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 21st – congested when we visited Shoshone – said it was allergies
April 6th - 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 7th – Called ENT, said she can’t wait until the 19th – apt with ENT (zpack antibiotic given)
May 13th – ENT again, new antibiotic and steroid
May 16th – stayed weekend with me – wasn’t great
May 18th – not feeling better but felt better than when went to ENT on the 7th – going to ride it out a couple days
May 20th – migraine, snotty – thinks she has a cold on top of sinus infection
May 26th – felt bad, thinks she caught flu virus, was really bad all weekend
May 27th – 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 31st – ER in Jerome, transferred by ambulance to Twin ICU – started treated for severe bacterial pneumonia
June 3rd – transferred to general ward – changed antibiotic
June 5th – Sinus surgery – ENT said it was the worst he had ever seen, abscess on the septum
June 9th – BAL procedure, suspect fungal pneumonia, anti-fungal started – results could take a week to return – weren’t able to culture anything
June 11th – infectious disease dr involved
June 13th – discharged from Twin Falls hospital – not getting worse but didn’t know what else to try
June 14th – resting in Nampa at Dad’s house – coughing very badly, gagging, puking
June 15th – ER in Meridian, transferred to Boise ICU – suspect Wegener’s, wanted to to kidney biopsy
June 16th – kidney biopsy – I noticed her septum collapsing (saddle nose)
June 17th – 1 gram steroids started, will have 3 days of 1 gram, moved to general ward
June 18th – 2 of 3 kidney results came back negative – 3rd test will take a couple more weeks
June 19th – VAT lung biopsy (http://www.healthcommunities.com/lung-surgery/thoracoscopy.shtml) done – sent samples to Mayo Clinic in Arizona, will take a week or more to have results
June 22nd – 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!

[Dirty Don]

Standard service/protocols seem to vary a bit by hospital/doctor/clinic. Medical facilities that don't have to send out as it were tend to have better return times on labs. The only reason Mayo was a bit slow on this simple dx was I crashed on a Sat. nite & he didn't see it till Monday morning...still better than most. All other tests/labs run on/for me have been on my tablet before I saw the doctors the same afternoons. Go there if you can. Best to you.

[mishb]

Hi Big Sister and welcome. Thankyou for being here for your Little Sister :thumbsup:

That is great news that there is no WG in the kidneys. Hopefully there is none in the Lungs either, but then that wont give a reason as to what is going on.
I hope the results come in soon.

[renidrag]

Dang, forgot the Creatinine, I always make sure they test for it. Nice save Anne.
Dale

[BigSister]

Thanks all!

Oh the biopsy-every-part-of-her-body saga!

When she was in the hospital the first time being treated for "pneumonia" she had a "reaction" to one of the many antibiotics they gave her (blood and urine tests quickly started showing signs of kidney failure). So that is now considered something she is allergic to.

I think when she was admitted into the second hospital, where Wegener's was the suspect off the start, she might have had residual markers from that incident?

Or that "reaction" to the antibiotic was in fact a kidney flare from GPA that we didn't know she had. Ugh.

I don't know what the values were either time but I do know they improved during her second hospital stay.

So they took a kidney biopsy, assuming kidneys were affected and because it was lower-risk procedure. She had sinus and lung BAL procedure in the earlier hospital but since they didn't suspect Wegener's at that time, they didn't check for it in either procedure. (ugh) And we didn't want to do those again since the the results cannot be trusted as much. When 2 of the 3 kidney tests came back negative, they recommend the lung. I know now why they didn't do that first - very painful procedure and risk, although she didn't have any problems.

I would like to know what her blood and urine showed on the test yesterday - I guess they would call if it was not good.

Still no results from Mayo - I'm just worried sick waiting. I feel like she is a ticking time bomb and they are just dilly-dallying around. I hope that isn't the case but the wait seems so excessively long.

Thanks again for all of your replies, and for reading my jumbled thoughts! I wish I could pick up on the lingo and proactive approach quicker than I am.

[Dirty Don]

I would call Mayo or whoever sent it there and get on their case. We (wife & I) found that by 'harrassing' the intended caregiver with repeated phone calls, one gets better/quicker service. Mayo didn't have room for me at the time; wife kept calling, got in 1&1/2 months before they would schedule me as a patient! As for the proactive lingo, etc., you've already done more than most relatives would have done. Good on you, keep it up!!! It's a high learning curve!

