Introduction: Clinical Nurse Specialists (CNS’s) are commonly the primary point of contact in cystic fibrosis (CF) centres. Aim: To identify and compare preferred modes of contact to paediatric (P) and adult (A) CF CNS’s for healthcare users. To identify when patients take responsibility for their own management. Method: P and A CF CNS’s prospectively recorded how, why and by whom they were contacted over a 30 day calendar month. CNS’s were available via telephone and email. The P team have a pager for messaging, A CNS’s have a mobile telephone. Results: 340 contacts were received (130 P, 210 A) from clinics of 130 P and 147 A patients. Contact methods: phone 57% P vs 75% A, email 12% P vs 7% A. Pager 31% P vs mobile texts 18% A. No children initiated contact in the P clinic. 2/12 patients <18 years in the A clinic initiated contact. All contacts were from the patients after the age of 18 years. 12 contacts to the P CNS were from Health Care Professionals (e.g. GP, community nurse). Reasons for contact: Respiratory management 42% P vs 39% A. 30% of the A contacts were for administrative issues, e.g. outpatient appointments or admission dates, vs 6% in P. Referral on to other team members was necessary in 12% P vs 21% A. Conclusions: Patients <18 yrs rarely take responsibility for their own management by contacting their CF CNS, leaving this to parents. Email contact was lower than anticipated in both P and A centres. There was a higher per capita rate of contact from the adult patients 1.4 vs 1, as well as a higher need for further MDT involvement, possibly reflecting their increasing medical needs. This age group utilise technology to contact their CNS’s less than expected.

Introduction: Physical activity in CF can help offset the limitations of the disease and optimize quality of life.Despite this, many patients have habitually low activity levels: we wished to study whether this was related to their exercise capacity.Method: We used an interview-based physical activity scoring system in 66 patients (mean age 25 years [SD±4.8],36 female), to assess their weekly occupational and leisure time activity.Patients then underwent assessment of their maximal steady state exercise capacity (VO2 MSS), peak exercise capacity (VO2 Peak) and functional aerobic impairment (%FAI).FEV1 and BMI were also noted.Results: Activity scores (AS) indicated that the group was mainly sedentary (47%), including those with good lung function and nutritional state, and only 11% had high scores.AS correlated strongly with MSS exercise capacity (r = 0.745, p < 0.001), and VO2 Peak and %FAI (both r = 0.52, p < 0.001), less well with FEV1 (r = 0.371, p < 0.01), but not with BMI (r = 0.195) or age (r = 0.001) (both p=NS).Males were more active than females (mean AS 1.7 versus 1.2), and were capable of higher sustained exercise (mean MSS VO2 21.5 v 15.7), but there was no difference in peak exercise capability (VO2 Peak 64.8% v 63.9%) or %FAI (mean 35.2 v 36.1). Conclusion:This study shows that, although many patients have a good lung function and nutritional state and are capable of exercise, they adopt a sedentary lifestyle which may in the longer term have a negative impact on disease progression and subsequent quality of life.More work needs to be done to aid patients to adopt a more active lifestyle including regular, higher-intensity activities, especially in those who appear to be limited by physical fitness and behavioural choices rather than by respiratory or nutritional factors alone.

Objectives: Few studies have documented relationships between caregiver depression, treatment adherence and medical outcomes in CF.Further, no studies have employed electronic monitors to measure enzyme adherence.This study examined the effects of caregiver depression on electronically monitored enzyme adherence over a three-month period in relation to changes in weight percentiles for children with CF.Methods: As part of a larger intervention study at 3 CF Centers, 88 children with CF ages 1 to 11 and their parents participated.Parents completed a standard measure of depression (CESD) at enrollment and were provided with pill caps recording the date and time of each bottle opening.Standard health outcomes were also assessed at enrollment and 3 months later.Results: Caregivers reported elevated levels of depression, with 30% scoring in the clinical range.Rates of adherence to enzymes was poor (43% at home and 48% at school).Caregiver depression was negatively associated with adherence, with depressed caregivers demonstrating lower rates of adherence (11 percentage points).Enzyme adherence was associated with changes in weight, with a 100% adherence translating into 5 percentile points of weight gain.Conclusions: Rates of adherence were surprisingly low and were associated with more caregiver depression and a loss of weight over three months.Caregiver depression appears to be under-diagnosed and these results suggest that more attention should be focused on parental adjustment.Improvements in weight percentile with better enzyme adherence highlight the need for interventions that promote adherence in children with CF.