Understand and describe the pathophysiology of bleeding and clotting disorders.
Describe the clinical manifestations of bleeding and clotting disorders.
Understand and discuss the treatment of bleeding and clotting disorders.
Understand and discuss the nursing care for bleeding and clotting disorders.
Hemochromatosis
Definition
Hemochromatosis is a disorder leading to excess iron accumulation in the body.
Causes
Genetic Defect: Most common cause.
Other Causes: Liver disease, chronic blood transfusions.
Clinical Manifestations
Early Symptoms
Fatigue
Joint pain (arthralgia)
Impotence
Abdominal pain
Weight loss
Late Symptoms
Liver enlargement due to excess iron accumulation
Cirrhosis of the liver
Bronzing of the skin
Heart disease (cardiomyopathy)
Right upper quadrant (RUQ) enlargement
Diagnostics and Treatment
Diagnostics
Increased iron levels (Fe)
Genetic testing
Complications
Liver failure
Cirrhosis
Liver cancer
Heart failure
Treatment
Remove excess iron through:
- Phlebotomy (500 ml/week)
- Iron chelating agents
- Limiting Vitamin C, iron supplements, and iron-rich foods
Polycythemia
Definition
Polycythemia is characterized by the absolute increase in red blood cell (RBC) mass.
Types
Primary Polycythemia
Erythropoietin (EPO) levels are decreased or normal.
Example: Polycythemia vera (all blood cells elevated).
Secondary Polycythemia
EPO levels are increased due to:
- Hypoxia-driven causes (high altitude, cardiopulmonary diseases, defective O2 transport, renal cysts or tumors)
- Hypoxia-independent causes (extrarenal tumors)
Common mutation: Jak-2 mutation.
Complications and Clinical Manifestations
Complications
Stroke due to hyperviscosity and increased risk of thrombus formation.
Splenomegaly and hepatomegaly due to the accumulation of RBCs.
Bleeding disorders due to fragile platelets.
Clinical Manifestations
Hypertension (HTN)
Fatigue
Feeling of fullness
Pruritus: It worsens with a warm bath.
Painful burning and redness of hands and feet
- Elevated hematocrit leading to thick blood (hyperviscosity)
- Risk of thrombus formation
- Overproduction of uric acid.
Diagnostics and Treatment
Diagnostics
Complete Blood Count (CBC) showing all blood cells elevated in primary vs. only RBC elevation in secondary polycythemia.
Bone marrow biopsy.
Treatment
Phlebotomy: Weekly until hematocrit is less than 45%, then every 2-3 months for life.
Medications:
- Aspirin (ASA)
- Hydroxyurea
- Myelosuppressive medication to reduce RBCs and platelets.
Bleeding Disorders
Thrombocytopenia
Definition
Thrombocytopenia is defined as a decrease in platelet counts to below 150,000/uL.
Heparin-Induced Thrombocytopenia (HIT):
- Occurs 5-14 days after initiating heparin therapy.
- Affects 5% of patients on heparin, causing a reduction of platelet count by more than 50%.
If history of HIT exists, the patient should not receive any heparin products again.
Care involves monitoring platelet count during therapy.
Treatment and Nursing Care for HIT
If HIT Occurs
Stop Heparin immediately.
Administer Protamine sulfate as a reversal agent for heparin.
Consider alternative anticoagulation options like Apixaban (direct thrombin inhibitor).
Platelet transfusion is considered based on risk/benefit (usually if platelet count is <10,000 unless active bleeding exists).
Math Example
Heparin Infusion Calculation
Order: Infuse heparin at 18 units/kg/hr.
Heparin bag: 25,000 units in 250 ml of 0.9% NS.
Patient weight: 180 lbs (approximately 81.65 kg). Adjust pump rate based on this calculation.
PTT Level Management
Levels
30-44 sec: 40 units/kg bolus, increase rate by 2 units/kg/hr after 6 hours.
46-65 sec: No bolus, no change.
66-85 sec: No bolus, decrease rate by 1 unit/kg/hr.
>105 sec: No bolus; hold until PTT <90 sec.
Hemophilia
Definition
Hemophilia is an X-linked recessive disorder passed from mother to son, resulting in deficiency of clotting factors.
Types:
- Hemophilia A: Factor VIII deficiency (factor VIII is produced in the liver).
Clinical Manifestations and Complications
Bleeding Symptoms
Any bleeding episode can be life-threatening.
Acute, prolonged, or delayed bleeding may occur hours to days after an injury.
Complications
Hemorrhage
Hemarthrosis (bleeding into joint spaces)
Nerve compression leading to pain and paralysis from distal bleeding.
Diagnostics
Deficiency of Factor VIII in blood tests.
Prolonged activated Partial Thromboplastin Time (aPTT).
Normal platelet count and prothrombin time (PT).
Treatment and Nursing Care
Prevention Strategies
Recommend non-contact sports and use of soft-bristled toothbrushes.
Use gloves during household chores.
Treat minor bleeding episodes for at least 72 hours.
DDAVP (desmopressin acetate):
- Synthetic analog of vasopressin, can be administered IV, SC, or intranasally.
- Stimulates release of factor VIII; effects evident within 30 mins and may last over 12 hours.
- Possible need for repeated doses.
- Patient should wear a Medic Alert bracelet.
Acute Care Strategies
Stop bleeding via direct pressure and topical thrombin agents.
Volume replacement with PRBC transfusions and IV fluids (IVFs).
Administer DDAVP IV – effects noted within 30 minutes and lasting for about 12 hours.
Follow RICE protocol for management of joint swelling: Rest, Ice, Compress, Elevate.
- Avoid NSAIDs.
Disseminated Intravascular Coagulation (DIC)
Definition
DIC is not a distinct disease but a complication caused by an underlying disease or condition. It results in serious bleeding and thrombotic disorders due to abnormally initiated and accelerated clotting.
Risk Factors
Trauma
Sepsis
Pregnancy
Pathophysiology
Triggering of abnormal response of the clotting cascade, causing excessive fibrin and platelet aggregation that leads to thrombosis and subsequent depletion of clotting factors, resulting in bleeding.
Events Leading to Thrombosis and Bleeding
Thrombotic Events
Activation of coagulation cascade leading to thrombotic occlusion of microcirculation across all organs.
Fibrinolysis in the microcirculation leads to circulating fibrin degradation products.
Bleeding Events
Consumption of platelets and coagulation proteins.
Signs of thrombosis can include delirium, coma (neurological), ischemia and gangrene (integumentary), oliguria and azotemia (renal), and ECG changes (cardiovascular).
Clinical Manifestations
Bleeding Signs: Petechiae, purpura, ecchymosis, bleeding from venipuncture sites, increased respiratory rate and shortness of air, hemoptysis, decreased blood pressure, increased heart rate, GI bleeding, GU bleeding.
Thrombotic Signs: Increased respiratory rate and dyspnea, ischemia, cyanosis, tissue necrosis, oliguria, and venous distention.
Diagnostics and Treatment
Diagnostics
Prolonged PT, PTT, and aPTT.
Decreased platelet count and fibrinogen levels.
D-dimer blood tests to identify blood clotting issues.
Treatment
Provide oxygenation and volume replacement with Fresh Frozen Plasma (FFP) and platelets if there is significant bleeding or prolonged bleeding time.
Critical to treat the underlying cause, as DIC is a complication rather than a standalone disease.
Learning Checks
Scenario 1: Thrombocytopenia
Which lab value is consistent with diagnosis: B. Platelet count of 120,000.
Scenario 2: Hemophilia
Prioritize nursing intervention for a client with severe hemarthrosis: C. Rest the affected extremity.