UConn Health Stands at the Forefront of Comprehensive Sickle Cell Treatment in the U.S.

Written by Steven Blackburn

UConn Health’s New England Sickle Cell Institute (NESCI) was already providing more comprehensive care for sickle cell patients than most U.S. clinics when the Farmington campus recently announced the expansion into a new, fully refurbished space.

“This new space gives us a permanent home to continue providing and even improving the individualized level of care we always have for even more of our sickle cell patients, particularly those who arrive with severe bodily pain,” says Biree Andemariam, M.D., who founded NESCI in 2009. “It’s another way for us to fast-track their management of pain for same-day urgent care rather than going through the Emergency Department.”

Dr. Andemariam and her team have already gone above and beyond to ensure their sickle cell patients admitted into UConn Health’s Emergency Department (ED) receive specialized, expedited treatment by linking their information and pain management plans to UConn Health’s electronic records.

“When our patients come to the emergency room during an episode or crisis after hours, staff know exactly who they are, their treatment of choice, the required dose and what steps need to be taken,” explains Genice T. Nelson, DNP, MSN, APRN, ANP-BC, an early member of Dr. Andemariam’s team who helped establish NESCI. “It can be a disheartening event for our patients if the ED doesn’t know what to do immediately, and we’ve helped address that.”

Providing Well-Rounded, Comprehensive Care Few States Offer

This transparency between UConn Health’s NESCI and ED is just one integral part of the innovative program by Dr. Andemariam and Dr. Nelson. Another is their newest addition, Oladipo Cole, M.D. Dr. Cole recently finished his fellowship program at Washington University School of Medicine and residency at Florida Atlantic University’s Charles E. Schmidt College of Medicine. 

“Not a lot of programs offer what NESCI does,” Dr. Cole says. To him, UConn Health is ahead of the curve with its treatment of sickle cell disease. “Many clinics are only able to provide patients with pain management and don’t have such a well-thought-out or well-rounded comprehensive program focused on the overall care of sickle cell patients. The team here is second to none, and that’s paramount to this program.”

UConn Health’s Courage to Pioneer Sickle Cell Treatment

Before NESCI began trailblazing sickle cell treatment, it was common for patients across the U.S. to not receive constant care. This is what prompted Dr. Andemariam to change her area of focus from blood cancer to sickle cell disease. “Every time I made the rounds at the hospital, I kept seeing the same young people with sickle cell disease, who were in and out of the hospital in pain,” she remembers. “This is when I realized that I could make a difference in their lives and give them a voice.”

As she was treating and studying sickle cell disease at UConn Health, she identified five critical areas for improvement: build a transition program from Connecticut Children’s, develop a fast-track system of care in the ED, create a day hospital for managing acute complications, provide a clear pathway of care for pregnant sickle cell patients, and perform specialized blood transfusions. 

Over time, UConn Health recognized Dr. Andemariam’s contributions and provided her with the means to expand officially, an initiative that eventually led to the NESCI. 

“NESCI wouldn’t have been possible without UConn Health’s support,” says Dr. Andemariam. “It took courage from our executive leadership to do the right thing upfront without necessarily knowing if there was going to be any financial benefit.”

That leadership team includes Dr. Pramod K. Srivastava, director of the Neag Comprehensive Cancer Center; CEO Dr. Andrew Agwunobi; and Chief Nursing Officer Caryl Ryan.

Child-to-Adult Program Achieves 99% Success Rate 

In addition to collaborating closely with UConn Health’s ED, another major component of NESCI includes its innovative transition program in partnership with Connecticut Children’s. Since babies are born with this genetic disease, hospitals are required by law to screen for sickle cell at birth. The program places a dozen or so Connecticut newborns diagnosed with sickle cell disease every year into Connecticut Children’s pediatric sickle cell center. Since sickle cell is a lifelong disorder, those patients will eventually grow out of Connecticut Children’s once they turn 21. 

