When Monique Hinchcliff, MD, MS, associate professor of medicine (rheumatology, allergy and immunology) and director of the Yale Scleroderma Program, first came to Yale, the only way to care for patients with severe, resistant-to-treatment Raynaud phenomenon, also known as Raynaud's phenomenon, whose fingers were turning black was to send them to the emergency department for hospitalization and treatment.
The system wasn’t ideal. “We wanted to be able to help these patients receive the care they needed in a relaxed, non-urgent setting, in a way that could be planned ahead of time,” Hinchcliff said.
Raynaud is a condition that involves decreased blood flow to certain body parts, usually fingers and toes. Common triggers of the disorder include cold exposure and stress. Raynaud phenomenon—in which the disorder is associated with underlying conditions such as scleroderma or other autoimmune diseases—can be severe, causing tissue damage and even finger loss.
“Patients with scleroderma get tiny vessel disease that can lead to painful ulcers and black fingertips that, if not treated, can cause tremendous disability and, in the most serious cases, result in amputation,” Hinchcliff said. “But the medication epoprostenol, administered intravenously, can prevent or treat these ulcers and gangrene by relaxing blood vessels and improving circulation.”
The treatment is used as a last resort in patients who have autoimmune disease and acute circulatory problems, that have not responded to standard medical therapy, explains Vaidehi Chowdhary, MD, associate professor of medicine and clinical chief (rheumatology, allergy and immunology).
To avoid sending patients to the emergency department, Hinchcliff developed a process to have them treated in the medical intensive care unit. But during the recent pandemic, the beds were needed for COVID patients. So Hinchcliff set out to find a place where epoprostenol could be given intravenously in an outpatient setting.
There were challenges along the way, Hinchcliff recalls. The medication is FDA-approved to treat patients with pulmonary arterial hypertension at significantly higher doses that come with side effects. Hinchcliff had to educate other physicians about the much smaller doses—and lower risk—involved in treating Raynaud phenomenon, which is the standard of care in Europe. In addition, administering epoprostenol requires specially ordered pumps that staff would need training to learn how to use.
After developing an EPIC order, creating a sequence of steps, and conducting educational sessions about the treatment, Hinchcliff’s efforts have paid off. Epoprostenol is now given by the Saint Raphael Hospital Outpatient Infusion Therapy (HOIT) team. “In April, we had our first patient get her infusions at HOIT, which is currently the only place in the Northeast and the second place in the U.S. to administer this medication in an outpatient setting,” Hinchcliff said.
Both Hinchcliff and Chowdhary emphasize the amount of collaboration that went into establishing the process. It was a big ask of the nurses and the pharmacist at HOIT, Chowdhary says, but they rose to the challenge.
Now that the outpatient treatment is in place, Yale researchers are assessing its effectiveness by administering a newly developed patient questionnaire specifically for Raynaud phenomenon and using a handheld digital camera to capture images of patients’ finger nailfold capillaries. Patients also undergo hyperspectral imaging—through a special camera lent to Hinchcliff and her team by Bauer Sumpio, MD, PhD, professor of surgery (vascular) and of radiology and biomedical imaging and of medicine (cardiology)—that shows the amount of oxygen in the fingers.
The goal, Hinchcliff says, is to identify which patients benefit most from epoprostenol and to determine the optimal duration of treatment, including how many times to administer infusions each month and for how many months. Also unanswered, Hinchcliff says, is if, once the treatment is successful, it will need to be repeated after a certain amount of time.
Chowdhary hopes it won’t be long before they roll the treatment out to other infusion centers. “We can improve access for patients who are in remote parts of Connecticut, especially during the winter, when Raynaud becomes a big problem,” she said.
Hinchcliff sees Yale as a model for how epoprostenol can be offered intravenously in an outpatient setting in the US. “I hope that the treatment can be launched throughout the country,” she said.
The Section of Rheumatology, Allergy and Immunology is dedicated to providing care for patients with rheumatic, allergic and immunologic disorders; educating future generations of thought leaders in the field; and conducting research into fundamental questions of autoimmunity and immunology. To learn more about their work, visit Rheumatology, Allergy & Immunology.