The first Sri Lankan under 'Biological' drugs for Ankylosing Spondylitis (AS):

He is now pain free...!

by Shanika Sriyananda

This is a story of a small boy who lost his childhood cried in pain, not just for days and weeks but for years. This is also story of a father who took his 'bundle of joy' to the four corners of the earth to cure his one and the only offstring, suffering from a crippling disease.

This is also a story of a mother who became the tower of strength for the sick child. Finally, this is a story of a 'magic drug' which miraculously gave a 'pain free life' to a young boy, whose dream was to help hundreds of others who suffer from the same agony.

Nine-year-old Siddarth Bahal was a very active boy of the St. Wycherly International School until that day, when he had a fall and taken to the family physician for treatment for the swelling of left ankle. The doctor prescribed some pain killers, but, by them both his ankles had swollen and had spread upto the knees next day.

Bahal family took little Siddarth to India for treatment, consulted from a doctor to doctor in search of remedy but in vain. As the ESR was high, they treated him for tuberculosis of bones. Heavy doses of antibiotics and anti-inflammatory drugs were prescribed with several tests, including an Aspiration of the knee'. Results were negative and Siddarth was referred to a rheumatologist, whose diagnosis was juvenile rheumatoid arthritis.

Not answering any drugs, the pain and the swelling had now spread to the hips and had shown no improvement during their six-month stay in India. Then the Bahal family flew to England, where Prof. Patricia Woo of the Great Ormond Street Hospital diagnosed that Siddarth had Ankylosing Spondylitis (AS).

The painful days seemed to be coming to an end with simple pain killers prescribed by Prof. Woo, who advised him to do exercises and avoid wrong food items that might result in a bad stomach. Unkind

But they were not blessed with long lasting happiness as after few years later Siddarth's illness came back in a big way, affecting his hips, jaws and shoulders. The AS, which normally attacks the small joints, was not so kind to the boy and it attacked all the big joints as well as the small ones.

Siddarth's life was not a bed full of roses. It was one with pain and suffering, not, a few months but 10-years. The only remaining solution for the doctors to bring down the pain was to a prescribe cocktails of drugs.

Those drugs were very effective to ease the pain for a short period of time but each dose lost its effectiveness after a while. When one dose was withdrawn another was given while internal bleeding in the stomach was continuing.

"It would have been so much of fun going with my little son abroad to France and the UK. I, like other dads, had big dreams about my son. I wanted to give him the best and see him doing well in his studies", recalls Harry Bahal, Assistant Technical Services Manager of the Sri Lankan Airlines.

Not only their happiness but also that of innocent dreams and hopes of Siddarth were robbed by the AS. He, as a small boy dreamt of becoming a Formula I car driver.

The pain rising from almost all the joints of the body and with the side effects of drugs - abdominal bleeding- could not stop Siddarth going to school who never missed his classes. Tolerating the pain he studied... played and even engaged in all extra curricular activities. Harry and Karin and even the doctors found it difficult to explain the pain.

Pain killers, hot compresses, physiotherapy, relaxation therapies and psychological pain management were the strategies that used to combat the pain.

"We suffered a lot with my son. He was a very mischievous little boy and how a mother can tolerate the agony of a son who is battling with such an illness. When somebody has HIV/AIDS or cancer or any other life threatening disease, you know that person is going to die and he or she won't suffer long. But, here, AS patients won't die but they will have to suffer throughout their lives. So, imagine the situation of a mother seeing her son crying out in pain and asking for help. I was so helpless" says Karin, trying to hide her emotions.

'Iron lady'

She is a mother who had bottled up her worries and cried alone for several nights. She became an 'iron lady', a steady and strong mother for her weeping child. "In front of him, I never cried as I thought that will demoralize my son in his battle to bare the pain. I always tried to be strong and I never let him be alone", she says recalling how she read out not only the story books but all the text books loudly seated by his side as he was unable to move about with his stiff spine.

