Hematology Commons^™

The Role Of Social Determinants Of Health In Hydroxyurea Adherence Among Persons With Sickle Cell Disease: A Systematic Review, Doreen O. Ugwu, Lori L. Vick, Terika Williams, Aurela Nikaj, Seyi Omeh, Benjamin Becerra Dr., Tilicia Mayo-Gamble

Journal of the Georgia Public Health Association

Objective: Georgia has one of the highest prevalences of sickle cell disease (SCD). For Georgians living with SCD, inadequate medication adherence is associated with important clinical outcomes. One important area that is understudied in SCD is the impact of social determinants of health (SDoH) on medication adherence. A foundational understanding of SDoH is needed to introduce, inform and carry out programs and interventions designed to facilitate hydroxyurea adherence through the state of Georgia.

Method: We conducted a systematic literature review to synthesize the literature on SDoH addressed in studies to improve hydroxyurea adherence in persons with SCD. SDoH were based …

Low-Dose Aspirin For Preventing Intrauterine Growth Restriction And Pre-Eclampsia In Sickle Cell Pregnancy, Lydia Pecker, Sophie Lanzkron

Cardeza Foundation for Hematologic Research

No abstract provided.

National Landscape Of Pediatric And Adult Inpatient Sickle Cell Disease Care, Ashley M. Jenkins, Spoorthy Vangala, Matthew Mcintosh, Robert Legros, Sophie Lanzkron, Kevin Fiscella

Department of Medicine Faculty Papers

No abstract provided.

Sickle Cell Disease, Children's Health School Services

School Guides

Educator guide about how to support students with sickle cell disease.

Ash Sickle Cell Disease Cpkd Guidelines Report, Robert I. Liem, Madeleine Verhovsek, Ted Wun, Sophie Lanzkron

Cardeza Foundation for Hematologic Research

No abstract provided.

Creation And Evaluation Of Standardized Electronic Health Record Templates For Transition Of Care In Sickle Cell Disease: A Pilot Study, Stephanie Howe Guarino, Mohan Madisetti, Payal C. Desai, Akshat Jain, Kenneth Rivlin, Jerome A. Osheroff, Robin Miller, Brandi Campanella, Sophie Lanzkron, Julie Kanter

Cardeza Foundation for Hematologic Research

Objective The objective of this pilot project was to create a standardized Transition Note in the electronic health record (EHR) to facilitate clinician communication during the transition process from pediatric to adult care for youth with sickle cell disease (SCD). Methods Using a modified Delphi process, consensus was reached among 78 member centers from the National Alliance of Sickle Cell Centers (NASCC) on the essential health data elements for a standardized EHR Transition Note. Templates were created in 2 different EHR systems and piloted at 2 SCD centers. Perceived clinician acceptability, appropriateness, and feasibility were assessed using the AIM-IAM-FAM measures …

Acute Pain Treatment In Patients With Sickle Cell Disease Transitioned To Buprenorphine: Evidence Of Equivalent Pain Relief, Christopher P. Carroll, Elizabeth J. Prince, Ashley P. Lauriello, Lydia H. Pecker, Sophie M. Lanzkron

Cardeza Foundation for Hematologic Research

Objectives Buprenorphine is a novel opioid analgesic with complex receptor pharmacology. It is safer than high dose chronic treatment with full agonist opioids. However, buprenorphine can inhibit conventional opioids, complicating acute pain treatment. Methods We compare acute pain treatment outcomes in 55 sickle cell disease patients in the year before and after transition to buprenorphine. Infusion center visits, ED visits, and hospital admissions were compared before and after transition, and pain outcomes were compared using linear mixed effects models with patients entered as random effects to account for differential contribution to the outcomes by visit rates. Results Acute care visits …

The Development Of Consensus Recommendation To Improve Practice Harmonization For Sickle Cell Disease Through The National Alliance Of Sickle Cell Centers, Julie Kanter, Melissa Frei-Jones, Deepa Manwani, Marsha Treadwell, Mohan Madisetti, Robin E. Miller, Seethal A. Jacob, Sana Saif-Ur-Rehman, Andrew O'Brien, Sophie Lanzkron

