Surgical Relief - By S.S. Yoga
HAVING a 90kg tumour wrapped like a boa constrictor around your stomach and back seems like a horrible nightmare. For Lori Hoogewind, a mother of two from Michigan, this was true life. She was diagnosed with neurofibromatosis (NF) in May of 1999 and by December, the tumour had grown to that size.
According to doctors then, it was probably the biggest tumour they had ever seen for that condition. It took a serious of highly dangerous operations to remove the tumour; she would have lost her life otherwise as the tumour was leaching her blood and food supply.
Most doctors here in Malaysia do not want to undertake any dangerous procedures to remove the tumours for cosmetic reasons. Plastic surgeon Dr Alizan Abdul Khalil says some patients with extensive facial/neck tumours or lesions would seek advice to remove them.
NF is a genetic disorder of the nervous system which causes tumours (or neurofibromas) to form on the nerves anywhere in the body. It affects all ethnic groups and both genders equally. It is an inherited disease and is not contagious. It is estimated that 50% of the cases are the result of spontaneous genetic mutation.
There is no known cure though the reason for it has been identified. A gene that produces a protein called neurofibromin undergoes mutation. This results in too little or zero production of neurofibromin. This protein actually acts like a kind of brake on cell division and if the “brake” is faulty, it means the cells will keep on multiplying. Hence the tumour keeps on growing.
There are two types of NF. NF1 occurs as cafe-au-lait spots and bumps (six or more is a danger sign) under the skin or even nodules that protrude above (many of which require no treatment), or freckles in the armpits and groin, while for others it can be bone deformities, or even blindness.
NF2 involves tumours in the brain, spine and peripheral nerves and can lead to facial paralysis, deafness, partial blindness, and poor balance.
Both Hong Siew Hui and Tan Kia Khim have NF1.
Trials and research are being done to look at drugs to control the cellular growth and check if cancer treatments can be used.
Source: Children’s Tumor Foundation (www.ctf.org) and Neurofibromatosis Inc (www.nfinc.org). - The Star
Monday September 7, 2009
A Life They Can Face - By S.S. YOGA and G.C. TAN
They are a model of courage, spunk and tenacity as they take adversity in their stride.
ONCE in a while, we come across an individual who reminds us of how grateful we should be for what we have. Hong Siew Hui and Tan Kia Khim are two such individuals. Despite living with a disfigurement for a good part of their lives, the spirited duo display a tenacity and strength of character that will take them far.
Hong and Tan have a condition called neurofibromatosis (NF), in which large tumours grow on the face. Most people associate NF with Joseph "Elephant Man" Merrick, an Englishman born in 1862. Merrick was treated as a freak most of his life and ended up as a sideshow attraction. He was befriended by a surgeon and became a permanent resident of London’s Whitechapel Hospital. He died at age 27.
When we met up with Tan, 28, at his home in a rented unit above a shophouse in Kampung Berjaya, Alor Setar, he had just woken up and was still drowsy. But pretty soon he was all smiles and opened up easily.
However, he remembered the bullying he endured in primary school. “They hit me with a ruler, pulled my hair, shot at me with rubber bands. They called me char siew pau (barbecued pork dumpling) and ghost.”
Children can be cruel and the taunts and bullying continued into secondary school.
“But there were a lot of students who were my friends and all of my teachers were nice to me, except one, who asked me what I would do after SPM since I didn’t have a brain and couldn’t think properly,” recalled Tan, whose parents run an “economy rice” stall at a coffeeshop.
Hong, 21, who also hails from Alor Setar, was more stoic in approach when we spoke to her at her single-storey terrace family home in Taman Bersatu, Simpang Empat, Kedah.
“In school there were a lot of naughty pupils who passed all kinds of remarks. I just ignored them. There’s no need to cry over it; it won’t solve any problems. It’s not like you can change anything.”
The year 2006 marked a turning point in their lives. Both were given the chance to have their tumours removed. Hong had a growth of about 3kg covering most of the left side of her face, including the left eye, most of the nose and mouth.
