husband diagnosed with wegeners

[Peacefulfrog]

my husband (68) was diagnosed with Wegeners in December. He was in stage 4 kidney disease.He was given a plasma exchange and started on Rituximab. Due to insurance the second infusion was delayed and it was a month before he got the second infusion. I’m wondering if this is why it failed to work? He was hospitalized with lung involvement in February and was started on cyclophosphamide. His wbc went down to .75 last week so they’ve stopped that treatment as well. We are waiting on insurance to ok shots for his low wbc. Are there any other treatments if those two didn’t work. Thanks for letting me talk. And thanks for providing a place.

[piglette]

Your poor husband, I am so sorry for him. Having to wait for the insurance cannot help either.

[Peacefulfrog in reply to piglette]

It is frustrating for sure. Thanks for the reply.

[Nadine99]

Rituximab does not 'cure' and it's a long process. It's not just a 'one off'. Sorry but you are in this for the long haul and if your doctors are calling it Wegener's then they are out of date and you should find someone who knows what they are dealing with. It's Granulomatosis with Polyangiitis, GPA.

[Peacefulfrog in reply to Nadine99]

I understand there is no cure for Granulomatosis with polyangiitis it’s just a lot easier to spell wegeners. I’ve read through a lot of posts on several different forums, was just looking to see if there were others out that had a similar journey

[philtw]

Hi, So sorry to hear that your husband has GPA.

There are lots of people here who can give you advice.....I've had GPA since 2013.......

Generally Prednisone (steroids), methotrexate or Imuran are the drugs that are used to control GPA......Whilst Rituximab is used used and a reboot to kill off certain white blood cells that cause the autoimmune response......

There is also plenty of information online.

I had PAN when I was 21yrs old, full remission after 5yrs, however I was diagnosed with GPA in 2013 when I was 51yrs old and have relapsed x3 times.......

GPA has a 75% change of relapse.....so you have to have the mind set that you are likely to be on drugs for life.

I also live and work a normal life at 62 yss old, going out for long walks with my wife and dog.......

[Peacefulfrog in reply to philtw]

Thanks for the reply. It’s good to hear that normal life will be possible. Good luck to you!

[Support_helps]

I am 61 and was dignosed with renal limited vasculitis on Apr'24. We started corticosteroids and rituximab when my eGFR was about 24. I thought I was headed for dialysis. The Rituximab worked for awhile and my eGFR climbed to 33 over 3 months. But then it stopped working well and my Creatinine shot up to 2.81 in July. So we switched to cyclo 5mg/day to complete Induction. Thankfully it worked and by Aug-Oct by Creatinine dropped and eGFR improved as high as 57. By Dec I was over 60 and even hitting 70s by Jan. During this time I was getting lots of white and red blood cell injections especially the WBC which take a beating from the cyclo. I am now in Maintenance where we are trying Ritux again. But it may not be best for me so we are also looking at other options like Imuran....jury still out. Diet wise I cut out alcohol which was very hard but helped I think. I also stopped all processed food and went basically for fruits, vegetables, tofu, white fish...boring as heck. Now I recently got hit with Lupus but hopefully skin limited, still tbd. I also spent 40 days in hospital with a bout of pancreatitis where we still don't know the exact cause...either chronic alcohol consumption over years or the vasculitis. No gall stones so was not that. It's been a pretty awful year frankly but I'm hoping the ANCA does not come back and I have a successful maintenance. My cANCA is < 1:10 and has stayed constant there. pANCA still around but low at 1:20 after being as high as 1:160 I recall. I was told by the nephrologist that fortunately my type of vasculitis has a lower level of reoccurence so I am praying for that. I hope this is somewhat helpful and very best to you and your husband.

[Peacefulfrog in reply to Support_helps]

Thanks for the reply. My husband’s pANCA was also 1:160 when he was diagnosed. His creatinine went up to 7 but it is now down to 2.4. Small miracles! Good luck to you!

[BlueTheCat]

I'm sorry to hear you are at the mercy of your insurance company.

My experience... I too had a plasma exchange followed shortly afterwards by my first rituximab infusion. My second infusion was some 4 weeks later.

It took a while for the therapy to take effect on me. It is not an immediate response. It took 6 months or so for my symptoms to start to alleviate, during which time I was on a reducing dose of prednisolone/steroids.

[Peacefulfrog in reply to BlueTheCat]

Thanks for the reply. I guess I just need to learn patience. It’s just so hard to watch him struggle with one thing after the other. Good luck to you!

[oldtimer2]

It's terrifying having to deal with a disease that hardly anyone has heard of - including most of the medical profession and probably the officials in your insurance company too! You sound pretty clued up already about the disease - we all have to do our own reseach to find out about rare diseases.

I do hope that your insurance company up their game. Have you thought about complaining about the delays in approval of treatment? It might mean that your husband's case is transferred to someone more competant, but I don't really know how these things work (mercifully for me, I only know all too much about delays in the NHS).

It is, I'm afraid, a matter of you and your husband keeping up with all the available treatments nd being pro-active with pushing for them. I would, too, caution against anyone who offers miracle results. It's a long hard slog. Best of luck!

[Peacefulfrog in reply to oldtimer2]

Thanks for the reply. Insurance is certainly a battle in itself. I’m hopeful we get a call today that he can get started on the shot today. His Dr told him to “hide” until he can get started on the shots. Good luck to you!

