No matter how old you are, it is difficult to live with a chronic illness—especially one as unpredictable as sickle cell disease. Thirteen-year-old Alexis Gordon understands—she was diagnosed with Sickle Beta Thalassemia when she was two months old. Unlike most of her friends and classmates, Alexis has to juggle the demands of her schoolwork and extracurricular activities with the symptoms and side effects of sickle cell disease. “When I was younger, I didn’t like to talk about it,” says Alexis. Now I know that I can share my story and help educate other people about this condition.”
Sickle cell disease is an inherited blood disorder characterized by defective hemoglobin, the protein in red blood cells that carries oxygen to the tissues of the body.
“Normal red blood cells are smooth, round, and flexible, like the letter ‘O,’ so they can move through the vessels in our bodies easily,” says Dr. Monica Hulbert, director of the Pediatric Sickle Cell Disease Program at St. Louis Children's Hospital and assistant professor of pediatrics at Washington University and the. “Sickle red blood cells are stiff and sticky, and form into the shape of a ‘C’. These sickle cells tend to cluster together, and cannot easily move through the blood vessels. This causes a blockage in small arteries or capillaries and stops the movement of healthy, normal oxygen-carrying blood. When these blood vessels are blocked by sickle cells, that part of the body can’t get enough oxygen, leading to the painful and damaging complications of sickle cell disease. The disease can affect any and all major organs—the bones, brain, lungs, eyes, liver, heart, and kidneys. Common problems include anemia, strokes, damage to the kidneys and eyes, and other life-threatening infections.”
“Sickle cell disease affects people in different ways,” says Alexis. “One complication I have had to deal with is avascular necrosis in my hip. My sickle cell disease has caused part of the bone tissue in my hip to die and it is something that I am always going to have to watch very closely.” This can be very painful and causes reduced mobility. Alexis also had her gallbladder removed recently.
“I try to take care of myself so I don’t end up in the hospital. A couple of things I can control is drinking lots of water to stay hydrated and avoiding too much heat or cold outside.”
“Temperature extremes can lead to a sickle cell crisis,” says Alexis’ mom, Cassandra. “A ‘sickling’ crisis is very painful and usually results in a hospital stay for monitoring and pain management. Fortunately, Alexis is very conscientious about her health and her school work. I don’t have to remind her about taking care of herself—she is on top of it and does what she can do to get plenty of sleep, eat right and stay healthy. She does not want to miss school and get behind in her classes. Her dad and I are so proud of what she has accomplished so far. She is a member of Duke University’s TIP Scholars Program and the National Junior Society. She is also on the Principal’s Honor Roll—despite the challenges of her sickle cell disease.”
Alexis receives ongoing care and monitoring as a patient of the Pediatric Sickle Cell Disease Program at St. Louis Children's Hospital. “Patients are seen every three to six months,” says Dr. Hulbert. “We work closely with the patient and his or her family to see how they are doing, identify problems and offer supportive services to help them be successful in school. Because we are a teaching hospital, our patients have access to the most up-to-date information to treat symptoms, such as pain, as well as prevent complications of sickle cell disease, such as strokes, vision loss, and severe infections.”
Families and caregivers value this team approach to pediatric sickle cell care. “Terianne Lindsey, a Pediatric Nurse Practitioner on the sickle cell team has cared for Alexis since she was first diagnosed,” says Cassandra. “She has played a key role in Alexis' ongoing healthcare. Terianne knows the ins-and-outs of her medical history and can communicate this to the other members of the team—especially since Alexis does not always fit into the category of the ‘typical’ sickle cell patient.”
St. Louis Children’s Hospital also offers supportive programs to help patients live with the day-to-day emotional issues surrounding sickle cell disease. Debbie Woods, Pediatric Nurse Practitioner with the Pediatric Sickle Cell Disease Program, has helped launch a program for adolescents and young adults with sickle cell disease. “After relying on their parents to oversee their health care for most of their lives, teens can find it difficult to make the transition to being responsible for their own health. Teens and young adults need to gain confidence in their skills, ranging from refilling their own medications to discussing their concerns with their doctor or nurse practitioner. We have put together a multi-disciplinary team of hematologists, an adolescent medicine specialist, the sickle cell disease team psychologist, the sickle cell coaches and additional supportive staff to help our young adult patients take control of their health and build a support network. We also sponsor Camp Crescent—a summer camp for children ages 8 through 13 who have sickle cell disease. It is a place where they can join in traditional camp activities while under the care of medical specialists and become more confident about living with the disease.”
Alexis encourages her peers to take advantage of these programs. “Now that I am older, I plan on participating in the program for adolescents and young adults. I have really enjoyed my time at Camp Crescent. It has been great to connect with other kids with sickle cell disease—they know exactly how I feel. I met one of my best friends, Bobbi, through camp. It is good to have a best friend, but it is the ultimate to have a best friend with sickle cell disease—like Bobbi!”