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

[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 (http://shop.neilmed.com/Products/Sinus-Rinse/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

[annekat]

I might mention another thing about breathing, in addition to the excellent points Gab made above. Shortness of breath, wheezing and coughing, can also be caused by acid reflux from the digestive tract. Prednisone can cause excess stomach acid, and some doctors will prescribe an acid blocker such as Prilosec to take along with it. Others, including mine, don't routinely do that. I thought all my coughing and wheezing was due to mucus in the throat and asthma, which I have a history of. And some of it probably was. But I recently had an endoscopy to try to find a source of bleeding, since I have become anemic. The results were that I had an inflamed esophagus, including some small ulcers, as a result of acid reflux, and that was causing a lot of my problems. In fact, I was coughing so much after the endoscopy, that they decided not to do the planned colonoscopy for fear of puncturing the wall of my colon. The doc prescribed Prilosec (or generic, omeprazole), 20mg. twice a day, and after a couple of weeks, my breathing and coughing have gotten so much better, I'm amazed! I'm not on a high dose of pred anymore, but this has been going on a long time, without my being aware of the extent of the problem. Just another possibility for people with breathing issues.

[Gab122]

Another thought I had - could you ask the dr to try a medication called Rituxan? It is administered by infusion. Some medications work better than others for some patients. Some studies have shown that it is helpful in both bringing a patient into remission and helping keep a patient in remission. Wishing your mom the best!

[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 !!!

[Gab122]

I think Annekat might be on to something. I just noticed that my shortness of breath is worse after a big meal/ eating in general. And of course I'm eating a lot because of the prednisone. Despite being on Prilosec since early Aug. I'm now thinking my breathing problems might be GERD related. I was put on fosomax to protect my bones and I think that worsens reflux. Fosomax was just started early Sept and reflux started shortly after. Breathing worsened around then too.

Adith, Does your mom have reflux? How is she doing?

Gab

[annekat]

I think Annekat might be on to something. I just noticed that my shortness of breath is worse after a big meal/ eating in general. And of course I'm eating a lot because of the prednisone. Despite being on Prilosec since early Aug. I'm now thinking my breathing problems might be GERD related. I was put on fosomax to protect my bones and I think that worsens reflux. Fosomax was just started early Sept and reflux started shortly after. Breathing worsened around then too.

Adith, Does your mom have reflux? How is she doing?

Gab I'm currently taking twice as much Prilosec as the packaging instructs, by doctor's orders, while my inflamed esophagus heals. I haven't been on Fosamax for a couple years, but that's interesting that your reflux started after taking it. I sure feel a lot better with the Prilosec, but also figure our shortness of breath could have multiple causes. The symptoms Wegs causes are bad enough by themselves! But I'm really happy with my progress on this and will see the gastroenterologist in a little over a week, to find out whether I'll decrease the dose, or what. My best advice is not to eat right before bed, because being horizontal gives the acid a better chance to come up from your stomach. For some reason, yogurt was especially problematical that way; I'd wake up coughing with a horrible, burning taste in the back of my throat. Best of luck in dealing with this!

[adith]

Hello members ,

First of all a big thanks to @annekat , @Gab122 , @MikeG-2012 and others for the valuable feedback posted to the thread . My mother is doing alright although severe fatigue and shortness of breath continues to exist . For a change i have got her to Malaysia and will be taking her to Singapore 2moro for a welcome break from her regular routine and monotony. I will update more once i am back in Bengaluru i.e my hometown.

@Gab122 , I cant exactly understand what is meant by reflux ??? If you don't mind please help me understand what it is so i can comeback with information as to if it is the case with my mother or not. Meanwhile cheers to all.

Stay healthy!!!!

[Gab122]

@adith reflux is when you eat food and it comes back up into your esophagus or mouth. (It is heartburn/ indigestion) Perhaps due to spicy food/fatty food, caffeine, certain medications, eating too much, eating before lieing down, etc.

I imagine your mother is more hungry when she is on high doses of prednisone. So the prednisone and over eating could be culprits. I am hungry all the time right now. Also if she is on fosamax to preserve bone density that can cause heartburn (indigestion/reflux) (prednisone is damaging to bones) Is she on Prilosec to protect her stomach while on prednisone?

Wishing you a wonderful time in Malaysia and Singapore.

Gab

[annekat]

Adith, the stomach produces acid to help digest food, and Prednisone is one thing we all take that can increase that acid, which may then come up our esophagus and irritate the trachea and bronchi, causing breathing problems as well as possible stomach and esophagus ulcers. Prilosec is one of the acid blockers used for this, with generic name of omeprazole, and there are others, such as Nexium, with different generic names. If your mother is on pred and not taking one of these, you might speak to her doctor about it. In the US, these are available at drug stores without a prescription. Good luck.!

