Sickle cell pain management

Sickle cell disease is the most common genetic hemoglobinopathy worldwide, characterized by a single-nucleotide mutation that predisposes to hemoglobin polymerization and erythrocyte sickling in hypoxic states. This report describes a 62-year-old male obese patient with a history of sickle cell disease, who presented with worsening nocturnal pain crises without any apparent triggering factor. A thorough evaluation at the outpatient department revealed obstructive sleep apnea. Airway obstruction or decreased respiratory effort during sleep may induce hypoventilation and hypoxia in the context of sleep-disordered breathing, with severe cardiopulmonary complications...

PURPOSE: This study assessed the feasibility of an in-home virtual reality intervention for chronic pain in adults with sickle cell disease. DESIGN: Two-group, parallel, randomized, multiple methods design with surveys, and interviews. METHODS: Participants were randomized to virtual reality or audio control, with 2-16-minute daily modules for 8 weeks, a daily pain diary survey, and a post study interview. Chronic pain and pain correlates were evaluated at baseline and every 4 weeks for 3 months...

Ketorolac, a nonsteroidal anti-inflammatory drug, is used in combination with opioids to manage vaso-occlusive episodes (VOEs). The relationship between ketorolac use and kidney injury in pediatric patients with sickle cell disease (SCD) remains incompletely understood. We hypothesize that ketorolac is associated with acute kidney injury (AKI) in patients with SCD presenting with pain. All nonsurgical hospitalizations for VOEs treated with ketorolac between January 2014 and December 2022 were included. We used optimal matching methodology to identify control admissions (2:1 ratio) and used nonparametric tests to compare ketorolac administration between cases and controls...

In this short review we have presented and discussed studies on pharmacogenomics (also termed pharmacogenetics) of the drugs employed in the treatment of β-thalassemia or Sickle-cell disease (SCD). This field of investigation is relevant, since it is expected to help clinicians select the appropriate drug and the correct dosage for each patient. We first discussed the search for DNA polymorphisms associated with a high expression of γ-globin genes and identified this using GWAS studies and CRISPR-based gene editing approaches...

The coexistence of diabetes mellitus (DM) and sickle cell anemia (SCA) poses significant challenges in clinical management due to the complex interactions and overlapping complications associated with both conditions. Managing diabetes in individuals with SCA requires a comprehensive approach that addresses the unique physiological and pathological aspects of both diseases. This paper reviews the challenges encountered in the management of DM in patients with SCA and explores therapeutic strategies and approaches to optimize patient care...

This report of two cases confronts the longstanding perception of Sickle Cell Trait (SCT) as a clinically benign condition, highlighting its complex and severe clinical manifestations, particularly in the context of blood loss anemia and vaso-occlusive crises (VOCs). The hallmark of sickle cell disease is the severe pain caused by acute vaso-occlusion of the microvasculature that leads to bone marrow infarction. We report two cases of patients with SCT and severe anemia in the setting of blood loss secondary to uterine fibroids subsequently causing VOCs with likely bone sequestration...

OBJECTIVES: The psychosocial aspects of chronic pain among youth with sickle cell are poorly described and may be better understood within a biopsychosocial model of chronic pain as applied to youth living with sickle cell disease. DESIGN: A systematic literature review was performed to synthesize the psychosocial factors contributing to chronic pain in this population. Criteria for study inclusion were primary quantitative research studies focused on psychosocial aspects of chronic pain among youth with sickle cell disease...

Crizanlizumab, a monoclonal antibody against P-selectin, has been shown to reduce vaso-occlusive crises (VOCs) compared to placebo in patients ≥ 16 years with sickle cell disease (SCD). However, there have been rare reports of patients experiencing severe pain and subsequent complications within 24 hours of crizanlizumab infusions. These events are defined as infusion-related reactions (IRRs). Informed by current literature and clinical experience, a group of content experts developed clinical guidelines for the management of IRRs in patients with SCD...

Background: Painful vaso-occlusive episodes (VOEs) are the hallmark of sickle cell disease (SCD) and account for frequent visits to the emergency department (ED) or urgent care (UC). Currently, the early administration of analgesics is recommended as initial management; however, there is a need for further understanding of the effect of prompt analgesics and hydration during VOEs. The objective of this study is to analyze the factors associated with the rate of hospital admission in the setting of time to intravenous (IV) analgesics and hydration...

Sickle cell disease is characterised by episodes of vaso-occlusive crisis, a painful complication. Regional anaesthesia has shown promising results in reducing opioid consumption and pain scores. Patients with vaso-occlusive crises who underwent regional anaesthesia in the paediatric intensive care unit were studied. Data regarding pain location, regional analgesia technique, the local anaesthetic used and dose, daily opioid consumption, daily pain scores, use of adjuvants and complications were recorded. The primary outcome was to evaluate the effect of regional anaesthesia on opioid consumption...

