1000 NIGHTS AGO...

 

1000 nights ago…

 

She went upstairs to her bedroom.

And closed the door.

1000 nights ago.

Not left the room since.

Well, perhaps three times.

Once a week she moves to the other bedroom for a couple of hours, while her room is cleaned and bed sheets changed.

Otherwise, only summer heatwaves have driven her from her bedroom haven, to seek cooler climes – in the cellar for a few days and nights.

To a spare bed set up especially, plus medical supplies, drinks, toilet.

That migration, down the stairs, can take up to  two hours.

With a rest period or even a night stopover in the living room, before the final descent.

One day, we hope, she will return, to join us downstairs.

But in the meantime, her life is on hold. 

Halted because of complicated medical issues these past two decades. It was thought to be ME. Took her out of school for her teenage years.

Then came a blessed recovery of sorts, a hopeful interlude 15 years ago, allowing a measured return to education, mindful to take regular rest periods.  Her health improved, even to the extent she travelled to the States, but convalescing was an element during her stay.

The high point came in moving away from home to digs for college.  Culminated in a degree in (TV and film production). She did a mid-term spell with CNN, London.

A driven character, full of energy, an organiser. Her managerial skills made her a natural leader of production teams making films as part of her degree course.  Well liked, she had many friends. She doesn’t suffer fools gladly, even now!  Used to be able to talk anyone under the table on current news issues.

Then came relapse, and a return home, with her furniture – now stored in the garage.

At last, she had the first diagnosis: POTS (Postural Orthostatic Tachycardia) – when too little blood returns to the heart).  Triggered by movement.

This from a private heart specialist, who subsequently arranged further tests at Kings College Hospital, Denmark Hill.

The second diagnosis – from the Royal Orthopaedic Hospital at Stanmore –Ehlers Danlos type 3 joint hypermobility syndrome. This leads to chronic muscular pain, can cause dislocations.

1000 nights.

EDs weakens the entire body, makes moving around painful.

A gradual degrading process.

When POTs kicks in the heart will suddenly increase to beat very fast and is combined with drop in blood pressure: result, dizziness, risk of feinting.  

When this occurred while out, she became scared. No idea what it was. She learned to sit down on a bench until it subsided.

In the beginning a few days rest would provide respite, until the next time, perhaps in a day or two.

Days regularly became punctuated with stops to allow rest and recovery.

She became fearful of travelling, of even going outside, so she stopped,

1000 nights ago.

Ehlers Danlos is a genetic condition, which weakens the connective tissue.  That’s the glue which holds bones, muscle, and all our internal organs together. Imagine a bendy chassis of a vehicle. It wouldn’t roll so well, if at all.

There is no cure.

Sensory disorders followed – hearing: too loud; sight: sudden movement too fast; colours – too bright.

This led to curtailing social contact with friends. There would be no more visitors to the house.

It became a quiet house.

She no longer viewed or sent emails, would not take phone calls. No television, no radio, very little reading. No newspapers.  No playing CDs, until recently – slow, quiet meditative sounds.

She maintains a simple, short exercise routine, as recommended for her condition.

Time has stood still. 

1000 nights and counting.

Birthdays go unacknowledged, as does Christmas, Easter, neither is celebrated. Too much for the brain to take in.

But she does whisper greetings, with a smile, to her parents, her carers.

And every day there are hugs.

She will permit herself a laugh occasionally, at the juggling antics of her parents taking things into and out of her room. Rare light moments. Usually she is lying still, to control POTS.  Eyes closed. Sound deadening headphones on for much of the time.

Very occasionally, she has expressed a wish to be free again.

One day is much the same as another.  Day is followed by night, which is often sleepless.

1000 nights.

Punctuated throughout each day by her carers to maintain her many needs.

After a while it was realised she also has PDA – Pathological Demand Avoidance. Which means avoiding doing anything. It is  common on the Aspergers / autism spectrum. This is undiagnosed, but no matter, she ticks all the boxes.

Also ticks the box for Obsessive Compulsion Disorder, when everything has to be done in a certain way.

Anxiety is of a higher order than you can ever imagine, is perhaps the most wearing and tiresome for the patient – and for the carers.

A scheduled home visit by anyone, gas boiler engineer, electrician, doctor on a rare occasions – there is no regular medical review.  These visits cause days of anxiety beforehand, and days of exhaustion afterwards.

Research into anxiety and its cause among those with EDS Joint Hypermobility has revealed that the area of the brain controlling anxiety is much larger than normal: making it Impossible to “feel the fear and do it anyway”.

Researchers have decreed that there is “the genetic predisposition to anxiety.  It seems there is a significantly higher prevalence of autonomic nervous system symptom (dysautonomia) in joint hypomobility patients.”

… “Processes compromising function in neuro-developmental conditions may occur in individuals with hypermobility – enhance vulnerability to stress and anxiety.”

1000 nights.

Then there are meltdowns; frenzied fury at something or other, flying fists, kicking - screaming, crying, swearing… fucking this and fuck that, Shiting this shitting that……

When it’s over it’s as though nothing happened.

She takes care to avoid Hypoglycaemia, which can lead to sugar crash.  She knows the symptoms – often has crackers or protein drink to hand, or will call for immediate food.

Her slow digestive tract means rather than say three set meals a day, she requires several small meals during the day.

The kitchen can be a busy place from morning, noon, through until late.

1000 nights.

 

Hypermobile Ehlers-Danlos syndrome type 3 (hEDS) is generally considered the least severe type of EDS, although significant complications, primarily musculoskeletal, can and do occur. The skin is often soft and may be mildly hyperextensible. Subluxations and dislocations are common; they may occur spontaneously or with minimal trauma and can be acutely painful. Degenerative joint disease is common. Chronic pain, distinct from that associated with acute dislocations, is a serious complication of the condition and can be both physically and psychologically disabling.

 

Postural orthostatic tachycardia syndrome (POTS) is a condition characterized by too little blood returning to the heart when moving from a lying down to a standing up position (orthostatic intolerance). Orthostatic Intolerance causes light-headedness or fainting that can be eased by lying back down.

 

HOW COMMON IS PoTS? The incidence in the UK is unknown. However, it is probably under-diagnosed due to lack of awareness and non-specific symptomatology. It is five times more common in women and tends to affect people age 15 to 50.1 Apr 2016

Dysautonomia International estimates that POTS affects between one and three million people in the U.S. The majority of them are women.