I was 31 years old with a two year old daughter. I was planning
on having a second child, going back to work and raising a family.
In 1981 I started to experience a problem with pruritus, fullness
in the head and an increase in blood pressure. I was young, thin
and in shape. I worked for Social Security Disability as a medical
analyst knew the itching I was experiencing could be symptomatic
of a blood disorder. I had gone to several doctors who said my
symptoms were due to allergies. In October of 1982, after several
miscarriages, my gynecologist performed routine blood work. My
hematocrit was found to be 71% and my platelets were 590,000.
My physician decided this must be a lab error. I thought differently.
Suddenly all my symptoms made sense. After a second round of tests confirmed my suspicions, a visit to a hematologist was immediately made. He said I had a classic case of P.V. (polycythemia vera). My spleen was 7cm below the coastal margin. The hematologist told me on that day that I should not get pregnant and that my life would change. I had a husband and a baby, and I was frightened I would die. I was given phlebotomies to bring my blood count down into the normal range. This was an unknown disease to me and I could not imagine what my future held. I was just 32 years old.
I was treated by a local hematologist until 1984. At that time
I decided to go for another opinion. Phlebotomies were the only
treatment I had received, however my spleen was increasing in
size. A biopsy in May of 1985 confirmed a diagnosis of post Polycythemia
myeloid metaplasia. In November of 1985 I had a bone marrow harvesting
for cryopreservation so that it would be available for autologous
bone marrow transplantation. Because of my young age and aggressive
disease this was suggested.
Unfortunately, after the harvesting was performed a hematoma formed
on my hip. I had bled after the procedure and after a few days
I started to experience a great deal of pain. An MRI confirmed
the hematoma. I was told I had to stay in bed until the clot dissolved.
That took until March. It was a very difficult time. The week
after I was able to move about I adopted a baby.
In March of 86 because of increasing myeloproliferative disease
activity, and increasing spleen size to 18cm below the costal
margin, myelosuppression was suggested. In Nov. of 86 I was started
on Interferon. I believe that I was one of the first patients
in the U.S. to be put on IFN for polycythemia. I was taken off
IFN in September of 1987 because my blood counts were stable and
spleen size had decreased.
I endured four repeated cycles of IFN. The last cycle started
in Sept. 1991. At that time my spleen was once again increasing.
I was thin and now had trouble eating because my spleen was down
to the pelvis. In December of 1991 my spleen was not shrinking
as it previously had with IFN. I was continually having night
sweats and fever from the disease and the IFN. The other side
effect included hair loss. I was depressed and feeling ill. I
desperately wanted a good quality of life so that I might enjoy
my family and good fortune.
Interferon is a wonderful drug, but it wasn't for me. I went to
another hematologist in New York City for another opinion. He
recommended that my spleen be removed and sent me to the Mayo
clinic for still another opinion. The physician at the Mayo Clinic
immediately recommended that my spleen be removed. It was also
found out at that time that I had Von Willebrand's disorder, probably
secondary to the polycythemia. This was probably the reason I
had the hematoma after my harvesting.
It was now January of 1992. My husband and I had to make a difficult
choice concerning my treatment. I decided to switch hematologists,
go off the interferon and have my spleen removed under the care
of my new hematologist in New York. After several consultations
with clotting experts, I finally had a splenectomy in July of
1992. My hematologist-oncologist started me on hydroxyurea because
my platelets had increased to over one million. I was experiencing
fungus infections of the throat and skin changes of the hands,
because of the Hydrea.
In October of 1993 I was admitted to the hospital for TIA's (Transient
Ischemic Attacks). My platelets were over 2 million, and they
needed to be under better control. In April of 1994 I was again
admitted to the hospital due to blood clots of the hepatic and
portal veins. I was started on heparin therapy.
Over the course of time it was found that I was having a drug
toxicity. I experienced every side effect possible to every drug.
My hematologist came up with the idea to give me several drugs
at low doses. Therapy included Hydrea, anagrelide, interferon,
heparin and aspirin. Priloseca and propulsion were added after
several cases of aspirating pneumonia. A visit to a G. I. doctor
in the fall of 95 revealed severe gastric reflux. I believe this
was a result of my massive spleen. In July of 95 I started to
experience hair loss even with a small dose of interferon. (1mu
every few days). I also had low grade fevers.
My hematologist is a great believer in quality of life. He decided
to take me off the interferon, anagrelide and Hydrea. He started
me on a drug called thioguanine. It is useful in treating leukemia.
Thioguanine had helped control my counts. Platelets for most of
1995 were stable at 250,000-400,000. Anagrelide has been added
again because in May of 1996 platelets started to rise and I became
symptomatic. Heparin was discontinued due to bone loss of my hip.
Coumadin was started in its place. My hematologist switches my
medication often depending on my counts and how I feel.
After having so many years of ups and downs, my counts have been
under fairly good control for the past year or so. I know now
that I made the right decision in having a splenectomy. I have
learned that this disease acts differently in all of us. Decisions
have to be made on an individual basis. I am now back to biking,
swimming, walking and to having a good quality of life. My hematologist
comments that I came from being very ill to being able to function
pretty normally again. I am now 45 years old, and live a very
active life. My hematologist sees me often and gives me liver
function tests at every visit.
I have learned that this disease is not a death sentence. It is important to be under good control and have a physician who allows you to ask questions and to make joint decisions. I do not dwell on the future. I am enjoying today with my husband, children and friends. I do not know where and when my next road block will be. The world wide web and my internet friends have been added support. The disease is no longer frightening. It has been 14 years since the diagnosis and I know I need to keep a positive attitude. I am always looking into new ways to be treated. Myeloproliferative patients are living long lives with doctor supervision.
Sharon
Shay@northeast.net