The Obando girls never know when or where the pain will strike, only that sooner or later, it will. This is the life that Haylee and Helen Obando — ages 15 and 13 — have known since before they can remember.
The girls, who live in Lawrence, have sickle cell disease, which causes red blood cells to deform into a sickle shape and stick to vessel walls. When that clumping happens, a sudden blockage can occur, depriving nearby tissue of oxygen and causing sudden bouts of pain that last days.
Haylee says it feels like heavy books piled on her. Helen feels needles stabbing.
Among childhood pain syndromes, sickle cell disease stands out as an especially bedeviling adversary, with intermittent attacks starting in infancy. The pain isn't constantly present, just constantly a threat. (For some people with sickle cell, the pain does become chronic.)
About 100,000 Americans have sickle cell disease, an inherited condition that strikes mostly African-Americans, but also affects people with Hispanic, southern European, South Asian, and Middle Eastern origins. There is no treatment for children.
"The really cruel thing about this disease," said Dr. Matthew M. Heeney, director of the Dana-Farber/Boston Children's Sickle Cell Program, "is that . . . in between crises, you're essentially 100 percent able. You have one of these crises, you're 100 percent disabled."
For families, that means a day-by-day challenge, as Haylee and Helen's mother, Sheila Cintron, can attest. Even taking turns with her husband, Cintron said she has lost more than one job because of her unpredictable absences.
Pain crises can be triggered by exposure to the cold, dehydration, or stress. Or they can strike out of the blue. "The pain itself won't let them move, at all," Cintron said. "And they're crying. You're trying to comfort them, waking up at 2, 3 in the morning to give medication, rubbing them. It's hard to say when it's going to hit."
To ease their suffering, Cintron first tries hot packs. If that doesn't work, then ibuprofen; next, acetaminophen plus ibuprofen. As a last resort, she will give an opioid. The girls hate the way opioids make them feel — dizzy, nauseous, sleepy. When the pain persists after two or three opioid pills, it's time for the hospital, where the drugs are administered intravenously.
Haylee and Helen say they don't live in dread of their next pain crisis. "When I have pain, I wish I didn't have it," Helen said. "Other than that, I don't think about it."
Felice J. Freyer can be reached at firstname.lastname@example.org.