For Ronald Johnson and Dr. Gwendolyn Poles, the pain of sickle cell disease never really goes away. In fact, they’ve dealt with it for as long as either can remember.
“I have very few good days without pain,” said Johnson, of Harrisburg. “On my worst days, I can’t move. I thank God every morning for being in the land of the living.”
For Dr. Poles, most days range somewhere between “mild pain” to “10-out-of-10, emergency room pain.” Days without pain for the Lower Paxton Township resident are few and far between.
“I’ve been sick as long as I can remember,” said Poles. “I was diagnosed at 18 months old. My mother noticed that I had swelling of the feet.”
Poles, though, is not just a sickle cell sufferer—she’s also an activist. She is president of the South Central PA Sickle Cell Council, a Susquehanna Township-based organization that helps and supports those who live with this painful inherited blood disorder.
The council offers clients and their families disease testing and consultation, psychosocial assessment, social work case management, individual counseling, advocacy and educational resources. Support groups are available for youth, adults and families.
Sickle cell disease is an inherited blood disorder that affects red blood cells. Patients with the disease have red blood cells comprised mostly of the abnormal hemoglobin “S.” At times, some cells convert into a sickle shape that can cause great pain when passing through small blood vessels. Patients often experience chronic or acute pain of crippling proportions.
“I’ve heard some people say it’s like having a baby,” Johnson noted of the disease’s highest pain levels.
Sickle cell is relatively common within numerous ethnic groups, particularly those of African and Middle Eastern origin. In the United States, 1 to 3 million African Americans carry the trait.
“It’s important for communities of color to know their sickle cell status,” said Debra Bizzard, case manager and social worker for the South Central PA Sickle Cell Council. “Sometimes, two carriers find out their status only when they have a child with full-blown sickle cell.”
Joseph Robinson, Jr., the council’s executive director, doesn’t have the disease, but he knows well how it affects the lives of patients and their families.
“The marker for sickle cell is pain,” Robinson said. “Many have to go to the emergency room and wind up staying in the hospital for six to eight days. A family who has to endure this often misses school or work, as well.”
As the council’s case manager/social worker, Bizzard provides support services for 148 clients.
“They call sickle cell ‘the invisible disease,’” she said. “There is no general cure for it at all. We just try to manage the illness.”
Upon intake, potential clients are given a comprehensive psychosocial assessment. Depression and anxiety are common for patients who try managing their lives around the disease.
“I’m totally disabled,” Johnson said. “I have no control over this disease. I never know when it’s going to hit.”
Median life expectancy for people with sickle cell is only 45 years, which tends to discourage some from pursuing higher education or careers.
“Some young people figure they’ve already lived half of their lives,” Bizzard said.
Poles and Johnson, however, have well exceeded this statistic.
Dr. Poles, 65, retired in 2013 as a physician with PinnacleHealth. She led a long and successful medical career in the area after earning a doctorate from the Philadelphia College of Osteopathic Medicine.
Johnson, 55, said that he tried holding jobs in the past but was derailed by a sickle cell crisis that required lengthy hospitalization. He tried to hide his illness from others while working because of the stigma it would entail, he said.
“Many (patients) have to go to the emergency room for morphine and stay for three to eight days,” he said. “That makes it difficult to hold a job. A boss may not be down with it. Others may not understand what’s going on because the person looks healthy to them.”
Bizzard added that sometimes hospital staff members don’t understand patients’ extreme pain due to a lack of knowledge about the disease. Sickle cell patients also tend to request high levels of narcotics to combat the pain, which can create even more stigma.
“We educate everyone about sickle cell disease: lawyers, physicians, nursing classes at Harrisburg Area Community College,” Robinson said. “Our function is to spread awareness and help anyway we can psychologically.”
The South Central PA Sickle Cell Council is located at 2000 Linglestown Rd., Harrisburg. For more information, call 717-234-3358 or visit www.scpascc.org. Office hours are 8 a.m. to 5 p.m. weekdays.