Cancer: An Unexpected Consequence

Jerry NiebaumBy Jerry Niebaum, Ph.D., September 2007

In early 2003 I was diagnosed with Wegener’s granulomatosis and have been in continuous treatment for the disease since that time. Treatment has included Cytoxan, Methotrexate, and Prednisone to suppress the immune system to treat the symptoms of Wegener’s. This article is to help alert other vasculitis patients to one potential side effect of the long term use of immune suppressive drugs.

It was a small bump on my arm that I first noticed in early Summer 2007. Being a redhead with freckles I’m very used to seeing funny things on my skin. I was concerned about an itchy spot on my left temple just above my glasses line, so I made a June 15 appointment with my primary care physician. He thought the face spot looked suspicious and advised its removal. He was considerably less certain about the bump on my arm, but advised that he could remove that as well. After removal, the facial spot was determined to be a squamous cell carcinoma. We left the arm bump for later.

My physician recommended further surgery on the facial spot by a plastic surgeon to help insure that all cancerous tissue had been removed. I delayed any action on the bump on the arm until the surgeon visit on July 13. In his examination and without my prompting he noticed the bump on my arm, which had grown to the size of a dime and exclaimed, “What’s that?” We both laughed about that as I remarked that “I had hoped you could tell me." We scheduled surgery on both face and arm for August 10.

My Merk 8/10/2007On August 20 he reviewed the pathology report with me. “Good news on the facial spot. No additional squamous cells were found.” However, the good news was short lived because the bump on my arm was a Merkel cell carcinoma, a rare and aggressive form of cancer. According to one report there are only about 1000 cases of MCC (Merkel cell carcinoma) in the U.S. each year versus about 60,000 cases of melanoma. According to Web site: http://www.merkelcell.org/  “The exact causes of MCC are not known. MCC is associated with a profoundly weakened immune system, such as in patients with HIV, or in patients who have received an organ transplant who are on medications that suppress the immune system. Factors strongly associated with the development of MCC are:

Age over 65 years  (I’m 67)
Fair skin (I’m a redhead with light complexion)
History of extensive sun exposure (worked outside on farms as a youth)
Chronic immune suppression (for me, more than 4 years)

Yes, I fit all four factors.

My physician noted that further tests and additional surgery would be required. He proceeded to visit with a team of physicians for collective advice on how to proceed. They advised that I have a positron emission tomography (P.E.T.) scan. The procedure involves an intravenous injection of a radioactive sugar substance, FDG (fluorodeoxyglucose). Because cancer cells absorb sugar more than normal cells, they show on the P.E.T. scan. The FDG is injected and the patient then waits approximately 1 hour for it to circulate throughout the body. For me the entire process on August 23 took slightly over 3 hours, including the scan time.

On August 24 I got the good news that the P.E.T. scan did not reveal cancer cells in other parts of my body. The surgical team determined that on August 28 they would perform three surgeries: a sentinel node biopsy of the lymph nodes under my right armpit, excision of the area around the spot where the Merkel cell tumor was removed, and a skin graft from my leg to cover the wound area of the excision on the arm. For the sentinel node biopsy they injected a radioactive dye in my arm and used a gamma ray scanner to trace its movement to the lymph nodes under my arm. The node which collects the most dye is considered the sentinel node for fluids draining from that area of the arm. The surgeon removed that lymph node and one adjacent for biopsy. The pathology was negative, so gratefully, no further lymph nodes were removed. The arm and leg surgery went well. Further pathology on the arm tissue showed no additional Merkel cell presence.

Ok, so what would I have done differently? I would have and will be more alert to unusual growths or skin changes and have them checked immediately. Our own intuition about our health can be an important diagnostic tool for health care workers. Having said that I should also note that there is only a very fine line between such intuition and hypochondria. Modern diagnostic tools coupled with surgical skills and treatment options have helped remove some of the dread of the “C” word.

[Jerry Niebaum is the contact person for the Kansas City Chapter of the Vasculitis Foundation.]

Making the Right Decision - A Hip Replacement

By Kathy Savickas, November 2006

When I moved to Michigan almost 10 years ago I was in a flare, subglottal stenosis was rearing its dragonhead and I was on Cytoxan for the third time and prednisone. Five years later I still had the roving joint pain and had also started having tremendous pain in my left hip. I figured it was just part of the Wegener’s (WG) pain and did not pay much attention to it until I read an article about prednisone on the Internet. It stated that if you develop hip pain during steroid use then get it checked out because you may be facing avascular necrosis (AVN).

I called my primary care physician (PCP) and he ordered x-rays. Sure enough, I had AVN in both hips. It was about this time that I decided I needed to make a change in my health care. My roving pain was getting worse and I was living the life of “work and sleep” - nothing else. My doctors at home just did not know enough about WG to treat me. I searched the Internet and read about the Cleveland Clinic Foundation and my PCP contacted Dr. Gary Hoffman. I met with Dr. Hoffman three weeks later and he ordered scans taken of my hips. He confirmed the AVN diagnosis. Here comes the hard part - Dr. Hoffman told me it was up to me to decide when I wanted to have the surgery. I wanted someone to tell me I had to have it done; deciding to undergo surgery one more time was not a decision I wanted to have to make.

Over the next two years I lived with the pain but in February of this year I finally made the decision to have the surgery. Once I decided to proceed with the surgery I felt a sense of relief I cannot describe. Once I had the courage to make the decision, I had the courage to face the surgery. One simply followed the other quite naturally.

The deciding factor in having the surgery was the pain. I was tired of spending most of my days in bed eating Percocet after Percocet and barely getting any relief at all. Finally, one day I just decided I had had enough. I did not want to live like that anymore. Although I have always loved spending time in bed reading, napping and watching TV when I have the time, I was now spending all my time in bed because I couldn’t move - the pain was so bad I couldn’t concentrate to read, do stained glass or enjoy camping with my husband.

I felt the most important thing to prepare myself for my hip replacement would be to visit all my doctors and get an OK from them to proceed with the surgery. This meant seeing my ENT, Dr. Robert Lorenz, for the status of my subglottal stenosis and Dr. Martin Citardi, my sinus specialist, who keeps an eye on an opaque area in one of the sinuses above my eye. I also made an appointment with an ophthalmologist, as I had not seen one in over ten years. I had my yearly gynecological exam and a check-up with my dentist to make sure my teeth and gums were healthy. I also saw my primary care physician because he would be my physician at home after the surgery and would need to coordinate with the doctors at the Cleveland Clinic should the need arise.

I spent a tremendous amount of time on the Internet researching hip replacements. The Cleveland Clinic, Mayo Clinic and The Johns Hopkins websites all have extensive information on hip replacements. One site I found particularly helpful described in detail the steps through the day of surgery - and what to expect when you wake up - telling me where there would be tubes sticking out, how my pain would be controlled, what would happen each step of the way before and after surgery. I was glad to know what to expect when I woke up - there were no surprises and that was the best part of reading up on what was involved with the surgery.

The day of surgery went smoothly; the entire procedure only took an hour. My pain was always under control. To help control infection, the surgeon did not insert a catheter although everything I read said to expect one. So, for the day of surgery I had to use a bedpan. I also left the hospital a day early, surgery was on Wednesday and I went to rehab very early Saturday morning as my wound was seeping and they wanted me out of the hospital and away from infection.

The Cleveland Clinic alerts all patients to things they can do to prevent infection. Most emphasized was that your caregiver, be it a nurse, an aide or your spouse, should wash their hands before they touch you. I knew I would not be able to get out of bed by myself for awhile once in rehab, so I made a point to bring a bottle of hand sanitizer to keep by my bed and used it several times a day - on my hands, my table top, my phone, etc.

The day after surgery I was wheeled over to the physical therapy (PT) room for my first session. I stood up and it actually felt good. Best of all, Dr. Hoffman came to visit me and when he didn’t find me in bed, he followed my trail all the way to the physical therapy room. It was so good to see a friendly and familiar face! I did a few exercises - the scariest part was thinking that I would faint or not be able to accomplish much in rehab but I surprised myself and always felt better after a PT session.

When I left the rehab facility in Cleveland, a local hospital in the Detroit area called me to set up physical therapy at home for two weeks. My physical therapist came three times a week and followed through with the exercises I had started at the Cleveland Clinic. The rest is up to me to finish on my own over the course of the year.

Family and friends can help by asking if there is something they can pick up for the patient at the pharmacy - I always needed something. Offer to bring over dinner but do not stay to eat and keep all visits short. Keep hospital visits short too - I LOVED my visitors but got so tired after about 30 minutes I could barely keep my eyes open. Offer to clean the bathrooms or run the vacuum. My husband was great and kept the household going but a little help with cleaning would‘ve been great.

My AVN was a direct result of long-term use of steroids. Collectively, I have spent more than half of the past 22 years on prednisone - most of that time on high doses (over 20 mg). I have been on Fosamax and Vitamin D for just over a year and had a bone density test done a couple of months ago which showed an increase in mass. (A bone density test measures the mineral content of your bones and my tests always focus on the lumbar region of my spine and my hips. I have one done every year.)

I was never told exactly how long to expect my hip to last as each case is different depending on your level of activity. However, I do have a ceramic hip with a titanium shaft and chances are it will last 15 or more years. This is what I can tell you now - I have NO pain in my hip. I move better than I have in six years. Good thing as I have a new puppy to train and it takes a lot of quick moving.

This story would not be complete without complimenting ALL the people at the Cleveland Clinic, especially my surgeon, Dr. Peter Brooks.  I have no qualms about returning to have the other hip done as soon as the pup grows up.

Editor’s Note: When I met Kathy in 2005 she spent most days in bed, controlling the pain with medication.  She couldn’t travel or enjoy her hobby of creating stained glass art.  Now, only months after surgery, she volunteered at the Symposium and donated two new pieces of stained glass for the silent auction. Kathy also adopted a 2-month old puppy, Mr. Spoons, which she never would have been able to do before the surgery. When I pointed this out to her she said, “I’m 95% back to being normal. I feel great!”    

Participating in Research Study

A Patient's Perspective: Participating in the VCRC Patient Longitudinal Study 

By Jane Dion, March 2006

As a long time patient of Dr. Peter Merkel, who is the principal investigator of the Vasculitis Clinical Research Consortium (VCRC), and as one of the founders of the Churg Strauss Syndrome Association, I was given an opportunity to become one of the first participants in the VCRC Churg Strauss Syndrome (CSS) Longitudinal study.  This is my experience.

I registered for the VCRC contact registry in February 2006. I had procrastinated about registering because I thought it would be a long, involved process. In actuality it took all of two minutes to answer the questions on the contact registry. I was surprised at how easy it was!

Now patients may also register by mail or by calling a toll free number (866-313-9879).

I was contacted by letter in April 2006 and told that I had an opportunity to participate in a CSS longitudinal study.  When I agreed to participate in the study, I was sent pages of detailed information about every aspect of the study as well as permission forms. The informational packet made for interesting reading and was presented in non technical terms that made it easy to understand.    

The first visit took place at the BU General Clinical Research Center. Since BU Medical Center is a massive facility, I was glad to be met by Ashley Leavitt, Clinical Research Coordinator, who led me to the Research Center.

I met with Dr. Merkel on this first visit. He took a detailed medical history and examined me thoroughly. He worked with me to complete several questionnaires. He also gave me details about my role in the study and encouraged questions. I then gave blood and urine for testing. The first visit took about two hours but as I was fascinated by the whole experience the time passed quickly.  The second visit took 10 minutes as it was only a lab visit.

