Question: Hello, I have suffered for 8 years now with worsening medical conditions, I have spent more than $120,000 trying to find out what is going on, what I have been diagnosed with thus far, is a possible Immune Deficiency, for which I currently undergo Intravenous Immunoglobulan Replacement Thereapy, I have also been diagnosed with Psoriatic Arthirus and Possible Lupus - I also underwent a very dodgy half nissen fundiplication (that went wrong) and have diagnosed swallowing problems My symptoms have been; 1/ Chest Pain under the left last rib 2/ Lowering O2 Saturations, reducing each year, starting at 97 and now down to 92 3/ Abnormal Liver function tests 4/ Swelling in legs when sitting for long periods (even without being on prednisone) 5/ Hypoxemia - borderline ABG hypoxic 6/ Shortness of breath when stationary for long periods 7/ Crackle noise on exhale 8/ 3 x Proven and worsening shunt study tests - tested with 100% O2 9/ Unproductive Cough 10/ Constant fluid in bottom of ariways causing mutliple infections 11/ Feeling of clunking fluid when breathing 12/ Noise and popping feeling in chest. 13/ Heomoptasis It was recently suggested that I might actually be suffering from Hepatapulmonary Syndrome, there are no Heptologists on Health Care magic, so I am sking you your thoughts. I have had far too many CT screens of my chest, they have always not found much, but fluid in the bottom of lungs and patchy infultrates at most.. however the last CT showed that the contrast had not travelled into the left side of the pulomonary areas by the time of the CT scan - ie it was delayed in getting there - which may suggest widened pulmonary veins. Infections have cleared from the IVIG therapy, however I can still feel fluid when breathing, and I have squamous metaplasia which is found from the washings of serveral bronchoscopies, I have had bleeding airways for some time but this was written off due to the infections - which could be caused by back up of fluid from HPS. I can NOT have any more radiation and not many here in my country have even heard of this syndrome, I have had an MRI of the liver and spleen and liver seemed relatively fine while the spleen was enlarged - I further had a fibroscan done and it was suggested that if there is no cirhossis during this scan then a HPS was unlikely, however I recently learned that this scan was performed by someone filling in who had never done the scan before - but generally there has never been signs of liver cirhossis. I was a very heavy drinker for many years, but that has ceased, and I have had many drugs like Methrexate etc that affect the liver. I know it is important to find HPS issues quickly as transplant is the only option and I must get that done before the liver worsens. Although it is obvious I have autoimmune issues, it is strange that my sats and ABG are lowering constantly - I am a very active person, but overweight, which i think is why LFT exceptions are always seen as possible fatty liver. The spleen enlargement may suggest liver disease, but why would that not be seen on MRI or Fibroscan? The fundiplication was a disaster and took 3 and 1/2 hours to complete, My heart stopped on the table - apparently vasal vegal reaction - but a nurse from the operation came to my room in the hospital and suggested I look into the operation, I did but could not find anyhting untoward I recently had motility study done, as I could feel that water would not seem to go past my oesuphagus, it proved that only half my swallows work.. I also had a PH study done, and found something very interesting - I swallowed a glass of Orange juice, which would obviously change the PH sensor to a lower number as it passed down the oesuphagus, however i note that it stayed at 4.3 for a very long time... in fact until I actually ate something solid and the oesuphagus opened up - the OJ seemed to just sit above it - when I breathe I can feel water sitting above or mucus and I wonder if I am aspirating this into my airways? Biopsies from the airways always show chronic inflamation in the cells and metaplasia - I also have a very weird hiccup, which is deep down in the chest - not up high like normal - I tried dilating the sphinkter with no succes and now get a very strange sound that is akin to a bath plug being removed from a metal bath tub full of water. i really need to know exactly how to diagnose the possibility or exclude HPS - I have had a bubble study before but that was done to exclude a cardiac shunt - please help as I am at a loss as to how to stop the progression. LFT's always indicate high ALT AST and Albumin Regards, XXXXXXX

Hi - thanks for your input - I will chase the reports you require, could take some time. 1/ 2D Echo with bubble study - I had this done a while ago to rule out a left to right cardiac shunt, I dont think they were looking at the time delay for bubbles showing up, they would just be seeing if there was a hole in the heart.. I will chase the report all the same. 2/ HRCT of lungs, I have this report and I have the scans on hard drive, would you be able to look at the scans (happy to pay extra) - I assume that the format for the digital images is not the same for every CT machine, so perhaps you need certain software for this - I will send through the report tonight 3/ Last LFT rsult was reasonable, for a change - only AST and ALT elevated... but that is not always the case, I will chase the older ones from my GP, hopefully I can get into him in time before my reply time wears out. 4/ Pulmonary Angiography - I might have a report on this, but not much is ever seen on that - no PE's etc not much noted I think the HRCT of lungs also included the CT PA 5/ Technetium-99m–labeled albumin transit test - I have never done this test 5/

Two qtns if you dont mind. 1/ I have been given notroglyceryn to help with the shest pain, I rarely use it at all but should I stop using it? 2/ The fundiplication was a bit of a mess - could somehting have been damaged arounfd the liver when it was done?