Cystic Fibrosis Core Strengthening and Respiratory Exercise Program
Condition: Cystic Fibrosis
MeSH terms: Cystic Fibrosis; Fibrosis
Study type: Interventional
Phase: Phase 2

Summary: 
      The purpose of this project is to determine the short-term effects of a customized Core
      Strengthening and Respiratory Exercise Program (CSREP) on children with cystic fibrosis (CF)
      between the ages of 10 and 21 who are receiving outpatient care at Children's Hospitals and
      Clinics of Minnesota. The CSREP, which will be provided by a physical therapist and a
      physical therapist assistant, consists of specific breathing techniques and core
      strengthening exercises, designed to improve rib cage mobility, pulmonary function, aerobic
      capacity, posture, and core strength. Currently the CF population at Children's receives
      physical therapy on an inpatient basis only.

      The overall goal of this program is to prove the viability of an outpatient exercise program
      for this population. Specific aims include:

        -  To customize a CSREP protocol per each patient

        -  To measure patient outcomes at baseline and six months

        -  To develop a satisfaction tool in order to measure patient experience and satisfaction
    

Detailed description: 
      Study Design: Phase II clinical trial.

      Objective: To determine whether a customized physical therapy exercise intervention titled
      Core Strengthening and Respiratory Exercise Program (CSREP) clinically improves the following
      outcome measures for children between the ages of 10 and 21 with cystic fibrosis: pulmonary
      function, aerobic capacity, rib cage mobility, posture, and core strength, thus warranting
      more extensive study and development.

      Background: Previous research conducted in pediatric populations has focused on the effects
      of aerobic training, anaerobic training, and strengthening of the extremities on the health
      of children with cystic fibrosis. However, the disease process of cystic fibrosis secondarily
      causes impairments in the musculoskeletal and nervous systems. No intervention study aimed at
      positively impacting these systems has been done to date with children with cystic fibrosis.

      Methods: A 6-month CSREP will be customized to meet the specific needs of each participant.
      During Part I of the program participants will attend weekly CSREP treatments for 4 to 6
      weeks; in Part II they will attend monthly CSREP treatments over a five-month period. Each
      session will last for 45-60 minutes. During these treatments, participants will be given
      instruction on a daily home exercise program and will receive hands on physical therapy
      interventions. Handouts with pictures and how-to instructions for assigned exercises will be
      provided. Participants will be given calendars to track completion of home exercises and
      level of compliance. During Part II, a researcher will call participants every week in order
      to encourage compliance with the home exercise program and to provide clarification as
      needed. All participants will continue to receive regular medical care as well as PT
      treatments.

      A respiratory therapist will perform the pulmonary function tests, a respiratory therapist
      along with an exercise physiologist will perform the VO2max testing, and a physical therapist
      will perform the physical therapy evaluation, including assessment of rib cage mobility, core
      strength, and posture. Another physical therapist as well as a physical therapist assistant
      will provide the physical therapy treatments, instruct the participants in their home
      program, and make the weekly phone calls to all participants.

      An experience survey will be designed to evaluate both satisfaction on a scale of 1 to 10 and
      self scoring of outcome measures including: respiratory function, cough strength, activity
      tolerance, posture, and strength on a three point Likert scale of: 1) Got worse; 2) Stayed
      the same; and 3) Got better. Additionally, the survey will include a question on the
      likelihood of continuing with the home program after the study has ended. The survey will be
      administered at 6 months upon completion of the program.

      Relevance: The results of this inquiry as to the viability of the CSREP will prove relevant
      to the above-mentioned patient outcomes within the cystic fibrosis population. The customized
      CSREP protocol will provide a non-pharmacological outpatient treatment option that can be
      completed on an outpatient basis, independently, in the patient's home.
    

Intervention name(s): Physical Therapy
Intervention type(s): Other
Intervention description(s): Treatment
Intervention model: Single Group Assignment
Masking: None (Open Label)
Primary purpose: Supportive Care
Keywords: CF