Big sister trying to help with possible new diagnosis of GPA

[BigSister]

Hi all - I've been browsing the forum for the last week - so thankful to have found a community like the one you have here! (Thanks Andrew and Anne for getting me registered!)

My name is Allison, my sister's name is Ashley. She's 29 yo, married, mother to a spirited, beautiful 3 yo girl.

It's a long story, as they all are, but we're now just waiting for lung biopsy results from the Mayo Clinic in Scottsdale, AZ. I'm 99% confident this will be her diagnosis based on her symptoms and the last 3 months. I was on the fence until her septum collapsed creating a saddle nose - pretty much sealed the deal for me - no need to wait for Mayo.

We live in Boise, Idaho - technically she lives in a smaller town 120 miles away but she's been here for the last few weeks and they are arranging to move back here. Boise isn't known for medical specialists but it beats her town by a long shot. Plus...I'm here, her parents are here and we are all anxious to help her in any way we can.

Below is the timeline I've been keeping for the last few weeks:

March 21^st – congested when we visited Shoshone – said it was allergies
April 6^th - Easter – sick (cold virus)
**Some time here went to family doctor in Shoshone twice – given two different antibiotics – referred to ENT, appointment for ENT made for May 19th
May 7^th – Called ENT, said she can’t wait until the 19^th – apt with ENT (zpack antibiotic given)
May 13^th – ENT again, new antibiotic and steroid
May 16^th – stayed weekend with me – wasn’t great
May 18^th – not feeling better but felt better than when went to ENT on the 7th – going to ride it out a couple days
May 20^th – migraine, snotty – thinks she has a cold on top of sinus infection
May 26^th – felt bad, thinks she caught flu virus, was really bad all weekend
May 27^th – stayed home from work
May 29th – Urgent Care – took chest x-ray, sent for analysis - prescription codeine cough syrup, sent home
May 3oth- Very weak, sweating tons, called urgent care doctor - they said they can't do anything since it's a Sunday, come first thing in the morning. Apparently this triggered the doctor to see if her chest x-ray results were in - he called her back and told her to go to the ER.
May 31^st – ER in Jerome, transferred by ambulance to Twin ICU – started treated for severe bacterial pneumonia
June 3^rd – transferred to general ward – changed antibiotic
June 5^th – Sinus surgery – ENT said it was the worst he had ever seen, abscess on the septum
June 9^th – BAL procedure, suspect fungal pneumonia, anti-fungal started – results could take a week to return – weren’t able to culture anything
June 11^th – infectious disease dr involved
June 13^th – discharged from Twin Falls hospital – not getting worse but didn’t know what else to try
June 14^th – resting in Nampa at Dad’s house – coughing very badly, gagging, puking
June 15^th – ER in Meridian, transferred to Boise ICU – suspect Wegener’s, wanted to to kidney biopsy
June 16^th – kidney biopsy – I noticed her septum collapsing (saddle nose)
June 17^th – 1 gram steroids started, will have 3 days of 1 gram, moved to general ward
June 18^th – 2 of 3 kidney results came back negative – 3^rd test will take a couple more weeks
June 19^th – VAT lung biopsy done – sent samples to Mayo Clinic in Arizona, will take a week or more to have results
June 22^nd – released from hospital – prescription for 40mg steroid, Bactrim antibiotic

Now we wait for results I guess….?

Future scheduled appointments:
6/30 - Kidney dr - post-kidney-biopsy appointment
7/14 - Lung Biopsy post-op appointment – remove stitches
7/16 - Pulmonlogist

[andrew]

Bumping this because it got stuck in the approval queue.

[Pete]

Hi Allison,

Welcome to the Forum. It sure does sound like Ashley has GPA/Wegener's. Since you're already working with Mayo, I recommend that your local docs consult with a Vasculitis specialist there for ongoing treatment.

As far as next steps are concerned, Ashley should expect an additional med to bring the disease under control and induce remission. This additional med could be a cytotoxin such as cyclophosphamide/cytoxan or a biologic such as rituximab. An additional antibiotic could be prescribed to cure any residual infection. (Bactrim is used as prophylaxis against the type of pneumonia associated with immunosuppression.) The steroid dose may be increased if needed to control inflammation and help her feel better.

The best advice I can give Ashley moving forward is to take her meds as directed, follow instructions regarding nutrition and managing side effects, and live life as fully as possible. There will be a "new normal" eventually, and it may be close to her pre-GPA lifestyle.

Keep in touch and let us know how she's doing.

[Dirty Don]

Morning Allison, looks like your sis is on a familiar trip. Lots of guessing till Mayo nails it for her. Mayo, in Phx, did the same for me. Other hospitals/docs had little idea what was going on, so finally someone got smart and sent labs/xrays to Mayo, they got it within 48 hours. They treated me and got me to where I am now. Dr Lester Mertz is head of Rheumatology there, and his NP have done the lion's share of work with me. You can have your docs consult with them and/or visit Mayo in Phx...good hotels nearby. Best to your sister and hang tough...patience & procedure go a long way with WG.

[BigSister]

Thank you Andrew, Pete and Don!

Mayo, in Phx, did the same for me. Other hospitals/docs had little idea what was going on, so finally someone got smart and sent labs/xrays to Mayo, they got it within 48 hours.

This kinda touched on some anxiety I have - the fact the lung biopsy result is taking so long - does this mean her condition is not severe (based on other symptoms/values) or is it someone just following standard biopsy protocol and not understanding that time is critical with GPA?

