an oral glucose tolerance test (OGTT), which is done to see if Madeline has developed CF-related diabetes (CFRD). About 15-20 percent of teens and 35 percent of adults with CF develop CFRD, so it's important to stay on top of this (you can read more about CFRD here). This screening was added to our routine when Madeline turned eight, so this summer will be her third glucose test. The OGTT starts bright and early because she has to fast beforehand. We arrive at the lab at 8:00 and they draw her blood before sending us upstairs for the glucose drink. She gets her blood drawn again at the one-hour mark and then at the two-hour mark. They also go ahead and draw her annual labs that check vitamin levels and other things to make sure she is healthy.
We have to stick around the general area during her glucose test, so we chat, read books, and maybe play a game. She also gets a chest x-ray done during this time. The x-ray is done once every year to check the health of her lungs and see if lung damage has progressed over the year. The x-ray is easy to do, but I always dislike this part because while her x-ray looks good, there are always little signs of CF doing its thing. This part of the visit is so much nicer now that Madeline is older and doesn't require any wrangling.
Last summer we had a cute little baby to keep us company. He was so small! You might notice that Madeline is wearing a mask in some of these pictures. She has to wear a mask if she's in any open areas of the hospital, including all halls and waiting rooms. This is really important for infection control and to reduce the risk of cross-infection of people with CF. The rooms are fully cleaned after each patient, and all of the doctors and nurses wear disposable gowns and gloves when they come in. It's serious business. The patients who have the most dangerous bacteria have special clinic days so that other patients have even less risk of being exposed to them. When Madeline was a baby, they always had her as the first patient of the day so the clinic would be as clean as possible.
By the end of all this waiting and drawing of blood, it's late morning and Madeline is absolutely ravenous! As soon as the glucose test is over, she can eat a quick bite and then it's on to the main portion of the visit. From this point on, everything that happens is done at each clinic visit, not just the annual one. First a nurse gets her height and weight.
Then the nurse checks her blood pressure and oxygen saturation levels. She also gets a throat swab done every time. This swab is cultured to check for any bacteria growing in her lungs. It takes about a week to get the results back, and the clinic uses the results to know if they need to treat any particular bacteria with antibiotics to prevent lung damage.
After all the vitals are done, Madeline does a Pulmonary Function Test (PFT) to test her lung function. It's a little complicated to explain, but basically she blows into a tube and it measures her lung volume.
After that, we go back to the room and meet with Madeline's CF team. It includes her pulmonologist (main doctor), a nurse, a dietitian, a social worker, a physical therapist, and sometimes a pharmacist or resident. We manage to underplay CF in our day-to-day life, but when you look around and realize that six highly educated and well-paid adults are gathered in a room to discuss every aspect of your daughter's health, well, it's clear that CF is a pretty big deal.
The nurse goes over Madeline's medications and makes sure that their list is current. One of my primary goals as a mom is to make sure that Madeline is prepared to take charge of her own CF care, so for the past few years she has gone with a checklist so she can do this part with the nurse herself. Then the nurse and/or doctor ask all kinds of questions about her health, including recent illnesses or various issues regarding her gut and lung health. We discuss her growth and appetite and any eating concerns, with the dietitian taking the lead as appropriate. Often this becomes a brainstorming session to see how we can maximize her calorie intake. We talk about any necessary adjustments to her enzyme dosage, whether she has had belly pain or headaches, wheezing, shortness of breath, etc. We discuss her activity level and what kinds of physical activities she is currently involved in.
The doctor listens to Madeline's lungs and gives her a complete check-up. We discuss her x-rays, PFT results, glucose results, and any adjustments to medication. The social worker will often check with us to see if we need any help with school or insurance issues or anything else. The physical therapist spends some time doing various exercises with Madeline and teaching her proper techniques for coughing or doing her breathing treatments. After that, we make sure we're all on the same page for a treatment plan, and then off we go. We pretty much always eat somewhere before heading back home. We used to always go to the cafeteria, but lately Madeline has wanted to check out some other places in the area, which is fun.
We usually get home late afternoon, where my angel father-in-law often has dinner waiting for us, and then we wait another three months before doing it all again! I always have visions of doing some fun things in Philadelphia before going home, but the truth is that we're both usually pretty wiped out by the end of the day! So grateful that we live near one of the best pediatric CF clinics in the entire country. I love Madeline's doctor and have full confidence in the care she receives there.
