… But Her Mother Is Uptight and Has Begun A Sickle Cell Advocacy
By Amy Schwabe, Milwaukee Journal Sentinel
When you meet 2-year-old Evelyn Islam, the first thing you notice is her grin. And her friendliness. And her curiosity.
You can’t help but smile as she mischievously steals phones to play with. You can’t help but be drawn into a conversation as she explains her surroundings to you in earnest toddler-speak. And you can’t help but play right along with her as she shows you her collection of beads from Beads of Courage, an organization that supports children who have serious illnesses.
Although Evelyn’s sickle cell anemia is a serious chronic medical condition, the fact that it isn’t obvious from the outside makes it easy to see Evelyn for the person she is. It’s easy for her to be defined by something other than her illness.
Evelyn’s mom, Melissa Goins, explains that Evelyn’s ‘body only makes sickle hemoglobin and no normal hemoglobin. When normal blood breaks down, it moves easily through the veins. But as Evelyn’s blood breaks down, it looks like sickles, sharp-edged things that clog up in different parts of the body. They’re sharp, and that sharpness can create damage as they flow through the body.’
Even though the shape of her blood cells isn’t obvious from the outside while this happy-go-lucky girl is playing with her family, for her mom, Evelyn’s sickle cell anemia is always at the front of her mind, even if she would prefer it wasn’t. Goins is Evelyn’s advocate, her caretaker, her mom. She doesn’t have the luxury to forget about the extra care that Evelyn’s illness requires.
For Goins, one of the most difficult parts of raising a child with sickle cell anemia is playing the waiting game. Because the symptoms of sickle cell are varied and happen at different points in people’s lives, Goins doesn’t know which symptoms will strike or when.
People with sickle cell anemia are prone to several problems as the blood cells clog different parts of the body, including strokes, organ damage and blindness. Many of those complications become more likely as the person gets older and the symptoms persist. For children, the biggest concerns are pain and a higher risk of infection as a result of a compromised immune system as the spleen becomes enlarged.
Evelyn’s first spleen crisis happened when she was 7 months old, causing her blood levels to drop dramatically, which resulted in an emergency blood transfusion.
Goins explained that the treatment plan at that point was chronic blood transfusions, which Evelyn had until she turned 2 years old.
She said, ‘Evelyn had 20 blood transfusions between the time she was 7 months and when she turned 2.’
Her spleen became enlarged because the sickle cells built up in it, so doctors gave her transfusions. The healthy blood cells flowed through her body the way they were supposed to. At age 2, the transfusions were stopped in hopes that her spleen wouldn’t become enlarged again, but it did because of the buildup of sickle cells. After two more blood transfusions, she finally had her spleen removed in November 2017.
Now Evelyn has been blood transfusion-free for several months, the longest she’s ever had her own blood instead of donated blood.
‘The benefit of living with donated blood is that we haven’t had to worry about what her own blood was doing to her,’ said Goins. ‘I wouldn’t say I’m a nervous wreck, but I’m kind of like, ‘OK, when will this happen?’ Because a symptom could happen at any time.’
Another thing that could happen at any time is pain. The Mayo Clinic says that pain in sickle cell patients is common as the sickle-shaped blood cells block blood flow to the chest, abdomen and joints.
Goins said, ‘In our situation, it makes me uncomfortable because she hasn’t had any pain crises, but it’s inevitable that it will happen.’