Management Of Sickle Cell Disease Research Articles

An Ayurvedic Management of Sickle Cell Anemia in Children: A case study

Introduction:- Sickle cell anemia is a term for a group of genetic diseases characterized by the production of hemoglobin (Hb) "S". This is the result of replacing the base pair thymine at the 6th position of the β-globin gene on chromosome 11 with adenine and replacing valine with glutamic acid. A single amino acid substitution is the reason for the profound changes in the stability and solubility of the hemoglobin "S" molecule. Due to its chronic nature and painful crisis, children’s quality of life is difficult. From now on, there is no final treatment other than a successful bone marrow transplant. Its symptoms and the nature of the disease can be compared with Pandu. If a drug improves the quality of life and maintains the health of the patient, then the drug and efforts will be beneficial to society. Material and Methods:- A 9-year-old male patient was diagnosed with sickle cell anemia. Symptoms include joint and limb pain, anorexia, fatigue, loss of appetite, and weight loss. approached the OPD, department of Kaumarbhritya, Parul Institute of Ayurved, Vadodara. Observations and Results:-The patient is taking modern medicine (1 mg folic acid once a day). The Childs parents also wanted to take Ayurvedic medicine, so they came here for 2 months of Ayurvedic medicine treatment, during which he did not have a pain crisis.Weight improved From 16 kg to 18 kg.The quality of life of a patient is also improved. Conclusion:-Therefore, the purpose of this case study is to study the pathophysiology and treatment of sickle cell anemia from an Ayurvedic perspective and to study the possible mechanism of action of the drug.

Turmeric and Fenugreek - Herbal Agents to Alleviate Sickle Cell Disease

Introduction: Sickle cell disease (SCD) is a genetic disorder with autosomal recessive inheritance causing abnormal hemoglobin. It is an important medical problem globally as well as in our community at Eastern province of Saudi Arabia. It accounts for large numbers of morbidity and to a lesser extent mortality. Several natural plant products over the world, which were known in some communities and their traditional healers, were studied and found to have properties in improving SCD manifestation. This research was planned to explore if any plant product under our study could prove being an effective anti-sickling agent for therapeutic use in SCD management. Objectives: Studying the effects of some plants on sickled RBCs as well as comparing between efficacies of these different plants. Material and Methods: The 49 fresh whole blood samples, extracted through venipuncture in EDTA tubes, were collected from known SCD patients whose homozygosity were confirmed by hemoglobin electrophoresis. These patients were not on recent blood transfusion or hydroxyurea, and aged 14 years or above of both sexes on clinical follow up. The dried rhizomes of turmeric (curcuma longa) powder and dried seeds flour of fenugreek (trigonella foenum-graecum) were used to evaluate the reversal type of anti-sickling activity on RBCs. Results: Out of 49 blood samples, 28 (57.1%) samples were from males and 21 (42.9%) samples from females. The average (±SD) age of the cases was 28.2 (±9.97) years with range 14 – 60 years. Significant anti-sickling activity was found using Turmeric rhizomes extract treatment with 66.81% reversal effect, and 61.28% anti-sickling action when fenugreek seeds extract was used. Conclusion: The findings of this study suggest that turmeric (C. longa) rhizome extract and fenugreek seed extract could have promising anti-sickling properties for SCD treatment.

Management of sickle cell disease in pregnancy. A British Society for Haematology Guideline.

This guideline was compiled according to the British Societyof Haematology (BSH) process at https://b-s-h.org.uk/guidelines/proposing-and-writing-a-new-bsh-guideline/. The Grad-ing of Recommendations Assessment, Development and Evaluation (GRADE) nomenclature was used to evaluatelevels of evidence and to assess the strength of recommenda-tions. The GRADE criteria can be found at http://www.gradeworkinggroup.org

Descrição do serviço de gestão da condição da saúde voltado a crianças recém-diagnosticadas com doença falciforme

