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!