In the spring of 2020, the coronavirus pandemic first gripped the world by the throat and its deadly menace continues to unfurl with renewed ferocity. In the United States, medical and scientific experts issued a series of early recommendations to slow or halt the spread of the virus that causes the disease COVID-19. Such public health measures are clearly warranted. As of this writing, over 285,000 Americans have perished from COVID-19 and the infection numbers are soaring across much of the country. Recommendations to combat the virus spread include handwashing, covering the face when coughing and sneezing, wearing a mask when in public spaces, and social distancing. The most challenging anti-virus measure was the lockdown or stay-at-home orders issued by state and local governments. In many communities, people rushed out to stock up on food, water, and household supplies before they began sheltering in place. The lockdown preparations and implementation clearly highlighted the pervasive and persistent inequalities impacting every aspect of American life that are attributable to social determinants of health (SDH). The World Health Organization defines SDH as “the non-medical factors that influence health outcomes. They are the conditions in which people are born, grow, work, live, and age, and the wider set of forces and systems shaping the conditions of daily life. These forces and systems include economic policies and systems, development agendas, social norms, social policies and political systems.” During the current public health crisis one key question for researchers, policymakers, and clinical providers alike to ask is: Which populations in the U.S. are most likely to experience adverse effects from SDH?
As an academic nurse researcher with expertise in health disparities, I closely monitored news about how the spring lockdowns were impacting various population groups. I paid special attention to the dramatically different experiences of white-collar employees, many of whom had the privilege of safely sheltering in place while working from home, and front-line essential workers who had no such option. This later group, often employed in grocery stores, meat-packing plants, bus stations, and other crowded environments, had higher risk of exposure to the coronavirus. Many of these essential workers are people of color and the devastating consequence of this reality was all too predictable. According to the U.S. Centers for Disease Control and Prevention, communities of color have experienced considerably higher rates of infection, severe illness, and death from COVID-19. This population includes approximately 100,000 individuals, largely African American, with sickle cell disease (SCD), an inherited red blood disorder. The major symptom of this disease is persistent, disabling pain, including excruciating episodes known as a pain crisis. SCD is a lifelong illness with a life expectancy of 48 years for women and 42 years for men.
With a primary research focus on pain management disparities experienced by SCD patients, I worry how coronavirus-related stressors are exacerbating the considerable pain already endured by these individuals. My research indicates that systematic stressors such as healthcare injustice—defined as unfair treatment an individual receives from important medical figures such as healthcare providers — predicts increased pain in patients with SCD. They are particularly vulnerable at this time because the coronavirus pandemic can magnify the negative SDH already experienced due to their race and disease trajectory.
I am concerned about how SCD patients are coping with today’s magnified societal stressors, particularly when trying to avoid a stress-related pain crisis that would require hospitalization and potential exposure to the coronavirus. Given the comprised hematologic profile of patients with SCD, which reduces oxygen circulation, these individuals are at elevated risk for both COVID-19 severity and mortality. Statistics from early in the pandemic document this grim reality. A recent study found that between March and May 2020, 178 infected individuals were entered into the SCD-coronavirus disease case registry. Of these, 122 (69%) were hospitalized and 13 (7%) died. (These hospitalization and death rates are much higher than for infected individuals in the general population.) Healthy stress management techniques that decrease emotion-triggered pain crises could greatly improve the well-being of SCD patients and potentially reduce their hospitalizations and deaths. Healthcare professionals can play a key role in encouraging patients to consistently utilize non-drug coping strategies to complement medication regimens for pain management.
Our research team found that patients with SCD who experienced healthcare injustice from nurses reacted to this psychological stressor by isolating themselves. Meanwhile, those who experienced healthcare injustice from doctors reacted by both isolating and pain catastrophizing. These negative coping strategies are associated with poor health outcomes. For example, pain catastrophizing corresponds to lower health-related quality of life for patients with SCD. On a more positive note, patients who experience healthcare justice also cope with prayer and hopeful thinking. These healthy strategies have long been endorsed within African American communities, where deep spiritual beliefs and regular religious practices have helped them deal with the harsh realities of slavery and the systemic racial discrimination and injustice that sadly remains a powerful force in American culture.
Another coping strategy for SCD patients dealing with stress and pain is guided relaxation. This can include deep breathing and counting backwards from 10 to 1 while focusing on a specific spot within an object. This technique has been shown to effectively reduce stress and pain for adults with SCD. Another recent study found that music therapy also reduces pain and improves mood. It is important that these vulnerable patients know there are a number evidence-based drug-free strategies they can utilize during this unprecedented and pressure-filled coronavirus pandemic.
