Care For Sickle Cell Disease Patients Research Articles

Enhancing the Management of Pediatric Sickle Cell Disease by Integrating Functional Evaluation to Mitigate the Burden of Vaso-Occlusive Crises

Sickle cell disease (SCD) imposes a significant health burden, particularly in low- and middle-income countries where healthcare professionals and resources are scarce. This opinion paper delves into the management strategies employed for vaso-occlusive crises (VOCs) in pediatric patients with SCD, advocating for the adoption of a transformative strategy. We explore the integration of functional assessment approaches into existing procedures, highlighting the potential of technology-assisted rehabilitation, including wearable sensors and digital biomarkers, to enhance the effectiveness of managing and preventing VOCs. Rehabilomics, as a comprehensive framework, merges rehabilitation-related data with biomarkers, providing a basis for personalized therapeutic interventions. Despite the promising advantages of these approaches, persistent obstacles such as the limited availability of rehabilitation programs, especially in resource-limited settings, pose challenges. This paper underscores the importance of a collaborative strategy to effectively address the unique obstacles faced by patients with SCD. This collaborative approach involves improving accessibility to rehabilitation services, incorporating technology-supported therapy, and fostering focused research endeavors. The primary objective of this comprehensive approach is to enhance the overall care of SCD patients, with a specific focus on preventing VOCs, as well as providing tailored (neuro)rehabilitation services in resource-limited settings. By examining the current state of SCD management and proposing transformative strategies, this opinion paper seeks to inspire collective action and collaboration to improve outcomes for pediatric SCD patients globally.

Distinguishing the Variances in Patient Care between Hematologists and Hospitalists for Individuals with Sickle Cell Disease Hospitalized Due to Vaso-Occlusive Crisis

Introduction: Sickle cell disease (SCD) is a chronic condition characterized by multiple vaso-occlusive complications. Patients often experience recurrent, severe pain that can be disabling and necessitate opioid treatment. Hospitalization for vaso-occlusive crisis (VOC) is a common occurrence for individuals with SCD. In recent times, the care of hospitalized SCD patients has increasingly shifted from adult hematology specialists to hospitalists. At our institution, we are yet to make this transition, with hospitalists rarely taking over the care of inpatients with SCD. Initially, recommendations to the hospitalist service were provided through informal opinions or formal consultations when requested. Hospitalists have demonstrated efficient inpatient management; however, some may have knowledge gaps and biases that can influence their interactions with SCD patients. To gain a better understanding of the differences in care practices between hematologists and hospitalists and to identify potential areas for improvement in the care of SCD patients, we conducted a formal comparison of their approaches. Methods: We performed a single-institution, retrospective review of patients with SCD hospitalized for VOC between January 1, 2014, and September 31, 2022. Results: Table 1 portrays the significant results. Conclusion: We observed significant distinctions in the care provided by hematologists and hospitalists to patients admitted for VOC in SCD. Hematologists tended to transfer fewer patients to the intensive care unit compared to hospitalists. On the other hand, hospitalists more frequently sought consultations from the pain service than hematologists. Interestingly, patients under the care of hematologists received longer hours of patient-controlled analgesia for pain management. Additionally, patients managed by hematologists exhibited higher hemoglobin levels at the time of discharge when compared to those treated by hospitalists. Notably, only 39.8% of patients had a formal hematology consult requested during their hospital stay, indicating a potential need for better collaboration between hematologists and hospitalists. This study emphasizes the importance of enhancing cooperation between these two specialties to elevate the quality of care for patients hospitalized with VOC.

Knowledge, attitude, and practice of physicians regarding pain management in patients with Sickle cell disease

Sickle cell disease (SCD) is a hemoglobin disorder inherited in an autosomal recessive pattern. Pain from vaso-occlusive crises (VOCs) is the most common symptom experienced by patients with SCD; thus, pain management constitutes a significant role in this patient population. We hypothesized that physicians with less favorable attitudes toward SCD patients are less likely to follow pain management guidelines. In this cross-sectional convenience sample survey, we aimed to assess the adherence of physicians in our institute to guidelines for the management of SCD pain crises and study the factors that are associated with non-adherence to these guidelines. Most surveyed physicians were early in their career and were found to adhere to the recommendations of using opioids and NSAIDs as a first-line therapy and avoiding using meperidine. On the other hand, some analgesic practices deviated from the recommendations, including less frequent assessment and reassessment of pain and response to pain medications and less frequent use of opioid patient-controlled analgesia (PCA). It is noteworthy that the frequency and severity of untreated pain in patients with SCD are associated with higher mortality, thus appropriate comprehensive care for such a vulnerable population should be prioritized and optimized. Although we did not find an association between the providers’ attitudes toward SCD patients and their pain management practices, improving these attitudes will promote the provider–patient relationship and its therapeutic outcomes. We conclude that the physicians taking care of SCD patients in our institute adhere to some of the guidelines in the field more than others. We also conclude that they have negative attitudes toward SCD patients; nevertheless, these did not affect their pain management practices.