Sent from my MotoE2(4G-LTE) using Tapatalk

[drz]

Info on GERD GERD - Mayo Clinic (http://www.mayoclinic.org/diseases-conditions/gerd/basics/definition/con-20025201)

What Is GERD? Pain, Symptoms, Causes, Remedies, and More (http://www.webmd.com/heartburn-gerd/guide/reflux-disease-gerd-1)

[nagesh]

Hi Adith,

I am Nagesh from bengaluru, my son has WG since 6 years, you can call on my mob 9341215692

[adith]

Hello friends ,

So we finished our Malaysia+Singapore trip sometime back . It was good for me personally but my mother hardly enjoyed as severe fatigue took over for every 7 or 8 steps she took . Somehow she managed to see the most famous attractions of Malaysia and Singapore. I wish we could travel to more countries in the future but my mother seems to have lost all confidence after our recent trip and that is due to her feeling awkward about getting help in the form of wheelchair . I am sure she never imagined herself with a wheelchair on a happy holiday . I think it was taking a toll on her psychologically as it kept on reminding her of the ailment and condition she was in. My eyes are getting wet as i write this but my mother informs me at this stage she can hardly taste any food and enjoy as all her taste buds have gone numb . She cannot eat what she wants and enjoy , she cannot go where she wants and of course it is very difficult for her to be of help to people around her as she herself needs help. She finds it very difficult to feel satisfied in any way and thus feels life is not worth living anymore. Whenever she gives me this line of thought i breakdown myself as i simply do not know how to handle or respond or reason with her .
Anyway i wanted to share some feelings with you all and interested to know how fellow members have found reasons that help you stay motivated positive and filled with hope .

[Gab122]

adith I'm so sorry your mom is still struggling so much. Has her breathing improved at all. I understand her feeling despondent. I feel like I am on a bit of a roller coaster ride emotionally. Some days I feel stronger than others. I am currently being tapered lower on prednisone and I know that prednisone can cause anxiety and depression. I also think "coming to grips" with having this disease can cause anxiety and depression. Furthermore I believe the tapering off of prednisone process comes with its own set of challenges. So really it's no wonder she feels sad. I am taking an anti-anxiety medication - which I believe is somewhat helpful. Would she consider this? I also turn to my family and friends and share with them when I am having difficulties. I also rely on my faith in God to help me. I believe God loves me and that there is a purpose to this challenge that He has let me have. A good purpose. These are the things that help me. Perhaps you could share with your mom and let her know she is not alone in her struggles. I wish she could understand that her value is not only in giving care but she is a blessing to you all when she allows you to care for her. That is what family is for. Helping each other. She will once again be able to give care but perhaps her role right now is to be cared for by you. And let you love her, which is very evident. You are a good son or daughter. (I don't know if adith is a man or woman's name) [emoji4]

May God bless you with wisdom and your mom with healing.

Gabrielle

[annekat]

Adith, I think Gabrielle has made some good points. I also feel that WG is a challenge that has been given to me possibly for a reason, to further my growth as a human being. I would not expect everyone to feel that way. I have a lighter case of WG than some, though it is not without its problems. I really empathize with your mother on the difficulties of having the energy to lead a full life and enjoy traveling and such. I just have no desire to travel, I think it would be stressful and I wouldn't enjoy it. I hope that will change someday. I know there are people on this forum with WG who enjoy traveling and have even done things like climb Mt. Everest. We are all different. I just like to stay close to home and enjoy my familiar routines and surroundings. I don't remember how long your mom has had WG, but I certainly think there is a chance that things will get better for her. In the meantime, perhaps she should try not to do too many things, not demand too much of herself, and let others help with the everyday issues of life. And maybe she can regain some of her hope and find enjoyment in simple pleasures. May you both be blessed with her eventual recovery from the worst of this disease.


g

[adith]

@ Gab122 , annekat .... Thanks for those replies ... I have shared with my mom as to how she is not alone in her battle and that many across the globe feel much the same way. Well she does take a tablet called Anxit to keep herself calm and also to get some sleep. Beyond all these medications there are some unavoidable irritabilities and some inevitable downcycles in mood . I understand that it has to be dealt with . @ annekat , like you said my mother too is not very keen on travelling but we did the singapore trip as it is closest to my country and Singapore is one of those countries which has been praised so much and rightly so and hence we went there for a change . No more travel for my mother as she saw the place she always wanted to and now the rest does not matter to her . @ Gab122 Also I am Adith (her only son) and it is very much a male name :smile1:.

@ Gab122 can you tell us what medication you are taking to keep anxiety or mood swings under control???

Also friends , i wanted to know how many of you have issues with taste buds going numb . It is relatively less discussed and so I was curious to know.

Overall my mother is doing fine (new normal for her) and she still cooks for us and does most of her daily routines herself but yea with difficulty. Its just that the medication for almost 3 years after diagnosis worked pretty great to the extent that she or for an outsider it was as though she had no ailment and was quite healthy . Anxiety only in the recent past for about past 6 months where the normal for her is going down as days and weeks pass by.

