Thalassemia
What lab value becomes elevated as leukemia progresses?
WBC (bonus, if >100,000 can block circulation, aka leukostasis, - life threatening)
How does a patient get sickle cell and what population is it more prevalent in?
Inherited, autosomal recessive disorder (genetic link). More common in African ethnicities.
What are risk factors for iron-deficiency anemia? (name at least 2)
Poor diet intake (including vegetarians), women in reproductive years, blood loss, surgeries that alter surface area of intestine
What type of solution is given with blood via y tubing?
Normal Saline/0.9% Sodium Chloride
What should you do first if you suspect a reaction to the blood is occurring?
Stop the transfusion
What is the normal range for platelets?
150,000 - 400,000
What should definitely NOT be given with thalassemia?
Iron supplements
How is DIC treated?
Treat underlying cause mainly. Monitor for and control bleeding. If hemorrhaging, may need blood products.
Define leukemia and what happens in simple terms.
Group of cancers affecting the blood and blood-forming tissues of the bone marrow, lymph system, and spleen. Overproduction of immature or abnormal WBC occurs.
What is a common trigger for a sickle cell episode/crisis?
Low O2 tension in blood (which can be caused by infection, stress, surgery, blood loss).
Dehydration
Define hemophilia.
X-linked recessive genetic d/o caused by a defective or deficient coagulation factor (Bonus - factor VIII deficiency in hemophilia A).
Define thrombocytopenia.
Reduction of platelets below 150,000
What is aplastic anemia and how is it treated?
Decrease in all blood cell types - RBCs, WBCs, platelets (pancytopenia) often due to autoimmune causes. Immune therapies and bone marrow transplantation (HSCT)
How long should you stay with your patient you've just started giving blood to?
At least first 15 minutes to monitor for reaction
A nurse suspects a transfusion reaction so they stop the transfusion, maintain IV patency with NS, notified the blood bank and HCP, monitored VS and urine output, and disposed of the blood bag and tubing. What did they do wrong?
They should send the blood bag and tubing to the lab for testing.
How is thalassemia major treated?
Blood transfusions (or exchange transfusions) with chelating agents that bind with iron.
What some clinical manifestations of DIC?
Signs of bleeding in the skin, respiratory and cardiovascular systems, GI and urinary tracts, neurologic and musculoskeletal systems - i.e. petechiae, hemoptysis, abdominal distension, bloody stools, hematuria, etc. Thrombotic signs in the skin, respiratory and cardiovascular systems, GI tract, kidneys - i.e. PE, tissue/organ ischemia w/ pain, etc.
How would a symptomatic patient be treated vs a asymptomatic patient?
Symptomatic - initial goal is to obtain remission. Chemotherapy mainly, can use radiation and biological therapies as well.
Asymptomatic - Watchful waiting with active support.
What are clinical manifestations of non-crisis sickle cell and sickle cell crisis?
Non-crisis: Anemic but asymptomatic except during crisis. Pallor and jaundice. During crisis - severe pain (r/t ischemia of tissue) often accompanied by other sx, such as fever, swelling, tachypnea, HTN, N/V.
What are some clinical manifestations of hemophilia?
Related to bleeding. Slow, persistent, prolonged bleeding after minor injury. Delayed bleeding after minor injury. Epistaxis, GI bleeding, hematuria, bruising, hemarthrosis (which may lead to joint deformity)
What are some considerations when taking iron supplements? (at least 2 points)
Enteric-coated or sustained-release tablets are ineffective. Take an hour before meals with vitamin C to enhance absorption. Black stool may occur. Constipation is likely - increase fiber, stool softeners and laxatives if needed.
You've just picked up the blood from the blood bank. How much time do you have to start administering it?
30 minutes
A patient receiving a blood transfusion develops the following symptoms: Fever, severe back pain, acute jaundice, tachycardia, tachypnea, and oliguria. What type of reaction is it?
Acute Hemolytic Reaction
What some general symptoms of anemia?
