mod 4 Flashcards
Anemia
hemoglobin is less than 10 or 11 g/dl
Iron-Deficiency Anemia
Predictable at developmental periods
In premature infants, due to low fetal supply
At 12 to 36 months, due to ingestion of large amounts of cow’s milk and diet
In adolescents, due to rapid growth and poor eating habits
Sickle Cell Crisis
Precipitating factors
Anything that increases the body’s need for oxygen or alters the transport of oxygen
Trauma
Fever, infection
Physical and emotional stress
Increased blood viscosity due to dehydration
Hypoxia
Types
Vasoocclusive (VOC) thrombotic
Splenic sequestration
Aplastic crises
Sickle Cell Vasoocclusive (VOC) thrombotic
Vasoocclusive (VOC) thrombotic
Most common type of crisis and is very painful
Stasis of blood with clumping of cells in the microcirculation leads to ischemia and then infarction
Signs are fever, pain, and tissue engorgement
Sickle Cell Splenic sequestration
Life-threatening type; death can occur within hours
Blood pools in the spleen
Signs are profound anemia, hypovolemia, and shock
Sickle Cell Aplastic crises
Diminished production and increased destruction of RBCs
Triggered by viral infection or depletion of folic acid
Signs include profound anemia and pallor
Diagnosis of Sickle Cell Anemia
Cord blood in newborns
Newborn screening
Genetic testing to identify carriers and children who have the disease
Sickle turbidity test
Quick screening in children over 6 months of age
Hemoglobin electrophoresis
Thalassemia (Cooley Anemia)
Anemia results from defective synthesis of hemoglobin, structurally impaired RBCs, and a shortened life of RBCs
Inherited blood disorders of hemoglobin synthesis
Classified by the hemoglobin chain affected and by the amount of effect
Autosomal recessive disorder with varying expressivity
Both parents must be carriers to have offspring with the disease
RBC changes are often seen by 6 weeks of age
beta-Thalassemia
Four types
Thalassemia minor: Asymptomatic silent carrier
Thalassemia trait: Mild microcytic anemia
Thalassemia intermediate: Moderate to severe anemia plus splenomegaly
Thalassemia major (Cooley anemia): Severe anemia requiring transfusions for survival
Greeks, Italians, and Syrians
alpha-Thalassemia
Occurs in Chinese, Thai, African, and Mediterranean people
Medical Management of Thalassemia
Blood transfusion to maintain normal hemoglobin levels
Side effect is hemosiderosis
Treat with iron-chelating drugs such as deferoxamine (Desferal)
Binds excess iron for excretion by the kidney
Aplastic Anemia
Aplastic Anemia
In hypoplastic anemia, there is profound depression of RBCs but normal levels of white blood cells (WBCs) and platelets
idiopathic
Hemophilia A
Classic hemophilia (deficiency of factor VIII) Accounts for 80% of cases of hemophilia
Hemophilia B
Christmas disease (deficiency of factor IX)
von Willebrand disease (vWD)
Deficiency, abnormality, or absence of vWF and factor VIII
Affects both males and females
Etiology of Hemophilia A
Males are affected
Females may be carriers
Manifestations of Hemophilia
Bleeding tendencies range from mild to severe
Symptoms may not occur until 6 months of age
Hemarthrosis
Bleeding into joint spaces of the knee, ankle, or elbow leads to impaired mobility and, eventually, bony changes and disability
Symptoms include warmth, pain, bruising, and decreased movement
Epistasis
Bleeding in the gastrointestinal tract
Hemophilia Diagnostic
diagnosed through amniocentesis
Genetic testing of family members
low levels of factor VIII or IX and a prolonged partial thromboplastin time (PTT)
Platelet count, prothrombin time (PT), and fibrinogen levels are normal
Management of Hemophilia
Desmopressin (DDAVP)
IV
Increases factor VIII activity by two to four times
Used for mild hemophilia
Interventions for Hemophilia
Shave only with an electric razor
For superficial bleeding, apply pressure for at least 15 minutes and ice to promote vasoconstriction
If significant bleeding occurs, transfusion for factor replacement
Managing Hemarthrosis
During bleeding episodes, elevate and immobilize the joint
Range-of-motion exercises after the bleeding stops will help to prevent contractures
Avoid obesity to minimize joint stress
Idiopathic Thrombocytopenic Purpura
An acquired hemorrhagic disorder characterized by
Thrombocytopenia (excessive destruction of platelets)
Purpura (discoloration caused by petechiae beneath the skin, with no other signs of bleeding)
NORMAL bone marrow
Occurs in two forms
Acute, self-limiting (usually following a viral illness)
Chronic (lasting longer than 12 months)
Diagnostic evaluation
Platelet count is less than 20,200/mm
Rule out other diseases and conditions
Wiskott-Aldrich Syndrome
X-linked recessive disorder with triad of
Thrombocytopenia (usually at birth)
Eczema
Immunodeficiency of select B and T lymphocytes
Blood transfusions
Monitor vital signs
Baseline, initially; 15 minutes after initiation and completion
Use an appropriate filter and saline flush
Use blood within 30 minutes of arrival
Infuse a full unit over 4 hours maximum