Living life to the fullest

Chan, an avid mountaineer and myelofibrosis patient, with a photo of himself (in red jacket) and fellow climbers at the summit of Mount Kinabalu. Photo: UU BAN/The Star >>

Despite having a rare blood disorder, Tan Sri Chan Choong Tak not only continued his active lifestyle , but also took up mountain-climbing.

FORMER Dewan Negara president Tan Sri Chan Choong Tak’s motto in life is to live it to the fullest.

Not surprising then that among his many accomplishments are two Malaysian Book of Records titles as the oldest Malaysian to reach the top of Mount Kilimanjaro’s Uhuru Peak (on Aug 31, 2003, at the age of 70) and the oldest Malaysian to reach the top of Mount Kinabalu’s King George Peak (on Aug 29, 2004, at the age of 71).

Uhuru Peak is the highest point on Mount Kilimanjaro, which is the tallest freestanding mountain in the world (from sea level) and the tallest mountain in Africa, while King George Peak is located on the more challenging and lessclimbed Eastern Plateau of Mount Kinabalu, Sabah.

What makes these two records more significant – aside from the impressive fact that Chan only took up mountain-climbing in his sixties – is that he was suffering from a rare bone marrow disorder at the same time.

His condition, primary myelofibrosis, is one of a group of diseases called myeloproliferative neoplasms, which are caused by abnormal production of blood cells in the bone marrow.

In the case of myelofibrosis, the problem lies in the abnormally-increased production of megakaryocytes, which are the cells that directly give rise to platelets. This results in an initial increased number of platelets in the body.

Cytokines – protein growth factors that are produced by megakaryocytes – are also correspondingly increased.

And as these cytokines are what stimulate the bone marrow’s fibroblasts to produce collagen, this results in an excessive amount of collagen being made.

The collagen deposits in the bone marrow as webs of fibre – similar to scar tissue on the skin – resulting in the disease’s characteristic fibrosis of the bone marrow.

With the collagen taking up so much space in the bone marrow, regular blood cell production is disrupted.

Red blood cells (RBCs) are usually decreased in number and abnormally formed, resulting in anaemia, while white blood cells (WBCs) are abnormal and immature, resulting in increased infection rates.

With production of blood cells in the bone marrow disrupted, the spleen, which is the body’s secondary supplier of blood cells, steps up to meet the body’s needs.

This extra work usually causes the spleen to enlarge (splenomegaly), resulting in pain or a feeling of fullness below the left rib.

Occurring commonly in those above 50 years of age, myelofibrosis is caused by a spontaneous genetic mutation (i.e. not inherited) in the affected person’s blood stem cells. This is what causes the uncontrolled production of megakaryocytes.

The cause of the mutation itself in primary myelofibrosis is, as yet, unknown.

Accidental discovery

As the symptoms of myelofibrosis, like fatigue, shortness of breath, pallor, frequent infections and easy bruising, are quite vague, diagnosis can be quite difficult.

In Chan’s case, he did not notice any signs or symptoms of myelofibrosis prior to his diagnosis.

In fact, it was a combination of a road accident and his wife, Puan Sri Cecelia Chia’s sharp eyes that alerted them to the possibility of a problem.

He shares: “My son gave me a racing bike for my 60th birthday – that was 21 years ago. So, I used to cycle around. Then, I met with a road accident.”

Chan was cycling along the narrow, winding roads of his hillside residential area in Seremban, Negeri Sembilan, when he suddenly met an oncoming car.

With no space to avoid the car, he braked hard and was thrown to the ground in a head-first fall.

“My helmet broke and I thought I would be paralysed. My friend, who is a doctor, straightaway rang up the hospital and they sent the ambulance,” he says.

Fortunately, Chan suffered no major injuries from the accident.

However, his cardiologist son insisted that he be checked more thoroughly for brain injuries, which resulted in him seeing a neurologist.

