My Recent Admission Among the COVID-19 Pandemic Originally I wasn't going to share about being in the hospital, but I think my experience might help someone else. Before I went to the emergency room (ER) on April 14th, 2020, I had been in pain (chest, legs, arms, back, and heel of my foot) for days but I was SCARED to go to the ER because of COVID-19. My chest pain wouldn't let up, so to be safe I went. The precautions the hospital had in place made me feel a little more at ease. A nurse took my temperature before allowing me into the waiting area. Also, I sat alone in a secluded area until a room was ready for me. As we walked to my room I noticed nurses and security guards posted at every entrance and in the hallways controlling access to rooms and patients. As I arrived at my room I noticed an iPad. The doctor spoke to me on the iPad until we knew the results of my COVID-19 test. Initially, I was not nervous about taking the test because I assumed it was like the flu test—but I was wrong. The test is an uncomfortable 15 seconds of a nurse swabbing your nostril as deep as possible. Until my test results returned, all of the nurses wore gowns, gloves, and masks. Registration was done verbally as well—so I could tell they were limiting extra contact as much as possible. Once my test came back negative the nurses took off the gowns and treated me as they normally would. I was ultimately admitted to the hospital. The staff told me that all the COVID-19 patients were on one floor, the 12th floor. I was on the 6th, a floor I'm not typically on. I felt safe and I was glad I finally went in. I had been sitting at home suffering and in fear, which triggered my anxiety—all for what seemed like nothing! I know that our “new normal” is scary and uncomfortable, but we have to remember to seek medical attention when needed. I suggest calling your hematologist or primary care doctor before going to the ER. If you do not have a healthcare provider, I suggest calling your local ER to ask what is the protocol for patients not experiencing COVID-19 symptoms. Many hospitals nationwide have new protocols in place.   Shamonica Wiggins Former CEO & Founder, #BoldLipsForSickleCell Media-Marketing Intern, Sickle Cell Community Consortium Dallas, Texas

Many people with sickle cell disease (SCD) had a hospitalization or an emergency department (ED) visit with a vaso-occlusive episode (VOE), or pain crisis, diagnosis code. People with SCD: • Go to the hospital more than once a year, with an average stay of 5 days per hospitalization. • Go to the ED an average of 2–3 times a year. • Go to the ED and hospital most commonly for VOEs. More than 4 in 10 (41%) ED visits and more than 7 in 10 (73%) hospital admissions for people with SCD in California had a VOE diagnosis code. Nearly 1 in 2 (48%) ED visits and 3 in 4 (75%) hospital admissions for people with SCD in Georgia had a VOE diagnosis code.   Abstract source

We are facing a global pandemic as cases of COVID-19, the disease caused by the novel coronavirus, rise around the world. More severe disease, including respiratory failure and the need for mechanical ventilation, is seen in patients with co-existing illnesses. Individuals with sickle cell disease (SCD) are a particularly vulnerable group of patients, with a higher risk of severe complications than the general public. Sickle cell disease is an inherited disorder causing normal red blood cells to deform into stiff, crescent shapes that get stuck in small blood vessels blocking blood flow and oxygen delivery to many parts of the body. This ‘sickling’ is triggered by events such as viral illnesses, cold weather, stress, and low oxygen, among others. If this sickling happens in the lungs, it triggers a cascade of events that causes a severe complication called acute chest syndrome.   Abstract source

