Sometimes we will never know the true value of a moment until it becomes a memory. I wish I could 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. We are from Malaysia. Being the elder and only sister for Darmash, a Lysosomal Storage Diseases (LSD) patient, I used to put all his needs before mine’s. Same goes to my parents who never failed in providing him with all his needs and desire.
LSD are 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 autosomal recessively inherited, meaning the disease is passed down from the healthy carrier parents. 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, my family and I had saw how cruel and devastating the 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 look like a child looking at the moon, probably. He was a beautiful baby without any problems. But, with time, we noticed there was something wrong with him and his body. After a lot of appointments at various hospital, in 2006, we were informed that, Darmash was diagnosed with a disease which even the doctors are not familiar with.
Our whole family was fearful and confused and we do not know what was happening to him. Darmash never grow up like other children does. He was short, generally around 4 feet. His arms and hands can’t be straighten. All his joints was stiff, including his fingers. With a protruding stomach and short neck, he was totally different from all of us. Without comprehending what is wrong with him, my parents spent a lot of money, hoping to cure Darmash for about 4 years. But everything was useless.
In May 2011, all our questions was answered by General Hospital of Kuala Lumpur, Malaysia when they introduced us to some other families with children same as my brother. Doctors of the hospital explained to us in details about this rare LSD disease. Despite different races and cultures, we and other families, founded a non-profit organization, Malaysia Lysosomal Storage Diseases Association (MLDA), which advocates for LSD patient’s 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 required no bone marrow matching of a healthy donor, is preferred. ERT is a lifelong periodical treatment that requires ultra-high medical cost. For each individual, ERT cost averages about RM 1 million a year. No family will be able to afford this cost on their own. No insurance companies in Malaysia are willing to cover the risk. All of us worked hard to procure funding for ERT for Darmash and his fellow friends, led by MLDA president Mr. Ir.Lee Yee Seng.
Despite the unhealthy and unfavorable health condition, Darmash was a strong child who never give up on anything. This rare disease, never been a stopping point for him to be himself. Back when he was in primary school, his hobby was cycling and playing football. And yes, he does go to a normal school like all other children. Even though he was not a bright student at school, he was not a fool either. He was good in writing and surfing the net. Moreover, even with hands that can’t grip the bicycle handle bar properly, Darmash did not give up and finally was able to cycle. He used to ride bicycle all over our residence area with our neighbours. He was an ambitious person too whose ambition was to become a successful car racer. His love for cars was uncontrollable and unlimited. He often says to me that he will only get married with a modified car. Not with a women he says.
When he enters secondary school, the fun and outgoing Darmash that my family and I used to know, changed into a whole new person. He began to distant himself from others except three of us. He stopped playing football and cycling and starts to play CSI Criminals and surf internet for modified cars. He changed due to his worsening body condition that comes with age as well. Furthermore, I could understand his teenage heart. At age about 14 and 15, seeing boys around same age as yours with healthy body and yours are not is very hurtful. It hurts him, when he saw youngster around his age. As a sister and parents we don’t know what to do other than working hard to pledge rich people and corporation to cover the treatment cost. Fighting over 4 years, finally in 2015, on his age of 15, Darmash commenced ERT at HKL. His first ERT was on August of 2015.
Thursday of every week, we will travel about 115km, to General Hospital which was situated in Kuala Lumpur, by car, an old model Proton Iswara, for Darmash ERT treatment. Even though the long drive was tiring and dangerous, we still go on, no matter what. There was too much hardship to re-count on our weekly trip to HKL for the infusion. Our car broke down, money problems and so much more. Because of this every week treatment, my father had to take leave from his work as a factory worker every Thursday and this leads to many problems to him on factory. But we still carry on with a hope that Darmash will have a better more quality life.
Darmash was a dreamer with no hope until he got ERT. After ERT his body started to improve. His stomach is no longer swollen, hand joints and fingers become less stiff and facial features becomes soften. Undergoing all this changes in himself, he started to dream with hope. ERT gave him hope. He started to believe that one day he will become normal and will drive a modified car in racing track and win his match. He started to believe that one day he can buy a big bungalow and Audi R8. He often says to me that we two will living in a big bungalow with indoor swimming pool.
With ERT he started to laugh, started to speak, started to spread his wings, started to live his life. My family and I was estatic. I saw how a successful treatment could change a patient’s mind. But all was for a short time only. Everything, our four’s hope, dream, and life, was gone in a blink of an eye on 16 June 2016. Everything was gone. Why? Because Darmash died. Everything happened at lightning speed. One second, he was walking happily with us towards our car, after ERT and the next second he was lying on the floor unconscious. Like mad people, we carried him and runs towards the hospital only to know, he had left us three alone; he had left all of his dreams and hopes.
What suddenly happened to him? Want to know the reason? The reason is like I said, LSD is life-threatening. If LSD patients are not treated early, they will end up having a variety of complex disorders that involve multiple organs, which will lead to death. Darmash got his treatment at the age 15 which was quiet late, as LSD patients average life span generally does not exceed 20 years. If they are treat in early age, they are bright chances for them to live longer. Due to ultra-high medical cost, we a middle class family, are unable to afford the ERT treatment for him in his early days. If ERT commenced when he was diagnosed back then, he may have a better chance of living a longer a life. But it’s too late now. He is gone. But not his dreams and hopes.
All I could do now as a sister is, to make all his dreams and hopes come true. Others things may change, but we start and end with our family. An ounce of blood is worth more than a pound of friendship. Despite Darmash absence, the love in four of us will grows strong and deep, leaves special moments of Darmash to treasure and keep.
Death ends a life, not a relationship.
Written by Darmash’s sister Vinodini.