So I went back to Papworth yesterday to get my results of recent tests, have been left with mixed feelings really.
CT scan showed no worsening since previous scan - air trapping and atelectasis with the obvious lung reduction from scoliosis but no evidence of bronchiectasis thankfully
LFT's showed the usual 11% functioning of small airways with lung volume of around 25%, despite this, gas exchange was as good as can be expected So, no apparent worsening.
Nasal nitric oxide was just in the normal range (8ppm over threshold) so that should safely rule out ciliary motility disorder which was being considered.
Heart echo showed right ventricular hypertrophy this wasnt present on my last echo about four years ago, and is due to the severity of my obstructive and restrictive lung disease. Trying to not think about that one too much. Having a nurse and doctor in the house isn't fun at times.
Bone density scan showed a reduction of 4% since the last one a couple of years ago (2 yrs is the minimum time between repeats). Really p'd off about this but its also unsurprising given my need for steroids. Will be starting IV infusions of bisphosphonate and have been given the usual advice about not falling over or doing silly things. Pants.
It seems then that a lot of my deterioration has been due to recurrent aspiration pneumonia and reflux - and consequent unstable asthma. This has improved a bit since being nil by mouth and getting an electric bed so I sit up at night, but waiting for an opinion now re jejunostomy so nothing goes in my stomach.
Im now dreading the summer because it usually means several months on oral steroids, and now I dont want to take the damn things because of my bones. Because of my spinal fusion and instrumentation osteoporosis is a biggy I wanted to avoid - I was always told that if I got it, it would cause big problems.
Also now being referred to the RSSC for a sleep study to see if I need night time ventilation (CPAP/BiPAP). If I need that, my life will be bloody complete. argh. Been referred because Nick has had to wake me because I've stopped breathing, and Im tired all the time no matter how much sleep I get. Im also high risk due to my scoliosis and lung volumes.
All in all a very mixed bag of a day, but at least some results were unchanged. Hardly normal, more consistently crap!
Need to do some weight bearing exercise now then