Niamh's Story in Detail

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Niamh's Journey

Underlying Disorder & Loosing her Fight

In 2008, a routine MRI revealed a progression of damage to part of Niamh’s brain called the basal ganglia and brain stem. Her doctors explained that this indicated that her underlying problem was probably metabolic and likely to be of mitochondrial origin. They said that the images they had seen indicated that her condition was fairly far advanced, and that it was degenerative. They likened the changes to Leigh's Syndrome - a mitochondrial disease.
We were given an approximate timescale of a further 1-2 years so we decided to make the most of our time left with her.

We took her to Lanzarote for our summer holiday (oxygen, suction machine, saturation monitor, feeding pump, drugs and all) which she loved and when we got back we threw her a big party for her 4th birthday.

niamh in float
Conor giving Niamh her dummy-1

In July 08 we nearly lost her when she stopped breathing at school. It seems that this episode was only a precursor to a prolonged state of Status Dystonicus where she went into permanent dystonic spasm and her rescue drugs would not work.

She ended up being taken into Great Ormond Street Hospital where she was kept heavily sedated in an attempt to stabilise her. After this episode, she never fully recovered.

Although we were discharged from hospital with her (now oxygen dependent) and heavily sedated, over the next few months she had very little time awake and not in spasm.
She needed sedating 2 or 3 times a day and night but she was able to enjoy a few hours a day of quality time with us, her nurses and her friends before having to be put back under.

Christmas 08 she was on good form during her waking hours, but over New Year she caught a bug that was going round and it developed very quickly into pneumonia.
Niamh struggled with this pneumonia and remained unconscious for nearly 2 weeks. We were warned that this may be the end for her but as usual Niamh proved them wrong and by mid January 09 she began to waken and had dropped from needing 12litres per hour of oxygen to only 4litres per hour.

We though she had done it again - defied medical science and pulled through the un-pull-through able.

However during her pneumonia her stomach and gut had failed. Tragically she was not able to kick these back in and she could not tolerate even the tiniest feed, diluted milk, Dioralyte and then not even water.

During this period her seizures came back with a vengeance and she required intra-muscular phenobarb-morphine-midazolam via a syringe driver to even keep her comfortable. All her other medications were stopped as they could not be given by any other route other than into her stomach which was bleeding heavily now.
Over the next few weeks Niamh's breathing became erratic and irregular, changing from very slow (8 breaths per minute) to
rapid (40+ breaths per minute). She lost control of her temperature and would spike to 40.5'C then very quickly her arms and
legs would become freezing.

Her heart rate would also range over the day from very fast (over 200bpm) to below 50bpm.
We realised that finally her brain stem - central to her vital functions - was just not working.

On the last few days she was with us, her heart rate dropped as low as 30bmp and no amount of oxygen could keep saturation
levels above 60. She held her breath frequently and her respiration rate was very slow.



Niamh passed away on 9th February 2009 in the arms of her Mummy, while her Daddy kissed her head.