Also - I don't know a lot of her GPA-specific readings, like her ANCA, etc. What are those that I should be concerned with so that I can ask the doctor specifically? I know her vitals, bloog sugar. But I don't know what showed up in her kidney nor what showed up that eventually led them to suspect GPA. Is there anything else?

[drz]

Thank you Andrew, Pete and Don!


This kinda touched on some anxiety I have - the fact the lung biopsy result is taking so long - does this mean her condition is not severe (based on other symptoms/values) or is it someone just following standard biopsy protocol and not understanding that time is critical with GPA?

Also - I don't know a lot of her GPA-specific readings, like her ANCA, etc. What are those that I should be concerned with so that I can ask the doctor specifically? I know her vitals, bloog sugar. But I don't know what showed up in her kidney nor what showed up that eventually led them to suspect GPA. Is there anything else?

They can diagnose Wegs even without a positive biopsy if all the symptoms point to it and they rule out alternative causes. I got my kidney biopsy results the same day I think but my kidneys were hard hit. She may be lucky and not have Wegs yet in her kidneys which would be a nice break. A positive ANCA suggests Wegs but also is not definitive since some people don't seem to show much correlation between Weg symptoms and ANCA scores. I did but not everyone does. WBC helps track infections. Readings for anemia due to bleeding are also important to monitor if she is bleeding in lungs or kidneys.

[renidrag]

Welcome Allison, what a great sister you are, I know because I have a sister that goes to bat for me. I look to CRP, SED Rate and PR3 blood results for inflammation. A lot of folks also look to ANCA. I was never ANCA positive and have attained drug free remission. There is always hope.
Dale

[annekat]

Hi, Allison! I'm glad you got successfully registered. Thanks for your detailed timeline of your sister's journey, so far, with all of this. The fact that her septum has collapsed, combined with all the other stuff, is pretty much a dead giveaway that she has WG. My story was similar, with it starting in the sinuses and eventually progressing to the lungs, but it was all stretched out over a much longer time, two and a half years, probably because my symptoms were less intense than your sister's for most of that time and were pegged on allergies and that sort of thing. So, I had a delayed diagnosis, as is quite common, and it took the escalation of lung involvement for them to get me properly tested with blood work, a lung CT scan, and a nasal biopsy, which was positive for WG. Strangely, my saddle nose appeared right around that time, and I only noticed it when I got home from an overnight hospital stay. No one had mentioned it, if they noticed it at all. But it was pretty much the clincher for knowing what I had, and when my ENT saw it before performing my biopsy, he knew it, too. There is a similar disease, MPA, which I think can also cause this, but WG seems to be a lot more common. BTW, I do not have kidney involvement and hope you don't either. There are quite a few of us on here who don't. Although we know that it could happen later on, so we continue to have that monitored regularly.

Anyway, what I meant to say was mostly that it is good that your sister's symptoms all happened and were paid attention to within a relatively short period of time. This improves her chances for stopping or greatly reducing disease activity before much more damage can be done to her sinus area, lungs, or any other tissues or organs. I'll be keeping her in my thoughts, and let us know when you know more!
And I would not worry too much about the length of time it is taking to get the lung biopsy results; I would just chalk it up to bureaucracy and the fact that you are doing it from a distance, using a well-known and respected facility that probably has lots of cases to handle. Best wishes to you and your sister!

[BigSister]

Thanks again for your help Anne!

Well the good news is that we had her kidney appointment this morning - the 3rd and final result from the kidney biopsy was negative. The Dr said there was a 3-5% chance they biopsied a healthy part of the organ but feels optimistic this means her kidneys are not affected. She gave blood and urine this morning, returns in August for same thing. So thankful and remaining optimistic in that regard.

Otherwise still waiting for lung biopsy - I asked around a few other forums and heard 2-3 weeks is pretty common unfortunately. She has some major decisions to make about her location and job while we wait. She lives about 120 miles from where she is now and where the better hospitals are (still not great). Her job is great as far as support and friendship goes but the pay is nothing. Right now her husband and daughter are 120 miles away and that is stretching everyone VERY thin. He is able to transfer jobs. We just don't know what to expect for treatment and how close she needs to be to her doctors?

(Oh and thank you for the blood tests to look at - I did find out she is positive for PR3 but I didn't get the values, next time!)

[annekat]

Thanks again for your help Anne!




(Oh and thank you for the blood tests to look at - I did find out she is positive for PR3 but I didn't get the values, next time!)

Good to hear her kidneys don't seem to be affected. There should be a value for creatinine in the blood test results. If the value is somewhere between .6 and 1.3 mg/dL, that would indicate that her kidneys are OK, according to the lab that does my testing. Mine is currently at 1.0, so I'm not worried. I get it tested every 3 months. I don't know why they would do a kidney biopsy if they have those numbers and they are normal. They probably did a lung biopsy because they are more reliable and accurate than a nasal or sinus biopsy. But a sinus biopsy is a lot easier to do. What's done is done, and may be for the best, but I'm sorry you have to wait. I would think that with the saddle nose, there are WG specialists out there who would just go ahead and start her on a standard WG immunosuppressant, along with the steroids and Bactrim. I think a lot of us were on more daily steroid at this point, too, more like 50-60mg. The sooner she gets proper treatment, the better, though this has all happened so fast that at least she hasn't had WG smoldering in her system for many months or years before getting treatment. Good luck!