Cystic Fibrosis for One Day: It Gets Real
I shadowed a mom and her 5-year-old son with CF from afar for 24 hours.
It taught me more than I could have imagined about living with a life-shortening disease — and about myself.
Erin and Drew made it to Cincinnati Children’s Hospital on time for his annual check-up, a milestone appointment since it would be the first time he would do a pulmonary function test (PFT).
For the PFT, Drew and a nurse sat inside a glass box the size of a telephone booth and he blew as hard as he could into an instrument called a spirometer. (And yes, I am linking to Wikipedia articles on both items since there is a good chance some readers have never seen either one in the wild.)
I’ve been online a long time, but the fact that Erin could text me photos and videos from within their exam room is still pretty mind-blowing. We could not have done this exercise without smartphones. Thanks, Steve Jobs.
People living with cystic fibrosis have to be extremely careful about germs. A “common cold” can cause a killer infection in their lungs.
Drew has an all-access pass to the iPad today — anything to keep him amused while they sit for hours in a small exam room.
Meantime, in DC, I am running late, just arriving at Georgetown University Hospital.
Parking did indeed cost me $25 for the morning. It’s a victory for Erin that Drew finally drinks his “milkshake” (in reality, it is high-calorie Ensure).
Erin meticulously tracks Drew’s meds and symptoms, allowing her to have a high-level discussion with his clinicians.
If you are curious about Erin’s monitoring techniques, you can hear her describe it in this interview: Self Tracking: Quantified Self Movement (skip ahead to minute 24).
In the video below I could see (and hear) the ever-present iPad and the super-annoying music that goes along with it. But Erin will do pretty much anything to keep Drew happy!
Cincinnati Chidren’s is a top-notch hospital. Is it a bug or a feature that they follow procedures to the letter, even when they don’t make sense?
Notice the mask on every clinician who enters the room — a key infection control measure.
So far, so good. This is supposed to a routine appointment. But that’s about to change.
Erin uses a term that might be unfamiliar to some readers: the “fellow check.” A fellow is a doctor who has completed medical school and residency (their initial training in a specialty, such as internal medicine) and has decided to go even further into one aspect of medicine, such as endocrinology or pulmonology.
Drew is smiling, but Erin is absorbing bad news about his physical health. He is not doing as well as she had hoped.
Unfortunately the fellow delivered bad news.
A quick tutorial on a technical term: BMI stands for body mass index, the “measure of relative size based on the mass and height of an individual” (Wikipedia). BMI is directly correlated with improved outcomes for people living with CF: the higher the better. And the persistent cough that Erin has been monitoring at home is an indication of a worrisome infection in Drew’s lungs.
This routine appointment has changed very suddenly into a serious discussion.
I can tell Erin is upset and I feel totally helpless, so far away and unable to do anything but text her back.
Notice the clock on the wall: Drew and Erin have been up since 5:40am and in that room since 8am.
I’m hitting Dr. Google pretty hard at this point, trying to understand what Erin is facing. “IVs” = intravenous, meaning into a vein. “PICC” = peripherally inserted central catheter. A long, thin tube is inserted into a vein in the arm and gently pushed through until it reaches the heart. Yes, it’s a big deal and no, it’s not pleasant.
Erin has three other kids and an active work and social life. Drew’s care takes priority, but it has a significant ripple effect on the rest of the family.
Again, Cincinnati Children’s Hospital provides a top-notch team, including a social worker to help people manage the complexity of CF care. The hitch is that Erin appears to be so competent and “together” that everyone assumes she doesn’t need help. Yet she does.
People with CF have a wide range of health issues so they have a host of specialists assigned to their care. Endocrinology is a sub-specialty of internal medicine dealing with hormones and associated issues such as metabolism, respiration, and growth — in other words, everything that Erin is worried about.
While Erin and the endo look at one screen, Drew is busy with his screen, the iPad. Again: Thanks, Steve Jobs!
I’m trying to make Erin smile by sending this selfie of me and my “kid” (a teddy bear), stuck in a tiny “room” (a library carrel). Obviously it’s not at all the same, and I feel like this empathy exercise has become a farce. There is no way I can recreate the stress she is feeling.
Intravenous antibiotics are a necessary treatment when an infection just won’t quit, but maintaining the cleanliness of the PICC line is a round-the-clock job. Erin will have to juggle even more balls to keep Drew well and her life in order.
Drew is an experienced patient. He heard the word “PICC” and immediately knew it meant no gymnastics or swimming for the next two weeks. A reminder: he is just five years old.
I hate to end on a cliff-hanger, but that’s our installment for today, folks. If you have questions for me or for Erin, please leave us a note (click on that little plus sign on the right margin) or write a response if you have more to say (click on that button below).
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The outpouring of love and support for Erin and Drew has been really beautiful. We want as many people as possible to learn about life with cystic fibrosis. Thanks for helping to spread the word!
And if you’re ready to read the end of the story, Part 3 is up: A Brave Kid.
For more examples of how people shared their Cystic Fibrosis For One Day experiences, see: #CF1day on Symplur.