Author Archives: Emma Day

Women’s History Month: Dr. Angella Dorothea Ferguson

This #WomensHistoryMonth, we are highlighting prominent women who have made lasting contributions to the SCD community. #WomenInMedicine #WomensHistoryMonth

Meet Dr. Angella Dorothea Ferguson, a pediatrician and sickle cell pioneer. Born in 1925, Dr. Ferguson dedicated her life to researching sickle cell disease, an unknown condition at the time. Her groundbreaking work led to the development of a blood test for infants, which is now the standard in most states. Thanks to her research, we better understand which symptoms to look for in children and can start treating sickle cell earlier. Thanks to Dr. Ferguson for the work she has done on behalf of our community!

 
 

#BlackHistoryMonthHeroes: Carlton Haywood Jr.

SCDAA’s #BlackHistoryMonthHeroes highlights prominent Black members of our community who have made their mark on our history. Stay tuned this month to learn more about these inspiring individuals. 

#BlackHistoryMonth Meet the late Carlton Haywood Jr. – a trailblazer in the field of health equity and bioethics. Born with sickle cell disease, Carlton never let his illness hold him back. He was an all-star student and held many degrees, including a Ph.D. in Bioethics and Health Policy from the John Hopkins Bloomberg School of Public Health. His research, informed by his own experiences with SCD, earned him a competitive National Institutes of Health grant and recognition as a leader in his field. Despite the challenges he faced, Carlton’s unwavering spirit and passion for health equity and bioethics inspired many. His legacy will live on as a shining example of resilience and dedication.    

#BlackHistoryMonthHeroes: Dr. Kwaku Ohene-Frempong

SCDAA’s #BlackHistoryMonthHeroes highlights prominent Black members of our community who have made their mark on our history. Stay tuned this month to learn more about these inspiring individuals. 

#BlackHistoryMonth Meet the late Kwaku Ohene-Frempong, M.D., – a renowned pediatric hematologist-oncologist and expert in sickle cell disease. Dr. Ohene-Frempong grew up in Ghana, where he excelled as a track-and-field athlete. He completed medical school and a residency in pediatrics, followed by a fellowship in pediatric hematology-oncology in the U.S. He dedicated his career to serving patients with sickle cell disease at Tulane University School of Medicine and the Children’s Hospital of Philadelphia. Upon retiring, he returned to Ghana to establish public health initiatives for SCD screening and treatment. Dr. Ohene-Frempong’s passion for improving the lives of those affected by sickle cell disease has left a lasting impact. His dedication to improving access to care, education and public health initiatives for SCD has inspired many to continue his work and carry on his legacy.   

#BlackHistoryMonthHeroes: Hertz Nazaire

SCDAA’s #BlackHistoryMonthHeroes highlights prominent Black members of our community who have made their mark on our history. Stay tuned this month to learn more about these inspiring individuals. 
Meet the late Hertz Nazaire – a talented artist and author who used his brush to spread awareness about sickle cell disease. At just 9 years old, Hertz began honing his skills in NYC and later perfected his craft at The Art Institute of Fort Lauderdale and the University of Bridgeport. Known for his advocacy work and his “Finding Your Colors” adult coloring book series, Hertz was dedicated to using his art as therapy for those impacted by stress. Hertz lost his battle to sickle cell in late 2021, but his commitment to SCD advocacy and his impact on the art world will not be forgotten. 

 

MARAC Statement: Penicillin Shortage

Penicillin VK solution is suffering from intermittent supply shortages. This can affect children with sickle cell disease. Penicillin VK in liquid form is prescribed for babies and young children with sickle cell disease who are unable to swallow pills as standard care, twice a day, starting as early as 6-8 weeks of age. Children less than 3 years of age should receive oral penicillin 125 mg twice daily, and the dose should be increased to 250 mg twice daily for children older than 3 years.

WHY DO CHILDREN WITH SICKLE CELL DISEASE NEED PENICILLIN?

The strategy of using an antibiotic daily to prevent infection in people with lower immune defense is called prophylaxis. Without medical care, young children with sickle cell disease have extremely high risk of hospitalizations and death from infection. Strong scientific evidence shows that penicillin twice a day helps young children with sickle cell disease avoid an overwhelming bacterial infection, especially the bacteria called pneumococcus. Newborn screening diagnosis of sickle cell disease leading to penicillin prophylaxis are the two fundamental starting points of sickle cell comprehensive care.

WHAT CAN THE HEALTH CARE TEAM DO ABOUT PENICILLIN SHORTAGES?

  • Urge the manufacturers and distributors to improve the supply of penicillin so that we do not lose out on this effective, evidence-based standard of care.
  • Prioritize sickle cell disease as an immune-compromised condition that has specific guidelines stating the necessity for penicillin prophylaxis.

WHAT DO FAMILIES DO ABOUT PENICILLIN SHORTAGES?