Unfortunately, SCD patients in the United States, like other citizens the world over, cannot individually control the course of the pandemic and the havoc it is wrecking. However, these patients do wield tremendous control over how they choose to cope with coronavirus-related stressors that can intensify their SCD pain. In addition to the non-drug options described above, individuals can explore and try other safe coping strategies to better manage their physical and emotional health challenges. It is vital that patients are proactive on an ongoing basis to reduce their stress and pain and improve their overall well-being as the world awaits better coronavirus treatments and an effective vaccine.
Today is World Sickle Cell Day—a day designed to spread awareness and understanding of sickle cell disease–an often painful inherited blood disorder.
The Sickle Cell Disease Association of America (SCDAA) is marking the 10th year of this recognition. There is no cure for sickle cell disease right now, although some patients have had success with various treatments including hydroxyurea treatment and bone marrow treatment.
According to SCDAA, complications from sickle cell disease (SCD) happen when a normal blood flow is reduced or prevented from flowing normally throughout the body. With SCD, some normally round red blood cells become crescent shaped (sickle shaped) and because of that change, can no longer flow through small blood vessels. These misshapen cells can actually cause a blockage, thereby reducing a normal blood flow that tissues need to stay healthy and to keep the body functioning properly. A common result is that reduced blood flow damages tissues and can lead to debilitating pain.
Blood cells with sickle cells don’t live as long in the body (about 16 days vs. 120 days for normal cells according to the SCDAA), so that rapid turnover can lead to the myriad complications that come with a sickle cell diagnosis including anemia and jaundice. Because SCD affects nearly every organ in the body, systemic complications can involve major systems including the lungs and kidneys. And the damage can also leave the body less able to fight off and control infections.
Current treatments are often a reaction to the complications, although some, like a preventative antibiotic and vaccinations in children, can help prevent complications from beginning. Patients may find various combinations of blood transfusions, pain management, antibiotics, breathing treatments, proper vaccinations, and other medications.
As with other chronic conditions, people with SCD have to take extra care with their own health. Because people are born with sickle cell disease, the disorder is a lifelong condition and can be especially challenging for children to cope with. Generally, a hematologist will manage the condition and any treatments. According to the Centers for Disease Control and Prevention, staying well-hydrated is especially important as is good nutrition and rest. Anyone with sickle cell disease can stay active to help overall health, but just be especially aware of fatigue and fluctuations in body temperature—moderation is the best approach.
According to the CDC, sickle cell disease affects about 100,000 people in the United States with a greater diagnosis rate in minority populations. Sickle cell disease happens in approximately 1 out of every 365 Black or African-American births and 1 out of every 16,300 Hispanic-American births.
More common is being diagnosed as a carrier of the sickle cell trait (SCT) which is not sickle cell disease. A carrier of SCT has one copy of the defective gene that can cause sickle cell disease (you need two copies to have the disease).
For those with SCD, finding a provider who is familiar with the disease and with cutting-edge treatment is important. Research information, through organizations such as the Foundation for Sickle Cell Disease Research is essential. But the emotional struggle of coping with a complex, chronic, and invisible disease that can have such disruption in someone’s life is big. Getting the emotional and social support of a caring community is going to make a difference in developing coping skills and getting through rough times.
If you know someone who is impacted by sickle cell disease in any way, today makes it easier to spread the word, provide education, and let them know they aren’t alone.
It was never fully explained during the movie Beasts of the Southern Wild, but we can surmise that Hushpuppy’s father may have suffered from sickle cell anemia. Her father, Wink, after being missing for days, finally strolls back to ‘The Bathtub’ still donned in his hospital gown and it is obvious something is seriously wrong with him. What, exactly, remains a mystery, but when Hushpuppy presents her father’s hospital identification bracelet to her schoolteacher, her teacher quickly gathers a type of folk-remedy to give to Hushpuppy that is supposed to help her father.
Of course, her father refuses his daughter’s ministrations, which only confounds Hushpuppy. Even after she faces the brutal aurochs, Hushpuppy still cannot prevent her father’s eventual demise. And as illustrated in real life, sickle cell is a very ruthless and painful genetic condition, most commonly found among the African American community.
According to the Centers for Disease Control and Prevention, it is estimated that 1 out of 12 African Americans have some form of the disease, either carrying the sickle cell trait, which generally has no symptoms but can be passed along to their subsequent offspring, or the full-blown case of it, which can entail a shortened lifespan as well as enduring days in a diminished capacity and longsuffering state. Tests are available that a pregnant mother can have performed during an amniocentesis to screen for sickle cell. Of course, if it comes back positive, she will still be faced with more questions than answers, among them treatment options, quality of life, and severity of the disease.