Managing Kidney Disease in Patients with Sickle Cell Disease: A Quality Initiative at the Cleveland Clinic

Background Sickle cell disease (SCD) when complicated by sickle cell nephropathy and chronic kidney disease (CKD) is associated with high morbidity and mortality. Early intervention with appropriate specialist care and timely evaluation for transplant are essential to improve outcomes. There is considerable variability in the screening, diagnosis and management of renal complications in SCD. Current guidelines from the NHLBI (2014), ASH (2019) and expert opinion endorse (1) annual screening for proteinuria, (2) the initiation of angiotensin-converting enzyme inhibitors (ACEi) or angiotensin II receptor blockers (ARB) in patients with significant proteinuria, (3) early involvement of nephrology for declining renal function, (4) testing for alternative etiologies of kidney disease, (5) referral for renal transplant in advanced CKD with regular red cell exchange in those being considered for transplant, and (6) combination therapy with hydroxyurea and erythropoiesis-stimulating agents (ESA) for patients with worsening anemia from CKD. We sought to audit our care of SCD patients along these metrics to devise effective interventions to improve outcomes in SCD patients with comorbid kidney disease. Methods We conducted a retrospective quality review of renal disease screening, referral, and management in adults with SCD who were seen in the outpatient setting at the Cleveland Clinic in the past 3 years. Results We reviewed 258 adults with SCD who were seen between July 2019 and July 2022. Of these, 16 patients had insufficient laboratory data and were excluded from further analysis. Our final cohort of 242 patients was comprised of 169 (70%) female patients and the median age was 42 (18-90) years. The majority [213 (88%)] had appropriate screening for proteinuria. Of 46 patients with significant proteinuria, 18 (39%) were on ACEi/ARB in part reflecting challenges with tolerance (hyperkalemia, cough, hypotension). CKD was present in 43 (18%) patients; 25 (58%) had CKD stage 3a, 5 (12%) stage 3b, 6 (14%) stage 4, and 7 (16%) were on dialysis. Nephrology was involved in 27 (63%) of those with CKD. Most [31 (72%)] were evaluated for other causes of CKD, however the extent of testing was inconsistent. Referral for renal transplant evaluation was made in 7 (16%) patients with CKD and approximately half [3 (43%)] of these patients were receiving regular red cell exchange transfusions. Only 2 (5%) patients received a kidney transplant. Of the subset of 13 patients with advanced CKD stages 4 or 5, 9 (69%) were receiving ESA and 3 (23%) were on combination hydroxyurea and ESA. In our larger cohort of 242, an additional 30 (12%) patients had isolated persistent microscopic hematuria and 44 (18%) patients demonstrated a trend of chronically rising creatinine within the laboratory normal range. Additional analysis is planned to evaluate screening and management in this at-risk cohort with more subtle presentation. Discussion The management of renal disease in SCD is complex and a paucity of data to support evidence-based guidelines presents a major challenge. We found that rates of screening for proteinuria and declining renal function in SCD patients were high, however the subsequent management was inconsistent. CKD in SCD is highly morbid, and patients with SCD continue to experience low rates of transplant evaluation and eligibility with devastating consequences. A root cause analysis, patient and provider education campaigns, and additional activities are planned to improve the management of kidney disease in patients with SCD.

