RESILIENCE IN ADOLESCENTS AND YOUNG ADULTS WITH CYSTIC FIBROSIS: A PILOT FEASIBILITY STUDY OF THE PROMOTING RESILIENCE IN STRESS MANAGEMENT (PRISM) INTERVENTION

Abstract

Background:

Disease burden in cystic fibrosis (CF) impacts quality of life, distress and treatment adherence. The Promoting Resilience in Stress Management (PRISM), is a brief patient-focused intervention to promote resilience in adolescents and young adults (AYAs), which may mitigate the negative outcomes, and is proven to be feasible and acceptable in other diseases.

Objective:

Our aim was to test the feasibility and acceptability of PRISM among AYAs with CF in addition to collecting pilot data regarding patient-reported resilience, distress and quality of life.

Methods:

Eligible English speaking, 12-21 year patients admitted to the hospital were enrolled. We defined feasibility as 80% completion of all sessions. Acceptability was defined qualitatively based on feedback about timing, content and delivery of intervention. As an exploratory aim, questionnaires measuring resilience (Connor-Davidson Resilience Scale/CD-RISC), distress (Kessler-6 scale/K6) and disease specific health related quality of life (Cystic Fibrosis Questionnaire-Revised/CFQ-R) were given at baseline and post-intervention.

Results:

10/17 (59%) patients consented to participate. 8 were Caucasian, 8 female with age range 13 - 20 years (median: 18). Nine completed all PRISM sessions with universally positive feedback. Health perception and respiratory domain scores of the CFQ-R improved (47.2 to 65.1, 95% CI 2.6, 35.6; 50.9 to 61.9; 95%CI 1.7-19.9, respectively), however in the setting of inpatient exacerbation treatment it would be hard to attribute these changes to PRISM.

Conclusion:

PRISM was feasible and highly acceptable among AYAs with CF. Future research is needed to test the efficacy of PRISM among a larger group of CF patients in a multicenter trial.

INTRODUCTION

Cystic fibrosis (CF) is one of the most common life-shortening autosomal recessive chronic illnesses, leading to frequent respiratory infections and requiring daily maintenance therapies, making it one of the most difficult chronic conditions to manage (1, 2). Although the expected life span has been increasing with better management and new medications, the daily required treatment regimen is complex and time-consuming which has major impacts on psychosocial health-related quality of life (HRQoL), distress and adherence ^1-3.

In addition to this intense daily self-care regimen, adolescents with CF face the additional burden of a developmental period that includes significant social, biological, and cognitive changes. When compared with healthy peers, the experience of serious illness among adolescents and young adults (AYAs) is exceptional because of the distinctive developmental challenges, transitions, and choices related to education, employment, identity, relationships and family³. Research has shown that adults and children with chronic conditions are at higher risk for depression and anxiety compared to healthy peers⁴, and individuals with CF have higher reported rates of both depression and anxiety with a prevalence of 8-29% among children and adolescents, and 13–33% among adults with CF^{5, 6}. Psychological symptoms in both CF patients and parents have been associated with decreased lung function, lower body mass index, worse adherence, health-related quality of life, more frequent hospitalizations and increased healthcare costs ^{5; 7-10}.

There is growing international concern for the impact of stress on CF outcomes, such that the Cystic Fibrosis Foundation and European Cystic Fibrosis Society International Committee on Mental Health in CF (ICMH) ^8-12 recommend that ongoing education and preventative, supportive interventions, such as training in stress management and the development of coping skills, aligned with appropriate developmental stage and disease events be offered for all individuals with CF.

Resilience describes an individual’s capacity to maintain psychological and physical well-being in the face of stress, and provides a novel platform for interventions designed to improve health outcomes ^{12, 13}. The Promoting Resilience in Stress Management (PRISM) intervention is a brief, disease non-specific, skills-based intervention developed for AYAs with chronic or serious illness. PRISM has demonstrated feasibility among populations of AYAs with cancer and diabetes^14-16, and a phase 2 Randomized Controlled Trial (RCT) comparing PRISM to usual care among AYAs with cancer suggested PRISM is also associated with higher patient-reported resilience, quality of life, hope, and benefit-finding, and with lower risk of psychological distress 6 months following enrollment ^{17, 18}.

