Sickle Cell Disease (SCD) Patients Often Mistreated: What Nurses Can Do

September is National Sickle Cell Awareness Month. Sickle Cell hits close to home for me. My nephew has the trait. My goddaughter has the disease and is frequently in crisis. Several other family members and friends are also fighting this illness. 

One of the most common issues they all deal with is treatment by healthcare professionals — particularly nurses — when they seek treatment for this disease. It’s shameful, but often their symptoms often cause them to be mistaken for:

• Opioid seekers
• Drug abusers
• Liars
• Over exaggerators

These are all terms thrown at people with Sickle Cell Disease (SCD). The treatment — or mistreatment — of Sickle Cell patients contradicts our title as the most trusted profession. 

So, nurses, what can we do? How do we advocate, treat, and care for this population of patients? 

What is Sickle Cell Disease?

SCD is a group of red blood cell disorders. Unlike healthy red blood cells (RBC) which are round, patients with SCD have RBC that take on a “C” or sickle shape. Sickle cells die early — which causes a constant shortage of red blood cells. 

SDC commonly affects those whose ancestors came from: 

• Sub-Saharan Africa
• Spanish-speaking regions in the Western Hemisphere 
• Saudi Arabia
• India
• Mediterranean countries 

The only cure for SCD is bone marrow or stem cell transplant. 

Stigma and Treatment

When sickle cells travel through small blood vessels, they get stuck and clog the blood flow — causing excruciating pain and other serious problems including:

• Infection
• Acute chest syndrome
• Stroke
• Death

SCD warriors and their caregivers report being stigmatized when they seek care. With patients showing signs as early as birth, nurses’ attitudes can contribute to negative stigmatization and may affect patients' response to sickle cell cues, potentially causing patients not to seek care and negatively impacting patient outcomes. 

Cleverly Changing founder Elle Cole's daughter has SCD. She gives a brief description of an ER visit after a physician’s assistant at their primary care office suggested her daughter go to the nearest hospital via ambulance. 

Elle recalls, “In the ER, the nurse was upset and asked why we were there and which clinic sent us. She stated my daughter didn’t need any oxygen, the hematologist was busy (but would come in about an hour), and she needed to get a mucus sample. My daughter was scared and started to cry. Then, the nurse told four nurses to join her, and they proceeded to hold my daughter down and extract the mucus from her nostrils. I was completely terrified! My husband was at work. I felt alone and scared with my daughter.”

One mom, Shaynise Robinson, drives three to four hours to seek care for her daughter, because of the lack of understanding from nurses and other healthcare professionals at their local hospital. 

In an article posted on Pubmed, researchers found that sickle cell patients in one hospital waited for 60% longer to get pain medication although other patients reported less severe pain. They were also triaged into a less serious category. 63% of nurses surveyed said many patients with sickle cell are addicted to opioids, according to another study. But according to Dr. Alexis Thompson, president of the American Society of Hematology, rates of addiction among SCD patients are no higher than the general population’s. 

SCD Patients & Caregivers Describe Their Pain

• Ayana Spearman, RN, BSN, was diagnosed with Sickle Cell SS at just three months old. Asked to describe her pain, she says, “As a child, I knew when I was going to have a crisis because I would start getting the feeling of general malaise, then the pain would come. The pain was mainly sharp in my chest and/or arms and legs. As an adult, the pain is usually a sudden onset and is sharp. The pain starts in my arms and legs or starts in my lower back then moves to my legs. After college, I was in a bad crisis. I went to the hospital — they gave me pain medications and sent me home. The next day I went to a different hospital, and again the same thing happened.  At this point, I’d been in a pain crisis so long that I kept passing out from the pain. I even asked my husband to kill me because the pain was so bad.”
• Jessica Desire, RN, diagnosed with SCD at three years old, says, “The best way I can describe crisis pain is like I'm a Barbie doll and my arm or leg has been pulled off. If I do not find a comfortable position during a crisis, it feels like someone is hitting me with a hammer, and my joint is shattering.”
• Elle Cole’s ten-year-old daughter was diagnosed at birth. When asked to describe the pain her daughter experiences, Elle says, “Watching my daughter have a pain crisis is debilitating. A full-blown crisis for her has lasted several days. While in crisis, she continually cries — which sounds more like screams — and has a hard time walking or moving. The pain itself sometimes starts in her back and moves towards her legs. During her first crisis, I could even see her blood vessels pulsating where the red blood cells were clumped together. It’s a terrible feeling and horrible to watch because once in a full crisis it’s hard to stop them.”
• Shaynise Robinson’s daughter has SCD. The description she gives is haunting: “Imagine your one-year-old screaming at the top of her lungs, and nothing you do can console them. Nothing is working, and your baby is screaming and crying the worst pain cry you've ever heard in life! Her little body shakes and quivers, riddled with so much pain. There have been times I sat in the same spot and position, overnight, because she was finally able to find a comfortable position. I was afraid if I moved one inch it would disturb the little peace she’d found.”

What Can Nurses Do to Help?

Elle Cole, who has a blog that brings awareness to SCD, wants nurses to be patient. Asking questions that indicate that you are genuinely concerned about your patient’s wellbeing can help open communication with your patient. 

Shauna Chin, RN, says, “In my experience, in addition to the pain, many patients with SCD exhibit symptoms of depression. The nurse needs to distinguish between solemness due to pain and solemness due to despondence. Many symptoms of depression go undiagnosed and can be remedied by encouraging health providers to engage in dialogue with the patient.” She expresses that nurses can advocate best for patients with SCD by identifying early non-verbal signs and symptoms of pain and anticipate their patient's needs.

Ayana Spearman, RN, BSN, believes more education is needed during nursing school. Noting in the last five years, few co-workers were adequately informed about the disease. “SCD was just 'glanced over' and not taught about in-depth, in nursing school,” she says. Spearman believes that SCD patients need to have a holistic care approach.

Shaynise Robinson encourages nurses to go through diversity training. “Give patients the benefit of the doubt. Understand that they are looking for pain relief and equal treatment,” she says. Proper bedside manner is a concern for her as well. 

Sickle Cell Disease doesn't just affect the body, but the holistic health of those with the disease. Education and awareness are critical for nurses to provide proper care. Shaynise Robinson leaves us with this: “The same compassion that’s shown to cancer patients should be shown to sickle cell patients.”

To learn more about sickle cell visit:

Sickle Cell Disease Association of America

American Sickle Cell Anemia Association

Sickle Cell Society

Portia Wofford is a staff development and quality improvement nurse, content strategist, healthcare writer, entrepreneur, and nano-influencer. Chosen as a brand ambassador or collaborative partner for various organizations, Wofford strives to empower nurses by offering nurses resources for development–while helping healthcare organizations and entrepreneurs create engaging content. Follow her on Instagram and Twitter for her latest.