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Health Outcomes of Parents With Cystic Fibrosis-Aim 2

Recruiting
Conditions
Cystic Fibrosis
Parenthood Status
Registration Number
NCT06296394
Lead Sponsor
University of Pittsburgh
Brief Summary

The goal of this observational prospective study is to determine the health impact of parenthood on United States (US) people with CF in the era of CF transmembrane regulator protein (CFTR) modulators. The investigators will collect physical and mental health data to comprehensively evaluate the impact of parenthood in CF with widespread highly effective CFTR modulator use. The main hypotheses this study aims to examine are:

H1: Parents with CF and moderate-to-severe depression have more rapid change in ppFEV1 (percent predicted forced expiratory volume in one second) versus those with mild or no depression.

H2: Parents with CF who have more parental responsibility and/or stress have more rapid ppFEV1 (percent predicted forced expiratory volume in one second) change than those with less responsibility/stress

H3: Parents using CFTR modulators have decreased ppFEV1 (percent predicted forced expiratory volume in one second) change versus those not using CFTR modulators

Participants will complete quarterly surveys during the first year of parenthood and biannual surveys, thereafter, using the computer-based survey system on an iPad protected for infection control or via personal device or computer via emailed survey link.

Detailed Description

The investigators will follow 146 new parents of children \<5 years of age at 18 participating US adult CF centers to assess the primary outcome of percent predicted forced expiratory volume in one second (ppFEV1) up to 5 years after becoming a parent. A prospective approach will capture the immediate and long-term impact of the use of the highly effective CFTR modulator ETI (elexacaftor/tezacaftor/ivacaftor) by \~90 percent of US adults with CF. By combining objective health measures and participant surveys, the investigators can comprehensively assess the psychosocial impacts of parenthood and explore the interplay between the parenting role and physical and mental health. The investigators anticipate identifying modifiable factors that may ameliorate negative health impacts of parenthood. The investigators will conduct hypothesis-generating, semi-structured dyadic interviews with a subset of parents and their key supports (partner/family/friend) to inform future interventions. The investigators have selected qualitative methodology to avoid preconceived theories/hypotheses.

Recruitment & Eligibility

Status
RECRUITING
Sex
All
Target Recruitment
146
Inclusion Criteria
  • Diagnosed with cystic fibrosis via sweat test or genotype analysis
  • Became a first-time parent (including foster parent, step parent, adoptive parent, or legal guardian) to a child under 5 years of age within the last 90 days
Exclusion Criteria
  • Undergone a lung transplant
  • Does not speak/read English or Spanish

Study & Design

Study Type
OBSERVATIONAL
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
Forced Expiratory Volume (FEV1)Annually from 2 Years prior to enrollment and annually for Year 1 through Year 5

Rate of change in ppFEV1 as reflected in the medical record from Year 1 to Year 5

Secondary Outcome Measures
NameTimeMethod
Length of HospitalizationsAnnually from 2 Years prior to enrollment and annually for Year 1 through Year 5

The length of each hospitalization, as reported in the medical record, will be used to compute the rate of hospitalizations.

History of CF DiagnosisYear 1

Date of diagnosis, as reported in the medical record.

Number of HospitalizationsAnnually from 2 Years prior to enrollment and annually for Year 1 through Year 5

The number of hospitalizations, as reported in the medical record, will be used to compute the rate of hospitalizations.

Microbiologic ProfileAnnually from 2 Years prior to enrollment and annually for Year 1 through Year 5

Number of positive screenings of specific types of bacteria, mycobacteria, fungus in cultures as reported in the CFFPR

BMIAnnually from 2 Years prior to enrollment and annually for Year 1 through Year 5

Height and weight combined, as reported in the medical record, will be collected to calculate BMI and BMI percentile

Parental StressUp to 4 times in Year 1, Up to 2 times in Year 2 through Year 5.

The Parental Stress Scale is a self-reported questionnaire which assesses feelings and perceptions about the experience of being a parent. Eighteen-item measure with 5-point Likert scale questions from 1 (strongly disagree) to 5 (strongly agree). Score is determined by the sum (possible range of 18 to 90) with higher scores indicating a higher measured level of parental stress.

Life StressorsUp to 2 times per year in Year 1 through Year 5.

Holmes-Rahe Life Stress Inventory (HRLSI) is a forty-three-item measure which assesses life stressors by accounting for life events over a 1-year period. Each life event corresponds to a point value (range 11-100). Scores are determined by the sum of the values for all checked life events. Scores of 150 or less refer to a relatively low amount of life change and low susceptibility to stress-induced health breakdowns. Scores of 151 to 300 imply about a 50% chance of a major health breakdown in the next two years. Scores greater than 301 raise the odds of a major health breakdown to about 80%.

Pulmonary Function Tests (PFTs) - Forced Vital Capacity (FVC)Annually from 2 Years prior to enrollment and annually for Year 1 through Year 5

Occurrence of PFTs at each clinic visit/hospitalization. Measures include FVC (Forced vital capacity), and the percent predicted.

