Current characteristics, challenges and coping strategies of young people with cystic fibrosis as they transition to adulthood

Background Research has shown that broad cognitive functioning in individuals with CF is intact. Specific executive functioning (EF) deficits have been identified, however, and adults with CF report more symptoms of ADHD than the general population. EF skills are critical to the management of a complex disease like CF although studies have not adequately examined EF mechanisms in CF. This manuscript (a) described EF in a small sample of children with CF, (b) summarized relations found between EF and psychosocial variables, and (c) presented a conceptual model by which to understand EF's impact on adherence in CF. Methods Data for this preliminary study were collected from 19 children with CF and their caregivers (ages, 6‐18). Caregivers completed questionnaires assessing their child's physical and mental health, their own functioning, and overall family functioning. EF was measured using a parent‐report rating scale. Patient health data were collected from the electronic medical record. Results This sample did not demonstrate elevated levels of EF impairment. Worse EF was related to poor family communication/cohesion, as well as higher treatment burden, worse lung function, poorer adherence, and older age. From these findings, a preliminary model was developed describing EF in the context of CF and adherence. Conclusions Findings from this preliminary study suggest that the CF regimen and associated symptoms may overload otherwise adequate EF skills. Reducing disease burden and preventing burnout should be a focus of treatment. A better understanding of EF in CF and the impact on adherence would allow for better clinical management and effective design of interventions.

Section: Discussionsupporting

confidence: 85%

Executive functioning in pediatric cystic fibrosis: A preliminary study and conceptual model

Borschuk

Molitor

Everhart

et al. 2020

“…A more recent study performed structured interviews with 45 recently transitioned patients, and noted that although there was significant individual variability, the mean FEV1 and BMI did not change 1 year after transfer. Importantly, there was a sense of optimistic acceptance about the future . As more centers are becoming engaged in the creation of a formalized transition program, it would behoove the community to study post‐transition outcomes further, including BMI, FEV1, pulmonary exacerbations, and patients lost to follow‐up after transfer.…”

Section: Cf Risementioning

confidence: 99%

Overcoming barriers to a successful transition from pediatric to adult care

Goralski

Nasr

Uluer

2017

As life expectancy for people with cystic fibrosis (CF) has increased dramatically, so has the need for a guided, structured transition from pediatric to adult-focused care. A formalized transition program allows for seamless transfer of patients between providers, helping to ensure continuity of care, and avoid potential declines associated with inconsistent medical care. New CF Center guidelines issued by the CFF strongly recommend that each center establish a transition program for age-appropriate transition to an adult CF clinic. In this article, we explore the remaining barriers to establishing a transition program in a CF Center and offer examples of several successful models. We describe CFF-sponsored and other initiatives that exist to support centers in establishing a transition program and discuss the need for ongoing research in this area.

“…In the Netherlands, around 1500 people live with cystic fibrosis (CF) and more than half are over 18 years of age . Their life expectancy has improved and is likely to improve further with the advent of CF transmembrane conductance regulator modulator therapies . CF care in the Netherlands has been concentrated in seven centers, each with an adult and a pediatric department.…”

Section: Introductionmentioning

confidence: 99%

“…Although there is no shared model, the TC's core principle is that professionals from both pediatric care (PC) and adult care (AC) deliver outpatient care in preparation for the upcoming transfer . Studies evaluating TCs in CF care have reported improved health status, self‐care and self‐advocacy skills, and more independence . However, most studies did not include a controlled pre‐post outcome evaluation, and the body of evidence for effects is still small.…”

Section: Introductionmentioning

confidence: 99%

“…1 Their life expectancy has improved and is likely to improve further with the advent of CF transmembrane conductance regulator modulator therapies. 1,2 CF care in the Netherlands has been concentrated in seven centers, each with an adult and a pediatric department. Although treatment protocols are standardized across these centers, 3 differences in the organization of care continue to exist.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Controlled evaluation of a transition clinic for Dutch young people with cystic fibrosis

Peeters

Sattoe

Staa

et al. 2019

Background Transition clinics (TCs) are advocated as best practice to support young people with cystic fibrosis (CF) during transition to adulthood and adult care. We aimed to research the functioning of a TC for young people with CF compared with direct hand‐over care and to evaluate whether those treated at the TC have better transfer experiences and outcomes compared with the control group. Methods Mixed‐methods retrospective controlled design, including interviews with professionals, observations of clinics, chart reviews (at four measurement moments), and patient surveys. Qualitative data analysis focused on organization and daily routines, and barriers and facilitators experienced. Young people's transfer experiences, self‐management, health care use, and clinical outcomes were assessed quantitatively. Results The most notable feature distinguishing the TC and direct hand‐over care comprised joint consultations between pediatric and adult care professionals in the former. A transition coordinator was considered essential for the success of the TC. The main barriers were lack of time, planning, and reimbursement issues. Young people treated at the TC tended to have better transfer experiences and were more satisfied. They reported significantly more trust in their adult care professionals. Their self‐management‐related outcomes were less favorable. Conclusions The TC had several perceived benefits and showed positive trends in transfer experiences and satisfaction, but no differences in health‐related outcomes. Structured preparation of young people, joint consultations with pediatric and adult care professionals, and better coordination were perceived as facilitating elements. Further improvement demands solutions for organizational and financial barriers, and better embedding of self‐management interventions in CF care.