Dr. Merkel or an associate will see me every third visit for a full clinical work up including a physical examination, a history and labs. During the first year I will return to BU Medical Center on a monthly basis for lab work as well.* After that, I will return every three months. If my disease flares, I will be seen more often. The Research Center at BU Medical Center is a comfortable and friendly place. The nurses are experienced, gentle and considerate. Once, when it was lunchtime, I was offered a complimentary meal.

After each visit I was sent a check for $25 to help pay for gas and parking. Although my disease is mostly well controlled with medication, I often hear from people who are struggling because their diagnosis was delayed, or because their disease is resistant to standard treatment. As one of the “luckier” patients with CSS, I feel an obligation to do what I can to advance an understanding of this disease. In addition, I realize that advances in understanding one of the vasculitic diseases may bring greater knowledge of others.

Being in the VCRC longitudinal study feels empowering to me as I feel that I am actively doing something that might eventually help others with vasculitis. 

* April 2007 - Protocol has changed to visits every three months.

To learn more:

Vasculitis Clinical Research Consortium

http://rarediseasesnetwork.org/vcrc

Toll Free: 866-313-9879

Boston University School of Medicine

715 Albany Street, E533

Boston, MA 02118-3294

Self Advocacy

By Dianne Shaw, Past President VF

January 2006

Email: dgs@med.unc.edu 

Self advocacy. Not a new idea to vasculitis patients. Self advocacy is about helping the medical system work well with you and for you.

Because our diseases are rare and difficult to diagnose, locating and developing a strong relationship with physicians who know about and routinely treat these diseases is key to successful disease management. In our situation, the relationship with our doctor(s) is probably more collaborative than most. As patients, we learn what steroid dose works for us, what signals indicate the possibility of a flare. Communicating this "body knowledge" is critical to managing our diseases.

For example, in my experience with vasculitis, there have been times that I just felt something was wrong, even though tests did not (yet) confirm it. Not feeling well, joint pain, lower exercise tolerance. When this happens, my doctors trust my judgement- and I have on occasion been thankfully wrong that a flare was imminent- and work with me to develop a plan of therapy. Teamwork is essential.

Many of us keep medical diaries, an excellent idea, especially when you're asked, "has this happened before, when did this last happen or what steroid dose works best for you?" Also, since most of us have had countless procedures, steroid tapers and numerous rounds of therapy, keeping a record is helpful. With steroids, "chemo brain" and aging, my memory can use the factual help that the medical diary provides.

There are times when I am going into a flare or am on a large dose of steroids that my self-confidence dissipates. I don't have the courage to stick up for myself, Also, when I am in the hospital and am quite ill, self advocacy is a bit more challenging. I remember one stay when the team treating me was determined to send me for a procedure that my doctor had told me was not necessary. I had to have him paged so that he could tell this team that the treatment plan no longer included that specific procedure.

With me at that moment during my hospital stay was my sister, equally determined that my doctor's orders would be followed. An effective self advocate seeks and values the support of family and friends. They help you remember medical history, tell you jokes, listen to you and buoy you up when things are tough. An Internet listserv can also provide much-needed feedback and support. I see it time and time again.

Expert care is critical. The Vasculitis Foundation has 29 medical consultants, all experts in the care of vasculitis. Not every patient is able to visit with these physicians, and many patients receive excellent care from community or academically based doctors. I recently spoke with a young man whose airway was narrowing making it difficult to breathe.  He ended up in a hospital where the doctors had not performed the type of surgery he needed. Our consultants communicated with his physician, and plans were then made for him to have the appropriate surgery for his condition. Self advocacy at work - getting the expert opinion for the best care. Other patients ask their physicians to talk with the VF consultants, availing themselves of the latest knowledge while having their care provided closer to home.

Two strategies to keep up with current vasculitis practices and research are: (1) read the Vasculitis Foundation newsletter; and (2) attend the biennial symposium. Both the newsletter and the symposium offer the best ideas and thorough discussion of everything medical. Our consultants author articles for the newsletter and attend the symposia where patients and families have a chance to hear from them about the latest advances and to ask questions of these experts. Those of you who have the pleasure of knowing Bruce McDonald value his constant web surveillance about vasculitis and posts to the WG Internet Mailing List. "Dr." Bruce is the most knowledgeable patient I know.

Self advocacy can help you have some control despite the roller coaster ride of these diseases. Knowing your medical history through a medical diary, seeking the support of family and friends, learning as much as you can about your illness and receiving expert care - all make you a leader in your team of care.

Vasculitis and Fertility – A Personal Story

By Christy Abele, October 2005
Email: christyabele@sbcglobal.net

My name is Christy Abele and I'm a newly elected board member and area contact for Northern California. I was diagnosed with Wegener's in October 2002, six months after the birth of my daughter, Claire. I had just turned 34 years old. As any parent can attest, the first year of parenthood is a beautiful but difficult time. Developing WG during the first year of Claire's life was particularly challenging. In fact, one of the most difficult parts of that year was the realization so shortly after giving birth to my daughter that I most likely would not be able to have another child. But as I write this today, three years later, I am eight months pregnant and eagerly awaiting the birth of my second child. Below is the story of my journey from diagnosis, treatment, infertility problems and miraculously, conception. I am not a doctor, or a researcher, just a Wegener's patient, and a mother, telling her story.

My diagnosis and treatment were fairly straightforward. I spent 4 ½ months on Cytoxan and high doses of prednisone. After Cytoxan, I stepped down to a less toxic, maintenance drug called CellCept. Knowing that I wanted to have another child, my rheumatologist thought that this drug would be safer for my reproductive system than Methotrexate. I spent the next 18 months taking CellCept, the last six of which I began the tapering off process. It was around this time that I started to get serious about having another child. I saw an OB/GYN whose specialization is women with autoimmune diseases. She did some hormone testing and we discovered that my FSH level was borderline high at 10. FSH (Follicle Stimulating Hormone) is a hormone which, when tested can tell you if/how well you are ovulating and even possibly how close a woman may be to menopause. At this point, I was still six months away from being off my medication, so there was nothing I could do.

Six months later, I was retested and my FSH was up to 30. I was shocked! How could my fertility decline so rapidly? I was given the news by a nurse who, to put it lightly, was not the most sensitive person in the world. She point blank told me that I would never have any more children. With those words, I stopped using contraception and was surprised to learn that I conceived a couple of days later. The only issue was that I was not completely off my medication. I quickly tapered off of CellCept. However, seven weeks later I suffered a miscarriage. I'll never know why I miscarried, but I vowed that the next time my husband and I tried to conceive, I would follow my doctor's advice. I would be off my immunosuppressant medication for at least six weeks (and in remission, of course) before trying to conceive again.

A few months later when we began trying again, I started doing acupuncture and changed my diet. This was an attempt to naturally bring my FSH level down and possibly conceive. Western doctors will tell you that once a woman's FSH level goes up (and fertility declines) there is no way to reverse the process. I stuck to this strict regiment for three months and then had my FSH retested; this time it was at 42! At the age of 36, I was "officially" going into menopause. The doctor did an ultrasound of my ovaries and found very few egg follicles left.
 

The Cytoxan had definitely done a number on my reproductive system. While the medical community has made great advances in infertility treatments due to "structural problems", they haven't been able to figure out a way to preserve egg quality in women who are older or in women whose eggs have been damaged due to chemotherapy or other toxic treatments. The conversation with my fertility specialist quickly turned to egg donation and adoption.

Upon leaving the doctor's office, he handed me a prescription for a drug called Clomid, which is used to help a woman ovulate better. By ovulate better, I mean it helps the follicle inside the ovary to fully mature the egg that will be released in that month's cycle. The following month, I took the Clomid. I figured, what did I have to lose? Then the miracle of all miracles occurred, I became pregnant with our second child! As of this writing, I am 32 weeks along (8 months) and am doing great. I am having a very normal pregnancy. I have seen a perinatologist (a high risk doctor) once, my rheumatologist every few months, and of course my OB monthly. So far so good!

Conception after Cytoxan is possible although in my case, more difficult than before. There are some things to consider if possible, before starting treatment. For men, freezing sperm is a safe and relatively easy process (did anyone read Lance Armstrong's first book?) There have also been advances made in male infertility. In my research, I remember reading about a new treatment whereby an egg could be fertilized even using just one live, viable sperm. This would suggest that even men with a low sperm count could potentially father a child.

For women, it's obviously a little bit more complicated. Women with partners could consider freezing embryos before treatment. This process takes a couple of months however, and in many cases (including mine) there is not enough time. The doctors at Stanford University are developing a program whereby they can remove one ovary of a cancer patient before treatment, cryogenically freeze it and re-implant it after treatment. Today, they have had only one live birth from this procedure and the doctors aren't sure from which ovary the egg came. But, it's important to note that fertility issues in seriously ill women are being studied. In lieu of any pre-treatment options, my advice would be to stay in good physical condition, eat fresh fruits and vegetables and limit processed foods while trying to conceive. You may even want to try acupuncture to help with your fertility. I cannot discount its effectiveness in my own case. I can recommend some books on the subject to anyone who may be interested.

Once pregnant, there are a few things to consider. It is generally believed that in the case of women with other autoimmune diseases, 1/3 of women improve during pregnancy, 1/3 of women's condition remains the same, and 1/3 get worse. Due to lack of data, the medical community cannot say with certainty that the same is true for WG patients, but it's possible. So, if you are in remission and in good physical condition when you conceive, you may indeed have a successful pregnancy. Factors such as kidney function play a major role, however. Women with compromised kidneys have a higher chance of developing preeclampsia later in pregnancy (weeks 24-26 are especially critical). Also, women with autoimmune disease are at a higher risk of having the placenta "poop out" before term. Non-stress tests of the placenta should be done in the last eight or so weeks of pregnancy.

My long and sometimes painful journey has enlightened me, and has led me to places I never thought I'd go. If I can help or even bring hope to one person by sharing my story, then my journey has been worth it. One of the most important points I'd like to stress is that just like with Wegener's, you must be your own best advocate when it comes to your fertility. Keep asking the difficult questions, keep researching, and keep hope in your heart. It is possible to conceive after treatment. If you have any questions, please contact me. It's been an honor and a privilege to share my story with you. Thank you.

Update from Christy. May 2007

I gave birth to McGregor Dean Abele on December 8th, 2005. Delivery was very normal and Mac is a healthy, happy 17 month old. Unfortunately, just like with my older daughter, Claire, my WG flared about 14 weeks post partum. My doctor started me back on CellCept, but I successfully avoided prednisone. I battled arthritis for about 6 months last year but my symptoms have subsided. My numbers are in the normal range now and I feel great! I am currently on CellCept but hope to taper down sometime soon. Life in the Abele household is chaotic, but we have lots of fun and laugh a lot.

Vasculitis Symposium 2008

By Suzanne DePaolis aka Karen

 

I hope you are not expecting your traditional 417 people attended the

biannual Vasculitis symposium held at the Mayo Clinic in Rochester,

Minnesota on June 6th -8th. Many famous faces from the world of vasculitis

practice and research were there sharing information and giving timely

advice. Everyone enjoyed the diverse topics that ranged from Yoga to

Modern Treatment Protocols. The Saturday evening banquet was a success

with much discussion between new found friends.