Objective: To assess the results of the health condition management service provided for children with sickle cell disease (SCD). Methods: a cross-sectional study of the health condition management service offered to newly diagnosed children with SCD treated between January 2016 and December 2019 at the Blood Center in Belo Horizonte, Brazil. The service was provided by a clinical pharmacist and an academic from the pharmacy course, with data from the initial consultation (start of drug use) performed in a pharmacist’s office and telephone contact carried out eight days later. All children seen at the service were evaluated, and their demographic characteristics, compliance with the beginning of the use of prophylactic drugs for sickle cell disease (phenoxymethylpenicillin and folic acid) according to the protocol of the Ministry of Health (within 90 days after birth), and documented reason in case of non-compliance. The doubts regarding the medications and the standardized interventions of the service to the children´s guardians were also described. The difference in the distribution of variables in the adequacy and non-adequacy group to the MS protocol was assessed using Pearson’s chi-square test (categorical) and Mann-Whitney (numerical). Results: In the SCD condition management service, all children together with their guardians (N = 298) had their first consultation with the pharmacist (initial dispensation day), however, only 185 (62.1%) participated in the pharmaceutical service by telephone (eight days after the initial dispensing). Approximately 88.3% of children started using drugs for sickle cell disease according to the protocol of the Ministry of Health; the most frequent reason for non-compliance was the late diagnosis in the complementary health system (22.9%). The guardians pointed out 63 doubts about the medications, highlighting their non-acceptance by the child (28.6%), and for all doubts standard pharmaceutical guidelines were provided in the service. Conclusion: It was identified that the health condition management service provided is relevant, since most of those responsible for the children with SCD had doubts about their medication after the administration of medications began. The pharmacist contributes positively to the pharmacotherapeutic management of SCD, promoting empowerment about the correct use of medicines.

A double blind, placebo controlled randomized evaluation of the efficacy of a Polyherbal Preparation (FaradinR) in treating sickle cell anaemia in Nigerian children

Introduction: The goal of management of sickle cell anaemia (SCA), for many years, has been to manage acute intermittent crises and slow down chronic end organ damage. In the past few decades, with increasing understanding of its pathophysiology, compounds primarily preventive in action are being investigated and used. Faradin® (a poly-herbal traditional supplement mixture) has been used aspreventive measure against painful episodes by SCA patients as an over the counter medication and anecdotal evidence suggests that it reduced the frequency and severity of painful crises as well as transfusion requirements. Alternative medications that are both affordable and available should be considered viable alternatives provided safety and efficacy are assured because of the high disease burden in Nigeria.Methods: This was a double controlled randomized study was carried out on twenty children. Each enrolled patient was randomized into either the herbal mixture or placebo after permission to participate in the study was obtain from the parents/guardian for children below 15 years or from both parents/guardian and the patients where the latter are older than 15 years. The main exclusion criterion was prior use or exposure to Faradin. Primary end points were pain alteration, death during study and blood transfusion frequency. Secondary endpoints were hemoglobin levels, neutrophil count, platelet count, hemoglobin F and A2 levels, serum bilirubin, nitric oxide concentration, drug toxicity and severe complications of sickle cell anemia reported during the study.Results: There was no severe adverse event, deaths or transfusion recorded in the two groups throughout the duration of the study. Mean hematocrit was increased in the Faradin group and reticulocyte count was increased by 12 %. Faradin reduced the total white cell count to half its baseline level and increased hemoglobin F levels by 10%. Weight and appetite were reported to increased and engenders a generalfeeling of wellbeing.Conclusion: Faradin appears to be an efficacious, nontoxic, available and affordable remedy for treating SCA patients in our setting.

Age-related prescription medication utilization for the -management of sickle cell disease among Texas Medicaid patients.

