Fellow Chalk Talk: The Bleeding Patient w/ Dr. Espinoza
/Objectives
Develop a schema for the evaluation of bleeding patients
Manage a patient with hemophilia
Questions From the Talk
+ Why are some factor activity goals 100% and others 50%?
The best way to understand this is to think about the pathophysiology of the disease and the goal. I think of these patients in a similar vein as chronic heart failure patients who present with an acute exacerbation - they can either be "compensated" (hemostatic) or "decompensated' (bleeding). Our goal acutely is to make them compensated - stop the bleeding, but we cannot change the factor level in the long run.
In minor bleeding, getting someone to 50% is adequate to achieve hemostasis. If the patient has a severe bleed, then you need a higher level of factor activity to achieve hemostasis.
+ What happens to hemophilia patients after discharge?
Once we have acutely achieved hemostasis, the patients are discharged, but what happens next...?
In Mild Hemophilia (5-50% activity) patients rarely have spontaneous bleeds, but they will bleed with trauma or procedures. These patient's are usually stable unless something happens. After an acute bleed and factor infusions, they are usually stable unless there is another precipitating event.
Severe hemophilia patients can have spontaneous bleeding, and even achieving hemostasis with recombinant factor in the hospital is only temporary. Often these are patients who get prophylactic factor infusions either weekly (or some up to every other day!) as outpatients.
+ How do you know when to give factor vs desmopressin in Von Willebrand disease deficiency?
Use only give if the patient has a DOCUMENTED response to DDAVP in the past. This should be easily found in the hematology outpatient clinic note. Be cautious if giving it for more than 3 days - it can cause tachyphylaxis.
This depends on the level of bleeding. Desmopressin can be given in patients with minor bleeding, some even have intranasal desmopressin at home to use during menstruation. If the bleeding is more severe, the pharmacy is able to get factor fairly quickly if needed.
+ How do you know when to stop giving factor once you reach your goal?
This is part of the "art of medicine" or certain prescribing guidelines. There are no clear guidelines - but if you look at Dr. Espinoza's slides, after a certain number of days of treatment, with stable levels of factor activity at the goal level, and hemostasis - then you can likely discharge them.
+ What is Hemlibra?
This is a new medication used to treat hemophilia that will give patients a dramatic improvement if quality of life because it is subcutaneous instead of an infusion and can be dosed up to once per month! This is used as a prophylactic medication - not an acute treatment. The main thing that generalists should know is that CANNOT check VIII levels, PTT or Bethesda titers in these patients as the medication disrupts the results.
Additional Resources
MKSAP 18
Heme Onc 72
Heme Onc 59
Heme Onc 71
Heme Onc 46