 

 Ok, anyone that knows me realizes that I am incapable of writing that way. I

would rather tell you how it felt to be at the conference. How it made a

difference in my life and the way I will deal in the future with my illness;

How a simple thing like a conference has empowered me to go out there and

not just live life but to BE ALIVE!

 

Some of us got good news at the conference (oh, good it’s just a side effect

of a med), some of us got bad news (the numbness means some nerves are

gone and probably won’t come back). Overall the importance of a

conference like this is that we got information and this information came

from the Top Doctors in the United States on vasculitis. We got to meet

them, hear them talk, touch them (Oh god! I am sounding like a stalker…)

and they listened to us. Truly listened and answered our questions. They

did not have one hand on the doorknob ready to exit the room; they stayed

for every question and anything they could not answer came with promises

of sending it to a doctor that could answer.

 

We are coming home feeling empowered and less alone with our illnesses.

We have seen others with moon faces, budda bellies, odd noses and thinning

hair. We sat and ate with people who also finished everything on their plates

due to being on prednisone I wrote the first night in my journal how

everything seemed right in the world, how I didn’t feel so lonely in my

illness anymore and that is still true. I have made friendships that I expect to

last a lifetime. I now can hear their voices and see their faces as we

exchange emails. And when things get really bad or I am just having one of

those truly awful Vasculitis days…. I know now… “Who YA gonna call?”

My Vasculitis Buster friends!

Vasculitis Humor

By Dianne Shaw, 2006

You know you have vasculitis when:

• Any conversation can suddenly turn into a round of "Charades".
• A "good hair day" is when you realize you have some left.
• You tell your kid to "clean up the floor" and they just get the broom out and start sweeping.
• You make a grocery list so you won't forget anything, and then forget where you put the list. (On a REALLY bad day you also forget where the grocery store is!)
• You bathe the lawn, fertilize the dog, and brush the kids.
• You use the smoke detector to tell you when dinner is done.
• You try to type and discover that you've invented a whole new language.
• You keep sunscreen by every door.
• Getting some fresh air means sitting near an open shady window.
• You have a temperature and moisture-controlled room for keeping your large quantity of meds.
• You're the only one who believes you're THAT sick.
• You sit in the car for three hours wondering what you needed to do, not even sure where you are.
• Someone asks you what vasculitis is and you've forgotten.
• It takes so long to get one project done, because in the meantime you've been distracted by at least a million other things.
• You put the ice cream in the cupboard (and then wonder why somebody else did something THAT stupid).
• You know every doctor, nurse, within 50 miles of your home - AND you've financed most of their vacations.
• The pharmacist sees you coming down the aisle and doesn't even have to ask your name.
• You decide to buy stock in pharmaceutical companies (because you buy their products so much they ought to make lots of money) but you can't afford to invest (for the same reason).

Riding the Behcet's Rollercoaster

By Joanne Zeis, VF Education and Awareness Council

February 2007

http://www.behcetsdisease.com
jzeis@charter.net
 

When you're 23, fresh out of college and just starting your first real job, you think that you have your whole wonderful life ahead of you. You have your friends, your boyfriend, a job that you like, your first car, and a cheap apartment with a nice roommate. Life is good, and you think that nothing's going to stand in your way.

Well, things didn't quite work out like that for me. I had the job and the friends and the car and the roommate, and life was good - at least until the day that one of my eyes started to hurt. Over the course of a few hours, the pain got worse, and looking at any bright light felt like someone was shoving a hot poker in my eye. When I looked in the mirror, I saw that there was a bright red ring surrounding the iris, which is the colored part of my eye. Luckily, there was an emergency room nearby that specialized in visual problems (what are the odds?). The doctor that I saw provided steroid drops for me to use for the next couple of weeks. A follow-up appointment confirmed that the inflammation was gone and that my eye was back to normal. There was no explanation for what had happened, but I didn't care as long as I could see again without pain.

A few weeks later, though, the red ring was back, and this time the pain was worse. I returned to the emergency room, and that was the beginning of a 17-year odyssey to find out what was wrong.

I would have been OK if the only thing wrong was with one of my eyes. After all, with the indestructibility of youth, I knew that I always had a second eye as backup. But I only had one mouth to use for eating, and that mouth was starting to get painful on a regular basis, too. It's hard to eat and drink when your mouth has ulcerations in it. The sores could be anywhere - on the top or bottom of my tongue, or on the roof or sides of my mouth. There could be up to 100 sores at one time. How can you talk with your friends when every part of your mouth hurts? How do you eat? (You don't.) How can you kiss your boyfriend or be intimate when he thinks you must have picked up herpes from being with someone else? - even if your herpes test is negative.

My joints started to hurt all the time, especially my knees, ankles and hands. There were strange red rashes on my skin, and painful bumps and lumps that came and went on their own, even in the genital area. Blood test results were abnormal, and finally the vision in my other eye was affected. Now I had foggy vision on one side, and distorted vision on the other. I panicked. By then I was in my late 20s, but I felt like an old lady. I couldn't see normally, walk normally, talk normally or live normally. Any plans that I tried to make were constantly disrupted with trips to the doctor, or to the hospital, or the emergency room. How many people at that age have regular appointments with ophthalmologists, rheumatologists, dermatologists, gynecologists, retinal specialists, neurologists, and cardiologists? It was just ridiculous. But the medical problems I was experiencing were, for the most part, internal -- I felt terrible on the inside but looked fine on the outside. As a result, I was often seen as an attention-seeker, a hypochondriac. It reached the point that even my parents didn't believe me. My primary care doctor finally told me to make an appointment with a psychologist for an evaluation, because she felt that I was spending too much time on the internet and in libraries trying to find answers for what she felt were my supposed health problems.

Seeing the psychologist was a turning point. I was told that I had real health issues that weren't being addressed, and that I was acting appropriately by searching for information to help myself. Fortified with support from our meeting, I decided to continue my quest for answers. I read everything that I could get my hands on. There seemed to be three possible options for my health problems -- lupus, multiple sclerosis, or Behcet's disease (which had actually been suggested by one doctor years earlier, but immediately dismissed as too rare). I finally joined an online support group for Behcet's patients, as my symptoms fit most closely to this disease, and then I joined the American Behcet's Disease Association.

I attended my first ABDA medical conference in 1996, 17 years after my first visit to the emergency room. This conference was a life-altering event: there I was in a room in Phoenix, surrounded by 30 other people from across the U.S. whose experiences matched my own. No one thought that I was imagining my symptoms, or just looking for attention. I left the 2-day meeting energized to try to help other patients who were hitting brick walls in their own search for answers. 1996 was a life-altering year in another respect - I finally met with a Boston ophthalmologist, an expert in diagnosing and treating cases of Behcet's disease. He provided final confirmation that the painful patchwork of symptoms I'd been experiencing was most likely the result of Behcet's disease; it had been poorly treated by doctors who had never taken the time to communicate with each other about my extensive health problems. After all the years of frustration, I had a diagnosis, and I now had a medical expert who could help resurrect my failing vision and overall health.

During the next year, I underwent cataract surgery and other repairs to my iris and retina, while also interviewing Behcet's patients for a book I planned to write: "You Are Not Alone: 15 People with Behcet's." This book was followed six years later by the research-based "Essential Guide to Behcet's Disease", and an assortment of other patient-oriented support materials. I've worked closely with the ABDA to make sure that people know that these books are available to help them. The more I learn about Behcet's disease, the more I want to share this information with others, so that no one else has to go through my 17 years of hell.

I am one of the privileged few. The symptoms that I've experienced for so long have slowed down and, in some cases, stopped entirely. After 28 years with Behcet's, I'm not blind and I'm not disabled. I'm a self-employed wife and mother of two healthy teenagers, and I enjoy life on a daily basis. All people with Behcet's should be so blessed.

This work has become my ministry, and I feel privileged to be able to offer up what I've learned. It's hard to take that first step in educating others, though. Not only do you have to overcome the fear of "going public" with the intimate details of your illness, you also have to face any fears you may have of speaking in front of groups at all. I've had some practice dealing with these fears over the last couple of years, though. Producers from the Discovery Health Channel came across my behcetsdisease.com website in 2005, and based an episode of their "Mystery Diagnosis" series on my 17-year search for a diagnosis. And I'm proud to say that the American Medical Association recently presented me with their 2006 national "Citation for Distinguished Service" award, for my ongoing work in educating people about Behcet's. My most recent project was to carry out a research study on "Behcet's disease, pregnancy and postpartum." The abstract for this study was accepted for presentation at the International Behcet's Disease Conference in Portugal in 2006; the information that I gathered effectively doubled the amount of data that had been available to date in the world on pregnancy and Behcet's.

Joining the Vasculitis Foundation in their educational endeavors was an easy choice for me to make, and a natural extension of the outreach that I've already been doing for the last 10 years.

My journey with Behcet's disease has never been easy, but the emotional rewards that come from helping other patients has made the trip an overwhelming experience.

 

An Unfamiliar Path in Life

By Cindy Smoot, VF Education and Awareness Council

January, 2007

 

Our family has been dealing with Churg Strauss (CSS) for many years now. My 13 year old daughter, Lauren, has CSS. Although CSS has been a part of our life most likely since Lauren was just a baby, it took many, many years to unravel the mystery of what was really wrong with Lauren and to finally get a firm diagnosis.

Lauren's problems mostly started with bad asthma, or what seemed "asthma". Actually, if I go all the way back her problems really started at birth when she had a systemic bacterial infection with group B strep, and also developed pneumonia as a neonatal. Lauren was given a 50 percent chance of surviving the night she was born. When she finally recovered from these life threatening infections, she was very weak and left with fragile lungs prone to infection. This turned into chronic infections and horrible asthma into her toddler years. We took the nebulizer everywhere we went, because her asthma was so severe and unstable. The asthma became markedly worse when Lauren was around 3 years old, and she would have severe attacks several times daily. Looking back, I cringe to think how bad she was, when I should have had her in the hospital. We would do treatments non stop for hours every day just to keep her breathing. It just became our way of life for everything to revolve around asthma treatments.

By the time she was five she had constant sinus infections that would not clear, and then had her adenoids out and her first sinus surgery at seven. That was also the year we started seeing her current asthma/allergy/immunology specialist, who still treats her. He knew how severe her asthma was the first day he saw her, and that day is when she started daily prednisone (steroids), and has been on it ever since then. That began my love/hate relationship with steroids, I loved how they helped her, but hated what they did to her with side effects. Even on steroids her asthma did not get better as it should have. She kept getting pneumonia, by that time she had had pneumonia over 10 times. Her stomach killed her all the time, she vomited all the time, and things just seemed to be getting worse. Her GI problems finally put her in the hospital for 2 weeks in Feb of 2002 when she was 8 years old. We found she had multiple bleeding ulcers (from steroids), and that is when they did a surgery called a nissen on her to tie off her stomach, to keep reflux and vomit down. She was better for a month, and then got pneumonia again.

