Retinoblastoma is a malignant tumour of the eye originating from the retina which is the light sensitive lining of the eye.
Retinoblastoma affects approximately 40-50 children per year in the United Kingdom. About 6000 children will develop retinoblastoma each year throughout the world. It generally, occurs in children under the age of 5.
Retinoblastoma may affect one eye, this is called unilateral retinoblastoma.
If both eyes are affected, it is called bilateral retinoblastoma.
How did my child get it?
Retinoblastoma, like many cancers is not caused by external factors such as smoking or drinking.
The genetic basis of retinoblastoma is now well understood, arising as either heritable or sporadic (non-heritable) form. Chromosomes contain the genetic codes that control the way cells grow and develop. If a part of the chromosome is missing or altered in some way, abnormalities or cancers may occur.
Approximately 40% of children with Retinoblastoma have the heritable form of the disease, of these about 10 –15% will have a family history of retinoblastoma. The remainder of the heritable form are new “germ-line”, mutations of the retinoblastoma susceptibility gene in the affected child.
With the heritable form of Retinoblastoma there is a 1 in 2 chance that the sufferer’s own children will inherit the mutation and will also have a high risk of developing Retinoblastoma.
The risk of having the heritable form is much lower for unilateral cases. Only about 1 in 20 of the offspring of adults who had unilateral retinoblastoma will have a retinoblastoma. In known families where retinoblastoma has previously been diagnosed it is important that regular eye checks are considered for close relatives in childhood. For many children, especially siblings and offspring, it will be necessary that examinations start at birth so that if tumours occur they can be identified at an early stage and treated.
Will my child die, is it curable?
Retinoblastoma is one of the most curable childhood cancers, with over 95% of affected children in the U.K being cured.
How can you confirm that it is retinoblastoma?
An Ophthalmologist will have a look at the back of your child’s eyes, usually whilst they are asleep. This is known as an examination under anaesthetic of the eyes. The Ophthalmologist will be able to diagnose the condition at this procedure.
Will my child lose their sight?
The amount of vision affected by retinoblastoma is individual to each child.
Some children will only have one eye affected, whilst others have tumours in both eyes. The size and position of the tumours will affect how much vision is affected. Some treatments may also affect the degree of vision retained by your child.
Can you remove the tumour from the eye?
As retinoblastoma is a malignant tumour, it is not possible to remove the tumour from the eye.
What treatments are available?
There are a number of treatments available for retinoblastoma. The type of treatment given depends on a number of factors.
These include:
- Whether the tumour affects one eye or both eyes
- The size, position and spread of the tumour
- The potential for vision
Frequency and duration of treatment will vary from child to child.
The types of treatment that are available may be used either alone or in combination. The Ophthalmologist and the Oncologist will discuss the treatment options available for your child with you.
Types of treatment:
Laser or photocoagulation – the use of laser light to destroy blood supply to the tumour and the tumour itself.
Cryotherapy – the use of extreme cold to destroy tumour.
Chemotherapy – the use of drug treatment, given via the bloodsteam to kill cancer cells.
Enucleation – surgical removal of an eye.
Radiotherapy – the use of high energy radiations to destroy cancer cells. This treatment can be done in a number of ways.
External beam radiation – where radiation is given via a machine.
Plaque therapy – where radioactive material is placed onto the affected eye.
What about my other children?
All siblings and parents of children with retinoblastoma will have their eyes checked by an Ophthalmologist.
The frequency of checking the eyes of siblings will depend on their age.
All newborn siblings will be screened shortly after birth.
Who will help us cope?
When your child has been diagnosed with cancer the impact of the news and the treatment affects the whole family. There will be a lot of new information for you to know and understand. You and your child will be able to talk with several different specialists who will be able to offer you and members of your family support in a variety of ways. The specialists can be hospital or community based. They include play specialists, psychologists, social workers, orthoptists, geneticists, all of whom work alongside the doctors and nurses you meet.
A representative from CHECT, your parent contact and support group will be happy to meet you during your visit. You can contact the family support worker from CHECT (details at http://www.rbsociety.org.uk/about_us/whos_who.htm).
Where can I find more information?
The specialists looking after your child are the best people to answer your questions. However, there are several useful websites that provide background information, the best of which is :
The Childhood Eye Cancer Trust :
http://www.chect.org.uk/about_rb/understanding_rb.htm
How can I contact other families with retinoblastoma?
The team caring for your child will be able to put you in touch with other families that are happy to be contacted by you; the charitable support organizations for retinoblastoma can do this also.
There are two North American Web-based discussion groups for families. Registration is required:
http://listserv.acor.org/archives/r-blastoma.html
http://listserv.acor.org/archives/rb-survivors.html
Author: Sister M McCalla on behalf of the Retinoblastoma Team, Birmingham children’s Hospital.
Sources.
Retinoblastoma Team, Birmingham Children’s Hospital.
Childhood Eye Cancer Trust, London.
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