Participation in a clinic to specifically address starting hydroxyurea after a SCA complication increases hydroxyurea use.
Background: Annual transcranial doppler (TCD) screening is strongly recommended for patients with sickle cell anemia (SCA) between the ages of 2 to 16 years to identify children at highest risk for stroke. Implementation of this screening and treating identified patients with chronic transfusion has decreased the incidence of overt stroke. Nonetheless, adherence to TCD screening guidelines is poor and many children with SCA do not receive an annual TCD. The purpose of this study is to evaluate adherence to TCD screening among a cohort of patients with SCA seen in the emergency department (ED) for an acute problem. Previous work has demonstrated that SCD-related outpatient visits are important "missed opportunities" for TCD screening. We hypothesized that ED encounters also represent potential opportunities to identify patients in need of TCD screening who do not attend clinic regularly. Methods: We conducted a retrospective chart review of the medical records of all patients with sickle cell disease (SCD) seen in the ED at a large, urban pediatric institution between February 2016 and April 2017. Patients were identified using an ED clinical registry that includes all ED patient encounters. We excluded patients who do not need TCD screening (sickle cell disease genotypes other than SS and Sβ0 thalassemia, age <2 or >16 years, on chronic transfusions, history of hematopoietic stem cell transplant). We also excluded patients documented to previously have inadequate TCD bone windows and patients who did not receive their regular hematology care at the study institution. For eligible patients who had multiple ED encounters during the study period, data was extracted at the time of the first ED encounter during the study period. Eligible patients who had received a TCD in the last year (adherent to TCD screening) were compared to patients who had not received a TCD in the last year (nonadherent to TCD screening). Categorical data was analyzed with the chi-square test. Continuous data was analyzed using the two-sample t-test. P value of <0.05 was considered statistically significant. Results: During the 64 week study period, 739 patients with SCD were seen in the ED. A total of 482 patients were excluded for the following reasons: non-SCA genotype (n=164), age (n=139), followed at outside institution (n=129), chronic transfusion (n=38), prior TCD window problem (n=10), history of transplant (n=2); leaving 257 patients with SCA aged 2-16 years for study. Among this study group, 63 patients (25%) had not received a needed TCD in the last year, including 19 patients (7%) who had never had a TCD. When excluding patients aged 2-2.99 years (n=33) in whom a first TCD may have been planned soon after the ED encounter, a similar proportion of patients still had not received a TCD in the last year (53/224, 24%) but a slightly smaller proportion had never had a TCD (9/224, 4%). Patient age and sex were not associated with TCD screening adherence (p>0.7). Patients adherent to TCD screening were more likely to be taking hydroxyurea (67% vs. 29%, p<0.0001). A recent hematology clinic visit was significantly associated with TCD screening adherence. All patients adherent to TCD screening had a clinic visit in the last year compared to 75% of nonadherent patients, p<0.0001. The mean interval time since the last hematology clinic appointment from the ED encounter was greater for TCD nonadherent patients, 70 vs. 270 days p<0.0001 (Figure). Conclusion: Patients with SCA who present to the ED and are nonadherent to TCD screening guidelines are less likely to have had a recent hematology clinic visit. Therefore, the ED may be an important location for identifying patients lost to regular clinic follow-up in need of a TCD. An intervention that specifically targets this patient population will likely improve TCD screening rates and stroke prevention. Figure. Figure. Disclosures No relevant conflicts of interest to declare.
Introduction: Hydroxyurea decreases many complications of sickle cell anemia (SCA) but is underused in treatment-eligible patients. Barriers to hydroxyurea initiation occur on the health care system, provider, and patient level. Novel strategies to increase hydroxyurea use in patients with SCA are needed. To address this challenge at our center, we implemented the Quick Start Hydroxyurea Initiation Project (Q-SHIP). Methods: Patients with SCA were eligible to participate in Q-SHIP if they presented to the Children's National Health System (CNHS) emergency department (ED) for pain or acute chest syndrome and were not taking hydroxyurea. Patients <9 months old, on chronic transfusions, pregnant, or not followed by CNHS hematology were excluded. Eligible patients were referred to a weekly Q-SHIP clinic visit focused on hydroxyurea education and were offered initiating treatment at the visit's conclusion. Participants completed a pre-session questionnaire, discussed hydroxyurea with a hematologist using a handbook developed by CNHS, and watched videos featuring patients and parents of children with SCA sharing their experience with hydroxyurea. Subjects were classified as starting hydroxyurea if they had a clinic visit for hydroxyurea monitoring within 3 months of participation in a Q-SHIP session. Results: Over 13 months (2/1/2016 - 3/31/2017) 65 eligible patients participated in Q-SHIP a median of 5 days (IQR 2, 20 days) after ED or hospital discharge. Although 44% (28/64) of participants reported no previous hydroxyurea offer, provider clinic documentation indicated that 61% (17/28) of these families had declined a previous hydroxyurea offer. After Q-SHIP, 55% (36/65) of participants started hydroxyurea. Subjects who started hydroxyurea after Q-SHIP were similar to those who did not, except subjects who started were more likely have a history of an intensive care unit admission (Table 1). After a median follow-up of 11 months, 81% (29/36) of participants who started hydroxyurea after Q-SHIP continued on therapy. Among Q-SHIP participants continuing treatment, mean corpuscular volume increased by a median of 8.6 fL (IQR +5.4, +17.7, p<0.0001) and hemoglobin F increased by a median of 5.8% (IQR +3.0, +11.3, p<0.001). One year after implementation of Q-SHIP, the proportion of treatment-eligible patients with SCA who presented to the ED with pain or ACS who were receiving hydroxyurea increased; February 2016: 56% (32/57) vs. February 2017: 73% (43/59), p=0.059. Conclusion: Addressing indications for hydroxyurea therapy in a clinic encounter exclusively for this purpose soon after a SCA complication is a meaningful time to meet with families of children with SCA to initiate treatment. Disclosures No relevant conflicts of interest to declare.
