New Member... possibly. In need of an expert in the field.

[Hope003]

Hi Everybody,

I'm not even sure where to start. I have ARVD/ARVC, Cardiomyopathy. Ventricular Tachycardia. Heart Failure, DVT's, Pulmonary Hypertension and my docs thought this was all from the ARVD.
Blood tests in the past showed no clotting disorder but every time they took me off Coumadin I threw another clot. So lifetime Coumadin now. I've had lower back pain for 2 yrs and kept telling my internist something was wrong. He took some x-rays and told me everything was fine and dismissed it. I have recurrent bouts of pleurisy and cough up blood. I have been to the Cardiologist, who sent me to the lung doc, who told me maybe asthma and gave me some inhalers. He told me it was my heart and back to the Cardiologist I went. He sent me to my PC, who kept telling me, I don't know what to do with you and would throw his hands in the air. He blamed my HF and increased my water pill which really made me sick. This has been going on for years and finally I was so sick and weak sitting in my Cardiologist's office this May. He said, Why isn't anyone checking into your Pleurisy and why you keep coughing up blood. I sat there and shrugged my shoulders. He said, I want you to go to the PC and tell him to run some blood and check for Vasculitis. I did and my PC asked me what tests he should run. I asked him to please call my Cardio and ask him. So frustrating! Anyway I finally had the tests done.

My ANA was a little high and he told me it's probably nothing. He said my ANCA's are elevated and when I asked what that was for he snapped at me and told me to call a Rheumatologist, he doesn't know. I was done with that PC. I did go to a Rheumy and he ran his own tests. ANA, Lupus,... You all know. They were neg but the Anca's are all positive. He mentioned a lung biopsy to me and told me to go to my lung doc and ask. My lung doc said I had some scarring and inflammation but nothing to biopsy. He told me to go see an ENT and let them do the biopsy. So I went to the ENT, he checked me and found a granuloma on my voice box. He said it is not related and is probably from GERD. He even did a CT scan and told me I had broke my nose in the past! My eyes went wide! I didn't even know. LOL Anyway he didn't find anything suspicious in my mouth or nose.

I called my cardio that Friday because I was very SOB and having bad chest pains. he said go to the hospital. I did not! I have V-tach! So when I go to the hospital, it's all they see and panic! My breathing has been getting worse as well as the chest pain. I went back to the lung doc on Monday. He put the pulse ox on me and the nurse walked with me as I told her I was having trouble breathing. she kept showing me the reading. 99%. I kept telling her I can't breathe and she let me sit down. My heart rate went up to 140 and my 02 dropped to 90% I was having a hard time breathing. The lung doc told me it's not low enough to get me oxygen. I have to be at 88 or 89%. My head was spinning at this point.

I went back to the Rheumy, who wondered what happened to me since May. I developed Bronchitis and the lung doc had to put me on prednisone to help me breathe. I just came off the prednisone late August and the Rheumy realized the second set of tests he ran was when I was on the prednisone. And they all came back negative. He just repeated the test last Monday and He said the ANA and lupus were negative and the ANCA's positive again and this time higher. He wants me to start on Plaqunil for the pleurisy and Methotrexate. I'm just so confused in all this and I really need a good doc to help me figure this all out.
I thank God for my cardiologist! He never gave up on me and always spoke the truth. He explained I have HF and VT and PH and all but he didn't think it was severe enough to be causing all my symptoms. All this time we thought it was my heart and now I would love to find out it's not and there may be a treatment to help me feel better! : )

I have SOB, chest pain, recurrent pleurisy, hearing loss and ear infections, eye pain and burning, (This is new) Liver pain, back pain, nose bleeds, unintended weight loss and just feel really weak. Oh and I don't think it has anything to do with this but a few months ago my feet were killing me! I actually went to the foot doc because it hurt to walk on them. It felt like really bad cramping, so strange.

I live in NY, Long Island if anyone knows of a good doc? PC too! I'm open to all advice.
I thank you all for listening and being so bold as to share your stories on this board. As I sit here and type this I realize it's a Godsend... for many!
Thank you Andrew for encouraging me to share!
Thank you All!

Blessings,

Hope003

[MaxD]

Hope,

See Dr. Robert Spiera in Manhattan, he's one of the top experts. He works closely with Dr. Robert Lebovics - a top ENT specializing in Wegener's, also in Manhattan. They're both wonderful people too. If you don't get an appointment right away, have your doctor call their office to get you in soon.

You can find list of specialists in Wegener's at Vasculitis Foundation

Good luck, and keep in touch.

[Hope003]

Thank you Very much MaxD. I appreciate all your help and information.

I'll keep in touch! : )

[Birdie]

I'm not even sure where to start.

You did a terrific job of explaining what you've been thru, expecially for not knowing where to start. Sorry I don't have any great insight. I just wanted to say that your story is typical of many cases of Wegeners in that nobody really knows what's wrong and the patient is numb with frustration. I was fortunate because it only took five months to get past that stage.

If you've got ANCA's then the next step for a firm Wegeners diagnosis is a specific type of granuloma in a tissue biopsy. Many patients don't get either of those, resulting in a more difficult diagnosis and a much higher frustration factor.

