What group of our patients is diagnosed with a devastating illness in early childhood which gets increasingly worse with age and leads to debilitating pain which is both chronic and acute in nature?
Colleen Castelein, AOCNP
Colleen Castelein is an advanced practice nurse with the GI and sickle cell disease teams at Seidman Cancer Center, University Hospitals Case Medical Center, and a member of the clinical faculty at the Frances Payne Bolton School of Nursing, Case Western University in Cleveland, Ohio.
Here is a quiz for hematology/oncology nurses: What group of our patients is diagnosed with a devastating illness in early childhood which gets increasingly worse with age and leads to debilitating pain which is both chronic and acute in nature? Every organ system is affected. These patients lose their spleens at an early age and are at risk for infections which may prove fatal. The disease has far-reaching effects for both the patient’s ability to hold down a job and lead a full life. While lifespan for these patients has increased from a mere 20 years 2 decades ago to well into their 50’s and 60’s today, most patients live with the knowledge that they are at risk of sudden death from infection and acute chest syndrome at any time.
You likely guessed it: sickle cell patients. They have the most common inherited blood disorder, but their suffering is often under-recognized and under-treated by health professionals. Sickle cell disease is not rare. The disease itself affects one out of every 500 African Americans, and one out of 12 is a carrier of the gene and has the sickle cell trait.1 Approximately 100,000 Americans have the disease.1
Some advances have been made to improve the care of sickle cell patients, improve quality of life, and increase lifespan. Comprehensive vaccination programs have prevented infections with devastating consequences, and hydroxyurea therapy has contributed to some decrease in both painful crises and acute chest syndrome episodes. Increasingly, patients are getting appropriate pain management interventions, but still pain crises are woefully underappreciated and undertreated. More research and concentrated clinical care programs are needed to help these patients throughout their lives.
At the Seidman Cancer Center we are trying to address this need. We established a formal program for caring for sickle cell patients. Under the leadership of a dedicated hematologist, Jane Little, MD, and two nurse practitioners, Tara Alin NP, and myself, we coordinate the care of all the adult sickle cell patients in our system. In the 5 years that we have been working together, we have learned a lot. Here are some of the lessons learned:
The National Heart, Lung and Blood Institute has recently published new evidenced-based guidelines for the care of the patient with sickle cell.2 Review them when you can. Reach out to your hospital administrator or head nurse to take steps to improve care for this underserved population. Help us address the suffering. Join us in treating these patients with the compassion and attention they deserve.