2. Blood Dyscrasias Mrs. Christa Cowen MSN, RN

3. Types of Bleeding Disorders
Hemophilia A (Factor VIII deficiency)
Hemophilia B (Factor IX deficiency)- also called “Christmas disease”
von Willebrand Disease (vWD)

4. What is Hemophilia? Hemophilia is an inherited bleeding disorder
Absence or ↓ in coagulation factors prevent secondary hemostasis :. ↑risk bleeding

5. Hemostatic System Blood vessels Platelets Plasma coagulation system
Proteolytic system
Fibrinolytic system

7. Inheritance of Hemophilia
Hemophilia A and B are X-linked recessive disorders
Hemophilia is more commonly expressed in males and carried by females
Severity level is consistent between family members

8. U. S. Incidence of Hemophilia
Hemophilia A:
Severe: 50-60%
More common than B
Hemophilia B:
Severe: 44%

9. Basic genetics

10. Genetics of Hemophilia
Deficient /Defective Factor VIII / IX
Inherited X-linked traits

11. Detection of Hemophilia
Family history
Symptoms
Bruising
Bleeding with circumcision
Muscle, joint, or soft tissue bleeding
Hemostatic challenges
Surgery
Dental work
Trauma, accidents
Laboratory testing:
plt count, PT & aPTT. Only the aPTT is abnormally prolonged (↑) in hemophilia A or B:
Further immuno-absorbant assays assist in the dx

12. Degrees of Severity of Hemophilia
Reference value range:
Factor VIII (F8) or IX (F9) level = %

13. Assessment: Types of Bleeds
Joint bleeding - hemarthrosis
Muscle hemorrhage
Soft tissue - hematomas
Life threatening-bleeding
Other (easy bleeding/ bruising; with hemarthrosis “target joints” have recurrent bl)

14. Life-Threatening Bleeding
Head / Intracranial *most dangerous*
SIGNS/ SYMPTOMS
Neck and Throat
Abdominal / GI / GU
Iliopsoas Muscle

15. Other Bleeding Episodes
Mouth bleeding 2⁰ to:
Epistaxis
Scrapes and/or minor cuts
Menorrhagia

16. Complications of Bleeding
Flexion contractures
Joint arthritis / arthropathy
Chronic pain
Muscle atrophy
Compartment syndrome
Neurologic impairment

17. Treatment of Hemophilia
Replacement of missing clotting protein
On demand (on-the-spot)
Prophylaxis
Factor concentrate/ recombinant factor
DDAVP / Stimate
Antifibrinolytic Agents
Amicar
Supportive measures:
Icing
Immobilization/ compression
Rest, elevate

18. Nsg: Infusions of Factor Concentrates
Verify product with physician order
Dose may be +/- 10% ordered
Reconstitute factor per package insert
Do not waste factor even if the dose is not exactly what is ordered:
Infusion rate per package insert or pharmacy instructions
Document lot number, expiration date, time of infusion, and exact dose given in units

19. Prophylaxis
Scheduled infusions of factor concentrates to prevent most bleeding
Frequency: 2 to 3 times weekly
Use of IVAD necessary

20. DDAVP (Desmopressin acetate)
Synthetic analogue of vasopressin (ADH) that lacks pressor activity
Useful in Mild hemophilia A- ↑s circulating Factor VIII (F8) (& vWF) levels 2-4X above baseline, via release from endothelial storage sites
Therapeutic Effect->generates substantial but transient ↑ Factor VIII (F8) (& vWF) levels
NOT effective in Severe Factor VIII (F8) deficiency
Persistent antidiuretic activity can lead to water retention and serious degrees of hyponatremia

21. DDAVP (Desmopressin acetate)
Administration
Intravenous: 0.3 mcg/kg (maximum 20 mcg) diluted in 50 mL of NSS, infuse over 20 to 30 minutes; the same dose is given with subcutaneous therapy; no single injection to exceed 1.5 mL
Subcutaneously: same as above
Nasally (Stimate)
Given every 12–24 hours in limited numbers due to its tachyphylactic properties
Persistent antidiuretic activity can lead to water retention and serious degrees of hyponatremia

22. Stimate® (desmopressin acetate) Nasal Spray, 1.5 mg/mL
Usual dose is one puff (150 mcg) in patients weighing <50 kg and two puffs in patients weighing >50 kg
Dosing
Every hours prn
< 50 kg body weight - 1 spray (150 mcg)
> 50 kg body weight - 2 sprays (300 mcg)
Persistent antidiuretic activity can lead to water retention and serious degrees of hyponatremia

23. Amicar (epsilon amino caproic acid)
Antifibrinolytic- inhibits fibrinolysis->stabilizes clot
Uses- Mucocutaneous bleeding & helpful after oral surgery
Dosing: mg/kg every 6 hours
Side effects increased risk for thrombosis(58%)
WHY IS RISK ↑’d?

24. Complications of Treatment with factor concentrate
Inhibitors/Antibody development
Hepatitis A
Hepatitis B
Hepatitis C
HIV

25. Inhibitors/ Antibody Development
Definition
IgG antibody to infused Factor VIII (F8) or IX (F9) concentrates, which occurs after exposure to Factor VIII (F8) or IX (F9) proteins.
(Prevalence
20-30% of patients with severe hemophilia A
1-4% of patients with severe hemophilia B)
National Hemophilia Foundation. (2012). Inhibitor challenges revisited. Available at:

26. Nursing Considerations
Factor replacement to be given on time
Laboratory monitoring
↑ metabolic states will _ factor requirements
Factor coverage for invasive procedures___
Document - infusions, response to treatment
Avoid NSAIDS/ plt aggregation inhibitors
Utilize Hemophilia Center staff for questions / problems

27. Post-operative Nursing Management
(pre-op: monitor administration of factor replacement)
Assess
Monitor
Cold or heat?
Pain management (pharm/non-pharm):
Avoid

28. Nursing Interventions
Teaching
Epistaxis
Apply pressure (to minor wounds if minor factor deficiency)
Hemarthrosis
Other

29. (1)-Treatment Centers (2)-Hemophilia Foundations
(1)-Hemophilia Treatment Centers: Hemophilia Treatment Centers are all across the United States. Below and on the right are three centers in North Carolina with contact information:
East Carolina University East Carolina University Brody School of Medicine PCMH 288 West Greenville, NC > HTC ID: 256 Region: Region IV - North Phone: (252) Fax: (252)
University of North Carolina at Chapel Hill School of Medicine Medical School Wing E, Room 128 CB # 7016 Chapel Hill, NC > HTC ID: 262 Region: Region IV - North Phone: (919) Fax: (919)
Wake Forest University School of Medicine Wake Forest University Health Sciences Department of Pediatrics Medical Center Boulevard Winston-Salem, NC > HTC ID: 251 Region: Region IV - North Phone: (336) Fax: (336)
(2)-Hemophilia Foundations: Hemophilia Foundations are available in almost every state (one in North Carolina) to help those dealing with hemophilia:
Hemophilia of North Carolina P. O. Box 70 Cary, NC Phone: Toll-free phone: Fax: mail to: Website: Matt Barnes, President Sue Cowell, Executive Director

30. What is von Willebrand Disease (vWD)?
Most common inherited bleeding disorder
Inherited: parent to child genetically
1-2% of population affected; affects M & F equally
↓plt adhesion to injured vascular endothelium
↓Factor VIII (F8) [ ] --> ↓ clot stability
↑bleeding time, slightly ↑aPTT
abnormal aggregation tests incl; ristocetin cofactor

31. Detection of vWD Family history Symptoms Hemostatic challenges
Mucosal bleeding* (gums, primarily)
Recurrent epistaxis/ easy bruising
Heavy menorrhagia/ prolonged bleeding from scrapes/ cuts
Significant bleeding post-dental extraction/Heavy bleeding after major surgery or injury
Dx of anemia
Hemostatic challenges
Major Surgery
Dental work
Post Partum
Type 3 vWD (most severe hemostatic challenges)
Laboratory testing: TBD

32. Role of vWF & Hemostasis
von Willebrand factor (vWF)- glue-like protein; plays critical role in both the primary & secondary stages of hemostasis:
During primary hemostasis- vWF helps build the clot by binding platelets together at injury site
During secondary hemostasis- vWF helps stabilize the clot by binding & circulating with factor VIII and protecting it from clearance

33. vWF & primary hemostasis

34. The platelet and its interactions

35. vWF & secondary hemostasis
During secondary hemostasis:
vWF binds with Factor VIII (F8)
vWF then circulates with bound Factor VIII (F8), protecting it from clearance
Action promotes clot stabilization
*FvW* depicted

36. vWD Diagnostic tests Plt count- normal (WHY?) PT- normal aPTT- mild ↑
↓Ristocetin cofactor- measures vWF function
↓vWF antigen- measures plasma vWF level
↓Factor VIII (F8)- clotting activity
(vWF multimers (for type 2)- loss of HMW forms)

37. Diagnostic findings

38. Treatment of vWD
Desmopressin (DDAVP)- releases vWF from endothelium, increases Factor VIII :. helps w clot formation
avoid DDAVP in CAD ->causes plt aggregation:._____
Amicar- Antifibrinolytic (inhibits fibrinolysis-> stabilizes clot)
Cryoprecipitate (replaces vWF, Factor VIII (F8)
(sm risk viral infections, despite screening- monitor)
Estrogen-progesterone WHY?

39. Treatment/ nsg care of vWD
Replace Factor VIII (F8) and vWF with commercial concentrates as necessary/serum level dependent (bleeding crisis/ surgery):
Humate -P®
Alphanate®
Ice/ direct pressure w sterile gauze
Topical hemostatic agents
Biting on teabags (Tannin hemostatic)

40. Nursing Care/ Treatment of vWD
Avoid NSAIDS & meds that interfere________
Avoid full contact sports
Avoid unnecessary surgeries
Prevent bleeding episodes
Teach s/s bleeding & control of bleeding
Support client & family
Encourage people to live as normal of a life as is practical