ARDS

[nelnorm]

I am 68 years old and have alpha one . I have been diagnosed with pseudomonas in the lungs . I was treated in February with IV and oral therapy together for three weeks and did very well afterward until September when I again was coughing much mucus . extreme lethargy and night sweats and the sputum again showed pseudomonas and I took Cipro orally for three weeks which worked very well. Now, I am having to be checked for Acid reflux as the doctor thinks I must have it although no symptoms because he thinks that is how I received the pseudomonas in the lungs (from the stomach). I am now scheduled for endoscopic placement of a device to check for reflux . However, the GI doctor says that the pseudomonas did not come from the stomach as the acid in the stomach would have killed the pseudomonas. I am at a loss as to why I have to continue to prove that I do not have reflux if the pseudomonas could not have been aspirated from the stomach. Your thoughts on this would be appreciated.

[Davy9]

You could have occult reflux with aspiration. No the pseudomonas did not come from the stomach but any aspiration of stomach content is be terribly irritating to the lungs. It is a good idea to get it explored.

I am intrigued by your alpha one diagnosis. In my experience that disorder shows symptoms prominently early in life, in ones late 30's or 40 by the latest. It is one of the few diseases so severe that lung transplantation is about the only and ideal treatment (or it used to be). You are the oldest person I've heard of having alpha one. Are you certain the diagnosis is correct?

[nelnorm]

Yes, I am sure.... I am registered with the Alpha one Foundation who actually tested me. My family, (6 siblings total and living Mother) have all been tested.... 3 children have it, 3 are carriers, my Mother a carrier and my Dad who is not living is now known to have had it due to the phenotypes of all of us. The diagnosis was after years of symptoms ..... diagnosis of asthma etiology unknown, copd, etc. My brother who has severe alpha had gone to doctors for 17 years with testing for asbestosis, etc with no doctor having known what was causing his deteriorating lung problem... he is a candidate for lung transplant but at the moment he is receiving augmentation therapy. At last , in 2003, a doctor at Baylor in Houston tested him for alpha one and when he tested positive, he suggested the siblings be tested. It so happened at that time, I was having a very bad lung infection and so I wrote to the alpha one foundation and was tested. For the next few years , even though I knew I was an alpha one, I did not find any doctor who thought it was something to be concerned with until I had pneumonia in Nov of 2007 .... that is when the alpha one foundation suggested I go to the National Jewish Lung Center where the director of Alpha one clinic is located....I had never in all those years prior to being tested nor since being tested was asked to give a sputum specimen although I was constantly tested for TB with ppd testing and chest x-rays. When I started to NJC, the first thing they did was request a sputum specimen.... every specimen I have given them has had pseudomonas. I am symptom free at the moment...no cough, evening fevers, night sweats... they have checked for MAC but have not grown out any at this time.... now, I am to see if I have gotten the pseudomonas from the stomach and since the GI doc told me I had not, I was wondering if anyone else has had such as I have....thank you so much for your timely reply.

[nelnorm]

Would you give me some ways of getting pseudomonas in the lungs if not from the stomach?

[Davy9]

Do you know if your genetic alpha one signature is SZ or a ZZ? A most interesting account and I appreciate your information.

Having talked around about pseudomonas and aspiration I have this to add. It can be part of an aspiration syndrome but the source is not from the stomach. It would come from an infection in the oral pharyngeal area. A similar example would be people with chronic sinus infections. They can present with aspiration pneumonia that started from a nasal drip.

Yet, I know you understand the need to avoid any pneumonia from any source. So it is still prudent to investigate gastric aspiration as well.

[nelnorm]

My phenotype is SZ. I am the only one that has the "S".... my other siblings as well as my living Mom has either ZZ or MZ . So, the alpha one docs know that my Dad who is not living had SZ. My Mom is a carrier... MZ as well some of my siblings. My sister who has ZZ has not had the problems that I have had but my brother who is ZZ is extremely severe. My two daughters are MS. I became worse when I got pneumonia last November . My symptoms were "classic" I was told by the alpha one doctors for MAC but they have not grown that out but have grown out the pseudomonas. I have not had noticeable sinusitis , pharyngitis, etc but the GI doctor who is going to check me for reflux says that he thinks that is how I have it in my lungs....that is, through the nose . My last catscan in September showed consolidation in the right lung whereas before it has been in the left only. When I had the pneumonia last November , I had no audible breath sounds from the left lung and lots of crackles. After the IV and oral therapy in February, I was much much improved but still have crackles which I have had for years on that left side. This September , as I said before, I had symptoms again and received oral only Cipro.I am a retired pediatric RN. So, I know how to listen to my chest with the stethoscope . Of course, I have done a lot of research on Alpha One and now I am researching the pseudomonas . I am having a hard time believing I have reflux but will go ahead with the test to rule that out. I am thin , 117# , and the doctors would like to see me gain weight but I have never been overweight and I am much more active than most people... that is I do all my yard work , take care of my Mom who has alzheimers (a full time job) and do all the housework, cleaning , cooking , ironing by myself. ..... so , I actually expend more calories than I put in which is something very difficult for me to change. Therefore, I would love to be able to find the source of this pseudomonas so that at least I can change whatever I am doing to end the chance of getting that again ...(It may not be eradicated anyway according to the clinical director of alpha one... it may be there waiting for a trigger to get active again). Thanks for your input.

[Davy9]

Just a few thoughts for your consideration. As an Alpha One you know the importance of not getting lung infections and of knocking them down ASAP when they occur. Your recent history suggests they they are frequent and advance to the point of leaving areas (lobar?) of the lung consolidated. I have seen where COPD patients have had Porta Caths placed to provide immediate access for IV push and IV medications. As a nurse you could access your own Porta Cath at home and push meds. I don't know how long a Porta cath can remain in situ but as long as they are flushed monthly they do last quite a while. They last longer than PIC lines as I recall. They only have one port though whereas a PIC can have more than one.

You are most likely already familiar with postural drainage and percussion techniques to aid mucous removal/management. There is available now a percussion vest (often used in CF) that is a really effective tool to loosen secretions. Perhaps usage of some such tool could keep infections from advancing to consolidation.

Ensure+ (plus) has the highest caloric content of their product line. 3 a day is around 1200 calories more and more protein, etc. Your infections suck up calories. The fever alone will raise metabolism 7% for every degree F. You surely want to avoid catabolism and need more reserves. Being underweight is not a problem for me personally.

It is nice to have a knowledgeable person to discourse with. I get to learn things and don't have to deeply explain my input either.