Wegener's Granulomatosis, Wegener's Disease Blog

Information for those that are new to Wegener's Granulomatosis Disease

This was posted in the WG-Discussion email group that I am a member of. I thought it worthy of posting here as it contains some great info! You can find the Discussion Group at www.weareb.org.

Andrew

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NEW TO WEGENER'S GRANULOMATOSIS OR TO THE WG-DISCUSSION EMAIL GROUP?

Hello, and welcome to the wg-discussion e-mail group. It's good you found this group as the many members have lots of support, information, and experience. Mike and Zandra Clay set it up about nine years ago, and keep it going for which we're all grateful. I'm glad you are already a member of the wg-discussion group so you are able to be in contact with other WG patients.

This "canned" email that I usually send to newly diagnosed WG patients is expanded in my web page on WG (See address at the end). Here I just try to hit the high points.

I address this directly to you because it's lengthy and because most other email members have already seen information such as below. I'm not medically trained, so NOTHING I say here or anywhere is to be taken as medical advice. All medical problems, symptoms, and questions should be referred to licensed medical professionals.

GETTING CONNECTED

If you haven't already, you can join the Vasculitis Foundation (VF) formerly the Wegener's Granulomatosis Association (WGA), for $25/yr in the U.S. (U.S. $30 to foreign addresses) and receive their bi-monthly newsletter. If you can't afford a membership, VF (WGA) will subscribe you to their newsletter at no cost.

You can get a "patient packet" by downloading from the WGA web page, the 150-page file in either Microsoft Word or Adobe Acrobat format, or by requesting a hard copy from the WGA.

Contact them regarding membership and the patient packet at their web page at http://www.wgassociation.org/ or phone 1800-277-9474. Outside the U.S. telephone 816-436-8211

There are some support groups around the U.S. that meet. A list of contacts for various states and countries is on the WGA web page at http://www.wgassociation.org/aboutwga/areacontacts.jsp The contact for your area or state or province can help you get in touch with other WG patients in your vicinity and tell you of any WG support groups near you.

Also, there's a Monday night live chat on Internet Relay Chat (IRC) at 9 PM EDT on Newnet, channel #wegener (note-- no 's). Instructions on downloading and installing a client IRC program are at http://www.weareb.org/WG/chat.jsp

Or one can use the "Chat" button on the Newnet web page at http://www.newnet.net/. You might have to type "/join #wegener" in the command line after connecting to Newnet, or ask an IRC operator (IRC OP) for assistance.

In July of 2006, there will be a WGA Vasculitis Symposium in Baltimore, Maryland. If you are able to attend, you'll have a chance to meet other WG patients, to hear and question leading WG experts, and eat and drink too much (a danger with all medical meetings).

WHAT ABOUT WEGENER'S?

As you may know, getting diagnosed is often the hardest part of having WG.

And as you may know, WG is chronic and incurable (excepting perhaps by a very risky and expensive process called stem cell transplant).
Consequently, a WG patient has to be followed the remainder of his/her life, with periodic blood, urine, and imaging tests.

As the details of the mechanisms of WG remain incompletely known, it's not possible to know exactly what triggers a relapse. Relapses are always possible, and often seem to be triggered by exceptional physical or emotional stress, or sometimes perhaps by infection. Remission of WG can last years, or months, or weeks. A few lucky ones never seem to have a relapse.

It's only natural to feel frightened and perhaps despondent because of the damage WG has done or might do. Over time WG, patients find that their spirits improve and the disease isn't so scary. Most WG patients get the disease into remission and go on to live satisfactory lives, even if not exactly as before.

A positive attitude can be a great help in adjusting to having a chronic
disease. Some patients find antidepressants to be useful.

WHAT'S THE FUTURE?

The outcome depends on how much damage the disease has done and whether early and aggressive treatment prevents further damage.

