Sickle cell disease (SCD) is among the most common genetic diseases in the United States, affecting approximately 100,000 people. In the United States, SCD is characterized by a shortened life expectancy of only about 50 years in severe subtypes, significant quality-of-life impairments, and increased healthcare utilization and spending. SCD is characterized by chronic hemolytic anemia, vaso-occlusion, and progressive vascular injury affecting multiple organ systems. The pathophysiology is directly related to polymerization of deoxygenated hemoglobin, leading to a cascade of pathologic events including erythrocyte sickling, vaso-occlusion, tissue ischemia, and reperfusion injury as well as hemolysis, abnormal activation of inflammatory and oxidative pathways, endothelial dysfunction, increased oxidative stress, and activation of coagulation pathways. These multifactorial abnormalities have both acute and chronic clinical consequences across multiple organ systems, including acute pain episodes, chronic pain syndromes, acute chest syndrome, anemia, stroke and silent cerebral infarcts, cognitive dysfunction, pulmonary hypertension, and a wide range of other clinical consequences. Abstract source

Tapping Into the Warrior Mentality My life has been characterized by tenacity, perseverance and determination; these values have been instilled in me through overcoming several challenges and obstacles. The role with the utmost significance to me is that of being a patient advocate for people with sickle cell disease. Being born with this illness myself, I felt compelled to be of service in this area. In 2009, I created the online community, sikcell.com, where people affected by sickle cell disease can relate to others who grapple with similar hardships. This platform established an avenue for people across the globe to connect with others who understand their pain, to share experiences that uplift each other, and to inspire in times of desolation. I am always delighted to receive messages on how the site has had a positive impact on the lives of and has inspired members not to give up through their adversities. Although sickle cell tries to prevent me from achieving my goals, through my determination I decided not to be a victim of it. Dealing with the unforeseen pain, hospitalizations and other complications of this condition has played an important role in shaping me into a resilient, resourceful and ambitious individual. This mentality has enabled me to achieve my goals of completing graduate school twice and advancing in my career. My life experiences and perspective have also prepared me to be of value to my community and the society at large. My resolve from overcoming strenuous conditions has been effective in encouraging others through challenging times, such as the current pandemic. Let’s all tap into our warrior mentality to help get us through these unprecedented times together!   Ade Adeyokunnu Founder of Sikcell.com and @sikcell on Twitter Philadelphia, Pennsylvania

Define yourself, define your life. No matter how old you are or how severe your sickle cell disease is, you can start where you are and learn more about sickle cell disease. The more you know, the more options you have and more options exist now than ever before. Advances are being made, and there is hope. This booklet was inspired, designed and created by your brothers and sisters in the sickle cell community to encourage, uplift and inform you. We want you to know you are not alone. Sickle cell is part of our lives, but it does not define us and it cannot break us. Join us as we redefine what it means to live with sickle cell.   Abstract source

This handbook was developed to answer common questions about BMT. Please share any questions you may have after reviewing this brochure with your health care team. In the past, only children who had major complications from their sickle cell disease (such as a stroke) were eligible for BMT. Since we know most children do very well after BMT using a matched sibling donor, more children are undergoing BMT today. In addition, we are now learning that adult sickle cell patients can tolerate BMT better than we expected with positive results, particularly those with a matched sibling donor. Most children and many adults with sickle cell disease may be eligible for BMT if they have a matched sibling donor. What if I or my child do not have a matched sibling?   Abstract source

In this installment, The Hematologist Editor-in-Chief Dr. Laura Michaelis has a conversation with Dr. Ifeyinwa (Ify) Osunkwo of The Levine Cancer Institute/Atrium Health. They discuss unique issues facing the sickle cell disease population during the COVID-19 public health crisis as well as some benefits of using telemedicine and telementoring.   Related story

Objectives: The hallmark of sickle cell disease (SCD) is acute and chronic pain, and the pain dominates the clinical characteristics of SCD patients. Although pharmacological treatments of SCD targeting the disease mechanisms have been improved, many SCD patients suffer from pain. To overcome the pain of the disease, there have been renewed requirements to understand the novel molecular mechanisms of the pain in SCD. Methods: We concisely summarized the molecular mechanisms of SCD-related acute and chronic pain, focusing on potential drug targets to treat pain. Results: Acute pain of SCD is caused by vaso-occulusive crisis (VOC), impaired oxygen supply or infarction-reperfusion tissue injuries. In VOC, inflammatory cytokines include tryptase activate nociceptors and transient receptor potential vanilloid type 1. In tissue injury, the secondary inflammatory response is triggered and causes further tissue injuries. Tissue injury generates cytokines and pain mediators including bradykinin, and they activate nociceptive afferent nerves and trigger pain. The main causes of chronic pain are from extended hyperalgesia after a VOC and central sensitization. Neuropathic pain could be due to central or peripheral nerve injury, and protein kinase C might be associated with the pain. In central sensitization, neuroplasticity in the brain and the activation of glial cells may be related with the pain. Abstract source