Sickle cell disease (SCD) is associated with recurrent complications and healthcare burden. Although SCD management guidelines differ based on age groups, little is known regarding actual utilization of preventative (hydroxyurea) and palliative therapies (opioid and nonopioid analgesics) to manage complications. This study assessed whether there were agerelated differences in SCD index therapy type and SCD-related medication utilization. Texas Medicaid prescription claims from September 1, 2011 to August 31, 2016 were retrospectively analyzed for SCD patients aged 2-63 years who received one or more SCD-related medications (hydroxyurea, opioid, or nonopioid analgesics). The primary outcomes were SCD index drug type and medication utilization: hydroxyurea adherence, and days' supply of opioid, and nonopioid analgesics. Chi-square, analysis of variance, and Kruskal-Wallis tests were used. Index therapy percentages for included patients (N = 2,339) were the following: opioids (45.7 percent), nonopioids (36.6 percent), dual therapy-opioids and nonopioids (11.2 percent), and hydroxyurea (6.5 percent), and they differed by age-groups (χ2 = 243.0, p < 0.0001). Hydroxyurea as index therapy was higher among children (2-12:9.1 percent) compared to adults (26-40:3.7 percent; 41-63:2.9 percent). Opioids as index therapy were higher among adults (18-25:48.0 percent; 26-40:54.9 percent; 41-63:65.2 percent) compared to children (2-12:36.6 percent). Mean hydroxyurea adherence was higher (p < 0.0001) for younger ages, and opioid days' supply was higher for older ages. Texas Medicaid SCD patients had low hydroxyurea utilization and adherence across all age groups. Interventions to increase the use of hydroxyurea and newer preventative therapies could result in better management of SCD-related complications and reduce the frequency of pain crises, which may reduce the need for opioid use.

Systemic Complications and Imaging Challenges of Sickle Cell Disease in Sub-Saharan Africa

With improved life expectancy of people living with sickle cell disease (SCD) as a result of modern interventions, chronic problems such as pulmonary, abdominal, cardiovascular, musculoskeletal, and obstetric challenges are more often encountered. The need for radiological examination is, therefore, growing for the early diagnosis of these complications and improved prognosis. The emphasis, therefore, should be toward safer diagnostic options such as ultrasonography, transcranial Doppler ultrasound, and echocardiography as repeated radiation is counterproductive. This review is aimed at highlighting the imaging challenges encountered in the management of SCD in sub-Saharan Africa and possible ways of mitigating them. We reviewed available electronic literature in the last 20 years, especially articles from Google Scholar and PubMed with search keywords of imaging challenges, sickle cell disease, and sub-Saharan Africa. The challenges identified in this review include lack of institutional infrastructure, poor equipment availability, weak health and imaging personnel capacity, treatment affordability, service accessibility, and poor research and training facilities. With the increasing longevity of SCD individuals in sub-Saharan Africa, imaging requirements are rising. Repeated exposure to radiation should be minimized as much as possible. Engagement of the private sector through public-private partnership in equipping health institutions with radiological equipment, as well as training of appropriate personnel for their deployment is strongly recommended to enhance care, prevent complications, and improve outcomes for SCD in sub-Saharan Africa.

Complete RH and Kell matching related to low alloimmunisation risk in sickle cell disease: prevalence and risk factors of alloimmunisation in a Spanish Tertiary Care National Reference Centre.

Red blood cell (RBC) transfusion remains an essential part of sickle cell disease (SCD) management but it can lead to alloimmunisation, with an increased incidence in this population. Prevention is based on RBC antigen phenotype matching, with complete RH and Kell matching being a standard of care. We performed a retrospective, single-centre study analysing alloimmunisation prevalence and risk factors in a cohort of transfused SCD patients. Eighty-seven patients (96.5% of paediatric age) received 1,781 RBC units (RBCu). Complete RH and Kell matched RBCu represented a median of 100% among total transfusions per patient. Of the 87 patients, 52 (59.8%) underwent chronic transfusion therapy, whereas 35 (40.2%) were only episodically transfused. Seven patients were alloimmunised (8.4%) and eleven antibodies were detected (alloimmunisation rate: 0.62/100 units transfused). 54.6% of these antibodies corresponded to RH-Kell despite the high accomplishment of the RH-Kell matching transfusion protocol. Alloimmunised patients had a median of 90.9% RH-Kell matched transfusions vs 100% in non-alloimmunised patients, but no statistical differences were observed (p=0.127). Number of transfused RBCu (19 vs 7; p=0.023), number of episodic RBCu (8 vs 2; p=0.006), episodic to chronic RBCu ratio (0.57 vs 0.09; p=0.045), number of vaso-occlusive crises (VOC) (4 vs 2; p=0.011), and autoantibody presence (57.1 vs 0%; p<0.001) were all statistically related to alloimmunisation. We report a low alloimmunisation prevalence (8.4%) related to a high grade of RH-Kell matching. However, deviation from 100% translates into alloimmunisation, with >50% of alloantibodies corresponding to RH-Kell. Alloimmunisation risk increases with transfusion burden, particularly during acute complications, and in patients with a higher number of VOC, probably reflecting underlying inflammation and disease severity. Further studies will be needed to elucidate additional risk factors and help prevent alloimmunisation in these patients.