Cardeza Foundation for Hematologic Research

Introduction SCD remains the most common inherited blood disorder. Due to a lack of strong evidence, the management of sickle cell is often anecdotal, local to individual centers, states, and countries. Even in areas of practice with high-level data to improve practice, implementation has not been consistent. The historical lack of an agreed-upon national registry has compounded this problem and resulted in a lack of comparative effectiveness data to identify areas of deficiency and improve practice. These barriers have resulted in centers developing local policies and procedures, often with limited communication with other centers, resulting in disparate and inconsistent care. …

Neurocognitive Gains Among Ugandan Children With Sickle Cell Anemia On Hydroxyurea: 18-Month Trial Interim Results, Shubaya Kasule Naggayi, Dennis Kalibbala, Vincent Mboizi, John M. Ssenkusu, Zhezhen Jin, Caterina Rosano, Deogratias Munube, Bill Wambaka, Ruth Namazzi, Robert Opoka

Paediatrics and Child Health, East Africa

Children with sickle cell anemia (SCA) frequently develop progressive neurocognitive impairment. We aimed to determine effects of hydroxyurea therapy on neurocognitive function in Ugandan children with SCA by comparing levels at enrollment to a planned 18-month interim assessment. Ugandan children (N=264) ages 3-9 years were enrolled from a SCA clinic and treated in a 30-month singlearm open-label trial with escalation to maximum tolerated dose (MTD). Primary outcome was the effects of hydroxyurea on cognition, attention and executive function, along with transcranial doppler ultrasound (TCD) blood flow velocity. Sibling controls (N=110) without SCA underwent neurocognitive testing in parallel to establish age-normalized …

Association Of Unmet Social Needs With Disease-Related Outcomes In Pediatric Patients With Sickle Cell Disease, Molly Sonenklar, Sarah Marks, Cerelia Donald, Cecelia Valrie, Wally Smith, India Sisler

School of Medicine Faculty Publications

Background: Social determinants of health (SDoH) are socioeconomic factors that influence health and well-being, though when unmet can greatly contribute to health disparities. Individuals with sickle cell disease (SCD) are at increased risk of mortality, disability, and healthcare utilization. However, there are limited data linking specific social needs with disease outcomes in this population. Therefore, we sought to identify the unmet needs in one institution and their association with healthcare utilization. Methods: Children with SCD and their guardians answered demographic and SDoH questionnaires during routine Sickle Cell Clinic appointments. We defined an unmet social need as any positive answer to …

Physician Perspectives About The Diagnosis And Management Of Acute Chest Syndrome, Neha Bhasin, Dana Marie Leblanc, Sean Yates, Quentin Eichbaum, An Pham, Deva Sharma, Li Zhang, Elliott P. Vichinsky, Ravi Sarode

School of Medicine Faculty Publications

Background: Acute chest syndrome (ACS) is the leading cause of mortality, accounting for 25% of all deaths among individuals with sickle cell disease (SCD). There is a lack of evidence-based laboratory and clinical risk stratification guidelines for the diagnosis and management of ACS. Study Design and Methods: To better understand physician practices for the management of ACS in the United States, we created an ACS Working Group including hematology and transfusion medicine physicians from four different SCD treatment centers in the United States. The working group created a physician survey that included physician demographics and ACS diagnostic criteria that they …

Eculizumab For Management Of Hyperhemolysis Syndrome In Pediatric Patients With Sickle Cell Disease: A Single-Center Case Series, Ajibike Lapite, Saleh Bhar, Titilope Fasipe

Faculty, Staff and Students Publications

Chronic hemolytic anemia and vascular occlusion are hallmarks of sickle cell disease (SCD). Blood transfusions are critical for supportive and preventive management of SCD complications. Patients with SCD are at risk for hyperhemolysis syndrome (HHS), a subtype of delayed hemolytic transfusion reactions. HHS management includes intravenous immunoglobulin, corticosteroids, and avoidance of further transfusions. Not all patients respond to first-line agents. Eculizumab, which blocks terminal complement activation, has been proposed as second-line management of HHS. We describe two patients who received eculizumab for refractory HHS. In our experience, eculizumab is a safe and effective option for refractory pediatric HHS.

