Progeria.

Hello from me, it's been awhile :)

Progeria or also known as Hutchinson-Gilford progeria syndrome is a genetic disorder or condition that is typically characterized by the dramatic and rapid appearance of significant aging that starts during the patients’ early childhood. Children with progeria grow more slowly than other children their age normally would and do not gain much weight at the normal rate which is one of the signs of failure to thrive although children with progeria usually look normal when they are born and also, during early stages of infancy.

            The prominent signs in progeria can be seen from their features; protruding ears that may look like that of pixies; they have small chins, thin noses that resemble birds’ beaks, thin lips, and very prominent eyes. This genetic disorder also causes aged-looking skin, baldness due to hair loss or in scientific terms, alopecia, a loss of fat and adipose tissues under their skin (subcutaneous fat) and also, joint abnormalities. The development of motor movement or skills of children affected with this genetic disorder are however, not affected as they are able to stand, walk, sit and do things on a daily basis well. Progeria also does not interfere with children’s intellectual development and children with progeria are known to have bright and cheerful personalities and sharp minds.

            Progeria is derived from ‘geras’ which means old age in Greek. It is assumed to typically affect one in four million newborns around the world. As progeria is extremely rare, not much has been hear about it before and society is usually not aware that this genetic disease or genetic disorder even exists. Doctor Jonathan Hutchinson first described and logged down this disease in the year 1886 and then in the year 1904, Doctor Hastings Gilford also did the same. The disease is named after the two of them as they were the ones who first mentioned about it and left an impact in the medical society. Coming back to the signs of progeria; within the short period of a year, children with progeria have slower growth rates and soon become shorter than most children their age.

Children with progeria are also known to be sufferers of symptoms old people usually suffer from which include hips dislocations, severe progressive cardiovascular diseases and also stiffness in their joints. Thankfully, children that have progeria are not known to have osteoporosis or cataracts which are symptoms atypical of that of an old person. In attempts to help with the life threatening cardiovascular complications that are caused by progressive atherosclerosis, some children affected with progeria have to undergo coronary artery bypass surgery and/or angioplasty. Death comes morbidly early for progeria sufferers as most die at an average of 13 years normally from strokes or heart attacks.

What are the causes of progeria? NHGRI researchers combined their knowledge with researchers at the Progeria Research Foundation in the New York State Institute for Basic Research in Developmental Disabilities and also, with the University of Michigan in 2003 and thus, discovered that progeria is caused by a minute point mutation in a singular gene called the lamin A or LMNA. The genetic mutation appears in nearly all the cases to occur in sperm right before conception. The substitution of only one base pair among the estimated 25 000 DNA base pairs that form the LMNA gene is found to arise in almost all the cases of recorded progeria sufferers and is amazing. This gene codes for two proteins which are the lamin A and the lamin C; both which are known to play significant parts in stabilizing the inner membrane of the cell nucleus. Researchers found that the mutation responsible for progeria causes the LMNA gene to create an abnormal kind of the lamin A proteins; in laboratory tests involving cells extracted from progeria patients. That single abnormal protein may destabilize the cell’s nuclear membrane that might be injurious to the tissues that are subjected to aggressive physical force on a routine basis like the cardiovascular and musculoskeletal systems.

However, at least half a dozen other genetic disorders which include two not so common forms of muscular dystrophy have been shown to share the same common mutations in the LMNA gene. Most scientists that are involved in aging in the genetics department usually use the research on the underlying genetics of the progeria prototype in order to discover how the aging process works. Sufferers of Hutchinson-Gilford progeria have a very severe hardening of the arteries or rather severe arteriosclerosis that starts right from childhood. The condition increases terribly, the probabilities of experiencing heart attacks or even strokes at very young ages. These conditions may become life-threatening for certain individuals with this type of syndrome.

Progeria thankfully is very rare and only 1 in 4 million newborns worldwide have been known to suffer from it. Only slightly over 130 cases have been reported and recorded in scientific journals since the first time the genetic disorder was first described in 1886. People with progeria inherit this disorder by one copy of the altered gene in each cell which is small enough to cause the disorder. If a person wants to know if he/she has Hutchinson-Gilford progeria, they have to be diagnosed since before the age of one as progeria is a significantly noticeable disorder and children with progeria do not develop normally and have an average lifespan of only 13 years old, sadly.

How should society deal with these disorders? According to Ashley Hegi (deceased); the society should not judge people who suffer from progeria and they should not stare as people with progeria are no different from others, except that they have very aged looks for their age and bald heads. Societies should also be more aware of this genetic disorder although it is rare for their knowledge so they would know how to handle it and also how to treat a person with progeria. As people with progeria do not have lower intellects than a normal human being, they like to be talked to normally and lead normal lives although sometimes it may be painful to do so. They need a lot of positivity in their lives to encourage them to continue going on despite their disorder and all of its setbacks. As progeria sufferers are known for their bright and sunny dispositions, the people around them should be supportive too and their families should be trained and well learned on how to handle them with care and how to provide good and stable emotional support for them.