By investigating the origins of sickle cell anemia, we can understand how it went from one of the most shamefully neglected conditions to one promising scientific breakthrough, where perhaps one day, we will have something close to a cure for those future generations who are afflicted.
Genetic Components
Interestingly enough, the sickle cell gene originated along the equatorial belt as a natural selective means to thwart the devastating effects of malaria. However, though it protects the carrier from mosquito-borne illness, sickle cell disease is a highly debilitating illness and its effects can diminish the stricken with a lifetime of painful joint, organ, and bone complications. In the human body, normal red blood cells are smooth-surfaced and round, hence the simplicity in which they slide through veins. When someone has full-blown sickle cell anemia, their hemoglobin is deficient, creating cells that are inherently sticky-surfaced and like a farming tool, sickle in shape. The problems caused by the damaged cells come in the form of chronic pain or ‘attacks’ where the cells have lodged together, either in certain organs, bones, or blood of the infirmed. The patient’s blood vessels are blocked by clumps of sticky, sickle cells, which is the source of the patient’s chronic pain.
Treatment Options
This can range due to the severity of the disease and can consist of treating the pain with anti-inflammatories, or if needed, a bone marrow transplant. There have been some promising results with the use of Hydroxyurea, a drug commonly used in the treatment of cancer. It can help prevent the sickle cell buildup, reducing the amount and occurrence of painful attacks, but it has its side effects as well. Symptoms can include nausea, constipation, hair loss, fever, weight gain, leg ulcers, and bleeding. With children some experts are cautioning the use without knowing the long-term effects; some studies have demonstrated genetic changes and an increased risk for cancer. For those extreme cases there is bone marrow transplantation, but of course, this requires finding a compatible donor as well as extensive treatments.
Care
Unfortunately, due to factors like medical discrimination, a lack of awareness, care, and treatment options, sickle cell anemia has historically not received the recognition among our scientific and medical community. On May 16th, 1972, President Richard Nixon took sickle cell anemia to center stage with the signing of the National Sickle Cell Anemia Control Act. But at the end of the day, it rests upon those in the medical community who can be that critical segue for pain management and controlling symptoms, especially young children who are suffering from the effects of this genetic disease. Treatment options, education, and cultural respect should be clearly understood for those caring for the afflicted.
Certainly, we have come a long way in recognizing the needs of a longsuffering community. And by implementing quality care, facilities, and adherence to treatment for those in the throes of this disease, we can undoubtedly help improve the overall quality of someone’s
Few things can be more heartbreaking for a nurse than watching a patient who is in pain and knowing there’s little that can be done about it. Every day, however, patients come to hospitals across the country with severe, debilitating pain—yet instead of receiving compassionate care, they’re met with cold indifference. These patients have sickle cell disease.
“A lot of nurses and health care professionals in general have preconceived, erroneous ideas that sickle cell disease patients are just drug seekers,” explains Bonnye Johnson, RN, MSc, community health educator and outreach coordinator at the University of Illinois at Chicago (UIC) Sickle Cell Center. In reality, these patients are hurting, and it’s often up to nurses to make sure they receive the pain control and disease management care they urgently need.
Sickle cell disease, also known as sickle cell anemia, is the most common inherited blood disorder in the United States, affecting 70,000 to 80,000 Americans. It causes the body’s red blood cells to harden and become shaped like crescents, or sickles, instead of resembling healthy, round discs. As these sickle cells travel through the bloodstream, they tend to get stuck in narrow blood vessels, cutting off the flow of blood and causing excruciating pain.
This serious disorder is most common among people whose ancestors come from Africa, South and Central America, the Arabian Peninsula, India and Mediterranean countries, including Turkey, Italy and Greece. In the U.S., it is especially prevalent in the African American population, where an estimated one in 500 people has the disease.
The only cure for sickle cell disease is a bone marrow transplant, but it’s a risky procedure that’s not available to most patients. The alternative is to simply manage the chronic discomfort. Even with excellent home care, many patients regularly suffer sickle cell crises that cause terrible pain, typically in the back, chest, legs or joints. The pain often forces patients to rush to the emergency room to be treated with powerful pain medicine— usually narcotics, such as morphine and oxycodone.
