Pain affects all of us at some point. If we’re lucky, we may escape it for many years. But that’s not the case for everyone – it certainly hasn’t been for 17-year-old Jaire Rodriquez. He has sickle cell disease, and excruciating pain has been present in his life since infancy.
Sickle cell disease is an inherited blood disorder in which the hemoglobin takes on a distorted sickle shape, making it inflexible and more likely to cause blockages that stop or slow the flow of blood. A blockage will prevent oxygen from getting to tissues, causing a sudden, severe attack known as a pain crisis.
In North Carolina, all newborns are screened for sickle cell disease. And there is now a program in place in which community workers throughout the state get notifications of the results of newborn screenings, so that parents who have children with sickle cell disease can start receiving active support right away.
When Rodriquez was born, however, things were different. He wasn’t diagnosed until he was two months old, and it was between the ages of two and four months that he started having his first pain crises – although they weren’t confirmed until a year or two later.
The Diagnosis and Treatment
“It was very daunting,” said Matisa Farley, his mother. “At first we thought it was colic. There was no way we could hold him that could make him comfortable. Even once he was diagnosed, we weren’t sure what was going on. We were told the fetal hemoglobin should’ve stopped him from sickling, but it didn’t. He was still in pain, and we didn’t know why.”
Rodriquez’s early onset of pain was rare. Young babies tend to be protected from sickle cells forming because they still have fetal hemoglobin in their blood. “At birth, babies still have fetal hemoglobin from when they were in utero. Over the course of about six months, the fetal hemoglobin naturally leaves the system and sickle cell hemoglobin takes over,” said Ginna Priola, MD, pediatric hematologist/oncologist with Mission Children’s Hospital.
Because fetal hemoglobin has known benefits for children with sickle cell disease, another common treatment is a drug known as hydroxyurea, which helps the body make fetal hemoglobin. Rodriquez said he takes hydroxyurea, which has been very successful, according to Farley. “We have had very helpful treatment options that we are blessed to have available to us,” she said. “Jaire had a lot of pain crises when he was younger, but once he got acclimated to the hydroxyurea, they were fewer and farther between. He tolerates the treatment well.”
Throughout his childhood, Rodriquez stood out in ways he would’ve preferred not to. At one time, he had a port and its outline was noticeable beneath his shirt. As a result, he often had to answer the questions of his curious peers. He also had to take precautions that other children did not.
“I guess one of the main problems of my disease has been the things I can’t do or the things I have to watch out for,” said Rodriquez. “I can’t be involved in a lot of contact activities, so I can’t play most sports.”
Overall, Rodriquez has come a long way. He’s learned a lot of lessons about his disease, and – with the help of his family and care providers – he’s cleared many hurdles. Now, he’s confident, aspirational and more apt to stand out for his bright outlook and well-spoken manner than he is for his challenges with sickle cell disease. A rising high school senior, Rodriquez has ambitions to become a lawyer and video game designer.
Farley said sickle cell disease can be emotionally painful for parents as well, but it’s important to be there for your child – to “pray, participate and advocate.” She said this has been easy to do working with Mission. “They incorporate parents into the process. They are all about learning and awareness when it comes to this disease, but they also support families and make sure we’re comfortable,” said Farley.
According to Rodriquez, support has been the No. 1 factor for him: “Knowing that God’s going to get me through it, that my family is always there for me and that my doctors are always ready to help me out means a lot. If I were the only one trying to cope with this, I’d be much worse off, but I’m not; I have so much support.”