Pharmacy Hydroxyurea Education Materials for Patients with Sickle Cell Disease: An Environmental Scan and Assessment of Accuracy

BACKGROUND: Hydroxyurea (HU) remains a cornerstone of sickle cell disease (SCD) therapy however its clinical efficacy may be limited by poor patient acceptance and adherence. While barriers to HU use are multifold, patients often relay fears relating to potential side effects of HU as a contributor to drug non-adherence. A common source of information on HU side effects is the patient information handouts (PIHs) that are routinely distributed by pharmacies when dispensing a prescription. We sought to formally evaluate the content and accuracy of HU pharmacy PIHs, including comparison of content with a reference standard PIH developed by the Canadian Haemoglobinopathy Association.METHODS: We obtained a comprehensive list of all accredited pharmacies in Ontario, Canada in 2016 from the Ontario College of Pharmacists (OCP), which totalled 4,150 pharmacies. A representative sample of pharmacies were subsequently contacted by telephone or in-person to obtain pharmacy PIHs on HU. PIHs were assessed according to listed uses of HU, number of inaccurate statements, essential statements based on comparison to the CanHaem reference standard PIH, and reading level (using Flesch-Kincaid and SMOG scales).RESULTS: Six unique HU PIHs were identified, which represented approximately 95% of Ontario pharmacies; PIH A and B were used by 58% and 36.7% of pharmacies, respectively (Table 1). While most of the PIHs listed uses for HU (60% of Ontario pharmacies in total), only 58.3% identified SCD as an indication for drug use. All PIHs contained statements that could be classified as inaccurate and misleading. The number of inaccurate statements ranged from 2-5 per PIH; 95% of distributed PIHs had an average of 4 inaccurate statements per handout. The most frequently encountered inaccuracy related to development of cancer in patients taking HU, namely acute leukemia. Additional inaccuracies included special handling precautions, vaccine inefficacy and overstating the likelihood of developing rare side effects such as, “deadly bleeding problems and infections,” hepatic insufficiency and infertility.On average, pharmacy PIHs included 45% of essential statements present within the reference standard HU patient handout. Missing information themes involved use of HU for management of SCD and specifically, benefits of HU in preventing SCD complications, long term safety data on HU and differentiation between common and uncommon side effects of HU use.The reading level of each pharmacy PIH was also assessed and compared to the grade 6 reading level formally advised for written patient information materials by the National Work Group on Literacy and Health. The average reading level across pharmacy PIHs was 7.85 and 7.83 according to the Flesch Kincaid and SMOG scales, respectively. However, 58% of Ontario pharmacies distributed PIHs with a reading level of 9.2 and 8.2 (Flesch Kincaid and SMOG, respectively) in comparison to the grade 6.2 and 6.7 (Flesch Kincaid and SMOG, respectively) reading level of the CanHaem PIH.CONCLUSION: SCD patients in Ontario who are prescribed HU are likely to receive written information materials from pharmacies that contain inaccurate and misleading statements weighted heavily toward risks of HU therapy and run contrary to published literature. Moreover, the PIHs reviewed in this study contained only 45% of the content deemed necessary for patient education on HU by a panel of experts in SCD. Oftentimes, this information requires a comprehension level beyond that recommended for patients. Through the itemization of written information provided to patients when obtaining their HU prescription, this study identifies a potential gap in the care of SCD patients and an opportunity to improve on the quality of HU PIHs to help patients make well-informed decisions about their health. [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Sickle cell disease: knowledge and clinical practice of dental surgeons at Family Health Units

ABSTRACT Objective: This study analyzes dental surgeons’ knowledge and clinical practice regarding sickle cell disease (SCD) at Family Health Units in Recife, northeastern Brazil. Methods: This cross-sectional study was conducted in a representative sample of dentists using a standardized questionnaire. Data were analyzed by descriptive statistics. Results: Out of 132 eligible dental surgeons, 124 (93.9%) were interviewed. Most of them were women (81.5%), aged between 28 and 39 years (53.2%), with more than 15 years of experience (52.4%). A percentage of 89.5% knew the definition and oral manifestations (53.2%) of the disease, but only 17.7% knew the dental care protocol. It was verified that 13.6% of the dentists who knew how to conduct the treatment had acquired this knowledge during their undergraduate studies. Conclusion: Regarding clinical practice, 71.8% said they would treat patients with SCD; however, 67.7% had never done that. Most dentists felt confident of performing dental procedures (82%) and their major difficulty was their lack of knowledge about the disease (57.1%). The findings suggest the need for the implementation of public policies for improving the qualification of dental surgeons who work in primary healthcare, thus minimizing risks during the dental care of SCD patients.

