Chronically Fabulous · Femme

What Are HNPP and MCTD? // My Disabilities

In today’s blog: HNPP, MCTD and POTS! What the hell are those I hear you ask? Exactly.

Exactly.

I’ve been procrastinating awfully about making this blog for three main reasons: (1) It makes me sad to think about sometimes (2) my disabilities are just part of me, knitted into the very fabric of my being, so it’s difficult to explain the differences to other people because… this is just who I am. And (3) I find disabilities very boring.

But I’m going to keep this Vaguely exciting for you!

I have two disabilities from which other symptoms and conditions come. They’re both genetic, I didn’t catch them from anywhere and I can’t pass them on to anyone through contact although sometimes I pretend I can when people are rude because that’s always faintly amusing. They are: Hereditary Neuropathy with Liability to Pressure Palsies (HNPP) and Mixed Connective Tissue Disorder (MCTD).

HNPP is a disorder of the peripheral nerves- that’s the part of your nervous system outside the brain and spinal cord. It includes the cranial nerves and spinal nerves as well as their roots and branches. These nerves control the functions of sensation, movement and motor coordination.

In the most simplified terms:

Our nerves are supposed to have a coating around them called the myelin sheath, much like plastic insulation around electrical wires. I’m missing one of the two genes that creates that sheath so my myelin is very fragile and more susceptible to injury. Just the slightest pressure, stretch or repetitive movement on the nerve causes sections of the myelin to be lost leading to weakness and palsies.

Due to this, signals don’t get to my brain properly. It can take me a very long time to process anything and I have to be vigilant with my limbs, making sure they’re not too cold or have picked up an injury I didn’t notice.

Palsies (bits of me being paralysed) can last for days, weeks or even years. There is no way of knowing when they will come back or to what degree.

Next!

Mixed Connective Tissue Disorder (or ‘Disease’- doctors can switch between the two in the same sentence, oddly) is an autoimmune disease in which the body’s defence system attacks itself. It can have arthritic, cardiac, pulmonary and skin manifestations.

MCTD causes chronic joint pain, muscle inflammation, hyper-mobility, pulmonary hypertension, random fevers, digestive issues, fatigue, problems healing, joint dislocation, swelling and malaise.

Malaise is possibly the weirdest symptom you’ve ever heard of. It means ‘my body is saying something isn’t right. I don’t know what… but it isn’t right’.

As such, my immune system is terrible. If I stand next to an ill person I will get whatever they’ve got. If they sneeze near me then let’s just write off the next month. I’ll be in bed.

Around 80% of people with MCTD will have problems with their oesophagus- Personally, I find it incredibly hard to keep food down and sometimes I struggle swallowing. Dry food is my enemy and yogurt is my friend. I will eat soya yogurt with savoury food if I have to and it’s actually surprisingly delicious.

80% of people will also have problems with their lungs.

Not necessarily the same 80% as the oesophagus!

Only 10% of people have neurologic abnormalities and I’m unfortunately one of them. I have cognitive issues and I get vascular headaches- which feels like all of the muscles and blood vessels across my head are tightening and crushing my skull!

Secondarily is POTS, Postural Orthostatic Tachychardia Syndrome,

This affects the body’s autonomic nervous system- the things your body does without you having to think about it. My body thus just doesn’t regulate itself. I get hot flushes in the snow or on the beach, I start freezing on a warm summer’s day or a crisp autumn one and I randomly faint because my body has suddenly realised I’m standing. 

It’s worse if I’ve done something the day before or haven’t gotten enough sleep but it can also just happen whenever for no reason.

The crossover of my disorders also affects my hearing, vision and energy levels. Very sweetly, my body is just constantly trying to heal itself.

 

Frustrating.

New stuff seems to go wrong with me all the time and it’s horribly frustrating because just when I think everything is under control… suddenly it isn’t again!

But life with a disability isn’t all horrible and it doesn’t mean I have to be miserable.

 

 

The Happy Ending

If you’re reading this because you or someone you love has just been diagnosed with one of these conditions, the most important thing I can say to you is: it’s going to be okay. As a teenager I had all of these big plans about how my life would be, but then I paralysed both of my arms for a year and a half, had a medical procedure that went wrong and spent two years going in and out of hospital. It took me twice as long to do my A Levels as everyone else and it was bloody hard- I had to dictate my answers whilst lying on the floor in the dark. But I did it. I was determined to go to university and I did. I was determined to find someone who would love me regardless and I did.

It is going to seem like such an uphill struggle after your diagnosis, but this is just a part of who you are and it’s your life now. Find the fun, find what makes you happy, give yourself a break but keep achievable goals in mind and you’ll be okay.

And yes, some days all I do is vomit but I’m married, and I own a house and I have two adorable little doggies so I’m proud of how far I’ve come.

Classical Film Reviews · Film Reviews

Classic Film Review: 12 Angry Men Review [1957]

Most directors, when handed a script, will consider a variety of locations in order to broaden the story. They will insert as many different backgrounds as time, money and narrative will allow. After all, wouldn’t it be terribly boring to watch all of the action taking place in just one room? The genius of Sidney Lumet’s potent 12 Angry Men is that we forget all of the action takes place in just one small, claustrophobic space because the dialogue and story move at such a pace!

As the film progresses, Lumet gradually moves the walls of the studio set closer and closer in; he changes camera lenses to reduce the apparent distance between the backgrounds and the figures prowling the room- ramping up the tension even further.