As a school going child with a stoop, sometimes with a limp due to stiffness and swelling of the ankles, Siddarth did not get a kind welcome from his school mates. The boy, who could not carry his own school bag let alone the water bottle, became the centre of jokes at the school, on some days. But he was lucky to have some good and kind friends mango friends, and teachers who cared for him.

Apart from the physical trauma, he suffered psychologically and on several days he told his parents that he is not keen to go to school.

"They used to tease him a lot. Teachers were aware of his ailment and they told us that he is a brilliant child but lives in a dream world. But, that was due to effects of active AS and also the side effects of the drugs. They cause dizziness and he was mentally shut out because of the unbearable pain in joints", Harry says.

Helplessness

Tears well in Siddarth's eyes when he recalls his painful life, where he used to take extra dose of pain killers to get relief just to finish his lessons. This courageous boy came to know the reality of the crippling disease as he grew older and determined to battle with his fate. But, when he got up after just 3-hours of sleep and just lying on the bed screaming in pain he realised more about his helplessness.

There, with every sleepless night he was more determined to live that day and keep hopes for a better future.

Without cursing his life, Siddarth, managed with his pain, passed his O/Ls and A/Ls successfully. When there is less pain after taking pain killers he had a period of six to seven hours with less or no pain. He did all the studies which he had missed and was very active till he comes around again.

The painful disease could not cage the boy in his room filled with bottles and vials of pain killing drugs.

He, who did not fear the disease was motivated when he met famous people like Michael Atherton, former England Cricket captain, Michael Slater, former Australian batsman and Dr. M.A.Khan who have the same illness. They are still his role models to face life.

No one would believe that Siddarth won the under 17 Badminton Championship once. Studying for a post graduate degree in Mechanical Engineering at the University of Nottingham, now 19-year-old, Siddarth's eyes brighten when he says that he enjoys a pain free life now. "It is a miracle.

After giving biological drugs all my pains which hurt me for over a decade had disappeared within two hours", says, Siddarth, who is now brimming with new hopes.

Bahal family kept so much of trust on one doctor, who had paved the way to their dear son to become pain free. His careful healing touch had also made the studious boy to manage his pain to do his studies well. "The last doctor we consulted was Dr. Lalith Wijeratne, who treated Siddarth for years and finally directed us to a doctor in India who knew well about biological drugs", say Harry and Karin.

Siddarth, who believed in a life, amidst of suffering saw the light at the end of the tunnel after a decade-long battle, has now started another battle - to speak on behalf of thousands of youth, who suffer from AS but do not have the means to access biological drugs due to high cost. They had spent over Rs. 150,000 to get the biological drugs - Infliximab (Remicade) and Etanercept (Enbrel) from the company, Wyeth. He is now on the second drug Enbrel.

Top priority

Contributing to improve the quality of life of AS patients, who have joint deformities, is top priority among Siddarth's list of long term plans. "The patients who have this disease need to demand from the government to make these drugs available in the country.

These drugs will make one's life, pain free and allow them to lead a very normal life", says Siddharth pointing out that in UK and the USA the government has sponsored programs for AS patients, especially for the youth, to give these drugs free of charge every year.

He says there, the patient had to be between the ages of 20 to 30 to qualify to receive biological drugs.

International researches have shown that these drugs are 99 per cent safe and their side effects are minimal, says Siddarth adding. "It is better to live a pain free life for 40 years rather than living for ages suffering from terrible pain with deformities in joints."

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Consultant Rheumatologist of the Department of Rheumatology and Rehabilitation (DRR) of the Colombo National Hospital Dr. Lalith Wijayaratne in an interview with the Health Track said that nearly 20 new cases of Ankylosing Spondylitis (AS) were reported to the DRR annually.

Two new AS patients seek treatment monthly. According to Dr. Wijayaratne, 90 per cent of the patients are below the age of 30 and from lower middle class families. There is no national survey on AS yet to count total number of AS patients in the country. Here are the excerpts of the interview:

Q: What is AS?