Given the high risk for elevated stress resulting from CF-specific daily management in conjunction with developmental stressors of adolescence, the main objective of this pilot study was to test the feasibility and acceptability of PRISM among AYAs with CF. We conducted brief, 1:1 qualitative interviews with participants to assess participant-perceived helpfulness, content appropriateness, timing, and opportunities for improvement and/or refinement in both inpatient and outpatient settings. As an exploratory aim to guide future studies, we assessed changes in patient-reported resilience, distress and disease-specific health-related quality of life.

MATERIAL AND METHOD

The “Promoting Resilience in Stress Management” (PRISM) Intervention

PRISM is a manualized, skills-based, brief intervention targeting stress management, goal-setting, cognitive reframing, and meaning making (Table 1). PRISM skills were chosen based on iterative qualitative work with AYAs with chronic disease, prior behavioral/skills-based interventions and stress and coping theories ¹⁴.

Table 1.

PRISM Intervention Session Details

	Topic	Strategies
Part 1: Managing Stress	Session 1: Stress management	Breathing techniques, relaxation strategies, obtaining social support
	Session 2: Goal setting	Setting specific, realistic, desirable goals, planning for roadblocks, strategies for dealing with roadblocks, identifying how parent/caregiver can help meet goal
Part 2: Building Resilience	Session 3: Cognitive restructuring	Recognizing negative self-talk, identifying unrealistic/negative thoughts, replacing these thoughts with positive/manageable ones
	Session 4: Benefit finding	Reframing current experience into a meaningful one, self-reflection/mindfulness, identifying and tracking gratitude, legacy, and purpose.
Coming Together (optional)	Reflection	Discussion of strategies practiced
	Celebration	Identification and recognition of successes
	Resources	Identification of further needs, referrals to additional resources
	Sharing	Shared conversation with parents: what works, how family can help

The content of PRISM is grounded in cognitive behavioral therapy methods and leverages brief, skills-based intervention techniques. It targets four resources known to promote coping and positive wellbeing, termed “resilience resources” within the intervention (Table 1). These resources include (1) stress-management, including relaxation and mindfulness techniques designed to recognize stressors, physical sensations, and emotions; (2) goal-setting skills, including strategies to set “SMART” (Specific, Measurable, Actionable, Realistic, and Time-dependent) goals and monitor forward progress; (3) cognitive re-framing, including techniques to recognize negative self-talk and re-appraise experiences realistically or optimistically; and, (4) benefit-finding, including skills such as identifying gratitude, meaning, and purpose in the setting of challenges, including CF. Participants receive worksheets to practice skills in between sessions, and intermittent brief “booster” visits from study staff to practice individual skills. Whenever possible, skills are taught in person by a trained interventionists who meets privately with the patient. The same interventionist delivers all sessions to a given patient. At patient’s requests and for convenience, sessions could be delivered by videoconference or phone.

PRISM is administered by trained, non-clinical professional staff with a Bachelor’s degree and clinical research experience ¹⁴. Like the intervention itself, PRISM delivery training is standardized. Specifically, all interventionists receive at least 8 hours of supervised training, including didactics, workshops, and role-play scenarios. In this study, all sessions were delivered to all patients by the same interventionist (LN). For fidelity purposes, each session was audio-recorded and reviewed by the study team for adherence to the protocol, inclusion of required elements, and presence/absence of additional information ¹⁴.

Each skill is taught directly to the AYA by a trained interventionist over a 30-45 minute period. Sessions were offered 1-10 days apart based on patient and family preferences, clinical status, and the anticipated length of hospitalization. All first sessions were conducted in person in the hospital; sessions not completed during the inpatient stay were delivered by phone or in person in the Pediatric Clinical Research Center. These were scheduled in advance by the interventionist and at the patient’s convenience. Following each session, participants received “cheat-sheets” (paper handouts) describing the session and providing opportunities to practice the skills. An optional “coming together” segment was offered for participants to share what they learned with parents or loved ones. All study participants were compensated for their time and contribution for each session using a reloadable debit card in accordance with CF research compensation standard practice.