Sleep disturbance is common among children with sickle cell disease (SCD) and is related to neurocognitive difficulties. However, research on sleep disturbances and related variables among adults with SCD is extremely limited. The present study examined the relationship between sleep, executive functioning, and emotional functioning among 62 adults (29 females; M age = 32 years, SD = 7.79) with SCD preparing to undergo a stem cell transplant. Participants were administered a neurocognitive evaluation that included objective and subjective measures of executive functioning, and they completed PROMIS self-report measures of anxiety, depression, and pain intensity. Results showed that about 17% of participants endorsed clinically significant sleep disruptions, while 16.1% and 8% endorsed clinically significant symptoms of anxiety and depression, respectively. Sleep disturbance in these adults was not significantly correlated with objective or subjective measures of executive functioning. Moreover, anxiety, but not depression, was a significant mediator between self-reported sleep difficulties and both objective and subjective measures of executive functioning while controlling for pain intensity. Future research on sleep interventions will be essential for ameliorating the effects of sleep disturbance on executive functioning and anxiety among adults with SCD. Abstract source

This paper reports on the findings of a study designed to establish website‐based self‐management recommendations for sickle cell disease. Google and Yahoo search engines were used to search the World‐Wide‐Web. Purposive sampling was utilized to select 28 websites that met the inclusion criteria. Data were manually collected from health education materials and subjected to qualitative content analysis. Self‐management was conceptualized as actions involving preventive health, self‐monitoring, self‐diagnosing, and self‐treatment. The results show that the websites recommend more self‐management actions for preventive health and self‐treatment than for self‐monitoring and self‐diagnosis. Frequent oral fluid intake, limitation of overactivity, eating a healthy diet, avoiding extreme temperatures, and infections were the commonest preventive health recommendations. Abstract source

Importance: Sickle cell disease (SCD) is the most common inherited red blood cell disorder in the United States, and previous studies have shown that individuals with SCD are affected by multiple health disparities, including stigmatization, inequities in funding, and worse health outcomes, which may preclude their ability to access quality health care. This needs assessment was performed as part of the Sickle Cell Disease Implementation Consortium (SCDIC) to assess barriers to care that may be faced by individuals with SCD. Results: The SCDIC sites did not report the number of individuals approached to participate in this study; thus, a response rate could not be calculated. In addition, respondents were not required to answer every question in the survey; thus, the response rate per question differed for each variable. Of 440 individuals with SCD, participants were primarily female (245 [55.7%]) and African American (428 [97.3%]) individuals, with a mean (SD) age of 27.8 (8.6) years. The majority of participants (306 of 435 [70.3%]) had hemoglobin SS or hemoglobin S β0-thalassemia. Most respondents (361 of 437 [82.6%]) reported access to nonacute (usual) SCD care, and the majority of respondents (382 of 413 [92.1%]) noted satisfaction with their usual care physician. Abstract source

Introductions: Sickle cell disease (SCD) is the most commonly inherited blood disorder in the United States, and it predominately affects the Black community. SCD is characterized by a number of symptoms, including unpredictable pain, which can lead to hospitalizations. Data indicate that people with SCD experience racism and ample data indicate that racism predicts depressive symptoms and impaired health-related quality of life (HRQOL). In contrast, research suggests that social support might buffer the impact of racism on depression. The aim of this study was to explore associations among perceived racism, depressive symptoms, and HRQOL in adolescents with SCD. Consistent with the literature, it was predicted that perceived racism would predict HRQOL through depression, and social support would moderate the negative impact of racism on depression. Methods: In a cross-sectional design, 75 inpatient adolescents with SCD completed measures of perceived racism, depressive symptoms, HRQOL, and social support. Results: This results indicated a moderated mediation model with greater perceived racism predicting more depressive symptoms, which in turn related to poorer HRQOL; greater access to social support from individuals with SCD strengthened the perceived racism—depressive symptom association. Abstract source