Author Archives: Emma Day

MARAC Advisory: COVID-19 and Sickle Cell Disease

March 2022 – The Sickle Cell Disease Association of America’s Medical and Research Advisory Committee notes that news about COVID-19 continues to emerge. Monitor your state or local health department for more information. Below are some updates and suggestions for individuals with sickle cell disease.

Several studies have examined the effects of COVID-19 infection in individuals in the sickle cell community and shown concerning results to include:

  • Individuals with sickle cell disease do have higher risks for severe COVID-19 disease and hospitalization compared to those of the same age without sickle cell disease. Poor kidney function raises the risk for severe COVID-19. A blood flow problem in the lungs called pulmonary hypertension also raises the risk for severe COVID-19.
  • COVID-19 infection often triggers sickle vaso-occlusive pain in adults. This type of crisis can cause the most intense pain an individual with sickle cell has ever experienced.
  • COVID-19 infection can also trigger sickle acute chest syndrome. Not everyone gets the same problems and children might have mild cases, but severe disease and death are unpredictable.
  • The American health care system has not solved the issues of racial and ethnic disparities in health
    during the pandemic. People of color have a higher risk of COVID-19 complications and are dying from COVID-19 at younger ages.

The positive news is:

  • The risk of death from COVID-19 for those with sickle cell disease is not as high as the risk of death from COVID-19 in patients who are receiving an organ transplant or are in active chemotherapy for cancer.
  • Sickle cell trait does not present a higher risk of severe COVID-19 or death.

The COVID-19 vaccine is safe, and the vaccine’s risks to individuals with sickle cell disease are very low when compared to the risks of a COVID-19 infection.

COVID-19 vaccines have now been given to tens of thousands of people with sickle cell disease across many countries for at least 14 months. People with sickle cell report about one to two days of side effects, which is about the same as reports from the general population.

SCDAA MARAC members have found that very rare cases of sickle cell pain have been reported after vaccination for COVID-19.

ACTIONS YOU CAN TAKE

  1. Stay up to date with your COVID-19 vaccines.
  2. If you feel comfortable wearing a mask, you can. You are at risk. Don’t be bullied.
  3. Continue medications and preventive care for sickle cell disease.
  4. Seek care when needed. Do not delay getting care for your medical condition. Health care professionals have infection prevention plans to help protect you from getting COVID-19 if you need care. If you have sickle cell disease and do not have a regular doctor, we strongly recommend that you connect with one.
  5. Keep up with your state or local health department for more information.

TEST SOON AND TREAT EARLY (CDC infographic in English and Spanish)

  1. Americans can request free COVID-19 tests mailed to your home. To learn more, visit COVIDtests.gov.
  2. If you have symptoms that are suspicious for COVID-19, take a home COVID-19 test or get tested in a medical facility.
  3. Several treatment options are now available for an individual with sickle cell disease who tests positive for COVID-19. Treatments can prevent a mild infection from leading to severe disease, hospitalization or death. Click here to learn more. Ask your doctor or pharmacist for the treatment that is used for your location, age and medical condition.

This statement has been provided by members of the Sickle Cell Disease Association of America’s Medical and Research Advisory Committee.

Download this statement.

SCDAA partners with Phi Beta Sigma Fraternity

The Sickle Cell Disease Association of America (SCDAA) partnered with Phi Beta Sigma Fraternity Inc. to increase awareness about sickle cell disease and raise funds to support education about the negative effects of sickle cell disease on the physical, economic and social well-being of individuals with the condition.

“SCDAA is excited to again partner with the Phi Beta Sigma Fraternity, and we are honored to have been chosen as a recipient of their donations,” said Regina Hartfield, president and CEO of SCDAA. “Phi Beta Sigma is deeply committed to serving and supporting the community, as is SCDAA. Their support will go far to benefit those living with sickle cell disease.”

The partnership will support the SCDAA and sickle cell community through raising funds, mobilizing blood and bone marrow drives and collaborating to educate the community about sickle cell disease and sickle cell trait. Phi Beta Sigma Fraternity will encourage chapters to collaborate with their local American Red Cross to host blood drives and the Be The Match Registry to host bone marrow drives. Chapters will conduct screenings to help adolescents and adults detect sickle cell and organize fundraisers for sickle cell research, education and children’s services.

