Hi there! I'm Alun Ackery. I'm an
Emergency Department physician at
St. Michael's Hospital in Toronto, and we put
together this video discuss the five key
points you need to know when providing
urgent or office based care to
hemophilia patients.
If you're an Emergency Department doc or family
physician and not sure how to manage a
hemophilia patient who walks in your
clinic or office, you'll find helpful information here.
Let's dive in. [splash sound]
First of all, the term hemophilia refers to
deficiency of either coagulation factor eight or nine.
These two deficiencies are
very similar clinically, and together
they comprise the most common of the
inherited bleeding disorders.
When it comes to management, the first point is
hemophilia patients can help you
design their care plan.
Most hemophilia patients are highly knowledgeable about
their disease, after all they've been
living with this congenital disease
their whole life.
They're usually able to self-diagnose
a bleeding event, they're familiar with
their treatment regimen and schedule, and
most severe hemophiliacs self-manage
their disease at home.
Also, most bleeding events are hidden in joints or muscles
and cannot be easily validated.
So, if the patient says,
"I know I'm having an early bleed,"
they're probably right.
You may be wondering, if hemophilia patients
self-manage their disease,
why would they even come in for an
urgent care visit?
For starters, not all hemophiliacs have been trained to do
their own infusions at home, so they may
come in needing factor replacement for
a typical joint or muscle bleed.
Others may need treatment for an exceptionally
severe bleed, or after an accident of trauma.
So there are hemophilia patients
out there who need your care, and often
they can help you design that care.
The second point is, don't delay
treatment by doing lab work and imaging studies.
While this may seem like a
departure from standard emerg
department routine, if a patient comes in
identifying themselves as a hemophiliac,
you should assume that any disturbance
of function is a bleed unless proven
otherwise.
And you should treat them with factor
replacement therapy as quickly as
possible.
You don't have to prove that they have
their congenital disease by waiting for
a PTT result.
If you think an imaging
study is indicated, go ahead and order it,
but treat the patient first, then do your test.
The longer treatment is delayed, the
greater the morbidity, and the worse the outcome.
So focus on treatment, not investigation.
If your blood bank doesn't
have this
specific treatment in stock, in a pinch,
you can use cryoprecipitate for
factor eight replacement and FFP for factor nine replacement.
You can add tranexamic acid too,
which helps in some cases.
Point 3 is a related one. When in doubt, treat.
The message here is not to
worry about overtreatment.
When it comes to hemophilia, overtreatment of a
bleeding event with replacement therapy
is typically innocuous.
The risk-benefit ratio favors treatment even if the
probability of a bleed is not terribly
high in your mind.
There's no harm in giving factor eight or nine to a
hemophiliac who turns out not to be bleeding.
On the other hand, leaving a
bleed untreated can lead to serious
consequences for the patient.
For example, a patient presenting with a severe
headache could be having an intracranial
bleed, a large muscle hematoma can cause
nerve or vascular compression, and even a
few bleeds into a single joint may
eventually lead to crippling arthritis
requiring surgery.
Okay, point four. When providing medical services to a person
with hemophilia, in the large majority of
cases, it's possible to completely
reverse the clinical phenotype of hemophilia,
and make a patient temporarily non-hemophilic.
Therefore, hemophilia
patients should not be denied necessary
diagnostic or therapeutic procedures for
medical conditions
just because of their hemophilia.
If there's an operational procedure that
your hemophilia patient needs,
one that you would do on any other patient,
with proper coverage you'll be able to
do it safely on this patient too.
The fifth and final point is, support is available.
Any hemophilia patient who
comes to you is most likely registered
with one of Canada's 23 hemophilia treatment centres.
They usually carry a
hemophilia ID card that contains the
contact information of their home hemophilia centre.
If you're not sure how to proceed,
do not hesitate to call the doctor or
centre where that patient is registered.
The patient may also show you a quick
reference guide developed in conjunction
with this video that summarizes the key
points covered here.
You can also go to the website of the
Association of Hemophilia Clinic Directors of Canada,
and click on the members list.
There, you can find the hospital or doctor where
your patient is registered, or you can
locate the hemophilia expert nearest you,
and contact them for advice.
So that's the overview of the steps you
should follow when treating a hemophilia patient.
Let's recap the five
points of hemophilia urgent care 101.
Number one: hemophilia patients can often
help you design their care plan.
Two: don't delay treatment.
Three: when in doubt, treat.
Four: don't change your treatment algorithm.
And number five: support is available.
We realize treating hemophilia is not something you usually do.
Our goal with this video is to help you
feel as comfortable as possible, if, and
when that situation arises.
Thanks for watching!