Nasal Wegeners diagnosed in August 2013 . Losing confidence in the recent past.

[adith]

Hello Forum ,

I am from Bangalore , India and i am here on behalf of my mother looking to get some opinions and expertise from fellow members.

Brief Case history of my mother (3 years since diagnosed)

Sex: Female
Age : 53
Weight : 79 kgs
Height : 5 feet 3

1> Ear pain and infection back in june 2011 . She had lost complete hearing ability along with a disturbing noise in the ears(both) . Tried various antibiotics from ENT , did not seem to solve the issue. Ent later wanted to investigate the case further and did a biopsy from her nose clog. At this point it was diagnosed as Nasal Wegener . Thankfully the diagnosis was quite early on and then started the journey of Wegeners in the month of august 2013.

2> Rheumatologist started with the appropriate treatment of 40 mg prednisone with MMF (Mycophenolate Mofetil) . She got her hearing ability completely restored without any noise in the ear. About 1 month she continued with the dosage and then started the taper of prednisone and increased MMF .

3> The tapering of Prednisone reduced weekly and eventually when the dosage became low enough the rheumatologist switched to another medicine Methotrexate . Up until this point my mother was doing healthy with all the ear infection and noise issue solved.
Methotrexate when tapered to 2mg per day she started having degradation in her eye sight. Immediately we consulted an ophthalmologist and he suggested Intravenous Steroid therapy instead of tablets. She recovered her eye sight to the extent of 20 percent.

4> We met another rheumatologist who started my mother on a series of Infusions. Each infusion consisted of 500 mg of Cyclophosphamide and 6 such infusions were done once every fortnight. At this point my mother was doing much better and eye sight had more improvement with continued usage of prednisone 10 mg /per day and also added another tablet called Azaron 50mg per day for maintainence. Along these lines at some point prednisone became 5mg per day while azaron became 150 mg/ day .

5> With prednisone 5mg /day and azaron 150 mg/ day , CRP increased and hence prednisone dosage increased again to 10mg / day and Azaron 200 mg/day. My mother continued being healthy for about 6 months with the same 10mg /day prednisone and 200 mg of Azaron .

6> For 2 months the tapering of Prednisone began again with continued usage of azaron 200 mg/ day. Again when the tapering came down to 5mg prednisone , she started having shortness of breath and loud sound of breathing and severe fatigue. Again prednisone was stepped up to 10mg/day . Lung xray and nose xray was done and suspected infection of trachea . Azaron was stopped and replaced with MMF again of 1000mg/ day. CRP levels did not show improvement and hence another step up of prednisone to 20 mg and MMF 2000mg/ day was suggested. Another tablet Folitrax 5mg per week was also recommended .

At this point my mother complains of following persistent issues

a> Shortness of breath , chest pain

b> Severe loss of energy , fatigue and usual swelling of face.

c> Throat pain, cough , continuous running nose , clogging etc.

d> Watery eyes , loud breathing sound (loud enough that everybody around can easily recognise)

Questions i have regarding my mother case

1> Can her breathing ease? Will the sound of her breathing reduce ??

2> Can she regain her energy ??? (Relatively acceptable energy and enthusiasm)

3> Is she really deteriorating or is it the new NORMAL???

4> Can she get a higher dose of prednisone and do better ??? Is it really required to taper when every single time prednisone was tapered she got difficulties.

5> Can more infusions be done????

Looking forward to opinions and suggestions from fellow members and survivors and doctors for advice.

Thanks in advance.

[vdub]

I'll see if I can answer your question. First off, it sounds like she is getting excellent care similar to what most of us have. Hats off to your ENT and Rheumy.

Generally speaking, I would say the fatigue is the new "normal" just as you suggest. She will have days/weeks/months where she might return to periods of higher energy, but will probably return to periods varying from light fatigue to heavy fatigue. My experience has been cyclical periods of feeling well and feeling very poorly. I'm currently on an up cycle of feeling well, so I'm doing as much as I can, while I can, because I'm relatively certain I will be going into a down cycle at some point -- maybe weeks, maybe months.

The "moon face" is a side effect of the prednisone. Pred is a wonderful and terrible drug both at the same time. It cures or aides many, many diseases, but the side effects can be quite devastaing. Google pred side effects and you'll see what I mean and you'll probably recognize some of them in your mom. As for tapering pred, yes, you want to get off pred as soon as possible due to the side effects. Some of us have hit a point where we are at a moderately low pred dose of 3/4/5 mg per day and we'll probably stay there for a very long time (years).

The shortness of breath is probably a result of granulomas in her lungs, but you have to ask your rheumy about that.

The other things you mention (watery eyes, dry throat, cough, etc) are mostly likely side effects of some of the drugs she is on.

