All around the world there is a big problem with a disease called retinoblastoma. This is the most common eye tumor in children, and the third most common cancer affecting children. This disease causes the growth of malignant tumors in the retinal cell layer of the eye.
“One in every 12,000 children in the United States is affected by retinoblastoma,” said Dr. Timothy Murray of Bascom Palmer Eye Institute, University of Miami. It is important to check for it the first five years of life. Seven thousand children die worldwide each year because of a late diagnosis.
In family photos, Joey had one red eye and one white eye. At the time, Pam Bergsma, his grandmother, had no idea what caused it. “I just thought it meant it was a lousy picture,” she said. The white dot was a reflection of a tumor that was forming in Joey's retina. It was a painful discovery. In picture after picture, Joey’s family had no idea these photographs held the key to save his vision and life.
After severe eye pain, Joey’s right eye was removed. Bergsma said, “It was one week after his second birthday on Monday, July 12th, 1999 that Joey, my beautiful grandson, told me his eye hurt. Wednesday he had a CAT scan and we were told to go to Bascom Palmer in Miami. Thursday afternoon Dr. Timothy Murray said he felt Joey had retinoblastoma and we needed to bring him in at 6 a.m. for an eye inoculation. They needed to remove his eye immediately in order to save his life.” At that time, there was hope in Joey’s family.
After months of treatment at nationally known cancer centers, the disease invaded Joey's brain and spinal fluid. The cancer was aggressive. He died Dec. 22, 2000, at the age of three.
If retinoblastoma is caught in time, it can be treated. If it spreads beyond the eye, as it did in Joey's case, there is no cure. As Bergsma said, “If I would have known about this disease and what the white spot represented, I would have saved his life.” It was right there in front of everyone, but nobody knew what it was.
“A study from Children's Hospital Los Angeles in 1958 reported a mortality of 9/50 patients (18 percent) with unilateral retinoblastoma and 11 of 22 patients with bilateral retinoblastoma (Carbajal UM. Observations on retinoblastoma. Am. J Ophtalmology. 1958;45:391-402). By 1984, the overall mortality had decreased to 9 percent (Nelson LB. Pediatric Ophthalmology. Philadelphia, PA: WB Saunders;1984;207-212). Survival is now 90 percent. The incidence is approximately 1 in 14,000 to 34,000 live births per year. It is hard to tell the exact number of patients who were diagnosed or died all over the U.S. We can extrapolate from the incidence as I noted above, and the mortality which is about 10 percent,” said Dr. Lilibeth Torno, one of the oncologists from Children's Hospital of Orange County, Calif.
Children die of metastic retinoblastoma, which means the cancer has spread beyond the retina into the body. When this happens, the survival rate is low.
“Retinoblastoma is the most common primary eye tumor in children. When detected early, survival is excellent. It is important to educate the public on signs and symptoms to look for, as well as establish guidelines for the pediatrician to screen for this condition,” said Dr Torno.
Snapshots may help to save children or grandchildren from retinoblastoma.
Everyone is familiar with the "red-eye" effect, which flash cameras produce. There are processes in printing to remove the "red-eye," and some new cameras have a device that causes multiple flashes that eliminate the distortion. But, as Bergsma explains, the “red-eye” has a life-saving value.
At home in Lake Worth, Fla., Bergsma is doing her best, trying to tell people about this disease. Wherever she goes, she hands out photos of her grandson, Joey.
Bergsma is pushing legislation that would require pediatricians to screen all newborns and infants for eye disease by dilating the pupils and using an ophthalmoscope, which magnifies the retina.
She has canvassed the state, collecting resolutions and letters of support from cities, counties and health officials for this legislation, House Bill #1117 and Senate Bill #2062.
“This will be a wake up call to parents,” she said. She wants eye screening to become a law in Florida.
“We have to change our standard. Every child born has the right to healthy vision,” said Bergsma. She wants a month dedicated to education, and a law requiring all physicians to perform the simple eye exam to try to detect the growing cancer.
Last spring a grandmother in West Palm Beach saw Joey’s story on TV and saved her granddaughter, Elexis,’ life.
According to Bergsma: there are three subjects parents need to be aware of:
1. You can take a picture of a tumor or cataract. Always be alert to your photos.
2. The ophthalmoscope needs to be used at every exam in a darkened room. It is the stethoscope for the eyes. A tumor or cataract can start to develop at anytime. (When you see the stethoscope come out for the heart...you need to see the ophthalmoscope come out for the eyes.)
3. An infant needs an eye dilation exam. One in every 677 live births in the United States has a treatable eye disease that will cause blindness if not detected and treated. Early detection and referral is the key to saving vision and, as in Joey's case, life. We would like to this done before they leave the hospital, at the 6-to-8 week well-baby exam, the 6-to-9 month well-baby exam and hopefully, one more time at the 18-month well-baby exam.
“We needed to be heard in appropriations when they stopped hearing bills in committee and went to session,” she said. “We tried to get the chairman to pull us out of committee to get to the floor. This did not happen. We had the votes on the floor. Time stopped us from getting there. We will be back in 2003 and Joey’s Bill will pass.”
“He is inside my heart, and my soul. He is my hero,” Bergsma said. She misses her little grandson, Joey.
“It is so simple. Joey should be alive. Please help me to stop this from ever happening again,” she said. “Joey is smiling...his message is being heard.”
For more information call Pam Bergsma (561) 586 2094 or www.lovejoey.com.
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