Imagine the worst pain you’ve ever felt — a gnawing, aching, sharp and burning pain — and you have little control over it. The pain is so bad you can’t lift a spoon to eat. Sometimes the pain is so unbearable you can’t manage it at home and have to go to the emergency department.
Of course, you would rather stay home, but you know the pain could be very serious, even life threatening. You’re tired of fighting to be believed, fed up with being labeled a drug seeker because you have an invisible illness.
This is what life is often like every day for more than 6 million people worldwide who live with sickle cell disease (SCD). September is National Sickle Cell Awareness Month, and it’s important to raise awareness of SCD and sickle cell trait (SCT).
Most of the 100,000 people living with SCD in the U.S. are either Black, or they are African Americans who can trace their lineage back to Africa. However, SCD affects people of all ethnicities and backgrounds. SCD, which includes sickle cell anemia, sickle cell beta thalassemia, sickle hemoglobin C disease and SCT, is an autosomal dominant genetic disorder that is passed from both parents to their children. Sickle cell anemia, the most severe form of SCD, occurs when both sickle cell genes (hemoglobin S or HbS) are inherited from each parent. SCT occurs when only one HbS gene is inherited and typically doesn’t cause symptoms. People with SCT can pass HbS to their children.
When a person with SCD is exposed to conditions that cause hypoxia and/or dehydration, low blood pH, changes in temperature, high altitudes, stress, infections, pregnancy and other medical conditions, HbS causes the erythrocyte to change into a sickle shape and become sticky. This can cause sickled erythrocytes to become occluded in smaller blood vessels anywhere in the body, leading to severe pain and organ infarction. Hemolysis and subsequent hemolytic anemia occur because erythrocytes that are repeatedly sickled have a life span of only 17 days (compared with 120 days for a typical erythrocyte). Once the causative condition has been reversed, the sickled erythrocyte changes back to the typical round, concave shape.
Since blood travels almost anywhere in the body, the complications from SCD are vast. Following are some of the more common sequelae that may be seen by progressive and critical care nurses.
Pain is the number one reason people with SCD seek emergency care. While pain can be acute, such as in a sickle cell crisis, people with SCD can also have chronic pain.
Acute Chest Syndrome (ACS)
ACS is the leading cause of death for people with SCD. These patients can deteriorate quickly, requiring critical care due to rapid respiratory compromise and multiorgan failure. ACS can be caused by bone marrow necrosis and/or fat emboli, infection, asthma and COVID-19.
Silent Cerebral Infarctions and Stroke
People with SCD can experience cerebral infarctions that are detected only with an MRI. The patient has a normal neurologic exam. People with SCD can also have ischemic strokes.
Chronic Kidney Disease
One-fourth to one-third of people with SCD have chronic kidney disease. Nephrotoxic agents should be avoided, if possible.
Currently, the only approved cure for SCD is an allogeneic hematopoietic stem cell transplant. Lack of donors and the risk of graft-versus-host disease or graft failure limits this option for many people. Emerging gene therapy using clustered regularly interspaced short palindromic repeats (CRISPR) or lentivirus vector is in the investigational stages. CRISPR therapy has been used to cure two people of SCD. Hydroxyurea can help reduce an acute sickle cell episode, but it has many adverse effects that may limit its use.
Racism and SCD
Black Americans and African Americans with SCD face the double-edged sword of an invisible disease and systemic racism. Systemic racism doesn’t single out a specific person, but the way our institutions, practices and laws have been created over the centuries creates a disadvantage for certain groups of people. Consequently, Black Americans and African Americans often don’t have equitable access to the tools needed to live a healthy lifestyle, including quality medical care, a clean living environment, safe housing and quality education. SCD inequality is represented in philanthropic expenditures, approved medications, the number of academic publications and industry-supported clinical trials when compared with other genetic diseases that affect people of a white or Caucasian ethnic background.
As progressive and critical care nurses, we are responsible for advocating for our patients. Learn what you can do to advocate for patients with SCD by reading Alexandra Power-Hays and Patrick McGann’s article, “When Actions Speak Louder Than Words – Racism and Sickle Cell Disease” (2020).
More Information on SCD and SCT
For a more in-depth discussion on SCD and SCT, access the 2021 AACN NTI session that I co-facilitated with Dianna Copley, DNP, APRN-CNS, ACCNS-AG, CCRN.
How have you advocated for more equitable care for your patients?
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