Pulmonary Function Tests (PFTs) - Forced Expiratory Volume (FEV1)Annually from 2 Years prior to enrollment and annually for Year 1 through Year 5

Occurrence of PFTs at each clinic visit/hospitalization. Measures include FEV1(Forced expiratory volume in one second), and the percent predicted.

Number of Pulmonary ExacerbationsAnnually from 2 Years prior to enrollment and annually for Year 1 through Year 5

The number of pulmonary exacerbations as reported in the medical record will be used to compute the rate of pulmonary exacerbations.

, hospitalizations (number and length)

Severity of Pulmonary ExacerbationsAnnually from 2 Years prior to enrollment and annually for Year 1 through Year 5

The severity of pulmonary exacerbations (mild, moderate, severe), as reported in the medical record.

Rate of Hospitalizations Due to Pulmonary ExacerbationsAnnually from 2 Years prior to enrollment and annually for Year 1 through Year 5

The number and length of hospitalizations, combined, due to pulmonary exacerbations will be used to compute the rate of pulmonary exacerbations.

CF-Related Diabetes Control - Insulin Prescription UsageAnnually from 2 Years prior to enrollment and annually for Year 1 through Year 5

Insulin prescription use and dosage as reported in the medical record

Cystic Fibrosis Questionnaire to evaluate quality of life domainsUp to 2 times per year in Year 1 through Year 5.

The Cystic Fibrosis Questionnaire-Revised (CFQ-R) is a disease-specific instrument designed to measure the impact on overall health, daily life, well-being and symptoms. It consists of 50 individual measures assessing 9 quality of life domains: physical, role/school, vitality, emotion, social, body image, eating, treatment burden, and health perceptions. Each question is scored with the following scales: for questions 1 - 6: Very True = 1, Mostly True = 2, Somewhat True = 3, Not at all True = 4. For questions 7 - 17: Always = 1, Often = 2, Sometimes = 3, Never = 4. For questions 18 - 30: Very True = 1, Mostly True = 2, Somewhat True = 3, Not at all True = 4. For questions 31 - 35: Always = 1, Often = 2, Sometimes = 3, Never = 4. Scaled scores are calculated using the following formula: (Sum of responses - Minimal Possible sum (n x 1)) / (Maximum possible sum (n x 4) - Minimum possible sum (n x 1)) x 100.

Parental ResponsibilityUp to 4 times in Year 1, Up to 2 times in Year 2 through Year 5.

Adapted Parental Responsibility Scale (APRS) is a forty-item measure which assesses responsibility forms of parental involvement. Each form of responsibility is to be rated on a 5-point Likert scale (I always do, I usually do, co-parent and I equally do, co-parent usually does, and co-parent always does) to designate who had primary responsibility for each task.

DepressionUp to 4 times in Year 1, Up to 2 times in Year 2 through Year 5.

The Patient Health Questionnaire depression scale (PHQ-8) is an 8-item screening tool used to assess depressive symptoms and severity over a 2-week period. The frequency of symptoms is evaluated using a 4-point scale (not at all, several days, more than half the days, nearly every day). Total score (range of 0 to 24) is determined by adding together the scores of each of the items. Scores of 5, 10, 15, and 20 represent the cutoff points for mild, moderate, moderately severe and severe depression, respectively.

Pulmonary Function Tests (PFTs) - Forced Expiratory Flow at 25 and 75 percent (FEF25-75)Annually from 2 Years prior to enrollment and annually for Year 1 through Year 5

Occurrence of PFTs at each clinic visit/hospitalization. Measures include FEF25-75 (forced expiratory flow at 25 percent and 75 percent of the pulmonary volume), and the percent predicted for each.

History of CF Genotype InformationYear 1

Genotype information, as reported in the medical record.

History of CF MutationYear 1

Mutation selection, as reported in the medical record.

Treatment of Pulmonary ExacerbationsAnnually from 2 Years prior to enrollment and annually for Year 1 through Year 5

The type of treatment used for pulmonary exacerbations (IV antibiotic, oral antibiotic), as reported in the medical record.

Clinic Visit AttendanceAnnually from 2 Years prior to enrollment and annually for Year 1 through Year 5

Number of outpatient clinic visits attended, as reported in the Cystic Fibrosis Foundation Patient Registry (CFFPR).

Medication useAnnually from 2 Years prior to enrollment and annually for Year 1 through Year 5

Number of positive screenings of medication use, as reported in the medical record, including CFTR modulator therapy, chronic antibiotic use, and home IV treatment.

History of CF-Related Diabetes StatusAnnually from 2 Years prior to enrollment and annually for Year 1 through Year 5

Instances of positive screenings for CF-Related Diabetes as reported in the medical record.

CF-Related Diabetes Control - Hemoglobin A1CAnnually from 2 Years prior to enrollment and annually for Year 1 through Year 5

Highest Hemoglobin A1C values, as reported in the medical record.

CF-Related Diabetes Control - Glucose ToleranceAnnually from 2 Years prior to enrollment and annually for Year 1 through Year 5

Oral glucose tolerance test results, as reported in the medical record.