I was so tired of her being sick, and tired of all the specialists, so I just went to her good old pediatrician, (who had guided me so well all these years), and asked him what in the world was wrong with Lauren. He told me to start over, and go to a pulmonologist and just focus on the lungs, as all her other problems seemed to stem from that. So that is what we did, and our first visit with that pulmonologist changed our lives. He immediately told me "I don't know what she has, but it is not asthma". He sent us for many tests, including her first CT scan, then in June 2002, and that finally showed the white patches all over her lungs, called ground glass infiltrates. This appearance is usually what is seen with a fatal lung disease called pulmonary fibrosis, and he was very worried. I got "the" phone call that the tests had come back and it did not look good. She was immediately scheduled for an open lung biopsy, and while she was in ICU we heard for the first time the word eosinophilic. The pathology showed these eosinophils were infiltrating her lung tissue and jam packed in her airways; she was diagnosed with eosinophilic pneumonia. Her blood counts with these eosinophils were also very high. We thought we finally had the answers. Little did we know we were just beginning this long walk that to this day is still unclear. She began massive doses of IV steroids, 750mg a day. She did horrible with these huge doses of steroids. She went into psychosis, and developed fluid on the brain, and her doctor was concerned because her CT's of her lungs were not improving.

At this point I started researching and reading everything I could get my hands on about eosinophilic lung disease. It did not take very long in that research to come across articles about Churg Strauss. I knew when I read the first article I saw that mentioned CSS, that was what Lauren had as it matched her symptoms perfectly.

Her doctor wanted us to see a more experienced physician who had dealt with these diseases before. So we began taking trips to experts in eosinophilic diseases. We went first to a specialist at Texas Children's in Houston. This doctor disagreed with the numerous pathologists and radiologists who had studied Lauren's results, and even with physicians he asked for opinions at his hospital. That left us all very confused, so the very next month her doctor at home sent us to National Jewish in Denver. We stayed there for over 2 weeks. It was there that we learned there was more to this than just lung disease. They discovered her auto antibodies were high, and she still, after all the months of huge doses of steroids, had very high amounts of eosinophils in her lungs. They found them in her sinuses too when they did her second sinus surgery, and they also were the first to realize she had nerve problems, with neuropathy in her legs. While we were at National Jewish we were told it looked like Lauren could have Churg Strauss, but that they did not know of any cases ever reported in a child. Lauren turned 9 the week after we returned from National Jewish.

A few months after going to National Jewish, the pulmonologist was unsure of what to do as Lauren began to decline in her lung functions. So I went back to that same immunologist we originally saw before her lung disease was found, then in the spring of 2003. During that time she began having more GI issues, and was found to have gallbladder problems, so it was removed and also a nerve biopsy was done to confirm if her strange feelings and pain in her legs was neuropathy. The biopsy showed she had active and chronic nerve degeneration of neuropathy. She then had three organs involved; lungs, sinus, and nerves, and it was suspected that her GI problems were linked to eosinophils too.

Her immunologist then consulted with her rheumatologist, and they both thought she had Churg Strauss (CSS), but wanted to confirm it with the doctors in Boston who had actually seen another case in a child. So yet another trip, that summer of 2003. In Boston we met a CSS expert, who turned out to be a great help over the years. We also met with several other pediatric doctors, but they never did quite get it because Lauren looked normal. I have learned when you are really sick, and looking good, it is not always a good thing. They literally said they could not believe that she was the child that the records were about. They showed us her CT's, and talked about her pathology and said it seemed to show severe disease, but they could not think a child could look that good and still be as sick as her records showed. So we came home with no real answers or help from Boston.

During this same summer Lauren stopped her steroids for the first time in 2 years for a couple of months and her blood counts shot up very high, she got serious infections in her lungs, and her lung disease worsened. We tried her first chemo drug. She also began having terrible stomach pain, nausea, and vomiting again. Her doctors worried she had bleeding ulcers again, but when they would scope her and take biopsies not much would show up but some nonspecific chronic inflammation. After a short while she went back on steroids, and has not stopped them since. Her lung functions slowly improved, but her GI problems persisted.

It was that next spring of 04, that her immunologist went to a conference and heard a doctor from Cincinnati Children's Hospital speak about research they were doing into eosinophilic diseases, especially eosinophilic GI disease, and he thought we should go see the group of doctors involved in this research in Cincinnati. That year Lauren's GI problems became horrible and unexpectedly her lung disease began to get somewhat better. So that summer Lauren and I went to Cincinnati, and we finally found a place that really understood these eosinophilic diseases. They told us her pathology looked like it was from an autopsy, and Lauren had an amazing ability to go on and function despite overwhelming disease. They found all the years of stomach problems, that had been looked into five times with biopsies were eosinophilic inflammation in her GI, it was all the way through her GI tract. She now had four organs involved. In Cincinnati, they also thought Lauren most likely had Churg Strauss, but again they said it was almost unheard of in children, so they could not be 100% certain without a specific finding of vasculitis on biopsy, which is the final stage of CSS. We continued going to Cincinnati Children's every three months for two years, to help guide her doctors at home treat Lauren's rare condition.

The GI disease has been very hard to control, and in January 2005 Lauren had a severe flare again in her stomach, despite treatment with steroids and Methotrexate, another immunosuppressant drug. At that time Lauren did daily IV steroids in a high dose for several months. She can no longer tolerate high dose steroids by mouth; they are too hard on her stomach. Unfortunately, the constant use of her central line led to Lauren getting sepsis, which is a very serious, systemic blood infection. This infection was a huge setback for Lauren that took months for her to recover from. When she finally completely recovered from the bout with sepsis, she began feeling much better after all the months on high dose steroids, but was having terrible side effects. She had developed cataracts, osteoporosis, Cushing's syndrome, and looked nothing like herself anymore. Her doctor began to try and lower her steroid dose. After a few months she began having symptoms again in her stomach. In December of 2005, she spent 3 weeks in the hospital unable to eat or drink without vomiting. At the same time she began to leak urine, and lost the reflexes in her legs. Her neurologist thought she was having progressive nerve disease from CSS, and had developed autonomic neuropathy, which affects the way your organs function, and can cause a paralyzed state in your GI tract called gastroparesis. The next month at the end of January 2006, Lauren could not be woken up for 24 hours, she was incoherent, and lost control of her bodily functions without being aware of it. She would fall asleep mid sentence while talking. Again her neurologist felt she was having serious progression of CSS vasculitis into her central nervous system. Several tests were run, a MRI of her brain and spinal cord, but they were normal. With CSS affecting small vessels it is usually not found on MRIs.

This past summer of 2006, the doctors we had been seeing for 2 years in Cincinnati felt too much was going on with Lauren, and she needed more immediate care close to home. Again her team of doctors at home felt she needed an expert to guide them, as none of them had ever had a CSS patient. So it was decided to then send us to an adult expert that had actually seen and treated CSS. All the doctors we had seen before were pediatric specialists, and although they were experts in eosinophilic disease, none of them had a case of CSS in a child. So we finally went to the Cleveland Clinic, and saw Dr. Hoffman, one of the leading vasculitis experts in the world, and he confidently diagnosed Lauren with CSS. We will continue seeing a team of doctors in Cleveland every 3 months until Lauren is well into remission.

Lauren continues to have a very difficult case of CSS, particularly with her GI tract involvement. She continues to flare frequently, and it is very hard to keep her disease controlled. She has one of the more severe cases her doctors tell us, and we have been told by most doctors she will most likely need life long treatment. She has a good outlook on life though, and is anxious to move on past CSS. We are hopeful that in much of the new drugs that are being researched for CSS, a cure for Lauren will be found.

In the process of dealing with this disease over the years, I found help from a wonderful group of people around the world, who also suffered with CSS, on the CSSISG support group. I continued my research of CSS and the mechanisms behind it, as well as the drugs that can potentially treat it.

When the CSS Association was formed I was asked to help by serving on the Board of Directors. With a great deal of help from Jane Dion, who started the CSS Association, and several others, a website and organization was put into operation to help serve other patients with CSS, and to bring awareness of this disease.

A year ago, the Vasculitis Foundation, formerly the Wegener's Granulomatosis Foundation, made the decision to add all the other vasculitic diseases to their umbrella organization. Fortunately, for those with CSS, we are now also represented by the Vasculitis Foundation (VF). Recently I was asked to serve on the Education and Awareness Council for the VF, as a representative for Churg Strauss. I would love nothing more than for CSS to be a common name known by all doctors and the general population. The long, painful journey we have been on with Lauren could have been very different if doctors all those years ago had known about CSS, and if we had known about CSS. Like any disease, the earlier it is caught, the better off you are.

We are hopeful for Lauren, and feel great breakthroughs are around the corner for CSS.

CSS - My Story

By Ken Abbott, February, 2007

 

I'm better now, but I spent 12 months battling Churg Strauss Syndrome. I'm telling my story below in the hope that it will be helpful to others going through the same thing. Of course, these are just my personal recollections, they are not intended in any way as medical advice. So, with that caveat, here's the scoop on Churg Strauss from my perspective..

How it began

I had some dental implant work done and the procedure was a bit messy because the implant head fractured and my implant dentist had a tough time. Anyway, a few hours after I got home I developed a sudden rash around my ankles. My dentist thought it was an allergic reaction to the antibiotic. I took Benedryl for a day or so and it went away. I thought no more about it. About a week later I spent a day painting a room in our house, and that evening as I showered I noticed the rash had reappeared. It had developed during the day. It was a Sunday, so I stopped by my medical practice and saw the doctor on duty. He immediately mentioned vasculitis. It was the first time I had ever heard the word, and he explained that it was an autoimmune reaction that attacks blood vessels. On the Monday I saw a dermatologist, who immediately pronounced the rash "probably produced by something internal, it's out of my area". He started me on prednisone and sent me to see a rheumatologist.

My Rheumatologist

I belong to a large medical practice, so all the specialists are in-house and it was easy to see a Rheumatologist. He was new to the practice. (By now, of course, I had been Googling vasculitis like crazy. I discovered there were many different forms, but the one that most closely matched my situation was a form called Churg Strauss.) I explained my full story to my Rheumatologist. I explained the rash, how it had occured. I also mentioned that a few years earlier I had surgery for nasal polyps. There was an history of asthma in my family, and although I had never had full asthma I had been suffering from light bronchial asthma for several years . He ran blood tests, which showed I had a very high eosinophil level. By now I was also having occasional blurry vision episodes, and also numbness in my palms and ankles. He told me that taken together with my medical history, my symptoms could be a form of vasculitis called Churg Strauss (thanks Google!) He said it was very rare, that he had never actually seen a case so was out of his depth, but he referred me to a Churg Strauss specialist in New York City. He said I must continue to take prednisone, a standard first line treatment for autoimmune problems. I did not realize it at the time, but I would be on prednisone for 12 months. I made an appointment to see the New York City specialist.

The official diagnosis

It was a few weeks before I got to see the New York City specialist, and by then my symptoms were much worse. I had difficulty breathing and walking, with numbness and discoloration of the feet. Also, I had swelling of the face. The specialist reviewed by medical history and blood tests, gave me a physical exam, and then officially confirmed I had Churg Strauss. He said he had seen about 40 cases in his entire career. He also said I had an aggressive case of the disease and so wanted to take strong action by putting me on Cytoxan. This is a drug first used for cancer patients, but later found to be very effective for autoimmune conditions. He explained there were "milder" drugs that could be used, but he felt we should treat the condition agressively from the beginning.

Cytoxan

I took Cytoxan for about 2 months and continued to take the prednisone which was now up to 60mg per day. After I phased off the Cytoxan my blood tests showed a significantly reduced eosinophil level. So progress was good. My feet and lower legs were still numb, which made if difficult to walk, and as a side effect of Cytoxan I lost a lot of my hair. A few weeks after stopping the Cytoxan I began a very slow reduction of the prednisone - a process called "tapering" and during this time my specialist put me on Imuran. I had a few attacks of breathing problems, and even an apparent return of the ankle rash, but my specialist explained that this was probably a reaction to the Imuran and so I stopped taking it. He was right. However, my numb feet caused a totally unnecessary complication.