Tan’s tumour was relatively smaller, starting from the left eye, disfiguring the nose and pulling down the mouth.
Hong first attracted media attention after her SPM results came out in 2006. “Actually, my SPM results were not really fantastic, but they exceeded my expectations,” she said. She scored 1A in Mathematics, her favourite subject, a 3B, two 4Bs and a 5C.
One of the newspaper reports which featured Hong was sent to Prof Xu Ke Cheng, president of Fuda Cancer Hospital in Guangzhou, China, which has carried out many successful procedures on NF patients.
Prof Xu initiated contact and flew down in April, 2006, to assess her condition.
Prior to the visit, her parents, padi farmer Hong Wah and home-maker Goh Jit Yang were reluctant for her to undergo any operation.
“Doctors in Alor Setar and Penang had told us that the condition could not be treated or the operation would be highly risky, so why take the risk?” said Hong in an earlier interview.
Tan, too, concurred that doctors in Penang and Alor Setar had discouraged him from removing his tumour.
After speaking with Prof Xu, Hong and her family changed their minds and agreed to go ahead with the procedure to remove the tumour. At the press conference to announce this, a member of the press who knew of Tan’s case brought him along. He was excited to meet someone who had the same condition as him. As it turned out, Prof Xu had a quick look at Tan and said his tumour was more easily operable.
Tan had no hesitation about going through with the operation. “Why should I turn down the chance to look handsome?” he quipped, flashing a beaming smile.
Meanwhile, the Kedah Chinese Assembly Hall (KCAH) had initiated a donation campaign with the co-operation of the Chinese Press (and others like The Star) for the duo to be sent to Fuda. The collection raked in close to RM360,000 and in May 2006, Hong and Tan were flown to Guangzhou. The hospital had waived many of the charges and provided free room and board for the entourage. Tan was accompanied by his father while Hong’s mother kept her company.
In June of that year, both of them underwent surgery – Hong first followed by Tan the next day. Tan’s operation was done in two hours, while Hong’s was spread out over several months as her case was more complicated.
A team of 14 surgeons worked on her. It was apparently the biggest and most challenging operation in the history of the hospital.
One of the procedures undertaken was cryosurgery, a technique where extreme cold (below -160°C) was used to destroy tumours.
After the tumours were removed, both had to undergo plastic surgery to reconstruct parts of their faces.
Tan returned home in mid-July that year, while Hong stayed back for more follow-ups. Tan returned to Fuda in November for cosmetic surgery and both eventually flew home on Dec 12. In March 2007, both went back to the hospital for more follow-ups.
Hong’s tumour started out with a pretty big bump on her face, and grew over the years. At the age of six, she had some semblance of sight in her left eye which was not enveloped by the growth yet.
“Now I’m so glad that I don’t have to carry all that weight around. Before I could not even use a crash helmet but now I can. Oh, and I don’t have to wear loose T-shirts now,” she says with a big grin on her face.
Her mother seems to display symptoms of someone who has NF as she has small bumps and cafe-au-lait spots all over her face and body. Her father has misshapened legs, which are sometimes a sign of the condition.
As for Tan, his father has cafe-au-lait spots and a few bumps so chances are he has the condition, too. His mother and three younger siblings do not have any sign of the condition. Tan’s tumour developed when he was still a baby.
Hong now spends most of her time on her studies. She is a diploma student at Politeknik Sultan Abdul Halim (Polimas) in Jitra, and expects to complete her three-year accountancy course early next year.
“I have had no problems there. Everyone is my friend. In fact, I’ve just finished doing my practical at (KCAH president) Mr Cheng Lai Hock’s accounting office. It was good training for me,” she beamed.
Some people have the knack of making friends and Tan is one of them. He proved so popular with the hospital staff and media in Guangzhou that they flew him back last year for press interviews that even took him to Beijing.
The gregarious and genial young man spends his free time hanging out with friends.
The future is looking bright for Tan and Hong as they look forward to Prof Xu’s visit to Alor Setar this Thursday to check on their progress. - The Star