A day with a cystic fibrosis patient

In my first quarter of medical school, I learned about prenatal screening for cystic fibrosis (CF), a progressive multisystem disease caused by an autosomal recessive mutation in the CF transmembrane regulator gene. We also learned about bronchiectasis, a common pathology of CF that consists of the irreversible enlargement of airways, promoting continuous low-grade bacterial infection and causing deadly respiratory failure.

But despite my hours of studying and memorization, these clinical facts and scientific findings did not prepare me for the #CF1day experience to learn about the daily challenges of life as a CF patient. This is part of the #1day program from Smart Patients. CF1day is a program through which you are paired with a cystic fibrosis patient for an entire day via text message, email, and video, learning about their disease management, treatment, and the way their illness impacts their daily life. During my CF1day experience, I was fortunate to be connected to Emily, a cystic fibrosis patient and a strong advocate for other cystic fibrosis patients through her organization, Emily’s Entourage.

The text message from Emily read, “Morning Paula — just waking up, and the first thing I do is test my blood sugar.”

I had received my nebulizer (a drug delivery device to administer inhaled medications) in the mail to do treatments alongside Emily. But despite my previous training as an engineer, I was unsure even how to set up the device properly!

I followed Emily’s texts through a complicated regimen of albuterol, hypertonic saline, inhaled vancomycin, and a handful of prescription pills.

As her texts rapidly appeared, I tried out the nebulizer, wearing a tight shirt to simulate the difficulty breathing with CF because of thick, sticky mucus in the lungs.

Emily’s shortness of breath is aggravated by some of the medication that she must take to minimize problems from infection. She explained that:

I’m always on an antibiotic, and I rotate between doxycycline, which is a pill, and inhaled vancomycin to help prolong resistance from forming … the inhaled vancomycin … causes very severe bronchospasms which also causes pretty bad shortness of breath… I try to time important things in my life to coincide with my doxycycline weeks so that I feel better.

Emily also taught me about lung bleeds, which she described as “not painful so much as extremely uncomfortable and scary. You feel a gurgling in your throat, which is literally the blood spurting up and pooling, and you feel like you’re drowning.”

As the day proceeded, I was stunned by the extent that Emily’s schedule and daily activities were dictated by her CF.

Even her food choices were selected to optimize the activity of her medications:  “I don’t like almonds, but I need a certain amount of fat for one of my meds and it’s an easy, healthy source.”

On her way home, Emily mentioned the need to protect herself from germs in public spaces, proclaiming herself the “Queen of Purell,” adding another dimension of risk to something as basic as riding the trolley.

Spending the day with Emily taught me some things that were beyond the scope of my first-year clinical curriculum. As CF progresses, CF-associated diabetes can occur due to pancreatic scarring, so on top of her CF treatments, Emily is also managing diabetes.

Diabetes is the primary health concern of many Americans, such as my health coaching patients at a local Bay Area community clinic. When patients are even on the stage of prediabetes, we present patients with a variety of diet and lifestyle changes to prevent further progression, and once diagnosed, a diabetes panel management team works to counsel patients on blood sugar control, insulin injection, and foot and eye care. Yet diabetes seemed almost an afterthought to Emily compared with her cystic fibrosis: “I manage my diabetes as well as I can but other than that, it’s wayyyy lower on my radar/list of priorities. [Diabetes (most likely) won’t be the thing that kills me!]”

As a medical student, I have learned about the benefits of patient support groups for disease management and social support. Yet, CF patients often carry secondary bacterial infections, making it dangerous for them to be around each other. Emily explained: “It’s one of the saddest and hardest things about CF, but as a result, there’s this thriving “underground” online community.”

As a future physician, I was encouraged to hear about Emily’s partnership with her own physician who “realizes that life with chronic illness is a marathon, not a sprint, and he treats me holistically — as a person and not just a patient with lung disease. We make plans together that value my LIFE, not just my lungs.” Emily’s insights inspire me to show compassion and understanding for my own patients, and to enable them to live their fullest life despite their illnesses.

The CF1day experience provided immense depth of knowledge beyond what could ever be provided to me in my genetics, physiology, and pathology courses. Expansion of such programs within the medical school curriculum would increase awareness and empathy among physicians, enabling them to provide better care to patients like Emily.

Paula Trepman is a medical student.

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