“SCDAA is proud to continue its partnership with Phi Beta Sigma in raising awareness about sickle cell disease,” said Thomas L. Johnson, chair of the SCDAA board of directors. “Its dedication to the mission of SCDAA is exemplary of providing support and assistance for those in need.”

Phi Beta Sigma Fraternity was founded at Howard University in Washington, D.C., in 1914 to exemplify the ideals of brotherhood scholarship and deliver services to the general community. The fraternity’s motto is “Culture for Service and Service for Humanity.”

“This disease effects one out of every 365 Black or African-American births and one out of every 16,300 Hispanic-American births,” said Chris Rey, president of the fraternity. “Phi Beta Sigma is dedicated to doing all we can to change these grim numbers. We are excited to continue our partnership with SCDAA to continue to fight for a cure.”  

SCDAA Celebrate Black History Month: Prodigy

Our final #BlackHistoryMonth highlight is dedicated to rapper and sickle cell warrior Albert “Prodigy” Johnson, one-half of the iconic 90’s rap duo Mobb Deep. Prodigy was born in the 1970s to a family of musicians and was diagnosed with sickle cell SS at three months old. He grew up in Queens, New York City, and formed Mobb Deep with his partner Kejuan “Havoc” Muchita while attending the High School of Art and Design in Manhattan. They released their breakthrough album “The Infamous” in 1995, which is widely recognized as a hip-hop classic.
 
Mobb Deep’s street-smart rap style and emotionally charged lyrics are credited with the renaissance of the East Coast hip-hop scene. Mariah Carey, Jordan Knight, Kanye West and Jay Z have all sampled tracks from the group.
 
Prodigy made music with Mobb Deep and on a solo basis until the mid-2010s. He lost his battle to sickle cell in 2017, but his music lives on. Hear what he had to say about living with sickle cell in this 2011 interview.

 

SCDAA Celebrates Black History Month: Paul Williams

Meet Paul Williams, one of the founding members of the legendary Motown group The Temptations. Williams began singing as a kid with Eddie Kendricks in their church choir in Birmingham, Alabama. The two pursued music careers as members of various groups, eventually moving to Detroit to join Otis Williams, Melvin Franklin and Elbridge Bryant as The Temptations.

With Williams on board, the group released many hits, including “My Girl.” Williams was considered the best dancer in the group and choreographed many of their moves. He also served as the original choreographer for The Supremes. His battle as a sickle cell warrior was hidden from the public, and his health issues eventually caused him to leave the group. Williams’ indelible impact helped cement The Temptations’ place in history as one of the greatest vocal groups in modern times and trailblazers in the evolution of R&B and soul music.

 

SCDAA Celebrates Black History Month: Billy Garrett, Jr.

Happy Black History Month! Sickle cell can be painful and hard to manage, but it doesn’t need to stop you from following your dreams. This month, we’ll share the inspirational stories of household names and notable figures who didn’t let sickle cell hold them back. 

Meet Billy Garrett Jr., the first known person with sickle cell disease to play in the NBA. Billy grew up in Chicago, playing competitive basketball despite words of caution from his doctors. To keep playing, Garrett learned to balance hydration, rest and exertion through a process of trial and error that would occasionally lead to crises. His passion and dedication carried him through these obstacles and onto the court as a member of the DePaul Blue Demons, the New York Knicks and the Lakeland Magic. Off the court, we’re honored to have him on our team as one of our celebrity ambassadors. Hear his story and how he learned to play despite the pain.

 

SCDAA Celebrates Black History Month: Miles Davis

Happy Black History Month! Sickle cell can be painful and hard to manage, but it doesn’t need to stop you from following your dreams. This month, we’ll share the inspirational stories of household names and notable figures who didn’t let sickle cell hold them back. 

We kick off our celebration recognizing world-renowned jazz artist Miles Davis. He struggled with health issues – arthritis, hip-joint problems and pneumonia – but wasn’t diagnosed with sickle cell disease until middle age. He began playing the trumpet at age 13 and at 18 convinced his parents to allow him to move to New York City to study at the prestigious Juilliard School. It was a ploy so he could jam with the musical masters of his day and join the band of his idol, Charlie Parker. He was soon leading his own projects and bands, solidifying a career that spanned decades. His album “Kind of Blue” is one of the most successful and influential jazz albums in history. He passed away in 1991, but his music lives on. Take some time to listen to his music today and pay tribute to this influential sickle cell warrior.

