Flashcards in Chapter 40 Hematologic Power Point Deck (82)
Describe Sickle Cell Disease (Anemia)
-Genetic disorder with autosomal recessive pattern
-Formation of abnormal hemoglobin
--Healthy adults with normal hemoglobin have two alpha and two beta chains
--Sickle cell disease hemoglobin contains abnormal beta chains
-Most common in African Americans
What is the primary symptom of Sickle Cell Disease?
What are some other symptoms of Sickle Cell Disease?
Hart murmurs (S3 heart sound)
--Cyanosis around lips
--Roof of the mouth and sclera (African Americans)
Ulcers on legs
Damage to spleen and liver
--Enlarged lumpy liver
What are some other symptoms of Sickle Cell Disease?
Decreased urine output
--Chronic kidney disease
--due to vessel occlusions.
--Look for joint swelling and temp change
--Low grade fever
--Seizures or stroke symptoms (due to frequent hypoxia
What are some interventions you can provide for Sickle Cell Disease? (Chart 40-2)
Pain-hospitalizations/ opioids(no dependency problems)
Hydroxyurea (Droxia) increases # of HbF (may lead to leukemia)
Warm room(because they have problems with O2, they are colder)
What are some more interventions we can provide for Sickle Cell Disease? (Chart 40-3)
Prevent sepsis and multi organ dysfunction
-Hand washing and aseptic technique
-Flu shot and pneumonia shot
-Respiratory problems –wear a mask
-Assessment q 8hrs: skin, mucous membranes, lungs (crackles, wheezes), urine output, antibiotics
-HSCT (Hematopoietic Stem Cell Transfusion aka bone marrow transfusion-costly, many side effects)
Describe Glucose-6-Phosphate Dehydrogenase Deficiency Anemia.
-X link recessive disorder
-Missing enzyme which leads to destruction of RBCs
-Triggered by infection or drugs (aspirin, diuretics, high doses of vit C)
-Symptoms: Dark urine, enlarged spleen, pallor, SOB
How can you prevent G6PD deficiency?
-Prevention: Genetic testing
--Prevent infection (triggering agents)
-Hydrate: Prevent hemoglobin collection in kidneys and Acute kidney injury
--Mannitol- osmotic diuretic helps prevent AKI
Describe Immunohemolytic Anemia
-Abnormal immunity that results in destruction of RBC membranes
-May be due to trauma, infections, drug exposure
-Warm antibody- excess of IgG
-Cold antibody-IgM protein fixation
--Arteries in hands and feet constrict in response to cold or stress
--Therapy: Steroids; Splenectomy; Plasma exchange
Describe an Anemia due to an Iron deficiency.
-May result from blood loss, poor GI absorption or inadequate diet
-Evaluate for abnormal bleeding
-Increase Iron rich food, Iron supplement, Parenteral supplement
What are some manifestations related to an iron deficiency anemia?
-Manifestations: pallor, weakness, fatigue, fissures in corners of mouth, exercise intolerance
Describe a b12 deficiency
Failure to absorb Vitamin B12
Slow onset (vegan diets- lack of dairy products)
What are some manifestations of B12 deficiency?
Manifestations: pallor, jaundice, glossitis, fatigue, weight loss, paresthesia of feet and hands
What are some interventions for B12 deficiency?
Increase dietary intake
Vitamin supplements : B12 shots
B 12 shots are given once a week at first and once a month later as a maintenance ( therapy for life)
What is a Folic Acid Deficiency?
Caused by poor nutrition, drugs, malabsorption, Crohns’ disease
Management: diet and scheduled replacements
Describe Aplastic Anemia?
Bone Marrow failure to produce enough RBCs
Unknown cause ( exposure to toxic agents, viral infections)
What are some interventions for Aplastic Anemia?
-Bone marrow biopsy-replacement of cell forming marrow with fat
---Hematopoietic stem cell transplant
What is polycythemia vera?
-Cancer of RBCs
--Massive production of RBCs
--Massive production of Leucocytes
--Massive production of platelets
What is the assessment you do for polycythemia vera?
Assessment : cyanotic, flushed, itching,
Poor perfusion (blood flow may actually stop)
Increased demand on heart (hypertension)
What are some interventions for polycythemia vera?
Phlebotomy with apheresis
Increase hydration and promote venous return (chart 40-4 p805)
Describe hereditary hemochromatosis.
Autosomal recessive disorder
Excessive absorption of Iron leading to organ failure
What are some manifestations of hereditary hemochromatosis?
Manifestations: abd pain, enlarged liver, hyperglycemia, darkening of the skin
Later signs: diabetes, liver cirrhosis, endocrine gland failure, heart disease & death
What is an intervention for hereditary hemochromatosis?
What is Myelodysplastic Syndrome (MDS)?
Abnormal cell formation in the bone marrow
Cells have short life (pancytopenia)
Unknown cause (risk factors: smoking, aging, radiation/chemo therapy)
What si the therapy for Myelodysplastic Syndrome (MDS)?
-Therapy: hematopoetic stem cell transplant
Medication takes 3-6 months to work so supportive therapy is necessary- transfusion and erythropoiesis stimulating medications
White blood cell disorder
Uncontrolled production of immature white blood cells (WBC)
Acute or Chronic
What are some patient considerations of leukemia? (Chart 40-5)
-Cardiovascular: increased heart rate, low BP, murmurs, bruits, slow capillary refill
-Respiratory: increased respiration rate
-Skin: pale & cool, petechiae
-GI : weight loss, nausea, anorexia, decreased bowel sounds, constipation, abdominal pain and enlarged liver
What are some labs you can perform with Leukemia?
What is an intervention for leukemia?
Prevent infection: frequent thorough hand washing, Aseptic technique, respiratory mask, personal hygiene, fever, urine, VS, (q8hrs)-inspect mouth, chest