UConn Health Helping Adults With Sickle Cell Live Longer

UConn Health’s New England Sickle Cell Institute is improving care for sickle cell patients and helping them beat the odds of the disease.

Dr. Biree Andemariam with patient Chad Bruce, 25, of Middletown, at the New England Sickle Cell Institute at UConn Health. (Chris DeFrancesco/UConn Health File Photo)

Millions worldwide live with this devastating and painful inherited red blood cell disease, including 100,000 Americans and more than 1,000 Connecticut residents. For the majority of those patients, life expectancy is less than age 40.

Individuals with the disease have abnormally hook-shaped red blood cells that impact their blood circulation. When these cells get stuck in smaller blood vessels they form blockages, causing severe pain. These blockages put patients at high risk for stroke, damage to vital organs, and life-threatening blood infections. Sickle cell patients are also prone to profound anemia, an unhealthy level of oxygen-rich red blood cells in their body. Many sickle cell patients experience a lifetime of sudden intermittent pain, daily pain medicine regimens, and monthly blood transfusions.

The New England Sickle Cell Institute at UConn Health helps sickle cell patients better manage their pain and other complications of the disease. It is the first and only dedicated center of its kind in the region, and one of only a very few in the nation.

Turning the Big 4-0

Sickle cell patient Marcus Cornelius Brown at the UConn John Dempsey Hospital. (Frank Barton for UConn)

Marcus Cornelius Brown, of Bolton, Conn. has a lot to celebrate this September.

The husband and father of two is turning 40 years old this month. But as a sickle cell disease patient, he says he was never expected to live this long.

“For so long, they put an age limit on our disease,” says Brown. “When I was young, life expectancy for me was only about 13 or 14 years old. Now reaching 40, 50, or 60 years old has become a big celebration. I look forward to being around for many more years.”

Brown was diagnosed with sickle cell disease at 18 months of age, and shortly after started to experience the disease’s paralyzing pain complications, including severe back and joint pain, along with fatigue.

“Everyday things are difficult with sickle cell disease,” says Brown, recalling the difficult experience of the sudden onset of pain during his childhood. “It was almost like a sixth-sense, I would just stop what I was doing and think uh-oh. It’s the worst, experiencing that type of intense pain.”

As a teenager, he started undergoing blood transfusions to manage his disease, and now continues them monthly at UConn Health’s New England Sickle Cell Institute.

Brown has been treated by UConn Health since 2003. “I like the doctors here that listen,” he says. “They make a big difference. I definitely have more energy.”

“I spend time with my family and don’t worry any more about being in the hospital, thanks to UConn Health,” said Brown. “I haven’t been in the Emergency Department in years. I can call UConn Health in the morning and they get you right in. Plus the nurses know you, which makes the experience, especially when you are in severe pain, better.”

He sticks to his daily regimen of pain medicine, goes for walks, stays in shape, watches what he eats, and incorporates a lot of green vegetables and fruits into his diet.

“It makes a difference with the team of doctors you have caring for you, such as Dr. Biree Andemariam,” Brown says. “I am feeling good, I have been through a lot, and thank God I am still here.”

This summer, the Institute opened a brand new comprehensive outpatient center in the new, state-of-the-art hospital tower at UConn John Dempsey Hospital, offering scheduled and same-day urgent patient visits to improve comprehensive care and also reduce dependency on the emergency room and hospitalizations for pain episodes. In addition, UConn Health recently partnered with the Red Cross to develop a new identification card program to speed sickle cell patient care during emergency situations or for emergency room visits.

60 Bound!
Evelyn Richard, 59, of Hartford is 60 bound and looking forward to her big birthday in January.

“Dr. Biree Andemariam at UConn Health is the reason I will be turning 60 years old,” she says.

Thanks to the care she has received at UConn Health, 59-year-old Evelyn Richard has learned how to avoid deep pain, and no longer has to be hospitalized for sickle cell disease. (Janine Gelineau/UConn Health Photo)

Almost six decades ago when Richard was born with sickle cell disease, doctors and her mom didn’t know it.

“I would scream all the time from my pain, but my Mom didn’t know what was wrong,” said Richard. “Each doctor in Hartford would only tell my parents ‘I don’t know.’”

Eager to find out what was wrong, her parents visited many local hospitals. Finally, when she was three months old in 1957, they went to the formerly city-owned McCook Hospital in Hartford’s North End (which in 1975 later moved to become John Dempsey Hospital). A young doctor there went out of his way to ask if he could examine the baby and take a sample of her blood.