 

In Memory of Carlton Haywood, Jr., Ph.D.

We are devastated to share the news of the passing of Carlton Haywood, Jr., Ph.D., on December 31, 2021. Carlton was an accomplished academic whose research focused on bioethics and sickle cell. His experiences as a sickle cell warrior guided his work as a scholar and an advocate. As such, Carlton was a well-respected and celebrated voice in both the sickle cell and research communities.
Carlton grew up in Atlanta, Georgia, and never let sickle cell get in the way of his achievements. He received a bachelor’s and master’s degree from the University of Virginia and completed a doctorate of philosophy in health policy and bioethics in the department of health policy and management at the Johns Hopkins Bloomberg School of Public Health. After completing his studies, he became a faculty member at the Berman Institute and the department of hematology at the Johns Hopkins School of Medicine.
“Carlton had a unique perspective as both an academic professor at a prestigious institution and an individual living with sickle cell disease who could link the academic theories to ‘lived experience,'” said Lewis Hsu, M.D., chief medical officer of the Sickle Cell Disease Association of America. “He generated some of the first scholarly publications on the injustices suffered by individuals with sickle cell disease. He called attention to issues that inhibit sickle cell disease care and research and worked effectively to resolve them. His quiet determination was inspiring to me.”
He was a dedicated sickle cell advocate and spent his career studying important issues to the sickle cell community, including patient-centered care and trust in the medical industry. His research also addressed the intersections of bioethics and clinical research, and his work earned him national recognition and helped inform sickle cell policy. We invite you to read more about Carlton’s life and career in this article.
SCDAA mourns the loss of Carlton, and we send our deepest condolences to his family and friends. A memorial will be planned at a later date.

MARAC Advisory Statement: Update About COVID-19

December 23, 2021 – The Sickle Cell Disease Association of America’s Medical and Research Advisory Committee reminds the sickle cell community that the COVID-19 pandemic is having another increase in infections. New variants like omicron are emerging, as are common for RNA viruses, and vaccination rates need to consequently improve. Vaccinations can protect against severe illness. A new medication might also help as early treatment for infected individuals.

