Life on a teaspoon: S’pore teen with rare condition drinks cornstarch water to stay alive

SINGAPORE – Thirteen-year-old Kayden Soh must follow a strict diet because his liver cannot process sugar. He can have only small amounts of fruit, milk and candy. He also drinks plenty of cornstarch water.

He mixes about a teaspoon of uncooked cornstarch with water and drinks the concoction every few hours, even in the middle of the night.

Without it, he could have dangerously low blood sugar, which is life-threatening.

Kayden suffers from Glycogen Storage Disorder Type 1A (GSD1A), a rare condition that affects the way his body breaks down food into glucose or energy for the body, due to a missing liver enzyme. It affects about one in 100,000 people worldwide.

Uncooked cornstarch is a complex carbohydrate that the body digests slowly. When consumed, the cornstarch releases glucose slowly into the bloodstream over several hours.

This slow release acts as a continuous, external source of glucose, mimicking the function of the missing liver enzyme and helping to manage blood sugar levels between meals and during fasting, especially overnight.

“Kayden first showed symptoms when he was nine months old. His belly was bloated. We took him first to the polyclinic and the doctor told us that it was quite common for babies to have a bloated tummy and asked us to just apply ointment. That was what we did,” said his mother, Ms Yong Xiao Ping, a 35-year-old interior designer.

However, her maternal instinct told her the problem could be more serious.

“His tummy continued to bloat, so that is when I decided to just take him to Mount Alvernia (Hospital) to consult the paediatrician there. The doctor wrote a referral letter and asked us to take him to KKH,” she told The Straits Times.

After some blood tests and a biopsy at KK Women’s and Children’s Hospital (KKH), Kaden was diagnosed with GSD1A.

The condition is caused by a deficiency in an enzyme called glucose 6-phosphatase (G6Pase), which is mainly found in the liver and kidneys. It is a crucial enzyme that converts stored glycogen – a form of sugar – into glucose.

Without G6Pase, individuals with GSD1A cannot release glucose from glycogen. As a result, their blood sugar levels drop during fasting or while sleeping, leading to severe hypoglycaemia or abnormally low blood sugar.

Dr Mildrid Yeo, a consultant with the Department of Genetics at KKH, said Kayden not only had an enlarged liver, but his blood test and biopsy results also showed low blood sugar and a high accumulation of glycogen in his liver.

Dr Yeo, who is currently Kayden’s doctor, said that when a typical person fasts, the body taps its glycogen stores, slowly breaking them down and converting them into glucose to maintain stable blood sugar levels.

“Kayden’s condition results in a block in the body’s ability to break down glycogen into glucose for energy... The only thing keeping his blood sugars stable is actually his food intake. But he needs to be very careful with how much carbohydrate he eats because, while it is good to keep blood sugars stable, any excess will be stored as glycogen in the liver that cannot release it. So then that’s bad,” she said.

Kayden has to tread a very fine balance, Dr Yeo added.

The teen has to adhere to very strict fasting intervals between meals to keep his blood sugar stable, and take regular meals that include uncooked cornstarch.

It is “the usual thickener bought from the supermarket because it acts as a long-acting carbohydrate to try and keep him safe within that five hours (without food)”, Dr Yeo said.

Kayden is also on medications such as allopurinol to help control high uric acid levels, a common effect of GSD1A that can cause symptoms like painful joint inflammation, kidney stones and fatigue.

He cannot go without eating for more than five hours, Dr Yeo said, though fasting times can vary among patients.

“So you can imagine that overnight, he has to wake up (to eat), or his parents need to plan his meals very carefully so they do not disrupt his sleep too much. In some cases, (the fasting times) can be as short as 1½ hours. These are very severe cases,” she said.

It is a strenuous schedule that can take a toll on caregivers.

Ms Yong said: “I do miss (the feeding) sometimes because I was too tired and did not hear the three alarm clocks going off. I do not really panic, but I would just quickly wake up to feed him.”

Kayden keeps emergency packs – usually a glucose drink prescribed with a specific amount for him – to use when he is unwell, or when he is in school for long hours. Sometimes, he substitutes it with milk and potato chips.

“We encourage patients like Kayden to try and live normal lives, and all it takes is a bit more planning. He uses continuous glucose monitoring through his mobile phone,” Dr Yeo said.

Despite the new rule in Singapore

banning secondary school students from using smartphones and smartwatches outside lesson time

, Kayden’s teachers at Fuhua Secondary School made an exception, allowing him to monitor his glucose level regularly via his phone.

As for Kayden, he has learnt to manage his condition.

“When it comes to drinking the cornstarch (water), I usually monitor the clock... Most of the time, I would be on time, but there are times when I would be late,” he told ST.

“Once or twice, I missed my 4pm feed while visiting a friend, so when I reached home, I quickly drank the cornstarch at around 6pm.”

GSD1A also caused his body to prioritise essential functions over growth, making him much smaller than his Secondary 2 classmates.

“Kayden is lucky because not only do his classmates look out for him, but his older brother’s classmates do the same too,” Ms Yong said.

Kayden has also made friends with the biggest boy in his class.

“I can’t really call him my best friend yet because it has only been a year since we became friends, but I hope we do become fast pals,” he said.