scholarly journals Transcranial Doppler Screening Adherence Among Children with Sickle Cell Anemia Seen in the Emergency Department

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 3569-3569
Author(s):  
Julie K. Weisman ◽  
Carrie Diamond ◽  
Sarah Kappa ◽  
Robert Sheppard Nickel
Keyword(s):  
Emergency Department ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Transcranial Doppler ◽  
Clinic Visit ◽  
Cell Disease ◽  
Screening Guidelines ◽  
History Of ◽  
Screening Adherence

Abstract 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.

scholarly journals Targeted Education after an Emergency Department Visit Increases Hydroxyurea Initiation in Children with Sickle Cell Anemia

Blood ◽  
2017 ◽  
Vol 130 (Suppl_1) ◽  
pp. 868-868
Author(s):  
Sarah Kappa ◽  
Lydia Pecker ◽  
Deepika S. Darbari ◽  
Robert Nickel
Keyword(s):  
Emergency Department ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Conflicts Of Interest ◽  
National Health System ◽  
Clinic Visit ◽  
Acute Chest Syndrome ◽  
Hemoglobin F ◽  
History Of ◽  
One Year

Abstract 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 &lt;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&lt;0.0001) and hemoglobin F increased by a median of 5.8% (IQR +3.0, +11.3, p&lt;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.

Transcranial Doppler Screening Adherence among Children with Sickle Cell Anemia Seen in the Emergency Department

2020 ◽  
Vol 217 ◽  
pp. 172-176.e1
Author(s):  
Julie K. Weisman ◽  
Carrie E. Diamond ◽  
Sarah Kappa ◽  
Robert Sheppard Nickel
Keyword(s):  
Emergency Department ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Transcranial Doppler ◽  
Screening Adherence

Sickle Cell Disease

2018 ◽  
Author(s):  
Ann Ng ◽  
Erin S. Williams
Keyword(s):  
African American ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Sickle Cell Trait ◽  
Organ Damage ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Systemic Involvement ◽  
History Of

Sickle cell anemia (sickle cell disease) is a common hemoglobinopathy with anywhere from 90,000 to 100,000 Americans affected. This chronic condition has a predominance in populations of African descent, occurring in approximately 1 out of 365 African American births, compared to 1 out of 16,300 Hispanic births. The sickle cell trait can be detected in 1 of 13 African American births. One of the most common complications associated with sickle cell anemia, vaso-occlusive crises by sickled cells, results in severe pain. Other issues associated with this condition include acute chest syndrome, lung infections, end organ damage, and stroke. With improvements in the management and prevention of pain crises, infection, and other systemic involvement, these patients are living longer, thus increasing the potential for surgical needs. Whether it is for routine surgeries or surgeries that are due to the natural history of the disease; the pediatric anesthesiologist must be knowledgeable of the management of these patients in order to prevent morbidity and mortality.

Adherence to transcranial Doppler screening guidelines among children with sickle cell disease

2012 ◽  
Vol 60 (2) ◽  
pp. 270-274 ◽  
Author(s):  
Michael J. Eckrich ◽  
Winfred C. Wang ◽  
Elizabeth Yang ◽  
Patrick G. Arbogast ◽  
Anthony Morrow ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Transcranial Doppler ◽  
Cell Disease ◽  
Screening Guidelines

scholarly journals Investigation of Chest X-Ray Use in the Emergency Department in Pediatric Patients with Sickle Cell Disease Presenting with Fever Compared to Age Matched Controls

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 26-26
Author(s):  
Urania Dagalakis ◽  
Henna Butt ◽  
Natalie Davis ◽  
Regina A. Macatangay
Keyword(s):  
Emergency Department ◽  
Chest Pain ◽  
Sickle Cell Disease ◽  
Radiation Exposure ◽  
Sickle Cell ◽  
Pediatric Patients ◽  
Asthma Diagnosis ◽  
Inclusion Criteria ◽  
Cell Disease ◽  
History Of