  • Alternatives to penicillin liquid exist, although all are suboptimal. Discuss with your doctor:
    • Penicillin VK tablets, crushed (Miss out on residual uncrushed portions, takes time twice a day while the family is already busy with parenting a young child)
    • Penicillin G injection, once a month (Cannot be administered at home/requires visiting a medical facility, needle stick)
    • Other alternative antibiotics: Amoxicillin 20mg/kg/day or Erythromycin (Broader effects on normal bacteria than penicillin, supply shortages might also be a problem)
  • Make sure your child has all immunizations as recommended for sickle cell disease. SCDAA MARAC strongly encourages full immunization, especially pneumococcal vaccines PCV and PPSV-23.
  • Fever needs prompt attention in individuals with SCD. Go to the Emergency Department (not urgent care) for blood counts, blood cultures and injection of a strong antibiotic like ceftriaxone.

HOW STRONG ARE THE RECOMMENDATIONS FOR PENICILLIN PROPHYLAXIS?

In the United States, penicillin prophylaxis is written into public health policy, National Institutes of Health guidelines and even state laws. Brazil, Canada, France, Italy, Jamaica, Ghana, Nigeria, Tanzania, Uganda, the United Kingdom and other countries have penicillin prophylaxis in their national guidelines for sickle cell disease.

IS IT SAFE TO GIVE ANTIBIOTICS DAILY?

Penicillin prophylaxis has an excellent track record and has been used to provide safe care to thousands of children for over 30 years. Studies have showed that giving penicillin prophylaxis continuously does not cause harmful bacteria to become antibiotic resistant. On-and-off periods of penicillin prophylaxis could cause antibiotic resistant bacteria to emerge. Penicillin was chosen for prophylaxis because it is focused on the pneumococcal bacteria that can cause problems in sickle cell disease.

IS PENICILLIN EXPENSIVE?

Penicillin is one of the oldest and most affordable antibiotics. Business analysts say that the low prices of penicillin make few companies motivated to manufacture penicillin.

For references and highlight points, download the full statement.

Sickle Cell is Not a Joke

The Sickle Cell Disease Association of America, Inc., joins the Foundation For Sickle Cell Disease Research (FSCDR) in condemning the use of sickle cell disease (SCD) as a punchline on the HBO Max show Velma. For the over 100,000 Americans impacted by sickle cell and their families, this disease is anything but a laughing matter. Stereotypes and misinformation reinforced by media clips such as this have real-life consequences. Sickle cell patients struggle to be taken seriously and receive proper care, even when they present with life-threatening symptoms in the emergency room. As we work to change the perception of sickle cell and increase education surrounding this condition, insensitive and inappropriate jokes like these work against progress and contribute to the spread of misinformation. We must do better and encourage people to treat rare diseases with the respect they are due.

Click here to read the full statement from FSCDR. 

#SickleCellIsNotAJoke  

CMO Speaks: Fertility Care and SCD

CMO Speaks is a blog featuring the voices of SCDAA’s clinical leadership team. The below article was written by Dr. Lewis Hsu, SCDAA chief medical officer, with input from Dr. Lydia Pecker.

NPR recently produced this segment with sickle cell warrior and advocate Teonna Woolford on fertility care and SCD. This is an underdiscussed topic and an important aspect of sickle cell care. We congratulate Teonna and the Sickle Cell Reproductive Health Education Directive (SC RED) for getting this message out, and we are grateful for the attention from NPR. In light of this discussion, we would like to reinforce a few points about reproductive health and SCD:

  1. Even without a transplant or gene therapy, sickle cell disease can damage patients’ bodies in ways that can affect their ability to have children. We agree with the statements by Drs. Lydia Pecker and Leena Nahata in this NPR report.
  2. Currently, insurance coverage for fertility preservation is highly variable and differs from state to state. We agree with statements in this NPR report by Dr. Irene Su about the vagueness of these policies. This is a sickle cell advocacy opportunity to get coverage in more states. SCDAA praises the efforts of SC RED and the Alliance for Fertility Preservation as allies in fighting for coverage. However, just as the NPR report described for intrauterine insemination, there are high costs and many other barriers of access to these procedures. Shared decision-making should be the model for information, choices and policies.
  3. SCDAA agrees wholeheartedly with the statements in this NPR report regarding funding disparities and the lack of information dissemination about sickle cell disease. The NASEM 2020 Report has much more information about action steps to take.

