Paula
The law of unintended
consequences hit me hard the day before the 9/11
terrorist attack on our country. It was the day I
was told I had Pemphigus Vulgaris.
The chain of events leading
to my diagnosis started a year earlier. I was 42
and had finally lost 30 lbs and gotten into the
best shape of my life. The weight loss made my
(until then) normal blood pressure go up to
borderline moderate levels. My family physician
insisted that this was not acceptable, and
persuaded me to begin taking medication. The
first drug prescribed was an ACE inhibitor, and I
spent time adjusting to it over the next months.
Two months into this period I noticed that my
gums were rough. When I asked my brother-in-law,
a dental hygienist, about it, he told me that
tartar-reducing toothpastes could cause that to
happen. Several months later I noticed a small
sore at my hairline on my forehead. I didn’t
remember where or when it happened, and it took
weeks to heal. So, about a year after I first
started the ACE inhibitor, a known trigger for
the disease, pemphigus finally showed up in
full-blown vengeance.
One hot August weekend in
2001 I got mildly sunburned watching a soccer
tournament. With Mediterranean olive skin, I
never worried about burns, but this was an
unusual one. I actually got a blister on my lip,
and my skin below my neck was strangely
different; it looked stiff and crackly. My
dermatologist gave me antibiotic creams to get it
to clear up, instructing me to come back in if
they didn’t work. They didn’t, and the
next time I went in I had a very small blister
that was excised from my skin and sent for
biopsy. Ten days later the dermatologist called
my home and asked me to come in as soon as
possible. So on 9/10/01 treatment started for PV.
One month after starting
treatment I had made no progress even though I
was taking 80 mg of prednisone a day. Most of the
skin on my body was loose, and I had sores everywhere,
some of them very painful. My husband and family
could not even touch me. At that point I was
referred to Dr. Sergei Grando at UC Davis Medical
Center in Sacramento, CA. He increased the
prednisone to 100 mg per day and told me I would
be coming in once a week until the PV was under
control. His method of checking for active PV was
to perform the Nikolski test, which involved
placing his thumb on the skin of my back and
pressing hard as he moved it down. Wherever he
pressed, the skin disintegrated and came
completely off. A week later my skin still
slipped off at the slightest touch, and I was
downgraded to a severe case of PV. I checked into
the hospital in my hometown that day and began
receiving 500 mg of prednisone a day
intravenously for the next three days. Checking
the strength of my skin at that point revealed
that it still came off with the least amount of
pressure. For the next three days I received 1000
mg of prednisone a day, again intravenously. I
checked out of the hospital and traveled to Dr.
Grando’s office, where he finally pronounced
my skin “normal and holding together on
it’s own”.
The side effects of the
large amounts of steroids did a great deal of
damage and caused massive swelling to my body,
particularly my face and neck. While I was glad
the disease had reversed course, and the sores
disappeared over the next few months, the
craziness of steroids set in for a long time. I
didn’t leave my house for months except to
go to appointments with Dr. Grando and my family
physician. I also was taking Imuran and having
blood work done once a month. A bone density test
revealed enough damage to my once normal bones to
cause me to start on Fosamax and other
osteoporosis medications. My blood sugar
skyrocketed, and I began taking glucophage to
control it. My internal organs were swollen, my
tongue was swollen, and my eyes were almost
swollen shut. I gained 40 pounds in four months.
And my very supportive husband told me I looked
fine!
I was also put under quite a
few restrictions. No extreme heat, no extreme
cold. No hot tubs, no lotions or creams. No
stress, no sweating. And no sun at all under any
circumstances, for I was told it was suicide for
me to be out in the sun.
Over the next 18 months I
tapered down from the 180 mg of prednisone I
began taking upon my leaving the hospital. I kept
a positive attitude, thinking that once I tapered
down to a normal level of steroids the disease
would never reappear. I was wrong. Just when I
thought I was getting my life back by stopping
the glucophage, walking three mornings a week,
and preparing for my eldest son’s college
graduation, a tiny blister appeared, and on the
day of my 25th wedding anniversary, it
was confirmed as a flare of PV. This was a very
low point for me. I knew exactly what lay ahead,
which was more steroids. The Imuran had caused my
liver enzymes to rise too high, and I had not
taken it in months. The new treatment protocol
was to include Cyclosporine, cyclophosamide, and
methotrexate at different times over the next
five years along with the prednisone,
tetracycline and niacinamide I had already been
taking for 18 months. I also began IVIg in
three-month on/three-month off cycles that would
also last for 5 ½ years. Most of the sores from
this flare disappeared, but not all. I
learned to live with sores on the top of my head,
something that had not happened the first time
around. The Cyclosporine caused excessive hair
growth, and I decided that it should be given to
bald guys. After that drug, the cyclophosamide
caused most of my hair to fall out, resulting in
large hairless patches on my head. I felt a great
deal of admiration and sympathy for women
undergoing chemotherapy. And, my very supportive
husband told me I looked fine!
The amount of time it took
for the second flare to come under control was
over five years. The weight gain had never really
disappeared and the swelling was still bad. I
struggled with kidney stones at the five-year
mark. I was always exhausted and neuropathy took
hold in all of my muscles, causing pain. I looked
around and saw people worse off than myself. And
I thanked God for my family and friends that
never, ever wavered in their support of me. I was
able to start physical therapy, which I badly
needed. Dr. Grando agreed to stop the prednisone
once the methotrexate worked well and made my
last sores disappear in only six weeks. Slowly,
my hair began to grow back as well. I began to
walk five days a week in an exercise pool, which
was heated in the winter. Step by step, I
regained some strength, though for a full year
after quitting steroids the pain in all my
muscles was with me every single day.
At the seven-year point in
my illness, Dr. Grando relocated to UC Irvine,
but he left me with instructions on how to taper
gradually and completely off the medications I
was still taking. With the supervision of Dr. Liu
at UC Davis Med Center, I did taper down to no
medications at all for the pemphigus, and enter
full remission. In a very bittersweet twist, a
very good friend succumbed to cancer on the day I
was free of drugs. I had watched as she battled
the disease and underwent surgery, chemo and
radiation, entering a five-year remission while I
continued to plod along on steroids. Her health
had looked wonderful, but the cancer returned to
steal her from her family and friends. I marvel
today that my own health has returned to me, it
is truly a miracle that I never thought would
happen. I had been nervous about stopping the
methotrexate; it felt like a comfortable crutch.
But, not trying to live without it was not
really an option. I put away all the pills and
never looked back.
It has now been almost two
years since I entered full remission, no drugs,
no disease. At times my mind wanders back to the
difficult days on steroids, letting some fear
return to haunt me. But I turn away from that
fear, and continue on in gladness. I may have
lost most of my health and some of my time during
my seven year struggle with a disease no one
could pronounce or spell, but I never lost what
was truly important, my family and my friends. My
heart goes out to those who have to struggle
alone through an illness like pemphigus; I could
not have gotten through by myself. For the first
seven months of treatment by Dr. Grando I noticed
that he didn’t smile much. Then there came a
day when I had tapered below a certain point on
the prednisone, and he saw that I didn’t
have a flare. A very different look came over his
face; he relaxed and smiled and said I was going
to be okay. Dr. Grando assured me that without
steroids I would have died. I’m more than
okay; I’m so very glad to be alive and
experiencing fairly normal health again.
Paula Tomei
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