Tag: rare

A Disease That Doesn’t Let You Sleep And Kills

By Anupum Pant

Background

In my very first article on this website, I talked about a 17-year-old boy, Randy Gardner who remained awake for an incredible 11 days and set a world record which no one had beat for a very long time. And then, in the 90s, Toimi Soini, of Hamina, Finland, set a new record of 276 hours of sleeplessness.

Well, no one should even try to beat it because depriving yourself of sleep can have some serious health issues and can even kill you. The seriousness of health consequences associated with such attempts is the reason that these records are no longer recorded in the Guinness books.

The disease

Not always do people decide to voluntarily break sleeplessness records. Some times, they can be the victims of a horrific and an extremely rare disease called fatal familial insomnia (FFI) – A progressively worsening form of insomnia discovered only 10-15 years ago. When it affects someone, the person starts having bouts of insomnia at first and then they aren’t able to sleep at all. Hallucinations, delirium, and confusional states occur and a person usually dies within 18 months from the first insomnia. 

The disease is caused due to a defective gene and the people having it start seeing symptoms from the age of 30 (never at an early age). It has been believed to have originated from an Italian man in the year 1765 (not necessarily).

Symptoms in detail

  • For the first four months there is an onset of insomnia and the person starts getting panic attacks and unfounded phobias.
  • For the the next 5 months the hallucinations become severe.
  • In the next 3 months, rapid weight loss and serious deterioration of mental ability happens,
  • Next comes dementia, unresponsiveness, and may be death.

[A list of couple of people who never slept]

Here’s a 10 minute documentary which discusses FFI in detail.

[Everything else you need to know about it]

An Extremely Rare and Bizarre Disorder – Alien Hand Syndrome

By Anupum Pant

Like lakes, bizarre and rare disorders also fascinate me. Of course I would never want to experience one of these, but it’s good to know about them. Besides the horrible, body-turning-into-stone disorder, Alien hand syndrome is one of the most bizarre disorders I’ve heard of.

If someone has the Alien hand syndrome, they’d have a hand that would move around and do stuff on its own without the person even being aware about it. And I’m not talking about those involuntary muscular movements you have once in a while. In this, the hand moves as if it can think for itself. It moves as if it’s being moved by “someone else”. Some times, it becomes necessary to use the other hand to stop it!

Imagine your left hand grabbing an object and you just can’t let it go.

It happens when the two hemispheres of the brain get separated either surgically or by accident or disease. In that case, the left and right hemispheres are unable to move information between them.

It isn’t just rare and bizarre, it’s extremely scary too. Imagine if your left hand waking up at night to murder its own host. At night, it’d like sleeping with a stranger. In fact, it’d be like living with a creepy stranger all the time. Who would want that!

Like the following video puts it, it seems as if there is another intelligence at work here, the one which is not known to the patient.

[Wikipedia page]

Please hit like if you learnt something today.

Horrible Rare Genetic Disease Turns Humans to Statues

By Anupum Pant

Background

At an early age, the horrible, rare and poorly understood disease that we’ll discuss today – FOP – starts producing tumours in the body. Doctors usually assume it to be the fairly common cancerous tissue but when it turns out to be FOP, everybody panics. Yes, this one is more ruthless than cancer. How?

Well, FOP (Fibrodysplasia ossificans progressiva) turns humans into statues. It is also known as the stone man syndrome. It is an extremely rare genetic disorder in which the muscles and other connective tissues in the human body start turning into bone. Some times it happens automatically, other times it happens when the tissues are damaged. Gradually, the patients who are affected by FOP are imprisoned in their own skeletal prison.

FOP is one of the most bizarre diseases. And has no solid cure.

Why does it happen?

It happens because of a gene that fails to turn off. This gene is responsible to form bones in a baby’s body when it is still in the womb. Once the job is done, the job of the gene is to turn off itself. But when this deactivation of the gene doesn’t happen, the gene keeps making bones even after the child is born and ultimately turns a human into a statue made of bone.

Ashley Kurpiel: When Ashley was just 3 years old. Tumours started appearing in her arm. Doctors thought they were cancerous tissues and started taking steps to deal with it. Only when they removed her arm, the doctors realized it wasn’t cancer, it was a horror. They found, she had FOP. She was slowly turning into a statue made of bone.

Harry Eastlack: When he was 5, he broke a leg and then there were other complications while the fracture was being treated. The fracture didn’t set correctly. This made his hip and knee stiff and bone growths started on the muscles of his thigh. The transformation of muscle/tendons to bone started happening in other parts of the body too. By the time he turned 20, his backbone had turned into a single stiff piece of bone. When he was 39 years old and was almost turning 40, his body had turned completely into bone. His jaws were locked and the only part he could move in his body was his lips. Harry died from pneumonia before he could turn 40.

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Understanding the Impending Helium Crisis

by Anupum Pant

There is too much Helium?

Helium is the second most abundant element in the observable universe, present at about 24% of the total elemental mass. Helium is also the second lightest element. So, 24% by mass is too huge a mass for a single light element. It equates to a measure that is probably millions of times more than what humanity could use up in millions of years. Close to about 12 times the mass of all the heavier elements combined, this element will almost never run out. But, that is only when we talk about the universe. Back in Earth, it is completely a different story.

Helium sources for us

On Earth, Helium is relatively rare. It amounts to only a 0.00052% volume of the earth’s atmosphere. Although 0.00052% is not too less, you also can’t consider it as an abundant element. Moreover, extracting Helium from air is almost 10,000 times more costly than fractional distillation (mentioned in the next paragraph). So, all that Helium in air is nearly useless to us till better methods of extraction are invented.
Thankfully, Helium is also present under the surface of the earth. The source of this kind of deposit is, radioactive decays which take place down there. It mixes with the natural gas and is lost to space, if released into the atmosphere. It is separated from natural gas using a process called fractional distillation – The best process to make Helium.

The largest known underground reserve estimated to contain about 10 billion cubic feet of Helium is a federal reserve (mostly under Texas and Kansas). For years US reserves had been the largest global suppliers of Helium (90%). Even today, these reserves contribute to more than 35% of the total global supply. The price of Helium coming from this source has remained almost unchanged for a long time. While during the same period (10 years) privately held Helium prices have tripled. The gap in prices is increasing every day, creating a big distortion in the market.

Helium Usage

Uses of Helium range from manufacturing smart phone screens (all LCD screens) to optical fibers (Internet cables) to health care (MRI scanners) to scientific research etc. [Uses of Helium]

The Problem

Since Helium has been made artificially cheap due to the Helium privatization act, it is popularly believed to be a cheap gas and is wasted a lot. Instead of using it up for important things, we consume it by filling up party balloons, distort voices and other entertaining activities. In fact, the warning issued by the Nobel Prize winner Robert Richardson that Helium could be depleted within a generation, seems to have had no effect on us. We still continue to waste a lot of Helium, release it into the air and keep losing it forever. Not many realize that it is a non-renewable resource.

We have almost reached a crisis already, but it was temporarily averted by the congress. In the future, after about 6-7 years, when the Federal Reserve stops supplying it (at below-market prices), it could be a big problem. I’m not very optimistic about market adjusting within such a small span either. In under a decade, we’ll probably see smart-phone prices, optical fiber prices and health care (MRI scans etc.) prices shoot up precipitously due to this artificial market distortion, if we do not start using Helium properly.