Participants

AYAs were eligible for this pilot study if they had an established diagnosis and treatment of CF for at least 6 months, were 12-21 years-old, fluent in English, cognitively and physically able to participate in interactive interviews, and admitted to the hospital for any reason at enrollment. The CF research team screened all consecutive eligible patients daily from the inpatient hospital census and approached between December 2016 – May 2017 until we reached our target enrollment of n=10 AYAs. Eligibility criteria were verified with the patients’ primary CF medical providers.

A CF research coordinator approached and consented all patients. All patients 18 years of age or older provided signed informed consent while patients 12-17 years provided signed assent along with additional consent from their designated legal guardian prior to any study specific activities. The study protocol was approved by the Seattle Children’s Hospital Institutional Review Board.

Outcomes

Primary Outcomes

We defined feasibility a priori as 80% completion of the 4 main sessions among enrolled participants. We defined acceptability based on qualitative patient feedback following each session. Specifically, feedback was queried at each session for thoughts, suggestions, and satisfaction with semi-structured questions and also in a separate interview at the end of the study (Table 2). The same interventionist conducted both feedback and interview sessions. All interviews were audio-recorded and transcribed verbatim for thematic analysis.

Table 2.

Questions asked during the individual and group interview at the end of the study

“What did you think of the intervention as a whole?”

“For the individual sessions, what did you think of a. Timing b. Content  c. Location  d. Handouts?”

“Do you have other suggestions for what we should include? What did you like or not like?”

“What would work better? What should we change? What are we missing?”

“Would you have done this without incentives?”

Exploratory Outcomes

Participants completed surveys at enrollment and following the completion of PRISM to assess the following:

• Resilience. Resilience is quantifiable, defined as the process of harnessing resources to sustain physical and emotional well-being in the face of significant stress, and influenced by health status ^{19; 20}. Patient-reported resilience was measured with the Connor-Davidson Resilience Scale (CD-RISC), a reliable and widely used 10-item instrument to measure self-perceived resilience ^{21; 22}. Questions revolve around personal problem-solving and approaches to adversity. CD-RISC scores range from 4-40 and higher scores suggest higher self-perceived resilience. The mean score among well US adults is 31.8. CD-RISC is also validated and used in populations of AYAs with chronic disease ^{23; 24}. In a previous research including AYA cancer patients receiving usual care¹⁷ CD-RISC was found to be 28 (SD 5.8), while it was 30.1± 4.2 in an AYA patient population with type 1 diabetes ¹⁴. Application of this scale could be in clinical practice with contemporary resiliency interventions and assessing response to the intervention ¹⁹.

• Psychological Distress. Distress was assessed with the Kessler-6 scale, a 6-item scale which measures “level of psychological distress experienced in the past month”¹⁹. The instrument strongly discriminates between community cases and non-cases of Diagnostic and Statistical Manual of Mental Disorder (DSM)-IV psychiatric disorders such as serious emotional distress or serious mental illness, generating a range of zero to 24 points. Higher scores suggest higher distress²⁵. Kessler 6 scores between 13 and 24 represent high psychological distress (with respondents very likely to have a corresponding psychiatric diagnosis), 8 to 12 moderate and 0 to 7 low psychological distress.

• Health Related Quality of Life. The CF specific health related quality of life questionnaire ²⁶(CFQ-R) is designed to measure impact of overall health, daily life, and perceived well-being and symptoms specifically for patients with cystic fibrosis. The CFQ-R measures daily functioning from the patients' perspective, and thus provides unique information to facilitate clinical interventions ^{27; 28}. It includes nine quality of life domains: Physical Functioning, vitality (energy/well being), emotional state, social perception/limitations, role limitations/school performance, body Image, eating disturbances, treatment burden and health perception. Additionally, it includes three symptom scales: Weight, Respiratory and Digestion. Domain and symptom scale scores range from 0 to 100, with higher scores indicating better health related quality of life or lower symptom burden ^{26; 27}. We asked all patients enrolled in the study to answer to a self-report format of the CFQ-R.

