Teen Survives Life-Threatening Sickle Cell Crisis
With the support of his family and Cook Children's care team, Caleb overcame the odds stacked against him.
By Ashley Antle
When the Ray family embarked on a cruise in April to celebrate Caleb Ray’s 18th birthday, their focus was on celebrating a milestone, not the sickle cell disease Caleb has lived with since being diagnosed shortly after birth.
Towards the end of the cruise, Caleb developed a fever and began to experience pain. The family knew the pain was likely associated with sickle cell, but none of the normal remedies Caleb used to treat a sickle cell pain episode gave any relief. After disembarking and arriving home, his pain grew so severe that his family took him to the Emergency Department (ED) at Cook Children’s Medical Center where he was admitted to the hospital.
Shortly after, Caleb’s lungs, liver and kidneys began to fail. He was transferred to Cook Children’s Pediatric Intensive Care Unit (PICU) where he was placed on a ventilator to support his breathing, and continuous dialysis to rid his body of the buildup of toxins from kidney failure. His prognosis was grim.
A Viral Trigger
A battery of tests revealed that Caleb’s life-threatening sickle cell crisis was triggered by a common childhood virus called Epstein-Barr (EBV). Many people are infected with EBV in childhood and experience little to no symptoms. In some cases, EBV can lead to infectious mononucleosis, also known as mono, according to the Centers for Disease Control. Healthy teens and adults who contract EBV and have symptoms usually recover within a few weeks. But for those living with SCD, any virus, even those that are common and typically mild, can trigger serious complications.
“The Epstein-Barr virus triggered something called EBV-induced sickle hepatopathy,” said Clarissa Johnson, M.D., hematologist and SCD specialist at Cook Children’s. “Essentially, that is a condition where you can develop acute liver failure, which he did. And when you develop liver failure, it can affect other organs, including your kidneys.”
Caleb also developed acute chest syndrome, a severe lung-related complication of SCD and one of the leading causes of death in individuals with sickle cell, according to the American Lung Association. In acute chest syndrome, sickled cells block vessels in the lungs, leading to a pneumonia-like illness.
“Any one of those organ systems getting critically ill can result in a person's death, particularly a sickle cell patient,” said James Marshall, M.D., a pediatric intensivist at Cook Children’s. Dr. Marshall cared for Caleb while in the PICU.
Caleb’s mother, Stephanie Ray, and grandmother, Tracy Ray, kept constant vigil at his bedside.
“I didn't know how he was going to come out of it,” Tracy said. “They were talking about his kidneys not working and possibly needing a transplant. It was just all horrible news. I just kept telling Stephanie and my family that we can't be moved by what we see, but stand on what we know and we know that God can heal.”
Caleb underwent a number of supportive treatments and therapies while in the PICU, including an exchange transfusion to quickly reduce the percentage of sickle cells in his body and prevent further damage to his organs. A host of medical specialists, including Dr. Marshall, Dr. Johnson, nephrology, pulmonology and many more, closely collaborated on Caleb’s case.
“Part of our method of caring for critically ill children at Cook Children’s is to freely bring in subspecialists to focus on specific organ system disorders with the critical care doctors kind of quarterbacking the team,” Dr. Marshall explained. “In most intensive care unit settings for children, the critical care doctors run it all with advice from the subspecialists. But we work as a team, so there's not someone telling the others what to do. It's a bunch of us getting together and pondering the patient, day and night, thinking of the best combined strategy.”
With the support of his family and care team, Caleb overcame the odds against him and was discharged from the hospital on May 25, one month after he was admitted to Cook Children’s.
“All patients like Caleb would have not survived,” Dr. Marshall said. “But his whole body and mind all wanted to get better. I was with him when we took out his breathing tube and let him wake up and was able to learn more about his personality. He was just as cheerful and vigorous coming out of the dark, dark night of critical care as any young man that I would meet. So a strong spirit and a positive approach to life, particularly when you have something that’s going to be with you for life, like sickle cell disease, is just absolutely required to get through the low spots.”