Brief Review: Regional Anesthesia For Vaso-Occlusive Pain Crises, Oluwatomi Alade

Rowan-Virtua Research Day

Vaso-occlusive pain crisis occurs with obstruction of blood vessels from sickled red blood cells. This results in ischemic injury causing in pain. Acute vasoocclusive pain crisis is one of the most common reasons for patients with sickle cell disease to present to the hospital for medical attention. Acute treatment involves IV opioid therapy, non-opioid therapy, and IV hydration. There is a known lack of trust between a patient in acute pain and a provider in the emergency department (ED) and hospital secondary to stereotypes regarding pain seeking behavior. Here we discuss a case of vasoocclusive pain crisis refractory to opioid …

Recurrent Nontraumatic Subgaleal Hematomas In A Pediatric Patient With Sickle Cell Disease, Irtiza N Sheikh, Olayinka Okeleji, Rabya Afzal, Eliana Bonfante, Monica Kodakandla, Neethu M Menon

Faculty, Staff and Student Publications

Spontaneous subgaleal hematoma in pediatric patients with sickle cell disease (SCD) is a rare occurrence that can present with symptoms mimicking ischemic stroke, a known complication of SCD. However, unlike ischemic stroke, subgaleal hematoma is nonlethal and can be managed conservatively without major sequelae. Here, we present the case of an adolescent with SCD who presented with 2 episodes of subgaleal and epidural hematomas, 2 years apart. The latter episode occurred while on crizanlizumab, an anti-P-selectin antibody, approved for use in SCD in 2019 to reduce the number of acute pain crises. We demonstrate the diagnosis of subgaleal hematoma and …

Pain Screening In Youth With Sickle Cell Disease: A Quality Improvement Study, Dennis C. Vroom, Samuel N. Rodgers-Melnick, Amma Owusu-Ansah, Jeffery A. Dusek

Student Scholarship

Background: Youth with sickle cell disease (SCD) face several challenges as they age, including increased pain frequency, duration, and interference. The purpose of this study was to (i) determine the feasibility of routine pain screening; (ii) identify and describe various clinical pain presentations; and (iii) understand preferences/resources related to engaging in integrative health and medicine (IHM) modalities within an outpatient pediatric SCD clinic. Methods: During routine outpatient visits, patients aged 8–18 completed measures of pain frequency, duration, and chronic pain risk (Pediatric Pain Screening Tool [PPST]). Participants screening positive for (i) persistent or chronic pain or (ii) medium or high …

Editorial: Developments In Sickle Cell Disease Therapy And Potentials For Gene Therapy, Robert W. Maitta, Hollie M. Reeves

Faculty Scholarship

No abstract provided.

Massage Therapy For Children, Adolescents, And Young Adults: Clinical Delivery And Effectiveness In Hematology And Oncology, Samuel N. Rodgers-Melnick, Mandy Bartolovich, Neha J. Desai, Smitha Hosahalli Vasanna, Amma Owusu-Ansah, Sanjay P. Ahuja, Jeffery A. Dusek, David W. Miller

Student Scholarship

Background: Children, adolescents, and young adults with hematologic and/or oncologic conditions experience multiple, significant symptoms (e.g., pain, stress, and anxiety), which may be addressed by nonpharmacologic approaches such as massage therapy (MT). The purpose of this study was to describe the clinical delivery of MT provided by a certified pediatric massage therapist and assess effectiveness in two patient groups: those with sickle cell disease (SCD) or hematologic and/or oncologic conditions excluding SCD (HemOnc). Methods: Investigators conducted a retrospective review of MT sessions provided to patients 0–39 years with hematologic and/or oncologic conditions at a large pediatric academic medical center. Results: …

