Andrea Reed Elmore and Doran Reed are siblings who have been fighting against sickle cell disease — an inherited, painful and sometimes crippling blood disorder — since they were infants. Health care providers at UCLA have been with them throughout their journey and continue to deliver care to them as adults.
It has been a long and difficult battle, but the brother and sister have supported each other throughout. “Their relationship is fantastic,” said Erica Martinez, a nurse practitioner at the Bowyer Oncology Center at UCLA. “They can advocate for each other and support each other because they understand what the other is going through. They can be each other’s backbone.”
Sickle cell disease is marked by defective hemoglobin — the protein in red blood cells that carries oxygen throughout the body. Cells with sickle hemoglobin are stiff, sticky and crescent-shaped. They often clump together, blocking small blood vessels. These snags cause agonizing episodes known as pain crises, centered on the limbs, back or chest.
Mary E. Sehl, MD, PhD, associate clinical professor at the David Geffen School of Medicine at UCLA and an oncologist at the Bowyer Oncology Center, has been an important member of the siblings’ care team. Treating the disease is complex, she explained.
“Comprehensive care for persons living with sickle cell disease involves careful attention to detail in addressing all of the organ systems that may be affected — including brain vasculature, eyes, heart, lungs, kidneys, liver, spleen, bone marrow and dental and bone health,” Dr. Sehl said.
Different medications, same resilience
Though both siblings have sickle cell disease, their physicians are taking different approaches to their medications. Reed has been on hydroxyurea, a medication that causes red blood cells to become bigger, rounder, more flexible and less likely to take on a sickle form. His sister is being treated with injections of a different medication, crizanlizumab, to reduce pain crises.
“We’re excited to hear about all the new medications that are coming out, but they don’t work for everyone,” said Reed Elmore. “I’ve tried a lot of different medications,” but because of the progressive nature of the disease, many lose their effectiveness over time. “We don’t know yet if this one will work for me, but that’s been the course of my journey over the past few years,” she said.
Pillars of support
In spite of the uncertainties, the siblings ascribe their ability to persevere to their strong family bond and their UCLA Health caregivers who have been with them every step of the way.
“The great thing about UCLA is that our doctors and staff have always educated us about how to deal with our illness,” said Reed Elmore. “We also know our bodies well enough to advocate for ourselves, and to have real conversations with our doctors. They are always striving to understand sickle cell disease and to improve the care that can be delivered to give us the best quality of life.”
Having a strong support system is essential for people with sickle cell disease, said Martinez.
“This disease is debilitating, and without a support system, it is very difficult to manage it,” she said. “There also is a stigma to sickle cell disease, and we are trying to break that so patients are treated with the care and compassion they deserve.”
Family support, too, is essential. Reed said their mother prepped family and friends how to respond if he or his sister had a crisis. “We’ve always had a great support system of friends and family who understood their assignment when things became hard,” he said.
The siblings got a chance to show how strong their family bond is when the Los Angeles Dodgers recognized them in front of a stadium full of fans — including members of their family — before a recent game at Dodger Stadium.
“It was amazing and emotional. To be presented with a jersey with our names on it was so unexpected,” said Reed. “It felt awesome to be honored by so many people. We really feel supported, and that’s a blessing.”
