Trouble breathing

[zizzcat]

Hi, I'm new to the group, my name is Hope.
I have been diagnosed with diffuse connective tissue disease.
My throat has been swelling up for over a year. I have had episodes of coughing and now munch on Halls Menthol Lyptus almost constantly to anesthetize my throat and keep it from closing up. When it does, I have coughing fits and try to catch my breath for a minute or two, while stuffing my mouth with Halls' and getting exasperated. My ENT ordered a CAT and it shows severe narrowing of the airways at the 4th and 5th vertebrae with some degeneration of these as well. When the ENT views the inside of the throat there is a lot of inflammation in the front and back of the voice box, sometimes w inflammation of the vocal chords as well. A month ago, lesions in my nose began to eat away at it again. The septum is already gone. She is concerned about Wegners as at this point the scabby lesions are at the bridge of the nose. She has written a letter documenting her concerns to my rheumatologist. The throat problem however, she believes is most likely due to the spine. I do not agree but am trying to get an appointment with a spine surgeon to evaluate this. I am ANA and ANCA negative but on a very high dose of Imuran. It has taken decades to get some relief but I feel like while I try to meet the various specialists sstandards my time might be running out. Would appreciate any info and/or advise.

thanks
Hope

[Dirty Don]

Time IS important here and someone needs to get a definite biopsy from you...the only real way to tell for sure. Docs need to quit guessing and get after it NOW.

[me2]

I agree with Don. I looked up diffuse connective tissue disease and from what I have read it is a diagnosis that says little more than "you are sick". That is not very helpful. I saw a large list of more precise diagnosis listed under diffuse connective disease and it would seem to me you should be pushing very hard to get a better diagnosis so your treatment can be more precise also. You need to lean hard on the doc you think is most likely to help you get this figured out soon. Be a pest, get angry if you have to but do everything you have to do to take good care of yourself.
I have had tracheal stenosis and several surgeries due to WG. In that regard the docs worked wonders for me and I have been able to breath good for over a year now. I'm hoping the same gets worked out for you soon. Be persistent. And stop in this forum to let us know how you are doing.

PS I have had WG for over thirty years. I have the same doc as back then. About two years ago I was having severe reflux problems and he didn't do his job of getting me an appointment. I tried him several times and got blown off. So, I got desperate, and for the first time in over thirty years I went over his head to the Medical Director of this University Hospital. The medical director was great. He listened to what was going on with me and within two days I had an appointment to be seen in Gastroenterology.
My doc was not real happy about me doing this but he also had no argument for what I had done. If doctors are not doing their job they need to be pushed sometimes.

[zizzcat]

They have been guessing that it may be something along the lines of lupus, and treating it as aggressively as if it were lupus. With the symptoms of the nose and throat resurfacing, my ENT has written a letter to my rheum docs advising that even without a positive ANCA, they consider treating it as though it were Wegner's regardless of what they might choose to call it. I need to document from the spine doc, to what extent if any, the degeneration of the 5 & 6 neck vertebrae, are affecting the severity of the stenosis, if at all, and then ask the ENT evaluate the spine doc's opinion and possibly add to that. I have an appt. with the pulmonologist to go over some pretty serious difficulties that have since been mostly resolved with medication, to see if Wegner's might be a consideration. My appt. with the rheumatologists is on the 10th of June. I am being seen at a very good teaching hospital. Last week I asked that the resident doctor treating me be removed from my case because she was not taking my case seriously and refuses to consider the points the ENT was making. I asked that the Sr. Doctor remain the same. She has been removed and replaced.

I've gone through many years of getting angry, and it got me nothing but doctors with busted egos. My experience from this is that doctors with busted egos don't care if you get better or not. During that time, I spent 5 years home bound and unable to drive.

I truly appreciate everyone's concern and comments.

hope

[me2]

Hope,
I can see you have been through a lot and I have been preaching to the choir. You have a great spirit. It sounds like you have a good handle on working with the system and working with doctors. I wasn't suggesting anger as the first choice but its not an option I would ever take off the table. Poor doctoring needs to be yelled at once in while , even if it doesn't help my case it might help the next patient get listened to. It sounds like you are aware that a negative ANCA is not conclusive. In the old days back when I became sick, biopsy was the only way to determine WG. My ANCA's have always shown nothing. My docs don't use them anymore for me they mean nothing. Unfortunately there is no test as to who gets useful results and who does not. A doctor who knows WG well would not use ANCA for diagnosis.

[zizzcat]

I began doing research on Wegner's 10 years ago when my nose was being eaten up and I was taking between 30-45 Aleve a day to thin my blood out enough to kill the pain in the joints and head. At that time, false negative ANCAs were not unusual and a few articles pointed out that often, even positive ANCAs were not detected until the illness was so advanced that a great many patients died within two years of diagnosis. So you, me2, must be a major miracle!

I was put on immune suppressants because I acquired a malar rash and had many lupus and vasculitis related symptoms.
The malar rash began to disappear as the dosage increased. After damage to the kidneys, the lungs, the pancreas and possible abdominal vasculitis, they gave me the max dose of Imuran and finally the pain began to disappear! How amazing to be pain free! However, scans of my lungs are showing that scarring to the kidneys has intensified and having tracheal stenoses is daring me to consider Wegner's again. The ENT says that tracheal stenoses only gets worse, never better. I had no choice but to file a complaint against the resident doctor and ask that she be removed. My acquiring the courage to file a complaint against that doctor is a miracle too! I have a feeling that for many of us, just waking up in the morning on certain occasions has been a miracle! I am learning to be "tougher than I know"

[HopeinTN]

This disease is so weird. Why can't it be cut and dry, positive or negative. This sounds horrible, but I'm guilt of saying, why can't it just be cancer? It's a yes or no, cut and dry and they know the treatment...BUT then I came to my senses. Cancer sucks and no way do I want it. I'm okay with being in the rare disease group and winging it.

I'm negative ANCA, positive MPO, negative PR3 and positive ANA. Positive Wegs diagnosis from lung biopsy. Sweet Al explained what this all meant, but it was still clear as mud.

The best thing to come out of this, would be my new friends on here that can relate to me and my funky lungs.