A sickle cell crisis is a painful episode that may begin suddenly in a person who has sickle cell disease. A sickle cell crisis occurs when sickle-shaped red blood cells clump together and block small blood vessels that carry blood to certain organs, muscles, and bones. This causes mild to severe pain. The pain can last from hours to days. "Painful event" and "painful crisis" are other terms used to describe these episodes. Some people who have sickle cell disease have many painful events, while others have few or none. The pain can happen in any part of the body. But the most common areas include the: • Bones of the spine • Bones in the arms and legs • Chest • Abdomen   Abstract source

After a recent stint in the hospital, I began researching documentaries and photo series about sickle cell. Disappointed by what I found - sad stories, stories that narrow in on the pain and, stories that tell how there is no cure; I decided it was time for a different narrative. One of strength, hope and joy. People living with sickle cell often call one another warriors. So I contacted my closest warrior friends and began discussing what it means to be a Sickle Cell Warrior. Being born with Sickle Cell Anemia has its ups and downs. This disease has humbled me, and through that humility I've developed a better spiritual relationship with GOD. My image represents a fighter who wins. Related content

“I'm 32 years old and have lived with sickle cell disease my entire life. Because it's a genetic disease and I was born with it, it really becomes part of your way of life. I've tried to live a normal life as much as possible. I had a stroke at 2 years old and after that, I was receiving at least a unit of blood every month to prevent more from happening. If I was in a crisis, I would sometimes need even more blood and be admitted into the hospital. I've had countless blood transfusions, and without them, I know for a fact I would not be here today. I guess you could say I’ve been a professional sickle cell advocate for the past several years. Sickle cell is what I call the invisible disease. You can tell people, ‘Oh, I'm in so much pain,’ but you can't totally see it. Related story

Marie was born in Kenya to a single mother, Josephine Ojiambo. She eventually had two younger siblings, but she bonded most closely with her older brother Peter. They were just one year apart. “We sheltered each other… and went through life teaching each other.” Their experiences were very similar. They went to the same schools. They had the same friends. And they both were born with sickle cell anemia, caused by a mutation in a single gene that turns red blood cells into rigid crescent or sickle shapes, instead of soft discs. Marie’s mother founded the Peter Ojiambo Foundation, of which Marie and her sisters are board members. Marie had done extensive work in the nonprofit space before this, focusing on advocacy and education for sickle cell disease with the Sickle Strong Initiative. Since beginning her advocacy work, the number of treatment options for sickle cell have significantly increased. Related story

As life gets more hectic, it is all too easy to go without sleep. In fact, many Americans only get 6 hours of sleep a night or less. You need ample sleep to help restore your brain and body. Not getting enough sleep can be bad for your health in a number of ways. Sleep gives your body and brain time to recover from the stresses of the day. After a good night's sleep, you perform better and are better at making decisions. Sleep can help you feel more alert, optimistic, and get along with people better. Sleep also helps your body ward off disease. Different people need different amounts of sleep. Most adults need 7 to 8 hours of sleep a night for good health and mental functioning. Some adults need up to 9 hours a night.   Abstract source

The podcast is presented by sickle cell warrior Ade Adeyokunnu. He is also an advocate for sickle cell. He is also an ambassador of Global Blood Therapeutics. Why I became an advocate for Sickle Cell Disease? The fact that I have it and my sister also has it is the reason for why I became an advocate for the disease. While I was at school, I felt lonely because I didn’t find anyone else going through the same things as I was going through with sickle cell. That was what drove me to start my advocacy for sickle cell and eventually lead me to create this online community for people living with sickle cell back in 2009. Related story

Sickle cell disease (SCD) is associated with increased risk of poor health outcomes from respiratory infections, including COVID-19 illness. We used US death data to investigate changes in SCD-related mortality before and during the COVID-19 pandemic. We estimated annual age- and quarter-adjusted SCD-related mortality rates for 2014-2020. We estimated the number of excess deaths in 2020 compared with 2019 using the standardized mortality ratio (SMR). We found 1023 SCD-related deaths reported in the United States during 2020, of which 86 (8.4%) were associated with COVID-19. SCD-related deaths, both associated and not associated with COVID-19, occurred most frequently among adults aged 25-59 years. The SCD-related mortality rate changed <5% year to year from 2014 to 2019 but increased 12% in 2020; the sharpest increase was among adults aged ≥60 years. The SMR comparing 2020 with 2019 was 1.12 (95% CI, 1.06-1.19). Overall, 113 (95% CI, 54-166) excess SCD-related deaths occurred in 2020. Abstract source

Background: Sickle cell disease is characterized by the painful recurrence of vaso-occlusive events. Gene therapy with the use of LentiGlobin for sickle cell disease (bb1111; lovotibeglogene autotemcel) consists of autologous transplantation of hematopoietic stem and progenitor cells transduced with the BB305 lentiviral vector encoding a modified β-globin gene, which produces an antisickling hemoglobin, HbAT87Q. Methods: In this ongoing phase 1-2 study, we optimized the treatment process in the initial 7 patients in Group A and 2 patients in Group B with sickle cell disease. Group C was established for the pivotal evaluation of LentiGlobin for sickle cell disease, and we adopted a more stringent inclusion criterion that required a minimum of four severe vaso-occlusive events in the 24 months before enrollment. In this unprespecified interim analysis, we evaluated the safety and efficacy of LentiGlobin in 35 patients enrolled in Group C. Included in this analysis was the number of severe vaso-occlusive events after LentiGlobin infusion among patients with at least four vaso-occlusive events in the 24 months before enrollment and with at least 6 months of follow-up. Results: As of February 2021, cell collection had been initiated in 43 patients in Group C; 35 received a LentiGlobin infusion, with a median follow-up of 17.3 months (range, 3.7 to 37.6). Engraftment occurred in all 35 patients. The median total hemoglobin level increased from 8.5 g per deciliter at baseline to 11 g or more per deciliter from 6 months through 36 months after infusion. HbAT87Q contributed at least 40% of total hemoglobin and was distributed across a mean (±SD) of 85±8% of red cells. Hemolysis markers were reduced. Among the 25 patients who could be evaluated, all had resolution of severe vaso-occlusive events, as compared with a median of 3.5 events per year (range, 2.0 to 13.5) in the 24 months before enrollment. Three patients had a nonserious adverse event related or possibly related to LentiGlobin that resolved within 1 week after onset. No cases of hematologic cancer were observed during up to 37.6 months of follow-up. Conclusions: One-time treatment with LentiGlobin resulted in sustained production of HbAT87Q in most red cells, leading to reduced hemolysis and complete resolution of severe vaso-occlusive events. (Funded by Bluebird Bio; HGB-206 ClinicalTrials.gov number, NCT02140554.). Abstract source Full text

Regina Hartfield is President/CEO of the Sickle Cell Disease Association of America, Inc. (SCDAA). Prior to joining the Sickle Cell Disease Association of America, Hartfield was a manager for federal, state and commercial contracts for CVP, an information technology consulting firm in Virginia and Maryland. During her time at CVP, she developed strategic outreach initiatives, wrote strategy and implementation plans for the national expansion of a federal health agency’s program and managed a $30-million disaster recovery grant contract for the state of Tennessee, overseeing staff and operations of CVP’s regional office. Hartfield serves on advisory boards and is a member of the Universal Sailing Club, where she has served on its board in different capacities. Hartfield graduated with honors from Lehman College of the City University of New York with a Bachelor of Arts in community outreach. Online profile