Fatigue, pallor, dizziness, cold intolerance, lethargy, tachycardia, tachypnea
What are some symptoms of thalassemia major?
General symptoms of anemia. Can cause growth and developmental deficits. Jaundice, splenomegaly, hepatomegaly, cardiomyopathy.
What is the etiology of DIC?
Abnormally initiated and accelerated clotting. Decreases in clotting factors and platelets ensue, causing increased bleeding. Always caused by an underlying disease or condition (such as some cancers).
What are some clinical manifestations of leukemia?
Bone marrow failure → sxs of anemia, thrombocytopenia, and decreased WBC number and function. Infiltration of abnormal WBC can lead to splenomegaly, hepatomegaly, lymphadenopathy, bone pain, meningeal irritation, and oral lesions.
What is the priority in treating a sickle cell crisis? What are other interventions?
PAIN MANAGEMENT (often continuous opioids). O2 therapy. DVT prophylaxis. Increased fluids (3-4L). Transfusion indicated with aplastic crisis. Infection prevention.
What are some patient teachings with thrombocytopenia? (give at least 2)
Encourage patients to seek prompt treatment for any symptoms of bleeding. Avoid blood thinners. Use electric razor. Do not blow nose forcefully. Do not puncture skin (tattoos or piercings) - (this includes no IM injections!!). Use soft bristle toothbrushes.
What are risk factors for folic acid anemia and how is it treated?
Risk Factors: Malnutrition, malabsorption, drugs, alcohol abuse, loss during hemodialysis
Treatment: Replacement therapy (usual dose is 1mg per day)
What size catheter should you use for blood transfusion? (___ and larger)
22 G or larger
A transfusion was given at a rate faster than the patient's body could handle. The patient began coughing, was short of breath, JVD was present, and crackles were heard in the lungs. How should the nurse treat this?
Upright position w/ feet dependent. STAT CXR. Diuretics, O2, morphine.
Name 3 foods that are high in iron.
Lean beef, turkey, pork, chicken, and fish. Legumes, beans, dark green leafy vegetables, whole grain and enriched breads and cereals.
How does thalassemia minor occur vs thalassemia major?
Minor - heterozygous inheritance (only 1 abnormal gene). Major - homozygous (2 abnormal genes)
What lab values are expected with DIC patients?
Elevated D-dimer, decreased platelets, decreased fibrinogen, clotting times prolonged
Classifications of leukemia: What does acute vs chronic mean, and what does myelogenous vs lymphocytic mean?
Acute = affecting immature cells and rapid onset; Chronic = affecting mature WBC and gradual onset. Myelogenous = from myeloblasts/granulocytes; Lymphocytic = from lymphocytes.
Bonus - Acute lymphocytic is the most common type in children
What are some teachings that can be done to prevent triggering a crisis?
Avoid high altitudes (low O2). Avoid overexertion. Infection prevention (i.e. avoid large crowds). Increase fluid intake.
A patient with hemophilia A has fallen and sustained a superficial laceration to their knee. Their bleeding is not slowing down, and they also report pain and swelling in their joint following the injury. How would you treat them?
Direct pressure or ice on wound. Administer clotting factor. With joint bleeding, R.I.C.E.
What is cobalamin (vitamin B12) deficiency most commonly caused by, what ancestry is commonly affected, and how is it treated?
Pernicious anemia; Northern European (notably Scandinavians) and blacks; parenteral or intranasal administration of cobalamin
Blood transfusions have to be completed within ____.
4 hrs
A patient receiving a blood transfusion develops the following symptoms: chills, rigor, high fever, vomiting, diarrhea, marked hypotension, and shock. What type of reaction is it and how would it be managed?
Sepsis; Blood culture. ABx IV fluids, vasopressors.
A patient with Hodgkin's lymphoma has lymph node involvement above and below the diaphragm. They present with weight loss, fatigue, fever, tachycardia, and night sweats. How would this be staged?
Stage III B