Improving the Number of Quantity Not Sufficient [QNS] Specimens in Pediatric Cystic Fibrosis [CF] Patients’ Sweat Chloride Testing Christi Dodd, BSN, RN Bennie McWilliams, MD Cindy Burns-Gold 10/27/2017

Autosomal recessive gene A disorder of the exocrine glands of the body Etiology of CF Autosomal recessive gene A disorder of the exocrine glands of the body Manifested by chronic pulmonary disease pancreatic insufficiency abnormally high electrolyte concentration in sweat sometimes abnormalities of other organs

CF is a Multisystem Disorder Pulmonary Sinus Hepatobiliary Exocrine pancreas Endocrine pancreas Rheumatic Reproductive Increased amount of salt in the sweat.

Sweat Test (“Gold Standard”) CF Diagnostic Testing Sweat Test (“Gold Standard”) <29 mmol/L (negative) = CF is very unlikely 30 - 59 mmol/L (intermediate) = CF is possible >60 mmol/L (positive) = CF is likely to be diagnosed Genetic Testing Newborn screen (40 panel mutation screen) Expanded genetic testing Other testing used to assist in the diagnosis of cystic fibrosis can include testing called Nasal potential differential. Absence of the vas deferens can also be included in the differential diagnosis of cystic fibrosis in males.

CF Sweat Testing Difficult to collect minimum amount of sweat specimen making test difficult to perform CF Foundation has standardized guidelines on performing sweat tests Sweat tests should be performed at accredited CF Centers According to the CF Foundation guidelines, Quantity Not Sufficient [QNS] rates should not exceed: <10% for patients < 3 months <5% for patients > 3 months (LeGrys, McColley, Li, & Farrell, 2010)

Sweat QNS Quality Improvement [QI] Background The 2014 CF registry report showed QNS samples 20% for patients < 3 months 9% for patients > 3 months A multidisciplinary QI team was formed to address elevated QNS rates CF center director CF nurse coordinator Lab manager The aim of the QI project To decrease the percentage of QNS sweat chloride tests to meet CFF guidelines. CF Registry is a national data base of that we use in our QI projects. A patient registry is a collection of observational data used to evaluate specified outcomes for a specific patient population defined by condition, disease, exposure, etc. serving an expressed purpose. In this case, we use the registry to assist us in directing our QI program.

Sweat QNS QI – Data Analysis The QI team met to review the preliminary data and processes. Initial observations by this team were that the inpatient sweat test tests were the primary drivers for the high QNS rate. Issues observed in the inpatient sweat testing were: Sweat samples collected in multiple inpatient units. No standardized inpatient collection protocol. There was a lack of ongoing supervision during the collection process by laboratory personnel and the unit staff were untrained. Inpatient studies were often performed on patients under suboptimal medical conditions. Repeat studies were often attempted on the same patients with little to no clinical changes. Additionally it was found that sweat test results were not consistently sent to program director for review. Patients transferred from one unit to another unit were being retested and receiving multiple QNS results within the same hospitalizations. Inpatient testing lacked supervision during the testing unlike the outpatient setting resulting in higher QNS rates. Many QNS rates resulted from inpatient testing performed on patients that were dehydrated (requiring IV fluids).

Sweat QNS QI - Actions As a result, the following actions were made: The QI Team developed a standardized specific protocol for inpatient sweat testing which included: Standardized collection methods NICU and PICU sweat tests require program director approval All repeat sweat tests require program director approval The QI team met with managers of inpatient units to explain and disseminate the standardized protocol. Inpatient checklist created and implemented in Dec 2016 for use with every inpatient sweat test. A system for securely emailing all results to the center director was developed.

Sweat QNS QI - Actions Inpatient checklist Specific patient condition requirements Room temperature Supervision Patient Sweat Test Preparation Instruction sheet for test preparation Testing process explanation

QNS Rates

Sweat QNS QI – Ongoing Monitoring Quarterly review of data in the CF Center QI meetings. The data will be formally reported quarterly until 2 successive quarterly periods with QNS rates below the threshold then formal review of data changes to annual basis.

References LeGrys, V.A., McColley, S.A., Li, Z., & Farrell, P.M.(2010). The need for quality improvement in sweat testing infants after newborn screening for cystic fibrosis. J Pediatr.157(6),1035-7. Epub 2010 Sep 16.PMID: 20843526