SINGAPORE \u2013 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.<\/p>\n
He mixes about a teaspoon of uncooked cornstarch with water and drinks the concoction every few hours, even in the middle of the night.<\/p>\n
Without it, he could have dangerously low blood sugar, which is life-threatening.<\/p>\n
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.<\/p>\n
Uncooked cornstarch is a complex carbohydrate that the body digests slowly. When consumed, the cornstarch releases glucose slowly into the bloodstream over several hours.<\/p>\n
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.<\/p>\n
\u201cKayden 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,\u201d said his mother, Ms Yong Xiao Ping, a 35-year-old interior designer.<\/p>\n
However, her maternal instinct told her the problem could be more serious.<\/p>\n
\u201cHis 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,\u201d she told The Straits Times.<\/p>\n
After some blood tests and a biopsy at KK Women\u2019s and Children\u2019s Hospital (KKH), Kaden was diagnosed with GSD1A.<\/p>\n
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 \u2013 a form of sugar \u2013 into glucose.<\/p>\n
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.<\/p>\n
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.<\/p>\n
Dr Yeo, who is currently Kayden\u2019s 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.<\/p>\n
\u201cKayden\u2019s condition results in a block in the body\u2019s 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\u2019s bad,\u201d she said.<\/p>\n
Kayden has to tread a very fine balance, Dr Yeo added.<\/p>\n
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.<\/p>\n
It is \u201cthe 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)\u201d, Dr Yeo said.<\/p>\n