Results
After the initial meeting with the hospital administration, two more examination rooms were allocated to the center. A strict IP&C policy was initiated with the support of the Hospital Infection Control Committee and administration. The required personal protective equipment like gowns, masks, and gloves were provided in both the outpatient clinic and the inpatient. A second CF nurse was added; both nurses are funded by donations raised by KIFDER. The fellow’s visit to UoM CFC was a good opportunity to see the multidisciplinary approach to CF care. She observed the importance of the teamwork in the follow-up of pw CF and recognized the importance of strict infection control.
To address the decreased pulmonary funtion for pw CF, UoM CFC standardized patient respiratory care protocols and algorithms were implemented at Ma CFC. The focus of the QI project was CF patients 6-18 years old with FEV1 pp <80, however, all protocols and algorithms were also applied to all patients in the center. The QI project was initiated in June, 2019, and ended on 10/2020. Flow charts from each member of the care team were created, standardized CF care algorithm was implemented and an individualized treatment plan for each patient was created to address barriers to adherence to the treatment plan that may result in FEV1 pp <80. Baseline, 6th and 12th month mean FEV1%pred was 62.2±15.1, 67.2±17.7, 70.3±19.0 respectively. Overall, there was significant improvement in mean FEV1 pp by 7.3 % in 6 months (p<0.001) and by 10.1 % in 12 months (p<0.001). 10 The algorithms and protocols became part of the center follow up. Table 2 shows the comparison of FEV1pp values of 2017 vs. 2020 according to age groups
The hospital administration increased the dietary support to 2 full time dietitians. Through the collaboration, the dietitians learned additional evaluation tools for a more in-depth assessment of patients’ nutritional status, including pediatric malnutrition criteria. In addition, a QI project was started to help improve the nutritional status of pw CF at Ma CFC. Growth and development were evaluated according to the protocol determined within the scope of the QI project. While height and weight were evaluated prior to the project, BMI was added to the evaluation. Recommendations for increasing caloric intake were made as indicated after the evaluation of the food consumption records of the patients. Oral nutritional supplements were recommended as needed. Pancreatic enzyme replacement therapy was maximized. In addition, nutrition education about the importance of maximizing the caloric intake was given to pw CF and their families.
Appetite stimulants started to be used for children in the higher risk group as defined by the QI project protocol. When enteral nutrition is indicated, pw CF and their families were informed in advance and the plan was discussed in detail to gain their agreement. Appropriate nutritional supplements were started through the G-tube, and appropriate enzyme dosing was ensured. Summary of the initial work was presented in North American CF Conference. 11 Table 3 shows Comparison of BMI Z-score values in 2017 vs. 2020 according to the age groups
To address the poor adherence to airway clearance techniques, patients’ low activity levels, and poor percussion and postural drainage, the 2 teams worked together to improve patient information materials, including developing booklets for patient education that also included section on improving hygiene and nebulizer cleaning and disinfection. In addition, the physical therapy team (PT) team at Ma CFC has implemented a one-on-one teaching to improve patient adherence and a regular evaluation of percussion and postural drainage (P&PD) and aerobic exercises regularly was started. Patients’ positive expiratory devices are screened in every visit, and their High Frequency Chest Wall Oscillation Devices (VEST), if available are screened yearly for proper use. The PT team also visits the patients during hospitalizations for follow up during acute exacerbations and for implementation of P&PD and aerobic exercises. Also, hearing, and vestibular dysfunction symptoms and bowel and bladder incontinence symptoms have been added to the evaluation during regularly scheduled clinic visits.
A focus on improving the knowledge of CF caregivers was done through standardized training programs supported by various educational material. To improve nebulized machines cleaning and disinfection, a half-day education program was started once a week for 10-12 caregivers every week. One hundred and ninety caregivers received this education till the start of the COVID-19 pandemic. Pre and post-tests were completed by the caregivers and the results of this project was published in Journal of Pediatric Pulmonology.12 New education material regarding infection control and aerosol treatment were developed and shared with pw CF and their care givers.
Annual depression and anxiety screening of pw CF and their caregivers was started via standardized questionnaires. Results of 2 years of screening were published in Pediatric Pulmonology.13Patients with high level of depression and/or anxiety levels were referred to the child psychiatry department for evaluation and treatment. Depression and anxiety were found to be common in pw CF and their families. As a result of the CoV2 pandemic, three virtual resources were developed and shared with pw CF and their families: breathing exercises for children 3-12 years old, group virtual therapy for adolescent, and support group for the parents of children 0-3 years old.