Teen survives life-threatening sickle cell crisis

by Ashley Antle

When the Ray family embarked on a cruise in April to celebrate Caleb Ray’s 18th birthday, their focus was on celebrities.Caleb RayThis was a milestone other than Caleb being diagnosed with sickle cell disease shortly after he was born.

Towards the end of the voyage, Caleb developed a fever and began to feel pain. The family knew the pain could be related to sickle cell anemia, but none of the conventional treatments Caleb used to treat sickle cell pain attacks provided any relief. After disembarking home, his pain became so severe that his family took him to the Emergency Department (ED) at Cook Children’s Medical Center, where he was admitted.

Soon 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 continued dialysis to remove toxins that had accumulated in his body due to kidney failure. His prognosis is grim.

viral trigger

A series of tests revealed that Caleb’s life-threatening crisis with sickle cell disease was caused by a common childhood virus called Epstein-Barr (EBV). Many people are infected with EBV as children but have few symptoms. In some cases, EBV can cause infectious mononucleosis, also known as mononucleosis, according to the World Health Organization. Desease control Centre. Healthy teenagers and adults who are infected with EBV and develop symptoms usually recover within a few weeks. But for people with SCD, any virus, even those that are common and often mild, can cause serious complications.

“EBV causes pathological liver disease caused by EBV,” he said Clarissa Johnson, MD, hematologist and SCD specialist, Cook Children’s Hospital. “Essentially, this condition can cause acute liver failure, which is what he did. When you have liver failure, it can affect other organs, including your kidneys.”

Caleb also suffered from acute chest syndrome, a severe lung-related complication of SCD and one of the leading causes of death in patients with sickle cell disease. American Lung Association. In acute chest syndrome, sickle cells block blood vessels in the lungs, causing a pneumonia-like illness.

“Critical illness in any of these organ systems can lead to a person’s death, especially in patients with sickle cell disease,” he said. James Marshall, MD, pediatric intensivist, Cook Children’s Hospital. Dr. Marshall cared for Keller while he was in the pediatric intensive care unit.

Caleb’s mother, Stephanie Ray, and grandmother, Tracy Ray, remained at his bedside.

Caleb Ray and family (second from right)“I don’t know how he’s going to get out of this,” Tracy said. “They were talking about his kidneys not working and possibly needing a transplant. It was all terrible news. “I kept telling Stephanie and my family that we couldn’t be moved by what we saw and to stick to what we knew, we Know that God can heal. “

While Caleb was in the Pediatric Intensive Care Unit (PICU), he received a variety of supportive care and therapies, including exchange transfusions, to quickly reduce the percentage of sickle cells in his body and prevent further organ damage. Many medical specialists, including Dr. Marshall, Dr. Johnson, nephrology, pulmonology, and others, worked closely on Caleb’s case.

“Part of Cook Children’s approach to caring for critically ill children is to bring in specialists at no cost to focus on specific organ system diseases, with an intensivist as the quarterback of the team,” explained Dr. Marshall. “In most pediatric intensive care units, intensivists operate on the advice of specialists. But we work as a team, so no one tells anyone else what to do. A group of us get together and think about the patient day and night. situation and think about the best comprehensive strategy.”

With the support of his family and care team, Caleb overcame many obstacles and was discharged from the hospital on May 25, one month after he was admitted to Cook Children’s Hospital.

“All patients like Caleb don’t survive,” Dr. Marshall said. “But his whole body and mind wanted to get better. I was with him when we took out his breathing tube and woke him up and was able to learn more about his personality. He was as good as any young man I’ve ever met. , walking out of the dark night in the ICU feeling cheerful and energetic. So a strong spirit and a positive attitude towards life, especially if you have something that will stay with you for your entire life, like a cell disease, is absolutely Need to get through the lows.”

lifelong care

Caleb Ray On the Beach 2Sickle cell disease (SCD) is a genetic blood disorder that affects the body’s hemoglobin, the protein in red blood cells that carries oxygen throughout the body. Mutations in the hemoglobin gene cause red blood cells to become sticky, stiff and shaped like crescents or sickles, instead of being round, pliable and able to flow easily through blood vessels like normal red blood cells. Sickle cells can clump together and block blood flow, reducing oxygen supply to the organs and causing inflammation and pain. Sometimes the pain can be so severe that it requires hospitalization, as was the case with Caleb.

September is National Sickle Cell Disease Awareness Month.one An estimated 100,000 people in the United States SCD. It primarily affects people of African descent, Central and South Americans, and people of Middle Eastern, Asian, Indian, and Mediterranean descent.

There are many types of SCD, the most common being hemoglobin SS, often called sickle cell anemia. Caleb has hemoglobin SC, the second most common type of SCD. Hemoglobin SC has similar symptoms to SS, although sometimes the symptoms are milder. Symptoms may be mild throughout childhood but worsen with age.

“Some of our families whose children have SC may think their children are not at risk for problems, but that is not the case,” Dr. Johnson said. “People with SC tend not to be the ones who have the most hospitalizations, and they don’t develop some of the more severe complications that we see, which would be our SS patients, but the older people who have SC disease, they may start to have more problems.”

That’s why Dr. Johnson says it’s important for families with hemoglobin SC to stay connected to and receive care from their doctors throughout childhood and adulthood.

family-centered

Although SCD limited Caleb’s ability to participate in some sports growing up— Overworked Dehydration can trigger SCD pain, but that hasn’t stopped him from living a full life. The key, he said, 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 a prayer.Caleb Ray wears white T-shirt with family

Lei family members pointed to doctors, nurses and staff Quinn Caring for Caleb also contributed to his recovery. They said everyone from the medical providers to the food service staff to the housekeeping team was dedicated to taking care of Caleb and making them feel like family.

“We can’t thank them enough,” Tracy said. “They touched us in a special way. I mean, we’re a black family and most of the staff are white. We didn’t feel any kind of pushback at all, that’s not our life. But those nurses took care of Caleb acted like he was their person. They were so respectful. Even when he was intubated, when they needed to move him or lift him, they talked to him like a human being. “It’s so beautiful . “

Dr. Marshall immediately returned the credit.

Caleb Ray and Mom“His family has been truly wonderful to have as a member of our health care team. “The way they interact with the health care team sets an example for other families. ” PhD. Marshall said. “The family is always there and always present. Even though the situation is stressful, they are always pleasant. I think what is really important is that a cooperative family makes the patient better. Not just being present, but actually engaging the patient care and help them recover. His family is a great example of that.”

Today, Caleb looks to the future. He will soon start coding courses in hopes of becoming a computer software coder.

“One thing I admire about Caleb is his resilience,” his mother said. “I think he’s always maintained a positive attitude throughout this whole process. He’s always been a happy kid. He’s definitely handled it better than we did. He’s not angry. He’s not bitter. He’s just going with the flow.”

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