Every course one can imagine has happened to WG patients. Some adults take early retirement. Some get less demanding jobs or switch to part time. A few are able to continue working full time, but not many until after successful treatment. Some find their spouses leave. A few have gone to live with relatives to reduce costs. Etc., etc.

Children may have to be home schooled or receive specialized instruction and tutoring. College students may have to skip a term or reduce their workload, or drop out for a time.

CLINICAL TRIAL

WG patients can register at the Vasculitis Clinical Research Consortium
(VCRC) web page at http://www.rarediseasesnetwork.org/vcrc/ The VCRC is a project of the National Institutes of Health. The VCRC was formed in Jan. 2005 and has yet to engage in clinical trials regarding WG. In the future, they will be conducting long term "longitudinal" trials as well as gathering detailed information from patients to enable the researchers to develop better means of diagnosing and determining the level of WG activity.

As of May 3, 2005, there were 450 registrants representing each of the vasculitic conditions targeted. There are registrants from 47 US states and 15 countries. 293 (65%) are female; ages ranges from 80 yrs (median range 46-50 yrs).

GETTING DIAGNOSED

Unfortunately most physicians (including ENTs and rheumatologists) have never seen a case of WG so finding the right rheumatologist (or appropriate specialist) is vital.

It's best to be treated by a rheumatologist who has lots of experience treating autoimmune vasculitis. If you can't find such locally, try a major medical center or teaching hospital. The WGA consultants can consult (at no cost) with any physician treating WG, so your physician has more expertise available if needed at http://www.wgassociation.org/aboutwga/consultants.html

Given the seriousness of WG and the medications used to treat it, it is wise for WG patients to get a second opinion. Two excellent centers that specialize in the autoimmune vasculitides are:

Johns Hopkins Vasculitis Center
http://vasculitis.med.jhu.edu/
Cleveland Clinic Center for Vasculitis
http://www.clevelandclinic.org/arthritis/vasculitis/default.htm


Patients can ask their current physician for a referral for a second opinion at those Centers, or other places where the needed expertise can be found. Many health insurance packages cover second opinions

SOME THINGS TO KNOW

Before treatment for WG, patients should be tested for Alpha-1 Antitrypsin Deficiency (AATD) as it is found in about 8% of WG patients and may require Alpha1 replacement therapy to avoid liver and lung damage. If that hasn't been done, the patient should ask his/her physician about ordering the test.

As both lungs and kidneys can have significant damage without overt symptoms, it's necessary for WG patients to be closely monitored for changes in those organ functions. CT scans can pick up lung abnormalities that x-rays miss. MRIs are better for soft tissues than ct scans. The fifteen-slice MRI is preferable to one doing fewer slices.

Kidneys can fail rather quickly at times, so while WG is active, a frequent urinalysis including urine creatinine level is appropriate to make sure no kidney damage is occurring or to assess improvements in functions.

Weekly or bi-weekly blood and urine tests you may be receiving at the start of treatment should be frequent until your disease is in remission. It's generally recommended that a WG patient test their urine even after the disease is in remission to make sure that silent kidney damage isn't occurring. Inexpensive urine dipsticks are great for that,

You should know that WG can affect virtually any organ in the body. A significant percentage of WG patients have "silent" heart damage, so it's reasonable to have your heart checked for damage caused by WG.

SOME SUGGESTIONS

If you didn't already, I suggest you now start a journal, noting symptoms, medications, copies of lab and imaging test results, appointment schedules, questions for your physicians, and answers to those questions, etc. Over time this will be valuable to you as details
are easily forgotten. Write up a medical history that shows all major
illnesses and surgeries.

Always prepare for appointments by preparing a list of questions and concerns. Ask your physician for copies of his report after each appointment.

Every interaction with your physician(s) should have an advocate in attendance to take notes and ask questions not thought of earlier.
Physicians tend to spend more time and be more thorough when an advocate is present. Your advocate can be a family member or friend who helps you by discussions of treatment options and other aspects of your treatment.

