Living with MPEI

Niamh's underlying condition has left her with severe brain damage. Although she can smile at noises and touch, she does not use her vision, she lost the ability to hold her head at about 2 years old and was never able to sit independently.

At the age of 2 she was able to take weight through her legs with support, but by the
age of 3 she had lost this and could only stand using her standing frame

Seizures for Niamh were continual. On a good day she would get away with about 20 and on a bad day over 100. Anti-convulsant drugs were never very effective for her but the\Ketogenic Diet worked well. Her seizures used to stop when she was ill.

She had weeks where her gut and bowel just work in reverse (anti-peristalsis) and she would only tolerate water. She is on lots of anti-reflux and anti-sickness medication, antispasmodics, and anti-inflammatories to stop her stomach bleeding (gastritis), and to reduce her sickness (reflux). She has also had two operations called "fundoplications" to tie knots in the top of her stomach to stop her being sick. Despite these she can still be sick.

From the age of 18 months Niamh experienced prolonged bouts of screaming, arching and throwing her head back. Ititially it was thought to be reflux pain but numerous hospital investigations could find no obvious source for these episodes considering Niamh was on every drug for reflux known to man and it was put down to cerebral irritation. - the medical profession call it cerebral irritation as they really don't know what the cause of her distress is.
These periods lasted for weeks and months at a time.

At aged 2 we started to notice that she had developed a curve in her spine. This is called scoliosis and this became progressively worse as she got older.
We attempted to limit the progression of this problem by careful positioning and wearing a back brace but Niamh's arching/spasms were just too strong and despite our intervention the curve became worse.
Niamh's curve was rotational which meant that her spine was twisting (in a clockwise direction). This led to knock on problems for other organs in her body.

She alos suffered from "windsweeping of her hips" which meant that she had a tendecy to let her legs fall over in one direction when she was lying on her back.
Niamh's direction was left which eventually led to her right hip becoming dislocated.

At the age of 2.5 Niamh started experiencing trembling of her fingers and toes (similar to that experienced in Pakinsons disease). This underlying movement was followed within a few months by much stranger movements that were quite frightening to watch. These were called Dystonic Spasms. 
 

Niamh is not consistently able to fix and follow object placed in her visual field. There is nothing wrong with her actual eyes, but instead, the area of her brain responsible for interpreting the information she sees, does not work correctly. This is known as Cortical Visual Impairment.