Sickle Red Blood Cell-Derived Extracellular Vesicles Activate Endothelial Cells And Enhance Sickle Red Cell Adhesion Mediated By Von Willebrand Factor, Ran An, Yuncheng Man, Kevin Cheng, Tianyi Zhang, Fang Wang, Erdem Kucukal, William J. Wulftange, Utku Goreke, Allison Bode, Lalitha V. Nayak, Jane A. Little, Umut A. Gurkan

Faculty Scholarship

Endothelial activation and sickle red blood cell (RBC) adhesion are central to the pathogenesis of sickle cell disease (SCD). Quantitatively, RBC-derived extracellular vesicles (REVs) are more abundant from SS RBCs compared with healthy RBCs (AA RBCs). Sickle RBC-derived REVs (SS REVs) are known to promote endothelial cell (EC) activation through cell signalling and transcriptional regulation at longer terms. However, the SS REV-mediated short-term non-transcriptional response of EC is unclear. Here, we examined the impact of SS REVs on acute microvascular EC activation and RBC adhesion at 2 h. Compared with AA REVs, SS REVs promoted human pulmonary microvascular ECs (HPMEC) …

Multispectral Imaging For Microchip Electrophoresis Enables Point-Of-Care Newborn Hemoglobin Variant Screening, Ran An, Yuning Huang, Anne Rocheleau, Qiaochu Zhang, Yuncheng Man, Zoe Sekyonda, Amma Owusu-Ansah, Umut A. Gurkan

Faculty Scholarship

Hemoglobin (Hb) disorders affect nearly 7% of the world's population. Globally, around 400,000 babies are born annually with sickle cell disease (SCD), primarily in sub-Saharan Africa where morbidity and mortality rates are high. Screening, early diagnosis, and monitoring are not widely accessible due to technical challenges and cost. We hypothesized that multispectral imaging will allow sensitive hemoglobin variant identification in existing affordable paper-based Hb electrophoresis. To test this hypothesis, we developed the first integrated point-of-care multispectral Hb variant test: Gazelle-Multispectral. Here, we evaluated the accuracy of Gazelle-Multispectral for Hb variant newborn screening in 265 newborns with known hemoglobin variants including …

Occlusionchip: A Functional Microcapillary Occlusion Assay Complementary To Ektacytometry For Detection Of Small-Fraction Red Blood Cells With Abnormal Deformability, Yuncheng Man, Ran An, Karamoja Monchamp, Zoe Sekyonda, Erdem Kucukal, Chiara Federici, William J. Wulftange, Utku Goreke, Allison Bode, Umut A. Gurkan

Faculty Scholarship

Red blood cell (RBC) deformability is a valuable hemorheological biomarker that can be used to assess the clinical status and response to therapy of individuals with sickle cell disease (SCD). RBC deformability has been measured by ektacytometry for decades, which uses shear or osmolar stress. However, ektacytometry is a population based measurement that does not detect small-fractions of abnormal RBCs. A single cell-based, functional RBC deformability assay would complement ektacytometry and provide additional information. Here, we tested the relative merits of the OcclusionChip, which measures RBC deformability by microcapillary occlusion, and ektacytometry. We tested samples containing glutaraldehyde-stiffened RBCs for up …

Safety And Efficacy Of Voxelotor In Pediatric Patients With Sickle Cell Disease Aged 4 To 11 Years., Jeremie H. Estepp, Ram Kalpatthi, Gerald Woods, Sara Trompeter, Robert I. Liem, Kacie Sims, Adlette Inati, Baba P D Inusa, Andrew Campbell, Connie Piccone, Miguel R. Abboud, Kim Smith-Whitley, Sandra Dixon, Margaret Tonda, Carla Washington, Noelle M. Griffin, Clark Brown

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Sickle cell disease (SCD) is a devastating, multisystemic disorder that affects millions of people worldwide. The earliest clinical manifestations of SCD can affect infants as young as 6 months of age, and pediatric patients are at risk for acute and life-threatening complications. Early intervention with treatments that target the underlying pathophysiological mechanism of SCD, sickle hemoglobin (HbS) polymerization, are expected to slow disease progression and circumvent disease-associated morbidity and mortality.

