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.