Questionnaires were completed by paper-and-pencil and entered into a secure REDCap database or completed directly in REDCap. Clinical data were also collected and managed using REDCap electronic data capture tools hosted at the University of Washington Institute of Translational Health Sciences. REDCap (Research Electronic Data Capture) is a secure, web-based application designed to support data capture for research studies.

Statistical Analysis

Basic demographic variables (gender and ethnicity) were collected from all participants and clinical data regarding the hospital admission was collected from medical records at the time of enrollment. Demographic and clinical variables were summarized descriptively. Scores for the self-reported instruments (CD-RISC, Kessler-6, CFQ-R) before and after the PRISM interventions were summarized using means and standard deviations. Change scores were calculated by subtracting baseline scores from follow-up scores. To describe change from baseline to follow-up, we estimated 95% confidence intervals for mean change. All statistical analyses were performed using Stata version 13 (StataCorp, College Station, TX).

The qualitative feedback obtained from the interviews was transcribed verbatim²⁹. Three authors (DT, ARR, and JYF) conducting directed content analyses of the data to identify exemplary quotes reflecting patient satisfaction (or lack thereof) and opportunities for improvement. Findings were confirmed by the full authorship team to for triangulation and face validity purposes.

RESULTS

Participants

Of 17 AYA CF patients approached over 5-months, 10 (59%) consented to participate. All approached patients were admitted for treatment of a CF pulmonary exacerbation with intravenous antibiotics. None of the admissions was planned ahead of time and a decision for a hospital admission was mostly made after an outpatient clinic visit or emergency room evaluation. Seven patients declined enrollment, citing ”not being interested”. Median time from admission to approach for patients who enrolled was 3 (range 1-7) days and was 4 days (range 1-10) for those who declined. The median time from admission to first session of PRISM was 6.5 days (range 3-12). Median duration of inpatient stay for enrolled patients was 12.5 days (range 7-24 days). Demographics of study participants are summarized in Table 3. Demographic and clinical characteristics of patients who declined to participate or accepted are summarized in Table 4.

Table 3.

Demographics of AYA CF patients participating in the PRISM intervention (n=10).

Age in years, median (range)	18 (13-20)
AYA age group, n (%)
12-13 years	2 (20)
14 Years & older	8 (80)
Female, n (%)	8 (80)
Race, n (%)
White	8 (80)
Unknown or declined to answer	2 (20)
Ethnicity
Not Hispanic or Latino, n (%)	10 (100)

AYA, Adolescent and young adult; CF, cystic fibrosis; PRISM, Promoting resilience in stress management.

Table 4.

Comparison of clinical characteristics of patients that have declined (n=7) and accepted (n=10) to be enrolled in the study.

	Declined patients (n=7)	Enrolled patients (n=10)
Mean age at approach (years)	17.4 (range 13-20)	16.8 (13-20)
Gender (F/M)
Mean time since diagnosis of cystic fibrosis (years)	15.6 (range 12-18)	14.4 (range 10-19)
Mean number of days spent as inpatient in the hospital in the prior 6 months	34.0 (range 0-60)	13.7 (range 0-36)
Days between admission to the hospital and approach for the study	4.4 (range 1-10)	3.3 (range 1-7)
Length of admission (days)	22.4 (range 6-57)	13.2 (range 6-24)
Average body mass index percentile (%) at admission	48.1%	41.4%
Average FEV1% predicted at admission (% pred)	57% (range 24-106%)	74% (range 38-107%)

FEV1, Forced expiratory flow volume in the first second.

Feasibility

Nine of 10 participants (90%) completed all 4 intervention sessions and follow-up questionnaires. One participant only completed two sessions due to employment scheduling conflicts. All participants completed the first session in the inpatient setting, and 4 of 10 participants completed all sessions in the inpatient setting. For those who did not complete all sessions while inpatient, sessions were completed either in the outpatient clinic (n= 1), Pediatric Clinical Research Center (n= 6), or by phone (n= 4). Nobody used the offered Skype option for the interventions. The median time from the first session to completion of all sessions was 15 days (range 4-29 days, IQR: 6-19). The median number of days between sessions was 4 days (range 1-7 days, IQR:1-7). Only 2 parents participated in the optional “coming together” segment. Reasons for opting out of the “coming together” included patient preference (n=3) and unavailability of the parent (n=5).

