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Tuning into Sickle Cell

UC San Diego Health offers the only adult program and same-day infusion services to manage the lifelong condition

By Yadira Galindo   |   August 29, 2019

​Growing up, Alfred Sturrup Sr., now 70, was unable to join “normal” children as they joyfully exerted themselves in play, sports and other activities for fear that he might go into a health crisis, spurred by an inherited blood disorder called sickle cell. Instead, Alfred embraced music and became a brilliant piano player who opened his first music school at the age of 10.

“Many people considered me a prodigy. They didn’t understand the work that went into building up my skills,” said Alfred. “I used to practice when I was sick and when I was well. I became a phenomenon based on how I could be so sickly and yet develop such skills.”

Alfred looks back at his childhood and the decades that followed not as an impairment but as the years that shaped him into the musician, husband, father and entrepreneur that he is today.

 

Alfred P. Sturrup at the piano.

“I had a wonderful mother and father who taught me not to focus on the sickness but to live between the crises because pain was only temporary,” said Alfred. “They instilled in me not to focus on the activities I could not participate in and instead focus on my strengths.”

Sickle cell is a lifelong condition, passed down from a parent’s genes, resulting in abnormally shaped red blood cells that are hard, sticky and clump together affecting the blood’s ability to carry oxygen throughout the body.

If a child inherits the gene from just one parent, the child becomes a carrier but is otherwise healthy. When both parents are carriers a child has a 1 in 4 chance of being born with active sickle cell. Although the risk of mortality is high in children age 5 or younger, with recent advances in health care, people with sickle cell disease are living well into adulthood, said Srila Gopal, MBBS, a UC San Diego Health hematologist who leads San Diego’s only adult sickle cell disease program.

“Sickle cell is a disabling condition that can cause an unexpected flair up anywhere, at any time and can be very painful,” said Gopal, who is also Alfred’s treating physician. “But it is manageable. Our goal is to improve preventive and supportive care for individuals with this disease to improve the overall quality of life of patients. We hope to create individualized plans so that when a person living with sickle cell disease arrives at the hospital, we have a treatment strategy in place that will help them bypass the emergency department and possibly avoid hospitalization.”

Sickle cell disease can cause chronic pain, pulmonary hypertension, chronic kidney disease, multiple organ failure and increased stroke risk, among other complications. Working together, a multidisciplinary team that includes hematology, emergency personnel, pain management and the infusion center team can treat patients more quickly and effectively to control pain and reduce the risk of complications, said Gopal.

Dr. Gopal with patient

Patients who experience a health crisis can now seek help from the only same-day infusion center in San Diego County. At UC San Diego Medical Center in Hillcrest, patients can receive their first dose of pain medication immediately, as well as oxygen, hydration or other necessary treatment instead of waiting in an emergency room.

As a child, Alfred remembers being admitted to the hospital once or twice a month. These days he’s learned to manage his condition by focusing on eating a healthy diet, getting enough sleep, drinking lots of fluids and trying to avoid known triggers.

This, however, has limited his ability to do strenuous physical activities so Alfred immersed himself in science, history, philosophy, and of course, music. He had a telescope and microscope that where his toys and learning tools growing up. Alfred said he was inspired by his father, who was himself a world-class violinist and played multiple instruments, including the accordion and piano.

“He stopped playing piano after I excelled above him,” Alfred said with a chuckle.

Alfred’s musical talents led him to perform in music halls but teaching music called to him.

“The skills that I developed would be much more effective through teaching and sharing my knowledge through teaching,” said Alfred.

Alfred continues teaching today. He employed his entrepreneurial skills and created a successful online business, The Piano Bug, to help students become more proficient on the piano.

Infusion Center

The infusion center at UC San Diego Medical Center in Hillcrest has dedicated appointment times for patients with sickle cell disease who are experiencing a crisis and need urgent infusion services. Please call 619-543-2639.

“What really inspires me about Alfred is his incessant optimism, resilience in the face of the challenges life has thrown at him, his ability to create a meaningful life despite all limitations, and most importantly, his willingness to share his experience for the benefit of others afflicted by this disease,” said Gopal.

At age 70, Alfred has already beaten the odds at surviving sickle cell disease, said Gopal. Improvements in preventative and supportive medical care has enabled meaningful changes in the quality and quantity of life of those with this condition.

The Centers for Disease Control and Prevention (CDC) estimates the average life expectancy for people with the most severe form of sickle cell is 30 years lower than that of people without it. In California, this gap is even wider, with an estimated lifespan of just 43 years for patients with sickle cell disease.

According to the CDC, the condition affects roughly 100,000 Americans and mainly touches people of African ancestry. One out of 365 African American babies are born with this condition in the United States. One in 13 African American babies are carriers of the sickle cell trait.

Alfred’s wife was not a carrier, so his children do not have sickle cell disease but they might well be trait carriers.

In addition to African Americans, Latinos are also known carriers, but much less frequently. One in 16,300 Latino babies are born with the disease, says the CDC. The gene has also been found in people whose families are from the Middle East, India, Mediterranean countries and in Native Americans.

NP Groesbeck with patient

In California, newborns are screened for sickle cell to allow treatment to begin as soon as possible to help reduce the risk of complications.

Treatment depends on symptoms, age, a person’s general health and the severity of the condition. This might include vaccines, antibiotics, blood transfusions or a bone marrow transplant.

Pain medications are a staple of patients with sickle cell disease. Unfortunately that means patients are often labeled as drug users because they are on narcotics their whole life, said Gopal.

“I noticed that when I was young if I said I had pain, doctors feared — rightfully so I presume — that I was high-risk of becoming addicted,” said Alfred. “When I got to the point when I could manage it, I refused to take pain medications. I only took it as long as I needed.”

Having a personalized treatment plan and a multidisciplinary team behind each patient, as well as a same-day infusion center available, means patients can get the care they need instead of having their pain questioned, said Gopal.

“My health became a part of who I am. Be who you are. Don’t deny your illness. Don’t be ashamed of it. Don’t focus on that. Focus on what you can do for humanity and those who are around you while you are here so that when you’re gone you’ll be worth remembering,” said Alfred.


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