    Go deeper – What is not brought out in this NPR report

    1. Sickle cell disease is an inherited condition. Another aspect of reproductive decision-making for individuals with SCD is understanding the risks of having a child who also has sickle cell disease (more about family planning in this infographic from SC RED). Having a child with sickle cell disease is not a mistake, but it should not be a surprise for lack of information. Tests are available to know whether the mate has the sickle gene or another hemoglobin variant. Tests are available for prenatal diagnosis. Fertility centers also can do preimplantation genetic diagnosis and embryo selection to choose an embryo without sickle cell disease.
    2. For individuals with sickle cell, choosing to have children also means gathering a support system so that they can be a caregiver for a child when they themselves have sickle cell problems. (Currently, the mother of one of my patients cannot be at the bedside for her child with sickle acute chest syndrome because she is hospitalized herself for sickle cell pain.)
    3. Making reproductive decisions would be best done in the format of “shared decision-making.” These are complicated decisions that depend on individual situations and individual values. Individuals with sickle cell disease should try to be well-informed. Try to explain your values and reasons to your health care provider(s), and ask questions until you have a solid understanding. Please be aware that some things are not certain in medicine and in reproduction and can only be described as chances and risks.

    Helpful links and resources

    Lewis Hsu, MD, PhD, is a pediatric hematologist who serves as director of the Sickle Cell Center and professor of pediatrics for the University of Illinois at Chicago. He has conducted sickle cell research, published over 50 peer-reviewed papers and co-authored “Hope and Destiny: The Patient and Parent’s Guide to Sickle Cell Disease and Sickle Cell Trait.” He currently serves as the SCDAA Chief Medical Officer. 

    MARAC Statement on Influenza

    December 6, 2022 — The Sickle Cell Disease Association of America (SCDAA) Medical and Research Advisory Committee (MARAC) shares the following:

    For individuals with sickle cell disease and their caregivers

    What is influenza, and why should I worry about it?

    Influenza (“flu”) is a contagious viral infection that can cause severe medical problems in anyone. It is worse than a common cold. The problems are potentially bigger for individuals with sickle cell disease. Influenza can trigger sickle cell vaso-occlusive pain or acute chest syndrome. This means that influenza has a high chance

    of sending you to the hospital and possibly to the intensive care unit with severe breathing problems. If you already have lung problems from asthma or from repeated acute chest syndrome, then influenza is even more likely to cause you to be severely ill.

    What can I do?

    Prevention Tip #1: MARAC strongly encourages all individuals with sickle cell disease (and their families) to get their flu shot every year. Although the shot might cause symptoms for a day or two (sore injection site, muscle aches and mild fever), these problems are small compared to the potential problems of influenza infection. To find a flu shot near you, visit vaccines.gov.

    Prevention Tip #2: Wash your hands. Stay away from people who are coughing or sneezing or wear a mask around them.

    Prevention Tip #3: During flu season, it is very important for individuals with sickle cell disease to take all their medications as prescribed. Hydroxyurea can protect against sickle cell pain or acute chest syndrome. Penicillin can protect against bacterial infection when a person is already weakened by influenza. Asthma medications can keep lungs functioning better.

    If you think you have the flu, see your health care team early. If you can be diagnosed with influenza in the first 48 hours of the illness, you will probably be eligible to start a prescription treatment for influenza called oseltamivir (Tamiflu). Oseltamivir can shorten the course of influenza and reduce the risk of severe problems.

    For health care providers and policymakers

    Sickle cell disease has a high risk for complications and high morbidity from influenza (references 1-6 below). Sickle cell disease has both altered immune response and vulnerability to lung inflammation, triggering hospitalizations for acute chest syndrome, pain or other sickle cell complications. Health care providers should monitor sickle cell patients with influenza for possible acute chest syndrome. Acute chest syndrome is greatly

    feared because it is the leading cause of death in sickle cell disease in the United States. Much of the clinical experience regarding sickle cell and the flu was gained from the 2009 H1N1 influenza but some is more recent, including a CDC report from 2021 (ref 2).

    SCDAA MARAC notes that there is a national shortage of the treatment for influenza called oseltamivir (Tamiflu) and stewardship of its use is important. Oseltamivir reduces complications in influenza (Lee 2020, Wiemken 2021). MARAC recommends that sickle cell disease should be included in the list of conditions to prioritize for oseltamivir. Sickle cell disease is a rare disease nationally and might be overlooked in national policy because of its rarity. However, the special needs of sickle cell disease should be acknowledged.

    For references and highlight points, download the full statement.

    Midterm Election Outcomes and What they Mean for SCD

    An update from John Otsuki, SCDAA government relations manager

    As I am sure you’re aware, the United States recently completed a midterm election. All members of the House of Representatives were up for election or re-election, as they are every 2 years, as were 34 Senators (roughly 1/3 of the total). 

    Before the election, the Democrats controlled both the Senate and the House, both by narrow margins. The 2020-2022 Senate was split 50–50*, with Vice President Kamala Harris breaking tie breakers, effectively giving the Democrats control, while the House was controlled by the Democrats 219 seats to 213 Republican seats. 