The effect of fixed oil extracts of Nigella sativa on sickle cells: An in-vitro study in Khartoum state -Sudan

In Sudan, sickle cell anemia (SCA) is one of the most common inherited disorders of hemoglobin that has complications and becomes world problem. Nigella sativa NS (black seed) has been reported to have calcium antagonist and antioxidant activities, both of which play a role in the management of sickle cell anemia. The aim of the current study was to evaluate of the anti-sickling activity of the NS extracts, forty patients with sickle cell anemia were recruited for the study. A total of 3ml of venous blood was collected from each patient after obtaining the consent and the ethical approval, the blood was treated with Nigella sativa (NS) extract, and sickling test was performed. Descriptive study and p-values were used, and the correlation was evaluated, data was analyzed by SPSS. Recent study found that the sickling test after treating blood with Nigella sativa extract showed a negative result in 75% of patients and a 25% of patients showed persistently positive sickling test result. The anti-sickling effect in relation to various hemoglobin concentration, gender and age group showed p values of 0.007, 0.672 and 0.853 respectively indicating significant relationship with Hb concentration. Our study concluded that the fixed oil extract from Nigella sativa has an in-vitro anti-sickling activity on patients with SCA and this finding could indicate the use of this extract in treatment.

Compliance with Expert Recommendations of Sickle Cell Pain Management—a Single Institution Review

Introduction:Sickle cell disease in a lifelong disorder associates with the occurrence of recurrent painful vasoocclusive crisis that often result in frequent emergency room trips and inpatient hospital stays. There are several goals in the management of sickle cell disease including early resolution of painful crisis and reduction of end stage events such as pulmonary hypertension and stroke. Unfortunately, the painful crisis leads to frequent ER visits and sometimes results in lengthy hospital stays. In 2014, The US Department of Health and Human Services came out with an expert report panel dictating recommendations on care for patient with sickle cell disease recommending pain medicine in the emergency room within one hour of triage and use of personal controlled analgesia (PCA) pumps. We opted to look at patient records within Orlando Health, a center with a large sickle cell patient population, to determine if we are following these recommendations.Methods: We reviewed emergency room and inpatient records of 158 patients. We documented if pain medications were started within two hours of arrival to the emergency room (we allowed additional time to account for registration and triage) and we reviewed inpatient records for PCA use. We also looked for trends in PCA use in terms of complexity of hospital stays and length of stay.Results:For sickle cell patients coming into the emergency room, 107/158 (68%) were given pain medication within 2 hours of registration.Of patients admitted to the hospital, only 10/158 (6%) were given a PCA pump. The patients given a PCA pump had an average length of stay of 16 days compared to the general population of 6.1 days. PCA use increased during lengthy hospitalizations rather than being started immediately.The patients who were given a PCA pump were assigned an ER acuity score of 3 compared to the general population of 2.1.Discussion:In caring for patients with sickle cell disease, we often aim to provide quick and effective management of pain crisis. In terms of emergency room management, triage to pain medication time is ideally within one hour. By looking at an extended window to account for registration time, we were able to provide pain medication on time 68% of the time. Per pharmacy staff, the pain medications are kept in the emergency room pharmacy system and should be immediately available upon request. Emergency room physicians identify overcrowding of the ER and the prospect of patient addiction as potential barriers to more immediate access. This thought process can lead to unnecessary suffering from patients.Inpatient PCA use was very infrequently started. Currently the hospitalist service is starting most of the inpatient pain medication. When asked about PCA use, the hospitalist physicians mostly stated that their experience hasn't shown a trend toward shorter length of stay. Additionally, patients frequently state that they prefer push medication to PCA use. Data does exist that the opposite is true, PCA use is associated with shorter length of stay. Education on this data and encouragement of PCA use with patients should be encouraged per expert recommendations. It does appear that PCA use is reserved for patients who are deemed “sicker” or have longer length of stay.We believe these trends are reflective of other institutions and broad education on best management of sickle cell pain crisis may be required. DisclosuresLandau:celgene: Speakers Bureau; sanofi: Speakers Bureau; alexion: Speakers Bureau; novartis: Speakers Bureau; BMS: Speakers Bureau.

