World Sickle Cell Day: What you may not know about the painful disorder by Angela Mulholland

Little progress has been made in finding a cure for sickle cell disease, a rare but debilitating blood disorder that typically shortens the life of patients by more than 30 years. But doctors say there are new treatments available that are offering some relief from the painful condition.
Sickle cell disease is an inherited disease in which the hemoglobin in one’s blood clumps together, causing red blood cells to become abnormally shaped. While healthy blood cells are normally round, in someone with this disease, they are C-shaped, like a sickle, and don’t flow properly.
Sickle cells die quickly, which causes the constant shortage of red blood cells known as anemia. While that aspect of the disease leaves patients feeling exhausted, for many, the most difficult part is the episodes of debilitating discomfort.
Sickle cells are sticky and block blood flow in vessels, which not only puts patients at risk of stroke, it causes intense pain, says Dr. Melanie Kirby-Allen, a hematologist from Sick Kids Hospital in Toronto.
“The hallmark of the disease is what are called painful vaso-occlusive crises, where patients have significant bone pain requiring hospitalization and a lot of pain medications,” she told CTV’s Canada AM.
Kirby-Allen says patients are trained how to manage the pain at home, but at times when it becomes so severe, they have to get intravenous pain relief in hospitals.
Ayanna Stanislaus, a high schooler from Ajax, Ont., says because sickle cell disease is life-long, patients like her just get used to the episodes.
“Throughout your life, you will have periods of time with pain and there’s nothing you can do about it,” she said. “So I find myself in that situation where sometimes I’m hospitalized due to fevers or pain in the bones. But you can’t do anything about it.”
Stanislaus says what she does look forward to every year is heading out to
Camp Jumoke , a volunteer-run camp just for kids with sickle cell disease. The camp is just like any other, with activities such as canoeing, arts and crafts camp songs, but Stanislaus says the best part is getting to meet other kids with sickle cell.
“You feel like you’re not alone. You feel like you’re not the only person with sickle cell disease,” she said.
The camp runs for two weeks every summer, with little or no expense to their families, and offers kids a chance to experience something they might never otherwise try. Stanislaus says many kids with sickle cell don’t go to summer camp because they know there won’t be people there who understand their condition.
“But at Camp Jumoke, there’s a team of doctors and nurses who understand and know how to treat it,” she said.
Sickle cell disease is a relatively rare condition, affecting approximately 5,000 Canadians. As a rare disease, there hasn’t been much funding into research for a cure.
But Kirby-Allen says there have been improvements in treatments. As recently as 30 years ago, the average lifespan for people with sickle cell disease was only 14 years. Today, life expectancy can reach 50 and beyond.
“We’ve made strides in the last 20 years, particularly with a drug called hydroxyurea,” she says.
The medication increases production of fetal hemoglobin, which reduces the chance that red blood cells will morph into sickles. While it doesn’t work for everyone, in some, it can reduce hospitalizations.
“By taking this medication on a daily basis, we can ameliorate these episodes,” she said, “and in some patients, they simply stop having these painful episodes.”

Culled from

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