What should researchers and society do to help progeria sufferers? They should continue researching for cures for this disorder as little to no drugs can help with this disorder especially that it is such a rare genetic condition. Research should be done on the LMNA gene and also on the protein Lamin A as these two might be clues as to how the aging process happens so rapidly in progeria sufferers. Geneticists should pay more attention to this particular disorder as aforementioned; might be a key to the aging process and all its setbacks including links to cardiovascular diseases and so on. Progeria albeit rare should not be overlooked. 



-The nocturnal one-

Why I support halal/kosher slaughter as an animal activist.

First things first, let me get some things straight. I am not a Muslim, neither am I a Jew. But I do support kosher/halal slaughter instead of normal inhumane ways.

Denmark has just issued a ban on the methods of kosher/halal slaughter. So, killing a perfectly healthy baby giraffe (Marius) in public is permissible but a perfectly healthy method of slaughtering animals isn't. Look at the bigotry oozing out there. I can feel it seeping out of their pores.


http://www.independent.co.uk/news/world/europe/denmark-bans-halal-and-kosher-slaughter-as-minister-says-animal-rights-come-before-religion-9135580.html

Despite resistance from RSPCA (a respectable animal welfare organisation) and many other groups, the ban has been called for. It really is anti-Semitic isn't it; anti-Muslim as well. Where is the freedom of speech, and religion that Europe boasts about so much?? So they only allow freedom AS LONG as it means religion is NOT involved?? There is no extremism/barbarism here, really. Only from the side of the ministers who have been involved in the banning of the production of these kinds of meat (kosher/halal).

Now, to be unbiased so as not rock the same boat as these idiots, I will show you the kosher/halal methods of slaughter and then the normal way of slaughter.

The Halal method

With the halal method of slaughter, there was not change in the EEG graph for the first three seconds after the incision was made, indicating that the animal did not feel any pain from the cut itself. This is not surprising. Often, if we cut ourselves with a sharp implement, we do not notice until some time later. The following three seconds were characterised by a condition of deep sleep-like unconciousness brought about by the draining of large quantities of blood from the body.  Thereafter the EEG recorded a zero reading, indicating no pain at all, yet at that time the heart was still beating and the body convulsing vigorously as a reflex reaction of the spinal cord. It is this phase which is most unpleasant to onlookers who are falsely convinced that the animal suffers whilst its brain does actually no longer record any sensual messages.

The Western method

Using the Western method, the animals were apparently unconscious after stunning, and this method of dispatch would appear to be much more peaceful for the onlooker. However, the EEG readings indicated severe pain immediately after stunning. Whereas in the first example, the animal ceases to feel pain due to the brain starvation of blood and oxygen – a brain death, to put it in laymen’s terms – the second example first causes a stoppage of the heart whilst the animal still feels pain. However, there are no unsightly convulsions, which not only means that there is more blood retention in the meat, but also that this method lends itself much more conveniently to the efficiency demands of modern mass slaughter procedures. It is so much easier to dispatch an animal on the conveyor belt, if it does not move.

-http://www.mustaqim.co.uk/halal.htm-

And then, from The Guardian itself.

• A friend did a documentary on slaughterhouses in the early 90's. He saw cows on an electric grid recieve a jolt. Workers then went through with hammers to bash in the skulls of those not dead. He instantly became a vegetarian. Kosher methods were created with the best interests of the animals in mind. (Taking as a given that man's status at the top of the food chain does not impart the right to cause extra suffering.) The knife must be free of nicks so that the blade moves as fast and cleanly as possible. There may be more humane ways to kill a cow or chicken, but I doubt they are cost effective from a mass-production standpoint. Kosher meat is double or triple the price of non-kosher. Perhaps you could find a rancher who would be willing to let you kill a cow as gently as possible (wait til it's asleep? or give a gentle sedative?). But from a practical standpoint, an animal slaughtered kosher is likely to have suffered less than the non-kosher one.
• 
  
• -Wraith, Salford, UK-

• I can't speak for Halal slaughter, but I do know about Kosher slaughter of animals. The "Shochet" - slaughterer - uses a special knife which has to be incredibly sharp and completely smooth (any nicks in the blade might tear the animal's flesh on the way down and cause pain). The knife should be able to cut smoothly and cleanly through the neck. The cut is made in a specific place in the neck, and although death isn't instantaneous (no method of slaughter is), the animal loses consciousness immediately due to the sudden drop of blood pressure in the head. Complete death results shortly after. As a comparison, let's look at some other methods of slaughter which are used. The "Captive Bolt Gun". Imagine a normal gun, but the bullet has a string attached to it so it flies out of the barrel, but can be reset and re-loaded again leaving nothing behind. Now take an animal, press this up against its skull and fire. Bolt goes into skull and pulled out again. Requires 4-5 shots normally to cause complete death, during which animal is in agony. Electrocution. Let's compare this with human executions in the electric chair. There have been cases where people being killed in the chair have caught fire and had smoke coming from their heads while still showing signs of life. Witnesses have been sick from the stench of burning flesh. Some researchers have said that being killed by electrocution would kill you by oxygen starvation (you couldn't breathe due to paralysis from the electricity), while at the same time being burnt alive. So much for a painless death! As long as people wish to eat animals (don't get me wrong, I'm a steak lover), we have to kill them first. And I truly believe that "Shechitah" - the method of slaughter for Kosher food - is the least painful for the animal concerned.