[BigSister]

I had a feeling that was the case which is so opposite of all of our personalities but we're going to have to buck up and start getting assertive and courageous! We're not talking about an under-cooked steak at a restaurant!

Well the good news is that the results are in...the bad news is that she missed the phone call. SIGH. Hopefully they can get in touch first thing tomorrow so we can get this show on the road.

[BigSister]

Results are in - positive. She'll start treatment very soon, Rituxan, 4x a week. She has some questions, not sure if you can help? Any input would be appreciated!

1. How long until she feels better? She has quite a bit of ear pain, facial pain, headaches, coughing. Will those subside quickly with the intensive treatment?
2. After the infusion can you drive? Can she drive herself to the treatments?
3. Has anyone had 4x a week treatments? Is it usually Mon-Thur?
4. Will she feel sick after the infusion? Like immediately or a day later? How sick? What symptoms?

Anything else about the initial course that you'd like to share we'd love to know. I'll head over to the other forums to read too!

[annekat]

I'm so glad you got results and now know for sure what is going on. RTX 4x/wk sounds a lot more intensive than I've heard of, for sure, but then I'm not among those who have taken it at all. I sure can't imagine my Medicare approving that much in such a short time, given the expense. In any case, I'll be waiting to hear how it works, and wishing your sister the best possible experience and results from the infusions.

[BigSister]

Thanks Anne!

I think she must've heard wrong (no surprise, she can't hear well and he has a heavy accent). It seems like 4x, once a week is the norm? I wish I could just live in her pocket and be there with every call and doctor visit.

[annekat]

Thanks Anne!

I think she must've heard wrong (no surprise, she can't hear well and he has a heavy accent). It seems like 4x, once a week is the norm? I wish I could just live in her pocket and be there with every call and doctor visit. That sounds more like what I've heard! Though I'm not sure there's a "norm". But we need to hear from some RTX users. I know some have side effects and some don't, and I think the length of time for it to make one feel better can vary. I can relate to the hearing loss, which I'm assuming is from WG, because I have it, too!

[BigSister]

Here are the responses I gathered from the FB group - putting them here more for anyone that runs across this forum as I did looking for information!

These are the questions I asked and the various answers!

1. How long until she feels better? She has quite a bit of ear pain, facial pain, headaches, coughing. Will those subside quickly with the initial treatment?


In my experience it took quite awhile for my pain levels to normalize due to nerve damage and the damage the disease did to my ears and sinuses.
How quickly you feel better varies for everyone so it's really hard to say. I started feeling a bit better after my first round of infusions of Rituxan, and then developed new symptoms. I did a second round a year later and have been doing well since then. Everyone is different though, and my experience will not be the same as your sister's.
it will take a week or so for the Rituxan to kick in


2. After the infusion can you drive? Can she drive herself to the treatments and home or should someone go with her?


I do drive myself to infusions now but I wouldn't the first few times in case of reaction.
I would not drive myself home after my infusions. They pre-medicate you with Benadryl and Tylenol and I am sleepy after my infusions because of the Benadryl. Also, reactions to the medication the first time are not uncommon. It's better to have someone go with you and drive you home if you have never had this medication before.
Not supposed to drive after treatment. I think it is because they give you Benadryl with the treatment.
(9 yr old) She is tired few days afterwards...but for the most part not bad
I don't recommend driving after the first infusion. Where I go, I don't think they'll even give it to you unless you have someone with you.


3. Has anyone had more than one treatment a week? I think she misunderstood - she said 4x a week but I think it must be 1x for 4 weeks?


I have done 1x for 4 weeks.
Typically the Rituxan infusions are once a week X 4 weeks, or once a week for 2 weeks repeated in a month. Some people do 1 infusion every 3 months. My guess is that she misheard and it is 1X a week for 4 weeks.
(9 yr old) Originally she got 4 weeks of infusions. After wards we have figured that if we do 2 weeks dosing...every 6 months...she has remained stable
My Rituxan has been two doses two weeks apart (one dose, then another two weeks later) for a round. Rounds tend to be 6 or so months apart unless you're mostly in remission. The once a week for 4 weeks is also a protocol that is s commonly used, just not one I've had myself.


