I'm New - Limited Wegener's Disease

[Brooke]

Hi, I am new to this site. My name is Brooke and I am 28 years old. I was just diagnosed with limited wegener's disease as it has only affected my sinuses and upper air way.
When I was 18 I got a blood clot in my right calf, my dad is a physician and had me get a bunch of blood tests done. Turned out I have a protein s deficiency. Well, I had no problems after that. I was on blood thinner for a while and then was able to discontinue that.
It all started about 7 years ago, when I was in college. It started with a tickle in my throat and I couldn't stop coughing and I also would lose my voice all the time, until it was completely gone. I saw many doctors, ENT, Allergists, pulmonologist, Voice Specialist, had ct scans, xrays, blood tests, bronchoscopy, biopsies of my nose and more. My ENT said I had Chronic Sinus problems, he actually did not believe me at first that I had not had surgery in my nose because it was so scarred up. In the back of his mind he thought Wegener's Disease but my ANCAS and biopsies always came back negative. Anyways for the past 7 years I have been on and off oral steroids, and antibiotics. Last year I had 2 sinus surgeries, after the first surgery, I developed saddle nose deformity right away. (didnt know if that was from surgery or wegener's disease) anyways I had that fixed this year. Doctor borrowed cartilage from behind my right ear to put in my nose. He did a good job. I should mention, I got pregnant when I was 24 and had a great pregnancy and have a healthy 3 year old boy now.
Finally I went to Mayo Clinic in September of this year. I saw pulmonologist and ENT there. I also had a bronchoscopy there. They also took a culture while doing that procedure. They said I have a narrow airway and inflammation which is consistant with Wegener's Disease. Even though my biopsies have come back negative for wegener's, there was so much inflammation from my nasal biopsy that it was enough evidence to conclude that I do have limited wegener's disease.
I am now on 25 mg methotrexate, prednisone, bactrim, and pholic acid and an anxiety medicine. I started this about 1 month ago.
My symptoms are, chronic cough, especially at night, and I cough up flem looking stuff. I have to do a sinus rinse everyday, a few times a day. I get big chunks out of my nose.
Wow, this got long. I have more to say but I will write back later. thanks for listening. anyone else have these symptoms?

[Jack]

Hi Brooke, sorry you had to find our site!

You will find a lot of people on here with sinus issues, it is how my own case started. I also had a lot of surgery including removal of my sinus linings! All a waste of time of course, because it was Wegener's causing the damage.
You are lucky to have been diagnosed so early and before the disease can spread. With any luck, the sinus problems should go when they get the disease fully under control. The current thinking seems to be to treate more gently than in my early days 25 years ago. I was treated with very high doses of drugs which stopped all the symptoms in their tracks, but it is not considered to be very safe these days.

[Brooke]

Thank you for responding so quickly. I do hope the methotrexate works. They say I will need to be on that for 2 years. I have been on the steroids for about 3 months now and I think I have one more month of that, then I will just be on the methotrexate.
Do you also cough "stuff" up?
I was also diagnosed with asthma, just last year. I do not feel I have asthma, I feel it is something else. (wegener's). I do get short of breath and sometimes it is hard to breathe.

[Brooke]

Also, do you cough a lot? Does the medicine help with coughing? I do have subglottic stenosis.

[elephant]

Hi Brooke, welcome. I myself had four maxillary(sinus ) surgerie and a bunch of other surgeries. Once your wegeners goes into remission alot of those symptoms should go away. I had a slight cough, but mine was more stuffiness,sinus headaches/shortness of breath. I had a lung function test and it showed that I had some lung damage from the WG. The lung doctor said it mimics asthma like symptoms. I have two inhalers but I only use one if I get short of breath. Sometimes the meds take a while to work and sometimes it's not the right medicine for you and they switch and give you another one or increase the one your on if your symptoms get worse. Make sure you let your Wegeners doc specialist know what your going thru. We are here to help and listen.

[Brooke]

Thank you. I live in Sioux Falls, South Dakota USA. I have seen a pulmonologist at Mayo Clinic in Minnesota, I don't know that she is a Wegener's Specialist or not but I know she does have more than just one patient with Wegener's Disease.
I use an albuterol nebulizer and am on another inhaler, symbicort.

[elephant]

I have the albuterol and symbicort, I have yet to use the symbicort. It took me awhile to use the albuterol, I was in denial. The pulmonolgist said just try it. I just saw my pulmonologist today and I'm suppose to start using the symbicort. I will try next week. You will see with this disease it's a full time job. You start to develop a sense of humor.

[Jack]

I use the same two inhalers, but they have little effect on my own lung condition. I have bronchiectasis which is not very common with Wegener's though not unknown and cough up a great deal of mucus. Lots of other members are troubled by coughing too, but due to different lung conditions.

I've mentioned it so many times before that I won't go into detail, but when I was suffering with my sinus problems, I had horrendous amounts of discharge, bleeding and crusting. The saline rince helped a good deal then. However, since being treated for Wegener's, all that has gone and left me with little more than a runny nose.

It took around 5 years to bring my Wegeners into full remission using lots of highly toxic drugs which I have had to continue with. Most of my problems now are due to side effects, hence the more gentle regimes now being used. However, in my case, diagnosis was so late that I would not have made it without this treatment.

[katwoman]

Welcome to the group Brooke!

I am sorry to hear you are having so much trouble and I hope the medication improves your situation quickly. As Jack mentioned many of us also suffer from serious sinus issues, like yourself I too suffer from severe crusty (due to a current flare its worse then usual and I also have saddle nose, which to date I am unable to get repaired). It is currently affecting my sinus area but also having joint pain and tiredness as well, I am also on methotrexate and Pred.

Not only do i use a sinus rinse (also always have a bottle of Fess handy in my handbag) I was also advised by ENT to use KY Jelly (yes I giggled at the doc) but believe me its amazing stuff if used regularly up the nose it loosens all that crusty and keeps the area moist.

It is a good thing your doctors diagnosed it so so early, my initial sinus and eye problems were over a year or two and it wasn't until I was admitted to hospital not in a good way with kidney/lung involvement, purple spots, fever and swollen joints was I diagnosed with WG.

I have found that everyone on this site is very helpful and offer great suggestions/advice for dealing with WG and so any questions ask away!
katwoman

[snooz23]

Hi Brooke,

I had to welcome you to the site and respond right away because I am from Mitchell, SD. That is where I grew up, I currently live in Columbus, OH. I was just so surprised to see SD here I had to write and say hello.

I am also recently diag.--Sept. 15--and am on 25 meth., tapering at 17.5 pred. (after 6+months), bactrim (3X week) and folic acid. My wegs presented with hearing loss, I have not had a whole lot of sinus involvement per se, except the mastoid. I do have some lung involvement and will be seeing a pulmon. at the Cleveland Clinic at the end of the month. I have chest pain and shortness of breath, I was hoping these would go away w/ treatment, but like Jack said on these more mild treatments I think remission is a loooooooong process that will take a while to see the effects. I do the nose rinses daily more so as a precautionary purpose.

I hope all goes well with your treatment. By the way I have two children, 2 and 4 years old.