Rachel Rangwala, MD
Chief of Clinical Pathology and Medical Director of Transfusion Medicine
Department of Pathology
Harbor-UCLA Medical Center
Los Angeles County has more adults with sickle cell disease (SCD) than anywhere else in California,1 making it an ideal and vital location for a treatment clinic. In 2018, Harbor-UCLA Medical Center stepped up to the challenge in a major way, and Rachel Rangwala, MD, led the establishment of a program offering outpatient automated red blood cell exchange (RBCX) transfusion therapy for patients with SCD.
Recently, the program earned a prestigious Top 10 County of Los Angeles Productivity and Quality Award, which honors programs that develop innovative ways to empower some of the county’s most vulnerable populations. Harbor’s use of RBCX for SCD resulted in better management of patients’ pain crises, reduced emergency visits and hospitalizations, and improved patient quality of life. In the process, the hospital also achieved significant Medicaid-related savings for the county.
Terumo Blood and Cell Technologies is also committed to improving therapy options for SCD, so we were excited to learn more. Rangwala, Harbor’s Chief of Clinical Pathology and Medical Director of Transfusion Medicine, generously shared her time in a recent chat to answer our questions.
Disease Overview at a Glance
SCD is the most common genetic disease in the U.S.2 It is often debilitating without treatment and can impact every aspect of a patient’s life. It can cause extreme pain and increased risk of infection, stroke, vision loss, and more. Lifespan for a patient with SCD averages just over 52 years — about 20 years less than the general population.1 In California, the situation is worse, with lifespans averaging nine to 11 years less than the national average for women and men, respectively.
While simple blood transfusion therapy has been a mainstay for managing the disease, the use of automated RBCX as a common therapy is also gaining traction. Harbor’s clinic is a prime example of how it can work well for patients.
Access Point: Congratulations on being recognized for your work and on the establishment of the Harbor-UCLA outpatient exchange transfusion clinic. Can you share some of the results with us?
Rangwala: Absolutely. First, emergency visits have declined tremendously for patients treated with RBCX. Hospitalizations decreased. And for patients who were hospitalized, length of stay was reduced, as well.
Harbor-UCLA Exchange Transfusion Clinic Patient Experience
Source: Data provided by Dr. Rachel Rangwala, September 2023.
Access Point: Are there any stories of individual patients that stand out for you?
Rangwala: All of the patients in our program have reported improvement in quality of life. One patient has been able to go back to college, while working, and now has a goal of visiting his parents' homeland in Africa. Another is now close to achieving the dream of becoming an auto technician and getting a real estate license.
Of course, our first patient was memorable. He was going to the ER almost every other month, about five times a year, before we started doing RBC exchanges. Now visits are down to zero.
We also provided monthly RBCX therapy for three pregnant women with sickle cell disease. It was really nice to see three healthy babies born because of our program.
Access Point: Those are truly inspiring stories. Looking back, how did you get started on the path to these successes?
Access Point:
Those are truly inspiring stories. Looking back, how did you get started on the path to these successes?
Rangwala: Before, our team was predominantly doing outpatient hemotherapy for people who are not hospitalized but need certain blood therapies, including blood transfusions and platelet transfusions. We also did apheresis treatments using the Spectra Optia® Apheresis System, but we weren't performing exchange therapies very often as an outpatient service.
In 2018, we got a request from a hematologist who is an SCD specialist. She asked if we would do ongoing, monthly exchange transfusions for one patient. He'd had a stroke as a child and was having a lot of pain crises, ending up with emergency visits and hospitalization. He was struggling as a young adult. We really wanted to help him, so we said yes, we'll figure it out.
One of the major hurdles was making sure we would have enough blood for him. Because each exchange requires about nine units, on average, for one patient. We also had to figure out ongoing vascular access and ensure he would come in for all the testing. Those were some of the early obstacles — but they helped us plan for our next patient.
Access Point:
Bring us up to date — what does the clinic workload look like today?
Rangwala: We're up to eight patients doing monthly exchanges and five nurses trained for apheresis. I will say we're limited by how many nurses we have who are able to do this and how many devices we have. We're hopeful this award will bring more attention to our program — because not only do we want to expand, there's a need for us to expand. We are now the only hospital in LA County that has the technology and capabilities to do these red blood cell exchanges.
Access Point:
It seems your team has handled a lot of change in a short time.
Rangwala: It has definitely been a learning curve. We knew how to do the procedures and had what we needed. But we had to learn how to transfer that knowledge from acute situations in the hospital to monthly treatments for chronic patients. In many ways, that's actually more complicated.
Access Point:
How have you dealt with the volume of blood needed for SCD exchange treatments?
Rangwala: With each of these exchanges using between six and 10 units of blood, that's a lot to give to one person at one time. Also, in the U.S., many sickle cell patients are African American, and there is an inherent mismatch between these patients and our blood donor population. That often forces us to use universal red blood cells, O-negative. Using 10 units at a time of that universal blood is a big ask at a trauma center like ours.
On the positive side, for one patient, we have been able to decrease the required number of units from 10 to eight by using a more realistic estimated pre-procedure hemoglobin S (HbS) percentage.
Still, we have to work very closely with our blood suppliers to manage the volume needs. They know how important this program is, and they're committed to supporting it. We work closely on the need for extended blood typing and testing.
Access Point:
How has your team reacted to the challenges and to the recognition from the award?
Rangwala: Our nurses are very dedicated. I think they are gratified to be able to provide this kind of care for these patients who have a disease that is historically under-resourced and doesn't get the attention it deserves. We all hope to expand our offerings, including partnering more with a new adult SCD clinic.
Access Point: What would you say to someone who may be thinking about starting an exchange transfusion clinic?
Rangwala: Collaboration is essential. You really have to work strongly with your hematology clinicians, the ones who are seeing the patients, and decide together who would be a good candidate. These patients have to be motivated and dedicated to coming in at least twice monthly. You also have to collaborate with your interventional radiologist to get the right ports, get them placed, and train your nurses.
Access Point:
Any final words of wisdom?
Rangwala: I think it's important to remember this is a quality-of-life issue. If people with SCD don't have access to this type of treatment, the disease may make them unable to go to school, to have a job, to take care of their families. The benefit to this group of patients is very clear and worth overcoming any potential barriers.
About Harbor-UCLA Medical Center
Harbor-UCLA Medical Center is affiliated with the University of California, Los Angeles, and operates independently as part of Los Angeles County. LA County-operated hospitals make up the second-largest public healthcare system in the US.
Sources
1Jiao, B, Johnson KM, Ramsey, SD, Bender, MA, Devine, B, Basu, A. Long-term survival with sickle cell disease: a nationwide cohort study of Medicare and Medicaid beneficiaries.
Blood Adv. 2023;7(13):3276-3284.
2Perkes, C. New UCLA Health sickle cell disease center aims to increase life expectancy for patients.
UCLA Health News & Insights. Sept. 14, 2022. Accessed Sept. 18, 2023.