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The story of an
unexpected crisis
- It began with urinary retention and emergency
admission to hospital for catheterisation. The doctor's skill is not in doubt
and, as seems quite usual, following the procedure there was some bleeding into
the urine. This bleeding normally clears in two or three days and in order to
ensure all is well the patient is often admitted to hospital until the urine is
seen to be clear of blood and a normal colour.
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- Unfortunately for him this was not the case and
after 4 days he was still losing blood and not very well at all. A few more days
passed and, since he was in considerable discomfort with the catheter regularly
being blocked by blood clots and an ultrasound showed a 'lump' in the bladder,
it was decided to perform a cystoscopy and have a look inside - a routine
operation usually lasting only an hour or two from leaving the ward to being
returned fully awake. The accumulated blood was removed and the surgeon did his
best to stop the internal bleeding, found that the 'lump' had been blood and
that there was no evidence of anything untoward.
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- That night he was in serious trouble with
continued bleeding and, to make matters worse, his heart was giving considerable
cause for concern because of the blood loss. By the morning it was essential for
him to undergo a second cystoscopy, despite his age (80+) and the condition of
his heart, to endeavor to stop the bleeding and his family were warned that he
may not survive the operation. There was no choice since without it he would
surely die and there was no guarantee that it would work. No further treatment
would be possible - nothing more could be done.
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- He was taken to theatre that morning, too ill
to be aware of the seriousness of his condition and in considerable pain,
leaving his family wondering whether they would see him again. Some six hours
later he was returned to the ward after a very troublesome operation and much
loss of blood. It was hoped that the bleeding would now cease and the site of
the blood loss would heal. A second, supra pubic, catheter had been inserted and
an almost constant irrigation system had been set up with careful watch kept.
All did not go well and he suffered much pain, deteriorating rapidly and
requiring blood and plasma transfusions.
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- The hematologist was consulted and an analysis
of his blood was completed. It was found that he was deficient in the necessary
clotting factors and, when questioned about previous injuries and dentistry, he
confirmed that he had not had any problems with bleeding before. This led to a
diagnosis of Acquired Von Willebrands Disease and, as you will be aware if you
have searched for information, this is a very rare disease.
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- I had been fortunate enough to have typed into
a search engine (Google) 'rare blood disorders' and found a case record of
acquired vWD in an 83 year old woman. I had printed it and brought it with me to
the hospital on the morning that we were told of the diagnosis and was therefore
able to understand that positive diagnosis was difficult and that problems were
likely in finding the right treatment for this acquired haemophilia.
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- Von Willebrand Disease, in it's various forms
from mild to severe, is an auto immune disorder where the body produces
antibodies to fight against and destroy the components in the blood that are
necessary for proper clotting and healing of a wound. References below are ones
that I found most useful in trying to understand the problems involved.
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- Factor
VIII was administered together with tranexamic
acid, steroidal anti-inflammatory drugs and hormones. Initially this had a
good result and he stopped bleeding. For a little while his urine became a
normal colour and all seemed well. The standard catheter was removed and so was
the irrigation. He became his normal self and began eating again after nearly
three weeks with little food.
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- After a few days things began to deteriorate
again with further bleeding and more clots blocking the supra pubic catheter
that had been left in place. The plan had been to clamp this catheter in a
couple of weeks and, if all worked normally, to remove it. The situation had now
become very serious again and the next choice of treatment serious too. Normal
Human Immunoglobulin for an immediate action to stop the bleeding and cyclophosphamide
in the hope of a longer term action, together with prednisolone, stilboesterol
and a number of other medications related to the prostate problem. In a day or
two this began to work and the bleeding stopped. Then we waited to see if the
situation would remain stable and the waiting was hard.
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- Five days after the new treatment began he was
transferred to a local hospital for recuperation. A week after this he had
gained enough strength to manage a visit home for a short while and was very
happy to do so after six weeks! At the end of the following week he returned to
the main hospital for the catheter to be clamped and possibly removed. But all
was not well and it was found that he had another urinary infection and that his
white blood cell count was very low due, almost certainly, to the action of the cyclophosphamide.
Another crisis, since he became very weak, stopped eating again and, apart from
the administration of anti-biotics to combat the infection, nothing could be
done to improve the white blood cell count except wait. He was in a very
low state indeed and still suffering badly from the pressure sores that had
developed some weeks before.
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- Fortunately the anti-biotic began working a day
or two later and with the infection now under control, and feeling a little
better, he was returned to the local hospital. If infections could be avoided it
was thought that he might be able to gain strength again and progress. Slowly he
began to improve
and, though very tired and still very weak, he gained sufficient strength to
return home. He needed carers daily and help from family and friends but was able to start living a relatively normal life
again 11 weeks after he was first admitted.
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During the latter part of his stay in hospital
his mental state deteriorated and it was thought that he may have suffered
another 'mini stroke' since he was, at times, unaware of what he was doing and
where he was, wandering around the hospital ward at night. It is possible that
his confused mental state was due to a combination of infection, drugs and
depression. Once the infections had been cleared up, medications re-assessed and
removed and, with the promise of going home, the depression reduced and he began
eating well and gaining strength.
He went home for a trial visit some two weeks later and, all seeming to be well,
was discharged about a week after that. He had carers each day to make sure he
took his medication - now much reduced thankfully - and to check that all was
well. There were various problems but these were coped withr. He
found it a little difficult managing on his own but enjoyed being at home.
Some two months later he suddenly began a nosebleed and when after 4 hours it
had not stopped he was admitted to hospital again. His nose was packed but the
bleeding worsened and it was decided to administer Factor
VIII(Y) which had a rapid effect - the bleeding stopped and he was
discharged home. Unfortunately this effect was deceptive since he began a
nosebleed yet again within 4 days and was re-admitted. This time ribbon packing
was tried, being less invasive, but eventually a Foley catheter was inserted
into the bleeding nostril and packing in the other since, again, the bleeding
had worsened. Factor VIII(Y) was again given and this was effective. A follow up treatment was
needed for a longer term effect and so he was given another course of cyclophosphamide
together with tranexamic
acid and re-started on prednisolone
(steroid).
He was discharged to his local hospital and then to a Residential Home since
recently he had been finding it difficult to cope alone and had had several
falls. We didn't know whether there would be a re-occurrence of the bleeding but
hoped it would not be for a few months and at least the method of treatment
would be more certain and quickly administered.
Six weeks later blood was seen in his urine and
he was re-admitted to hospital. An infection was diagnosed, anti-biotics given
intravenously and he rapidly improved. The dosage of prednisolone
was increased from 5mg to 60mg temporarily and, the urine having cleared, he was
discharged five days later. He was re-admitted two days after that but, all
seeming well, was discharged again. A few problems occurred with
infections, blocked catheter and small incidents of bleeding but it was possible to deal with these. The dose of
prednisolone
had been reduced to 40mg per day. Almost 8 months since he was first
admitted. Just two days later he was admitted again and remained in hospital for
one week for tests for possible kidney impairment and after being discharged he
was depressed and lethargic and became quite frail.
Anti-depressants had helped to lift his mood
but, at the end of February 2003, some 10 months after his problems began, he
developed a urinary infection again. This time, sadly, he did not recover.
We miss him terribly.
- Last updated: 22 July, 2008
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- Links:
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cyclophosphamide
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