Characteristics and Trends of Sickle Cell Disease Patients Once Presented to the Emergency Department, Reflecting on a Possible Burden: United States (2010-2015)

Background: Sickle cell disease (SCD) is an autosomal recessive, multisystem disorder, characterized by chronic hemolytic anemia, painful episodes of vaso-occlusion, progressive organ failure and a reduced life expectancy. SCD is the most common monogenetic disease, with millions affected worldwide. It is estimated that there are 100,000 people living with SCD in the United States (US). Patients with SCD often use emergency department (ED) services to obtain medical care. Limited nationwide information in the recent years is available about ED use among patients with SCD. Methods: Data from the National Hospital Ambulatory Medical Care Survey (NHAMCS) for the years 2010-2015 were analyzed, to examine the prevalence and care of SCD patients in the EDs across the US. The NHAMCS is a survey of hospital ED and outpatient visits. Cases were identified using ICD 9 codes and all analyses accounted for complex survey structure of the data. Results: On average, approximately 1.4 million ED visits were estimated to have occurred between the years 2010 and 2015 with SCD as one of the diagnoses listed, where 860,000 were attributed to sickle cell crises. 37.29% were of female sex while 62.71% were males. 11.62% were under 15 years of age, 28.22% were between 15 and 24 years of age, 51.15% were between the ages of 25 and 44 years and 9.02% were older than 45 years. The chief complaint for the majority of cases was pain. The expected source of payment were Medicare and Medicaid at 30.45% and 41.98% respectively, while private insurance coverage and self-pay were only at 11.71% and 5.81% respectively. Of all SCD ED visits, 68.5% were considered as initial presentations and up to 23.37% were considered as follow-up. 33.31% of the visits ended up needing a hospital admission while 66.69% got discharged from the ED.In 43.39% of the visits, a form of imaging was ordered, compared to 56.61% where it was not. Upon getting triaged in the ED, 67.62% of all SCD visits needed either emergent or urgent medical attention, while the rest were either none-urgent, or a regular check-up. Conclusion: In well-resourced countries, like the US, comprehensive care programs have increased life expectancy of sickle cell disease patients, with almost all infants surviving into adulthood. However unfortunately with poor control of the disease due to limited therapeutic options and with growing morbidity for the aging SCD patients, the burden on the medical system becomes extremely high. Our study highlights the significant burden SCD creates in the EDs across the US based on data collected from a national database. Disclosures No relevant conflicts of interest to declare.

Current Healthcare Utilization Patterns and Most Common Reasons for Admission Amongst Patients with Sickle Cell Disease