Wegs is a terrible disease and, unfortunately, the "cure" (there is no cure) can be just as bad as the disease itself. Good luck and visit us often! We'll try to answer as many questions as we can. None of us are doctors -- our advice comes from our own experiences. Hopefully, as you progress down this road you can offer some advice of your own. Cheers!

[drz]

Some of us found that we have to accept being on higher dosages of Prednisone meds for a longer period of time in order to control our symptoms along with maintenance meds like AZA or MTX. Only a few people are lucky enough to attain and maintain a drug free remission from Wegs. My Weg expert at Mayo Clinic wants me to remain on 175 MG of AZA and 5 mg of prednisone indefinitely to try control my Wegs and try avoid a flare up. His view is that if it (my current meds) is working then "don't rock the boat" (slang term meaning don't make any changes).

The concern is that if I tried going drug free I will most likely have another flare up, since most people do when they stop all maintenance meds, and I very likely will not attain the same degree of remission I currently have. Or in my case a serious flare may very likely be fatal for me since I was very sick initially with significant lung and kidney damage and barely survived. His special expertise and research is on Wegs and treatment of it and has a lot of experience so I trust his evaluation and recommendations.

I have been on it for over five years with no major flare ups although there are increases in residual symptoms that some times require small tweaking of meds like adding oral steroid nasal spray and often antibiotics for infections which cause an increase in my residual Weg symptoms like joint pain fatigue, nasal bleeding etc. like a mini flare up.

A drug free remission of Wegs is very nice if it can be done and many people do attain it. It is worth trying to do if Weg symptoms are not life threatening and the side effects of the maintenance meds are very troublesome and the patient is very motivated to be drug free. But some times it is better to accept having some side effects from the meds and stay on the maintenance meds long term in order to try control the Wegs and to feel more functional in ones daily life. My doctors have assured me that many people with organ transplants and other diseases often stay on 5-10 MG of Prednisone for rest of their life and do OK for the most part.

[adith]

Thanks @vdub , @drz for the responses and i really appreciate it . Mostly my mother has received good care since the diagnosis but however as a person who takes care of her i feel that she might be deteriorating despite the good care and the concerning part is that my mother too feels she is deteriorating especially with new developed problems like SHORTNESS OF BREATH , SEVERE FATIGUE , LOUD BREATHING SOUND . For an outsider observing the sound of her breath is especially concerning as it feels as though she is gasping for breath and as though she needs to put a lot of effort to simply inhale and exhale . My concern or anxiety is not towards getting my mother to be drug free or medicine free but rather my concern is if she can do better with higher doses of prednisone or more infusions.

I honestly request more fellow members to throw some light on SHORTNESS OF BREATH issue and also SOUND OF HER BREATH . Sound is not an issue if it were to be the case where she did inhale and exhale comfortably with the sound but it is quite evident for an observer that she is not able to INHALE and EXHALE comfortably.
I really want to know if she can do better with higher doses of prednisone or another round of infusion . I know about the subglottic stenosis that does occur to a few WEGENER patients. So i got her checked with ENT and he says that there is no need for stenosis at this point and the issue with her breathing is basically FLEM that exists in her wind pipe . ENT recommended nebuliser and my mother has been taking the steam vapour 3 times a day to flush out as much FLEM as possible. It does help her breathing issue but to a very less extent but the issue persists.

Looking forward to more responses from other fellow patients who have experienced the BREATHING issue and if they have it solved in any way. I will keep the thread updated for any new results from her tests and also any doubts fellow members could have in their own case, they are welcome to ask me as i have taken care of my mother closely and monitored her progress through her WEGENER journey .

Thanks in advance.

Thanks in advance

[MikeG-2012]

I honestly request more fellow members to throw some light on SHORTNESS OF BREATH issue and also SOUND OF HER BREATH . Sound is not an issue if it were to be the case where she did inhale and exhale comfortably with the sound but it is quite evident for an observer that she is not able to INHALE and EXHALE comfortably.

From my experience, shortness of breath means there is something else going on. Most of the times when my breath was short, at rest short, not working too hard short, I have had additional internal lung issues. Most of those times, there were more lesions/nodules growing.

A simple chest x-ray could be a good start to see if anything is going on in her lungs. Maybe, it is even time for a lung CT if she has never had one.

[im so blessed]

If your mom was not scoped by an ENT to actually look down her throat for a stenosis i would highly recommend it !!!

