Keisha’s world
Dealing with the varied symptoms caused by the tumours of neurofibromatosis type 2 is a neverending battle.
In conjunction with World NF Awareness Day last Thursday, we share the story of neurofibromatosis patient Keisha Somasundram Petrus.
TUMOURS are not always cancerous, or malignant. Those tumours that do not invade the surrounding tissues or spread to other parts of the body are commonly classified as benign or non-cancerous.
But these so-called benign tumours do not necessarily always have non-malignant consequences. Take neurofibromatosis (NF) for example.
This genetic disorder causes multiple benign tumours to grow in the nervous system.
Although they grow quite slowly, once they are large enough, they can wreak all sorts of havoc by compressing and choking off those vital pathways of information and communication within our body.
Treatment is not always easy either, particularly because our nervous system is so intricate and delicate.
One wrong twitch of the scalpel during surgery, or an inch too broad a field of radiation during radiotherapy, and the consequences could be the same as, or worse than, leaving the tumour alone.
Diagnosing NF
Twenty-two-year-old Keisha Somasundram Petrus is all too familiar with the effects of having benign tumours randomly grow on her nerves and spinal cord.
Her medical journey started when she was four.
Mother, Veena Petrus, 50, shares that when Keisha was an infant, the paediatrician discovered something wrong with her right eye.
“He referred her to an ophthalmologist, who couldn’t diagnose it, so we were referred to a doctor in Singapore, who diagnosed it as a naevus.”
A naevus is a defined malformation of the skin, which is usually benign, although it can turn malignant in certain cases.
After following up in Singapore for a couple of years, the family heard of paediatric ophthalmologist Datuk Dr Bajit @ Baljit Kaur.
“She disagreed with the (naevus) diagnosis,” says Veena. “Her mentor was here from England, giving a talk, and he said (after examining Keisha), just to rule out NF, do a CT scan and MRI.”
The scans revealed that Keisha had bilateral acoustic neuromas.
These tumours that grow on the acoustic or hearing part of the eighth cranial nerve, are diagnostic of neurofibromatosis type 2 (NF2).
Says Veena: “We had to take it from there as to what to do after that.”
Keisha found out years later, that the apparent naevus was actually a reticular haematoma, which is a common finding in NF2 patients.
Although Keisha did not have any symptoms then, her uncle – a paediatric neuroradiologist in California, United States – was concerned that the tumours would eventually affect her hearing.
He helped arrange a surgery to remove the left acoustic neuroma, which was the larger one, at the House Ear Institute in Los Angeles when she was nine.
Keisha shares: “The surgery was a success as the doctors managed to remove the tumour completely.
“However, my hearing nerve was compromised, and as a result, I was left completely deaf in my left ear, which persists to this day.
“In addition, I have (left-sided) facial paralysis as a result of the surgery.”
Then, when she was 11, Keisha started falling down frequently, and her left hand and leg became noticeably weaker.
Upon undergoing an MRI, a 12cm tumour was discovered on her cervical spine (at the neck).
“I was admitted and had emergency surgery to remove the tumour. I skipped school for about six months and that was (my) UPSR year.”
Although she eventually regained her ability to walk with the aid of physiotherapy and rehabilitation, she started experiencing intense pain in her neck.
The pain was initially dismissed by her neurosurgeon as her “being stiff”, says Keisha.
Then, she started falling down again, with her left hand again becoming weak.
“I actually fell down a flight of stairs in school because I blacked out from the pain. I also had problems lifting and turning my head, even until now,” she shares.
After undergoing an MRI, it was discovered that she had developed kyphosis – a separate problem from NF.
Unfortunately, no doctor she consulted was confident enough to surgically correct the problem, as she had too many lesions on her spine from NF.
“My scans were sent to many local and international surgeons who all came to the consensus that any surgery would, in fact, be extremely risky, and they decided that since I was walking fine, and since I had considerable body strength, it was decided that I should just soldier on.”
She learnt to live with the pain, and when it was really unbearable, she would lie down and take a painkiller.
The intensity of the pain also reduced over the years, and she later found out that this was due to the bones having fused due to the kyphosis.
Additional problems
Towards the end of the following year, when she was 14, Keisha started experiencing chronic headaches, followed by vision problems.
“My vision became blurry. It would go pitch black, and then come back,” she says.
An MRI revealed that a tumour along the trigeminal or fifth cranial nerve had grown enough to compress it and cause the symptoms.
By this point, Veena shares that friends had suggested they look at medical options in India.
Doctors at the Apollo Hospital in Chennai decided that stereotactic radiosurgery – a form of radiation therapy that focuses high-powered X-rays on a small area of the body – was the best option available.
“A few months later, the symptoms disappeared. I went back to India, did an MRI and found that the tumour had shrunk,” says Keisha.
Two years passed without inci-