    This November, the Democrats held the Senate, winning 50 seats to the Republicans 49. One race is going to a runoff in mid-December (GA). Regardless of the outcome, the Democrats will retain control, with Senator Chuck Schumer (D-NY) expected to remain as Majority Leader and Senator Mitch McConnell (R-KY) to remain as Minority Leader. In the House, the Republicans the Republicans have won 220 seats, while the Democrats have 213 seats. While two seats are still undecided, control of the House will flip to the Republicans. 

    Once the results are finalized, the House of Representatives will elect a new Speaker of the House, which will certainly be a Republican. Who that will be remains undecided, with Rep. Kevin McCarthy (R-CA) as the front runner. The current Speaker of the House, Rep. Nancy Pelosi (D-CA), has stepped down from leadership. Representative Hakeem Jeffries (D-NY) has been elected as the new Minority Leader of the House. The new Congress will begin January 3, 2023. 

    What does this mean for sickle cell disease?

    The Republican flip of the House Representatives means that the chair and composition of every committee and subcommittee will change. That means the person deciding which pieces of legislation make it to and through committee and to the floor for a vote will change. Republicans have been open to working on sickle cell disease issues in the past, however, the number of supporters of SCD is drastically lower. Republicans are also less likely to support increased spending for any program. The current pending bills have price tags of $50 million to $535 million annually. Republicans are less likely to support these numbers in their current form. 

    More importantly, the Senate will still be controlled by the Democrats and the Presidency will still be controlled by the Democrats. This means we will have divided government. As we have seen over the past 20 years, divided governments struggle to pass any meaningful legislation at all. Passing SCD legislation is about to become much more difficult. 

    I hope this summary was helpful! 

    -John Otsuki, SCDAA government relations manager

    *The Democrats technically have two independents, Senators Angus King and Bernie Sanders, but both caucus and vote with the Democrats. 

     

    NFL players spotlight Sickle Cell Disease Association of America

    The NFL’s My Cause My Cleats campaign features players showcasing important causes on their cleats each year during Week 13 games in early December.

    Tevin Coleman’s Cleats

    San Francisco 49ers running back Tevin Coleman and Arizona Cardinals linebacker Markus Golden will support the Sickle Cell Disease Association of America through the NFL’s My Cause My Cleats program by wearing custom-designed cleats with inspirational artwork and messages to raise awareness and engagement around sickle cell disease.

    “We’re thrilled and grateful to Tevin and Markus for recognizing the Sickle Cell Disease Association of America on their cleats this year,” said Regina Hartfield, president and CEO of the Sickle Cell Disease Association of America. “The visibility and support mean so much to us and the thousands of families living with sickle cell disease across the country.”

    Coleman is an eight-year veteran running back in the NFL. For him, supporting the Sickle Cell Disease Association of America means supporting his daughter.

    “As the father of a child who lives with sickle cell disease, I know how important it is for people affected by sickle cell to receive the high-quality health care and support they need and deserve. I’m proud that my cleats this year will help support the Sickle Cell Disease Association of America and also recognize the strength of my daughter, Nazaneen, who inspires me every day,” Coleman said. “Together with my wife, Akilah, our hope is that by raising awareness and connecting people to this amazing organization, we will help more individuals and families battling this condition to live well and thrive.”

    Off the field, Tevin and Akilah Coleman partner with Pfizer to advocate for sickle cell disease and share their family’s story at SickleCellSpeaks.com. Pfizer partners with the Sickle Cell Disease Association of America to advance education and awareness of sickle cell disease.

    Coleman worked with footwear artist and designer Dan Gamache, known as “Mache,” to create cleats honoring those who inspire his commitment to help improve the lives of people with sickle cell. The cleats include the name of Coleman’s 5-year-old daughter, Nazaneen, and her favorite animal, a purple butterfly, alongside sickle cell imagery in the style of Hertz Nazaire, a renowned artist and sickle cell warrior. The cleats will also feature the Sickle Cell Disease Association of America’s logo and a unifying hashtag, #SickleCellMatters.

    A Sickle Cell Disease Association of America celebrity ambassador, Golden grew up in St. Louis, graduated from the University of Missouri and was selected by the Arizona Cardinals in the 2015 NFL draft. He has featured the Sickle Cell Disease Association of America and sickle cell causes on his cleats in past years.

    “Sickle cell disease is a condition that’s often forgotten, but it profoundly affects so many families across the country,” Golden said. “I lost my cousin Jonah at only 10 years old to the disease, and that’s why I’m so driven to bring attention to sickle cell and the Sickle Cell Disease Association of America on my cleats this year. I’m proud to once again partner with such an important organization to support people with sickle cell. I hope every step I take on the field is a step toward finding a cure for sickle cell.”

    The NFL will auction Golden’s and Coleman’s cleats on the league’s website after the Week 13 games. Auction proceeds will support the Sickle Cell Disease Association of America.