A Land Called Hope: Assessing Positive Views in People Living with Cystic Fibrosis

photo used with permission from the cystic fibrosis foundation

Although I didn’t yet know it, my journey with positive psychology started in 1982, when I was three years old. That year, I was diagnosed with cystic fibrosis (CF), a genetic, progressive disease that primarily affects the lungs and digestive system. At that time, there was little hope for people with CF. Many did not live to adulthood. Even today, there is no cure for the disease, and only 11% of the CF population is over the age of 40 (Cystic Fibrosis Foundation, n.d). In 1982, most parents of children diagnosed with CF were given the same message: Take your child home and love them hard because you probably won’t have them for long.

The doctor who diagnosed me had a very different message for my parents. She told them to raise me to be a responsible adult because she believed there were promising advances in CF research on the horizon. It was a bold prediction, but maybe the most important one of my life. Instead of fear, my family chose optimism, hope, and the idea that CF was a problem to be solved. My grandparents started fundraising for the CF Foundation to help ensure that those advances in research would happen, and my parents encouraged me to dream big for my life. Just as my doctor had hoped, my family and I celebrated my high school and college graduations, danced at my wedding, and cried tears of joy when I gave birth to a beautiful baby boy 17 years ago. There have been many health challenges along the way: hospitalizations, IV antibiotics, surgeries, and clinical trials, along with countless daily pills and treatments. But here I am at the age of 45, alive and well, still dreaming big. One of my doctors told me that it is possible to deal with CF by living in a state either of denial or of despair, but in the middle is a land called hope. This is where I choose to live.

Mental Health in People with Cystic Fibrosis

I applied to the Master of Applied Positive Psychology (MAPP) program at the University of Pennsylvania, with the goal of developing a better understanding of the science of hope and its impact on health outcomes. Existing research shows that people living with chronic illness, including cystic fibrosis, have a 41% increased risk of having a psychiatric disorder (Guta et al., 2021). In people with cystic fibrosis, depression rates are twice the global average (Guta et al., 2021). This is concerning because depression is itself a health risk factor for people with chronic illnesses. Symptoms of depression in individuals specifically with CF have been linked to decreased lung function, increased pulmonary infections, lower adherence to medications, and decreased quality-of-life scores (Lord et al., 2022; Quittner et al., 2008). The five-year mortality rate for CF patients with depression is double that of patients who do not have depression (Schechter et al., 2021).

Research also shows, however, that hope and optimism offer a defense against depression (Fischer et al., 2018; Rajandram et al., 2011). In fact, optimism has been associated with the following benefits in people with CF: better quality of life and less distress independent of disease severity, higher adherence to medications, and less health-related stigma (Abbott & Hart, 2005, Oliver et al., 2014).

Positive Views in People with CF

With my MAPP capstone, I set out to understand if positive expectations of the future and positive views of the world correlate with higher levels of well-being in people living with cystic fibrosis and better health outcomes, including survival.

Using a cross-sectional survey design, I surveyed 117 adults with CF from across the US (ages 20-77, mean age of 41.3) using validated psychological questionnaires including the LOT-R (optimism), HHI (hope), CFQ-R (cystic fibrosis quality of life), PHQ-9 (depression), and the Primal World Belief scale and compared them to a set of control groups made up of the general population.

The results of the study were surprising considering CF is a life-shortening disease with a heavy treatment burden. The LOT-R and HHI surveys revealed no statistical difference between levels of hope and optimism in people with CF compared to the general population, suggesting that people with CF have the same capacity for the positive views of hope and optimism as the general population.

The primal world beliefs research showed something even more exciting for the CF community. Primal world beliefs (or primals) describe an individual’s beliefs about the general nature of their environment, understood as the world as a whole, not a specific place. As defined by Clifton and Baker and colleagues (2019), who introduced the concept in 2018, primals are simple and descriptive; they describe a belief about how the world is, not why.

My study revealed that despite higher levels of depression, people with CF saw the world as a better place, a more enticing place, and a more improvable place at statistically significant levels than the control group of the general population. The results from both studies were independent of FEV1 (a standard measurement of lung function), indicating that disease severity does not influence positive views. This finding is consistent with other studies in primals research, which suggest that privilege in the form of socioeconomic status, health, and gender are not good indicators of positive primals (Kerry et al., 2023).

Implications of this research

Together, these results depict a population for whom positive psychology could be an important and welcome resource. Optimism, hope, and positive primals all correlate with well-being. In people with CF, these positive views seem to be independent of health measures (FEV1), which may suggest that they are independent of circumstance and would therefore remain available at times of change, whether positive or negative. Positive interventions designed to enhance any of the three could increase flourishing and protect against depression. In addition, people with CF approach their disease both realistically and hopefully, with a strong sense of agency that is reflected in their adherence to often-burdensome treatment regimens. Positive interventions that build on this sense of agency could be important in helping people with CF to feel an internal sense of control about their future, while also offering pathways for a broader, more robust experience of flourishing than can be measured by both physical and mental health outcomes. These types of interventions could be beneficial for those living with chronic illness and the general population as well.

