You feel as if your bones are breaking, your blood is “stuck” and a rusty blade is sawing your insides.

This is how patients often describe the pain associated with sickle cell disease. Pain crises are hallmarks of this inherited blood disorder in which red blood cells, normally round, become crescent-shaped and block small blood vessels. Less blood flows to tissues and organs — causing excruciating pain that can last a week or more, and leading to permanent damage, increased infection and possible organ failure.

In 2015, Johns Hopkins Aramco Healthcare (JHAH) was seeing up to 150 adult sickle cell cases each month, often in its emergency department. These high patient volumes stem, in part, from the 25 percent prevalence of the genetic trait for sickle cell disease found in the Eastern Province of Saudi Arabia, the site of JHAH’s main hospital. By comparison, 12 percent of the U.S. population carries the trait.

Due to the high occurrence of the disease and the magnitude of resources required to treat it, JHAH requested the support of Johns Hopkins Medicine (JHM) to develop a comprehensive sickle cell program for its adult population.

“We didn’t have a standard practice of care for patients with sickle cell disease at JHAH, which led to limited access to specialists, poor communication and inadequate pain management during admission and at home,” says Kawthar Al Hussain, JHAH clinical nurse specialist in pain management. “As a result, patients relied on the emergency department, which often meant higher readmission rates, lower patient satisfaction and decreased patient quality of life.”

In February 2016, Sophie Lanzkron, director of JHM’s Sickle Cell Center for Adults, led a clinical visit to evaluate sickle cell care at JHAH. Together, JHAH and JHM clinicians focused on assembling the most effective care team, opening a dedicated infusion center and tailoring treatment plans.

Right Team

The challenge, as JHAH and JHM determined, was the need for more providers at JHAH to handle the high volume of patients. On JHM’s recommendation, JHAH created a care team that includes a second adult hematologist, a hospital medicine consultant, a nurse practitioner and two nurses with pain management and palliative care expertise, and a social worker who coordinates with psychiatric services.

The partners also provided opportunities for training and collaboration through observerships at JHM. “They came to Baltimore to see how we do things here — not just the physicians, but also the nurses, which was very helpful,” says Lanzkron.

Right Location

Nearly two-thirds of adults with sickle cell disease will experience pain every day, and the volume and complexity of their cases strained the busy emergency department at JHAH’s Dhahran Health Center. This led to JHAH and JHM working together to open an infusion clinic in April 2017, using Johns Hopkins’ sickle cell center as a model. The clinic not only treats patients with acute pain — it also provides routine outpatient care, including chronic pain management and social work support.

Today, JHAH provides more centralized sickle cell care, performed by clinicians who understand both the disease and the needs of their individual patients.

“No one protocol will fit all patients. We work with each patient to create a treatment plan to manage everyday pain, as well as crises,” says Zainab Mahr, who coordinates JHAH’s sickle cell program. The treatment plans include disease-modifying therapy and pain treatment during a crisis.

“One of the barriers to sickle cell care is that patients are referred to a different expert for each part of their treatment, and the patient’s care winds up fragmented,” says Dr. C. Patrick Carroll, director of psychiatric services for JHM’s Sickle Cell Center for Adults. To combat this, the sickle cell team collaborates with the emergency department so every site of care can follow a patient’s plan.

Right Outcome

The results have been dramatic. Between 2015 and 2017, both emergency department visits and hospital admissions dropped by 85 percent. JHAH directed the majority of patients to the new infusion clinic, where visits lasted less than 12 hours — compared with an average five-day hospital stay in 2015 — and where the average time to receive pain medication decreased by nearly half.

“We have begun to see significant improvement in pain management practices, lower rates of readmission, reduced ER visits and substantially increased patient satisfaction,” says JHAH’s Al Hussain. “We’re seeing this success because we’re implementing patient-specific treatment plans, standardizing and improving the care we provide, and working at the patient level to decrease the need for acute care.”

Mahr adds, “Observing the trends over the past two years has also provided us with a wealth of data that we can use to understand sickle cell better, conduct breakthrough research and create a model of patient care for the entire Eastern Province, where as many as one in five hospital admissions relates to this disease.”

This article originally appeared in Headlines, the newsletter about Johns Hopkins Aramco Healthcare.