Personalized Sickle Cell Plans | Corewell Health

More than a year ago, Kathleen Jarrett, MDmade a promise to a patient.

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

Now Dr. Jarrett and others Corewell Health medical providers sit on a sickle cell action committee to research and implement new practices to achieve this.

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

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

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

The sickle cells die early, leading to a chronic shortage of red blood cells and, consequently, anemia or low hemoglobin. They can also clog small blood vessels, causing pain and other serious complications, such as infections and strokes.

National health problem

The Centers for Disease Control and Prevention estimate that sickle cell disease affects about 100,000 Americans and occurs in about one in every 365 black or African American births.

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

Dr. Jarrett said sickle cell disease management has often been overlooked nationally, and in 2018 Congress took action to help change that with the Research, Surveillance, Prevention, and Treatment Act. treatment of sickle cell disease and other inherited blood disorders.

“We know that as a nation we were behind in managing these patients, and this law was intended to close that gap,” she said.

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

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

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

“When things get stuck like that, it causes pain because they can’t get enough oxygen,” she said. “It’s a chronic problem for them.”

Often it is everyday things that cause sickle cell crisis, such as weather, seasonal changes, stress, alcohol, caffeine, lack of sleep, lack of water, and menstruation.

Individual 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 this to a new doctor every time they’re in the emergency room or hospitalized can be frustrating for patients, she said.

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

Thanks to his 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 record under the problem list and includes a section for pharmacy, emergency department, and inpatient management if the patient is admitted for care.

Plans are new in the past two months, but Dr Jarrett said eventually more patients will receive one as they are treated or admitted to hospital.

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

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

This includes painkillers, dosages and more.

Better pain management

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

This allows patients to break through the sickle cell crisis more quickly, then wean off the narcotics and return to their regular medications as quickly as possible, she said.

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

Ketamine, a drug approved by the United States 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 the narcotics so that they feel like increasing the doses doesn’t work,” she said. “The ketamine drip is an opportunity to use the reset button to get these patients narcotic sensitive again.”

The committee has also partnered with the Department of Hematology at Helen DeVos Children’s Hospital to facilitate the transition of sickle cell patients from pediatrics to adulthood, Dr. Jarrett said.

Having procedures like these in place is already making a difference for patients, Dr. Jarrett said.

“I think we’ve gotten off to a good start, but I think there’s room for continuous improvement,” she said.