These comments are made for the purpose of discussion and should NOT be used as recommendations for or against therapies or other treatments. An individual patient is always advised to consult their own physician.
[posted 10/14/1999]
Question: My father is 60 years old. He has had P-Vera for over 15 years. We’ve been informed by his physician that the life expectancy of a P-Vera sufferer is about 15 years when the “accelerated phase” sets in. He told recently that my father is indeed in that phase now. The doctor explained that this phase ends in bleeding to death (due to low platelet count), leukemia, and 2 others of which I am not sure. His spleen is enlarged to the size of a fully grown foetus and his liver has also been affected by the “migrating” marrow. The marrow in his bones has almost completely gone through fibrosis. To combat the growing spleen he was given a “immino” treatment taken in 3 injections/week of dosages 6 mil units. that has been stopped and only pain treatment is being administered. The treatment for pain however does not seem to be working and he is taking more frequently than he should (resulting in nose-bleeds due to the thinned blood). His doctor has been reluctant to give us any support or advice in handling the situation. I feel we should be administering my father’s medication for him and limiting it to what has been prescribed. Any information or assistance is appreciated. (There are obviously many side-effects and other complications I hvae not mentioned but which have taken a back seat at this stage).
Answer: The pain medication should not be causing the nose bleed unless it is aspirin or a non-steroidal anti-inflammtory which I doubt. Usually in this situation, we control the pain since this is a rapidly deterioring situation and a cure is unlikely. I would discuss it with his md, but sounds like he needs more pain medication. If his md is unresponsive, revert to your primary care doctor to handle the politics/treatment. Often if he/she intervenes with the specialist the level of care will change markedly.
[posted 08/10/1999]
Question: I was diagnosed with polycythemia vera about ten years ago.
I developed deep ulcers on my ankles in the fall of 1998.
I became bed ridden and was put in a nursing home. The pain
in my legs was intolerable and I was put on heavy doses of
morphine, dilaudid and a 150 mg skin patch for pain that I
don’t recall. My son and his RN wife intervened to get me
out of the drug induced stupor and brought me to the
Mayo Clinic in January. Mayo said the ulcers were
from the hydrea and placed me in the care of a dermatologist
and a plastic surgeon. They healed the ulcers with some sort
of a vacuum pump and a skin graft. Unfortunately, the
dressing they used, some sort of a medicated foam wrap,
caused a rash and itching that has never gone away. My blood
counts are up to 950 again and my blood is too thick to be
drawn I am told. I went to the doctor and had my liver
functions tested. Everything was within normal ranges.
The itch is driving me crazy. My regular doctor said
my blood doctor should check for lymphoma and a liver tumor.
Am I experiencing unusual symptoms for polycythemia vera?
Is there anything you can recommend for the itch? Is the
itch related to the p-v? My ankles are beginning to ache
and are red the way they were before the onset of the
ulcers. I have been off the hydrea since checking into MAYO.
I feel as if I am going crazy with all the recent problems
I have experienced. Any advice will be appreciated.
Answer: Try some benadryl for the itch. It doesn’t sound like anyone is co-ordinating your care, this is probably the first step.
Polycythemia Vera [posted 8/11/98]
Question: My father is currently 57 years old and was diagnosed
with Polycythemia Vera 7 months ago. His treatment started
immediately by phlebotomy and baby aspirin to maintain a
hematocrit level below 47 ml/kg while decreasing blood
viscosity. Although this seemed like the solution to his
problem for the time being, his platelet level kept rising
from 400,000 at the time of diagnosis to 600,000 recently.
He also has a blood iron level of 4.3 Umol/L .
Besides the Blood Picture tests which he now does biweekly,
he also conducted the following tests:
Ultra Sound: Spleen, Liver, kidneys all appear to be normal.
Bone Marrow Examination (Conducted After Blood Drawing ): Normocellular and normoblastic marrow showing active
granlopoiesis with slight eosinophilia.
Megakaryocytes are slightly increased in number, but of
normal morphology. There is no evidence of hypoplasia,
leukemia or malignant infiltration at the site of
aspiration . There is no evidence of myelofibrosis.
Iron stores appear depleted.
Although the hematocrit is now under control by
continuous blood testing and intermittent blood drawing (as
needed), the platelet level seems to be in continuous rise.
If it continues to do so, at what level should he start to
take any further action and what would that action be?
I have heard that there are more than one possible
courses of treatment for cases that cannot be controlled by
phlebotomy and aspirin alone. These include Hydroxyurea
Interferon Alpha and P32. Can you please explain briefly
the advantages and disadvantages of these different
treatments and which would be the preferred one?
Should he be taking supplementary iron at this stage or
not? What are the names of the Prominent Institutions in
North America that treat this disease and how much time and
money (rough estimate) would be needed to treat such a case
for someone with no medical coverage and currently lives
outside North America ?
Answer: Most physicians usually start treatment above 500,000 and give chemo suppression at 1,000,000. Aspirin is usually given between 500,000 and 1,000,000, but some physicians will give chemo suppression at 500,000 – especially if there is a high risk of stroke (vascular disease etc.). The advantages of each mode of treatment really vary with the patient and their individual risks. The usual sequence is hydroxyurea, P 32 and Interferon – as each one loses its effectiveness – this is usually done by toxicity and cost. He should not take iron. As to the institutions, any of the large cancer centers would treat P-vera. This would include Sloan Kettering, Roswell Park, Massachusetts General, etc. Those would be my picks especially if coming from Africa/Europe/Middle East. The costs would vary with the needed treatment. Hydroxyurea is fairly cheap.
Polycythemia Vera and Marijuana
Question: My Mother has been very ill for three years now with three
MI’s and Polycythemia Vera. She is in severe pain and
nothing really helps her. Would marijuana help her control
some of the pain she is in? I would love to see her have
some quality of life.
Answer: Possibly, but why isn’t she getting some narcotics? If her doctor isn’t addressing her pain, she
needs another opinion. it’s very uncommon to have intractable pain if the physician is working on it
properly. Also, I’m not sure where the pain is or the cause.
Polycythemia Vera
Question: My friend has been diagnosed with Polycythemia Vera. Could you give me some information on this?
Answer: Polycythemia Vera is a relatively common disorder which affects the bone marrow,
usually in older individuals. This is a type of disorder called a myeloproliterate disorder and
results in the overproduction of blood cells, platelets, and sometimes the while cells. It is
usually seen after mid-life and the cause is not known. Therapy in the initial stages is merely
blood donations by the affected individual to lower the blood count. The risk of the high
counts (especially red cells) is a stroke or blockage of small vessels as the blood becomes too
“thick”. Hydroxyurea is the drug commonly used and achieves “remission” about three-
quarters of the time. Any hematologist would be familiar with and able to treat this disease.
It is relatively simple to treat for the first few years in most individuals and does not require
a “super-specialist” unless common treatment fails.
