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.