COVID-19 and African Americans: Dispelling the Myth with Facts In cities like Chicago and New York, coronavirus deaths in African Americans rank at 28% and 70% respectively; in Louisiana and Michigan the death toll among blacks stands at 70.5% and 40% respectively.1 The data alone show us that blacks are in no way immune to this virus, and that in fact, much of these findings support the notion that there are major disparities in outcomes. Before the coronavirus became a pandemic and overshadowed the year 2020, blacks experienced disheartening health inequities and reprehensible disparities. African American women experience the highest death rates from heart disease, breast and lung cancer, stroke, and pregnancy among women of all racial and ethnic backgrounds.2 Hundreds of black men were victims of a syphilis observational study and were left untreated without consent, and of course, during the dreadful period of slavery in the United States, blacks were not afforded equitable health care. When they were finally freed, the Freedmen’s Bureau established a health arm, and due to racial politics and mismanagement, the Bureau, and subsequently the health component permanently shut down.2 Therefore, it comes as no surprise to learn that this viral pandemic is showing the world that racial and ethnic disparities in health care are real and need to be addressed. Patients with sickle cell disease are presumed to be at a higher risk of infection with the coronavirus due to preexisting immunocompromisation. Respiratory issues such as acute chest syndrome are already a threat to patients with sickle cell disease and this virus could prove fatal in someone who has had acute chest syndrome in the past. I believe it is wise for all patients with sickle cell disease to be vigilant in this season and make sure that they are making the best choices possible to protect their health and safety. We understand that conspiracy theories will crop up any time but especially during a global crisis such as this. It is incumbent upon every black person to make sure that they vet all the information that they are inundated with on a regular basis. With the advent of social media, untrustworthy sources and erroneous information can spread just as fast as this virus has. I tell everyone that if it does not pass what I call the “smell test” then you should be skeptical and seek out more information. Most things that sound too good to be true, are normally too good to be true. The internet can be a double-edged sword; however, it can be and has been used for good. Whenever there is information spreading in your family or community, take to a search engine and find out the answers for yourself. The Centers for Disease Control and Prevention (CDC), United States Department of Agriculture (USDA), Food and Drug Administration (FDA), the U.S. Department of Health and Human Services (HHS), and the National Institutes of Health (NIH) and other governmental healthcare organizations have published information about the coronavirus pandemic on their websites for you to read, digest, understand and then disseminate to your own community. I believe that it is the individual’s responsibility to debunk myths and fallacies once they have heard them and found them to be untrue—this is one of the reasons that I wrote this blog piece. I wanted to personally have a voice in addressing the unfounded claim that black people cannot be infected with this virus. The data suggest that black communities are in fact contracting and dying from the virus more frequently and disproportionately.1 Unfortunately, at this point, the only tool that we have against the coronavirus is social distancing. It reminds me of the abstinence campaigns back in middle school and high school where they would tell us the only 99.9% way to protect against pregnancy was not a condom, it was abstinence. That point being made, the only sure-fire way you can be sure that you will not be infected by this virus is to follow social distancing and public health guidelines and mandates. What adds insult to injury is that possessing the ability to participate in true social distancing is in itself a privilege.1 The communities in which black people live tend to be poor, where there’s high housing density and more instances of food insecurity.1 This virus continues to expose the deep-seated impact that a legacy of racial divide, and inequality have in our healthcare system. This legacy of racial injustice has played a role in the disparities that black Americans and people of color in America face today. It was hypothesized that black people could not be infected with the virus because we “already had to deal with too much as a community of black people” from slavery, to segregation and Jim Crow laws, and we would get some divine “pass.” Unfortunately, these same horrific events that we as a people went through ended up being major historical determinants of our health. These determinants have inevitably influenced the impact of the coronavirus in black communities. It is crucial during this pandemic that we are good stewards of our own education and that we are responsible for feeding and informing ourselves with the most up-to-date and accurate information as possible. When you hear erroneous messages being passed around, do not let it go further than yourself. Take the time to gracefully educate, after you have searched out the answers from trustworthy and credible sources—besides your family members who “heard” something from their friends. 1. Yancy CW. COVID-19 and African Americans. JAMA. Published online April 15, 2020. doi:10.1001/jama.2020.6548 2. Dawes DE. The Political Determinants of Health. Baltimore, MD: Johns Hopkins University Press; 2020.   André Marcel Harris Community Manager, oneSCDvoice

The Be The Match® Patient Support Center offers free counseling services. A licensed social worker can provide one-on-one counseling support to help patients, caregivers, family members, and survivors of transplant cope with the blood and marrow transplant (BMT) process. At this time we are also offering this service to patients with sickle cell disease and their loved ones regardless of their treatment status. If you are interested in seeking counseling services from one of our licensed social workers, please complete this form. A social worker will reach out to you within two business days.   Abstract source

This registry is designed to capture pediatric and adult COVID-19 cases that are occurring across the world in patients living with sickle cell disease. The goal of the registry is to report on outcomes of cases of COVID-19 in this population of patients. We are asking providers caring for these patients to report all of their cases of COVID-19 to this registry. We expect the reporting of a case to take approximately 5-10 minutes. With our sickle cell disease community worldwide, working collaboratively, we hope to quickly identify the impact of COVID-19 on patients with sickle cell disease and understand the impact of risk factors such as genotype, prior sickle cell disease complications, or sickle cell disease related medications, on health outcomes. This is an international effort—we are counting on robust participation and collaboration. We will provide the sickle cell disease community with regularly updated summary information. The registry contains only de-identified data, in accordance with HIPAA Safe Harbor De-Identification standards. Abstract source