Red Cell Exchange: A Concise Review of Indications and Management

▪Sickle cell disease (SCD), a common hemoglobinopathy in the United States affecting approximately one in 500 African-Americans, is associated with numerous potential complications related to both anemia and the presence of hemoglobin S (HbS). Red blood cell (RBC) transfusion and exchange (RCE) to improve oxygen carrying capacity and to treat or decrease the risk of complications due to sickling has been an important part of treatment for many years. While some treatment guidelines exist, there is still significant variation in the management of SCD patients.Fifteen physicians in the active practice of apheresis from 12 institutions representing different geographic regions within the United States discussed how they manage RCE. Additionally, a literature review was performed and data from a prior practice survey were evaluated.Simple transfusion is recommended to treat symptomatic anemia with hemoglobin <9 g/dL. RCE is indicated to prevent or reverse complications arising from the presence of HbS.Indications for RCE in SCD : Common indications include treatment of stroke, acute chest syndrome, and sickle hepatopathy. Primary and secondary stroke prevention is an accepted, well studied indication, although optimal duration of treatment is unknown. RCE may be indicated prior to planned surgical procedures, may be necessary during pregnancy in women who have a history of severe SCD-related complications, and is indicated prior to stem cell mobilization. Pain crisis in the absence of other symptoms is generally not accepted as an indication. The role of RCE is not determined for pulmonary hypertension, end stage renal disease, and progressive sickle retinopathy.Goals of RCE : The most important goals are to reduce the level of HbS while also preventing development of hyperviscosity. In most situations, a target HbS of ≤30% and target hemoglobin <10 g/dL (or hematocrit <30%) will treat and/or prevent SCD related complications without causing hyperviscosity.Access: Peripheral veins are the preferred means of access when possible. Treatment of acute complications of SCD usually requires a temporary central venous catheter. Options for long-term access include tunneled central venous catheters, implantable ports, or arteriovenous fistulas.RBC units: These should be HbS negative and leukodepleted. Units are commonly matched for C, E, and Kell antigens to help prevent alloimmunization in addition to being negative for antigens against which the patient has already formed antibodies. RBC phenotyping commonly is performed and molecular typing may be indicated in selected patients.Complications: These include risks related to central venous access, transfusion reactions, iron overload, alloimmunization, transfusion-transmitted infection, development of warm autoantibodies, and hyperhemolysis syndrome.There are many issues that need to be taken into consideration when evaluating a patient with SCD for transfusion treatment. A prior practice survey indicated that there are variations in the clinical management of these patients. While every case must be evaluated individually, we have presented some common practices. DisclosuresBoggio:Grifols: Consultancy, Honoraria.

Prevention of Acute Chest Syndrome By Implementing a Standardized Process to Improve Incentive Spirometry Use in Hospitalized Patients with Sickle Cell Disease