BACKGROUND: Tissue necrosis from pentazocine abuse is becoming a burden in our environment. Pentazocine is an opioid of the benzomorphan class. It is commonly used for post-traumatic and postoperative pain and vaso-occlusive pain of sickle cell disease (SCD). Its prolonged use can lead to addiction and may result in severe injection site necrosis, often worsened by infection due to a lack of aseptic principles during administration. Although pentazocine is a controlled drug in our environment, it is readily available...

The acute pain crisis (APC) is the commonest complication of sickle cell disease (SCD). Severe episodes may require treatment in hospital with strong opioid analgesic drugs, combined with additional supportive care measures. Guidelines for APC management have been produced over the past two decades gathering evidence from published studies, expert opinion, and patient perspective. Unfortunately, reports from multiple sources indicate that guidelines are often not followed, and that acute care in emergency departments and on acute medical wards is suboptimal...

Sickle cell disease (SCD) has been identified as one of the most prevalent genetic conditions. It alters the shape and function of red blood cells. This brief case report presents a case of a five-year-old male with sickle cell disease who complained of pain in the left mandibular region due to deep proximal caries. Before dental management, a complete fitness evaluation was performed with the help of a pediatrician, followed by informed consent. Dental management includes pulpectomy followed by stainless steel crown placement and Glass ionomer cement (GIC) restoration for superficial caries...

Sickle cell disease (SCD) is a genetic disorder causing painful and unpredictable Vaso-occlusive crises (VOCs) through blood vessel blockages. In this study, we propose explosive synchronization (ES) as a novel approach to comprehend the hypersensitivity and occurrence of VOCs in the SCD brain network. We hypothesized that the accumulated disruptions in the brain network induced by SCD might lead to strengthened ES and hypersensitivity. We explored ES's relationship with patient reported outcome measures (PROMs) as well as VOCs by analyzing EEG data from 25 SCD patients and 18 matched controls...

AIM: Sickle cell disease has witnessed a 41.4% surge from 2000 to 2021, significantly affecting morbidity and mortality rates, particularly in children from regions with elevated under-5 mortality rates. Gut microbiota dysbiosis is increasingly recognised in SCD, exacerbating complications, particularly chronic pain, marked by significant alterations of proinflammatory bacteria abundance. This review explores the therapeutic potential of Akkermansia muciniphila and Roseburia spp. in alleviating SCD-related complications, emphasising their roles in maintaining gut barrier integrity, reducing inflammation, and modulating immune responses...

Background: Sickle cell disease (SCD) is an autosomal, recessive, genetic condition of the sickle cell genes. It affects about 100 000 people in the United States where an estimated 1 out of every 365 black children and 1 out of every 13 black children will be born with SCD and sickle cell trait, respectively. Severe and unpredictable pain crisis are the leading cause of emergency department visit for adult patients with SCD and account for 90% of inpatient hospitalizations and 85% of all acute medical care, as well as high usage of medical resources...

Sickle cell disease is a hemoglobinopathy often complicated by painful vaso-occlusive episodes, acute chest syndrome, stroke, and myocardial infarction. Sickle cell intrahepatic cholestasis (SCIC) is a rare and potentially fatal complication of sickle cell disease. SCIC is thought to involve progressive hepatic injury due to sickling within sinusoids. We present the case of a young patient with SCIC and acute liver failure, requiring prompt treatment with exchange transfusion. Our case describes features that should raise suspicion for hepatic failure in SCIC and highlights exchange transfusion as a successful management approach in similar patients with an otherwise high risk of mortality...

Acute painful vaso-occlusive crisis (VOC) is the common presentation of sickle cell disease (SCD) leading to emergency room visits, admissions, morbidity, mortality, and negative impacts on quality of life. Among various treatment approaches commonly employed to manage the condition, intravenous (IV) hydration is also frequently used in emergency and inpatient settings. Although helpful to overcome dehydration, IV hydration often leads to adverse outcomes like fluid overload, pulmonary edema, increased length of stay, transfer to intensive care unit, new oxygen requirement, etc...

BACKGROUND: In patients who experience frequent vaso-occlusive crises (VOC), opioid dependence may be due to a need for pain control as opposed to addiction; the implications of opioid use disorder (OUD) in this population are unclear. OBJECTIVE: To compare outcomes in hospitalizations for VOC in those with a history of OUD to those without a history of OUD. DESIGN: A retrospective assessment of hospitalizations for adults in the USA with a primary discharge diagnosis of VOC using the National Inpatient Sample database from 2016 to 2019...

Sickle Cell Disease (SCD) is a severe genetic disorder causing vascular occlusion and pain by upregulating the adhesion molecule P-selectin on endothelial cells and platelets. It primarily affects infants and children, causing chronic pain, circulatory problems, organ damage, and complications. Thus, effective treatment and management are crucial to reduce SCD-related risks. Anti-P-selectin antibody Crizanlizumab (Crimab) has been used to treat SCD. In this study, the heavy and light chain (HC and LC) genes of anti-P-Selectin antibody Crimab were cloned into a plant expression binary vector...