Background: Sickle cell disease (SCD) is an inherited condition affecting over 100,000 individuals in the United States with increasing prevalence, with annual healthcare costs of approximately $488 million dollars. Peak rates of healthcare utilization occur in the young adult population, with the highest mortality risk when transitioning to adult care. This has prompted increased efforts to improve the transition process from pediatric to adult care, in addition to optimizing overall care for SCD patients. The impact of these efforts on current healthcare utilization, including reasons for hospitalization, has yet to be explored. This study sought to explore current rates of healthcare utilization in SCD patients, measured by inpatient hospitalizations, as well as the most common reasons for hospitalization to inform continued efforts to improve outcomes in the SCD population.Methods: Data was obtained from the Healthcare Cost Utilization Project (HCUP) State Inpatient Database (SID) for New York from 2005-2014. All patients with a principal or secondary ICD-9-CM diagnosis of any genotype of SCD (282.41, 282.42, 282.60-282.64, 282.68 or 282.69) were identified. Patients with anemia or other hemoglobinopathies not due to sickle cell were excluded. The encrypted identifier was used to identify all hospitalizations in SCD patients (including those not coded for SCD), and analyze outcomes at a patient-level. Utilization outcomes examined were (1) total number of patient encounters and (2) 30-day and (3) 90-day readmissions following the index hospitalization. To examine reasons for hospitalization, variables for common conditions were created using standard ICD-9-CM diagnosis codes found in the Elixhauser Comorbidity algorithm, a tool used to predict hospital resource use and in-hospital mortality, in addition to acute or chronic conditions associated with SCD. Descriptive statistics and univariate analyses were performed using SAS Enterprise Guide version 7.1. This project was determined to be exempt from Institutional Review Board oversight per the Washington University Human Research Protection Office.Results: A total of 19,729 patients had at least one hospitalization from 2005-2014 coded for SCD, with a total of 140,779 hospitalizations. The SCD population was 57.6% female and the primary insurance type was Medicaid (49.9%). The mean age at index hospitalization was 26.5 years (SD 19.3, range 0-104) and the mean length of stay was 5.5 days (SD 11.4). At the time of the index hospitalization SCD patients aged18-30 years made up 29% of the population (Table 1). The largest percentage of all hospitalizations also occurred among patients in this age group (33.3% of total encounters). Hospitalization rates per 10 person-years of observation were similar among patients aged 10-17 years (13.2), 18-30 years (13.8), 31-45 years (12.9) and 46-64 years (13.3). Both 30-day and 90-day readmissions after the index hospitalization were highest in the 18-30 year age group (33.9% and 31.1%, respectively, Table 2). The most commonly coded diagnoses were pneumonia (23.3%), vaso-occlusive crisis (22.9%) and respiratory infection (20.1%, Table 3). The most commonly coded principal diagnosis was vaso-occlusive crisis (11.7%). Diagnoses associated with the respiratory system accounted for the largest category of hospitalization reasons, including: pneumonia (23.3%), respiratory infection (20.1%), asthma (14.5%), respiratory failure (11.0%) and chronic lung disease (14.6%). (Table 3)Conclusion: Analysis of 10 years of inpatient data shows that overall rates of hospitalizations in SCD patients have decreased as compared to those previously reported in the literature. Rates per person years were similar across most age groups, but 30-day readmission, a frequently used quality metric, was highest in the young adult age group. This suggests an improvement in utilization patterns, but also reiterates the need for targeted efforts towards improved care in the young adult group. Hospitalizations were frequently due to vaso-occlusive episodes, and a large number of hospitalizations were due to complications of the pulmonary system, both infectious and non-infectious. Directed efforts towards optimizing pulmonary health and aggressive treatment of respiratory infections may further decrease the utilization burden and hasten the improvement of outcomes in the SCD population. [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Psychosocial Care And Adjustment Of Children And Families With Sickle Cell Disease: The Nigerian Perspective

Nigeria is a country with one of the largest number of sickle cell disease (SCD) patients in the world. Many affected children and their families experience many adverse psychosocial effects. Most SCD clinics and other outlets of SCD care in Nigeria focus mainly on the physical well being of affected children with little or no concern about prevention or management of these associated psychosocial complications. This has negative implication for management and manageability of the disease. This paper is aimed at reviewing psychosocial care and adjustment in sickle cell disease with focus on the present status, problems and possibilities in Nigeria. This is a review article on psychosocial care of SCD children and their families, using manual search for literature in library, internet and textbooks. The psychosocial component of the care of SCD patients in Nigeria is still deficient and need to be improved. Major problems of psychosocial care of SCD patients in Nigeria include: insufficient attention to data gathering on problems and coping strategies, inadequate health education and public enlightenment on coping strategies and shortage of trained human resources, social welfare services and infrastructure. Sustained commitment to tackling these problems will upgrade psychosocial care for SCD children and their families in Nigeria. This is achievable if the knowledge and skill of psychosocial care is made available to all groups of health professionals involved in health care delivery to children; with the mental health professionals spearheading the initiative. Key words : Sickle cell disease, children and family, psychosocial care, adjustment.

Hydroxyurea in children with sickle cell disease: Practice patterns and barriers to utilization

Hydroxyurea (HU) is underutilized in adults with sickle cell disease (SCD) despite the Multicenter Study of Hydroxyurea in Sickle Cell Anemia (MSH) (1). Since little is known about HU utilization in children with SCD we sought to: 1) evaluate patterns of HU utilization; 2) elicit how providers define frequent pain when prescribing HU; and 3) identify barriers to HU use by surveying members of the American Society of Pediatric Hematology/Oncology. Data analysis included descriptive statistics and Chi-square. Of the 350 respondents, 63% care for SCD patients. Of these providers, only 9% have 50–90% of patients on HU, while 10% have 30% of patients on HU (35.2% vs. 20%; p=0.023; Chi-square). Provider-related barriers to prescribing HU included compliance with: HU (86%), laboratory monitoring (85%), and contraception (85%). Our survey suggests substantial variation in HU utilization in children. Providers accounting for pain managed both in and out of the hospital had more patients on HU. Existing barriers to HU utilization should be addressed to optimize care for children with SCD.