For people with sickle cell disease, add check-ins to checkups

Among the many lessons the Covid-19 pandemic has taught society are that individual behaviors matter; leadership counts; and the burden of Covid-19 is not equally distributed.

I’ve learned something more as a health care provider taking care of patients with sickle cell disease: Successfully caring for a vulnerable population with a chronic illness requires more than high-quality medical care. As a community health worker connected with the sickle cell population once told me, people need more than a checkup; they need a check-in.

Sickle cell disease is a genetic, inherited condition that affects millions of people around the world and an estimated 100,000 people in the U.S., primarily those of African and Middle Eastern descent. Even though the first patient with the disease was described in 1910, little meaningful progress in developing disease-specific therapies was made until recently. Scientists identified the gene responsible for sickle cell disease in the 1950s, but used their understanding of the inheritance and genetics of sickle cell disease primarily to advance the fields of molecular biology and genetics. Little was done to directly benefit those with sickle cell disease, primarily people of color.

It became another example of the disparities in health care that left racial and ethnic minority patients with few options to treat a disease that causes intense episodes of pain, strokes, organ damage, and premature death.

Research has advanced clinical care, focusing on children and young adults. Cutting-edge technology in the form of transcranial Doppler ultrasonography can now check individuals for their risk of stroke, an event that occurs in 11% of people with sickle cell disease before the age of 20. Yet according to a 2021 report from the Centers for Medicare and Medicaid Services, only 36% of children with sickle cell disease covered by Medicaid or the Children’s Health Insurance Program had at least one transcranial Doppler test in 2017.

Even with technological advances, the strides needed to improve life for people living with sickle cell disease on a national scale are not being made.

At Children’s Hospital of Philadelphia (CHOP), where I see patients, we perform transcranial Doppler ultrasounds for stroke-risk screening in all eligible patients. This test is needed yearly between the ages of 2 and 17 for those with the SS type of sickle cell disease, the most common form of the disease in which an individual inherits one sickle gene from each parent. Before the pandemic, more than 90% of our patients were getting these ultrasounds annually. During the pandemic, this only dropped to 85%.

We have managed to do so well by listening to patients and incorporating their voices into care plans. If patients have transportation barriers that make getting to an appointment challenging, then social workers and community health workers help them arrange logistics — and then might combine a clinic visit with a transcranial Doppler scan, saving a second trip. If someone cannot spend that much time at one visit, separate appointments are scheduled, and patients and their families are informed about the importance of these visits and preventive care.

This is coordinated through an electronic health record system that allows the sickle cell care team to better understand which patients need special testing or services. The team then follows up to make sure that patients have received everything they need in a timely fashion.

The pandemic has made providing these services even more challenging — and more essential. Sickle cell disease makes regular assaults on patients’ organs, including the lungs, leaving them even more vulnerable to severe Covid-19 complications. Early in the pandemic, concerns over exposure to the novel virus made it difficult to get some people with sickle cell disease into the clinic, especially those who were already living with pre-pandemic barriers to care. Yet because our hospital had in place the infrastructure — and, more importantly, the culture — to provide wraparound services to these patients, our team was able to maintain their care. We distributed masks and hand sanitizer, arranged direct calls from nurses, hosted virtual town halls, and started vaccine clinics once vaccines were available.

Isn’t this what anyone with a chronic, life-threatening illness not only wants but deserves? The warm touch, the open lines of communication, the extra step — these are the nuances of respectful, compassionate care that not only make a patient feel heard but also lead to better outcomes because health care providers are meeting patients where they are.

To be sure, not every hospital can afford or maintain interdisciplinary teams of doctors, nurses, social workers, financial advisors, psychologists, and community health workers. But it’s possible to establish relationships and networks between well-resourced comprehensive sickle cell centers and hospitals with fewer resources through a system like the University of New Mexico’s Project ECHO, which ensures that vulnerable populations are getting the high-quality health care they deserve.

Decades ago, 60% of newborns with sickle cell disease died before the age of 20. Today, more than 90% are expected to live to age 20 largely due to newborn screening, infection prevention, and disease-modifying therapies. That is commendable progress, but more can be made. Providers who care for people with sickle cell disease need to mark success not only by a month or a year without their patients visiting the emergency department for a pain crisis, but also by their patients living long lives to their fullest potential. I don’t just want to celebrate my patients’ graduation from high school. I want to see them meet their full potential in their careers and personal lives — I want them to return for social visits with me so that I can hear of their accomplishments and possibly meet their children and grandchildren.

How can the medical community do that? Providers must treat sickle cell disease aggressively in early childhood, so when life-saving therapies are finally available their patients have not sustained such extensive organ damage that they are either ineligible to receive them or too sick to truly benefit from them. Providers must also build networks and support systems for their patients so they are not only addressing their patient’s medical needs but also the psychosocial needs that accompany a chronic disease that affects those who are often under-resourced and underserved.

If hospital systems and medical providers are proactive about caring for patients with sickle cell disease and build teams with a culture of communication, empathy, and accountability, the medical community can truly address the disparities this population has faced for far too long. This pandemic will eventually end, but the need for compassionate, coordinated care for vulnerable patients — check-ins as well as checkups — will long outlast the hurdles of Covid-19.

Kim Smith-Whitley is a hematologist at Children’s Hospital of Philadelphia, a professor emeritus at the University of Pennsylvania Perelman School of Medicine, and executive vice president and head of research and development at Global Blood Therapeutics, a San Francisco-based company that focuses on sickle cell disease.