There is a lot more that needs to be done to improve outcomes of rare cancers. Diagnostic accuracy, better treatment and prognosis are the need of the hour
The Indian healthcare system has markedly improved in the last few years. However, one of the areas where it lacks is judicious treatment of rare cancers. The problem with this disease is multi-faceted and has many aspects to it. The conundrum in India begins with the lack of a correct definition for it. Then comes other issues such as difficulty in diagnosis, absence of registries, dearth of experts and the lack of data and newer drugs, approved as orphan drug elsewhere. All of these factors lead to poor outcomes. This disease may be rare individually, but collectively, the varied manifestations constitute almost 20 per cent of all cancers. Thus, they cannot be ignored.
There is no universally adopted definition for rare cancers. The National Cancer Institute, the US, definition is fewer than 15 cases per 100,000 people per year. A consortium from the European Union defined rare cancers as those with fewer than six cases per 100,000 people per year. Rare cancer definition or subtypes can’t be copied from any country. To cite an example, though melanoma may be a very rare cancer in our country, in Australia and the US, it is a common cancer.
Similarly, there are a few expert centres for diagnosis and treatment of rare diseases like sarcoma, thyroid cancer, melanoma and neuroendocrine tumors. There are many diagnostic investigations that are routinely done in many countries but are not available in India as the focus on rare cancers is less. Apart from investigations, even first line drugs for diseases like melanoma and thyroid cancer are not available in India. This leads to severe frustration among doctors and patients. The only option left for patients is to import drugs, which is not a viable option for many. Often, the situation forces them to buy drugs from grey market, the veracity of which is highly questionable. Further, research for rare cancers in our country is slow and a majority of data has come from the developed countries. Reason for this is a lack of funding for research. There is less incentive for working on rare cancers and this leads to apathy among researchers.
From a patient’s perspective, there are no specific guidelines for expert centres and a majority of them land up having inadequate or inadvertent treatment in non-expert centers. This leads to poor outcomes and psychological turmoil. Support groups for them, too, are very few and this leads to a feeling of loneliness during the arduous journey of their treatment. Lack of guidelines for this disease also leads to difference in opinions from various centres, which leaves the patients baffled. Similarly, insurance cover for this disease poses tremendous problems as patients undergo a hard time in getting the insurance cover for these lesser-known diseases.
How can we improve the outcomes? First and foremost, it is of utmost importance to address the above-mentioned shortcomings in a systematic manner. Collaboration and data collection/registry for all cancers with equal emphasis on rare cancers (which is not the case currently in India) should be the second approach to kick-start the progress in the management of rare cancers. This will help us define the subset of rare cancers and develop a uniform definition. All tertiary care centres must publish their experience in rare cancers and the challenges faced by them in diagnosis and treatment.
We need to have a consortium/core body soon in order to channelise the efforts and streamline them. Collaboration must be widespread between NGOs, patients advocates, tertiary cancer institutes, medical colleges and peripheral centres with sound referral system. We need indigenous guidelines, keeping in mind the resources available for the diagnosis and treatment constraints in our country. Similarly, we must spend on patient education resources in vernacular languages in order to educate them and help them understand the intricacies of rare cancers. Research bodies must keep some funding secured for the treatment of rare cancers and should encourage further studies.
Training programmes must be held for doctors to enhance their skills so that pathologists, radiologists and pharmaceuticals are also encouraged to bring drugs in India. Subsequently, they should be given due benefits. Generic brands must get due importance. India should lead the research in rare cancers and should set an example for other countries like Nepal, Bangladesh, Pakistan as these countries have similar epidemiology for cancer.
(The writer is Assistant Professor, Medical Oncology Department, AIIMS, New Delhi)