Unnecessary and annoying

One day, walking down some steps in my house with my numb feet, I tripped and tore my Achilles tendon. Long use of prednisone has several side effects such as an increased appetite and mood swings and even depression, but one that is not mentioned much is tendon weakness, and especially the Achilles tendon. This makes it prone to injury. I went through surgery to fix the tendon, followed by about 4 months of recovery and physical therapy. During this time I continued to slowly taper the prednisone. But the tendon injury was unecessary, annoying and an upsetting complication.

Nutrition

While I was at home in a cast recovering from the tendon rupture I had a telephone consultation with a nutritionist. I explained that I was specifically interested in any diet that would help reduce inflammation. Churg Strauss is an inflammatory autoimmune condition, so I was interested in anything that could help reduce inflammation. She said that such a diet exists and gave me the basic rules. Here's what she said (and what I did). Don't eat anything that's made with white flour - no bread, no cakes, no pastries, no pasta. But whole wheat flour is ok, so whole wheat bread is ok. And whole wheat pasta is ok. No potatoes. Cut way back on red meat, try to eliminate it if possible and eat fish and chicken instead. Eat lots of fruit, especially fruit rich in anti-oxidants such as berries. No cheese. Eat cereals rich in flaxseed. I also discovered a special very dark chocolate that's 60% or more cocoa content. It's advertised as rich in anti-oxidants. I checked this one with my Churg Strauss specialist in New York City and he told me, "go for it." I did.

Recovery

At about the same time I recovered from the tendon rupture I finally got myself off the prednisone. In the end I was tapering very slowly with a reduction rate of just 0.5mg per week. The whole process had taken over 12 months. Of course, I had frequent visits to my rheumatologist during the whole time. He was the primary doctor who managed my case, and he carefully monitored my condition with blood tests and chest X-rays to check lung conditions. As required, he also sent me to other specialists - a cardiologist to check heart condition, a dermatologist for a skin biopsy of the rash, a neurologist to check for nerve damage, and a nephrologist to check kidney function.

Hair

Not only did my hair grow back, but it grew back thicker, richer and slightly darker than before. My friends joked about asking their doctors for a Cytoxan prescription!

Maintenance

This consists of blood tests and urine tests. The main blood tests are sed rate, eosinophil level and ANCA. My rheumatologist also prescribed Bactrim - an antibiotic taken twice a week to help avoid nasal infections. I also see my ENT frequently to check my nasal passages and sinuses for any recurrence of polyps. I use Nasonex to help keep nasal passages clear, and I irrigate twice a day with a ph balanced saline solution. The basic message here is that nasal polyps are not a good sign! During my entire treatment I took Ambien every night to help me sleep and I still take it occasionally as needed. I still can't do real exercise, but I can do my usual lunchtime walks exactly as before this whole thing began.

Flare-Ups

It's not uncommon for some or all of the symptoms to re-appear, but usually in milder form and for short periods. These are called "flare-ups". So far I've had two. Both have been "mild" and nowhere near as bad as the original condition. Neither flare-up included the rash or nasal polyps, but there was some upper respiratory problems. The first time my doctor simply put me on prednisone (Medrol) for a couple of weeks. The second time, with my sed rate ok but with my eosinophil and ANCA levels showing signs of creeping up, my doctor put me on prednisone and Methotrexate.

I'm still not back to normal, but I feel I'm well on the way. And I'm very lucky to have had wonderful doctors.

 

CSS Vasculitis- A Life Changing Experience

By Jane Dion, Co-Director Churg Strauss Syndrome Association

Spring 2006
http://www.cssassociation.org/

  
My name is Jane Dion and I was diagnosed with Churg Strauss vasculitis in May 2001. I live in a small town of 800 people in the foothills of western Massachusetts. I've been married 36(!) years and have a 25 year old son, who lives in Barcelona and a 23 year old daughter. My passions are sea kayaking, travel to faraway places, photography, and going to the movies.

Like many people with vasculitis, I was sick for quite a while before I was diagnosed. Diagnosis was difficult because vasculitis can present in so many ways. I saw many different specialists, but none of the doctors put it all together. I saw a pulmonologist for late onset asthma, an allergist and a dermatologist for skin rashes, and an ENT for sinus problems. I lost a lot of weight, which made me very happy, but I often felt weak and lacking in energy. I had a constant dry cough - my husband said he could never lose me in a store because he could just follow the sound of my cough to find me. I had Raynaud’s syndrome and I sometimes experienced pins and needles in my hands and feet.

My primary care doctor was wonderful in referring me to appropriate specialists, but I did not improve. I lived a busy, full life and I didn't have the time to be sick, so I carried on the best I could. I ended up in the emergency room late one night after feeling that I just couldn't breathe - I felt as though there was a five-ton elephant sitting on my chest. Imaging studies showed infiltrates in both lungs. At first I was put in an isolation room at the hospital while TB was ruled out. After two weeks in the hospital and many, many tests, I was finally diagnosed with CSS. I met five of the six American College of Rheumatology’s criteria needed to be classified as having CSS (only four are needed) which are: 1) asthma; 2) eosinophilia (eosinophils are a kind of white blood cell) greater than 10% on differential WBC count; 3) mononeuropathy; 4) transient pulmonary infiltrates on chest X-rays; 5) paranasal sinus abnormalities; and 6) biopsy containing a blood vessel with extravascular eosinophil. I started on high IV doses of prednisone. Improvement was dramatic. I soon felt better than I had in years! I am very fortunate my disease is responsive to medications and that they also control my infrequent flares. I am currently on 200 mgs of Imuran and 10 mgs of prednisone. I would like to further reduce the prednisone, but it seems I flare whenever I go below 10 mgs

When I was first diagnosed, my husband and friends scoured the Internet for information on CSS. Because the disease is so rare, getting easy-to-understand information was difficult and finding an active patient advocacy group was impossible. The disease is so rare - with fewer than 900 to 3,000 patients in the USA - and there were just not the resources available for newly diagnosed patients. I was, however, fortunate to eventually find a Yahoo message board which proved to be a great support (cssisg@yahoo.com).

I was very lucky that Dr. Peter Merkel from Boston University Medical Center, one of the nation's top vasculitis doctors, became my specialist. When I first met Dr. Merkel he was involved in establishing the Vasculitis Clinical Research Consortium (VCRC). He urged me to find someone willing to act in an advocacy position for CSS patients within the VCRC. After there were no takers among the people on the message board, I, along with Dr. Carol Kavanaugh, a CSS patient from California and Cindy Smoot, mother of a young child from Texas, formed the Churg Strauss Syndrome Association in 2004. Its original purpose was to gain representation on the VCRC, which had recently received more than $6 million in funding for vasculitis research. At a meeting of the Rare Disease Clinical Research Network in Rockville, MD we were further encouraged and supported in our efforts to set up a CSS resource to maintain a presence within the rare disease patient advocacy community.

Because Churg Strauss Syndrome is so rare and because it manifests itself in many ways, diagnosis and treatment can be problematic and confusing. Many of us search the web for pertinent information on the disease and its treatment. This task can feel overwhelming to the newly diagnosed. In addition, there has not been much research on CSS and therefore it remains somewhat of a mystery. Because it affects everyone differently, treatment is not standard. The drugs used to treat the disease themselves need to be understood as they can have adverse effects. Some of the articles are written in “doctor speak” which is hard for the lay person to interpret. We hoped to make the CSSA website a place where patients and their families can easily find the information and support they need to better understand CSS. We also hoped that by establishing a strong presence we might stimulate and support research into CSS.

Helping to create the CSSA is one of the most difficult things I've ever done and one of the things I'm most proud of. I had no relevant experience at all before I undertook this endeavor. In two years we have created a very informative and much accessed website with readers from over 70 countries around the world. We often learn that patients are referred to our site by their diagnosing physicians who themselves have referenced it to learn more about the disease. We have created an international presence with board members from France and the Netherlands. Our Medical Advisory Board consists of the worlds experts in our disease. Many people have contributed patient stories to the news page of the website. Ben Watt, DJ, songwriter, singer and author of the compelling book "Patient - the True Story of a Rare Illness" took the time to write a letter of encouragement to CSSers and people with vasculitis (http://www.cssassociation.org/oldnews/watt.asp). Irina Slutskaya, an Olympic figure skater who has CSS, graciously met with me and offered words of hope to all vasculitis patients. (http://www.cssassociation.org/oldnews/Slutskaya.asp). On the news section we also publicize and promote research and share new information relevant to CSS.

Although the CSSA has accomplished so much in a short period of time, we recognize the need to band together with other vasculitis groups to better raise awareness of vasculitis. There is strength in numbers and together we can create a higher profile and stronger voice within the medical community and among the general population. Because CSS and other forms of vasculitis are so rare, many people are diagnosed late, or not at all, causing permanent damage or even death. We believe that by banding together, we will be more successful in raising awareness of all the vasculitides, resulting in earlier diagnosis and more effective treatment for each of the diseases. Someday, we hope that "vasculitis" will be a household name much as lupus, MS or Parkinsons is now. The CSSA is happy to have a close association with the VF as the WGA/VF was very helpful to our fledgling organization and provided advice, support and shared resources. We have a history of working well together and share many of the same goals.

 

My Thirty Year Story - So Far!

By Nita A., January, 2007

I am now 51, have had three distinct diagnoses of CSS and since hindsight is 20/20, I now believe that my Churg Strauss actually started in my 20s. I have been reading the other stories on this site, and I am amazed at the similarities with my own experience. I will try to keep my story succinct, and I hope that it is helpful to others.

As a college student I suffered from severe sinus congestion, lost my sense of smell and eventually had surgery for nasal polyps. Shortly thereafter I developed asthma, and needed to be hospitalized multiple times to get it under control. Throughout my 20s I continued to have severe asthma, nasal polyps and recurrent ear infections. Looking back, I also remember that I had itchy red bumps on the palms of my hands (which were later diagnosed as eosiniphiliac).

In 1986, when I was 31, I developed bizarre symptoms about a week after I gave birth to my first child: a purple rash on my chest, night sweats, low grade fevers, joint pain, itchy red bumps on the palms of my hands (which were biopsied and showed eosiniphils), and loss of appetite. I was unable to nurse my baby. I thought I was going crazy! After a couple weeks of many physicians and tests, I was given the news: Churg Strauss vasculitis. I was started on prednisone and felt better almost immediately. I was on the steroids for about 2 1/2 years, and was relatively well (except for asthma, polyps and loss of smell) until the next episode 10 years later.

The second episode occurred when I was on vacation with my husband and two children at Disney World! I experienced a tingling in my hands and a weird sensation in my butt such that I could not sit comfortably. There was nothing at all similar about this to make one think of CSS. We returned home, and I woke up the next morning with a foot drop!! Unfortunately I had switched physicians because my health insurance had changed, so I was being evaluated by docs who did not remember what I had experienced previously. This was a huge error in retrospect. I almost ended up with back surgery for a disc problem, before we realized there was something else going on. While a neurologist (who had not read my medical record) was performing an EMG she exclamed, almost to herself, "this almost looks like Churg Strauss"! My mother was in the room with me and we looked at each other in disbelief!! Who would think that such seemingly unrelated symptoms could be the same disease!!