▪BackgroundAcute chest syndrome (ACS) is the second most common cause of admission and the main cause of death in patients with sickle cell disease (SCD). Nearly half of ACS cases develop during hospitalizations. Incentive spirometry (IS) use decreases the incidence of ACS during hospitalization. The 2014 NHLBI guidelines for the management of SCD encouraged the use of incentive spirometry in hospitalized patients to prevent the development of ACS (strong recommendation, moderate-quality evidence). Despite the available evidence and recommendations, IS is underutilized and the use of this simple, inexpensive, and effective intervention in hospitalized patients with SCD is inconsistent. We sought to develop and implement a standardized process to ensure a reliable use of IS in hospitalized patients with SCD and measure its effect on the occurrence of ACS.MethodsBased on the model for improvement, we implemented a standard process that involved several interventions to improve the use of IS in all hospitalized children and young adults with SCD at Cincinnati Children's Hospital Medical Center. Interventions included the development of an electronic medical record-based order set to standardize IS orders, supplying inpatient units with incentive spirometers for timely delivery, increasing awareness through education of staff, patients and families, discussing IS use during daily rounds, and standardization of the nursing documentation of IS use. The primary outcome was the percent ACS diagnosis per inpatient encounters per month.ResultsWe analyzed all SCD inpatient encounters one year before (April 2015-April 2016; n=224) and one year after (May 2016-May 2017; n=214) the implementation of the new process. The frequency of ACS diagnosis was reduced from 22% (50/224) pre-intervention to 12% (25/214) post-intervention (P=0.002). The median rate of ACS diagnosis per month was reduced from 23% to 12% and this reduction was maintained over one year post intervention. There was an improvement in the reliability of all measurable key processes targeted during this initiative. The percent of inpatient encounters with accurate IS orders increased from 28% to 88% and the percent of encounters with documented actual IS use by the patient improved from 41% pre-intervention to 81% post-intervention. There also was a trend of decreased length of stay from an average of 3.3 days in the 12 months preceding intervention to an average of 3.1 days in the year following the implementation of the new process. Possibly due to increased awareness for ACS among the clinical staff, there also was an unintended increase in the number of chest radiographs (CXR) performed from 16% pre-intervention (35 total CXR) to 37% post- intervention (70 total CXR). Despite this increase in the number of CXR performed, the diagnosis of ACS was still decreased by nearly half.DiscussionThrough the implementation of a standard process, reliable and sustained use of IS in hospitalized patients with SCD was associated with a decrease in the frequency of ACS diagnosis. Identifying and reliably executing key processes through a multidisciplinary approach was associated with an improved outcome and was maintained over a 12-month period. Automation of the new process will further improve its reliability and sustainability and allow for better assessment of its impact on morbidity, cost, and length of stay over a longer duration. DisclosuresNo relevant conflicts of interest to declare.

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.

Developing a Problem-Solving Intervention to Address Methods of Self-Management and Transition Challenges in Adolescents with Sickle Cell Disease: A Qualitative Investigation

Introduction: Given recent advances in medical care for children with Sickle Cell Disease (SCD), families are now seeing their children survive into adulthood. This presents new challenges around transition from pediatric to adult medical care, with responsibility for self-management shifting from parent to child. Interventions to improve self-management skills, such as problem solving education (PSE), have been used to increase self-efficacy and medication adherence in youth with other chronic diseases. The present study sought to explore common barriers and facilitators of disease management during the transition period and the acceptability of adapting PSE to improve transition readiness in adolescents with SCD.Methods: We purposively sampled adolescents with SCD ages 13 to 18 who received care at either Boston Medical Center (BMC) or Boston Children's Hospital and their primary caregiver to participate in focus groups. Each focus group was audio recorded and transcribed. Data were analyzed through use of grounded theory and coded with NVivo, a qualitative analysis software. Transcripts were double-coded until thematic saturation was achieved.Results: A total of 17 adolescents and 15 adult caregivers participated in five focus groups. Groups initially met together to discuss barriers and facilitators of self-management and then split into separate groups of teens and parents to discuss PSE. Teens and parents identified common methods of disease management includingtaking medication, staying warm and dressing appropriately, maintaining hydration and a healthy diet, and staying rested. Teens identified having a daily routine and sharing responsibility with a caregiver as facilitators of self-management. Caregivers commonly mentioned using a set of reminder techniques to help teens manage their health. Barriers to increasing teens' self-management included wanting to fit in with peers and lacking appropriate knowledge. Parents found it challenging to allow teens more responsibility.Parents and teens unanimously approved the adaption of PSE to SCD management and transition. Though a small number of parents stated low interest in participating either for social reasons or lack of perceived need, many identified the potential benefits of the intervention for both parents and teens. Parents felt that educational support would be most beneficial to them, while teens prioritized the benefits of emotional support during the transition period. Most participants would prefer that parents and teens meet separately in a group setting at the hospital or a comfortable environment outside of the home, then join together for a final session. Groups would be held on a weekly basis for a minimum of two months. However, some participants felt the support was imperative and thus could be effective for a year. Participants favored a mentor-style structure where a knowledgeable, college-aged individual with sickle cell disease would serve as the interventionist. This appeared to be a common preference for both parents and teens as they felt it was important to work with an individual that excelled independently and academically, despite any challenges with SCD.Parents and teens willingly identified specific scenarios where they felt PSE would be most valuable. Common PSE scenarios for teens were related to disclosing the diagnosis to peers, enhancing methods of management, and addressing identified barriers to transitioning. PSE scenarios for parents involved working with healthcare and school systems on select adaption issues and learning to “let go” of their teen.Conclusion: Though parents and teens have adapted management techniques to facilitate their transition from pediatric to adult medical care, barriers still exist across medical, personal and educational environments. Applying PSE as an intervention for both parents and teens of a sickle cell population could improve self-management and transition readiness in this vulnerable population. DisclosuresNo relevant conflicts of interest to declare.