Background In sickle cell disease (SCD), acute chest syndrome (ACS) is associated with prolonged hospitalization, increased risk of respiratory failure, future lung disease and 25% mortality in hospitalized patients(Bakshi, & Krishnamurti, 2017; Vinchinsky et al. 1997). Pediatric patients with SCD frequently present to the Pediatric Emergency Department (PED) with complaints of fever, chest pain, and cough, all of which may or may not be related to ACS. It is challenging for PED providers to determine which patients are at highest risk of ACS, so chest X-Rays (CXR) are frequently ordered which increases radiation exposure and healthcare costs. The objective of this study was to identify incidence of CXR performance, as well as ACS diagnosis, in SCD patients presenting to our PED with or without fever. Our goal was to identify significant clinical predictors of ACS in this population in order to implement a diagnostic algorithm for PED providers. Methods This was an IRB-approved retrospective medical record review of subjects diagnosed with SCD with inclusion criteria: ages 2-12 years, who presented to the University of Maryland PED between 2016-2018. We performed bivariate analyses comparing these variables between subjects who were febrile vs. afebrile on presentation to the PED, as well as those who were ultimately diagnosed with ACS compared to those who were not. Analysis of categorical variables was performed using Chi-square or Fischer exact test as appropriate. We performed a multivariable logistic regression model to identify significant predictors of ACS diagnosis. Analyses performed using SAS 9.4. Results We identified 424 SCD subjects who presented to our PED meeting inclusion criteria, with 25% (n=108) presenting with fever. Of these, 69% received a CXR on presentation vs. 42% of afebrile subjects (p=&lt;0.0001). In our febrile group 21% (n=23) patients had more than 2 febrile episodes and 100% received CXRs. There were no significant differences between the febrile and afebrile subjects when it came to sex, asthma diagnosis/comorbidity, hydroxyurea use, folic acid supplementation, or pneumococcal prophylaxis. Overall, 10% of patients presenting to the PED were diagnosed with ACS (n=42), made up of 13% of those presenting with fever vs. 9% of those presenting without fever. Those subjects ultimately diagnosed with ACS were significantly more likely to present with chest pain (p=0.003), tachypnea (p=0.001), and hypoxia (p&lt;0.0001), and were more likely to have a past history of asthma (p=0.0085). Sickle cell variant, home medications, and history of splenectomy were not significantly associated with ACS diagnosis. Upon multivariable modeling, when adjusting for fever and pre-existing asthma diagnosis, the only significant predictors of ACS diagnosis were chest pain and hypoxia. Patients without chest pain had an odds ratio (OR) =0.3 of ACS diagnosis [95% Confidence Interval, CI 0.14-0.67], indicating they had 70% lower odds of ACS compared to patients with chest paint. Patients without hypoxia had OR=0.12 of ACS compared to those with hypoxia [CI 0.06-0.25], indicating an 88% reduced odds of ACS diagnosis. Conversely, those with chest pain had 3.3x the odds of ACS diagnosis [CI 1.5-7.4] and those with hypoxia had 8.4x the odds of ACS diagnosis [CI 4-17.9] compared to those without these symptoms. Conclusion In ACS, current guidelines recommend that patients presenting with fever, hypoxia, tachypnea, tachycardia and abnormal respiratory exam findings should be treated empirically as well as receive a CXR. However radiological signs can be delayed compared to physical signs so a normal CXR does not preclude the diagnosis of ACS if there is clinical suspicion(Howard et al. 2015). Our data demonstrate that clinical findings such as chest pain, tachypnea and hypoxia were most likely to correlate to a diagnosis of ACS. While 69% of our febrile patients received a CXR in the PED, only 13% were ultimately diagnosed with ACS, indicating that more CXRs and radiation exposure occurred in the febrile population than may have been necessary. When adjusting for fever and asthma, the most notable predictors of ACS were hypoxia and chest pain. When present, these findings are significant predictors of ACS; when absent, subjects had significantly decreased odds of ACS. Incorporating the presence or absence of chest pain and hypoxia may help focus the use of CXR on the appropriate patient population. Disclosures No relevant conflicts of interest to declare.

scholarly journals Results from transcranial Doppler examination on children and adolescents with sickle cell disease and correlation between the time-averaged maximum mean velocity and hematological characteristics: a cross-sectional analytical study

2011 ◽  
Vol 129 (3) ◽  
pp. 134-138 ◽  
Author(s):  
Mary Hokazono ◽  
Gisele Sampaio Silva ◽  
Edina Mariko Koga Silva ◽  
Josefina Aparecida Pellegrini Braga
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Transcranial Doppler ◽  
Analytical Study ◽  
Hematological Parameters ◽  
Cell Disease ◽  
Mean Velocity ◽  
Cross Sectional ◽  
Group I

