Hope, Jurgen Moltmann, Transformative Therapies and Cystic Fibrosis: Unlikely Ties that Bind and Inspire
Mary Dell, Institute of Living/Hartford Healthcare, Hartford, CT, University of Connecticut, Farmington, CT; Kimberly Pasley, Stephanie Sliemers and Rylie Young, Nationwide Children's Hospital, Columbus, OH; and Anne May, Nationwide Children's Hospital, Columbus, OH, The Ohio State University, Columbus, OH
Hope in chronic, life-limiting illnesses has been of keen interest to patients, health and pastoral/spiritual care providers for centuries. Major world faith traditions, especially Abrahamic faiths, have formulated hope as both noun and verb, linked to desire of God’s present and future favor, with the simultaneous expectation of behaviors consistent with pleasing the God wherein hope resides. In the 1990s, psychologist C.R. Snyder continued this two part conceptualization, asserting that hope is a two part entity consisting of a positive emotional state or cognitive set coupled with actionable pathways to attain desired goals.
Few conditions manifest the need for hope and the challenges and blessings for maintaining hope as does cystic fibrosis (CF). The prognosis and quality of life (QOL) of people with CF, a life-limiting genetic disease, was improved dramatically by the development of CF transmembrane conductance regulator (CFTR) modulator therapies, especially elexacaftor/tezacaftor/ivacaftor (ETI), which addresses underlying genetic mutations in CF that lead to malfunctioning proteins. While ETI may improve physical health, ETI is not a cure for CF. Adult patients continue to experience lung, pancreatic, GI, and other disease as well as effects on QOL. However, early clinical trials were promising for increased life expectancy, decreased daily treatment burden, and improved QOL with faithful adherence to ETI and accompanying therapies. These circumstances presented an opportunity to identify and study changes in hope in a large population of adult patients with CF over several years of ETI administration.
As part of a larger 5-year study of ETI treatment at Nationwide Children’s Hospital (NCH), a prospective, IRB approved study was designed to examine changes in hope in adult patients with CF clinically eligible for ETI. Study visits occurred at time of drug initiation and repeated at 1, 3, 6, 9, 12, 24, 36, 48 and 60 months after enrollment. Participants completed the Adult Hope Scale (AHS, Synder et al., 1991), a 12-item measure of a respondent's level of hope. This scale is divided into two sub-scales that comprise Snyder's cognitive model of hope: (1) Agency/Willpower and (2) Pathways/Willpower). Study results are based on patients >18 years who completed baseline and at least one additional study visit.
A total of 168 participants were eligible for ETI and 18 years of age or older at time of study enrollment. For this analysis, 20 patients were excluded for eligibility reasons leaving 148 patients (mean = 33 years, 53% male). At baseline, population results on the AHS indicated mean of 50, indicating that patients were “moderately hopeful” and all subsequent time points also remained in the “moderately hopeful” range. Agency baseline mean score of 25 (range of 4-32) and Pathways mean score of 25 (range 4-32) indicated strength in both areas. Thirty-seven (25%) patients reported low levels of hope (below 40) at least once during a study visit. At baseline, 16 patients experienced low levels of hope, with a majority (n= 11) experiencing persistent hopelessness over the next 5 years. Five patients experienced improvements in their hope outlook since initiation of ETI therapy. Overall, majority of adults with CF reported feeling moderately hopeful as they initiated ETI therapy and their hope persisted over 5 years of treatment.
While these results do not apply to all individuals with CF, certain individuals struggle with hopelessness as they continue to live with a chronic illness that does not the option of a cure at this time. The tensions between balancing cognitive aspects of hope with the perils of hopelessness while being faithful to daily treatment requirements and behaviors share much with the elements of hope and persistence described by the 20th century theologian, Jurgen Moltmann. Moltmann eschewed equating hope with optimism, favoring instead the association of hope with endurance.
Without endurance, hope turns superficial and evaporates when it meets first resistances. In hope we start something new, but only endurance helps us persevere. Only tenacious endurance makes hope sustainable … In hope we affirm the pain that comes with endurance, and learn to tolerate it. (Moltman, Theology of Hope).
