Hope is Darmash

Darmash Subaramaniam, age 16 (deceased)
Mucopolysaccharidoses (MPS) Type II
Malaysia

As his sister, I will make all his dreams and hopes come true.

Sometimes we will never know the true value of a moment until it becomes a memory. I wish I realized it earlier as the only thing left for me and my family are memories of my brother, Darmash Subaramaniam. Family, where our stories began. Story of me, my parents Subaramaniam and Sumathi, and my brother. Being the elder and only sister for Darmash, a Lysosomal Storage Diseases (LSD) patient, I used to put all his needs before mine. Same goes for my parents who never failed in providing him with all his needs and desires.

LSD is a group of inherited metabolic diseases where a defective or missing enzyme causes metabolism error in lysosome which is the recycling center within a cell structure. This causes unwanted buildup of various substances. Most of those disorders are passed down from the parents who are healthy carriers. There are altogether about 50 different types of LSD and Darmash was a MPS Type ll patient, which also known well as Hunter syndrome.

LSD is life-threatening. Believe my words, pals. My family witnessed how cruel that disease is.  When Darmash was born in 2000, he was a healthy baby. Words can’t describe how I felt when I touched his baby fingers, while looking at him for the first time. I looked like a child looking at moon, probably. He was a beautiful baby without problems. But, with the flow of time, we noticed there was something wrong with him and his body. After a lot of appointments at various hospitals, Darmash was diagnosed in 2006 with a disease which even the doctors don’t understand.

My whole family was in fear and confused. What was happening to him? Darmash never grew like other children. He was short, about 4 feet tall. His arms and hands could not straighten. All his joints were hard. Even his fingers were stiff. With a swollen stomach and short neck, he was totally different from all of us. For about 4 years, without comprehending what was wrong with Darmash, my parents spent a lot of money to get him cured. But all their efforts were futile.

In May 2011, all our questions were finally answered by when the staff of the General Hospital of Kuala Lumpur, Malaysia introduced us to other families whose children  were like my brother. Doctors of the hospital explained to us in detail about this rare LSD disease.  From there, we and other families, despite our different races and cultures, founded a non-profit organization, Malaysia Lysosomal Storage Diseases Association (MLDA), which advocate for LSD patients’ treatment rights and general welfare.

Currently, the available treatment is either by Enzyme Replacement Therapy (ERT) or through Bone Marrow Transplant (BMT). ERT being safer and not requiring bone marrow matching of a healthy donor, is preferred.  ERT is a lifelong periodical treatment of extremely high medical cost. ERT costs about RM 1 million a year for each individual. No family will be able to afford this cost on their own. Insurance companies in Malaysia are not willing to cover the risk. And so, we as association, led by MLDA president Mr. Ir.Lee Yee Seng, started to work towards raising money to treat Darmash and his fellow friends.

Despite his poor health, Darmash was a strong child who never gave up on anything. His rare disease never prevented him to be himself. Back when he was in primary school, his hobby was cycling and playing football. Oh yes, he did attend a normal school like all other children.  He was good in writing and navigating the internet. Despite having hands that couldn’t straighten enough to hold the handle bar of a bicycle, Darmash tried very hard and finally was able to ride one. He used to cycle over our estate with our neighbours. Such a strong boy he was. And he was an ambitious person too. His ambition was to become a successful car racer. His love for cars was uncontrollable and unlimited. He often said to me that he will only get married with a modified car. Not with a woman, he said.

At secondary school, the fun and outgoing Darmash whom my family am and I used to know disappeared. He began to distant himself from others except his family. He quit football and cycling, and started to play CSI Criminals and surf internet for modified cars. This was because of his worsening body condition, and I believe, his teenage heart. It was hurtful for him, as a teenager, to see other boys his age with healthy bodies. As his sister and parents, we could only  work hard to raise money for his treatment. Finally in August 2015, at the age of 15, Darmash was able to receive ERT.

Every Thursday, we would travel interstate 115km to the General Hospital in our old Proton Iswara to bring Darmash for ERT treatment. Even though the long drive was tiring and dangerous, we never failed to make the trip. We met with countless obstacles - accidents, car breakdowns, financial problems. My father, a factory worker, had to take leave every Thursday and endured many work related problems as a result. But we persevered with the hope that Darmash would be cured.

Darmash 01

Darmash was a dreamer with no hope until he began ERT. After ERT, his condition began to improve. His stomache did not swell as much, his hand joints and fingers were not as stiff, and his facial features softened. Having experienced theses changes, Darmash started to believe that one day he will become normal and will drive a modified car in the racing track and win.. He often said to me that the two of us will be living in a big bungalow with indoor swimming pool and he would drive an Audi R8.

With ERT, he began to laugh, speak and spread his wings. He began to live life.

My family and I were beyond happy. I saw how a successful treatment could change a patient’s mind.

Darmash 02

But all this was shortlived. Our hopes, dreams, and life were gone in the blink of an eye on 16.06.2016. Why? Because Darmash died. Everything happened lightning fast. One second, he was walking happily with us towards our car after ERT and the next second, he was lying on the floor, unconscious. Frantic, the three of us carried him and ran towards the hospital only to find out he had left us three behind; he had left all of his hopes and dreams; he had left his life.

What happened? LSD is life-threatening. Untreated, LSD patients will have a variety of complex disorders that involve multiple organs, which are fatal. By the time Darmash received his first treatment, he was already 15 years old, too late as the life span of LSD patients generally does not exceed 20 years. Treated early, they have a better chance of living longer. We couldn’t afford to give Darmash ERT when he was younger. If we had done that, maybe Darmash would still be alive. But it’s too late now. He is gone.

Darmash 03

But not his dreams and hopes. As his sister, I will make all his dreams and hopes come true. An ounce of blood is worth more than a pound of friendship.  Despite Darmash’s absence, the love in the four of us will continue to grow deep and strong. Our special moments with Darmash are for us to keep and treasure.

Death ends a life, not a relationship.

 

 

 

 


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