PROCEDURE: The HOPE-KIDS 1 trial (NCT02850406) is an ongoing four-part, phase 2a, open-label, single- and multiple-dose study to evaluate the pharmacokinetics, efficacy, and safety of voxelotor-a first-in-class HbS polymerization …

Antithrombin-Iii Mitigates Thrombin-Mediated Endothelial Cell Contraction And Sickle Red Blood Cell Adhesion In Microscale Flow, William J. Wulftange, Erdem Kucukal, Yuncheng Man, Ran An, Karamoja Monchamp, Charlotte D. Sevrain, Himanshu R. Dashora, Allison Bode, Jane A. Little, Umut A. Gurkan

Faculty Scholarship

Individuals with sickle cell disease (SCD) have persistently elevated thrombin generation that results in a state of systemic hypercoagulability. Antithrombin-III (ATIII), an endogenous serine protease inhibitor, inhibits several enzymes in the coagulation cascade, including thrombin. Here, we utilize a biomimetic microfluidic device to model the morphology and adhesive properties of endothelial cells (ECs) activated by thrombin and examine the efficacy of ATIII in mitigating the adhesion of SCD patient-derived red blood cells (RBCs) and EC retraction. Microfluidic devices were fabricated, seeded with ECs, and incubated under physiological shear stress. Cells were then activated with thrombin with or without an ATIII …

Regional Anesthesia For Sickle Cell Disease Vaso-Occlusive Crisis: A Single-Center Case Series, Cecile Karsenty, Venée N Tubman, Chyong-Jy Joyce Liu, Titilope Fasipe, Karla E K Wyatt

Faculty, Staff and Students Publications

Pain management is challenging for patients with sickle cell disease (SCD) who present in vaso-occlusive crisis (VOC). Opioid therapy is highly effective, nevertheless undesirable side effects can hinder their effectiveness. Regional anesthesia with deposition of perineural anesthetic offers nociceptive blockade, local vasodilatation, and reduces the inflammatory response. Among pediatric patients, continuous peripheral nerve block (CPNB) for perioperative adjunctive analgesia is safe. Herein, we describe the trajectory of a cohort of pediatric SCD patients with opioid-refractory upper-extremity VOC following placement of CPNBs for analgesia; highlighting reduced opioid consumption, improved pain scores, and decreased length of hospitalization.

Predicting Risk Factors For Thromboembolic Complications In Patients With Sickle Cell Anaemia - Lessons Learned For Prophylaxis, Salam Alkindi, Anwaar R Al-Ghadani, Samah R Al-Zeheimi, Said Y Alkindi, Naglaa Fawaz, Samir K. Ballas, Anil V Pathare

Cardeza Foundation for Hematologic Research

Objective: To assess the clinical and laboratory predictors of venous thromboembolism (VTE) in patients with sickle cell anaemia (SCA) and its relationship to morbidity and mortality.

Methods: This retrospective case-control study analysed data from patients with SCA that experienced VTE compared with matched control patients with SCA but no VTE (2:1 ratio).

Results: A total of 102 patients with SCA were enrolled (68 cases with VTE and 34 controls). Amongst the 68 cases (median age, 29.5 years), 26 (38.2%) presented with isolated pulmonary embolism (PE). A higher prevalence of splenectomy (73.5% versus 35.3%) was observed in the cases compared with …

Utilization Of Patient-Controlled Analgesia Reduces Length Of Stay Of Sickle Cell Crisis Hospitalizations, Brett M. Prestia, Talha Ramzan, Catherine Waldron, Ameer Malik, Robert M. Pallay, Candace R. Murbach, Mike Flynn, Eric Shaw Phd, Abdullah Kutlar, Daniel Lowe

HCA Healthcare Journal of Medicine

Background

Sickle cell crisis hospitalizations are emotionally and financially burdensome to patients and healthcare systems, and processes to decrease the frequency or length of stay of these crises should be examined.