While his brain turned out to be fine, his wife noticed that his platelet count from the blood test were quite high – between 600,000 to 700,000 platelets per cubic millimetre, when the upper limit for normal is 400,000.

His son then sent him to consultant haematologist Dr Ng Soo Chin, who prescribed hydroxyurea to bring down his platelet count.

That seemed to work quite well for Chan, and it was, in fact, shortly after this that he began mountain-climbing with a group of fellow MBA (Masters of Business Administration) alumni from Tenaga Nasional Bhd.

Chan was then a director of the company, and had gone to Ohio University, United States, to study his MBA along with other Tenaga Nasional executives.

“So, as I climbed, I continued to take hydroxyurea and everything was normal.

“But Soo Chin said, hydroxyurea will eventually bring down your red corpuscles (another term for RBCs), and recommended anagrelide,” he says. Anagrelide is a platelet-reducing agent.

Accelerating disease

Chan continued happily with the two medications, until the year 2011, 18 years after his initial diagnosis.

By then, he was seeing consultant haematologist Datuk Dr Chang Kian Meng at Hospital Ampang, Selangor, as Dr Ng had advised him to continue his follow-ups at a public hospital as his medications are quite expensive.

Chan shares that Dr Chang started him on epoetin alfa and pegylated interferon that year as his blood cell levels were fluctuating.

While interferon decreases the production of blood cells in general, epoetin alfa stimulates the production of RBCs to counteract the effects of anaemia.

However, his haemoglobin levels dropped even further, and he started requiring blood transfusions about once every two months.

The transfusions made a big difference as he reports feeling “very energetic” after receiving the first one. (Fatigue is a common symptom of anaemia.)

The following year, it was the WBCs turn to go “completely haywire”, when a blood test revealed that they had dramatically increased to about 56 from the regular range of about 4 to 10.

He also started experiencing profuse night sweats and cramps, along with the occasional itchiness that had started in his seventies – all of which are among the symptoms of myelofibrosis.

“Then, both Dr Chang and Soo Chin agreed that I had entered into myelofibrosis in acceleration,” he says.

The only cure for myelofibrosis is a bone marrow transplant, but aside from the difficulty of finding a suitable donor and the riskiness of the procedure, Chan’s age rendered him unsuitable for such a treatment.

Fortunately for him, a new drug had recently been approved by both the European Commission and the United States Food and Drug Administration for use in myelofibrosis at that time.

A new drug

The drug, ruxolitinib, inhibits certain enzymes in the JAK pathway, which regulates blood cell production. Half of primary myelofibrosis cases are caused by mutations in the JAK genes, which results in the dysfunctional production of blood cells in the bone marrow.

However, the drug was not available in Malaysia then. (It was only launched in the Malaysian market in 2013.)

This is where his political connections as a Gerakan life member and former secretary-general came in useful.

Then Minister in the Prime Minister’s Department and Gerakan president Tan Sri Dr Koh Tsu Koon offered to help pass on the letter Chan had written to the Health Ministry requesting approval to use the drug on compassionate grounds, to the Health secretary-general.

Four days later, Chan received the approval he needed, and received his first dose of ruxolitinib in October 2012.

Since then, after some adjustments in dosage, Chan’s blood cells are back in the normal range and his last transfusion was in December 2013.

He is currently doing well enough for his doctor to lower his dosage of ruxolitinib, while still taking epoetin alfa and interferon.

Life goes on as normal for this active 81-year-old, who still climbs hills, reads newspapers of various languages and blogs daily, works out in the gym and does regular morning calisthenics.

Of his condition, Chan shares that he never felt the need to know about the disease, being only interested in his blood test results.

“I didn’t know what myelofibrosis was all about until I was asked to do this interview. That was the first time I went into Google to see what was myelofibrosis,” he says with a laugh.

“But I knew it was a dangerous disease, but I wasn’t bothered. I continued to carry on with my normal life.”

He adds: “I’m not bothered with what happens because I have full trust in my doctors.

By Tan shiow China The Star/ANN

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