Pain and Prejudice
Because sickle cell disease disproportionately affects people of color, all too often these patients must overcome barriers of stigma, bias and cultural misunderstanding to receive the care they need. Sadly, once many patients arrive in the ER, they face hourslong delays. As an added insult, once they do receive attention, they often face skeptical nurses and doctors who believe they’re there to abuse the system. Sometimes the patients don’t receive any medication at all.
Tosin Ola, RN
“I’ve been discharged from the ER while still crawling on the floor in pain,” says Tosin Ola, RN, a nurse from Portland, Ore., who has sickle cell disease. “It felt like no one wanted to help me.” “I’ve worked in ERs at several hospitals,” says DeEtta Cook, RN, a graduate student and full-time nurse at DePaul Health Center in St. Louis. “Regardless of the patient’s clinical indicators, there seems to be a perception that sickle cell patients are just there because they’re seeking narcotics. When patients request pain medicine, I’ve sometimes heard nurses say to each other, ‘Oh, that person just wants to get drugs. They know they’re not in that much pain.’ This starts the beginning of what I would call culturally incompetent care.”
Part of the problem, says Michelle McCreary, RN, who works at the Banner Good Samaritan Medical Center in Phoenix, Ariz., is that sickle cell patients may not always “look” sick. “These patients are not swollen or bleeding. Instead, they have joint pain. Since they don’t always exhibit pain symptoms outwardly, they feel that they’re often ignored,” she explains. “I hear stories like this all the time. The patients become extremely frustrated because they feel like they’ve been stereotyped as drug addicts.”
In many cases, minority patients who have encountered this type of discrimination are reluctant to make subsequent visits to the hospital, even if they’re having a pain crisis. “What happens instead is that they’ll often wait until their pain level is too high before they come and seek treatment,” says Johnson. Unfortunately, waiting too long in a sickle cell crisis can increase the risk for severe—even deadly—complications.
Furthermore, patients who have had bad past experiences in the emergency room are more likely to become defensive or hostile on subsequent visits. This escalates the problem, creating even more barriers. “Nurses are often offended by how these patients [present] and how they communicate,” says McCreary. It’s important for nurses to walk in the patient’s shoes, Johnson emphasizes. Many people with sickle cell disease were told that they wouldn’t live past their 20s. They often live with chronic discomfort.
“Imagine that you’ve been in pain a large portion of your life. You’d probably have a lot of social, emotional and self-esteem issues,” she points out.
With that in mind, nurses shouldn’t take it personally if a patient reacts negatively. Instead, the best solution is to treat the person with dignity and empathy.
“Many people don’t understand how deep the connection is between the nurse and the patient,” says Ola. “But for me [as a sickle cell patient], it impacts how I see the whole admission process. If I believe my nurse is there for me, I feel a whole lot better.”
Here are some additional ways nurses can close the gap of pain management disparities and provide culturally competent care to all sickle cell disease patients.
Ask, Then Listen
Culturally sensitive sickle cell care begins with looking beyond the preconceived notions and stereotypes to see the real patient underneath.
“You have to become the patient’s advocate,” says Cook. “Ask them if they’re in pain, and keep asking them if they’re still in pain. Ask them even after the initial dose of medication has been administered. Don’t arbitrarily decide to stop treatment just because you don’t want to give them drugs.
“Nurses should not allow their subjectivity to determine how they treat patients,” she continues. “Just view the patient as someone who’s in pain and needs health care, not as someone who’s seeking drugs. The measure that you have for if a pain medication is working is what the patient tells you.” Adds Johnson, “These patients have been in pain all their lives. Anyone who lives with chronic and acute pain tends to know what works for them in terms of pain management.” So listen to your patient!
This may seem like obvious advice, but the facts suggest that physicians and nurses don’t always follow it. “When we look back at the data, we find that [sickle cell] children actually receive more narcotics than the adults,” says Evelyn L. Brown, MSN, RN, CNS, coordinator of the sickle cell disease program at Children’s Hospital of Wisconsin in Milwaukee. Of course, an adult would need more medicine than a child, but apparently health professionals are not providing the appropriate level of pain medication when asked.
Another reason why practitioners may withhold opioid pain medication from a sickle cell patient is fear that the patient will develop a narcotic dependency. “But we [doctors and nurses] don’t make a person a drug addict. That’s an internal mindset,” Brown argues. “Instead, the key is to monitor the patient and establish a relationship. If [I have a good relationship with the patient], we can talk and we can work together [to find the safest medical solution].”
While it is often easier to build a mutually trusting relationship when nurses and patients share the same ethnicity, it’s not an absolute necessity. Majority nurses who work with minority sickle cell patients say that listening to patients and treating them with respect can go a long way toward bridging cultural gaps.