Fidelity scores for the interventionist was 100% for delivery of the appropriate content. Fidelity did not differ for phone versus in person sessions.

No unexpected psychosocial or emotional issues requiring additional support from a social worker or psychiatrist were reported during the interventions.

Acceptability

Feedback from CF AYA participants was positive (Table 5). Of the participants who completed all 4 sessions, all indicated that they would recommend PRISM to other patients. No changes were suggested to the content, format and delivery of the interventions. All participants approved of PRISM’s overall structure. Five out of the 9 participants who completed the intervention considered the “stress management – square breathing” session to be the most practical for daily life. Participant suggestions included adding additional sessions with a physical component similar to the square breathing exercise and incorporating “hands-on” activities into the existing sessions (eg. stretching, posters to describe the square breathing, using a whiteboard during the session to organize thoughts). One participant suggested that it could be even more attractive to the younger CF patients if there was a “final prize” at the end of the “goal setting” session, however a “final prize” was not defined.

Table 5.

Quotes from participants regarding different sesions of the PRISM

Session 1: Stress management	“I thought [the sessions] were all really helpful especially the square breathing and the visualization. I was going into an anxiety inducing situation and it made me forget, not forget entirely. I had been crying and I tried that and it helped a bunch.” “I feel like the square breathing worked the best. As soon as I got on the pattern it was very relaxing and I could just feel everything relaxed. And my mind went blank.”
Session 2: Goal setting	“It helped me to plan out my steps to achieve my goal a lot because I saw what I needed to do. I wasn’t just kind of winging it, so that helped me a lot.”
Session 3: Cognitive restructuring	“It was easier for me to have more positive thoughts when I broke everything down and realized what triggered the feeling.”
Session 4: Benefit finding	“I used to be in the hospital and think it was a waste of time and not want to be there. Doing things like this make you realize you are here to make yourself feel better. Think about the positive side of being inpatient.”

Two AYA participants mentioned that since they have been dealing with CF complications and required treatments and hospitalizations for several years, they had created their “own version” of stress management and coping skills. Although they thought PRISM was additionally valuable to hone these skills, they also wondered if it would be even more impactful if delivered earlier in their illness experience during their younger teen years.

As for the setting, all patients reported appreciating the convenience of PRISM’s delivery in the inpatient setting and stated that sessions were more engaging when given in-person rather than over the phone. All agreed that approximately a week between sessions was ideal to learn and process the new skills before transitioning to the next session. Handouts that had been extended to the participants during the sessions were considered to be helpful to remind them of the structure of the interventions.

Exploratory Analyses

Resilience and distress total scores and individual item scores did not change after the PRISM intervention (Table 6). CFQ-R scores seemed to improve in the health perception (+19.1; 95%CI 2.6, 35.6) and respiratory domains (+10.8; 95%CI 1.7, 19.9) (Table 6, Figure 1). No instrument or domain scores worsened with PRISM.

Table 6.

CD-RIDC, Kessler-6 and CFQ-R scores before and after the PRISM interventions.