Antisickling effect of chrysin is associated with modulation of oxygenated and deoxygenated haemoglobin via alteration of functional chemistry and metabolic pathways of human sickle erythrocytes.

Sickle cell disease (SCD) treatment and management remain a challenging puzzle especially among developing Nations. Chrysin's sickling-suppressive properties in human sickle (SS) erythrocytes in addition to its effect on AA-genotype erythrocytes were evaluated. Sickling was induced (76%) with 2% sodium metabisulphite at 3 h. Chrysin prevented (81.19%) the sickling and reversed same (84.63%) with strong IC50s (0.0257 µM and 0.00275 µM, respectively). The levels of oxygenated haemoglobin in the two groups (before and after induction approaches) were similar but significantly (P < 0.05) higher than that of SS erythrocytes (the 'induced' control), with chrysin-treated AA-genotype showing no effects relative to the untreated. The level of deoxygenated haemoglobin in the 'induced' control group was significantly (P < 0.05) higher than those of the chrysin-treated SS erythrocytes. Normal and chrysin-untreated erythrocytes (AA-untreated) were significantly more resistant to osmotic fragility than the SS-untreated. However, treatment with chrysin significantly reduced the osmotic fragility of the cells relative to the untreated cells. Furthermore, chrysin treatment significantly lowers the high level of 2,3-diphosphoglycerate (2,3-DPG) observed in the sickle erythrocytes, with no effects on AA-genotype erythrocytes. Based on functional chemistry, chrysin treatment alters the functional groups in favour of its antisickling effects judging from the observed bends and shifts. From metabolomics analysis, it was observed that chrysin treatment favors fatty acid alkyl monoesters (FAMEs) production with concomitant shutting down-effects on selenocompound metabolism. Thus, sickling-suppressive effects of chrysin could potentially be associated with modulation of oxygenated and deoxygenated haemoglobin via alteration of human sickle erythrocyte's functional chemistry and metabolic pathways implicated in SCD crisis.

Sickle Cell Disease and the Respiratory System: A Tangential Perspective to the Hematopulmonological Dilemma.

Sickle cell disease (SCD) is a genetically inherited hematological condition that predominantly affects the African-American subset of the population. It leads to the precipitation of multi-systematic manifestations throughout the course of the life of the patient leading to an increased rate of inpatient admissions and decreased quality of life. This article has reviewed some of the most common pulmonary complications of SCD with a brief overview of the clinical features and their management and has also highlighted the fatality of the complications placing a strong focus on screening, monitoring, and the treatment of the disease. The article has also discussed the management of SCD from a pulmonological perspective rather than hematological alone.

Acceptability of neonatal sickle cell disease screening among parturient women at the Paul Moukambi Regional Hospital in rural Eastern Gabon, Central Africa.

Neonatal screening and the effective management of sickle cell disease (SCD) are now well established in urban areas in some sub-Saharan African countries. The high rate of sickle cell trait in Koula-Moutou, Gabon, prompted an assessment of the psycho-clinical context of the introduction of neonatal screening in this rural area in eastern Gabon. Interviews were conducted with 215 women from February to June 2016 in Maternity and Maternal Child Protection services at the Paul Moukambi Regional Hospital Center in Koula-Moutou. Few childbearing women knew about SCD (24%), very few (6%) knew their hemoglobin status and only 30% of parturient women authorized sampling for neonatal SCD screening. Young mothers aged 16-28 years (p=0.018) and those who were educated (p=0.002) were more likely to authorize neonatal blood screening. There was no association between acceptance of blood sampling and knowledge of SCD or the parturient woman's hemoglobin status. The barriers to acceptance for SCD neonatal diagnosis are related to the education and culture rather than the knowledge of this disease. Introduction of diagnosis in rural areas requires a team comprising a psychosocial worker and health workers known to the rural population, to remove inhibitions related to blood collection from newborn infants.

Psychosocial risk and health care utilization in pediatric sickle cell disease.

