Ever heard of MPS?

Never heard before...PMS women should know - Pre-menstual syndrome... but MPS seldom people know about it. Well, MPS stands for Mucopolysaccharidos. Such a long name, I wouldn't care about it until my children got diagnosed by MPS type IV A, which is also known as Morquio Syndrome. First time I heard of it, reminds me of mosquito but the sickness is not related to mosquito at all. It is a mucopolysaccharide storage disorder , taken after the Dr Morquio, a paediatrician in Montevideo, Uruguay, who described a family of four children affected by the condition in 1929.

The doctor at Genetics Clinic, City Hospital confirmed the diagnosis on Adnin & Aqilah on April 15, 2008, a day before Adnin's 8th birthday. How would one react to it? In actual fact, the doctor asked me what I felt about it. Well, I was very calm and steady, knowing that whatever happens is fate and the fact that I know about it, makes things better as I can really take good care of them. What actually causes Morquio disease? Mucopolysaccharides are long chains of sugar molecules used in the building of bones, cartilage, skin, tendons and many other tissues in the body. Muco - refers to the thick jelly-like consistency of the molecules, poly - means many, and saccharide is a general term for a sugar molecule. In the normal life there is a continuous recycling process of building new mucopolysaccharides and breaking down old ones. This process requires a series of biochemical tools called enzymes. Adnin and Aqilah lack this enzyme - galactosamine-6-sulphate sulphatase, essential in breaking down of Keratan Suphate. Incomplete broken down of Keratan Sulphate remains stored inside parts of the cells called lysosomes. The lysosomes become swollen and disrupt cell functioning causing progressive damage. There was no sign when they were born as the symptom start to appear as more and more cells become damaged by the accumulation of mucopolysaccharides. They were born normal - Adnin was 3.25 kg at birth, while Aqilah 3.3 kg. Adnin is now around 14 kg while Aqilah around 12 kg. Seems like I don't feed them well, in actual fact, even if I feed them the best food in the world, when their body lack the enzyme, they will still be small as they are. One advantage of them being small, any bus or train ride, they ride for free!