Retinoblastoma control and management

0
76


World Retinoblastoma Week is an opportunity to raise global awareness about this cancer that affects children’s eyes. During this year’s World Retinoblastoma Week from May 10-16, we focus on how important this message is globally.

By spreading awareness about this disease we hope that globally more people will know how to watch for the sign that could potentially be an indicator of Retinoblastoma. The white glow in the eye, also known as leukocoria, is yellow-white reflections in the pupil of a child’s eye that can show up in photos taken with a flash and could be a sign retinoblastoma. Due to lack of awareness about retinoblastoma in the country, many children are losing their eyesight and in some cases, even lives.

People in the rural areas ignore minor eye problems seen in their children as they do not have knowledge about the disease. By the time they bring children to an ophthalmologist, it’s too late. So in order to control such deaths, it is necessary to make people aware about the disease and inform them about the treatment procedure. It is equally important to make people aware about complications like loss of the eye ball or death that the disease can bring in the patient.

Retinoblastoma (RB) is the most common eye cancer in children, affecting approximately 5,000 of them each year, and India has the highest number of retinoblastoma -affected children in the world, with about 1,500 new cases each year reported. It is estimated that about 60-70 new cases are reported in Nepal every year. From the public health prospective, this is not a big number, and investing in this disease will be high cost and a low catch, but we should not forget that this could be a life-threatening cancer if not addressed in time.

In developed countries like the United States, the survival rate reaches beyond an astounding 96 per cent, with early diagnosis and treatment being key to saving a patient’s life and sight. However, this incidence rate is higher in the developing countries, where most of the children succumb to metastatic retinoblastoma. In areas where children and families have no means of travel to a treatment centre far away from them, these afflicted children often endure their disease untreated until there is very little hope for them left.

Retinoblastoma is a fast growing eye cancer affecting babies and young children.  Early diagnosis is vital to saving children’s life and sight, but very young children cannot tell parents their sight is changing, and the signs of vision loss in very young children can be subtle. A white glow in a child’s eye, seen in flash photos or dim light, is the most common early sign of this cancer.  Some 90 per cent of the children are diagnosed because a parent sees this sign, but the time delay between first seeing the sign and seeking medical help is often several months or more.

In the developed world, 96 per cent of children are cured today, but many lose one or both eyes or suffer significant vision loss due to late diagnosis. Curative treatment often has lifelong physical and psychosocial impacts on young children. About 90 per  cent of children with eye cancer live in economically less developed countries, and most are diagnosed too late to save their lives. Global survival is below 20 per cent.

Awareness about retinoblastoma is low, and it is completely curable with life, eye and vision salvage, if detected early. With improved techniques and technologies, survival of children with retinoblastoma has improved, but low awareness is still a cause of concern. If the child is examined early by a child specialist or an eye doctor and referred to an eye cancer specialist for treatment, then the cancer can be controlled, and the child’s life, eye and vision can be saved.

Retinoblastoma represents 3 per cent of all childhood cancers, and it is fatal, if untreated. Retinoblastoma is the most common intraocular malignancy in children, with a reported incidence ranging from 1 in 15,000 to 1 in 18,000 live births. A white eye reflex, also known as leukocoria, is the commonest sign, followed by strabismus. Paediatricians have a very important role to play in the diagnosis of this relatively rare, but easily detectable tumor. Early diagnosis yields better results.

The management of retinoblastoma has gradually evolved over the past few decades, with an aim to not only preserve life and the eye, but also to optimise residual vision. The treatment of retinoblastoma is multimodal, with chemotherapy, focal treatment including trans-pupillary thermotherapy, cryotherapy and laser photocoagulation, radiation therapy and surgery, all playing a vital role.

Till a century ago, retinoblastoma was uniformly a fatal disease. Thanks to advances in surgical techniques, external beam radiation, focal treatment and chemotherapy, the survival rate and preservation of vision have greatly improved. Future holds promise for further advancement in focal therapy and targeted drug delivery.

Retinoblastoma may occur in one or both eyes. It begins in the retina, the layer of nerve cells lining the back of the eye. It happens when nerve cells in the retina change, grow in size and number. These cells usually spread in and around the eye. There are high chances of the disease spreading to other parts of the body, such as the brain and liver. The disease doesn’t have many symptoms in the initial stage.

