St. Jude offers children with eye cancer the specialized care they need to envision a world beyond cancer therapy.
Once again, Brayden Shields is in the driver’s seat. Today’s vehicle is a late-model desk chair, although he has been known to commandeer a sporty little laundry basket.
“Get in!” the 3-year-old urges his mom, as he fastens an invisible seat belt. Misty Shields dutifully climbs into the passenger’s seat. Their excursion culminates at Brayden’s favorite destination: St. Jude Children’s Research Hospital.
Brayden was born with a rare eye cancer called retinoblastoma. The first sign of a problem occurred the moment Misty held her infant son in the delivery room.
“I told my husband that something was wrong with Brayden’s eyes,” she recalls. “They seemed to jitter and drift away from each other.”
Misty and Drew continued to worry about their son’s eyes. When Brayden was 7 months old, the pediatrician referred him to an ophthalmologist, who discovered large tumors. The physician warned that both eyes might have to be removed.
“If it were your kid, where would you take him for treatment?” Misty asked.
“I’d send him to Memphis,” the physician replied.
One less stressor
Within 24 hours, the Shields had obtained a referral and were walking through the doors of St. Jude. Soon, the family made an astounding discovery.
“At one point, Drew pulled out his insurance card and asked if the hospital bills would be sent to our house while we were in Memphis,” Misty recalls. “The lady said, ‘Oh, you won’t be getting a bill from us.’ She told us that they would also pay for transportation, would give us a place to stay and would pay for our food while we were here. It was just crazy.
“We began crying, and we probably said ‘thank you’ at least 100 times.”
It takes a team
With three large tumors in his left eye and two in his right, Brayden had already lost much of his vision. Treatment would require an intensive collaboration among a cadre of specialists, including oncologists, surgeons, radiation oncologists, ophthalmologists, occupational therapists and the hospital’s genetic counselor.
“In treating retinoblastoma, we have three main objectives,” explains Brayden’s oncologist, Ibrahim Qaddoumi, MD. “Our first objective, of course, is saving the child’s life. The second objective is to avoid removing the eye. And our third objective is saving as much vision as possible.”
St. Jude deftly bridges the gap between laboratory findings and the application of that knowledge in the clinic. Discoveries made in the Developmental Neurobiology lab of Michael Dyer, PhD, for instance, have identified potential targeted therapies that kill retinoblastoma cells more efficiently than compounds used in the past. These drugs may dramatically impact the treatment of patients such as Brayden and other children with retinoblastoma.
As the toddler received his chemotherapy in the hospital’s Medicine Room, Dyer, a Howard Hughes Medical Institute investigator, and his colleagues were collaborating to make additional discoveries to further improve retinoblastoma treatment.
Eradicating the seeds
In addition to the large tumors in his eyes, Brayden developed numerous small masses called vitreous seeds, which form when portions of larger tumors break away and float within the eye’s vitreous fluid.
The little boy received 11 cycles of chemotherapy, plus frequent evaluations under anesthesia. In conjunction with chemotherapy, St. Jude ocular oncologist Matthew Wilson, MD, treated Brayden’s eyes with a combination of laser therapy and cryotherapy, which uses a probe to freeze the tumor. Brayden underwent 26 laser therapy treatments and 24 cryotherapy treatments during a two-year period.
He also received a type of highly focused radiation treatment called brachytherapy, in which Wilson implanted radioactive plaques into Brayden’s eyes. For approximately 80 hours, the small disks delivered a prescribed dose of radiation only where it was needed, preventing radiation exposure to Brayden’s brain and surrounding tissues.
Preparing for the future
St. Jude offers a low-vision rehabilitation clinic to enhance the quality of life for children whose sight has been impaired by cancer. Mary Ellen Hoehn, MD, a St. Jude pediatric ophthalmologist, works with occupational therapists to optimize each child’s vision and share strategies that help the patient make the best of the vision that remains. Brayden also received physical therapy and speech therapy at the hospital.
“Like many other children who come to us, Brayden required the full gamut of services that only a multispecialty team can provide,” Wilson says. “This collaboration allows us to not only take care of the child, but also to equip him for his life going forward.”
Retinoblastoma is caused by a genetic defect. There are two forms of the disease: familial, which typically affects both eyes; and sporadic, which usually involves a single tumor affecting only one eye. Since Brayden has the familial form of the disease, every cell in his body lacks one of its pair of retinoblastoma genes. Although some children inherit this condition, others, like Brayden, develop a new mutation before birth. His big brother, Peyton, does not carry the mutation.
The hospital’s genetic counselor, Christine Odom, helped Drew and Misty understand the mutation as well as the importance of vigilance in monitoring Brayden’s health into adulthood.
“Most people assume that if you have the familial type of retinoblastoma, then one of your family members had to have had the disease,” Odom says. “But that’s not true. Nine times out of 10, it’s a brand new mutation in the child. Brayden’s chance of passing retinoblastoma on to his children, though, is 50 percent with each pregnancy.”
Because he has the hereditary form of the disease, Brayden also is at increased risk of developing second cancers, such as brain, bone, skin or soft-tissue tumors. As he moves toward adulthood, hospital staff will further educate Brayden about how to minimize his risks by adopting good health practices and obtaining regular medical evaluations.
In the driver’s seat
Today, it’s hard to believe that the energetic little firecracker with the Technicolor imagination has a visual impairment. Brayden runs, fingerpaints, rides a bike. He wiggles his hips to demonstrate his hula hoop prowess. He identifies colors and proudly counts to 14.
“We’ll never be able to say ‘thanks’ enough to the doctors, nurses and all the other staff who are helping our son,” she says. “We never imagined our child would be a St. Jude kid, but he is, and we are so blessed to have them.”
Abridged from Promise, Summer 2013