4. Will she feel sick after the infusion? Like immediately or a day later? How sick? What symptoms?


I didn't have any side effects aside from being sleepy from premeds.
I didn't feel sick after my infusions- I did have a reaction the first time and they had to slow down the infusion. I was tired for a couple of days so I scheduled my infusions for Fridays so I had the weekend to recover. I worked 40 hours in 4 days at my job Monday-Thursday and took Friday off for the infusions. I did develop severe headaches during my second round of infusions, but those subsided once I was done with the infusion set.
The Benadryl knocks me the eff out. Every time LOL
It makes me pretty tired for a couple of days generally, but nothing worse than that. I tend to have a day 4-5 days out where I feel flu-like (hit by a train) but I'm pretty sure that's the steroid leaving my system. It has helped so many people feel better, I hope it works as well for her.


5. How long is each infusion?


They should do her first infusions pretty slowly so it can be an all day thing. I've been at the infusions center from 8am-2pm several times.
The infusion time varies, but the first one will likely be scheduled for 6 hours because of the possibility for reactions. My last infusion took 2.5 hours total.
(9 yr old) She usually takes about 4 -5 ours to run the Rituxan.
The first one will be the slowest since they won't know if she reacts to it or not. (They typically give you Benadryl, a steroid, and Tylenol before the infusion to help counter that) I have gotten a tickle in my throat feeling (like when you're getting sick) and they've slowed it down and it went away, so they are able to continue.

[annekat]

Very interesting and fits with what I've read on here! Thanks for that. I may need it someday, so it's helpful. Hoping for the RTX'ers on here to chime in.

[kaysee]

Hi BigSister and welcome to the group. I've been on vacation for a while so have not been checking. I have had rituxan infusions and the info you have already posted seems about right. My infusions were once a week for four weeks. Then I had a second round 4 months later. The usual is a second round 6 months after the first, but I was having problems again so they did it sooner. My first infusion was the longest - about 5 hours. They go very slow on the first one because some people have allergic reactions. That is why they give benedryl, steroid and tylenol first. I felt a little faint the first time, but it passed. Some people get a headache, I did not. I felt fine after, but was tired. Tiredness comes with the disease. The rest of the infusions were less than 4 hours. She should do fine unless she has a reaction. They will slow it down or stop all together if that happens. The nurse that did my infusion said only one person he's done has ever had a reaction, so it is rare. She will see big improvement a week to two weeks after they start. Wishing you the best.

[PattyL]

Great information. I am waiting for insurance authorization for rituxan.. I learn so much from you guys. Thank you.

[lesa]

Hi BigSister. I had sent you a PM but in case you didn't receive here is the Rituxan co-pay card info. This is very helpful. Genentech will give you $10,000 a year for Rituxan infusions. Just give the card to the center or office where you are getting the infusion. The card can/should be renewed each year regardless if an infusions is needed or not. I always have it ready just in case.

Here is the link:
https://racopay.com/

If you didn't receive my PM regarding your questions just let me know. I can post or send again.

Best Wishes

[BigSister]

My sister finally has her first rituxan infusion scheduled for Aug 5th!

[annekat]

My sister finally has her first rituxan infusion scheduled for Aug 5th! Glad to hear it! :thumbup:

[kaysee]

My sister finally has her first rituxan infusion scheduled for Aug 5th!


How did the first treatment go? Thinking of you and your sister.

[BigSister]

Thanks for asking, I meant to post an update earlier!

Her first treatment went really well - lots of nerves but it went completely smooth. Took about 5 hours, she didn't have any reactions so they ramped up per the standard schedule. She did get a bit queasy so I'd definitely recommend having a tummy full of solid food or snacks with you. Saltines and a 7-up fixed it almost instantly. No side-effects other than being very tired, which isn't uncommon for her anyways!

Her Rituxan dose was 825 mg
She received oral benadryl
She received solumedrol via IV (I think it was 100mg)
Started the Rituxan at 20 ml/hr
every half hour increased by 20 ml/hr if she wasn't having any reactions (which she didn't)
Max ml/hr was 161 but I think it ran out before she got that high.
Next time they start at 101 ml/hr.

She *finally* heard back from the rheumatologist she was referred to - she'll see him after her 3rd Rituxan, before the final dose. Better than never I suppose... :/

She also started to see a new ENT who seems to be phenomenal. He specializes in chronic situations so much better care for her. She's using an antibiotic in her saline rinse and also on a new round of cipro because they did grow bacteria with her latest swab/sample. She thinks that is really helping with her sinuses.

Anyways, thanks for the support - so very much appreciated! I'll definitely keep you posted!

[kaysee]

Glad to hear all went well.