-David Dreaming Bear, Horesethief Canyon, California USA-

• 
  

• Studies have been conducted in which monitors were placed on the brain of a cow being slaughtered using shechitah (kosher slaughter) and it turns out that it is actually 100% painless. another interesting note is that 2500 years ago, when the rules of kosher were made, it was said that the animal must have split hooves and chew it's cud. interestingly, now 2500 years later we know a great deal of biology and it turns out that the only species of animals that have split hooves and chew their cud, also just happen to have a certain artery that goes directly to their brain, as opposed to their heart as in all other animals (including humans). when this vein is cut it will be a painless death, because the animal will be instantly unconscious, whereas if you tried to slaughter any other animal in this method it would cause a lot of suffering. so, to answer that question "would YOU like to be shot in the head or have your throat cut?" well, i being human would prefer a shot in the head for the least painless death, but if i was a cow, or any other animal with split hooves and a cud, i'd be all for the throat slitting for the painless death. also, to clear up the misconception about the animal still being alive while bleeding to death with shechitah - this is true that the animal may still be living, but with the brain entirely unconscious it still does not experience any of the pain of death. the food industry will do whatever necessary to reduce cost, but if you truly care about the well being of living things, buy kosher.
• 
  
• -Seth, Edinburgh-

• 
  

• Arguing over which methods of killing are more humane seems a bit pointless since the only ones who could tell you are dead.
• 
  
• -Lauri Reizes, Los Angeles USA-

I'll let these points speak for me. I'm not alone. Many other rational activists believe that kosher/halal meat is better than normally-slaughtered meat. For Denmark to have banned the more humane ways of slaughtering animals without further research shows how "shallow" the people there are (those who called for the ban). Also, Britain should really do more in-depth research regarding this topic instead of being all dumb about it and banning it because of what some irrational activist groups are saying. 

I agree that there are certain kosher/halal slaughterhouses that do not use proper equipment. Shut them down instead of banning them in whole. There is no point in making a huge controversy out of this. I understand that we all want these animals to die in the least painful ways possible. Therefore, research should be done first using scientific methods (EEGs etc.) before causing ruckuses about it. 

-Animal lover-

Popple the Wriggles and I :)

Desperation and Anwar.

All our news feeds have been littered with Teresa Kok's funny video and then, not long after, news of it being slanderous. 

It's strange isn't it, how our country's leaders find fault in such minute details like a video that was meant to be purely satirical and not just realize the motives behind stuff like that??

Firstly, Malaysians need to get a funny bone. Secondly, to attack one's person is a great fallacy, dear UMNO cronies. If you want to attack Teresa Kok for her video, do so but with a little more dignity please. It's an ad hominem to start attacking her for her name.

I have been so appalled by the state of the country that blogging seemed like a hugely, strangely, overwhelming task. They want to condemn Teresa for her satirical video which actually rang with truth but they allow the state of the country to worsen. They keep thinking that us Malaysians are stupid enough to have wool pulled over our eyes time and time again.

The police are now asked to investigate the "Onederful Malaysia" video. The police are so free to conduct such investigations and lose track of what they really are supposed to do; protecting citizens and ensuring peace in the country. My bag got snatched from me in 2012 and until today, NOTHING can be recovered and the snatch thieves weren't caught. It was such a traumatic experience and I wouldn't even wish it upon the people I despise the most. Why can't the police deal with issues like these?? Why can't they deal with issues like abuse and more important things?? When people report abuse, it's automatically domestic and has to go to a different office altogether. 

Some Malays of this country also have to stop being so butthurt and claiming that every single tom, dick (ooh I said a bad word, immolate me, right??) and harry are out to get them and their religion. They'd rather vote for people like Felixia Yeap and her wrapped up self because she is "oh so sweet" and vixen-like. And UMNO, you don't represent all the Malays in Malaysia because most of them are extremely intelligent and see past your nonsensical acts.

Another thing I'd like to mention is 'Anwar'. Look, we're not stupid alright?? We know HE was the one who truly started playing racial politics all those years ago. But fuck us right?? We have no leader now  and are desperate for a change. It's not like we can find anyone better anyway. We're in such desperation that anyone proposing a change for the betterment of Malaysia would do. I mean, if anyone proposes such huge changes for the country, of course, we'd jump at the idea of it immediately. We used to be a picky lot. Badawi was too cowardly and calm. Mahathir was too tyrannical. But now that we've seen THE ultimate WORST, I guess all we can do is hope for a better one. It's no longer a war of who's going to make a better prime minister. It's more like a war of survival for us other than the Bumiputeras in Malaysia, thanks to the cronyism of the current government who BLATANTLY promote racial politics and play it down to the core. 

If you're planning a Malaysian genocide, consider it done. Just DO NOT complain about a brain drain after that and all the other things you complain about. If we're that bad a bunch as non-Bumis, we'd rather take off than listen to you telling us how bad we are and how we're "stealing your economy" and shit like that.

Enough IS enough.