These 12 men are a jury who must decide the fate of an 18-year-old who is accused of stabbing his abusive father to death; if he’s found guilty, he’ll go to the chair. Eleven of the jurors are convinced he did it; juror No 8, played by Henry Fonda, isn’t so sure, and gradually his doubts infect the others. The bigotry and stupidity so well established at the outset are so convincingly overcome by a combination of compassion and clear thinking that the audience can marvel at how nothing but dialogue can affect change.

Although Fonda’s character has a great potential for sanctimoniousness, he admirably reins it in. Conversely, the “bad guys” in the room, Number 3. Played by Lee J Cobb and Number 10, played by Ed Begley, let rip with their monstrous creations – with mesmerising effect.

Yet we feel sympathy when No 3 is finally crushed by a realisation of his own inadequacy as a father.

By far the most spellbinding moment of the film, however, is when No 10’s racist rant prompts the others, slowly, one by one, to leave the table and stand with their backs to him, each an icy tower in this boiling hot room.

We’re in no doubt from the opening moments that the room is an oven: it’s the hottest day of the year, there’s no air conditioning, and the single, wall-mounted fan seems to be out of order. Beads of sweat glisten on foreheads; shirt backs become increasingly damp.  It’s no wonder these men are so desperate to leave the room and want nothing more than a, easy verdict- unfortunately, ‘easy’ seems to mean the death sentence for a teenager.

Screenwriter Reginald Rose’s fundamental thought is that the somewhat disturbing legal term, “beyond a reasonable doubt,” should not be regarded as a flat phrase casually coined by the law-makers. The defendant involved (whom we catch merely a glimpse of in the opening shot), is a tough 18-year-old from a broken slum home charged with having stabbed his brutal father, a former convict. All but one of the jurymen are convinced this is an open-and-shut case. Number 8 does not assert that the boy is innocent, but that the conduct of the trial, especially that of the defence lawyer, has left him with gnawing doubts.

It’s likely you won’t notice, so wrapped up will you be in the unfurling of an absorbing tale through dialogue that is both complex and unassuming, but the camerawork in this film is truly magnificent. At the beginning, the cameras are all positioned above eye level and mounted with wide-angle lenses to give the appearance of greater distance between the subjects. As the film progresses the cameras slip down to eye level. By the end of the film, nearly all of it is shot below eye level, in close-up and with telephoto lenses to increase the encroaching sense of claustrophobia.

 

Film Reviews · New Film Reviews

New Film Review: Battle Royale [2000]

In the near future, Japan’s economy has collapsed with unemployment soaring to 15 percent and gangs of youths acting in open defiance of authority figures- a particular ‘no no’ in Japanese culture. Although it is never specified whether this is a global issue, Japan has responded to the impending social catastrophe by passing the Battle Royale (or ‘BR’) Law, requiring that each year a ninth grade class be randomly selected and sent to a remote island where they’ll be forced to kill one another with a variety of weapons until only one student remains. Any student refusing to participate will be killed.

It’s a premise that promises a subversive smashing of taboos as children are killed on screen. But… it’s a fairly standard dystopian conceit beefed up by including children. The death of a child on screen is a social pressure point that retains its shock value despite repetition.

The film aroused both domestic and international controversy and was either banned outright or deliberately excluded from distribution in many countries. However, it was a mainstream domestic blockbuster and became one of the ten highest grossing films in Japan.

 

As tasteless as the premise is, it also has potential for satire, especially when it comes to technologically savvy children and how scary they can be for adults. Parents often respond to that fear by condescending to them: short-changing a child’s capacity for understanding as well as for cruelty.

The notion of children running around a creepy island gunning each other down can certainly resonate with a world that’s haunted by problematic gun-control laws, as well as the school shootings that periodically disturb our illusions of stability and sanity.

But…

Battle Royale squanders its considerable potential with a harsh juxtaposition between the smugly leering opening scene- involving last year’s smirking bloodsoaked winner- and a temporary trip into a moony, melodramatic mood that’s meant, one assumes, to reflect the dreams and fears of your typical moony, melodramatic 15 year old.

But the romanticism doesn’t mesh with the show-off nihilism of the opening and both distract from the ultimate story, which is of the current class that’s subjected to the Battle Royale.

This poor class is let into the bad news by a grave former teacher who explains the rules of the game to the new contestants while casually killing a few of them for insubordination— death staged here as a punchline. Each child must then snatch up a backpack containing food, a map, and a weapon of choice. And then flee into the wilds of the island! … and slaughter each other!

Unlike The Hunger Games, the ruthless opportunism of TV doesn’t appear to be the subject of the film’s satire, as the battle itself doesn’t appear to be televised. The last 30 minutes are awful, inexplicably morphing into an inspirational tale of transcending abuse. We’re supposed to suddenly feel empathy for the monstrous teacher previously shown as all too willing to kill his former students in the name of bureaucratic nonsense.

The film’s director, Kinji Fukasaku, decided to direct the film after connecting with the novel it is based upon. 15-year-old Fukasaku was made to work in a munitions factory during World War 2. When the factory came under artillery fire in July 1945 the children were unable to escape and dived under each other for cover. The survivors were forced to dispose of the corpses and at that point realised their government was lying to them.

THAT is the film I want to see. I enjoy the stylisation of Battle Royale- it’s just missing a beating heart.