Early diagnosis, treatment, and prevention of a vaso-occlusive crisis (VOC) is critical to the management of patients with sickle cell disease. It is essential to differentiate between VOC-associated pain and chronic pain, hyperalgesia, neuropathy, and neuropathic pain. The pathophysiology of VOCs includes polymerization of abnormal sickle hemoglobin,inflammation, and adhesion. Hydroxyurea, L-glutamine, crizanlizumab, and voxelotor have been approved by the US Food and Drug Administration for reducing the frequency of VOCs; the European Medicines Agency has approved only hydroxyurea. Other novel treatments are in late-stage clinical development in both the United States and the European Union. Development of agents for prevention and treatment of VOCs should be driven by our understanding of its pathophysiology. Abstract source

Options for High School The COVID-19 crisis has brought many changes to the lives of high school students—no more lunchtimes with friends, no more giggling over the latest fashion trends, no more worries about who is in the in-crowd and who is not. Lots of our teens would trade sleeping late and extra screen time to have these things back again—who wouldn’t? For the youngster with sickle cell disease, these things may take a back seat to the struggle to simply, physically, get through the day. High schools in many parts of the country consist of several two-story buildings on many acres of land. Now that our high school students have been home for several weeks, it’s a good time to take stock of how they are doing. Although many will complain of boredom with the new on-line courses and the difficulty of understanding without being able to get extra help, parents of kids with sickle cell disease understand that this new educational setting may actually help their students succeed in high school. In many parts of the country there is an alternative to the regular high school, the options high school or continuation high school. These schools are often located on a small corner of the high school campus and are taught by credentialed teachers. Classes are usually limited to four hours a day, with much of the work being done in an independent study format. Students may work in small groups or on their own with the teacher providing guidance along the way. Options schools are often part of a dropout recovery program, since the students enrolled may have had attendance problems that make them potential dropouts. Right now you may be thinking, “That kind of program is not right for my child, I want him to go to his regular high school.” But now might be a good time to have a deep conversation with your student. Kids have been out of school for several weeks now, does your child seem physically stronger, is it easier for him to understand what is being taught now that he is not fighting the physical demands of sickle cell disease? As a patient advocate and former high school principal, I always suggest that parents have a discussion with their pediatrician. A review of your child’s medical history may shed some light on what may really be going on. Remember that sickle cell disease is often called sickle cell anemia because the sickled red blood cells don’t carry enough oxygen to the body. It’s not surprising then that young people with sickle cell disease have little energy. A big school with flights of stairs and six challenging classes can be challenging if you’re tired all the time. Before the next school year begins, parents might want to ask for an IEP or 504 meeting. Identify the issues of concern as you meet with teachers and counselors. Perhaps a four-hour day in the regular high school setting will help, but if a continuation school is the answer, it could give your child the opportunity for individualized instruction that can address the specific issues of students with sickle cell disease and lead to a more successful high school experience.   Nancy M. René Co-Founder, JVT Advocates Supporting Autism, Sickle Cell Disease, and K–12 Education Los Angeles, California

We read with interest the letter from Skinner, Nader and Connes and their comments on our recently published study on the burden of type 2 diabetes (T2D) in patients with sickle cell disease (SCD). The authors call attention to variation in T2D risk across patients with different SCD genotypes. Despite the fact that SCD is caused by single gene disorders, patients with SCD demonstrate a complex spectrum of complications and phenotypic heterogeneity that can be partially explained by the variability of fetal haemoglobin levels and co‐inheritance of alpha thalassaemia. It is plausible to speculate that SCD patients of different genetic foundation could present distinct pathophysiology features, including lipid profile and anthropometrics, which in turn could influence the likelihood of being diagnosed with T2D. To answer the questions raised by Dr. Skinner and colleagues, we must acknowledge the inherent limitations of administrative health databases in performing studies of disease burden. Herein we describe the challenges of further exploration of these currently available data and the necessary methodological considerations for such epidemiological studies, particularly possible information and selection biases. Abstract source

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