Sage Tan, Age 13
Born maturely at 2.5kg on Christmas day, Sage was the ideal healthy baby that any parent could ask for. He might have drank too little and slept too much, but his parents did not think much of it. However, when Sage couldn’t wake up on the 10th day, Cindy did not need her mother’s intuition to know that something was wrong.
At two weeks old, little Sage was rushed into the Intensive Care Unit (ICU) and diagnosed with Maple Syrup Urine Disease (MSUD). It is a rare genetic metabolic disorder that prevents the body from processing certain amino acids which are formed when protein in food is digested. When the level of amino acids in the blood reaches a high level, it becomes toxic to the body. This can cause brain damage, seizures and, if left untreated, death.
The medical team finally identified the illness through the distinctive sweet-smelling urine where the disease got its name. The disease was so rare and almost unheard of in Singapore that it threw many doctors off and they did not know what to do.
Subsequent blood tests found that Sage’s blood was so toxic he needed to undergo dialysis. However, his 2-week old veins were so small that even the thinnest catheter could not be used. Thus, the doctors had to do the transfusion manually by stringing blood out and then pumping fresh blood into Sage’s body through his legs. This painstaking process took 4 hours.
Thereafter, the only thing Sage could consume was specially formulated milk modified specifically for children with MSUD. Unfortunately, back in 2006, Singapore had yet to produce such a formula and it had to be shipped in from the UK. At this point, Sage was in a coma in the ICU. To make matters worse, there was a complication with the information given to the UK medical staff and the fatal miscommunication caused the special milk formula to never reach Singapore’s shores.
Both parents were distressed but Cindy was guilt-stricken to know that the milk she had been providing her son for the first two weeks of his life was killing him as his body could not process the protein in the milk, and that Sage was now living off a drip in the ICU.
Before all could be lost, a call came from Sage’s aunt in KL bringing the first hopeful news: there was a similar modified milk formula in Hospital Kuala Lumpur. The doctors there, after hearing about Sage’s condition, were ready to ship a batch over. With the prompt help of Sage’s relatives in transporting the formula from KL to Singapore, Sage’s condition improved and he was discharged after spending a month in the hospital.
However, the worst of it was far from over for little Sage and his family.
After Sage was well enough to return home, Cindy took it upon herself to prepare Sage’s milk formula daily and care for him. The special formula contained 6 different ingredients whereby Cindy had to measure the exact dosage for each ingredient and note the batch limit for the day. Too much protein and Sage would be groggy; too little and he would be cranky and be unable to settle. Furthermore, the modified milk produced a rather fishy smell, causing an acid reflux where Sage would throw up 9-10 times daily. This was very stressful for Cindy as she had to make sure Sage met the daily quota of nutrients for his growing body, and each episode would upset the delicate balance of the nutrients he needs.
Cindy recalled once when she broke down and cried after Sage had thrown up all over the floor instead of into the plastic bowls that littered the house, making it impossible for her to measure how much protein her son had lost and how much milk she had to replenish. It might had sounded silly to others, but each episode had to be carefully quantified as it was a literal matter of life or death for her son.
In 2006, Singapore did not have a proper machine for blood testing for genetic conditions. Thus, the blood had to be flown or driven up to KL for processing where the results would then be sent back. Every 3 to 4 days, Sage would have to take a blood test to ensure that each component was within safe parameters. By the time Sage was 3, he had taken more than 150 blood tests.
Throughout his ordeal, Sage remained a happy child, easing his mother’s worry with his bright smiles and cheery personality. He was greatly entertained by toy cars and models and it was a common sight to see four-wheeler gifts of all shapes and sizes from relatives and friends decorating his hospital room.
Sage’s case of his rare genetic disease spread to his neighbors and community, and several articles covered his story. This reached Abbott Nutrition SG who pledged to sponsor 2 years' worth of the specially formulated milk for Sage, which costs $60 per can and lasts 2-3 days.