“When I first took this position, I wasn’t sure if I’d be accepted as a teacher and role model for a predominately African American population,” says Diane Batham, MSN, RN, PHN, a pediatric sickle cell nurse educator and coordinator at Kaiser Permanente West Los Angeles Medical Center. Batham is Caucasian. “But when I spoke to my patients’ families about it, they said, ‘Oh, Diane, don’t be silly, you know us.’ They told me that what mattered most to them was that I took the time to ask questions and to get to know them.”
Another important reason to establish a relationship with your patient is that you’re more likely to identify complications. Nurses can never assume that a patient who is having a sickle cell crisis just needs to get their medicine and go home. If a patient presents with chest pain, he or she could be experiencing acute chest syndrome (ACS). This extremely serious complication occurs when blood vessels in the lungs become clogged with sickle cells. ACS greatly increases the risk of infection and lung damage, and requires urgent care. Other complications include blindness, pneumonia and stroke. The results can be fatal.
“Many times, nurses are shocked when someone dies from sickle cell complications,” says Brown. But if nurses are knowledgeable about what to look for and make a point not to simplify the patient’s problems, they can recognize the signs of a life-threatening crisis and intervene appropriately.
Get Families Involved
“Sickle cell disease affects the whole family, so taking care of patients—whether they are pediatric or adult—involves everyone,” says Batham. “Nurses must do their best to make sure that all the people who are important to that patient are involved in helping to care for him or her.”
Families can also help by creating a home environment that eliminates triggers for sickle cell crisis, such as extreme heat, cold or increased stress.
Where to Go for More Information The Sickle Cell Information Center at Grady Health System Centers for Disease Control and Prevention MedlinePlus (National Library of Medicine) National Heart, Lung and Blood Institute Sickle Cell Disease Association of America International Association of Sickle Cell Nurses and Physician Assistants National Marrow Donor Program
Nurses should make sure patients’ family members know the importance of being on the national bone marrow donor registry. “Often a family member match for the patient, or for someone else,” says Brown. “We try to remind everybody about the registry. They could be the one to help save a life.” Because sickle cell disease is hereditary, nurses can help educate parents whose children have been diagnosed with the disorder, as well as sickle cell patients who fear that their future children may inherit the disease. Sickle cell disease is caused when each parent carries a recessive gene for the disease, and both parents pass the sickle cell gene on to their baby. Children who are born to parents who both carry the sickle cell trait have a one in four chance of inheriting the disease. Babies who receive the sickle cell gene from only one parent will not have the disease, but will be carriers of the trait.
“Our hospital tests newborns for sickle cell, and out of all our infants who test positive, probably only 40% of the parents knew they were carriers. The other 60% are just totally shocked,” says Brown. “Many of these parents have other children who are healthy, so they are especially surprised when they find out they have a child with sickle cell disease.”
Nurses who treat adult sickle cell patients who want to have children can assist them in finding resources, such as genetic counseling. It is definitely possible for mothers with sickle cell disease to have healthy pregnancies, but nurses can be an important link in helping them become better informed about their options.
Become an Advocate
Nurses as a group can use their powerful collective voice to speak up for more equitable funding for sickle cell disease research. There are huge disparities between the amounts of federal research dollars allocated to sickle cell when compared to other disorders.
For example, every year there are three times as many babies born with sickle cell disease than with cystic fibrosis—8,000 versus 2,500. However, according to the National Institutes of Health, for every baby born with CF, there are $2,733 research dollars spent to find a cure. In contrast, for every baby born with sickle cell anemia, only $345.58 is spent. That’s less than oneeighth of the CF budget per infant. While it’s important to find cures for both disorders, nurses can be important advocates in making sure sickle cell disease isn’t ignored.
Nurses can also become patient advocates by volunteering with non-profit organizations that help sickle cell patients, participating in committees to improve quality of care and donating money to sickle cell charities.
Perhaps most important, nurses can advocate for more culturally competent sickle cell care by educating their peers about the disease, so that other nurses don’t fall prey to the common misconceptions and prejudices that can create barriers to treatment for patients of color.
“As a young nurse, I was brought up to [be culturally knowledgeable about this disease], because I was mentored by some great people,” says Batham, who’s been a nurse for over 21 years. “I would encourage other nurses to become role models.” All patients who have sickle cell disease deserve to be treated equally, and the care they receive should be based on objectivity, compassion and respect. In many cases, it’s up to the nurse to make sure that happens. “We have to convey to the patient that they have rights,” says Cook. “If they’re in pain, they deserve to be treated.”
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