Hemophilia and Hospitalization

A Self Learning Program
 

University of Colorado Health

Sciences Center

The Children’s Hospital Denver

By:  Susan J. Geraghty, RN
 
 
 
 
 
 
 
 

This is a self-learning program designed to give the health care provider an overview of hemophilia and the care of patients with this

disorder.
 
 

DIRECTIONS

Read this module, and answer the post-test questions.  Review your answers; if you have any incorrect answers please refer back to the text.
 
 
 
 
 
 
 
 
 
 
 
 

Hemophilia A and Hemophilia B

Hemophilia A, factor VIII deficiency, and hemophilia B, factor IX deficiency, are hereditary bleeding disorders.  There are many proteins in the blood necessary for it to clot properly.  Factor VIII and factor IX are two of these proteins.  When a patient has less than the lower limit of normal of one of these proteins, they are given a diagnosis of hemophilia.  

The genes involved in the production of factor VIII and factor IX are carried on the X chromosome.  They are frequently referred to as sex-linked disorders.  Because this is an x-linked inherited disorder, it affects males and women are carriers of the disorder.  Approximately 20% of carrier women actually have low factor levels themselves and can have bleeding with significant trauma or surgical procedures just like a hemophiliac.

In the majority of hemophilia patients there is a family history of the disorder; however a new mutation can occur for the first time in the birth of an infant.  About one out of three persons with hemophilia have no family history of the disorder.

Hemophilia A and B can occur in different severities depending on the amount of clotting protein circulating in the blood, mild >5-50%, moderate >1-5% and severe 0-1%.  The severity of the disorder remains the same in families.  Patients with severe hemophilia have the most frequent bleeding and those with mild hemophilia typically bleed following trauma or an invasive procedure.  All patients with hemophilia regardless of their severity will require specialized treatment while hospitalized.

Bleeding episodes in patients with hemophilia can occur anywhere there is an injury to a blood vessel wall, however most bleeding episodes occur in soft tissue, muscles, and joints.  Most patients are able to identify a cause of their bleed, but this is not always true,  especially in patients with severe hemophilia, as they can have what is referred to as a “spontaneous hemorrhage”. In very young children the cause of the bleed may not be known due to non verbal communication.

Treatment of hemophilia bleeding episodes is most often done by replacing the missing clotting factor either factor VIII or factor IX, in adequate amounts to stop bleeding.  The clotting concentrates are administered intravenously.  The amount or dose of factor is determined by a hematologist or primary care physician caring for the patient.  Patients with mild hemophilia A may use clotting concentrates to treat bleeding episodes like their more severe counterparts, or they often are able to use a synthetic hormone known as DDAVP to treat bleeding.  This medication is administered intravenously, and also comes in an intra nasal high concentration form.  DDAVP works by causing the release of stores of factor VIII from endothelial cells into the blood stream. This raises the factor VIII level in the blood stream. The patient should have a documented response to this medication prior to its use.  DDAVP should never be given to a child under the age of 2. There is no equivalent product for factor IX deficient patients.
 

What Brings a Child with Hemophilia to the Hospital?

UNCONTROLLED OR SERIOUS BLEEDING

Patients with all types and severity of hemophilia can present to the hospital emergency room with a bleeding episode.  Most parents/patients with severe or moderate hemophilia have been trained in home infusion by the age of 5 and are able to administer the clotting concentrate at home when a bleeding episode starts.  Patients with severe hemophilia Generally present when a bleed is causing significant pain or is not responding to the treatment.  Children who are not yet on home infusion will come in for factor replacement when they are having a bleeding episode.  Most patients/families with mild hemophilia are not able to administer the clotting factor or intravenous DDAVP at home so they will present for evaluation and treatment.   Due to infrequent bleeding in mild hemophilia these patients often do not recognize a bleed until it has gone on for some time, so they often have a significant hemorrhage in progress when they present.

Int