CONTEXT AND OBJECTIVE: Transcranial Doppler (TCD) detects stroke risk among children with sickle cell anemia (SCA). Our aim was to evaluate TCD findings in patients with different sickle cell disease (SCD) genotypes and correlate the time-averaged maximum mean (TAMM) velocity with hematological characteristics. DESIGN AND SETTING: Cross-sectional analytical study in the Pediatric Hematology sector, Universidade Federal de São Paulo. METHODS: 85 SCD patients of both sexes, aged 2-18 years, were evaluated, divided into: group I (62 patients with SCA/Sß0 thalassemia); and group II (23 patients with SC hemoglobinopathy/Sß+ thalassemia). TCD was performed and reviewed by a single investigator using Doppler ultrasonography with a 2 MHz transducer, in accordance with the Stroke Prevention Trial in Sickle Cell Anemia (STOP) protocol. The hematological parameters evaluated were: hematocrit, hemoglobin, reticulocytes, leukocytes, platelets and fetal hemoglobin. Univariate analysis was performed and Pearson's coefficient was calculated for hematological parameters and TAMM velocities (P < 0.05). RESULTS: TAMM velocities were 137 ± 28 and 103 ± 19 cm/s in groups I and II, respectively, and correlated negatively with hematocrit and hemoglobin in group I. There was one abnormal result (1.6%) and five conditional results (8.1%) in group I. All results were normal in group II. Middle cerebral arteries were the only vessels affected. CONCLUSION: There was a low prevalence of abnormal Doppler results in patients with sickle-cell disease. Time-average maximum mean velocity was significantly different between the genotypes and correlated with hematological characteristics.

Natural History of Blood Pressure in Sickle Cell Disease: Risks for Stroke and Death Associated with Relative Hypertension in Sickle Cell Anemia

1997 ◽  
Vol 102 (2) ◽  
pp. 171-177 ◽  
Author(s):  
Charles H. Pegelow, MD ◽  
Linda Colangelo, MS ◽  
Martin Steinberg, MD ◽  
Elizabeth C. Wright, PhD ◽  
Jeanne Smith, MD ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Sickle Cell Disease ◽  
Natural History ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Cell Disease ◽  
History Of ◽  
Disease Risks

scholarly journals To Give or Not to Give: RhD Immunoglobulin for an RHD*39 Pregnant Woman with Sickle Cell Disease

2021 ◽  
pp. 1-5
Author(s):  
Justin E. Juskewitch ◽  
Craig D. Tauscher ◽  
Sheila K. Moldenhauer ◽  
Jennifer E. Schieber ◽  
Eapen K. Jacob ◽  
...  
Keyword(s):  
Pregnant Woman ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Pregnancy Termination ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Childbearing Age ◽  
Procedural Complications ◽  
History Of ◽  
Chronic Hemolysis

Introduction: Patients with sickle cell disease (SCD) have repeated episodes of red blood cell (RBC) sickling and microvascular occlusion that manifest as pain crises, acute chest syndrome, and chronic hemolysis. These clinical sequelae usually increase during pregnancy. Given the racial distribution of SCD, patients with SCD are also more likely to have rarer RBC antigen genotypes than RBC donor populations. We present the management and clinical outcome of a 21-year-old pregnant woman with SCD and an RHD*39 (RhD[S103P], G-negative) variant. Case Presentation: Ms. S is B positive with a reported history of anti-D, anti-C, and anti-E alloantibodies (anti-G testing unknown). Genetic testing revealed both an RHD*39 and homozygous partial RHCE*ceVS.02 genotype. Absorption/elution testing confirmed the presence of anti-G, anti-C, and anti-E alloantibodies but could not definitively determine the presence/absence of an anti-D alloantibody. Ms. S desired to undergo elective pregnancy termination and the need for postprocedural RhD immunoglobulin (RhIG) was posed. Given that only the G antigen site is changed in an RHD*39 genotype and the potential risk of RhIG triggering a hyperhemolytic episode in an SCD patient, RhIG was not administered. There were no procedural complications. Follow-up testing at 10 weeks showed no increase in RBC alloantibody strength. Discussion/Conclusion: Ms. S represents a rare RHD*39 and partial RHCE*ceVS.02 genotype which did not further alloimmunize in the absence of RhIG administration. Her case also highlights the importance of routine anti-G alloantibody testing in women of childbearing age with apparent anti-D and anti-C alloantibodies.

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