Living With Retinoblastoma ~ Part I

posters-sign-to-look-english-2Retinoblastoma is a fast growing eye-cancer which affects babies and young children. Tumours may form in one eye (unilateral) or both eyes (bilateral), and can spread outside the eye (extraocular retinoblastoma). Rarely, a separate tumour develops in the brain (trilateral retinoblastoma). This cancer typically develops before 3 years of age, and some children are born with tumours.

Retinoblastoma affects about 1 in 15, 000 live births, and an estimated 9,000 children develop the cancer globally each year.  Due to global population distribution, 90% of children with retinoblastoma live in economically developing countries.

The most common early sign of retinoblastoma is leukocoria, a white glow in the pupil (black circle) of the affected eye, seen in flash photographs. This reflection may be visible to the naked eye in dim light when cancer fills the eye, or in very young babies. Other signs include a squint (misaligned eyes), red, painful or swollen eye. Trilateral retinoblastoma causes a range of symptoms, depending on its location in the brain.

Treatment depends on the number, size and location of tumour(s), whether one or both eyes is involved, and whether cancer has escaped the eye or is at risk of spreading. Without early treatment, retinoblastoma quickly spreads to the brain or bone marrow.

Treatments include laser, cryotherapy (freezing), chemotherapy, brachytherapy (radioactive plaque), external beam radiotherapy and enucleation (surgical removal of the eye).

Prompt removal of the eye is standard treatment when only one eye is affected with advanced cancer, and is essential when there is a risk of cancer escaping the eye.

You can read more about this type of cancer on our Retinoblastoma Page


Living with Retinoblastoma

More than 96% of children treated in developed countries survive, but many children lose at least one eye. Even when eyes are saved, most children have severe vision loss.

Most children with retinoblastoma in the developed world are cured today. However, even after cure, the ghost of cancer walks with many survivors throughout life. Emotions, impaired vision and other effects of treatment, life-long second cancer risk and adapting to meet the challenges of the world can be hard. But life after retinoblastoma can also be richly blessed and full of living, with knowledge, understanding and the right support.

Believing in a better tomorrow is vital throughout the cancer journey. Hope is not a way around cancer, but a way through it, motivating, encouraging and sustaining us in difficult times.

Cancer treatment is a stressful and potentially traumatic experience that can overwhelm a child’s natural ability to cope.  This can delay healing and normal development, with lasting negative effects on their physical and mental health.


Pain Management

During a child’s cancer journey, their perception and tolerance of pain will grow and change.

After repeated procedures, many children become less able to tolerate even a minimally invasive or mildly painful experience. This is often because they are anticipating the pain, however mild, before the procedure. Anticipation causes stress, worry and anxiety, amplifying the child’s reaction to even mild pain.

The body and brain communicate to determine if touch or bodily sensations are interpreted as pain. We are able to help children change their experience of pain by interfering with the way the brain interprets sensations.

This can be done in two ways: pharmacological (giving medication) and non-pharmacological (psychological) strategies. You, your child and your care team need to work together to find the best combination of methods for your child.

Sometimes there is nothing you can do to prevent pain. For example, after an operation or during a painful procedure. You can, however, address factors that are often part of the pain, or make it worse.

Reducing fear and anxiety, talking with your child to clearly understand what they think and feel, relaxation and distraction all help to change your child’s experience of pain.


Living With a Special Eye

If the tumour is very large and has damaged the sight in the eye beyond repair, then the ophthalmologist will recommend that the eye be removed. This operation is called an enucleation. Enucleation is only recommended when it is felt other treatments would not be effective, and/or would put the child’s life at risk.

Approximately 70% of children with unilateral Rb will require enucleation.

Children recover very rapidly following this surgery. There are no stitches to be removed after surgery and  your child will be kept comfortable with regular paracetamol. Other painkillers can be given if necessary.

When an eye is removed, the socket must heal before a new artificial eye can be fitted 4-10 weeks later. There are many ways you can support your child through surgery and initial healing.

Children face a number of different challenges when they have an eye removed. These can range from embarrassment about their appearance or protest when someone tries to clean their special eye, to extreme fear, shame and non-compliance. These are all natural responses.

To reduce fear and anxiety, children need to be able to participate in their experiences.  There are many ways you can support and empower your child to live well with their special eye:

1. Seek Support

Many parents find this time before enucleation overwhelming. Firstly, receiving the news that your child has cancer, and then being informed that their eye needs to be removed as soon as possible can be very difficult to process.

It is important that you seek out support and assistance in coping with this news, from your partner, other family members, your child’s medical team or other parents who have been through the process and understand your feelings.

2. Talk you Your Child

You may feel that explaining to a very young child what will be happening may seem inappropriate since they may not fully understand, but it is important that you give your child at least some information about the steps and stages of going in for surgery to have the eye removed.

