Psychometric properties of the Mini-Balance Evaluation Systems Test and physical function measures in patients with Ehlers-Danlos syndrome/hypermobility spectrum disorders.

PURPOSE: Ehlers-Danlos syndrome (EDS) and hypermobility spectrum disorders (HSD) are associated with impairments in balance and physical function. However, the psychometric properties of relevant outcome measures remain largely unexplored. The objectives of this study were to evaluate the construct validity of the Mini-Balance Evaluation Systems Test (Mini-BESTest) alongside the test-retest reliability of the Mini-BESTest, Six Minute Walk Test (6MWT), and Lower Extremity Functional Scale (LEFS) in patients with the hypermobility subtype of EDS (hEDS) and HSD. MATERIALS AND METHODS: Participants with hEDS/HSD (n = 20) attended two visits scheduled one to two weeks apart. The construct validity of the Mini-BESTest was determined through Pearson correlations between force plate balance measures, 6MWT, and LEFS. Test-retest reliability of the measures was evaluated through intraclass correlation coefficients (ICC). Minimal detectable change values with 95% confidence (MDC95) were also calculated. RESULTS: Mini-BESTest demonstrated significant correlations with force plate measures, 6MWT, and LEFS (r = -0.41 to 0.66). Test-retest reliability was excellent for the Mini-BESTest, 6MWT, and LEFS (ICC = 0.91 to 0.96). MDC95 was 4 for the Mini-BESTest, 77 m for the 6MWT, and 11 for the LEFS. CONCLUSION: The Mini-BESTest is valid and reliable for assessing balance and mobility in patients with hEDS/HSD.IMPLICATIONS FOR REHABILITATIONThe Mini Balance Evaluation Systems Test (Mini-BESTest) is valid in capturing aspects of balance and physical function in patients with hypermobile Ehlers-Danlos syndrome or hypermobility spectrum disorders.However, the Mini-BESTest may show a potential ceiling effect for high functioning patients in this population.The Mini-BESTest, 6 Minute Walk Test, and the Lower Extremity Functional Scale also show excellent test-retest reliability in this population.The Minimal Detectable Change with 95% confidence intervals is 4 for the Mini-BESTest, 77 m for the 6 Minute Walk Test, and 11 for the Lower Extremity Functional Scale in this population.

Interdisciplinary Quality Improvement Project Increases Vitamin D Supplementation in Infants.

BACKGROUND: American Academy of Pediatrics guidelines recommend 400 IU of vitamin D supplementation daily for certain infants <1 year of age. We aimed to increase the proportion of reported appropriate vitamin D supplementation for infants born at our institution and those who followed up in our resident clinic through 6 months from 49% to 80% over 24 months. METHODS: Our interdisciplinary quality improvement effort included vitamin D medication delivery before nursery discharge and family and staff education. The process measure was the percentage of families discharged from birth hospitalization with vitamin D and teaching. The outcome measure was the percentage of families reporting appropriate vitamin D supplementation at 2-, 4-, and 6-month well child visits. The balancing measure was the percentage of infants discharged from the nursery by 2 pm. Data were displayed on Statistical Process Control p charts and established rules for detecting special causes were applied. RESULTS: Baseline and improvement data were collected for 587 hospital discharges and 220 outpatient encounters. The percentage of families discharged with vitamin D increased from 24.8% to 98% from 2016 to 2018. Percent of families reporting appropriate vitamin D supplementation at well child visits increased from 49% to 89% from 2016 to 2018. Overall, the percentage of discharges by 2 pm remained stable at 60%. CONCLUSION: Bedside medication delivery and education in the newborn nursery improved reported vitamin D supplementation rates in the first 6 months of life. The intervention did not delay newborn hospital discharge.

The Impact of a Clonidine Transition Protocol on Dexmedetomidine Withdrawal in Critically Ill Pediatric Patients.

OBJECTIVES: This study describes our experience with a clonidine transition protocol to prevent dexmedetomidine (DEX) withdrawal in critically ill pediatric patients. METHODS: Retrospective review of electronic medical records of patients in the pediatric intensive care unit of a single tertiary children's hospital. All patients up to 19 years of age, who received concomitant DEX infusion and enteral clonidine between June 1, 2016, and May 31, 2018, were included. RESULTS: Two of 24 encounters had DEX restarted for withdrawal (8.3%). Five of 14 encounters who were transitioned to clonidine 2 mcg/kg every 6 hours required an increased dose, and 1 of 10 encounters transitioned to clonidine 4 mcg/kg every 6 hours required an increased dose (36% vs 10%, p = 0.21). For encounters with clonidine dose increases, 5 of 6 had improvements in Withdrawal Assessment Tool-1 (WAT-1) scores. Of these 5 encounters, 4 had decreasing or stable opioid and sedative requirements and 1 was transitioned to methadone. No encounters required discontinuation of clonidine owing to adverse events. Two of 24 encounters met our safety endpoint. One received a fluid bolus during the clonidine transition with no change in clonidine dosing, while the other had clonidine dose decreased for asymptomatic bradycardia. CONCLUSIONS: The 24 encounters in our retrospective study add to the limited literature available to describe dosing, initiation time, and duration of clonidine to prevent withdrawal from DEX in critically ill pediatric patients. Further research is needed to clarify the optimal dosing and duration of clonidine to prevent DEX withdrawal in pediatric patients.

Pediatric Patient-Centered Transitions From Hospital to Home: Improving the Discharge Medication Process.

OBJECTIVES: Medications prescribed at hospital discharge can lead to patient harm if there are access barriers or misunderstanding of instructions. Filling prescriptions before discharge can decrease these risks. We aimed to increase the percentage of patients leaving the hospital with new discharge medications in hand to 70% by 18 months. METHODS: We used sequential plan-do-study-act cycles from January 2015 to September 2016. We used statistical process control charts to track process measures, new medications filled before discharge, and rates of bedside delivery with pharmacist teaching to the inpatient pediatric unit. Outcome measures included national patient survey data, collected and displayed quarterly, as well as caregiver understanding, comparing inaccuracy of medication teach-back with and without medications in hand before discharge. RESULTS: Rates of patients leaving the hospital with medications in hand increased from a baseline of 2% to 85% over the study period. Bedside delivery reached 71%. Inaccuracy of caregiver report during a postdischarge phone call decreased from 3.3% to 0.7% (P < .05) when medications were in hand before discharge. Patient satisfaction with education of new medication side effects increased from 50% to 88%. CONCLUSIONS: By using an engaged interprofessional team, we optimized use of our on-site outpatient pharmacy and increased the percentage of pediatric patients leaving the hospital with new discharge medications in hand to >80%. This, accompanied by increased rates of bedside medication delivery and pharmacist teaching, was associated with improvements in caregiver discharge-medication related experience and understanding.