Next steps

The results of this research made me fall in love with the CF community yet again. Despite living with a life-shortening chronic illness and higher levels of depression, this research suggests that people with CF see the world as a better place than the general population. Perhaps this is due to the incredible progress in research that has been achieved in recent decades. CF was once a disease where most did not live to adulthood. Now, the median age of survival for a person born today is 61 years old and counting. More people with CF than ever are able to attend college, pursue careers, have families, travel, and live at a fuller capacity.

Still, the levels of depression and anxiety are too high. Every few months, people with CF go to their local care center to be seen by a team of doctors—pulmonologist, dietician, respiratory therapist, and a mental health coordinator, to name a few. At each visit, we are assessed on our potential physical and mental health deficits. I am hoping that at some point we can also be assessed on our strengths.

What if people with CF left clinics knowing that they tend towards positive views even with their challenges? Could they harness their innate psychological strengths to combat depression and anxiety, which in turn could positively impact their physical health? In other words, what if all people with CF lived in this land called hope and had an expectation to flourish even while dealing with significant health challenges. This is my moonshot goal.

References

Clifton, J. D., Baker, J. D., Park, C. L., Yaden, D. B., Clifton, A. B., Terni, P., Miller, J. L., Zeng, G., Giorgi, S., & Schwartz, H. A. (2019). Primal world beliefs. Psychological Assessment, 31(1), 82.

Fischer, I. C., Cripe, L. D., & Rand, K. L. (2018). Predicting symptoms of anxiety and depression in patients living with advanced cancer: The differential roles of hope and optimism. Supportive Care in Cancer, 26(10), 3471–3477.

Guta, M. T., Tekalign, T., Awoke, N., Fite, R. O., Dendir, G., & Lenjebo, T. L. (2021). Global burden of anxiety and depression among cystic fibrosis patient: Systematic review and meta-analysis. International Journal of Chronic Diseases, 2021.

Kerry, N., White, KC, O'Brien, M. L., Perry, L. M., & Clifton, J. D. (2023). Despite popular intuition, positive world beliefs poorly reflect several objective indicators of privilege, including wealth, health, sex, and neighborhood safety. Journal of Personality. 1–14. https://doi.org/10.1111/jopy.12877

Lord, L., McKernon, D., Grzeskowiak, L., Kirsa, S., & Ilomaki, J. (2022). Depression and anxiety prevalence in people with cystic fibrosis and their caregivers: A systematic review and meta-analysis. Social Psychiatry and Psychiatric Epidemiology, 1–12.

Oliver, K. N., Free, M. L., Bok, C., McCoy, K. S., Lemanek, K. L., & Emery, C. F. (2014). Stigma and optimism in adolescents and young adults with cystic fibrosis. Journal of Cystic Fibrosis, 13(6), 737–744.

Quittner, A. L., Barker, D. H., Snell, C., Grimley, M. E., Marciel, K., & Cruz, I. (2008). Prevalence and impact of depression in cystic fibrosis. Current Opinion in Pulmonary Medicine, 14(6), 582–588.

Rajandram, R. K., Ho, S. M., Samman, N., Chan, N., McGrath, C., & Zwahlen, R. A. (2011). Interaction of hope and optimism with anxiety and depression in a specific group of cancer survivors: A preliminary study. BMC Research Notes, 4(1), 1–7.

Schechter, M. S., Ostrenga, J. S., Fink, A. K., Barker, D. H., Sawicki, G. S., & Quittner, A. L. (2021). Decreased survival in cystic fibrosis patients with a positive screen for depression. Journal of Cystic Fibrosis, 20(1), 120–126.

About the author | KC White (C’22) was elected Chair of the Cystic Fibrosis Foundation’s Board of Trustees in 2022 after serving on the board since 2005. This marks the first time a person living with cystic fibrosis has held this role. Diagnosed with cystic fibrosis at the age of 3, KC has been a committed volunteer and inspiring advocate for the Cystic Fibrosis Foundation since childhood, speaking at her first event at the age of nine.

A founding member of the Foundation’s Adult Advisory Council, she served as its chair from its inception in 2014 to 2022. KC was also the co-chair of the first BreatheCon, an annual virtual conference for adults with CF. In 2020, KC envisioned and created ROSE UP, a national virtual fundraising event led by a committee of adults with CF.

KC received her Master of Applied Positive Psychology from the University of Pennsylvania in 2022. She currently serves as an assistant instructor in the program. KC is the Head Varsity Women’s Lacrosse coach for the Chagrin Falls, Ohio, Tigers. She and her husband, Justin, have one son, Mac.