Finally, connections between hope of adults with CF on the new ETI therapy, Snyder’s two-part conception of hope, and Moltman’s theology of hope with endurance will be discussed.
References:
Cystic Fibrosis Foundation. https://www.cff.org/
Moltmann J. Theology of Hope. Minneapolis, Fortress Press, 1993. (German original, 1965)
Snyder CR, Harris C, Anderson JR, et al. (1991). The will and the ways: Development and validation of an individual-differences measure of hope. Journal of Personality and Social Psychology, 60, 570-585.
Few conditions manifest the need for hope and the challenges and blessings for maintaining hope as does cystic fibrosis (CF). The prognosis and quality of life (QOL) of people with CF, a life-limiting genetic disease, was improved dramatically by the development of CF transmembrane conductance regulator (CFTR) modulator therapies, especially elexacaftor/tezacaftor/ivacaftor (ETI), which addresses underlying genetic mutations in CF that lead to malfunctioning proteins. While ETI may improve physical health, ETI is not a cure for CF. Adult patients continue to experience lung, pancreatic, GI, and other disease as well as effects on QOL. However, early clinical trials were promising for increased life expectancy, decreased daily treatment burden, and improved QOL with faithful adherence to ETI and accompanying therapies. These circumstances presented an opportunity to identify and study changes in hope in a large population of adult patients with CF over several years of ETI administration.
As part of a larger 5-year study of ETI treatment at Nationwide Children’s Hospital (NCH), a prospective, IRB approved study was designed to examine changes in hope in adult patients with CF clinically eligible for ETI. Study visits occurred at time of drug initiation and repeated at 1, 3, 6, 9, 12, 24, 36, 48 and 60 months after enrollment. Participants completed the Adult Hope Scale (AHS, Synder et al., 1991), a 12-item measure of a respondent's level of hope. This scale is divided into two sub-scales that comprise Snyder's cognitive model of hope: (1) Agency/Willpower and (2) Pathways/Willpower). Study results are based on patients >18 years who completed baseline and at least one additional study visit.
A total of 168 participants were eligible for ETI and 18 years of age or older at time of study enrollment. For this analysis, 20 patients were excluded for eligibility reasons leaving 148 patients (mean = 33 years, 53% male). At baseline, population results on the AHS indicated mean of 50, indicating that patients were “moderately hopeful” and all subsequent time points also remained in the “moderately hopeful” range. Agency baseline mean score of 25 (range of 4-32) and Pathways mean score of 25 (range 4-32) indicated strength in both areas. Thirty-seven (25%) patients reported low levels of hope (below 40) at least once during a study visit. At baseline, 16 patients experienced low levels of hope, with a majority (n= 11) experiencing persistent hopelessness over the next 5 years. Five patients experienced improvements in their hope outlook since initiation of ETI therapy. Overall, majority of adults with CF reported feeling moderately hopeful as they initiated ETI therapy and their hope persisted over 5 years of treatment.
While these results do not apply to all individuals with CF, certain individuals struggle with hopelessness as they continue to live with a chronic illness that does not the option of a cure at this time. The tensions between balancing cognitive aspects of hope with the perils of hopelessness while being faithful to daily treatment requirements and behaviors share much with the elements of hope and persistence described by the 20th century theologian, Jurgen Moltmann. Moltmann eschewed equating hope with optimism, favoring instead the association of hope with endurance.
Without endurance, hope turns superficial and evaporates when it meets first resistances. In hope we start something new, but only endurance helps us persevere. Only tenacious endurance makes hope sustainable … In hope we affirm the pain that comes with endurance, and learn to tolerate it. (Moltman, Theology of Hope).
Finally, connections between hope of adults with CF on the new ETI therapy, Snyder’s two-part conception of hope, and Moltman’s theology of hope with endurance will be discussed.
References:
Cystic Fibrosis Foundation. https://www.cff.org/
Moltmann J. Theology of Hope. Minneapolis, Fortress Press, 1993. (German original, 1965)
Snyder CR, Harris C, Anderson JR, et al. (1991). The will and the ways: Development and validation of an individual-differences measure of hope. Journal of Personality and Social Psychology, 60, 570-585.