The children grow up with labels such as “weird” or “different”. It gets worse – some brand them evil because of their “evil eye”. Most have to struggle with the stares and teasing from other children wherever they go. Abby White recalls how when she was a child, her classmates taunted her because her eyes were “funny”.
“Children can be very cruel but it’s because they do not understand,” says Abby who is now 32 and running the Daisy Eye Cancer Fund (DECF), a worldwide fund that deals with a form of cancer unique to children. When she was eight days old, Abby’s parents spotted a white glow in her pupil, which confirmed to them that the baby had not escaped the cancer her father had suffered.
The condition, medically known as retinoblastoma, is a fast growing form of cancer that affects children from infancy. It manifests as a ‘glow’ in the baby’s pupil but this could easily be dismissed as a reflection of light. According to researchers in the UK, the cancer comes about when the retinoblast cells found in the eyes fail to divide and mature into retinal cells.
Instead, they grow out of control and form a tumour. This happens while the baby is still in the womb as the eyes and other organs are developing. Researchers say that four in 10 cases get this condition from their parents. They inherit the cancerous cell Rb or RB1 gene and develop what is called congenital (hereditary) retinoblastoma.
Because all the cells in the body have the changed Rb gene, these children also have a higher risk of developing cancers elsewhere in the body. Research studies still cannot account for what causes cancer in the remaining 60 per cent who develop sporadic retinoblastoma (that is despite having no family history of the disease) but note that it nearly always affects only one eye and is often diagnosed when the baby is older.
If not treated, the tumour spreads to fill most of the eyeball and other parts blocking the flow of liquid within the eye. Pressure built by the blockage can cause loss of vision. If not contained then, experts say, the cancer can spread to the brain, bones and lymph nodes.
Abby explains that in its incubation stages, the cancerous cells have almost no effects on a baby’s health. So spotting the disease almost entirely depends on the keenness of the child’s parents or care-giver. “It is very easy to miss the cancer because in the early stages the baby is not in any pain or discomfort,” she says. “The change in the eyes is so gradual that it is very likely the mother will miss it.”
Abby suggests the use of a camera flash to pick out the while glow. “The flash hits the tumour and the light bounces back as a reflection,” she explains. “Otherwise, looking at it against natural light one would easily dismiss it as a trick of the light.” Advanced symptoms include redness of the eyes, pain or the child’s eyes seeming to look in different directions.
The good news is that it is the only form of cancer that is almost entirely curable. Statistics from the American Cancer Societies show that more 90 per cent of children are cured from the cancer. However, once it gets out of the eye, the cancer takes the shape of other cancers reducing the possibility of a cure to about 10 per cent, says Abby.
Treatment depends on what stage the tumour has reached. In the most desperate situations, surgery is done to remove the eye; the hollow is then fitted with an artificial eye. Other treatments include radiation therapy (external beam radiation therapy), chemotherapy, photocoagulation (for small tumours, using lasers to kill them), cryotherapy (using small, very cold probes to freeze and kill the tumour), and thermotherapy (using infrared lasers to heat and kill the tumour).
Treatment for retinoblastoma is available in Kenya. The Kenyatta National Hospital (KNH) deals with a number of cases regularly but doctors fear the statistics they have grossly under-represent the actual occurrence of the condition in the country. Dr Kahaki Kimani, who is the head of the team that manages retinoblastoma diagnosis and treatment at KNH, says they receive about 60 cases of the disease in a year.
“This is a very big number for such a rare disease that is completely curable,” he says. Dr Kimani’s team includes an ophthalmologist, paediatric oncologist, radiotherapist and a pathologist. Unfortunately, of the retinoblastoma babies received at the hospital, only a few survive.
The doctors say this is because many of the children are brought in too late – when the cancer has already spread to other parts of the body. In some cases doctors say the glow is mistaken for cataracts, a common eye infection that is not life threatening and which is, therefore, treated as such.
