In 1993, at the age of two years, Nawal Khairil Anwar was diagnosed with optic glioma, a type of benign brain tumour that compressed her optic nerves.
She was treated at University Malaya Medical Centre (UMMC) in Kuala Lumpur with chemotherapy, which managed to shrink, but not completely eradicate the tumour.
Unfortunately, the tumour grew again three years later.
She underwent further treatment, which included radiotherapy.
This time, the treatment was successful in removing the tumour.
Sadly, Nawal did not come through this unscathed and was left blind.
At such a tender age, she realised she had to be a fighter and her family strongly encouraged her to pursue the things she wanted to achieve in life.
It was this very drive that made her learn to read Braille starting from the age of seven.
With the support of her family, she was able to adapt to her new life.
In the case of 12-year-old Chua Yong Sheng, it was discovered that he had a malignant brain tumour when he lost vision in both eyes in early 2016.
The diagnosis came as a shock, and left him and his family in absolute grief.
Over a five-month period, he underwent surgery and chemotherapy, followed by stem cell transplantation.
The treatment appeared successful and he regained his sight.
However, after two years, he developed a persistent headache and it was found that his brain tumour had recurred.
The news left the family utterly devastated as the prognosis for a recurrence of this tumour is very poor.
They feared that all hope was lost.
His consultant paediatric neuro-oncologist Dr Revathi Rajagopal started him on an intensive course of chemotherapy, followed by another stem cell transplantation.
The treatment, being considerably more intensive than the first, was so aggressive that it left him debilitated from painful mouth ulcers and persistent diarrhoea.
There were often times during treatment when he felt defeated and considered that he was better off dying.
Still, Yong Sheng fought on and defeated the cancer to make a remarkable recovery.
Brain or central nervous system tumours are the second most common childhood cancer after leukaemia.
No one actually knows what causes brain tumours in children, apart from rare cases where it is part of an inherited cancer predisposition syndrome.
Brain tumours may be primary, where it arises from the brain itself, or secondary, when a cancer starts at another part of the body and spreads to the brain.
There are many types of brain tumours, with specific histological (microscopic anatomical) subtypes.
Brain tumours can look, behave and respond differently to treatment, which is why it is critical to correctly identify the actual type of tumour.
Broadly speaking, there are two groups of primary brain tumours.
They may be benign, where they are slow-growing and do not usually spread to other areas, or malignant, where they are fast-growing and invade the surrounding tissue.
However, despite the name, it would be deceptive to think that a benign tumour is harmless.
The presence of any mass within the delicate structure of the brain can cause its own problems, as experienced by Nawal when her benign optic glioma compressed the visual pathway in her brain and left her blind.
Larger masses can cause an increase in pressure within the head (raised intracranial pressure) by virtue of its size or by obstructing the fluid surrounding the brain (cerebrospinal fluid).
Symptoms of a brain tumour can vary from headaches and vomiting to change of personality, loss of balance, seizures or loss of sight, as with Nawal and Yong Sheng.
In babies, the symptoms can be fairly non-specific, with them possibly presenting with irritability, an abnormally rapid increase in head circumference or bulging of their “soft spot” (i.e. the fontanelle).
Every year in Malaysia, about 200 children and their families are affected by this condition.
For them and their families, their hope lies in the treatment that medical science can offer.
In medical centres and laboratories around the world, including in UMMC, countless groups work on clinical trials and research to continually find new advances in the treatment for childhood cancers.
The choice of treatment, as mentioned before, is dependent on the type of tumour, as well as its size and the location within the brain.
The types of treatment are surgery, radiotherapy and chemotherapy.
Patients may receive one type of treatment or a combination of the various treatments.
Occasionally, surgical removal of a brain tumour is not always feasible due to the delicate nature of brain structures and challenges related to access to the tumour.
The immediate aim of all therapies is to cure children of their cancer.
In the 20 years that spanned between Nawal’s and Yong Sheng’s cases, the treatment of childhood cancer has radically changed.
Therapies are designed to be smarter so that they are more effective and safer.
As a result, there are now more childhood cancer survivors.
Unfortunately, while these treatments can be successful in achieving a cure, it is increasingly being recognised that survivors of childhood cancer suffer a multitude of long-term problems caused either by the tumour itself or as a result of side effects from either the chemotherapy or radiotherapy.
These long-term side effects include hormonal problems with short stature and delayed puberty, cognitive impairment and learning difficulties.
These children need to be supported and protected so that they have minimal long-term effects from both the disease and its treatment, and be able to lead fulfilling lives.
UMMC consultant paediatric oncologist and paediatric oncology and transplant service head Professor Dr Hany Ariffin is particularly concerned about the long-term effects of cancer therapy on survivors of childhood cancer.
She explains, “Key to the development of better therapies would be deeper understanding of tumour biology.
“This will enable doctors to offer more personalised therapy with increased precision.”
A lot of research is being done with regards to identifying targetable sites, i.e. the so-called Achilles heel of certain tumours, and this has led to the development of new drugs that only kill tumour cells while leaving other cells unaffected.
“As an example, a subgroup of the type of tumour that Nawal suffered from, may carry a unique mutation in the BRAF oncogene, which is sensitive to a specific inhibitor drug that is not toxic to normal cells.
“Additionally, the patient is spared from chemotherapy or radiotherapy,” she says.
At the same time, radiotherapy techniques have also improved, with increased precision to help prevent radioactive “spray” from damaging normal tissue adjacent to the tumour.
A multi-disciplinary approach represents the standard of care for patients and survivors of brain cancer.
In addition to the medical team, allied health professionals all play key roles in assisting patients and their families regain function and independence in daily activities, and cope with the physical, mental and emotional changes the condition has caused.
Currently, patients are regularly monitored for continued remission and early recognition of treatment-related health effects, as well as academic performance.
Special schools are beneficial for patients with specific impairments, e.g. sight-impaired, or vocational schools to match a patient’s level of interest and ability, so that children who survive brain tumours have the best chance of realising their potential.
Perseverance and hope
Today, Nawal, 29, is a Braille teacher. She hopes to teach English too in the future, which is her main passion.
She also loves baking pastries and has taken up classes to enhance her pastry-making skills.
Nawal is a firm believer that one should always plan for the future, regardless of whatever cards life may have dealt you.
She also believes that one can do anything if you set your mind to it.
She advises others going through similar hardships as she did that they should not lose hope and to find strength from the support of family and friends.
Nawal also stresses the importance of public support for childhood cancer research to help improve current treatments for children in the future.
Meanwhile, Yong Sheng is now 16 and happy to be back at school with his friends.
Even though he can still recall the many times he felt like completely giving up, he is proud that he battled his brain tumour until the end.
He says: “Despite the massive odds, I was given a second chance to survive.
“I kept on fighting and now I want to achieve all that life has to offer.”
He shares that even through our darkest moments, we should have faith that there is light at the end of the tunnel.
There is always hope.
Dr Syaza Zafirah Ab Rahman is a paediatric oncologist and Dr Diyana Sofea Nasir is the clinical trial coordinator of UMMC’s Children’s Cancer Unit. For more information, email email@example.com. The information provided is for educational purposes only and should not be considered as medical advice. The Star does not give any warranty on accuracy, completeness, functionality, usefulness or other assurances as to the content appearing in this column. The Star disclaims all responsibility for any losses, damage to property or personal injury suffered directly or indirectly from reliance on such information.