Methods

This is a multicenter retrospective hospital record review of sickle cell crisis hospitalizations as defined by ICD-10 codes (D57.1-4), from January 2016 through December 2019, examining inpatient medication administration records and length of stay among admitted adults aged 18–65 years. Patient controlled analgesia orders using morphine, hydromorphone, fentanyl and/or merperidine at any point of an admission (n=188) were compared to admissions without any patient-controlled analgesia orders (n=2,159). The …

Level Of Utilization And Provider-Related Barriers To The Use Of Hydroxyurea In The Treatment Of Sickle Cell Disease Patients In Jos, North-Central Nigeria, Akinyemi O.D. Ofakunrin, Edache S. Okpe, Tolulope O. Afolaranmi, Rasaq Olaosebikan, Patience U. Kanhu, Kehinde Adekola, Nantok Dami, Atiene S. Sagay

Department of Pharmacology and Experimental Therapeutics Faculty Papers

Background: Hydroxyurea is underutilized by sickle cell health-care providers in Nigeria despite available evidence of its effectiveness in reducing the manifestations and complications of sickle cell disease (SCD).

Objectives: To assess the level of utilization and provider-related barriers to the use of hydroxyurea in SCD therapy in Jos, Nigeria.

Methods: A cross-sectional study conducted among 132 medical doctors providing care for SCD patients. Data on so- cio-demographics, utilization and barriers to hydroxyurea use were obtained. The barriers were fed cumulatively into the logistic regression model as predictors of utilization.

Results: Of the 132 care providers, 88 (67%) had been in …

Determinants Of The Outcome Of The Transition Of Children With Sickle Cell Disease To Adult Programs., Samir K. Ballas, Gaye Riddick-Burden, Elisabeth Congdon-Martin

Cardeza Foundation for Hematologic Research

Transition of adolescents with sickle cell disease to adult programs is associated with increased morbidity and mortality. The reasons for this poor outcome are not well known. This report describes the various factors that affect the outcome of the transition process. These include four inter-personal factors: country of residence, region within the country of residence, the health care system and intra-personal factors. Each factor is described in some detail. Understanding these factors and the establishment of guidelines or recommendations could improve the outcome of this critical transition in the life of patients with sickle cell disease.

Sickle Haemoglobin: How Critical Are Laboratory Quality Measures For Accurate Identification?, Nazish Sana, Muhammad Shariq Shaikh

Department of Pathology and Laboratory Medicine

No abstract provided.

Opioids And Sickle Cell Disease: From Opium To The Opioid Epidemic., Samir K. Ballas

Cardeza Foundation for Hematologic Research

Sickle cell disease (SCD) is an inherited disorder of hemoglobin structure. The clinical effects of the sickle gene are pleiotropic in nature causing multiple phenotypic expressions associated with the various complications of the disease. The hallmark of the disease is pain that could be acute, chronic, nociceptive, or neuropathic that could occur singly or in various combinations. The acute vaso-occlusive painful crisis (VOC) is the most common cause of admissions to the Emergency Department and/or the hospital. Although progress has been made in understanding the pathophysiology of SCD as well as in developing preventive and curative therapies, effective pain management …

How I Treat Acute And Persistent Sickle Cell Pain., Samir K. Ballas

Cardeza Foundation for Hematologic Research

Sickle pain is the hallmark of sickle cell disease (SCD). It could be acute, persistent/relapsing, chronic, or neuropathic. Although there is a general consensus that pain is a major manifestation of SCD, there is a controversy as to the types of pain and their interrelationship between acute, chronic, relapsing, persistent, etc. This report first reviews the general approach to the management of acute vaso-occlusive crisis (VOC) pain, including education, counseling, pharmacotherapy, non-pharmacotherapy, and fluid therapy. This is followed by the presentation of five patients that represent typical issues that are commonly encountered in the management of patients with SCD. These …