Anyway friends , I am grateful that there is a community with which i can share and get some feedback. ALL the best for everyone and keep posting :smile1:

[Gab122]

adith you are a good son. [emoji4]

I take Effexor for my anxiety and it helps a bit with depression. I tried other meds too prior to this (Wellbutrin, lexapro, Prozac) and it may take a while to find the right med for your mom. I am mostly calm and not irritable (tho prednisone does make some people irritable) I am perhaps only slightly less patient with my husband and 12 year old daughter at times. But not usually. I think getting enough sleep and resting when I am tired is really important. I think I'm close to your mom's age. (?) I'm 49. It is a stage with hormone fluctuation already. Then when you add medication side-effects and the anxiety and depression of an incurable disease... it's a heavy burden at times.
Tell your mom, Gabrielle in America is thinking of her and saying a prayer. [emoji1374] maybe she would enjoy being on this forum herself as well? Does she speak English?
Peace

[annekat]

Adith, to reply to your question about taste buds; yes, I think mine have gone a little numb, but not to the point where I can't enjoy the taste of most food. But texture of food has always been important to me and I think now is even more so. I cannot smell, and I know smell and taste are related, so I guess I'm lucky I can taste pretty well. I know that smell and taste can return, though at this point I'm not expecting smell to return for me. Can your mom smell things? Everyone is a little different. I hope she will regain any of her senses that have been lost or impaired by this disease.

[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.

[drz]

Sorry to hear this. Hope you and family are getting the support you need.

[annekat]

Adith, I'm very sad to read this, and thanks for sharing with us. Your mother was too young to go this way. Treatment of WG has so many variables and doctors sometimes have to guess at the right course of action. You were right to return her to her previous pred dose. A jump from 15 to 10 is too fast for most people after being on it long term. What a let down after seeing her blood work improve. I'm so sorry. I wish you all the strength you can find to get through this difficult time and eventually be at peace. With love from all of us.

Sent from my MotoE2(4G-LTE) using Tapatalk

[vdub]

So sorry to hear about this. I have added a candle in honor of your Mom to our Wegs map.
https://www.zeemaps.com/map?group=242717#

[Alysia]

I am deeply sorry for your loss, adith. May your mother rest in peace. Thank you for sharing with us....

we just lost 2 more weggies only this week (friends on facebook)...

Do the docs tell what was the cause of her death ? Was it an adrenal crisis ? Something with her breathing ? Other ?



You did the best you could have done with her, including your good advices about the pred.
Sending prayers for strength and consolation.

[vdub]

we just lost 2 more weggies only this week (friends on facebook)...
Alysia, do you want me to put candles on the map? If so, give name, date, and location.

[adith]

Thanks everyone for sharing my grief ..... I met my mothers rheumy for about 5 to 10 mins 2 days after her passing to discuss what i could have done differently to keep my mother alive . Unfortunately in my mothers case it was a cardiac attack. Heart beats get to erratic patterns , mostly they recover but when it does not , it has to end in a few seconds. So basically difficulty to breathe was the main culprit . It triggered irregular heart beat and the irregular heart beat triggered death.

My prayers go out to everyone who is suffering weggies... Please remain strong.... God bless all

[Alysia]

Alysia, do you want me to put candles on the map? If so, give name, date, and location.

Thank you, vdub. You are very kind.

The names are:

Amber Nicole Hatfield,
Passed on March 6, 2017
From Owensboro, Kentucky.

Mark Mayfield
Passed on March 8, 2017
From Belleview, Florida

RIP warriors.

[Alysia]

Thanks everyone for sharing my grief ..... I met my mothers rheumy for about 5 to 10 mins 2 days after her passing to discuss what i could have done differently to keep my mother alive . Unfortunately in my mothers case it was a cardiac attack. Heart beats get to erratic patterns , mostly they recover but when it does not , it has to end in a few seconds. So basically difficulty to breathe was the main culprit . It triggered irregular heart beat and the irregular heart beat triggered death.

My prayers go out to everyone who is suffering weggies... Please remain strong.... God bless all

Thank you for sharing the info with us. It must be heart breaking for you.
Dont beat yourself over what you could have done. It sounds like an event which was sudden and not under medical control.
Your mother passed embraced by your love & caring. It was God's will.

God bless you and comfort you and your family.

[vdub]

Both are now on the map. Sad to see so many candles appearing....

[Alysia]

Both are now on the map. Sad to see so many candles appearing....

Thanks vdub. If there is a space for only one name, can you please change the name of the girl from Owensboro ? We call her Amber. I hope its possible.

[vdub]

Made the change!

[Debbie C]

Adith, I don't know why I didn't see this thread earlier. I just got done reading the whole story and I am now in tears. I am so sorry for the loss you and your family are going through, I hope in time you can find peace knowing she is no longer suffering and that you will forever have an angel looking over you. At least you were able to take her on a holiday where she always wanted to see and that you did all that you could. :sad: