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44 Cards in this Set

  • Front
  • Back
Hemophilia refers to a group of:
bleeding disorders resulting from a deficiency of specific coagulation proteins or clotting factor deficiencies
Hemophilia
-etiology?
-It is transmitted as an X-linked recessive disorder
What disease:
The patients have abnormal bleeding in response to any trauma because of a deficiency of the specific clotting factor.
Hemophilia
Hemophilia
-Tell me about the issue of bleeding.

-Patho of why they bleed the way they do comparead to people without the disease?
-Pts with hemophilia do not bleed more often or even more rapidly than those without the disease, but they do bleed for a longer period.

-They form platelet plugs at the bleeding site, but the clotting factor deficiency impairs the formation of stable fibrin clots. This allows excessive bleeding.
Hemophilia A: deficiency in what clotting factor?
factor VIII (cryoprecipitate) which is needed for clotting
Hemophilia B: deficiency of what clotting factor?
factor IX
- rarest also called the Christmas disease because it was discovered in a little boy in England and his last name was Christmas.
Von Willebrand disease results from what?
impaired platelets that adhere to damaged endothelium and deficiency factor VIII.
Von Willebrand disease
-missing which factor?
-why is that factor important?
Missing von willebrand factor (fWF) which needs to be present in order for clotting to take place.
-stabilizes factor VII but can’t be used by the body
Treatment for hemophilias
-avoid what (3)
-avoid injury
-avoid ASA
-avoid NSAIDS
Treatment for hemophilias

-control bleeding, how?
(pressure, ice)
cryoprecipitate is given for what?
Treatment for hemophilias

– contains all these factors (including what is needed for clotting): VIII, XIII, vWF, fibrinogen, fibronectin (usually given prior to surgery)
Treatment for hemophilias
-generally speaking, treatment includes administration of:
-clotting factors
Treatment for hemophilias
-what drug is given to stimulate more factor VIII and vWF?
DDAVP (desmopressin)

stimulate more factor VIII (Hemophilia A) and vWF (Von Willebrand Disease)
DDAVP (desmopressin) is given for what?
treatment for hemophilias

it is also called vasopressor or ADH
DDAVP (desmopressin) is used for hemophilias but can also be used for what other s/s?
can be used for bed wetting (enuresis)
Von Willebrand Disease
-Estrogen does what?
(increases synthesis of vWF )
CM of Hemophiliacs
-all relating to blood/bleeding
-abnormal bleeding in response to trauma or surgery
-Epistaxis (nosebleeds)
-Tendency to bruise easily
The most common problem that occurs as a result of hemophila is:

CM include:
degenerating joint function as a result of chronic bleeding into the joints

-joint bleeding causing pain, tenderness, swelling, and limited ROM
Labs of Hemophiliacs
-PTT?
-PT?
-prolonged PTT
-normal PT
Disseminated Intravascular Coagulation
-occurs with some horrific stress to the body, such as:
–Shock
–Septicemia
–Hemolytic processes
–Obstetric conditions
–Tissue damage
-Trauma
-Burns
What disease?

Abnormally initiated and accelerated clotting
Disseminated Intravascular Coagulation
Disseminated Intravascular Coagulation

-Pathophysiology
how does this result?
-the body starts clotting as a response to the bleeding that is occurring. The clotting cascade is activated resulting in clot formation. The rapid formation of clots causes the clotting factors and platelets to be used up so as a result of this you have more bleeding taking place and you have inefficient clotting. bleeding and clotting occur simultaneously
Disseminated Intravascular Coagulation
-clinical manifestations
-uncontrolled bleeding
-bruising, purpura, petechiae, and ecchymosis
Disseminated Intravascular Coagulation
-CM in the reproductive system?
-presence of occult blood in excretions such as stool
-hematuria, hematemesis, or vaginal bleeding
DIC - Diagnoses

PT, PTT, APTT
all increased
DIC - Diagnoses

Fibrinogen?
decreased fibrinogen
DIC - Diagnoses

Platelets?
decreased platelets (less than 100,000)
DIC - Diagnoses

factor V, VII, VIII, X, XIII?
decreased
DIC - Diagnoses

D-dimer
increased
- D-dimer is the end product of clot formation
DIC - Diagnoses

Hematocrit level?
decreased
Disseminated Intravascular Coagulation
- why should you monitor UO?
-maintain urine output of 30mL/hr because renal failure is a complication of DIC
if you are caring for pt and you leave a handprint on them, what disease should you expect?
Disseminated Intravascular Coagulation
DIC - Treatment
-what should you first do?
Treat cause (ex: sepsis, hemorrhage) then focus on preventing organ damage secondary to microemboli and replacing the blood clotting components) monitor for signs of microemboli (cyanotic nail beds, pain)
DIC - Treatment

What drug do you give?
Why?
Heparin or LMWH (used to decrease microclots from forming and using up clotting factors) you are trying to fool the body because the problem is clotting and breakdown of clots at the same time. if you stop the clotting process, the clotting factors can accumulate and you can form a clot.
DIC - Treatment:

Transfusions using:
• Transfusions: Cryoprecipitate – factor VIII,
PRBC,
FFP,
platelets
– you are giving the body clotting factors so they can build up
Episilon-aminocaprocic acid (EACA, Amicar) is given in what disease?
DIC because it inhibits fibrinolysis so can enhance thrombosis (stops bleeding so clotting factors can accumulate)
DIC - Treatment:

Avoid the use of what drugs?
-AVOID use of NSAIDS
CBC with Differential

RBC (range)
-men
-women
men 4.5-5.3

women 4.1-5.1
CBC with Differential

Hematocrit (range)
-men
-women
-men 37-49%;
-women 36-46%
CBC with Differential

Hemoglobin (range)
-men
-women
Hemoglobin: men 13-18; women12-16
WBC:
-range?
5 -11/mm3
WBC:
-Segs (PNMs):
45-75% involved in phagocytosis
WBC:
-Bands (stabs):
0-5% immature segs
Reticulocytes:
-range?
are immature erythrocytes.

-Normal is 0.5-1.5%


(test and diagnostic book says 0.5-2)