A: AS is particularly a disease of the spine. Most of the arthritis patients get affected in the peripheral joints. But there are few people whose joints of the spine too get affected. And that causes several problems to the spine. So, the AS patients have lots of pain in the spine with neck pain, back pain and shoulder pain.

There are veritable joints, peripheral joints and root joints including the shoulder and the hip joints. AS mainly affects the veritable and roots joints. The back pain, shoulder pain, hip pain and neck pain are the common symptoms of the AS.

Here, the patients get pain and stiffness and if they are not properly treated and these joints will get glued together. The movements stop and ultimate result is the stiffness of the spine and neck. It is like your spine converted into a concrete pillar.

Q: How long will it take one to get the stiffness?

A: It varies from person to person. If it is an aggressive type, it will be in five years time. Or others will get it in 10 years time. It varies depending on how young you are, how serious your disease at the beginning, what treatment you have taken and how much exercise you do.

The common arthritis is mainly among females who are in the middle age (40 to 60 years). But unfortunately the AS is predominantly a disease of the men. Usually they are in the age group of 20 to 30 of age, which is the productive age. Therefore the loss for the country is more with AS because most of AS patients are young people. Their future is shattered, if they do not take proper treatment.

Q: What are the real causes for AS?

A: No. Still no one knows the causes for the AS. It is a problem within one's body. There is a belief that a bacteria in the intestines may have a role in AS. Some say that this may be due to soriasis - a skin disease, which is common. About 8 to 10 per cent of Psoriasis patients can get AS.

There is another rare disease, which calls inflammatory bowel disease and they are also vulnerable to AS. But, the causes for majority of AS cases have developed within their own bodies. This is a condition where our own immune system is attacking our own joints. Why it happens is not known.

Researches are going on and lots of new things have been discovered. This also can run in genes.

Q: What is the role of biological drugs in curing AS?

A: Today, there are plenty of new drugs available to treat the arthritis in peripheral joints. But it is very sad because all these special drugs, which were produced in the West for the last 15 to 20 years, were mainly for the joints of the limps, shoulders, the knee, the feet and the elbows.

Nothing is available to treat arthritis of the spine. So, biologics are a major breakthrough. We call it a magic drug thanks to the Western researchers who found this new drug, which was able to give relief to AS patients. These are very special drugs, which are only meant for the joints of the spine.

Q: How long will it take to cure an AS patient with biologics?

A: It depends on the person's age, how long after the onset the patient is coming for treatment and how aggressive the disease is. There are many variables to determine how good are the biologics for the patient. This magic drug, like any other drugs has bad side as well. Biologics attack the immune system.

When one is given biologics, his chances to get infections and certain type of cancers can be more.

Biologics available for AS attacks the molecular called tumour necrosis factor. TNF is a molecule that is found in the immune system and also a fuel for arthritis. It has lot to do with arthritis but at the same time it has bad effects also.

It also helps to build the immunity against infections. And also helps us to keep the body against the cancers. The TNF can do a cancer surveillance to check whether we have got cancer cells. These biologics blocks the TNF.

But, one will be more prone to infections and certain cancers, due to the use of biological drugs. Cancers due to these drugs are very rare. So one has to be very careful when using biologics.

So, first the immunity of the patient needs to be checked. Especially, we need to be very careful about TB and lots of precautions need to be taken, and also need to examine the patient to see whether the person has cancer or not. The two major problems of using biologics are the high cost and the infections like TB, which might creep in.

Q: Do you have any suggestion to make the magic drugs available in the country?

A: At the moment Sri Lanka Association of Rheumatology has got together and plans to request the government to make this drug available in Sri Lanka. This drug has been in use for the last five to eight years.

At the beginning there was no hope for AS patients and they ended up cripples. But thanks to the Western researchers, the magic drug was discoverd but we have to wait and see the long-term effects of these drugs.

More researches are going on. This is a new avenue for AS patients, but need to be careful in selecting the right patient because with the wrong patient the consequences would be more serious.