So, since doctors are so concerned about having definitive evidence before they treat, I was asked to endure a biopsy of my leg to confirm the presence of eosiniphils. Although I had multiple incisions, and my seral nerve was cut (leaving me with loss of sensation in part of my foot), they happened to not find a spot that would give them the info they wanted. I was told that my sciatic nerve was involved and that it was as though my sciatic nerve had had a stroke because the inflammation of the blood vessels had cut off the blood supply to the nerve. I had to be fitted with a foot brace, and had to use a cane. I developed severe nerve pain that kept me lying in bed in agony, and was finally started on Elavil which was the only thing that helped the pain. Until I was diagnosed I thought I was dying! The diagnosis was confirmed on the basis of lab results and past history. My symptoms were quickly controlled with prednisone and, later, Imuran. Most of my neurological damage healed over the next year. After six weeks out of work on medical leave, I returned to a job that I love as a social worker in a facility for the aged, though I was barely recognizable to some because of the Cushing's Syndrome and the cane! I joined Weight Watchers a year later, started to exercise, and lost all of the weight that I had put on. Life was good.

The next 10 years were more of the same: asthma and nasal polyps, and now I started to have recurrent ear infections again. The past couple of years have been characterized by an increase in all of these problems. I had tubes put in both ears last March (2006). The ear infections continued despite the tubes. Every time I had an ear infection the asthma also was triggered, so I would end up on a short course of prednisone. This has been going on for quite some time. I also experienced a tingling in my fingers. In retrospect, I think the CSS was a factor, but my lab results have not been indicating that there was an inflammatory process at work. Perhaps they were suppressed from the frequent steroid use.

Soooo, this week as I was recovering from yet another ear infection, I developed symptoms of a UTI and pain in my left mid back. At the same time my sinus congestion became unbearable. My PCP thought that I had developed a fungal UTI from the frequent antibiotic use. Ultrasound of the kidneys showed inflammaton in the ureters. I do not know if this was unrelated or if it is symptomatic of the CSS. I also saw my ENT who (sorry in advance for the graphic description) said that my sinus was red and inflamed, the stuff coming out of my ears was the same stuff in my sinus, and he extracted a thick, white globule that he identified as consisting of eosiniphil cells that had come from the sinus. He was sure that this was Churg Strauss!! I have been so sick for the past week with urinary problems and kidney pain, sinus problems, fever, ear congestion and loss of hearing, but it never occurred to me that this was the CSS again! My lab results now showed an increase in eosiniphils to 17%. I started taking prednisone and immediately began to feel so much better. I have been out of work for the past week. My rheumotologist is probably going to wean me from the steroids onto Methotrexate. She and I had speculated last summer about the Churg Strauss being a factor in my recurrent ear problems, but did not have the lab results to back it up. I am now convinced.

I am looking forward to getting back to my life. I now understand that CSS is a chronic illness with flares, rather than distinct episodes. I can avoid the frequent and recurrent low grade chronic illness that has been making me sick for the past two years.

I hope that others find my story helpful. My thanks to everyone else out there who has shared theirs!

Surviving CNS Vasculitis

By Jim and Becci Bornac and Cindy Webber

October 2007

His head throbbed with excruciating pain as if someone was continually bashing it with a brick-every hour of the day and night. Jim's pain was beyond belief. Although he had experienced headaches for many years, these headaches never let up. He was no longer able to sleep or to function well at his job of 13 years as a movie theater manager. Then began the long journey looking for a cause for the extreme headaches and bizarre symptoms he endured. Thus, he searched for a doctor that could help him.

The first doctor suggested something for depression or nerve pills. The pain continued. The second doctor thought the root of Jim's pain was allergies.This treatment again failed and soon Jim was back in his office with extreme disorientation, speech defects and an unnatural balance. This allergy doctor called for an MRI which proclaimed a slight abnormality in the brain. They titled it "unremarkable". Stroke-like symptoms continued along with the severe headaches. Jim found a neurologist in his own home town. Again, the doctor could find no problems. Then one day, Jim came home disoriented and could find no communicable speech at all. He was glassy-eyed and was slobbering down his tie.

Jim's wife Becci kept an accurate report of events to try and help the doctors figure out what was wrong with Jim. Becci continues to be Jim's advocate to this day. She called the neurologist and explained to him the symptoms and he said, "I want to see you both in my office tomorrow. Jim told me he was fine."

After running every test imaginable, including: spinal taps, angiograms and blood tests, Jim's ailment was still unnamed. Eventually the neurologist convinced a neurological surgeon to do a biopsy. The surgeon was unconvinced to do a brain biopsy as it was very risky in the area that he would have to invade. He performed a muscle biopsy instead. It proved nothing in the way of a diagnosis. A decision was made by both doctors that a brain biopsy was in order for treatment at best! The day before Thanksgiving in 1993, Jim underwent the brain biopsy, a risky and invasive process.

The neuro-surgeon met with Becci in the waiting room and described his amazement to the particulars of the surgery. A diagnosis was finally found-- a rare form of Cerebral Vasculitis. It was called Granulomatous Angiitis of the Central Nervous System. (GACNS). Since it is so very rare, we refer to it more commonly as CNSV. Jim was immediately treated with prednisone and Imuran. And a barrage of other medications, including Dilantin and meds to thwart the side effects of the medications.

Things were looking better until the doctors tried to reduce the prednisone too drastically. Jim suffered the worst headaches in his life! While the doctors could not agree whose responsibility it was to handle the drastic call, Becci called 911 to get the help Jim needed.He suffered three brain hemorrhages and was hospitalized for many days. His neurologist called him a 'miracle'.

Jim has been off of prednisone for a year now and is now at half dose of Imuran. His next task of medicine reduction will be Methotrexate. He will never be off all medications with this disease but at least he may be able to eliminate the more dangerous ones.

Jim is a "miracle" in that he never gives up and has never lost his sense of humor. He never fails to help others with vasculitis and continues to research the disease.

For those newly diagnosed, he suggests that people keep positive and take life one day at a time- do not think too far ahead. This advice has served Jim well. He is now focusing his energies into supporting others with vasculitis find accurate information and support.

Profile of Andrew Green, Member of the VF Education and Awareness Council

By Kathy Savickas, February 2007

Andrew Green is a member of the Education Awareness Council and represents Central Nervous System vasculitis, which he was diagnosed with in 2001 at the age of 58. During his working years, Andrew was president and co-owner of a small plastic molding company in Cleveland, Ohio. He is now retired and lives in Shaker Heights, a suburb near Cleveland.

Andrew had been an avid runner and cross-country skier with 5 and 10k races under his belt and always spent a week every year cross-country skiing with friends. During the 1999 trip he lost hearing in his left ear and was diagnosed with an autoimmune hearing loss.

During the summer of 1999, his runs became shorter and shorter and he started to have trouble completing even short training runs. He tried a long bike ride with a friend and the following day he was sore from head to toe. What troubled him most was that his workouts were not out of the ordinary but his reactions to them were.

As summer progressed, things became worse and he was eventually unable to walk around the block. Then walking around the house became difficult and he started to fall asleep quite often during the day. One afternoon in mid December, while conversing with his wife, he had trouble constructing an answer to a question and kept putting his head down on the kitchen counter to sleep. She became extremely concerned and took him to the emergency room.

There were a few visits to the emergency room at the Cleveland Clinic and fortunately, Dr. Sudhakar Sridharan from the Rheumatology Department was working the night Andrew arrived at the Cleveland Clinic Emergency Room. He was admitted to the hospital and had several tests: 8 lumbar punctures (spinal taps), a bone marrow biopsy, brain biopsy, a biopsy on a nerve in his foot, echocardiograms of blood vessels, several MRI's of his head and other tests.

At first, the doctors thought he had Wegener's granulomatosis. However, the more tests they ran, the less he fit into the WG mold. Andrew asked that Dr. Sridharan "head up" his team of doctors that would help make the decisions on what tests and procedures would be performed. He feels lucky to have had Dr. Sridharan as his doctor and is indebted to him for all the help and guidance he provided. Andrew was in and out of the Cleveland Clinic from December to May and even went to the Mayo Clinic for a second opinion. In the end, it was Dr. Sridharan and Dr. Leonard H. Calabrese, also at the Cleveland Clinic, who made the diagnosis of Central Nervous System vasculitis. Finally, the doctors administered Gamaglobin IV and within a week Andrew finally began to feel better. He continues to receive Gamaglobin IV every six weeks.

Andrew is especially grateful to his wife, Judy for her love and support that helped him through this entire ordeal. Even when things were at their lowest, she remained by his side to help made some difficult decisions about his medical care while working with the doctors at the Cleveland Clinic.

Prior to his diagnosis of CNS vasculitis, Andrew was diagnosed with Hashimoto's disease, also an autoimmune condition where the thyroid becomes inflamed and often leads to an underactive thyroid. He uses a drug called Synthroid to try and control the Hashimoto's disease and has just recently started to inject the medication intramuscularly three times a week.

Andy does not run or cross-country ski any more. He says his battery just doesn't stay charged long enough these days. Now he uses Tai Chi, which is not only great exercise but also helps him work on his balance. He feels it is important to keep things flowing.

One of Andy's passions these days is his woodcarving. He has made beautiful bowls and artwork from what he describes as distressed wood - wood that has an interesting grain, is rotting or has insect holes. Andy and his wife also enjoy traveling. They visited Japan last spring and recently got back from San Miguel de Allende in Mexico. This June they will be visiting Barcelona.

Andy's advice to anyone with vasculitis is to first get one doctor to be the "quarterback". This doctor should coordinate all tests, medications and decisions with regards to your case. Andy asked Dr. Sridharan to do this for him. Secondly, make sure you always understand what tests are being run and what they hope to find out from the results. Become familiar with the drugs you are taking and their side effects just in case you have a reaction.

Judy, Andy's wife, suggests that you become as educated as possible and do not be afraid to ask questions or for clarification. Take responsibility for example, by learning the vocabulary and do not ever assume anything. And finally, don't be afraid to ask for help.

Although Andy has multiple autoimmune disorders, none of his doctors have suggested that they are in some way related. He also states that to his knowledge no autoimmunity runs in his family.

During his time with the Education Awareness Council, Andrew would like to work on helping patients get quicker, more accurate diagnosis when faced with a vasculitic disease. It saddens him to think of how many patients have lost their lives because of an improper diagnosis.

Giant Cell Arteritis – An Elusive Odyssey

By James W. Rupp, husband and caregiver

 

My wife, Pearl, became ill with Giant Cell Arteritis (GCA) and Polymyalgia Rheumatica (PMR) in 1998 at age 65. It was diagnosed after she lost vision permanently in one eye. We learned from that event that GCA is a serious illness and demanded our complete attention and vigilance. Her illness persisted for nine years until death due to unknown cause in 2007.

 

During her treatment many questions arose which could only be satisfied by increasing our knowledge of her illnesses and symptoms. Medical journal articles were identified via PubMed on the internet by entering the topic of interest in the search block. Obtaining the full text of the articles was more difficult.

 

We were fortunate to have the interest of a physician on the board of two of the hospitals in our area that have medical libraries. While many journal articles are available for copying in the library those that they don’t have journals for had to be ordered through the library. Those articles were ordered through the physician on their board. A few articles have full text available on the internet. The abstracts and articles were very helpful in understanding the illnesses and for discussing treatment options with Pearl’s doctors.