“Retinoblastoma is not yet well known in Kenya. There are even medical workers who do not know about it,” says Dr Kimani. He adds that efforts are being made to create awareness especially among mothers. “In our new maternal health booklet, for instance, information on retinoblastoma has been included alongside that of immunisation,” says the doctor.
Retinoblastoma is rated as the second most common cancer affecting children in Kenya after leukaemia, which is the most common worldwide. Yet treatment is readily available. “Any ophthalmologist can diagnose the cancer.
If a mother notices the white glow, squinting or that the baby is cross-eyed, she should have the baby checked,” the doctor says. If retinoblastoma is confirmed, laser therapy, cryotherapy or chemotherapy can be done on the child. “Chemotherapy is done to reduce the size of a large tumour,” explains the doctor.
Surgery is usually the last option, done when the eye cannot be saved and the child’s life is at risk. Following treatment, the child will grow up healthy just like any other child. However, radiation therapy may cause side effects. “Again, this normal development after treatment is possible only if the cancer is caught in good time, otherwise the child may be at risk of getting other cancers,” cautions Dr Kimani.
Abby, who has encountered many parents whose children have the cancer, say they too, contribute to the low turn out of affected children. “When we tell parents that their children require surgery some of them hesitate because they fear their child’s face will be mutilated,” she says. “They also worry the child will grow up discriminated against by the community.”
In Abby’s case, the doctors were able to remove her eye before the retinoblastoma spread to the rest of her body, but not before it affected her left eye. But unlike her father who had to have both eyes removed, Abby’s left eye was saved, leaving her only partially blind.
Over the years the young Abby underwent cancer treatment in England to ensure it was contained. At the time, chemotherapy had not been discovered so she had to go though rigorous radioactive treatment. “The radioactivity caused my eyes to shrink,” she says, pointing to the part of her face that looks sunken. “That is why the other children made fun of me in school,” she adds.
Having struggled all her life with looking different from others and watching other children go through what she herself had experienced, Abby took a keen interest in the study of retinoblastoma. She has especially concerned herself with creating awareness among African mothers on the existence of the disease.
DECF, of which she is the CEO, began as a funds drive to collect money for a 13-month-old girl named Daisy who needed retinoblastoma treatment. When the amount needed was reached and surpassed, Daisy’s parents, family friends and doctors decided to turn the fund into a global organisation that focuses on improving care for affected children and their families.
Abby came across the fund while looking for donors to help an 11-month-old baby girl from Botswana, Rati, who had been diagnosed with the disease. “I had begun a website with information on retinoblastoma. Rati’s parents contacted me because they wanted to find out how they could access treatment and that is when I met Daisy’s family,” says Abby.
In 2006, DECF chose Kenya as an ideal site to set up a Model Retinoblastoma Strategy for Developing Countries. Kenya was chosen because this was where Abby’s father was born and first diagnosed with the cancer. It was also considered an ideal location because Kenyatta National Hospital already had an established retinoblastoma working group.
According to DECF, the major problem causing failure to treat the eye cancer in Kenya is the lack of proper labs for examining specimens, and lack of specialist training. Dr Elizabeth Dimba, an oral pathologist and expert in cancer studies at the University of Nairobi, says proper equipment for determining the type of cancerous cells and extent of spread is what they lack.
“What we are using now is a lab at the university’s School of Dental Science,” says Dr Dimba, adding that this is not enough to manage the cases that come from all over the country. Dr Dimba says failure by pathology tests to detect the cancer or extent of spread is what is causing children to die.
But last year, DECF, the Ministry of Public Health, KNH and the School of Dental Science struck a deal that would see the creation of a centralised lab where all specimens would be tested. In addition, DECF has offered to train doctors and other professionals in Canada, which is known to have the best institutions for the study of the disease.
Dr Dimba is expected to travel to Canada in early May for a specialised three-month training course courtesy of two East African philanthropists, Rita and Charles Field- Marsham. But even with all these plans, success still depends on the mother and her ability to detect the disease early.
“We encourage mothers to keep checking their babies,” stresses Abby. “ With the right care at the right time, retinoblastoma can be cured and the child can survive and go on to lead a productive life.”