TREATMENT

For rapid reduction of inflammation due to WG, very seriously ill WG patients may require plasmapheresis in one or more of a series of treatments. It's very important that a person be tested for Alpha1 Antitrypsin Deficiency (AATD) before receiving plasmapheresis. Some seriously ill WG patients have responded well to intravenous immunoglobulin G (IVIg).

Most WG patients are treated with Prednisone and Cytoxan (or other corticosteroids and immunosuppressives). Recently physicians have realized that vasculitis such as WG can cause blood clots so many patients are put on a permanent daily low dose 81 mg aspirin.

Usually a strong immunosuppressive such as Cytoxan is needed for some time. Prednisone alone rarely gets the disease into remission.
Alternative immunosuppressives may be effective. Sometimes only Cytoxan will be effective and must be used. Where major organs are threatened, Cytoxan is commonly required. If you are put on immunosuppressive meds, you'll need frequent blood tests to see that your white cell count hasn't dropped too low.

Unfortunately, used in the dosages and durations required, Cytoxan and alternative immunosuppressives may cause female infertility. Women may want to discuss ways of preserving fertility with their physician before starting treatment. Before starting Cytoxan, females can have eggs frozen, or a section of ovary tissue taken for later replacement or there may be other methods to guard against infertility. Male fertility may be affected, but may recover after Cytoxan is stopped.

Parents of children and also young adults may need to discuss possible infertility effects of medications used to treat WG with an endocrinologist.

There is a recent biological medication called Rituxan (Rituximab). It is expensive, but has proved effective where conventional therapies have failed. It probably doesn't affect fertility. There's a clinical trial, RAVE, using Rituxan to treat WG. Dr. Specks at Mayo Clinic, Rochester, Minnesota has treated some WG patients using Rituxan with good success.

THEY JUST DON'T UNDERSTAND

It's good if your family and friends are supportive. That makes the trip back to health so much easier. They may not understand you are ill because WG patients often don't appear ill. Patients on prednisone often gain considerable weight that can lead to awkward questions.

Persons on medications used to treat WG can be very moody or irritable.
It takes some time and learning on the part of friends and family to realize that you don't have complete control of your emotions while on WG medications.

Sleep aids sometimes help to get a good night's rest so one can not be completely worn down the next day.

WARNING IF ON PREDNISONE OR SIMILAR STEROID

If on Prednisone (or similar), then you probably should be on calcium supplement (1200-1500 mg/day), extra vitamin D (400-800 units/day), and an osteoporosis prevention biphosphonate such as Fosamax, Aldronate, or similar.

If you are not on those supplements but are taking Prednisone, ask your physician about the need for those. (Apparently, children should not be on a biphosphonate).

Prednisone can cause glaucoma, so periodic measurements of internal eye pressure are required to prevent that. Also, ask your physician about the use of Bactrim DS tablets for prevention of Pneumocystis pneumonia
(PCP) and because it may be helpful in preventing relapses. It is prescribed in dosages varying from 3 tablets per week up to two tablets per day.

MORE BUMPS IN THE ROAD

Some WG patients find they have little trouble with their sinuses. Some find they need to do nasal irrigation to keep things clear. If let go, sinus inflammation can do serious damage. Once the disease is in remission, surgery can correct the "saddle-nose deformity" if that occurs.

Most WG patients find they don't require pain control once the disease is under control, though if damage has been severe, then pain medications may be required.

An endoscopic exam by an ENT who knows what to look for can detect sub-glottal stenosis (narrowing of the trachea) or other throat abnormalities that might be involved in shortness of breath.

AND FINALLY

For over six years, I've compiled information on WG into an .html web page. It's now available at http://www.wegenersgranulomatosis.net/

Note again that I have no medical training, so anything said there or to this e-mail group may not be correct and may not be applicable to you.

Best wishes for effective treatment of your WG followed by remission of the disease.

posted by Andrew MacLean @ 8:17 AM