 

Some were obtained for special medical concerns of Pearl that weren’t related to GCA or PMR. Some reflect avenues of study that were suspected of being contributory factors or secondary issues of GCA and PMR.

 

A significant effort was made to understand peripheral arterial disease (PAD). Pearl’s leg arteries became ischemic or stenotic (blocked or narrowed) within six months of her diagnosis and start of treatment with prednisone. Throughout her illness there was disagreement among her specialists (rheumatologists, vascular surgeons, orthopedists, cardiologist, ophthalmologist and internists) about the cause of the arterial circulation problems in her legs. 

 

GCA can cause peripheral (arms or legs) arteritic ischemia and stenosis while atherosclerotic plaque can be another common cause. The common opinion throughout her illness by the rheumatologists and some of the others was that Pearl’s blockages were due to atherosclerosis. Yet, the preponderance of evidence pointed to arteritic involvement. Her leg circulation was managed throughout those years with prednisone, indicating arteritis. Too little a dose and her circulation became impaired – increasing the dose improved circulation to her extremities. Her legs and feet were saved repeatedly during her illness by increasing prednisone when her doctors wanted her to taper off. The opinion expressed was that GCA is a self-limiting disease of one to three years duration. Another thought expressed was that GCA doesn’t affect leg arteries. Both opinions are proven false by many medical journal articles yet those attitudes persisted.

 

Other damage she experienced were impaired shoulders, necrosis of a shoulder blade likely due to GCA impaired circulation, and body pain and joint swelling typical of PMR.

 

Treatment options were studied for management of the illnesses including alternative medications and steroid-sparing ones. Article 130. was found to be of significant benefit in understanding GCA and management of the illness. Dr. S.S.Hayreh, the author, has published a significant amount of the content of his paper on a website of the University of Iowa Ophthalmology Department.

 

I have compiled Pearl’s experiences in a story titled “Giant Cell Arteritis – An Elusive Odyssey”. Click here to read or see below to download the article in PDF.

 

To view a detailed list of medical journal research articles compiled by the author, click here or see below to download document in PDF.  

Hoping and Coping

By Mryna Rootham, December 2006

I am 54 years old, a mother of two sons and one honorary daughter and a grandmother. I teach little kids to speak French, publish literary fiction and poetry, direct a choir, sing and travel a lot. I have had to give up figure skating, cross-country and downhill skiing, portage canoeing and some dancing because of this disease. BUT! Oh the life I do live is just grand!

I was first diagnosed in 1992, but back then MPA was still called PAN. My fist signs and symptoms appeared in 1991. I had fatigue (winded feeling), swelling in my ankles marked with red lesions, toes so hot, swollen and sore that I couldn't put a bed sheet over them, a frank arthritis and a strange feeling that I would sink through the floor. My family physician prescribed various NSAIDs to control what she thought was arthritis.

In January of 1992 the swelling became so marked that I could not wear my boots. I had a rash - lupine like lesions on my face. I couldn't even manage the Phys Ed component of the job. I went back to my family physician who told me that people in their forties often got arthritis like that, and that this was a natural part of aging. I managed to convince her that perhaps blood or urine tests might reveal something that would lead to a better treatment. Reluctantly, she ordered blood work and a urinalysis.

A few weeks following the tests, she called to tell me I needed to see a rheumatologist because I had a SED rate of 52. I made an appointment with the rheumatologist she suggested - for six months later.

The NSAIDs seemed to help and the swelling started to subside. In the meantime, I lucked into a cancelled
appointment at the rheumatologist's. She ruled out lupus and rheumatoid arthritis but wasn't yet sure of a diagnosis. She repeated blood and urine tests and as the swelling had abated, I could palpate thousands of little pea-sized nodules and so at my follow-up appointment, I asked the rheumatologist if that always happened after swelling subsided. At that point the rheumatolgist ordered a biopsy on one of the nodules. I had happily been teaching all this time, and in June- six months after my first doctor visit, I received a call from the dermatologist that the biopsy indicated PAN - a life-threatening form of vasculitis and that I needed to go to the hospital immediately for an angiogram, 24 hour urinalysis and a nerve conductivity test.

At that point the disease seemed to be abating of its own and my SED rate had dropped to 32. I was started on a course of prednisone (40) mgs and returned to work the next day.

I thought the meds had cured the disease as I recovered rather quickly and all went well for about two years until I flared again. The flare presented itself the same way. This time only 20 mgs of pred were needed. As I had concentration problems, the doctor ordered an MRI to
rule out cerebral involvement. I waited 18 months for the MRI. By then I was fine again. In the meantime an appointment with a nephrologist lead me to understand that my form of the disease was actually MPA. She could see
crescentic casts of microscopic blood in my urine. I was still
clearing enough protein, so no treatment change was necessary.

In 1999 I needed a total hip replacement - osteoarthritis? I guess too many doses of prednisone took the cartilage from my hip. I also lost the retinas in my eyes (could that have been the MPA?).

The next flare I had was the most severe. It came very suddenly and within 24 hrs I didn't have the strength to walk to the front door. I was prescribed 40 mgs of prednisone , but within a month things became worse. I had unpredictable and sudden shooting pains that would dissipate as quickly as they came, incredible fatigue, shingles, and mouth sores. I still looked pretty good and all around me family and friends and
colleagues whispered about how I was "faking" the illness or "having a nervous breakdown". I felt under siege both physically and emotionally and family and friends seemed only to add to my ills.

The rheumatologist ordered rest and 15 mgs of methotrexate along with the 40 mgs of prednisone. I lost an entire year of work and could only return on a half-time basis the following year. For four years now I have been on a combination of prednisone(10 mgs) and methotrexate (25mgs) which I inject to cut down on the nausea. Things are going well, except for some fatigue and some thinning hair.

I feel lucky. I have fatigue and some "blues" while on prednisone, but other than that, my life is good. All seems to hinge on self-discipline - daily water exercise, some weight work, no caffeine, no salt, adequate rest, no smoking or drinking and a really positive outlook. I find the humor and joy in every day and avoid negative people and situations. I live in a constant whirl of pleasurable activity with people I have discovered to be my true friends. Through these MPA years, I have traveled extensively, taught school, written and published, sung opera and been a spoiling grandmother. I believe the key to survival is to embrace life and to treat hip replacements, sight issues, the arthritis, the fatigue, the pain, the skin lesions and whatever this disease throws at me as simply little annoyances.

The Light Within

By Robbyn, June, 2007

 

I have to say that I am really not sure if I had symptoms of MPA in the beginning. I was awaiting heart surgery and had symptoms from that for two years prior. That being said I had open-heart surgery in February 2004. I was starting to feel great in April, because of the improvements in my heart. But soon after that I started going downhill. I thought I was just out of shape and started walking. Difficult and unsuccessful I might add. 

 

I went to the hospital in May 2004 because I was coughing up blood. There were a few diagnoses along the way. First TB, then Pneumonia, but I remember the doctor showing me the x-ray and saying how strange the location of the 'fluid' in my lungs. Not usually associated with Pneumonia. At this point my hemoglobin was also getting very low and I was getting blood transfusions every week. This went on for 2 months.

 

Then on July 2 everything in my life changed.  What a birthday that was. I remember wrapping up in a down quilt because I was so cold. It was 80 degrees out.  I thought I was getting the flu. The next morning I awoke with a severe bleeding nose. We went off to our local community hospital. I spent the day in Emergency getting several blood transfusions. Still no answers. They didn’t know what was wrong and I was getting very ill. So I was sent by air ambulance to a major teaching hospital in Toronto. My lungs were hemorrhaging at this point.  Once I was in Toronto my diagnosis was relatively easy, so fortunately I didn't have to see a lot of doctors.  They started me on large doses of Prednisone right away. They felt sure they knew what it was. They did a kidney biopsy, which is when they knew for sure it was MPA. I went to the hospital with a bleeding nose and came home 3 weeks later, with MPA.

 

Because of the mechanical valve they put in my heart I am on Coumadin, so this complicated the bleeding problems. I don't remember the next week. I was on life support in ICU. 

 

I remember my doctor telling me the drugs were as bad as the disease. Like most people 6 months of IV cyclophosphmide, along with 80 mgs Prednisone daily. Unfortunately I reacted very poorly to the Prednisone. It affected the muscles in my legs, which caused me to fall a great deal. Forty-nine years old and using a walker. I was too embarrassed to use it outside of the home. The most difficult part for me was not being able to care for myself. The doctor ordered a homemaker to come in 3 times a week to help me. You have to sit back, heal, and let the world go on without you for a while. 

 

I actually recovered quite well from the initial shock of diagnosis. But it seems this was just the beginning of my problems. You not only have to deal with the MPA but you have to deal with all the related issues. Shingles, Sleep Apnea (because of weight gain from Prednisone, I sure wish someone had warned me about that), severe mood swings, hepatitis (probably from a blood transfusion), Anemia, Pneumonia, numerous bladder and lung infections. I am still on a small amount of Prednisone and take Imuran.

 

I still deal with moderately high sed rates, and kidney function test that are not normal. I go for blood test every month and if something is going to happen to me, well, they will know right away. Part of the healing process has been for me is the realization that I won't be like I was before I got sick and you know what - that's okay. I am a different me now.  I lost most of my straight hair from the drugs but now I have this full head of curly hair, and when I glance at myself in the mirror I still wonder who I am seeing.  I take very good care of myself. I eat extremely well. I do aqua-fitness 2 times a week, which has made a tremendous difference in my life, along with the use of a stationary bike. I have lost all my Prednisone weight and then some. In some ways I am in better shape than I was before. I see that there is light at this end of this tunnel and that light comes from within myself. And each day it gets brighter and brighter. Everyday my body amazes me, what it has gone through and what it still has to give.

Wrestling the Unknown

Cindy Webber, VF Education and Awareness Council

December 2006

Cindy WebberFor the first 43 years of my life I was "boring" from a medical perspective. I was neither underweight, nor overweight. I worked out regularly and had no real medical history. In fact, my visits to a doctor were rare enough that I had no real name to write when asked to fill out the name of my doctor.

Even in my 43rd year, the disease entered into my life in a low key fashion. I had a mysterious "bruise" that persisted intermittently for months. An MRI showed "inflammation" and the doctors thought I had a deep tissue bruise. There were other events as well that barely registered a blip on the medical screen. I had occasional blurry vision that the eye doctor thought was allergies. At times my ears felt like I was underwater and the ENT felt I had a Eustachian tube problem. And of course that bruise would come and go....

Yes, my vasculitis was wily in those early days. Typically my symptoms would clear up to coincide with my doctor's appointments. I felt like a hypochondriac as I would describe my symptoms but lacked the physical evidence that the doctors would find helpful. I assumed that all these little physical annoyances were just that and that they did not relate to each other. As the saying goes, hind sight is 20:20. And, life was still pretty normal.

I can pinpoint the exact day that it stopped being low key. It was at 2:00p.m.on May 6th, 2005. Everything up until then was pale by comparison. That day, I felt a fever sweep over me and an intense ache in all my muscles. Although I had trained for a 10K race the day before, I could barely lift my legs to climb my stairs and plod into bed. At the school where I worked, Strep, Fifth's disease and assorted viruses were making the rounds. I thought I was a casualty of one of those culprits. Aspirin became my ammunition. Time, however, was not my ally. As each day passed, more and more was going wrong with me. Of importance, my bruise was back and was now surrounded by red dots which the doctors called petecheae. Other new symptoms included pins and needles in my extremities which attacked my limbs one by one. Fevers and night sweats taunted me each night. Soon, weight loss and swelling of my ankles and feet joined rank with the ubiquitous muscle and joint pain. I could no longer run, walk or stand.

