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 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).