Stop This Corroded World — I Want To Get Off!
Most of the time I love the world and appreciate its beauties. I honor the Creator, and appreciate all the blessings He showers upon me daily, but there are times, like today, when learning of some of the things that happen to people are just too much to bear!
Even Babies Can Die of Hemochromatosis?
How often I have declared that I was "letting go" of Hemochromatosis, only to be forced to discover that it will not let go of me, and here I go again! For many years, ever since my husband died a cruel death as a result of the destruction caused in his body by an overload of iron, I have agonized about how 'iron hides it’s evils behind a grinning mask of virtue,’ and I can never be grateful enough to Prof. Thomas Bothwell for his dedicated research which finally led to a diagnosis at the Johannesburg General Hospital, after he, my husband had been ill for eight years! It is comforting to know that since that time much has been said and written about this inherited disorder and its involvements; neverthless, although I am able to accept the fact that there are indeed those who need to take iron supplements and eat iron-enriched food, the very thought of it makes me cringe.
Of course, this is due to the fact that the greater part of my own life has been devoted to agonizing, writing, and talking about HH, and yet many doctors still remain ignorant and it infuriates me that many of those who have heard of it do not readily believe that it is anything but rare, which it is not! In Canada, for instance, it is estimated that one in every 300 people will die of hemochromatosis—although the solution is available—and, if patients are caught in time, and stick to the prescribed regime, there is no reason why they should not live to a ripe old age. However, after having also been equally voluble about chrondrocalcinosis, by which I, for instance, as a ‘heterozygote’ (meaning a carrier of one gene only for hemochromatosis) have been crippled, I must acknowledge that their existence will not necessarily be pain-free.
A New Horror! Even Babies Can Die of Hemochromatosis!
I found this hard to believe until Professor Patrick MacPhail, a former colleague of Professor Bothwell, confirmed it. “Most definitely true!” says the professor. “As we learn more about the very complex way that the body manages iron, more opportunities for things to go wrong are discovered. We used to think of hemochromatosis as a single disease, but it is not. When it presents and how bad it is depends on which gene/protein combination has gone wrong. We now know that many of the severe cases, presenting very young, are due to mutations in novel genes that have worse effects than the ‘common’ form. To my knowledge, the cause of neonatal haemochromatosis (not to be confused with ‘Juvenile Hemochromatosis’) is not yet known. It is very rare and usually fatal within the first few months of life.”