	Before intervention		After intervention		Score change
	n	Mean (sd)	n	Mean (sd)	n	Mean (sd)	95% CI
CD-RISC total score	10	25.7 (4.3)	9	24.7 (5.7)	9	−0.8 (4.8)	−4.5, 2.9
Kessler-6 total score	10	5.3 (3.0)	9	5.6 (4.6)	9	0.6 (3.1)	−1.8, 3.0
CFQ-R Quality of Life domain scores 26
Physical functioning	10	55.0 (27.1)	9	60.8 (20.3)	9	7.1 (20.1)	−8.4, 22.5
Role limitations/School Performance	8	54.2 (14.8)	7	51.2 (25.7)	7	−0.0 (28.9)	−26.7, 26.7
Vitality (energy, well-being)	8	38.5 (12.5)	7	52.4 (19.1)	7	13.1 (24.9)	−9.9, 36.1
Emotional state	10	70.2 (7.3)	9	70.7 (13.2)	9	1.7 (7.6)	−4.2, 7.5
Social perception	10	60.2 (15.5)	9	60.6 (15.1)	9	2.4 (9.0)	−4.6, 9.3
Body image	10	68.9 (18.0)	9	77.8 (13.6)	9	9.9 (14.1)	−1.0, 20.7
Eating disturbances	10	90.0 (14.3)	9	80.3 (21.4)	9	−9.9 (19.6)	−24.9, 5.2
Treatment burden	10	51.1 (12.0)	9	51.8 (13.6)	9	1.2 (6.7)	−3.9, 6.4
Health perception	8	47.2 (17.6)	7	65.1 (10.0)	7	19.1 (17.9)	2.6, 35.6
CFQ-R Symptom Scale scores
Respiratory symptoms	10	50.9 (22.3)	9	61.9 (25.0)	9	10.8 (11.8)	1.7, 19.9
Digestive symptoms	10	73.4 (16.7)	9	71.6 (20.1)	9	1.2 (23.2)	−16.6, 19.1
Weight	8	41.6 (38.8)	7	38.1 (35.6)	7	−9.5 (25.2)	−32.8, 13.8

CD-RISC: Connor–Davidson Resilience Scale; CFQ-R: (CF) health related quality of life questionnaire; CI: Confidence interval; PRISM: Promoting Resilience in Stress Management.

Figure 1.

Mean score change with 95% confidence intervals for CFQ-R health-related quality of life domains and symptom scales.

DISCUSSION

The PRISM intervention, a resilience-promoting intervention for AYAs, has been successfully utilized in AYA populations with cancer and type 1 diabetes (T1D)^{14; 17}. Our current study demonstrated that PRISM is also feasible and acceptable among AYAs with CF. The intervention was highly valued by participants and parents and was also endorsed for future patients. All of the participants considered stress management, goal-setting, cognitive restructuring and benefit-finding essential sessions for the structure and retention of an intervention designed to reduce stress and improve resilience.

The completion rate of PRISM in the enrolled AYA CF population was high with 90% of the pilot sample completing all four interventions either as inpatient or in the outpatient setting in the proposed time frame. The completion rate in this study was comparable to previous resilience studies in the chronic patient populations, suggesting that once they actually start the intervention they are very likely to finish it ^{14; 30}. Even though participants agreed that the intervention was more productive and engaging in person, the phone option provided flexibility and convenience and increased the completion rate for all sessions. Further, the use of a manualized intervention suggested a cost-effective approach, such that a research associate with limited professional experience was able to be trained to administer it.

Our study uncovered a few important suggestions for consideration moving forward. For one, several patients in our study reported that the PRISM intervention would be more motivating and effective if a younger CF population was targeted, since they described that they already self-acquired similar skills at younger ages. This reflects previous research showing pediatric patients with CF were emotionally more mature and gained coping skills and resilience sooner than their healthy peers in part because they reported living with CF for a long time and having faced life challenges ³¹. The International Committee on Mental Health in CF recommends that children with CF ages 7–11 be clinically evaluated for depression and anxiety when caregiver depression or anxiety scores are elevated, or when significant symptoms of depression or anxiety in the child are reported or observed by patients, caregivers or members of the CF multidisciplinary team ⁹. Insufficient evidence exists on preventative psychological interventions or approaches to support people with cystic fibrosis and their caregivers ³². This pilot study warrants future research to explore the efficacy of a resilience-promoting intervention in larger groups of youth with CF, including younger age groups.

Second, we did not identify a change in patient-reported resilience (CD-RISC) or psychological distress (Kessler-6), even though both outcomes have been associated with the intervention in other, larger studies ^{17; 33}. There are several plausible reasons for this. First, this study was not designed or powered to detect intervention efficacy. Second, in prior studies, PRISM was delivered over a longer time frame of 4-8 weeks. The shorter intervals between PRISM sessions in our study may have limited skills-development and corresponding impact. In this group, the brief format of the intervention may not provide a sufficient “dose” of treatment. Finally, the selected population may not have been distressed to warrant the beneficial activation of resilience skills. Distress was low at 5.4 and 5.6 (both normal range scores) before and after the intervention, respectively. Hence, we had little opportunity to determine if PRISM could alleviate distress as it seems to do among patients with newly diagnosed cancer who had a mean Kessler-6 score of 8 in the usual care arm which is in the range of moderate psychological distress¹⁷. Further, our follow-up time was too brief to determine if it might protect patients from later distress.