MARAC Recommendations

  • No general recommendation for all individuals with SCD to stay home nor for all to return to in-person activities. Patients and families need to make individualized assessments of the risks and trade-offs of returning to work or school in person. Factors to consider include vaccination status, the rate of COVID-19 and variants in your community, family socioeconomic situation, protective measures in the building (proper ventilation, physical distancing, etc.), mental health needs and educational needs.
  • Rare vaccine-related side effects, such as pain crises, have been reported after receiving a COVID-19 vaccine. However, they are not more likely to occur as a result of the COVID-19 vaccine compared to other common vaccines. No serious adverse events have been reported to date, and the risk/benefit ratio is in favor of vaccination for all individuals with SCD over age 5.
  • Continue to recommend vaccination against COVID-19.
  • Booster shots: All Americans are eligible, except children under 5. We recommend that individuals with SCD should receive a booster dose of the COVID-19 vaccine (Pfizer or Moderna).
  • Continue to recommend general precautions like wearing masks, maintaining physical distancing, promoting good ventilation and washing hands. These are public health measures.
  • The FDA has granted emergency use authorization of early treatments for people who test positive for coronavirus and who are at high risk for severe COVID-19, including hospitalization or death.
    • FDA announced on Dec. 22 and 23 that two new antiviral pills can be available to treat people with symptomatic COVID-19: Paxlovid (ritonavir plus nirmatrelvir, Pfizer) and Molnupiravir (Merck). Paxlovid is for patients ages 12 and up who weigh at least 88 pounds. It is available by prescription only and would be taken as soon as possible after diagnosis and within 5 days of the start of symptoms. Molnupiravir (Merck) is for adults, also byprescription only, also as soon as possible after diagnosis. MARAC is still learning more about these treatments.
    • Neutralizing monoclonal antibodies (bamlanivimab plus etesevimab (Lilly); casirivimab plus imdevimab (Regeneron); Sotrovimab (Glaxo Smith Kline) have been effective as early treatment for mild, symptomatic COVID-19 in some individuals with SCD [<10d after the test, age 12y+, wt 88 lb+ (40kg+), not hospitalized, not newly on oxygen]. However, these neutralizing antibodies might no longer be effective against the omicron variant of coronavirus. MARAC is monitoring the situation.
  1. Please remember that you can take precautions to stop COVID-19. The coronavirus spreads in the tiny droplets breathed out by an infected person. The person might not know that he or she is infected. COVID-19 spreads quickly because many people breathe these contagious tiny droplets in the air.
    • Please stay aware of local conditions that impact the risk of making contact with someone who is infected. Keep track of the rate of new cases of COVID-19, the rate of vaccination against COVID-19 and whether new variants of the coronavirus are appearing near you.
    • Where vaccination rates are low, the coronavirus spreads quickly. Continue to take precautions so that you can reduce your risk of COVID-19 infection: wear masks, maintain physical distance from others, and avoid people who are coughing or otherwise contagious as well as indoor spaces and crowds.
    • Wearing a mask is not officially required in some situations but MARAC strongly advises that individuals with SCD should still wear masks in many situations.
    • Wear a mask if:
      • you must be in a crowded indoor space.
      • you are surrounded by strangers or friends who might have been exposed to people who are sick.
        You can remove the mask when you and all those around you have been vaccinated and you are certain that nobody around you is sick.
  2. MARAC continues to advise individuals with sickle cell disease to get vaccinated against COVID-19.
    • Any of the three types of vaccines available in the U.S.A. are safe for individuals with SCD.
    • Vaccinations against coronavirus have now been shown to be safe for millions of people, with a known pattern of temporary aches that go away in about two days. It is rare for people to get severe side effects after receiving the vaccine.
    • The vaccines against coronavirus protect very well against serious symptoms of COVID-19 and reduce the chances of hospitalization and death. Break-through infections after vaccination have resulted in mild cases of COVID-19, not severe.
    • People who are not vaccinated may be barred from some travel and entertainment opportunities.
    • Protect the ones you love. Most senior citizen centers will not allow entry by people who are not fully vaccinated. Young children under 5 are not yet eligible for vaccination and depend on adults around them to be protected from infection.
  3. How much riskier is COVID-19 for individuals with SCD? Research indicates that the risk of death from COVID-19 could be 1.5 to 2 times greater for individuals with SCD compared to the general population (based on the Brandow & Panepinto SECURESCD database, U.S. single-institution reports, UK and French databases). While still significant, the risk is not as high as MARAC feared at the beginning of the pandemic.
    • Risk of complications for COVID-19 in SCD is probably higher than in the general population. The rate of COVID-19 hospitalizations is higher because pain or fever from COVID-19 could result in SCD hospitalization.
    • Death and moderate to severe complications are higher for younger SCD patients. Organ damage risk factors such as kidney damage are also higher compared to the general population.
    • There are probably excess deaths during the pandemic because of deferred care for other problems (e.g., poor care in pregnancy, delayed diagnosis of cancer).
    • Individuals with SCD and kidney or lung problems are more likely to have severe COVID-19 infections.
    • People with SCD-SC and SCD-Sbeta+thalassemia can have moderate or severe COVID-19 cases.
    • Over half of the reports of COVID-19 in SCD were associated with intense vaso-occlusive pain or sickle acute chest syndrome.
    • Individuals with SCD can have mild cases of COVID-19.
    • COVID-19 remains dangerous for everyone, especially minority groups.

    SCDAA News Advisory: Partial Hold on Gene Therapy Trial

    On December 20, the FDA placed a partial hold on bluebird bio’s clinical program for lovotibeglogene autotemcel (lovo-cel) gene therapy, temporarily stopping testing on study volunteers under age 18. This partial hold was a response to the investigation of one adolescent with sickle cell disease who has persistent anemia (not dependent on transfusion) a year and a half following treatment. Read more.

    Adults can continue enrollment and treatment in bluebird bio studies. Other clinical research studies of gene therapy for sickle cell disease are continuing.

    The clinical research process has many built-in safety precautions, which include the partial hold implemented by the FDA. MARAC will continue to monitor the situation closely on behalf of the individuals and families living with sickle cell disease.