The treatment for retinoblastoma is complex. Although cancer cure remains a priority, eye salvage and vision preservation are equally important. A program to treat patients with retinoblastoma in CA has been developed in El Salvador, Guatemala, and Honduras through the cooperation of local pediatric oncology centers, their supporting non-profit foundations, the International Outreach Program at St. Jude, the retinoblastoma team at UTHEI, and ORBIS. When the programs began, early diagnosis and referral to a trained practitioner were priorities. As the programs grew, the need to provide more sophisticated ocular salvage necessitated more input and resources. There is now a network of pediatric oncologists and ophthalmologists who have the knowledge to diagnose and treat retinoblastoma. Also, ophthalmologists can now work together at the pediatric oncology centers using modern equipment such as RetCams, which facilitate rapid consultation, and diode lasers and cryotherapy units, which allow focal treatment of intraocular disease in an effort to avoid external beam radiotherapy or enucleation. A concurrent program in Jordan, results of which have been recently reported, uses similar methods of multidisciplinary team development, acquisition of equipment, and telemedicine consultations to improve eye salvage.5
Treatment is provided at already established pediatric oncology centers that are supported by local non-profit foundations that provide some social services and financial help for families6
. This support is crucial to decrease the high rates of abandonment and refusal of therapy that occur among patients with retinoblastoma and their and families in countries with limited resources. Experts also provide consultation via collaborative online networks to ensure optimal therapy to patients with complicated disease. These consultations have also improved local expertise.
We found no statistically significant increase in rates of survival for patients with retinoblastoma for 2000–2003 and 2004–2008. This probably reflects the low number of patients after censoring for abandonment in the early period. Except for El Salvador, significantly fewer patients refused initial therapy or abandoned treatment over the most recent period, and we expect this decrease to ultimately contribute to increased rates of survival with continued follow-up. Late diagnosis resulting in advanced disease11
and abandonment of therapy continue to be major factors contributing to decreased survival. These problems are more social than medical; therefore, future efforts to improve survival for patients with retinoblastoma in these countries need to focus on more than medical issues (i.e., programs of awareness, identification of early signs by caregivers, supportive measures to decrease abandonment of therapy) while maintaining present infrastructures.
The program in El Salvador has been the most successful in terms of early diagnosis and reduced abandonment, perhaps due in part to the small size of the country, which decreases travel time, and the relative homogeneity of the population. Ongoing donation of prostheses, an important supportive measure, will not only improve cosmetic results but will hopefully also decrease both abandonment and refusal rates as parents are assured that children with enucleations can look normal. Parent groups can also provide emotional and educational support for new families. Improved data collection, including cost analysis, is necessary to develop cost-effective programs.
Technology, including case consultations on the Cure4Kids and ORBIS Web sites as well as development of training modules for RetCam, can provide both expert advice on individual cases and cost-effective education in low-income countries. Although our informal experience has shown that online education is cheaper than traveling in person to provide education, no formal comparative analysis has been done regarding this. The online case consultations have helped decrease morbidity by salvaging eyes that previously could be treated only by enucleation The ultimate goal of the program is to develop self-sufficient programs capable of giving care equivalent to that in developed countries. Such care consists of at least 90% survival, good cosmetic results, and salvage of vision when possible through local measures and brachytherapy . Although the system is still fragile, several components of sustainability are in place including equipment, trained physicians, data collection methods, foundation, and government support. Continuing to increase awareness by training ophthalmologists, promoting early diagnosis of disease, and receiving expert advice via real-time online networking will be essential to guarantee the long-term success of the initiative in CA. Purchase of contracts for maintaining equipment in the 3 countries has been added to the budget of the twinning program. Data collection is crucial for sustainability of outcome measures and demonstrating success or failure to donors and the government.
Another benefit of the program is the possibility of local ophthalmologists collaborating with investigators at St. Jude and other groups to analyze fresh retinoblastoma tissue samples and designing treatment protocols for patients with advanced disease, neither of which can be easily accomplished in developing countries.
Developing the program has been a learning experience. Initially, it was difficult to arrange to process donations of equipment, particularly the RetCam, through customs, to find appropriate locations to house the equipment and to keep it functioning. These problems were solved through help from the local foundations and technical support from Clarity Medical Systems. As is known to occur with other Internet exchanges, initial face-to-face meetings between participants facilitated online interactions. Early diagnosis campaigns have progressed more slowly in Guatemala compared to Honduras, but discussions regarding various approaches among pediatric oncologists and ophthalmologists have resulted in modifying successful programs for local conditions.
We used the pediatric oncology center as the focal point for program development, but progress was possible only because of the commitment of ophthalmologists. Similar programs to improve outcomes of retinoblastoma and brain tumors or other diseases can be developed by collaborative efforts of multidisciplinary teams using the strongest, most developed specialty as a focal point.