[stuc]

it can be quite scary and daunting, I can only imagine how frustrating this is for both of you with the insurance company.

I was diagnosed with GPA in 2017, I took unwell while on holiday in France. I spent 6 weeks in intensive care ( 2 weeks of that in a coma) . I got weekly plasma infusions and cyclophosphamide once I came out the coma but it did take several weeks to have a significant impact. I did not have kidney impact but both lungs packed in.

I have had 3 flares since returning home , each treated with 4 x rituximab infusions and prednisolone and each time it took 4 - 6 weeks before I improved.

Every persons journey is different and may differ depending on how his body reacts to each treatment. The one advise I can give is to question everything that your specialist explains so that you can fully understand what is going on , why and the impact each decision has. It is all too easy just to accept what you are being told without questioning it ( this is from my own personal experience)

[gooseflight in reply to stuc]

That sounds rough. Did you have haemorrhage in both lungs?

[Peacefulfrog in reply to stuc]

He doesn’t have a vasculitis specialist…was diagnosed by a nephrologist. I plan on asking for a referral when we go next week. Was told by the dr last week that he was in remission but he continues to have night sweats and TERRIBLE mouth sores.

Sounds like you had quite the time of it. Good luck to you!

[gooseflight]

Your husband is still early in the process. It can be eight to 12 weeks before RTX starts to work. Also, your husband’s induction was atypical: should be x2 500mg doses within two weeks. If he doesn’t improve you may need to look to cyclophosphamide as a means of inducing remission. Is he on a high but reducing dose of prednisolone?

[Peacefulfrog in reply to gooseflight]

Thanks for the reply. They started him on cyclophosphamide 2 weeks ago but had to stop because of his wbc going down to .75. He just went down to 50 mg of prednisone.

Good luck to you!

[gooseflight in reply to Peacefulfrog]

Sorry, yes, missed the bit about his cyclophosphamide. Difficult though it is, hang on a little longer to see if things pick up. Good luck to you too.

[Main1234]

so sorry to hear your husband is having a dreadful time with illness and insurance problems. From your email sounds like he has had the “heavy” guns plasma exchange and cyclophosphamide although he dropped his white blood count. Unfortunately Rituximab is a slow burner and can take a few months to slow effect. Had he been on high steroids 60mgs each day?

My husband has had GPA for over 7 years and it came out of the blue. He had all the above treatment but not plasmas exchange. He now is fit for his age and there is good treatment available as long as you are under a consultant who has experience with this rare chronic disease.

This site is informative and supportive. Keep in touch. In some ways it’s harder for the partner negotiating medical professionals and keeping positive.

Best wishes

[Peacefulfrog in reply to Main1234]

Thank you for the reply. He is struggling right now with awful mouth sores…… I read this morning that they could be due to his low wbc (.75). Still fighting with insurance for the filgrastim shot. I’m hoping to get a call today so we can get the shot going.

[Main1234]

mouth ulcers are really painful and unpleasant, often happen if you are immune suppressed. Have you any treatment for them ?

If you use the search bar you can find out what other people used to help with mouth ulcers

[Peacefulfrog in reply to Main1234]

I have been searching the forum. Have tried a lot of the things that were suggested. I called his Dr today because he still hasn’t been able to get the figrastim shot for his low blood count. Dr said to go have labs done and the white blood cell count was down to .36 from .75 on last Thursday. He’s done nothing but sleep all evening and I haven’t heard back from the Dr. not sure what I need to do.

Thanks for talking to me.

[Main1234 in reply to Peacefulfrog]

Can’t imagine how worried you must be in im no expert but if it was my partner I would like you be very proactive. Can you access any other facility? In the UK I would be taking him to A&E. Not good that his white blood count count is still falling. He really needs some intervention.

I had to advocate for my partner and be very assertive when he was so ill as I nearly lost him.

[Peacefulfrog in reply to Main1234]

You were right. Came to emergency last evening. He has sepsis and his wbc has gone down to .07

[Main1234 in reply to Peacefulfrog]

Sending love to you .

[Peacefulfrog in reply to Main1234]

Thank you❤️

[Erichtheviking]

Well done you - going to A&E. Hope he's doing better?My hubby's had vasculitis in his eye since 2016. We are a long way down the road now. He had low blood count, still has low iron to this day. His long term medication is Methotrexate. He's due to come off it in April. He is still having flare ups of Lupus. He had 2 operations, one to remove his lacrimal gland 2nd one was an eye lift and scar tissue removal. 2 x biopsy's. There have been times when he just wanted his eye removed due to the pain. He has poor vision now and very dry eye, drops at least 10 times a day.

I know how stressful it is being 'the carer' and sorting out appointments, meds and everything. Hubby is 62, had 2 x back operations, arm cuff repair op, prostate cancer, he's not maungy but his eye drives him up the wall. Hope you're taking care of yourself 🤗. Ask me anything x

[Peacefulfrog in reply to Erichtheviking]

Thank you so much for sharing. We are back home now. He ended up with neutropenic fever and panctypenia (sp)?

Anxiously awaiting labs and Dr visit this week. While in the hospital his heart showed some symptoms (low ef rate) cardiologist wanted to do a heart catch but his nephrologist said his counts were to low so we will address that next. Good luck to you and your hubby.