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:
Comprehensive, interprofessional and multidisciplinary outpatient care with specialists under one roof improves care.
Not only do we work together as a team, meeting once a week to discuss all patients seen that week, and twice a week with the inpatient team, we invite specialists from cardiology, nephrology, and pulmonary to join us in our clinic. They see our patients where we do, and we can collaborate in real time.
It takes a village—our patients deserve a wide range of services.
We found that our young adult patients were missing appointments and having problems navigating our complex healthcare system and the social system they live in. Now a lay navigator works with them to help them find their way, and a full-time social worker is a permanent part of our team. Music therapists are available at crucial points of care offering our young patients a special form of therapy they can readily relate to.
The emergency room is not the best place to treat painful crises. An alternative to the emergency room manages patients more effectively when they have a painful crisis, increases patient satisfaction, and keeps our patients out of the hospital.
Ten years ago we established a fast-track clinic in our infusion center. We see 6-8 patients per day there. We can treat pain for a longer period of time, see patients for multiple days, and get pain medicine to them more quickly. Today patients who go to our emergency room for a painful crisis are admitted approximately 50% of the time. We admit patients about 20% of the time. A dedicated group of nurse practitioners and clinical nurse specialists run the clinic and know the patients well.
Transition from pediatric clinic to the adult clinic is an important time and needs special attention.
This is a time when our patients are seeking independence from parents and learning to make their way in the world. We need to manage this transition and treat our young adult patients with respect and kindness. It is also a time when their disease symptoms increase in severity. We are still experimenting with ways to help in this transition. Music therapy has played a big role in our patients’ care.
More research is needed to treat both the symptoms of the disease and the disease itself.
Other centers, particularly Johns Hopkins, have led the way in bone marrow transplantation, the only known way to cure this disease. While we are planning to initiate this therapy for some of our patients, many will not be candidates. We are participating in a variety of research projects to improve care for our patients. Research is needed in all aspects of care, including quality of life strategies.
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.
US Department of Health and Human Services. National Institutes of Heath. National Heart, Lung and Blood Institute Disease and Conditions Index: What Is Sickle Cell Anemia? http://www.nhlbi.nih.gov/health/ dci/Diseases/Sca/SCA WholsAtRisk.html. Accessed November 17, 2014.
US Department of Health and Human Services. National Institutes of Heath. National Heart, Lung and Blood Institute. Evidence-Based Management of Sickle Cell Disease: Expert Panel Report 2014. https:// www.nhlbi.nih.gov/health-pro/guidelines/sickle-cell-disease-guidelines/. Accessed November 17, 2014.