Methods:
A variety of outcome measures were considered in the development of this quality improvement project. The tests that were considered included the Peabody (PDMS), Test of Gross Motor Development (TGMD), Movement Assessment Battery for kids (MABC) and BOT-2 (see Table 3 )(11). Ultimately, the BOT-2 was selected for the following reasons: 1) The BOT-2 is norm referenced, valid and reliable (8), 2) the age range of the test met the goal to assess comprehensive motor skills in school aged children and 3) the information that the BOT-2 provides is specific and relevant to the skills that are necessary for participation in school, recreational and peer activities. The BOT-2 also includes specific strength and balance sections, which are skill areas that may be impacted in children with CF due to gene expression in skeletal muscle, or side effects of medications on the vestibular system.
Once BOT-2 was selected as the screening tool, children between 4 and 12 years of age with a diagnosis of CF based on a sweat chloride ≥60mEq/L and/or the presence of two known disease-causing variants in the CF gene were eligible to participate in this quality improvement project. An algorithm was developed to guide timing of the BOT-2 assessment in CF clinic and follow up recommendations (Figure 1 ). Eligible children were identified in pre-clinic rounds. In an effort to identify a clinic visit where the number of additional assessments (i.e., labs, imaging) were not needed, we targeted the half birthday visit for the BOT-2 evaluation. To increase the number of BOT-2 assessments over the year, the BOT-2 assessment was performed at the end of a hospitalization in a small subset, with historically infrequent outpatient clinic visits, when acute symptoms of a pulmonary exacerbation were improved. Parents were educated on the purpose of the assessment and observations of their child’s performance and follow-up PT recommendations were made based on the results.
The BOT-2 requires a testing kit and scoring booklet, which costs $685(11). The equipment that is needed for the gross motor sections are a BOT-2 balance beam, a line on the floor, a tape measure, two cones, a scoring sheet, and a scoring booklet. An exercise mat can also be used for some of the strength tests but is not required. Testing was performed by two trained, board-certified PTs (only 1 PT required to do each test), and typically took 25-30 minutes to complete the 26 gross motor tasks to compile a full gross motor BOT-2 score. Children can perform the test in a clinic room or gym space, but testing does require a 50 foot shuttle run course. PTs found it helpful to partner with other CF care team members during clinic when BOT-2 testing was performed. With child and parent permission, PT would take a child to the gym to complete testing while other care team members (i.e., social worker, psychologist or dietitian) met with the child’s parents. Separating children and parents was not a barrier to completing testing, as children generally expressed excitement about participating in BOT-2 testing during their clinic visit.
Data collected during the BOT-2 evaluation included scale scores, standard scores, percentile rankings, descriptive categories (well below average, below average, average, above average, well above average), and the total motor composite (TMC), an average of all subsections of the test. As defined by the BOT-2 tool, a percentile ranking of less than 18% was considered below average (3 to 17%) (10). A percentile ranking of less than 25% was also recorded since this qualifies a child in Colorado for school district and state services. Scale scores are assigned for the following components of the BOT-2: bilateral coordination, balance, running speed and agility and strength with normal scale score values defined as 11 to 19. Standard scores (normal values 41-59) and percentile score are given for body coordination (bilateral coordination plus balance) and strength and agility (running speed and agility plus strength). The descriptive categories of below average and well below average (<2%) indicate impairment. We utilized these scores and rankings to then customize individual exercise plans and follow up.
Our CF center clinical database was utilized to collect demographic data for all participants including age, sex, BMI, number of lifetime hospitalizations (defined as a hospital stay of 3 or more days), CF genotype, lung function including forced expiratory volume in 1 second (FEV1) and forced vital capacity (FVC) and the presence of any of the following comorbidities: CF related diabetes, type I diabetes, asthma, Gastroesophageal Reflux Disease, history of intestinal surgery, anxiety, depression, Attention Deficit Hyperactivity Disorder, toe walking, gastrostomy tube, and other miscellaneous conditions (scoliosis, short gut, migraines, congenital heart disease). This quality improvement project was approved by Children’s Hospital Colorado Organization Research Risk & Quality Improvement Review Panel (ORRQIRP).