Abstract
Introduction
Sickle Cell Disease (SCD) is the most common genetic disease identified in newborns in the United States. It results from a single amino acid substitution in the beta globin protein. This results in severe chronic complications, long-term end-organ damage and reduced life expectancy.
Evidence Based Health Maintenance (HM) recommendations (HMR) provide the best framework for reducing chronic complications of SCD. Many barriers to healthcare utilization exists for patients with SCD and patients are often unable to access HMR. The decrease in comprehensive care often leads to poor compliance and outcomes. To address this we established a novel patient centered comprehensive SCD clinic (CSCDC).
Methods
CSCDC occurs the third Wednesday of every month. Sub-specialists commit to CSCDC and subspecialty clinic slots are reserved. Available services: Cardiology, Radiology, Ophthalmology, Pulmonology, Nephrology, Social Work, Immunizations. Other services are available as needed. Lunch and group patient education are provided.
There are 188 SCD patients in our program (the cohort). This includes HbSS, HbSC and HbSB thalassemia only. Patients electronic charts are reviewed to determine HM needs. Families are contacted by phone 4-6 weeks prior to the scheduled CSCDC. The HM needs are reviewed with families. The family has an opportunity to adjust work, school and other personal commitments in advance of the clinic date. Family and SCD clinic commits to the CSCDC date. Clinic flexibility is allowed.
Electronic medical records and Qlik Sense Software (Qlik ,Randor PA) were used to determine visit numbers and clinic no show rates (NSR). Patient initiated same day cancellations are included in the NSR. NSR date range was January 2016-July 2018. First we determined NSR for all SCD related clinic visits for the cohort (NSR-SCD). Then we determined NSR for the cohort on CSCDC dates only (NSR-CSCDC). We then divided the cohort into three groups based on NSR-SCD. Low, middle and high NSR. We then compared NSR-SCD and NSR-CSCDC for each group. The two proportion Z test was used to calculate p values
Volunteers unrelated to CSCDC contacted patients by telephone. Prior IRB approval was obtained. Family participation was voluntary and limited to families with CSCDC attendance. Parents or patients (18 years and older) were asked to answer questions. The questions evaluated 1.Patient overall satisfaction with CSCDC, 2.Likely to return to CSCDC, 3.Likely to recommend CSCDC and 4.Did CSCDC improve the patients individual SCD management.
CSCDC dates Aug 2015-July 2018 were used to select the sub-cohort of patients that attended two or more CSCDC clinics. We determined if the HM evaluations occurred according to published guidelines.
Results
There was a statistically significant decrease in NSR for patients attending CSCDC vs the general SCD clinic. The cohort consisted of 188 patients, 1243 clinic visits and 322 no shows(NS). In CSCDC there were 134 patients, 182 clinic visits and 18 NS. NSR-SCD vs NSR-CSCDC was 25.9% vs 9.9% (p 0.000002).(Table 1)When the cohort was divided into NSR subgroups. NSR decrease was most significant for the high NSR sub group.For high and middle NSR, NSR-SCD vs NSR-CSCDC were 50.8% vs 28.8% (p 0.002759) and 19.8% vs 4.2% (p 0.001187) respectively.(Table 2)The low subgroup had 0% NSR. Patients with the historically highest NSR had significant decrease in their NSR during CSCDC.
There were 170 phone contacts and 122 responses for a 72% response rate. Families were asked to grade their answers in a range of 1-5 with 5 being the highest (top box). Results: 86%, 91% and 92% top box scores for CSCDC patient reporting "extremely satisfied" with CSCDC, "extremely likely" to recommend CSCDC and "extremely likely" to return to CSCDC respectively. 97% responded "yes" that CSCDC improved their individual family SCD care.
CSCDC increased adherence to standard HMR. Total patient visits for the time range was 236. There were 56 patients (24%) who attended at least 2 CSCDC. 62% of these repeat attendees received health maintenance evaluations within 4-8 weeks of the recommended time.
Conclusion
Our CSCDC demonstrates increase in SCD patient compliance with clinic visits, high patient satisfaction and high level of HM adherence to standard. We believe this is an effective model for SCD comprehensive care and this data may be useful to initiate evaluation of a larger patient cohort.
Disclosures
No relevant conflicts of interest to declare.
Hydroxyurea use in sickle cell disease: the battle with low prescription rates, poor patient compliance and fears of toxicities