Pain and complications related to pediatric sickle cell disease (SCD) are associated with higher health care utilization. In other pediatric chronic conditions, psychosocial screening can help identify children and families at risk of increased health care utilization to guide resource allocation, address treatment needs, and improve care. This study aimed to investigate the utility of psychosocial screening in predicting increased health care utilization among youth with SCD. Youth with SCD (n=74, 8-18years) and their parents were recruited from comprehensive SCD clinics. Parents completed the Psychosocial Assessment Tool (PAT), which categorized family psychosocial risk into one of three categories: Universal (minimal distress), Targeted (elevated distress), and Clinical (persistent distress). Youth reported on their pain characteristics, and health care utilization was extracted from medical chart review. Differences in health care utilization were evaluated using analysis of variance (ANOVA) and moderation analyses. Based on PAT risk, families were categorized into Universal (56.8%), Targeted (29.7%), and Clinical (13.5%) risk groups, with no significant group differences across demographic variables. Patients in the Targeted group reported significantly higher pain frequency than those in the Universal group (F[2, 66]=3.7, p<.05). The association between pain frequency and health care utilization significantly varied on the basis of psychosocial risk, such that Clinical psychosocial risk strengthened the connection between pain frequency and health care utilization (β=.2, t=2.1, p<.05). Integrating the PAT into routine clinical care may help health care providers identify families in need of greater psychosocial or medical support to further optimize SCD management.

"It Is Not Easy": Cultural Influences of Sickle Cell Disease Management in Rural, Eastern Sierra Leone.

Sickle cell disease (SCD) is a chronic genetic disease that causes life-threatening complications and requires robust comprehensive management. Developing comprehensive SCD programs in sub-Saharan African countries requires knowledge of the cultural factors affecting health-seeking behavior. We utilized an ethnographic approach and the frameworks of Dutta and Habermas to explore cultural factors influencing SCD management in rural Sierra Leone. A purposive sample of 27 individuals with SCD and their family and professional caregivers were observed and interviewed from March 2019 to April 2019. We identified four domains (Cultural Beliefs, Cultural Values, Cultural Practices, and Dealing with SCD) of cultural influences on SCD management, and 12 sub-themes (related to collectivism; spiritual, traditional, and Western beliefs and practices; and lived experiences) that reflect the personal, social, structural, and contextual complexities of SCD management. Further research regarding roles of traditional and spiritual leaders, combinations of Western and traditional practices, and culturally centered interventions is warranted.

Utilization of Pneumococcal Vaccine and Penicillin Prophylaxis in Sickle Cell Disease in Three African Countries: Assessment among Healthcare Providers in SickleInAfrica

Sickle cell disease is a genetic disease with a predisposition to infections caused by encapsulated organisms, especially Streptococcus pneumoniae. Pneumococcal vaccines and prophylactic penicillin have reduced the rate of this infection and mortality in sickle cell disease. However, implementation of these interventions is limited in Africa. The objectives of the study were to assess health care providers’ behaviors with the implementation of pneumococcal vaccination and penicillin prophylaxis and to identify barriers to their use. A 25-item online questionnaire was administered through SickleinAfrica: a network of researchers, and healthcare providers, in Ghana, Nigeria, and Tanzania, working to improve health outcomes of sickle cell disease in Africa. Data was collected and managed using the Research Electronic Data Capture (REDCap), tools and data analysis was done using STATA version 13 and R statistical software. Eighty-two medical practitioners responded to the questionnaire. Only 54.0 and 48.7% of respondents indicated the availability of published guidelines on sickle cell disease management and pneumococcal vaccine use, respectively, at their facilities. The majority (54.0%) perceived that the vaccines are effective but over 20.0% were uncertain of their usefulness. All respondents from Ghana and Tanzania affirmed the availability of guidelines for penicillin prophylaxis in contrast to 44.1% in Nigeria. Eighty-five percent of respondents affirmed the need for penicillin prophylaxis but 15.0% had a contrary opinion for reasons including the rarity of isolation of Streptococcus pneumoniae in African studies, and therefore, the uncertainty of its benefit. Lack of published guidelines on the management of sickle cell disease and doubts about the necessity of prophylactic measures are potential barriers to the implementation of effective interventions.