Don't relax, find doctors you can work with as soon as possible.

[Hope003]

Thank you Birdie!
I'm thinking of trying the NY doc or JHH, only because they helped me in the past.
My Cardio has me going in for another Right Heart Cath and it's hard because I can't breathe as it is and when they lay me flat... Oh Boy! LOL I'm laughing because I remember that line from, Quantum leap! Ohhhhh Boy!
I can't imagine having Wegeners and ARVD! Two rare diseases! I think my Cardio feels the same. My potassium keeps dropping also and we are not sure why. Does anyone have low Potassium, Chest pain and SOB? And low Sats?
Thanks again for your help and support. I'm going one day at a time and trusting God as He puts the pieces of the puzzle together for me.

[Birdie]

I can't imagine having Wegeners and ARVD! Two rare diseases! I think my Cardio feels the same.

When first diagnosed I found a web site with a database of case studies. Not limited to Wegeners, it was a challenge to navigate. I found and read hundreds of Wegeners case studies. Well over half of those case studies were given more than one incorrect diagnosis before the Wegeners diagnosis. Most of those cases took years to get to Wegeners treatment and were in bad shape by that time.

I have a long list of things I was told I have... they were all side effects & damage from Wegeners or the treatment for it. Such is the case for the damage to my heart. When the heart issue showed up, it would not have been attributed to Wegeners if I had not already been diagnosed with Wegeners. I'd have some other heart related diagnosis.

So I learned the lesson right from the start, a diagnosis is not reality. From my experience it's wise to doubt. It's wise to get more than one opinion.

[Hope003]

Well I had the RHC and the docs do not think that my shortness of breath is coming from my CHF or PH. I have Mild PH I' being treated and on the story goes. This is all beginning to frustrate me... a lot!
I'm exhausted to the point I wake up and want to go back to bed! Dressing and taking a shower are becoming increasingly difficult and I never felt this weak before. I found a new PC, and he just happens to be a Nephrologist.
He is doing another work up, my fourth. I did my best to explain my exhaustion and weakness and he said, I look good. I asked him if there is anything I can do or take, for relief. He said no but if you looked bad I would give you something right now! Is it me or what. I have to look bad to get treatment?! Sigh! He also explained if it is Vasculitis, I could be gone in under 2 yrs then goes on to say that the medication could kill me as well! I have to have a sense of humor and say, that emoji, is exactly how I must have looked! Then he says, we need to know. I sat there stunned. I told him my Pulmonologist told me to get a second opinion and he immediately said no and told me he knows what he's doing, this is his field. So... I'm going to Manhattan to see Dr. Spiera on the 19th. I praying and hoping he can shed some light on all of this and possibly diagnose and help me. I was told to bring my medical records, any tests and biopsies I had done. From all I'm reading and learning, biopsy sounds like the only way to DX this disease? Is that correct? Isn't there any other non invasive way?

Thank you for giving me Dr Spiera's name, and for all your help!

Blessings!!!

[annekat]

Welcome to the forum, I have nothing much to add but am glad you are here! Come here any time to ask questions, tell stories, or vent, and use the search function. Add your name to the Weggie members map highlighted in blue at top of page, and see the distribution of members all over the world. I'm glad you got an appointment with Dr. Spiera so quickly! There are videos of him talking on youTube if you should feel inclined to look. Let us know how that goes and if you get a real dx!

[Birdie]

I told him my Pulmonologist told me to get a second opinion and he immediately said no and told me he knows what he's doing, this is his field. So... I'm going to Manhattan to see Dr. Spiera on the 19th.

I would run out of the office to find another doctor as soon as a doctor did anything but encourage me to get another opinion. You done good by going elsewhere without even using the first doctor for a refferal.

From all I'm reading and learning, biopsy sounds like the only way to DX this disease? Is that correct? Isn't there any other non invasive way?

You said in your first post that your ANCA's are high. Assuming that means c-ANCA, then you already have more indication of Wegener's than many patients ever get. A small percentage of people have a natural c-ANCA level. If you're not one of those people then the c-ANCA is likely caused by Wegener's.

Granuloma in a tissue biopsy can confirm Wegener's beyond doubt. There are a number of slightly different types of granuloma with different causes. They can be distinguished in the lab, each type can be positively identified. The Wegener's type of granuloma is only known to be caused by two things, Wegener's or pyostomatitis vegetans (associated with ulcerative colitis).

So... the most positive diagnosis is with both, c-ANCA and granuloma. No c-ANCA means nothing since many Weggies never produce it. No granuloma means nothing since it may have been missed in the tissue grab, or a dumb lab may not have known what it was looking at, or, or, or, ???. Negative result does not rule out Wegeners.

So you have reason to have hope... you're going to see a specialist who knows far better than any of us. You being educated on the subject now means you'll better understand what the expert say's, instead of having to decide if he knows what he's talking about (should I run away from this guy?).

[MaxD]

Hope,

Glad to hear you're seeing Dr Spiera next week. I would suggest writing notes of everything you can recall from your complicated history - Dr Spiera always spends time listening closely to patients, even before going through the medical records. So you wouldn't want to forget anything important. If you have someone accompanying you (highly recommended), have them take notes at the meeting after he examines you.

Good luck to you!