FACING THE PAIN OF SICKLE CELL DISEASE
By: Genice Nelson, D.N.P., APRN, ANP-BC
Imagine being taken to the hospital in severe pain, then being dismissed without treatment or care. Now, imagine if you were a physician who was in severe pain, and you were still ignored. How would you feel? Helpless? Angry? Desperate? All of the above? Unfortunately, this isn’t a made-up scenario. It’s exactly what happened to a physician I know, who happens to live with a debilitating illness that is common, incurable, yet one we know way too little about: sickle cell disease.
Sickle cell disease is a lifelong, genetic blood disorder that causes the body to produce abnormally shaped red blood cells. Normal red blood cells move easily through the blood vessels and carry oxygen to all parts of the body. Sickle cells, however, are hard, sticky and tend to clump together. The cells then get stuck and literally block the flow of blood to the organs and limbs, causing pain, anemia and organ damage.
Among black Americans, one in every 400 babies is born with the disease. And the incidence of the sickle cell trait, the carrier status, occurs in one in 12 live births. In the U.S., sickle cell disease affects more than 100,000 people, while globally SCD affects more than 3.5 million people. SCD is also common in Spanish-speaking countries in South America, Central America and parts of the Caribbean.
Because of the nature of SCD as a chronic illness, the burden of care can be tremendous on families and communities. The most common reason that people with SCD seek medical care and acute care services is that they frequently suffer from sickle cell crisis pain episodes. In fact, patients with SCD utilize more emergency department services than patients with other more common chronic illnesses such as diabetes, asthma and hypertension.
This sudden, severe, acute pain is also the primary cause of distrust between the medical health system and those living with SCD. It is ingrained in trained medical professionals to seek objective reasons related to patient symptoms and disease processes. Unfortunately there are no known tests or procedures that can definitively show whether an actual SCD pain crisis is occurring or not. That is why, when people engage the medical system, they are often given erroneous explanations for delays and subpar care such as “your blood counts look good, you can’t be having pain” or “we are waiting for your test results before we can give you any treatment” or “we can’t give any opioid medications for sickle pain,” which only leads to prolonged suffering of the person in pain.
My friend’s experience was all too common. After being released without treatment the first time she went to the hospital, she returned a few days later. She was fatigued and was struggling to breathe, but she wasn’t given any IV fluids, antibiotics or pain medications even though she was in pain with each breath. Fortunately, she had an advocate with her at the hospital who demanded the attention of her physicians — but not before she ended up in the intensive care unit with acute chest syndrome.
Acute chest syndrome is one of several serious life-threatening complications of SCD, and it needs rapid treatment to prevent fatal outcomes. My friend was blessed someone advocated on her behalf to save her life. Unfortunately these episodes occur too frequently with many people succumbing to the complications — not the disease itself. As my friend told me, “I’ve had two near death experiences, not because of my sickle cell disease but because as a patient I was never acknowledged.”
As providers we must do better. We need to remain empathetic and engaged to serve our patients as humanely as possible, recognizing there is very limited ammunition to fight debilitating pain.
Genice Nelson is the adult nurse practitioner for the UT Southwestern Medical Center Comprehensive Sickle Cell Disease Program and serves on the National Sickle Cell Disease Association of America’s Board. She wrote this column for The Dallas Morning News.