However, Sage’s condition went downhill days after his 2nd birthday in late 2007. He caught a stomach flu, which caused an influx of amino acids to accumulate due to the imbalance of nutrients in his body. He was then hospitalized due to multiple seizures where the doctors expressed their worry on the long-lasting effects on Sage’s brain if the seizures were to continue. They raised an option of a liver transplant, which would allow Sage’s body to process protein normally and finally give him a chance to live a normal life.
The family had a serious discussion about it, taking into consideration the many risks involved as there have been very little operations done on children with MSUD in the last 10 years. Eventually, Cindy made the tough decision to go through with the transplant.
Then, came the problem with finding a matching donor. As the donor cannot be a carrier of the defective recessive MSUD gene, neither Cindy nor her husband could be the donor since the disease was genetic. Everyone had to get tested. Sage’s Cousin (Cindy’s Nephew) was found to be a positive donor and at 3 and a half years old in April 2009, Sage went through his first liver transplant at NUH.
Unfortunately, complications arose during the operation, the hepatic artery was found to have a blockage and it caused a decrease in oxygenated-blood to flow into the organ. This also caused his bile ducts to clot and gradually, he developed jaundice.
In the wake of a failing liver, Sage was placed on the priority list for another transplant from a cadaveric donor. After 4 months with no matches and the toddler’s health steadily deteriorating, Sage’s aunt volunteered for the transplant despite the risks to both the recipient and the donor. Within 6 months of his first transplant, Sage went through his second transplant in October 2009. This time, a doctor who was an expert in the field was flown down from Japan to Singapore to carry out the operation on Sage.
To the relief of his parents and relatives, Sage’s second transplant was a success.
Today, exactly a decade after his successful transplant, Sage is a happy-go-lucky child with smiles for everyone. Although the many operations he went through left him with deep physical scars and noticeable knots and bumps from the non-dissolvable sutures, his optimistic and bright nature has held strong.
His early tumultuous years and late medical detection caused Sage to suffer from global development delays and language disorders. The immunosuppressant drugs he takes also caused him to have multiple food allergies to seafood and all nuts. He struggled through his studies but gave his best effort and completed his PSLE in Monfort Junior at foundation level. He is now studying in Spectra Secondary School.
Sage’s love for cars and Lego has followed him through his life and his house is decorated with a massive assortment of Lego models from Star Wars to Ferrari, which family and friends bought for him to improve his motor functions. Just last year, Sage put up a custom-built Lego battleship, that he put together with his older brother, for auction at a fund-raising event. His battleship was auctioned for $1,800 to Ferrari Challenge Driver, Mr. Martin Berry, before it was donated back to Sage where he has his Lego model taking pride of space in a glass case at home.
Sage has also shown a keen interest in visual art, participating actively in our programmes such as Acrylic Pour and Digital Drawing. He also took part in Dreamseeds Arts Fest 2018 to design a tote bag which is being sold to raise funds for his peers in Club Rainbow. To cultivate his interest and recognize his commitment, he has been receiving the Talent Development Fund for the past few years and was also selected to take part in the Collaborative Storytelling Illustration Programme with Darel Seow.
For Cindy, Sage’s battle with his illness has finally stabilized, and she is eternally grateful that it had a happy ending. While she still worries for Sage’s future, she is also positive that Sage will grow to be able to take care of himself fully and be more independent one day.
Cindy would like to stress the importance of early newborn screening and urges all young parents to send their babies for the screening to detect any genetic disorders early, which can then be addressed promptly. Regardless, she is immensely proud of how far her son has come and is looking forward to the many new experiences and opportunities that lay ahead for her son.
Keen to help other children and youths like Sage? Click here to donate to Ride for Rainbows 2019, our annual night cycling event that aims to raise funds for children who have chronic illnesses and their families.
Written by: Charlotte Leong
Photo credits: Josh Wijaya