Background
Methods
Study design
Quantitative phase
Qualitative phase
Integration phase
Results
Study cohort characteristics (quantitative phase)
Parents (N = 88) | |
---|---|
Age range in years, n (%) | |
18–25 | 8 (9.2%) |
26–30 | 17 (19.5%) |
31–35 | 31 (35.6%) |
36–40 | 18 (20.7%) |
41–45 | 9 (10.3) |
46 and older | 4 (4.6%) |
Female, n (%) | 75 (86.2%) |
Educationc, n (%) | |
Some high school | 4 (4.7%) |
Graduated high school | 16 (18.6%) |
Some college | 29 (33.7%) |
Graduated college | 30 (34.9%) |
Graduate school | 7 (8.1%) |
Multiple children with CF, n (%) | 17 (19.5%) |
Distance to CF Center in miles, med (IQR), n | 43.0 (19.3–90.7), 87 |
CES-D, med (IQR), n | 17 (9–26), 81 |
DUREL, med (IQR), n | 10.5 (7–14), 86 |
Negative Spiritual Coping, med (IQR), n | 1 (0–3.5), 84 |
Self-efficacy, median (IQR), n | 1071 (989.5–1097.3), 84 |
Adherencea | |
Aerosolized Medications, median (IQR), n | 0.8 (0.5–1.1), 68 |
Airway Clearance, median (IQR), n | 0.8 (0.5–1), 87 |
Children with CFb | |
Age in years, med (IQR), n | 4.9 (2.4–9.6), 88 |
BMI percentile, median (IQR), n | 58 (35–81), 87 |
Exacerbations in prior year, n (%) | |
0 | 46 (52.3%) |
1 | 20 (22.7%) |
2 or more | 22 (25%) |
Exploratory analyses
Adherence Outcomes | |||
---|---|---|---|
Aerosolized meds | Airway clearance | Self-Efficacy | |
Parents | |||
Age | −0.14 | −0.17 | −0.12 |
Gender | 0.02 | 0.06 | 0.09 |
Education | −0.08 | 0.15 | 0.07 |
Multiple children with CF | −0.03 | −0.01 | − 0.27 |
Distance to CF Center (miles) | −0.01 | 0.12 | −0.18 |
CES-D | −0.09 | −0.13 | − 0.19 |
DUREL | 0.06 | −0.08 | 0.09 |
Negative spiritual coping | −0.29 | −0.07 | − 0.26 |
Children with CFb | |||
Age (years) | 0.01 | −0.20 | −0.22 |
BMI percentile | 0.26 | 0.06 | 0.23 |
No. of exacerbations in prior year | 0.06 | 0.14 | 0.09 |
Segmentation models
Adherence (Model 1) | Self-Efficacy (Model 2) | ||||
---|---|---|---|---|---|
Components (clinical relevance) | I (Parental capability) | II / III (Barriers to CF care / child nutrition) | I / III (Barriers to CF care / child nutrition) | ||
Cumulative % of variation explained by each component | 57.0% | 81.0% | 98.5% | 68.2% | 96.3% |
Characteristics | |||||
Child BMI percentile | −0.43 | −0.47 | 0.33 | 0.61 | |
Distance to CF center (miles) | 0.12 | 1.24 | −0.88 | −1.26 | 0.83 |
Self-efficacy | 1.03 | −0.29 | – | – |
Clinical personas (qualitative phase)
Persona | Goals | Characteristics | Clinical Implications/Actions (Integration Phase) |
---|---|---|---|
Maria-mother, 1 y.o. Beatrice Age, 26, Hispanic, married, homemaker | • Child will become more independent • Child is happy and has a family of her own • Illustrative quote “I don’t put her in a bubble, what’s the point? We don’t limit her in what we let her do because of CF.” | • Minimal community support through social media and church • Live 6 miles from CF center • Regularly forgets enzymes and treatments • Child’s BMI in 30th percentile • Eats breakfast with family every AM • Often naps mid-day | • Immediate focus needed on familial support. • Medical intervention is not as important as mental health • Because family lives near the CF center, clinicians could offer monthly visits and coordinate with social workers and staff psychologists if applicable |
Liam-father, 4 y.o Maddie, 2 y.o Brian Age 28, Caucasian, married, homemaker | • Children should be true to themselves, make good decisions, and live to be an old age • Illustrative quote “We just treat them like every day is their last.” | • Active on social media for community support, but not in church • Live far from CF Center • Anxious about getting in every treatment, highly adherent • Children’s BMI in 60th Percentile • Regularly plays outside with children • Spends time with wife each night | • Family has good adherence with CF, but concerning that they live everyday as if it is their last, which may present as anxiety • Clinicians should partner with family to make care planning a team aspect, which can help normalize CF • Offering family clinical research opportunities to enroll, in order to help alleviate some anxiety and provide sense of purpose • Ensure family is properly connected with online groups. Imperative that the family is getting proper social media information and support • Connect family with a learning network monitored by the CF center to enable them to receive proper support |
Anna-mother, 3 y.o. Francesca Age, 31, Caucasian, married, works from home as engineer | • For Francesca to enjoy her life, get the best education, and be an outstanding adult • Illustrative quote: “CF fits in like anything else. I’m a germophobe, but I’m not OCD. That’s where my faith comes in. That’s a huge thing.” | • Not active on social media, but active in church community • Live 15 miles from CF center • Highly adherent to treatments • Child’s BMI in 50th percentile • Views relationship with husband as a team | • Family is adherent and likely to incorporate changes if needed • Type of family that should continue to receive support, but there is not a strong need to intervene |
