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Reducing Care Fragmentation in Sickle Cell Disease

Comprehensive, multidisciplinary services that provide whole-person care can be difficult to find. Experts are changing that.
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Sickle cell disease is a genetic blood disorder predominantly affecting people of color. The abnormal gene results in misshapen, sickle-shaped red blood cells that become “stuck” in tiny blood vessels throughout the body and block blood flow and oxygen to organs. Complications range from daily chronic pain and acute, unpredictable episodes of severe pain in the chest, back, abdomen, and joints lasting between a few hours to a few weeks — known as vaso-occlusive events — to stroke and organ damage predominantly affecting the lungs, heart, kidneys and brain. SCD impacts nearly every blood vessel of the body. As the disease persists without proper treatment and medical intervention, the body suffers irreversible damage.

For the 100,000 individuals living with sickle cell disease (SCD) in the United States today, their chances of survival in adulthood are not significantly greater than if they were living with the disease 40 years ago. It is a stark reality considering the improvements in survival rates for other serious conditions, including some cancers and HIV, in shorter periods of time. And while there are a few contributing factors — for example, up until 2017, there was only one FDA-approved treatment for sickle cell disease, and research remains largely underfunded — arguably the most detrimental of all is the fragmentation of healthcare.

Despite widespread knowledge about the harmful effects of SCD, the overall healthcare landscape for the disease is dangerously sparse. Sickle cell disease has long been associated with disparities in access, treatment, and outcomes. In many areas of the country, medical intervention is typically only available when a patient suffers a vaso-occlusive event and needs emergency care for pain management. As such, people may receive the majority of their care within the emergency department. If the hospital doesn’t have proper care protocols in place or misunderstands SCD, a patient might become so ill they require hospitalization, which often means missing work and school — an effect that has downstream consequences on quality of life and ability to thrive — or providers may mistake their requests for helpful pain medication as drug-seeking behavior, leaving them with longer wait times for the care they need.

“There remains a lot of work to do as we work toward improving the quality of life for patients with sickle cell disease, ” says Elizabeth Klings, MD, director of the Center of Excellence in Sickle Cell Disease at Boston Medical Center, the largest sickle cell center in New England. “That lands on shifting the paradigm of care from the emergency department and the inpatient hospital to an outpatient setting. We envision a future where patients can participate in their normal daily lives as much as possible, without disruption by visits to the emergency department and stays in the hospital.”

Care fragmentation vs. comprehensive care for sickle cell disease

Given SCD impacts all areas of the body, it’s especially important for numerous specialties to work together on an integrated care team. However, Klings explains that there is almost no longitudinal care due to the relative paucity of physicians who are qualified to take care of patients with this disease and who are interested in doing it — it’s not necessarily a lucrative practice, since a majority of patients receive their insurance through government payers.

The Center for Excellence in Sickle Cell Disease at Boston Medical Center cares for both pediatric and adult patients — approximately 190 children and adolescents and 345 adults — allowing them to provide healthcare across patients’ lifespans. Their goal is to help patients overcome some of the most significant barriers by providing comprehensive and tailored multidisciplinary care through individualized treatment plans. The plans are created as a collaborative effort among all members of the medical team and then embedded within the electronic medical record. This approach directs patient care, but is also a useful tool for collecting and evaluating clinical data to inform best practices and guidelines.

“Buy-in from numerous physicians in other specialties gives us the ability to create a multispecialty network,” says Klings. “We’ve assembled a nationally- and internationally-recognized group of hematologists and experts in other subspecialties, particularly pulmonary medicine, who are at the forefront of providing the best care possible for SCD patients.”

The Center’s Pediatric Sickle Cell Program involves hematology and pulmonology providers working closely together to ensure the family is equipped with educational tools and resources to manage the child’s disease. These experts liaise with providers in the Pediatric Pain Clinic, who help young patients cope with pain using integrative medicine practices such as aromatherapy, acupressure, and mind-body strategies.

For adult care, physicians in nephrology, orthopedic surgery, ophthalmology and other subspecialties work closely to help manage the complex, multisystem disease patients are facing, says Klings.

"Improving the quality of life for patients with sickle cell disease lands on shifting the paradigm of care from the emergency department and the inpatient hospital to an outpatient setting." Click To Tweet

Over the years, the Center has also launched a formal multidisciplinary clinic that takes place twice a month to allow patients to see their primary care and specialty physicians in the same visit. The team has implemented an outpatient alternative to the emergency department for vaso-occlusive event treatment. At the onset of an event, patients can make an urgent appointment in the Center’s Infusion Therapy Center, as it’s called, where they are seen by a care team specializing in protocols for treating vaso-occlusive events.

In addition to achieving better alignment among clinical specialties for patients, the Center also alleviates barriers to care through social determinant of health screening and referral. Transportation and food insecurity tend to be the most common issues patients face, so the Center provides gas and grocery gift cards. Because the hospital is a flagship for SCD care, many patients need to travel from great distances, which isn’t always easy. In response, the Center also recently partnered with Uber Health to arrange rides for patients who reported transportation challenges. The pilot program reduced no-show rates by 8.5%, indicating that more patients were able to receive their care — with just a bit of creative and low-cost problem-solving.

Looking ahead: Casting a wider net

While the increase in established primary care among adult patients that the Center has witnessed in recent years is promising, Klings and her colleagues are cognizant of the fact that not every person with SCD has access to care of the Center’s caliber.

A key goal for the Center over the coming years is to expand its reach to even more patients and clinicians. After seeing a positive reception to telehealth during the COVID-19 crisis, providers are no longer looking at geography as the limiting factor it once was. Now, efforts are underway to establish care with SCD patients in Western Massachusetts as well as Vermont, New Hampshire, and Maine.

Moreover, initiatives are in process to set and share best practices for care to bridge gaps. Protocols that were developed by Klings and colleagues are being used or modified for use at many other centers nationally, she says. For example, as part of the Medical and Research Advisory Council of the Sickle Cell Disease Association, Klings helped establish national protocols for caring for SCD patients infected with COVID-19. Through telementoring, physician experts from the Center offer their guidance, expertise and feedback about difficult SCD cases to healthcare professionals around the country. Many other physicians from the Center participate in the New England Pediatric Sickle Cell Consortium to discuss challenging cases and other topics relevant to SCD. These and other efforts ensure the clinical excellence taking place at the Center expands well beyond the walls of one hospital to transform care for SCD patients, wherever they are.

As Klings and her colleagues move full steam ahead to improve outcomes for SCD patients both locally and nationally, they recognize they are only at the beginning of a long, albeit rewarding, journey.

“We’re getting closer every day,” she says.

Friday, June 19 is World Sickle Cell Day, and BMC’s Center of Excellence in Sickle Cell Disease is hosting a webinar to highlight the impact COVID-19 has had on patients with SCD. Moderated by President and CEO of New England Blacks in Philanthropy Bithiah Carter, the virtual event will feature panelists Elizabeth Klings, MD; George Murphy, PhD, co-director of BMC’s Center for Regenerative Medicine; Melisa Ruiz-Gutierrez, MD, BMC pediatric hematologist and medical oncologist; and David Henderson, MD, chief of BMC’s department of psychiatry. To RSVP for this one-hour occasion, happening June 19 at 11am, please click here.

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Kristen Jackson


Kristen Jackson is a communications specialist in the Office of Development at Boston Medical Center. She has a BA in English with minors in Health Studies and Spanish from Siena College and an MA in Health Communication from Emerson College/Tufts University School of Medicine.