Right now Ella is on continuous bipap. There has been the results of tests coming through. there were a series of tests relating to metabolic mitochondrial disorders, on of them showed a reduction in the enzyme cytochrome oxidase. cytochrome oxidase help cells use oxygen as fuel. the reduction is not thought to be alarmingly low just enough outside of the normal range to be noteworthy . It is unclear as to whether this is diagnostically significant.

Ella had an immunological test done, which found an elevated number of memory b cells (cd19 + cd27) The significance of this is also unknown.

Her condition has deteriorated although she remains in good spirits considering she can not speak now, can only move her legs and hips and is bedridden apart from the short time she spends in a special chair on wheels that enables her to leave the hospital room she is confined to. she was at home for two weeks for the first time in 4 months but we took her back to hospital on monday as she had to have her monthly dose of ivig and she also was not looking too well.

blood tests revealed that he hemoglobin levels were seriously low at 60 where the normal would be 100-120, she was very pale, tired and irratable. it is not clear what happened but a blood transfusion got her up to 90 and over the next few days she has climbed to 98 herself which hopefully means that she is ok in this respect.

we were hoping that this might be a clue of some kind to her disorder which continues to remain a mystery. a nerve biopsy has shown severe axonal degeneration and secondary mylein damage. chronic inflamatory demyleinating neuropathy was a possible diagnosis but that seems to be unlikely or impossible when all test results are considered. i.e the nerve biopsy shows axonal damage not just demyleinating damage.

ella eyes have started to twitch now. not the eyelids but the eyeball themselves. it looks as though the optic nerve may be affected. the situation is very hard now, it feels sometimes like things are getting worse by the week but we want to believe that she will get better.

it is very upsetting when you think about her life now and in the future. she is such a character though and we are grateful for what we have right now but it seems to be slipping away slowly.