Use child-friendly language, words, actions, play and touch to enhance your child’s understanding and support their coping through these steps. Use language appropriate to your child and be consistent with the language that you use, such as saying “your eye has a boo-boo” or “your eye is sick” and “the doctor is going to remove your eye to fix the boo-boo.”

Stick to whatever type of language your family uses for when someone gets hurt and then “fixed” and repeat the same words as often as is necessary so that your child understands.

Some young children fear mutilation, so be clear that the doctor is not going to do anything but remove the sick eye, and put a large bandage over the area where the sick eye was. Stress that no other part of their face will be touched or affected by removal of their sick eye.

3. Ophthalmic Visits

Psychologically, both children and adults are unaccustomed to somebody putting things into our eye sockets. We spend our whole life protecting the eye in the socket, and the mind and body is geared to reject anything in the socket. Your child’s greatest fear therefore, will be that they will experience severe pain, so you need to put them at ease.

The procedure is actually not painful, although your child will feel some very strange, unfamiliar sensations in the process of making and fitting the new eye. Explain to them that it will not be sore, maybe just a bit uncomfortable.

The best way to explain what is going to happen is to tell your child that “the doctor will use some special squishy clay/play dough to make a mould of inside your eye socket. It may feel a bit funny but will not take too long and Mom/Dad will be there with you all the time”

Some children need support to feel and experience each aspect of the procedure. Ask questions such as “does the squishy stuff feel cool or warm?” or “I can see this feels funny for you, can you tell me about it?

Explain each step to your child, and the reasons for it. For example, “the doctor is fixing the shape to make sure it is comfortable and your eye can open and close well”.

Focusing on the procedure helps your child define and compartmentalise the sensation, and confirms that he is being listened to.


Making an Artificial Eye

The process of custom making an artificial eye for an individual child involves several stages and is done by an ocularist (someone who specialises in the fabrication and fitting of ocular prostheses for people who have lost an eye or eyes due to trauma or illness.)

Sometimes the mould is made during the enucleation, while the child is asleep, but making it when the child is awake often has a better cosmetic result.


Making the Mould

Making the Mould is the most difficult step psychologically, because the sensations are so unfamiliar, but the entire process only takes about five minutes.

eye_making_process-519x1030A special “plunger” that looks like a suction-cup arrow found on children’s toys is placed into the child’s eye socket. This will not hurt, but your child may feel a strange but painless sensation from the eyelid resting on the portion that sticks out.

A child will get used to the sensation within a few seconds if supported by a parent, basically as soon as he or she realises the plunger doesn’t hurt, it just feels strange.


Making the Impression

The mould is used to create a wax impression similar to the cavity in your child’s eye socket and placed into the socket and the ocularist will ask your child where he can feel any pressure – he will then remove the impression, make it smoother, and reinsert it.

This may need to be done a few times until the wax impression is the right shape, and the eyelids rests on it comfortably when the eye is open, and close properly when blinking. The wax will also be shaped to fill out certain areas around the eye socket so the eye has a natural look.

Once the wax impression is properly fitted, a plain white plastic insert the same shape as the wax will be made (this should take about an hour). Once again this will need to be inserted and removed from your child’s eye socket several times until the fit is perfect.


Painting the Eye

Once everyone is satisfied with the insert, the ocularist will draw on the insert how the eye should be aligned. He will then remove the insert to begin making the “eye” by drilling a hole in the plastic and inserting the coloured area which will be as close to your child’s other eye-colour as possible.

Continued in Part II



About LFCT

CHILDHOOD CANCER and CHILDHOOD CANCER AWARENESS Little Fighters Cancer Trust is a non-profit Childhood Cancer support organisation that offers support and aid to Children with Cancer and their families. When a child is diagnosed with cancer it affects the whole family. One of the parents, usually the mother, must give up their job to care for the child and this creates financial problems for the family. In South Africa especially the majority of these families are not well-to-do; many of them are rural. A diagnosis of cancer can wipe out any family’s finances, let alone a poor family. The costs of special medications, special diets, hospital stays, transport to and from the hospital or clinic and accommodation and food costs for the mother who spends most of the time at her child’s bedside are astronomical. These are the people and problems that fall through the cracks, and these are the people that Little Fighters Cancer Trust has pledged to help in any way possible. LFCT takes a holistic approach to assisting the Children with Cancer and their Families, with the main aim to be the preservation of individual dignity and pride. Little Fighters Cancer Trust also focuses on promotion and advocacy of National Childhood Cancer Awareness in an effort to increase awareness of Early Warning Signs of Childhood Cancer. This would result in earlier diagnosis, giving the Child with Cancer more of a chance at Treatment and Survival. See "About" for more Background info

Posted on 11 October, 2016, in Advice & Tips, Blog and tagged , , , , , , , , , , , , . Bookmark the permalink. Leave a comment.

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