[Lilly]

Hi, I have had wegeners for 26yrs. At first, when I was diagnosed, I had Granuloma tumors in my lungs. Oral Cytoxan took care of most of them, and they shrunk to little dots of scar tissue. No lung issues again until 2014, when I had pneumonia symptoms....and then it all started. I did not have any wegeners tumors in my lungs, but I was having a very hard time breathing, and I was wheezing a lot. I saw my PCP and he gave me breathing treatments and antiobiotics, and he said I had developed Asthma...Then when lungs were not getting better, I went to my Cardiologist....Same thing, CT showed no wegeners tumors, but areas of clusters of spiculated density. (They had NO IDEA what is going on) But that's not their fault, so I was put on home breathing treatments, and an inhaler, and a follow up CT Scan. 3 months later, CT scan no changes, but I am still having shortness of breath, heavy wheezing, especially at night. Sooooo, Cardiologist finally did a Bronchoscopy. Everything looked good, which surprised him, but when he rinsed my lungs with water and sent it off for culture, it came back that I had MRSA in my lungs. YIKES!!!!!! So I had a pretty heavy treatment for that for a month with the strongest antibiotics that will kill this infection. After a month, I was having some relief,(that was last year) but even now, I am on the inhaler "Spiriva" and I am still short of breath, and get tired easily. I also got a blood clot in my lung that was found in May. So now I am on blood thinners. I am going back to my cardiologist this month, on the 29th....I will let you know how all the tests on my lungs turn out. Best of Luck....So sorry that you are going through this.....its not easy, but we have to stay strong, listen to our bodies, do our best to not get depressed, and if you need to vent your aggravation, just do it here! We will listen :-) Blessings.... <3

[adith]

@ Lilly , @ annekat , @ Gab122 and others who have replied to this thread ...... Thanks a bunch for all the information shared . However i have to share a bad news to you all which is that my mother passed away on 25th feb 2017 morning around 7.30 am approximate in our house itself.

To give a brief history since i last updated this thread as to what unfolded in the last 2 to 3 months. So we got back from Singapore in October and as stated before the shortness of breath , wheezing , asthma were showing no signs of relief . So rheumy said he will start her on Rituximab . So she was given rituximab i guess 500 mg twice with a gap 15 days . Doctor had informed us that it might take upto a month or a little more for it to start reacting . So my mother held on to her life with the some hope that rituximab might work like how the cyclophosphomide infusions worked for her during early diagnosis . So after rituximab injections twice while all her breathing problems and other usual side effects of prednisone persisted she did get some releif and just a little energy sometime after 20-25 days after rituximab injections. As you all know the regular crp and esr reading check up was scheduled and was done a week before the unfortunate incident happened . Astonishingly when we had her checked up with the rheumy after 2 injections of rituximab for the last time , although she still was very weak and hardly had any energy and ofcourse breathing issue was still a nightmare , her CRP AND ESR readings were very much in control and at that point she was on 15mg of prednisone for maintenance. rheumy was very cautious with the dosage of prednisone drop in my mothers case always , however that last meeting with the rheumy when he saw that crp and esr reading were pretty much in control and that rituximab had probably started to work , rheumy took a little confidence from those readings and also to relieve those horrible side effects of prednisone he suggested that she drops her prednisone from 15 mg to 10 mg and as usual he scheduled us to meet him again in a month. While coming back after this meeting with the rheumy , since i had read information about the disastrous effects of dropping prednisone , i had discussed with my mother beyond rheumy suggestion that she can try dropping prednisone as the rheumy suggested only if she felt her body could handle it. So we got back and she dropped prednisone on the 1st day after the suggestion and she felt miserable . She complained that dropping of prednisone was making her even weaker . unfortunately on that same day she had taken this tablet called osteopos which is given to most WG patients but that tablet is not a regular one. From experience we knew that the day she takes osteopos , she always suffered and had a bad day on the day she took the tablet. So it became hard for us to decide whether she was feeling horrible due to usual osteopos tablet effect or the dropping of prednisone . Anyway i did not want to take a chance and with discussing with my mom , i told her to step up 5mg of prednisone back to 15 mg with which she had always maintained her condition . one day passed by and the situation somehow did not get better . Usual no sleep in the night , severe breathing issue and asthma continued.basically she dropped prednisone for just a single day before we ourselves stepped her back to original dosage. Although we stepped her back to original dosage , situation did not improve and on the early hours of 25 th feb 2017 around 4.30 am , her suffering was quite severe and my dad called me to come down to see her since he was also getting very tensed about her suffering the entire previous night. At 4.30 am in the morning , me and my mom and dad discussed amongst ourselves that we cannot wait for a month of this kind of suffering and then meet rheumy according to usual monthly schedule but rather we visit him that day itself . Usually she used to have no sleep in the night but was able to find some relief at around this time and get some sleep from 5 to 7 am. So my mother said finally on that morning after we discussed amongst ourselves that we meet rheumy after 7 since she always found some sleep during those hours . With this discussion she tried sleeping for a bit and asked me and my dad also to go to get some sleep and discuss again at 7 after coffee. At 7 my wife got up and has gone downstairs to prepare coffee. She has asked my mother if she wanted coffee and my mother said yes with nodding her head but not orally. At this point my mother has got up from the Drawing room where she was resting herself sitting (Offlate she used to get some sleep sitting rather than sleeping because of the breathing issue) and it seems with great difficulty she managed to come back to the bedroom where my dad was sleeping. She sat next to my dad with no reactions . My father tried telling her to sleep a bit but again there was no reaction from my mother . In the mean time my wife had brought coffee to the bedroom and observed nil response from my mother even while my father was making an effort to persuade her to lean on him for support if not sleep completely. Both my wife and my father were shell shocked to see total 0 response from my mother and it seems she was counting her last breaths. My wife shouted out to me to inform of the emergency and i immediately ran down to her bedroom and in the presence of all the three of us her multiple agonies came to an end within seconds and it was all over . With this my mothers journey came to a close at the age of 53 and we had lost the central support of the family.

