Scared Mom of WegGPA and widow of PAN

[Fifi]

Over the last 5 years, my daughter has been taking meds ranging from methotrexate to imuran to cellcept and loads of prednisone, yet none have been effective in treating Wegs. Recent excruciating flare ups are in sinus and ears. She will undergo first rituxan treatment next week (SEP 23). In her words, "Don't worry and trust the process is what I need to remind myself. I'm scared of what's to come but am trusting that everything will work out...There is so much more I want to do in this life and am hoping the chemo will put this disease into remission...."

My husband of 36 passed away on Aug 13, 2010 after a 4-day period: 1st day, saw specialists and had new round of tests done; 2nd day, admitted to hospital due to high levels of infection showing in blood; 3rd day, was administered heparin (blood thinner) to prep for invasive procedures next morning (note: he had prior artificial mitral valve so that's why had blood thinners); 4th day, had massive brain hemorrhage and was pronounced brain dead. The pathologist performing his autopsy declared his case to be very "difficult," finding polyarteritis nodosa (PAN - another rare vasculitis disease) as the culprit of his fevers, night sweats, body pain throughout the years. Finally, in death he was properly diagnosed. He was 58 years old.

Even with our past, current doctors for my daughter still went around in circles for 4 years until being diagnosed with Weg last year. Although most readings say vasculitis/auto immune diseases aren't hereditary and genetics are not a factor, we change the mold.

I am scared, my daughter is scared (currently 32 years old). I'll be going to her first treatment next week, flying from SF to NYC. Any words of wisdom, advice regarding rituxan treatment or in general are very much appreciated. Thank you!

[Pete]

Hi Fifi,

In general, most rituximab treatments are non-events. I've had three courses since 2013. My only side effect is mild fatigue for 24-48 hours following each infusion. The infusions are generally given following an IV steroid and an antihistamine (usually benadryl). The first treatment takes 4-6 hours. If it's tolerated well, subsequent infusions last about four hours. The drug is started at a very slow flow rate to see if there is any allergic reaction. If so, the treatment is stopped and treatment for the allergic reaction is started immediately. The treatment then resumes. From what I've read on this forum over the years, allergic reactions are relatively uncommon and not particularly problematic.

It will take 4-6 weeks after the last infusion for the drug to take full effect, so don't expect a dramatic improvement.

Treatment is contraindicated if infection is present.

Assuming she tolerates treatment well, she has a good chance to lead a normal life, but will require ongoing observation to make sure she stays in remission once it's obtained.

[Fifi]

Thank you, Pete, for your insights and sharing. It helps me to know a bit more about this process!

[Aneinu]

My son only had one dose of rituximab (two times over two weeks) and he responded well to it w/o side effects.
We hope and pray that it goes well with your daughter.
Welcome to the group.

[vdub]

Although most readings say vasculitis/auto immune diseases aren't hereditary and genetics are not a factor

That may not be the case according to newer studies... Some studies have shown a possible genetic link and they have found what they believe could be the culprit genes. Below are two links to studies that indicate a genetic connection for a predisposition....

https://secure.e2rm.com/registrant/t....google.com%2f

Association of Granulomatosis With Polyangiitis (Wegener?s) With HLA?DPB1*04 and SEMA6A Gene Variants Evidence From Genome-Wide Analysis

[Fifi]

I am still learning how to maneuver throughout this site, so I apologize if I'm sending double messages, etc.

Thank you, Pete, for your insights and sharing! It does help to know more about the process and be more comfortable with what's to come, especially for Friday, her first treatment.

Thank you, Aneinu, for your welcome and for letting me know that your son responded well to rituxan. I appreciate your prayers and pray that my daughter will come out fine, too.

Thank you, vdub, for the latest info on genetic's role. This is very interesting, and I only hope and pray more conclusive research can be found for preventive measures, or better yet, a cure! I hope this can happen in our lifetime.

I will be traveling from SF to NYC on Tuesday so will be inaccessible the rest of the week. Will communicate later about my daughter's first rituxan treatment in the medication forum. Thank you for the support. Wishing you all the best!

[annekat]

Welcome to the forum, Fifi. I'm so sorry about the loss of your husband and that your daughter has WG/GPA. This is the best place on the internet to come for support and information. I haven't taken RTX so can't speak to that, but there are plenty who have. There seems to be a range of variations in how people respond to it, how soon they feel better, etc., but it seems that most are quite happy with it. Let us know how things go after you get back from your trip.

[Alysia]

Welcome to our family Fifi. I am deeply sorry for the loss of your husband. May he rest in peace.
It seems like your daughter became sick after his passing ? Must be so heart breaking for both of you.
Imuran & Mtx didnt work for me either. But rtx is THE med. For most weggies it works great. Although it might take time until it start to work. Weeks and even months.
Getting the IV is mostly calm. For me it can take around 7-8 hours first IV and less in the next. The only side effect that I had was lower blood pressure. So I drink lots of water and enjoy some chocolate. It elevates my bp
She might feel like sleeping during the IV which is ok.
Sending prayers. Please update us.

[gilders]

So sorry to hear of the loss of your husband, I can see why this would make you so worried about your daughter and perhaps not have faith in hospitals.

As vdub mentioned, there could be a hereditary link. Auto immune diseases effect most of my mum's side of the family, such as Lupus, sjogren's and rheumatoid arthritis). It's previously been discussed on this forum and it does seem common for other family members to have auto immune diseases.

RTX seems to be the current best choice for Wegeners, so hopefully there will be some improvement, although it does take a while to become effective.

I hope you are now a little more relaxed now that you know she is starting the best treatment. Like Alysia said, she will probably sleep during infusion. I think this could be due to IV anti histamines rather than RTX.