NESCI developed a transition program for future patients that begins as early as 12 years old to familiarize them with UConn Health and the campus. “Our long-standing social worker, Teresa Works, is instrumental in ensuring that all children at Connecticut Children’s with sickle cell disease make their way to UConn Health to continue their care as adults,” Dr. Andemariam says. “She has really been the backbone of the transition process for the last 13 years.”

The NESCI team also confirms insurance and transportation. UConn Health will provide funding for transportation if none is available. Today, NECSI currently boasts that 99% of young adults graduating from care at Connecticut Children’s have successfully transitioned over to NESCI at UConn Health.

“Until we developed this adult program at UConn Health, Connecticut Children’s patients didn’t have anywhere to go. It’s not just a Connecticut problem, either,” says Dr. Andemariam. “Very few hematologists in the nation are comfortable or willing to treat sickle cell disease.”

During the transition process, Dr. Nelson, Dr. Andemariam and their team meet with prospective patients virtually to get to know them. UConn Health also hosts on-campus tours in Farmington so incoming patients can familiarize themselves with their new place of care. 

“We always encourage families to come to these tours, since it’s a time of high anxiety for everyone,” says Dr. Nelson. “Up until then, many of them had been with a team of providers for 21 years; now they are going to be taken to a completely different area with a different team. It’s important that families can rest assured that their loved ones will receive the same care and love they had at Connecticut Children’s. It’s a naturally difficult process, but we are trying to make it as seamless as possible.” 

Working Tirelessly to Provide a New Medication

Patients also travel to UConn Health from New York, Rhode Island and Massachusetts. Many patients come to Farmington for checkups every six months for heart ultrasounds, eye examinations, lung and kidney evaluations, and lab tests. Some patients visit as frequently as once per month, depending on their level of pain or need for blood transfusions and medications. 

“There are many parts of the body that serve as markers or indicators of sickle cell,” explains Dr. Nelson. “Sickle cell disease is very painful. There are some who live with chronic pain every day.” 

The team members at UConn Health can proudly say they provide every FDA-approved treatment for sickle cell disease. “Not every person with sickle cell has a physician who is knowledgeable enough or has the clinical resources to provide all the therapies as we do,” shares Dr. Andemariam.

Conducting Rare Clinical Trials That Patients Trust

Even with a growing number of new treatments for sickle cell disease over the last few years, the condition is still wrought with many complications and reduced lifespan. The average life expectancy remains only around 42 years of age. In order to address this, NESCI conducts clinical trials and research to streamline medical innovations and expedite future releases of newer medications and therapies.

“There are a lot of things we still don’t know about sickle cell disease,” says Dr. Cole. “But the reason we know as much as we do is because we collaborate with basic scientists and pharmaceutical companies to conduct groundbreaking research and clinical trials.” 

Very few clinics are even capable of conducting clinical trials. As a rare condition, a significant number of patients is required. UConn Health is one of the few centers that can, since it treats more than 300 patients with sickle cell disease.

Of course, not every patient is willing to participate in trials, a decision that Dr. Andemariam’s team always supports. But most of their patients are usually excited whenever an opportunity arises. “I think many of our patients volunteer because they trust us,” adds Dr. Cole. “They know we have their best interests in mind because we are always honest and transparent.”

A Vision of State-of-the-Art Treatments and Therapies

As clinical trials and research help propel UConn Health into the future, Dr. Andemariam is optimistic about where UConn Health is headed because of her newest hire, Dr. Cole. He is committed to bringing the latest innovations to NESCI, such as gene therapy and bone marrow transplants. 

“He is the future of our program,” says Dr. Andemariam. “When you’ve been doing it for as long as Dr. Nelson and I have, you need a fresh perspective like his. With Dr. Cole’s pedigree, he could have gone anywhere. And that says a lot about our program and its future.”

Steven Blackburn is a freelance writer with more than 10 years of journalism experience in various fields, including U.S. education and Connecticut community interest stories. He lives in Winsted.