On the other hand, certain domains of CFQ-R such as health perception and respiratory symptoms did seem to improve with PRISM. Since these self-reported CFQ-R questionnaires were done before and after a hospitalization in the setting of a respiratory illness, these domains may have also shown improvement not only with PRISM, but also with the extensive CF treatments during their hospitalizations and improvement of respiratory symptoms. We had no control group to compare the natural history of these changes; hence, we cannot attribute these changes to the intervention. Future studies will need to be sufficiently powered to test these hypotheses with appropriate control groups.

The PRISM intervention has been successfully researched in other AYAs with serious and chronic illnesses such as cancer and diabetes, in which the diagnosis is usually established later in life ^{14; 17; 18}. On the other hand, the diagnosis of CF is usually established during infancy and it is one of the most common life shortening diseases with increased morbidity which might reflect to their emotional status very early in life. Focused behavioral interventions could promote positive psychological outcomes and increase resilience in a lifelong chronic illness such as CF. The PRISM had never been applied to a CF patient population and in our pilot study our primary hypothesis was to test the acceptability and feasibility of PRISM and obtain basic data in a relatively short duration of time and in an accessible CF patient population who was admitted to the hospital with no significant time constraints. Eligibility criteria for the study included CF patients between 12-21 years-old and admitted to the hospital for “any” reason. Every eligible CF patient who was admitted to the hospital was approached for the study and invited to participate. However, patients who all accepted to enroll had an exacerbation. Our patient population was a “convenience” sample, which resulted with advantages and biases. Six out of 10 patients started the intervention as inpatient and completed the rest of the interventions as outpatient either in person or over the phone, which demonstrated that it was still feasible to continue in the outpatient setting. Our pilot study is not representative of the entire CF population especially in the outpatient setting. Future studies are needed to assess for generalizability of these interventions in a larger CF population in the outpatient setting with longer time intervals between sessions and when confounding factors such as acute illnesses are absent.

Our patient enrollment rate in this inpatient patient population was 59%. This low enrollment rate might be secondary to patient fatigue and respiratory symptoms especially during the first few days of their hospitalizations which required intensive medical treatment. We have not been powered to compare differences between the two groups of patients who declined and accepted to enroll, however, the findings do suggest that sicker patients may have been less likely to enroll (longer hospital stay and lower FEV1 % predicted values). Future studies will be more able to evaluate the role of illness severity on intervention feasibility and efficacy. Likewise, during a prolonged hospital stay for a CF exacerbation, caregivers usually resume their personal and professional duties and are not always able to accompany their children throughout hospitalization and provide consent for research studies. Indeed, two patients expressed willingness to enroll, but we were unable to include them because their adult caregivers were not available to provide written informed consent. This might also explain the low uptake rate for the “Coming Together” session which is found to be lower than the previously studied research populations with serious/chronic illness. Providing the PRISM intervention in the outpatient setting when patients present to their clinic visit with their legal guardians and when they are at their baseline health with less respiratory symptoms might increase the enrollment rate. Future iterations of PRISM may need to offer additional options for parents to participate in the individual sessions.

Another limitation of our study is the limited heterogeneity of our sample since the study took place in a large CF center at an academic pediatric hospital, which does not reflect enough diversity. Future studies are needed to assess for generalizability of these interventions in different ethnicities, non-English speaking patient populations and different socioeconomic statuses and education levels.

In this small pilot study we were able to demonstrate the feasibility for the PRISM intervention in the CF AYA population in the inpatient setting. Multicenter studies should be targeted to increase the sample size and examine efficacy. Future studies could be also directed to the younger CF patients with an adjusted PRISM model in the outpatient setting with longer time intervals between sessions and when confounding factors such as acute illnesses are absent. Involving the parents/caregivers in these modules could be also more often encouraged. As a whole, this is a promising approach to help youth with CF prepare for and address stress and improve resilience as they move through adolescence into adulthood.