my sister....

[Lila]

I posted something earlier..not sure where it went...
My sis has just been diagnosed with Wegeners...after over 10 years of suffering. Last week she could not breathe and was rushed to Jefferson (Philadelphia). They did an emergency trache on her and she was home 2 days later....with the trache too. She is STILL having problems breathing. It seems that once she 'coughs' it up...she is much better. This is not easy for her..she has to cough and cough so hard that last night blood was in her trache. However, this morning she said she feels much better since it has 'come up'.
She is scheduled to go back to the doctors on Thursday to have an Endoscopy and possibly remove the trache. I don't see how this is possible since she still has trouble breathing with it in. However, she is so depressed and crys often. Her doc there is an ENT specialist and he will be referring her to a Weg specialist. I hope it's a good one cause' she's been suffering too much.
Why do bad things happen to such good people?

[Doug]

Good. She should be seeing a WG specialist!

It sounds like the stuff I was coughing up in the early stages of treatment for WG- a congealed, bloody mass that reminded me of a banana slug, the huge slug that occurs in the Pacific Northwest of the US. Size-wise, anyway. Let's hope the WG specialist helps your sister.

When she feels better, you might encourage her to get on this support forum, where several people have experienced the same operation. The depression can be medication-related, too, though ten years of dragging around (and I bet she was!) until someone diagnosed her with WG in itself could cause her to feel a bit negative about her situation. Once again, I recommend this US site for more information on WG and much more. I don't recall giving this to you yet.

Front Page | Vasculitis Foundation

Read up on WG and help her to understand that once the doctors get it in control- and it's been working on her body, apparently for 10 years on top of everything- that she will experience recovery, slowly perhaps, maybe fast. Little by little most of her old world will come back to her. She needs patience now, and a change of attitude (which may come as she gets past this stage where breathing isn't easy yet). A positive and realistic attitude is her best friend after the doctors set her on the way to remission or whatever the "new normal" will be.

If you found the discussion on remission, you read that it isn't easily described. Even the doctors don't totally agree on a standard definition of it for WG. We on the forum tend to take this approach: "If I feel good, the test numbers mean nothing. They guide the doctors, but are subject to interpretation."

One of my numbers is consistently high, yet I am well, functioning, but use a cane in some circumstances just to feel safer from falls. That's my "new normal", and I can live with it. Your sister, "talking" with others on the forum can learn about their "new normal" after this surgery, and it'll help her get prepared a bit for her "new normal".

It may seem a brutal and unfair fate to come down with this disease, yet it is very encouraging to us on the forum that roughly 90% survive and thrive with treatment, compared with 30-40 years ago before diagnosis was more likely in the early stages and it was more like 90% died within two years of the initial flare!

[Lila]

Im really having trouble navigating around this web site.
The info you gave me is great. However, my sis is very negative about the whole situation. Her nose has also 'collapsed' and that needs to be reconstructed. Her toes and fingers are curled up and cause her terrible pain. She does get Enbrel injections, but due to all of this she hasn't had it in over 3 weeks. Since being released from the hosp. she is only on 10 mg. of Prednisone. She does not like to take meds.
Where do u live? Do u know any Weg specialists at Jefferson? Do u have a trache?
Sorry about all the questions. Im trying to find out whatever I can...
Thanks so much for all your info.
Maybe I should try reading the email you sent me to my sister...

[Doug]

Unfortunately, we are bad about staying on topic, which further complicates your job. We're trying, though. There are doctors listed in the Vasculitis Foundation site who can guide you to doctors or institutions in your area. Also, there are hospitals that specialize in WG listed. I'm not familiar with your area. Only one person (in the short time I've been on the site- since April) has reported a collapsed nose. Again, I encourage those people with specific experience of knowledge to join us in this discussion so you can get proper and useful information.

[Jack]

10mg of pred is not sufficient treatment for anyone with Wegener's unless they are already in remission! In the early days it needs heavy doses of imuno-suppressants together with steroids to knock the disease on the head. I started on a thousand mg of pred IV and also a chemotherapy drug called cylophosphamide also IV. Many people on the site started on similar medication, although perhaps not such large doses!
Your sis needs a vasculitis specialist before more damage occurs.

[onatreetop]

I am waiting to hear from Jon Hopkins Un. in Maryland. That was the closest WG spec. I could find to NJ. Any RA will take you on but and treat you but WG isnt common and most of them hear havent seen any cases. I was lucky, I hope to have found Dr. Soloway in Vineland. He is aggressive and has a big head, you may wait up to 4 hours to see him too. But he found the problem is treating it and is amazing. He has helped me get some of the meds even. He has been one of THE TOP DOCS on the east coast for years. he also has one of the largest infusion labs in the USA. He is a good man. A bit strange but good. JHU wants records then they call you for the appt.
The doseage does sound low even for me an early dx"s case. I recently had a FLARE and was bumbed to 100mg cty. and 60mg pred. It isnt fun but thats what it takes sometimes.
Were are you in NJ? I am near Ocean City. So Vineland is an hour drive. He is worth the trip and or day if you cant get her in to another specialist sooner. He will flat out tell you if he can help you or not the first time you meet. he says he has only seen a few cases 10 or 11. He currently has another besides me. But that's it all the other people are joint pain arth. peoples.

[Sangye]

Lila, I would urge you to call Johns Hopkins today. I think they'd get your sister in much faster since she had an emergency trach and is DEFINITELY not on proper meds. Like Jack said, a pred dose of 10 mg is completely inadequate.

I would also suggest that she not let anyone remove that trach until she's seen a rheumatologist who specializes in Wegs AND an ENT who does also. It typically takes months of treatment before they can safely remove it.

Her depression is normal. Can she join us on here? We've all been through it and can probably help.

[Lila]

I hope I'm sedin this to you correctly. Im really having trouble finding out where to find new messages and write new messages. I live in Galloway but my sis lives in Hammonton. She has seen Dr. Soloway in the past but did not care for him. Did you say that you DO have Wegeners? Wow, you are like right next store to me!

[Lila]

My sis does not have internet access. Im on the phone with her reading these messages. I hear what you are saying...about not removing the trache yet....however, you do not understand my sis's stubborness! If I try to suggest too many things to her she gets upset with me and ends the conversation. I told her that somehow, someway someone will get her to the Cleveland Clinc but her respose is "let me first just see what my doc at Jefferson says." I'm also afraid that if I suggest too much of something and she gives in to what Im saying...and it was a bad choice..I will never forgive myself. She wants to make her own decisions and I have to learn to back off. She is my older sis but Im very protective of her and her health.

[Lila]

I did post a respose...not sure if it goes to you...or for everyone..!