By Reeti Mahobe:
How would your reaction be if you are told that you must avoid ice-creams, sweets, chocolates, cold drinks etc. throughout your life, right from birth? And that you should never be starving? Sounds quirky, eh? Well, to an extent it is. This is what patients suffering from ‘Glycogen Storage Disease’ are advised to do. Recently the world, rather west, because very few in India are aware of it, celebrated the Glycogen Storage Disease Awareness week from 25th August to 31st August’13.
It’s a rare inherited disorder where liver lacks or is deficient in some enzymes that inhibits conversion of glycogen (stored energy) into glucose (readily available), when body needs to use it. It’s a life-long condition that the person has to live with forever. Sounds scary? No, it’s not that much actually. This diseases has several types, barring few types, most others allow the affected person to live a pretty normal life. It must be known here that there’s no cure for it yet in the world. Few basic precautions and some adjustments in lifestyle can help the person lead a better life. Having cornstarch at night or night feeds would not let you loose your energy during your sleep.
Cornstarch is the major part of the treatment. Some of the advises differ with different types but some are common. Few might need to have G-tube inserted for night feeds in their bellies. Many of the types involve some form of muscular weakness during adulthood and later, to compensate for which, one has to have high protein and low fat diet. Along with that, at least annual monitoring of vital parameters of the affected person is a must. Well, this might sound simple. Yes, it is. But if one doesn’t follow these and other specific advice provided, the condition might get complicated in no time, for instance, if one doesn’t have any food for long, she or he may end up hypoglycemic with headache, drowsiness, dizziness, vomiting, seizures or even death!
So, what’s really required is an enhanced level of awareness about this problem. Sometimes, the patients go undiagnosed for very long, that leads to complications. This really sums up the dire need of a wide awareness program, particularly for those in India, as there are some dedicated centres and groups in the west such as in UK and US while there’s none here in India. Even doctors don’t have enough knowledge in this regard while many in India are affected with this condition, only the big hospitals in metropolises have required resources about it. Many Indians who can afford to, fly to Florida wherein under world-renowned Dr. David A. Weinstein, they get all the essential advice and treatment. With this, I wish to initiate an awareness campaign for Glycogen Storage Disease, as that’s the key to a happy life ahead for affected ones and their families. I would urge the government and such other agencies to help in pushing this forward and make it a pan-India one to help many.