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.
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.
“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.
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.
“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.
Nurses should make sure patients’ family members know the importance of being on the national bone marrow donor registry. “Often a family member [is the best] 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.”