Barbara-mother, 7 y.o Henry Age, 35, Black/African American, married, homemaker | • Happiness • Good job later in life • Independence with treatment • Illustrative quote: “I just want him to be happy. Hopefully he’ll find a really good job and a husband or wife, whichever way he goes, and to have kids and be a good person.” | • Active in church community but not social media • Live 30 miles from CF center • Not consistently adherent with treatments • Does not plan ahead or have a regular routine • Conflicted marriage • Personal health issues, depression | • Seems family is in chaos on how to work in day to day treatments • Family tends to make spur-of-the-moment decisions, a likely reason they are forgetting to be adherent • Important that the team focus on the most important medical outcome of the child as to not overwhelm this family • Helpful to focus on improving family adherence to critical treatments, such as pancreatic enzymes • Educating this family is important; since they live further away from the CF center, offer telehealth with a dietitian and social worker to provide needed support • Inform family on financial aid options for CF care to help alleviate stressors and promote focus on care |
Charlie- father, 9 y.o Jacqueline (with CF) and her 2 sisters (without CF) Age, 30, Caucasian, married, accountant | • That child understands the importance of eating and nutrition • Illustrative quote: “Since Jacqueline is older now, it is easier because she is independent enough to do treatments herself.” | • Active in church community, regular attends bible study • Live near CF center • Adherent to treatments, child is independent enough to do majority of treatments • Childs BMI above 50th percentile • Plans family time around CF treatments | • High functioning family does good job of adhering to medication • Daughter is becoming more independent, so team can focus on educating the child to do her own treatments • Fostering her independence can make for a smooth transition into adolescence and alleviate some stressors this family may face |
Danielle-mother, 11 y.o, Luke Age, 43, Caucasian, married, part-time retail associate | • Typical life for child • Ability for child to have many friends • Wants Luke to be part of a sports team • Major goal is for Luke to graduate high school • Illustrative quote: “Things are just so difficult right now trying to change our lives around CF, but we are trying to make it work.” | • Not active in church or social media community • Lives 55 miles from CF center • Adherent and anxious about treatments due to Luke’s late diagnosis • Child’s BMI below 40th percentile • Feels late diagnosis makes it difficult for Luke to be involved in sports and other activities • Spend a lot of time together watching TV and making dinner as a family | • Often, when people are worried about disease progression, clinicians can see high adherence • Family still struggling with their son’s BMI. Using goal setting with providers and the child to incorporate him and the family onto the team could be helpful • Goal setting will also encourage independence with the child with appropriate oversight from the team • Family should identify long-term personal goals for Luke and work with the team to give him the medical support he needs to achieve these goals. • Offer family opportunities to discuss their situation with other families of children diagnosed later in life; may also be beneficial in order to help them adjust to this new aspect of their lives. |
Emma-mother 13 y.o, Molly Age, 37, Caucasian, single, Registered Nurse | • Understand the importance of treatments • Begin to get into a routine • Illustrative quote: “Well, I just had to change my life a lot (since CF). The feasibility of keeping drugs on you seems to get harder and harder at school. I think we have control over it. She doesn’t really think that far ahead, she’s more day by day. CF is not a bad thing in our life, just part of our life. It’s a routine” | • Not active on social media or in church community • Live near CF center • Adherent about 50–75% of the time • Child’s BMI in 10th percentile • Feels like they are constantly busy • Difficultly getting proper nutrition, often stopping to eat fast food | • Seems family has a good handle on their CF diagnosis and views CF care as part of their life • Room for nutrition improvements, and they are struggling with completing airway clearance treatments • Given family’s busy schedule, the team should focus on helping the family reschedule their routine and restructure their day to put more emphasis on improved nutrition and fitting in treatments |
Floyd-father, 4 y.o Oliver Age, 36, Caucasian, married, data analyst | • Oliver to have a typical life and good education • Continue to work to provide for his family • Illustrative quote: “What I’m focused on now is getting to work so I can continue to have insurance to afford healthcare.” | • Not involved in social media or church community • Live far from CF center • Mostly adherent to treatments, but not sure • Child’s BMI in 35th percentile • Works overtime most weeks • Tries to spend as much time with his son as possible • Doesn’t see lack of adherence as a problem | • Parents may come into the CF clinic and seem like they have high self-efficacy and are adherent; however, upon further probing it can be revealed that there are issues with completing treatments, as is the case with this family • Sometimes parents do not always think their lack of adherence is a problem because there is no visible evidence to support this • Offer a CT/MRI for evidence-based perception, which can show actual problems with non-adherence • Closer follow-up with this family is also recommended via monthly appointments and/or telehealth |