[annekat]

Welcome to the forum, Adith. The ups and downs your mother is experiencing sound pretty familiar, as taking the meds and then trying to wean from them becomes a balancing act. Especially the prednisone. Many of us have been unable to get below a certain amount of pred, like 5-10mg., without having at least a mild to moderate flare. Some of us have found we have to go slower in the tapering than our doctors suggest. Some of us have doctors who let us manage this for ourselves when we get down around 10mg. I'm currently at 10 mg and at one time tapered down to 7.5mg. I then had a moderate flare and went back up to 15-20mg, I don't remember exactly, and then tapered back down at my own pace to the 10mg. I'm at now. And sometimes the aches and pains and other symptoms may be a temporary result of the tapering and you can just ride it out until your adrenal glands start to work again (after the pred has taken over their function and put them out of commission). Everyone is a little different in how fast they are able to taper and whether they can get completely off pred. Sometimes the adrenals will just not come back on board, and one will just have to continue with a low dose indefinitely. I will likely try to taper below 10mg. pretty soon and think the doc will give me leeway to go very slow, like 1mg. per month, perhaps. I've heard of people tapering 1/4mg. at a time! Most can go faster, but it's true that the lower you go, the slower you need to go, because each drop is a higher percentage of reduction, if the drop is the same each time. So I'm just adding to the good advice already given by those above. Best wishes to your mother for eventually putting those symptoms behind her. And it can take some time; it has been 5.5 years for me since diagnosis, and I was sick with nasal and ear problems for a couple of years before diagnosis. Let us know how things go.

[Gab122]

Hi Adith,
I'm so sorry your mother is doing so poorly with her breathing! I can tell you that even though I had lung granulomas seen on CT scan and I was diagnosed with a lung biopsy, I feel more short of breath now and my lung granulomas are almost completely gone thanks to the Rituxan infusions I received and the high doses of Prednisone I've been on. I was just diagnosed with Wegener's GPA on Aug 1 2016. I did have some breathing discomfort but I feel more short of breath now and I think it's probably due to the prednisone side-effects. I was on 60 mg pred for 6 weeks and then 40 mg pred for 4 weeks and yesterday I was told to further taper my prednisone. I do not have the loud noisy breathing that your mom has however.

My trouble started in my ears too like your mom. I had really terrible ear pain and fullness and then they put tubes in my ears and I had profuse drainage until I was diagnosed and treated for Weg GPA. My ears then got better and my lung granulomas shrunk. I also have sinus drainage daily.

I wondered if your mom has tracheal stenosis. But you said the ENT does not think so. How do they know? Did they look down her trachea with a scope at all to see if there was airway constriction? I don't know what that procedure is called but maybe it should be done if it hasn't been.

If the ENT is correct that it is mucous in her throat, maybe it's from sinus drainage and a saline sinus rinse would help with that. I highly recommend the NeilMed Sinus Rinse kit (Sinus Rinse Regular Kit)

Have they done a CT Scan of her lungs or just an xray? Sometimes (and it happened with me) granulomas don't always show up on chest xrays. My chest xray only revealed 1 granuloma, but the CT scan showed 3 granulomas and 2 nodules. So she may need a CT scan to REALLY see what's going on in her lungs.

If there is no mucous, or lung granulomas then it could be the prednisone making her feel short of breath. Like was posted before, prednisone (while also very helpful) has many unpleasant side effects including shortness of breath, puffy face, eye pressure, and anxiety (which can also make you feel short of breath) .

I hope some of what I have posted helps you.

Sincerely,
Gabrielle