A white colour occurs in the centre of the eye (pupil), eyes appear to be looking in different directions, and redness and swelling are some of the symptoms. However, these symptoms often go unnoticed as the child also doesn’t complain.

However, I would like to emphasise that screening of children for the white reflex should be taken up along the lines of a public campaign, and dilated fundus, inner part of the eye, screening for children should become a standard clinical practice. The impact of a child going blind is enormous as it corresponds to the loss of a number of man years of productivity.

A six-month-old Rati was brought to me by her mother in June 2000 with a history of a painless white pupil and protrusion of the left eye in my hospital, the BPKLCOS Teaching Hospital in Kathmandu, while I was doing my ophthalmology training there and conducting a study on this vision and life- threatening disease, retinoblastoma.

Rati’s delayed diagnosis and poor care in the Tarai health centre threatened her life. After treating her by removing the left eye and chemotherapy, she was cured of her cancer. A few months ago, after 20 years, she came to me for her regular eye checkup. I was proud to learn that she had become an established entrepreneur as a fashion designer.

Rati has taught us that many opportunities to save children’s lives are missed in Nepal and other developing regions of the world. Limited resources, poor awareness and poverty condemn 8 in 10 children to a slow and agonising death. Yet simple changes can prevent this curable cancer from becoming a terrible death sentence. Rati inspires us every day on our journey to develop high quality sustainable care that protects life and sight for every child, and care for their lifelong needs.

Several aspects of retinoblastoma in Nepal remain enigmatic. Although extremely rare in the general population, retinoblastoma remains the most common primary intraocular cancer expressed in the paediatric patients amongst all populations. However, the incidence of retinoblastoma varies significantly according to the socio-economic status and between developed and developing nations. Discrepancy between the presenting signs of retinoblastoma also exists between developing and developed nations.

In the Western countries, retinoblastoma is typically diagnosed early, and the white pupil is the presenting sign in 40-60 per cent of patients. In Nepal, retinoblastoma is often not diagnosed until the later stages, with protrusion of the cancer mass from the eye ball, comprising still a small proportion of the mode of presentation. Thus prognosis is often muchworse due to increased likelihood that the tumor has spread to other parts of the body.

In Nepal, the discrepancy between occurrences of unilateral versus bilateral retinoblastoma in the Hills and the Tarai is highly significant. Regarding the increased frequency of bilateral retinoblastoma in the Tarai region, several factors may be involved. When compared with every district of the Hills, excluding Kathmandu and Lalitpur, the districts of the Tarai region have a substantially higher proportion of foreign-born inhabitants.

As there are few terrain barriers separating the Tarai region from northern India, majority of these foreign inhabitants have probably migrated from India. Thus, Nepal’s natural landscape and high population density may have kept retinoblastoma carriers isolated in the Tarai region. The increased prevalence  of  bilateral retinoblastoma in the Tarai  region  may  be due  to  consanguineous marriages, inducing  genetic mutation in this disease  amongst  the  Muslim  parents,  who comprised a  higher proportion  of  the  patients with retinoblastoma in the Tarai.

During the World Retinoblastoma Awareness Week this year, efforts are dedicated to generating public awareness about retinoblastoma. But due to the COVID-19 crisis in the world, the event could not make it big. The retinoblastoma treating task force is comprised of a dedicated team of an ocular oncologist, paediatric oncologist, ophthalmic oncopathologist, expert chemotherapy nurse and an ocularist (prosthetic eye maker).

Over the past two decades, we have treated about 500 retinoblastoma eye cancers at the Oculoplasty and Ocular Oncology Department in Tilganga Eye Hospital. We intend to reach out to more people, increase the capacity and build new technologies and new research. The aim is to provide the best possible holistic clinical care and cutting edge treatment to the retinoblastoma patients.

Dr Saiju is Professor of Ophthalmology (NAMS) and director of Tilganga Institute of Ophthalmology



Follow The Himalayan Times on
Twitter
and
Facebook





Source link

LEAVE A REPLY

Please enter your comment!
Please enter your name here