By now, I was having extensive amounts of lab tests run. More and more abnormal labs were "flagged" each time as if my body was surrendering to the disease. A neurologist, dermatologist and rheumatologist were working diligently on my behalf. I had MRI's, Doppler sonograms, skin biopsies and an angiogram--all yielding little information other than "inflammation". I gave my blood like a trooper hoping the doctors would know how to help me fight whatever was going on. I gave over 50 tubes of blood for lab tests that June. Many tests registered positive for inflammation but, where, why and when were still unanswered-even with a positive P-Anca. My enemy was still unnamed.

It was a simple routine test-an abnormal unrinalysis that solved the puzzle. My internist phoned me. "Cynthia, we need to add another doctor to the mix-a nephrologist." My nephrologist, Dr. Kittaka, recommended a kidney biopsy. He warned me that a biopsy is like finding a needle in a haystack and that it was possible we might not get a telling sample. The kidney biopsy indeed captured a piece of offending tissue and the diagnosis of Microscopic Polyangiitis (MPA) was nailed.

Although I was seriously ill, the news that I had a disease like this shocked me. In my mind, I hadn't expected anything as serious as MPA. Words reverberated in my mind: rare, life threatening, incurable... The drugs I needed to heal were explained to me. More words: hair loss, weight gain..... I was started on prednisone and oral cytoxan. Within 4-6 weeks, my lab tests returned to normal. I was now winning the battle! Physically, I was healing. Emotionally, I remained scared.

Attitude is important in winning a war. I still lacked positive belief and hope. A friend recommended I call the then Wegener's Granulamatosis Association. I remember apologizing that I didn't have WG when I called. I was assured it didn't matter. I will forever be grateful I made that call. I met people that had dealt with vasculitis for decades. They filled me with hope and inspiration. Having fought the vasculitis fight themselves, they alleviated most of my fears and worries about my future. Most of what I worried about never came to be-including hair loss. They are, and will forever be, my vasculitis mentors.

I continued the Cytoxan and prednisone for 6 months. I used various amounts of Imuran as my maintenance drug for the next 9 months. I am in remission and have been off all medications. Life is good.

Currently I am back to teaching, being a wife and a mother. I have even run a few 5 K's. I have received excellent medical care from Dr. Martin K. Kittaka from Elmhurst, Illinois and Dr. Gary Hoffman from the Cleveland Clinic.

Life has come full circle and is even fuller from this experience. I treasure each day and find time to pursue the dreams close to my heart. One of those dreams is to help others with MPA and vasculitis find support, hope and inspiration to face the challenges of vasculitis. Along these lines, it is my priviledge to be a member of the Education and Awareness Council with the hope of building solidarity for the support, research and awareness of vasculitis.

A Profile of Ed Becker

By Kathy Savickas, December 2006

"I'm sorry. Your Mother has a very serious and rare disease called Polyarteritis Nodosa (PAN)." The doctor's words were still ringing in our heads as we tried to understand the diagnosis. Our family looked at one another in shock. My Mom looked very scared. The doctor appeared deeply concerned.

We peppered the doctor with questions about a disease that was a total mystery to us. What does PAN mean? What is it doing to her body? How do you treat a PAN patient? How did she get it? What's the life expectancy of a PAN patient?

A week earlier we had brought my Mom, Florence Becker, into the hospital in serious condition. Nearly three months of tests and evaluations couldn't adequately explain the symptoms that continued to grow worse. Shortness of breath, terrible headaches, a persistent, low-grade fever and abdominal pains had taken their toll on her. Now she was at the point of collapse.

Her kidneys had all but ceased to function. Her blood pressure was unusually and dangerously high. Something was obviously wrong but the doctors couldn't identify the cause. She was quickly placed on dialysis three days a week.

A week later a biopsy on her kidney revealed the underlying illness--classic Polyarteritis Nodosa. She was placed on an aggressive course of Cytoxan and prednisone. Her doctor told us that in her whole career she had never treated a patient with this disease. It became clear that unlike cancer, PAN was not widely known or understood.

My Mom's health quickly deteriorated throughout August. The Catch-22 of treatment was damning. Treating PAN required suppressing the body's immune system that left her open to infection. Unfortunately she contracted such an infection and her ability to fight it was gone.

On September 12, 1998 Florence passed away from complications of PAN.

Ed Becker remembered the frustration at not finding adequate information about PAN when his Mom was ill so he decided to create a website--a central source of information about the disease. "Honestly, the site was initially created as a web design for a class project. I actually forgot it was out there. A couple of weeks later I checked the site only to find 60 people had visited the site and left messages in my guest book. They were thankful to find a site with this info. That was my awakening to the real need for the PAN site."

Ed learned that visitors needed more than just information. They wanted to connect with other patients. " I remember when my mom was diagnosed, one of the first questions she asked was if anyone else out there had the disease. She wanted to talk to someone who was going through the same thing. At that time there wasn't such a forum. Now, however, I realized I could fill that need. The PAN suppport mailing list became that venue to allow members to connect with each other."

Ed checks his email every evening and on Sunday evening he moderates chat sessions that give PAN patients the opportunity to ask questions of doctors and medical researchers. The PAN Research and Support Network draws more than 688 PAN patients, their families and friends, and physicians and researchers.

Ed Becker has never charged for participation nor has he ever earned a penny from the work he does. He feels this is what he has been called to do - to help others.

Ed was motivated to join the VF because he felt the VF Education Council would be the best way to not only increase awareness of PAN, but for ALL vasculitis diseases. There's a huge challenge ahead to educate the public about these diseases and he simply felt this was one of the best venues to do so.

Although Ed is working on formulating his specific goals he is willing to put his skills to work for the cause. One of his personal goals is to work with Dick Burns and Christy Abele to help increase awareness about the Vasculitis Foundation support groups throughout the world. He has already started a campaign to get the word out in his PAN Network that such groups are out there --- maybe in their own city! He feels it would be valuable for other group leaders to let their organizations know about these regional support groups and encourage them to not only attend, but start a new one.

Ed would like readers to know there is a small (but growing) group of dedicated people who are working intensely behind the scenes to make vasculitis a household word and to get the word out about these diseases.

The evolution of Ed's PAN Network is amazing and it shows the challenge that lies ahead for the VF as well. When he first created the PAN Network website he didn't even know if there were even three others out there with this disease. Now, he gets more members every week who have just been diagnosed. A week doesn't go by that he doesn't get an email or phone call from someone who has discovered our resource and is immensely grateful. Patient's lives have been changed--maybe saved-- because we have shared invaluable information, connected someone to a doctor or clinic, or simply provided a forum where patients can connect with each other.

Beating the Odds

By Rhonda Lane, December 2006

People beat the odds all the time, so there's no reason why we can't be among them. Still, the most useful advice I received as a patient pertained to the prednisone. A friend who is a lab supervisor for a pharmaceutical company told me, "Always remember - it's not you, it's the pills." But my other friend, a science fiction/fantasy writer reminded me of a motto from the world of swords and sorcery: "Heavy magic comes at a price."

Still, I know I'm one of the lucky ones. Things started going downhill into vasculitis land in March 2002 when I woke up one morning with a terrible pain in my neck and couldn't close my jaw. After six months of treating the pain - which would pop up elsewhere -- I ended up in a hospital at Thanksgiving 2002 with pneumonia and congestive heart failure.

A battery of blood tests revealed severe anemia, an astronomical sed rate and reduced kidney function. Because I was "an interesting case," I had visits from a cardiologist, a pulmonologist, a rheumatologist, a nephrologist and an infectious disease specialist. My kidney function improved, so the nephrologist then dismissed the idea of a kidney biopsy, posed by my PCP.

After that, I had six months of continued anemia, continued high sed rate and more blood tests. I felt better, but my PCP was adamant that I was not out of the woods yet. Finally, a summer cold slid into bronchitis which then plunged into, once again, pneumonia and congestive heart failure. CT scans showed that my kidney was mushy, but its function improved again while I was hospitalized.

A new nephroligist succumbed to my PCPs nagging for a kidney biopsy, all the while saying, "I don't see why but he wants it so we're doing it." A couple of weeks later, the nephrologist called me back, "I don't believe it. We found something in a tiny vessel deep inside your kidney."

I underwent treatment with prednisone and Cytoxan, with a battery of high blood pressure meds to counteract the side effects. I had trouble coming off prednisone because I developed pleurisy/costochondritis/whatever, depending upon who you asked, at low doses of steroids. One time, doctors mistook that for pneumonia and hospitalized me twice until a doctor with experience with lupus patients recognized what was happening.

Bottom line: diagnosis for me took about a year and a half (counting the debilitating case of TMJ that heralded the start of the downward slide). I was on Cytoxan for a year and a half and prednisone for three years. I'm still taking blood pressure meds, although my treatment for vasculitis itself is over.

Zest for Life

 By Steve Bernhardt, December 2006

My name is Steve Bernhardt and I was diagnosed with PAN in December 2004 by a radiologist after having a renal arteriogram. My symptoms started in August 1998, when I went to the hospital while on a business trip with what they thought were kidney stones. Over the next few years I made several trips to the ER with flank pain, groin pain and numbness in legs and arms and high blood pressure. Although small things were noticed with the various tests they ran, no diagnosis was ever made. During this period I also made numerous visits to urologists, internal specialists, nephrologists and cardiologists. Even met with a chief vascular surgeon at one point, but he chose not to run any tests. None of them came up with a diagnosis.

After another trip to the ER in November 2004, I asked my nephrologist what she would do. She suggested a renal arteriogram. Scheduled for December 6, 2004, it was then that I was finally diagnosed by the radiologist. "Conclusion: Constellation of findings (concentric narrowing of proximal celiac artery with surrounding soft tissue swelling, left renal micro aneurysms, and left common iliac arterial eccentric aneurysm are most likely related to endarteritis. Polyarteritis Nodosa typically presents with the micro aneurysms seen in the left kidney. Although considered less likely, mycotic aneurysms could have this appearance"

The radiologist recommended that I see a rheumatologist ASAP. We then received a call from my nephrologist who requested to see me ASAP. They had heard of the diagnosis from the hospital and immediately prescribed clonidine and diastolic to control my high blood pressure. They seconded the suggestion to see a rheumatologist. We did go to someone who came highly recommended, supposedly one of the top rheumatologists in the area. However, he had not ever treated anyone with this disease, nor did he know anything about it. We were then referred to the Chief of Rheumatology at UCI Irvine (University of California, Irvine Medical Center).
On December 15th I called my nephrologist because my blood pressure was very low and my pulse was 114-121.
I also had a bad headache and severe pressure pains behind my eyes. He saw me immediately and called UCI to explain the situation and get an earlier appointment that what we were able to get scheduled. Per the doctor at UCI, my nephrologist prescribed the following immediately:

Toprol XL-50 mg, Diovan - 80 mg twice a day, Protonix 40 mg (took me off Clonidine) and increased predisone to 60 mg per day.

On December 16th I saw a dermatologist, who diagnosed me with statsis dermatitis and provided me with medications for my legs, chest and head.

On December 17th the nephrologist called to follow up on my blood pressure and increased my Toprol XL to 75 mg per day and started me on calcium with vitam