Liver Disease StatusAnnually from 2 Years prior to enrollment and annually for Year 1 through Year 5

Liver disease status (gall stones, liver disease with cirrhosis, liver disease non-cirrhosis, acute liver failure, hepatic steatosis) as reported by the medical record.

Transplantation StatusAnnually from 2 Years prior to enrollment and annually for Year 1 through Year 5

Current transplant status of the patient (not pertinent, accepted on waiting list, evaluated with final decision pending, evaluated and rejected, or had transplantation), as reported in the medical record.

Sleep QualityUp to 4 times in Year 1, Up to 2 times in Year 2 through Year 5.

The Pittsburgh Sleep Quality Index (PSQI) is a self-rated questionnaire which assesses sleep quality and disturbances over a 1-month period. Nineteen individual items generate seven "component" scores: subjective sleep quality, sleep latency, sleep duration, sleep efficiency, sleep disturbances, use of sleeping medication, and daytime dysfunction. The seven component scores are derived, each scored 0 (no difficulty) to 3 (severe difficulty). The component scores are summed to produce a global score (range 0 to 21). Higher scores indicate worse sleep quality.

Transplantation TypeAnnually from 2 Years prior to enrollment and annually for Year 1 through Year 5

If participant had transplantation in previous years, the transplant type will also be recorded as shown in the medical record

Social SupportUp to 4 times in Year 1, Up to 2 times in Year 2 through Year 5.

Interpersonal Support Evaluation List-Short Form (ISEL-SF) is a 12-item measure that assesses the perceived availability of social support from family, friends, and others. Each statement is scored using a 4-point scale (definitely false, probably false, probably true, definitely true) to measure subscales of appraisal, belonging, and tangible support. All items are summed to a total score ranging from 0 to 36 with higher scores indicating greater perceived social support.

Daily Care Check-InUp to 2 times per year in Year 1 through Year 5.

The Daily Care Check-In Tool is an 18-item measure that assesses the occurrence and frequency of facing barriers to completing treatments over a 6-month period. The Daily Care Check-In Occurrence Scale score can range from 0 (no barriers) to 18 (experiences all barriers). Each barrier is further scored based on a 5-point Likert scale (always, often, sometimes, rarely, never) to assess the frequency of each interference to treatment. Scores for the Daily Care Check-In Interference Scale are calculated by summing the point values assigned to each score. Scores can range from 0 (no barriers) to 90 (experiences all barriers and they always interfere with completing treatments).

AnxietyUp to 4 times in Year 1, Up to 2 times in Year 2 through Year 5.

The Generalized Anxiety Disorder Assessment (GAD-7) is a seven-item measure that is used to assess the severity of generalized anxiety disorder over a 2-week period. The frequency of symptoms is evaluated using a 4-point scale (not at all, several days, more than half the days, nearly every day). The whole scale score can range from 0 to 21. Scores of 0 to 4 indicate minimal anxiety, scores 5-9 indicate mild anxiety, scores 10-15 indicate moderate anxiety and scores greater than 15 indicate severe anxiety.

Self-Reported AdherenceUp to 2 times per year in Year 1 through Year 5.

The Self-Reported Adherence to Highly Effective Modulator Therapy scale is a 4-item questionnaire aimed at identifying the class of modulator (ivacaftor, elexacaftor-tezacaftor-ivacaftor, tezacaftor-ivacaftor, lumacaftor-ivacaftor), presence of altered dosing schedules (specific time or days), and missed treatments (yes, no). Adherence is further evaluated by assessing the number of days that treatment occurred as prescribed (0-3 days, 4-6 days, 7-10 days, 11-14 days) over a 2-week period. This measure serves as descriptive data only, and therefore requires no scoring calculations.

Trial Locations

Locations (18)

University of Alabama-Birmingham

🇺🇸

Birmingham, Alabama, United States

National Jewish Health

🇺🇸

Denver, Colorado, United States

Northwestern University

🇺🇸

Evanston, Illinois, United States

Indiana University

🇺🇸

Indianapolis, Indiana, United States

University of Kansas Medical Center

🇺🇸

Kansas City, Kansas, United States

Johns Hopkins University

🇺🇸

Baltimore, Maryland, United States

Massachusetts General Hospital

🇺🇸

Boston, Massachusetts, United States

Boston Children's Hospital/Brigham and Women's Hospital

🇺🇸

Boston, Massachusetts, United States

University of Michigan

🇺🇸

Ann Arbor, Michigan, United States

University of Minnesota

🇺🇸

Minneapolis, Minnesota, United States

Washington University School of Medicine

🇺🇸

Saint Louis, Missouri, United States

University of North Carolina

🇺🇸

Chapel Hill, North Carolina, United States

Oregon Health and Science University

🇺🇸

Portland, Oregon, United States

University of Pennsylvania

🇺🇸

Philadelphia, Pennsylvania, United States

University of Pittsburgh

🇺🇸

Pittsburgh, Pennsylvania, United States

Medical University of South Carolina

🇺🇸

Charleston, South Carolina, United States

University of Texas-Southwestern

🇺🇸

Dallas, Texas, United States

University of Washington

🇺🇸

Seattle, Washington, United States

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