Sickle cell disease (SCD) is an inherited blood disorder that affects the body’s hemoglobin, or the protein in red blood cells that carry oxygen throughout the body. A mutation in the hemoglobin gene causes red blood cells to be sticky, rigid and shaped like crescent moons, or sickles, instead of round, flexible and flowing easily through the blood vessels like normal red blood cells. Sickled cells can clump together and block the flow of blood, resulting in inflammation and pain from decreased oxygen to the organs. Sometimes the pain can be so severe an individual requires hospitalization, like in Caleb’s case.
September is National Sickle Cell Disease Awareness Month. An estimated 100,000 people in the United States have SCD. It primarily impacts people of African descent, those from Central and South America, and people of Middle Eastern, Asian, Indian and Mediterranean descent.
There are several types of SCD, the most common being Hemoglobin SS, usually referred to as sickle cell anemia. Caleb has Hemoglobin SC, the second most common type of SCD. Hemoglobin SC has similar symptoms as SS, although sometimes less severe. Symptoms can be mild throughout childhood but worsen with age.
“Some of our families whose children have SC may think that their child is not at risk for problems and that is not true,” Dr. Johnson said. “People with SC don't tend to be the ones who are in the hospital the most and they don’t tend to have some of the more severe complications that we see, that's going to be our SS patients, but the older someone gets with SC disease, they can begin to have more problems.”
That’s why Dr. Johnson says it's important for Hemoglobin SC families to stay engaged with their doctor and their care throughout childhood and adulthood.
While SCD limited Caleb’s ability to play some sports while growing up — over-exertion and dehydration can trigger SCD pain episodes — it hasn’t stopped him from living life to its fullest. The key, he says, is to take things “day by day.” That’s what his family did during his latest health crisis, although his grandmother said it was more like one prayer at a time.
The Ray family points to the doctors, nurses and staff who cared for Caleb as contributors to his healing, too. They say everyone from the medical providers to food service and the housekeeping teams were dedicated to Caleb’s care and to making them feel like family.
“We cannot thank them enough,” Tracy said. “They touched us in a special way. I mean, we’re a Black family and most of the staff were white. We never felt any kind of resistance at all, and that's just not the life that we live. But those nurses took care of Caleb like he was theirs. They were so respectful. Even when he was intubated, when they would have to move him or lift him, they talked to him like he was a human. It was just beautiful.”
Dr. Marshall gives the credit right back.
“His family’s membership in our health care team was really fabulous. How they interacted with the health care team was a model for other families.” Dr. Marshall said. “The family was always there and always present. They were always pleasant despite the tenseness of the situation. I think the point really is that a collaborative family can make patients better. Not just be there, but literally take part in the patient's care and help them heal. And his family was a great example of that."
Today, Caleb is looking ahead to the future. He will soon begin coding courses in hopes of becoming a computer software coder.
“One thing that I admire about Caleb is his resilience,” his mother said. “I think that throughout the whole thing, he's always kept his positive attitude. He's always been a happy kid. He handles it better than we would for sure. He’s not angry. He's not bitter. He just rolls with the punches.”
For one week every summer, the cabins at Camp John Marc in Meridian, Texas, fill with campers who share one thing in common — they all live with sickle cell disease. Camp John Marc is a residential camp serving children and families living with a chronic illness and physical challenges, and a Cook Children’s Camps for Kids partner.
This special week is known as Camp Jubilee. In addition to traditional summer camp activities like arts and crafts, swimming, horseback riding and climbing a ropes course, campers get to connect with other kids who share similar challenges in growing up with and managing SCD. These connections help build a sense of belonging and, with the encouragement of their peers, resiliency.
The next Camp Jubilee is scheduled for July 7 - 12, 2024. There is no charge for campers, thanks to generous donors who support Camp John Marc and Cook Children’s Camps for Kids. If you would like to make a donation to support Camp Jubilee and Camps for Kids, click here.
To learn more about Camp Jubilee and how to register a camper, check out cookchildrens.org and campjohnmarc.org.