Background: Hydroxyurea is daily oral medication proven to decrease complications of sickle cell disease (SCD). While concerns have been raised about the safety of hydroxyurea, it is now generally viewed as a well-tolerated medication for SCD. The primary toxicity of hydroxyurea that requires holding of treatment is reversible cytopenia. Due to its classification as a chemotherapeutic agent and safety concerns regarding inappropriate chemotherapy ordering, hydroxyurea can only be ordered by "chemotherapy-certified" providers at some hospitals. At our hospital system, pediatric resident physicians were restricted from ordering hydroxyurea. Instead of being a part of a resident's hospital admission orders, hydroxyurea for inpatients had to be ordered separately by a hematology fellow or attending physician. In June 2016 our hospital changed its policy to allow residents to order hydroxyurea for patients with SCD admitted to the hospital who were already on hydroxyurea at home. We hypothesized that this change in policy to allow residents to order hydroxyurea would increase the proportion of patients with SCD appropriately receiving their home hydroxyurea by hospital day 1. We also hypothesized that this policy change would not result in an increase in the proportion of patients inappropriately receiving hydroxyurea when it should have been held based on the admission complete blood count (CBC). Methods: We conducted a retrospective review of the medical records of a random sample of patients admitted to the hematology service the year before (2015) and the year after (2017) the policy change in 2016. Patients were eligible for study if they were admitted to the hematology service and were taking hydroxyurea as documented by a clinic note within the last three months. Patients were excluded if they were admitted to the intensive care unit or for surgery. Patients were also excluded if discharged on hospital day 0 or 1. Institutional guidelines advise holding hydroxyurea if any of the following: absolute neutrophil count <1250/µL; platelet count <80K/µL; reticulocyte count <100K/µL, unless hemoglobin >8.0 gm/dL. Hydroxyurea was classified as "inappropriately given" if a patient received hydroxyurea despite having an admission CBC value below a hold parameter. Hydroxyurea was classified as "appropriately not given" if a patient did not receive hydroxyurea when having a CBC value below a hold parameter. Patients who were on hydroxyurea who never received hydroxyurea inpatient with CBC values above the hold parameters were classified as "inappropriately not given." Patients admitted in 2015 (before resident ordering) were compared with patients admitted in 2017 (after resident ordering) using a chi-square test or Fisher exact test. Results: In total, 217 hospitalizations of eligible patients were reviewed: 91 before the policy change and 126 after the policy change. Based on the admission CBC, hydroxyurea should have been held for 8 patients. Excluding these patients who should not have received hydroxyurea, patients after the policy change were significantly more likely to have received their home hydroxyurea by hospital day 1: before 62/90 (69%) vs. after 105/119 (88%), p=0.0005. The proportion of patients who inappropriately received hydroxyurea was very low in both groups: before 1/91 (1%) vs. after 3/126 (2%), p=0.64. No serious adverse clinical events occurred from this "inappropriate" administration of hydroxyurea. The figure graphically displays the proportion of patients in the two groups who: appropriately received on hospital day 0/1/2+, inappropriately did not receive, appropriately did not receive, and inappropriately received hydroxyurea. Conclusion: Resident ordering of home hydroxyurea for hospitalized patients with SCD appears to be safe. Policies that permit residents to order hydroxyurea as part of a patient's admission orders can help increase the proportion of patients who receive this important medication while inpatient. Figure. Figure. Disclosures No relevant conflicts of interest to declare.
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