Personalized sickle cell disease plans | Corewell Health

Over a year ago, Kathleen Jarrett, MD, made a promise to a patient.

That promise: she would investigate ways to improve care for adults with sickle cell disease.

Now, Dr. Jarrett and other Corewell Health medical providers serve on a sickle cell action committee to research and implement new practices to do just that.

“I wanted to keep a promise,” said Dr. Jarrett, an internal medicine hospitalist with Corewell Health’s Spectrum Health Medical Group in West Michigan.

Sickle cell disease is an inherited red blood cell disorder which causes healthy, round blood cells that easily carry oxygen throughout the body to turn hard and sticky.

When that happens, they look like a crescent moon-shaped farm tool called a “sickle.”

The sickle cells die early, causing a chronic shortage of red blood cells, and therefore, anemia or low hemoglobin level. They also can clog small blood vessels, causing pain and other serious complications, such as infection and stroke.

National health issue

The Centers for Disease Control and Prevention estimates that sickle cell disease affects about 100,000 Americans, and it occurs among about one out of every 365 black or African-American births.

One tool guiding the action committee is a report, “Addressing Sickle Cell Disease: A Strategic Plan and Blueprint for Action,” released in 2020 by the National Academies of Sciences, Engineering, and Medicine.

Dr. Jarrett said that sickle cell disease management has often been overlooked nationally, and in 2018, Congress took action to help change that with the The Sickle Cell Disease and Other Heritable Blood Disorders Research, Surveillance, Prevention and Treatment Act.

“We know that as a nation, we were behind the curve in managing these patients, and that act was meant to address that gap,” she said.

The goal, she said, is to meet the highest standard of care, and exceed it.

While the severity of sickle cell disease varies greatly in patients, most have one thing in common: pain, Dr. Jarrett said.

Sickle cell crisis occurs when the sickle cells become stuck in a blood vessel and block blood flow to an area, causing pain.

“When things are blocked off like that, it causes pain because they cannot get enough oxygen,” she said. “This is a chronic issue for them.”

Often times, it’s everyday things that cause sickle cell crisis, such as weather, seasonal shifts, stress, alcohol, caffeine, lack of sleep, not drinking enough water and menstruation.

Individualized care plans

Dr. Jarrett said treating patients with sickle cell disease presents challenges because each patient’s case is so unique, and many patients have learned over the years what works for them and what doesn’t.

Communicating that to a new doctor every time they are in the emergency room or hospitalized can be frustrating for patients, she said.

That’s where the committee, or expert improvement team, comes in. Their work has also involved the commitment of many people, including quality improvement administrators, pharmacists, physicians and more, Dr. Jarrett said.

As a result of its work, each patient now has an individual treatment plan called a sickle cell action plan, Dr. Jarrett said.

The plan is located in the patient’s medical records under the problem list and includes a section for the pharmacy, emergency department and inpatient management if the patient is admitted for care.

The plans are new within the last two months, but Dr. Jarrett said that eventually more patients will receive one as they are treated or admitted to the hospital.

The committee also hopes to expand the options with the plan so that a patient’s primary care doctor can also access and contribute to it.

“The plan stays with the patient so that a provider knows what to do each time,” she said.

That includes pain medications, dosages and more.

Better pain management

Included in that effort, she said, is the use of patient-controlled analgesia (PCA) for hospitalized patients with sickle cell disease. This a safe pain management option that lets the patient decide when to administer more pain medication, with controlled limits in place, Dr. Jarrett said.

It allows patients to break the sickle cell crisis more quickly, then wean off the narcotics and get back on their regular medications as quickly as possible, she said.

Another tool the committee has recommended to help hospitalized sickle cell patients with pain control is a ketamine infusion therapy, she said.

Ketamine, a medication approved by the U.S. Food and Drug Administration for anesthesia, can also treat acute pain in some cases.

“The idea is that sickle cell patients can get used to narcotics so that it feels like increasing doses are not working,” she said. “The ketamine drip is an opportunity to use the reset button so that those patients become sensitive to the narcotics again.”

The committee also partnered with Helen DeVos Children’s Hospital hematology department to help ease the transition for sickle cell patients from pediatrics to the adult world, Dr. Jarrett said.

Having set procedures such as these is already making a difference for patients, Dr. Jarrett said.

“I think we’re off to a great start, but I do think there is room for continued improvement,” she said.