Hours later, he announced that she had sickle cell anemia. “My parents were told I would be in pain for the rest of my life, that I will not live long, and to try to keep me comfortable,” says Richard. “My parents and my family all expected to bury me.”

From birth to 13 years of age, Richard would frequently stay in the hospital for three months at a time. “The pain was often so bad, I would ask my Mom to kill me,” Richard recalls. “The pain feels like 100 people with butcher knives all stabbing you at the same time.” The only relief was from pain medicine injections.

By the age of 18, Richard was tired of her disease and wanted to make herself feel better. She started researching the disease at the local library, and became a vegetarian.

When she surprised everyone by turning 50, she went to see a new, young doctor at UConn John Dempsey Hospital whom she had heard was interested in enhancing sickle cell patient care.

Dr. Andemariam has taught her how to avoid deep pain, and she no longer has to be hospitalized for sickle cell disease. She says Andemariam goes out of her way to make sure sickle cell patients are comfortable and getting the best possible care.

“I am being cared for in the best hospital and by the best doctor in Connecticut,” Richard says. “I wish I’d met Dr. Andemariam earlier in life.

“While I am never without pain,” she adds, “I now live my life through the pain to do I want to do.”

Richard’s advice is to “Eat, drink, and be healthy – and get into something you love.” She believes that when you are doing something you love, such as singing, dancing, or spending time with those you love, your pain disappears.

She also gives back as a volunteer, helping teach young sickle cell patients how to better care for themselves, deal with the pain of the disease, listen to their doctor’s orders, and communicate better with their medical care team.

Richard says she can’t stop raving about the new outpatient unit of the New England Sickle Cell Institute in the new tower at UConn John Dempsey Hospital.

“It’s a beautiful place to be,” she says. “There’s a lot of privacy, the patient care rooms are like being in your own bedroom. They got it all and the medical team is even nicer. As a teaching hospital, UConn will do the best to get you better.”

“Our team’s goal is to help patients better manage their health, reduce their pain symptoms and disease complication risks, and keep them out of the hospital so they can enjoy their lives more,” says Dr. Biree Andemariam, director of the New England Sickle Cell Institute.

Andemariam and her team at UConn Health provide comprehensive care for more than 220 adults with sickle cell disease, and are working to raise greater awareness of the disease with the help of the local chapter of the Sickle Cell Disease Association of America.

“We are committed to bringing increased awareness of this disease and enhancing care for sickle cell patients, while tirelessly conducting research to answer key questions,” says Andemariam. “With better and advanced health management here at UConn Health, the future now looks brighter for patients living with sickle cell disease.”

Baby #3 on the Way

Sickle cell patient Paulina Agyeman receiving care at the New England Sickle Cell Institute. Agyeman is expecting her third child this fall. (Ethan Giorgetti for UConn)

Paulina Agyeman, 35, of East Hartford is a mother of two and is getting ready to welcome her third child this November.

Born with sickle cell disease in Ghana, Agyeman had a difficult childhood before coming to the U.S. when she was 11 years old.

“There were no standard newborn screenings for sickle cell in Ghana like in the United States,” said Agyeman. “I used to cry a lot from pain, but no one could figure out why I was crying.”

At about 10 months of age she was diagnosed by a visiting doctor from the U.S., but there were no medications in her country to treat her pain. She would often spend two weeks at a time in bed unable to walk or eat because of the pain.

Agyeman says the pain is worse than being in labor. “The crisis pain from sickle cell feels like you are about to die,” she says. “It’s a very painful experience.”

As she grew up, doctors told her parents she would never be able to have kids. But nine years ago, she was surprised to find out that she could have a family. She beat the odds, and now has a 9-year-old daughter and a 6-year-old son. Both times she had a normal pregnancy and delivery.

Most women with sickle cell are considered very high-risk for pregnancy, as a growing baby in the womb can lower a mother’s blood supply level. For sickle cell patients this can be dangerous, as they usually have half the normal blood supply level. These moms can suffer increases in pain episodes and complications during the pregnancy that can affect their ability to carry the baby to term. Both mother and baby are potentially at risk.

Two years ago Agyeman learned that UConn Health specializes in sickle cell disease care and has a dedicated center.

Now that she is pregnant, she gets her blood levels checked often at UConn Health and every month receives healthy blood via transfusions.

“I am very blessed,” she says, noting that she hasn’t often had to be hospitalized, just once or twice a year when in a pain crisis. “Now I can spend more time with my family and be the best mother and wife possible.”

September is National Sickle Cell Awareness Month.

To learn more about the New England Sickle Cell Institute at UConn Health, visit here or call: 860-679-2100.

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