Cyndi - Week 12 - Exam 6 Flashcards
what are the four types of hematologic disorders?
- problems with RBCs
- problems with bone marrow
- problems with platelets
- problems with clotting cascade
what are the 3 problems with RBCs?
- Sickle Cell Disease
- Hemophilia
- Polycythemia Vera
what are the 2 problems with bone marrow?
- Aplastic Anemia
* Multiple myeloma
what is the one problem with platelets?
• Immune Thrombocytopenic Purpura (ITP) - brusing - small platelet cells
what is the one problem with the clotting cascade?
• Disseminated Intravascular Clotting (DIC) - idopathic
what are the basic diagnostics for hematological disorders?
- labs
- CT of liver, spleen, lymph nodes
- biopsies of bone marrow and lymph nodes (cancers → swelling in nodes)
what are the labs for hematological disorders?
- CBC
- Blood type
- Iron studies - Iron, TIBC (total iron binding capacity), Ferritin
- Coagulation, clotting studies
- Blood smear
what are the characteristics of a bone marrow biopsy?
Sterile procedure
• Bone marrow needle is inserted into the cortex of bone
• Severe pain by the patient during the aspiration
• Direct pressure to site after needle is removed
• May have the patient lie on the site if possible for 1 hour to compress
• Homeostasis is the goal
what are the preferred site for a bone marrow biopsy?
• Preferred sites ‐ Posterior or anterior iliac crest, sternum
what are the complications of bone marrow biopsies?
- Hemorrhage and infection are rare
- Position patient on side 30‐60 min.
- Tenderness can last for 3‐4days
- Analgesics
TEST: what nursing intervention can help with hemorrhaging after a bone marrow biopsy?
put the pt on that side in order to put pressure on the wound
what is sickle cell disease?
inherited autosomal disorder
- causes RBCs to “sickle”
- More than 2 million Americans have SCT ‐ (1/12 have SCT)
- 1 in 500 African American births SCD
- 1 in 36,000 Hispanic American births
is sickle cell disease a recessive or a dominant trait?
RECESSIVE!
how is sickle cell disease diagnosed?
• labs - CBC, peripheral blood smear, hgb electrophoresis
• newborn screening
***pt may be interested in genetic counseling if trying to start a family
what is the main issues with sickle cell disease?
main issue is ORGAN HYPOXIA
- reversible in the early stages with oxygen
- blockage of perfusion
- self-perpetuating hypoxia
- more sickling of RBCs
- triggers (infection, dehydration, stress, pregnancy) causes exacerbations
- person lives anemic
what are the crises of sickle cell disease?
- severe exacerbation
- painful - ischemia occurring in multiple areas
- vasospasms
- can lead to organ damage, MODS (multiorgan dysfunction syndrome), and death
TEST: when sickled cells in the system need to be cleaned up by the spleen, they begin to lyse because they can’t keep up, what will the pt look like?
JAUNDICE
what are the clinical manifestations of sickle cell disease?
• Pain • Fever • Swelling • Tenderness • Tachypnea • Hypertension • Nausea, vomiting • Signs of organ perfusion problems (↓ U.O., pain, altered LOC, respiratory distress, chest pain, hand or foot pain, etc.)
what is the tx for sickle cell disease?
maximize oxygenation, monitor organ perfusion, minimize complications
• O2 and rest
• Treat pain – usually opiates
• Lots of IVF – flush out occlusive areas of cells
• Blood transfusion
• Infection – prevent, avoid triggers, and treat asap
– Get immunizations
– Prophylactic antibiotics may be used
• Medications ‐ hydroxyurea
• Hematopoietic Stem Cell Transplant (HSCT)
• Genetic counseling
what is hematopoietic stem cell transplant (HSCT)?
- the only curative treatment for Sickle Cell Disease
- Sources of stem cells: bone marrow , peripheral, or cord blood
what are the risks of hematopoietic stem cell transplant (HSCT)?
- infection
- graft vs host disease
- immune suppression
what are the benefits of hematopoietic stem cell transplant (HSCT)?
- CURE!
- prevention of organ damage
what are the two types of dones for hematopoietic stem cell transplant (HSCT)?
- allogeneic - someone else is the donor
• Matched sibling donors have the best outcome
• Families members should be encouraged to have cord blood collected and stored - autologous - the pt him/herself is the donor
• Healthy bone marrow taken from pt before marrow ablation
• Rate of rejection very low and immune system response much quicker
T/F All patients with SCD should be evaluated for HSCT
TRUE
• Severity of disease can be underestimated – damage is occurring
• Allogeneic HSCT is not only curative in SCD, but abates progressive organ
dysfunction, including CNS complications
what is aplastic anemia?
pancytopenia - marrow stops producing blood cells
what are the causes of aplastic anemia?
- congenital or acquired
what are the clinical manifestations of aplastic anemia?
- Abrupt or insidious onset: fatigue, pallor, tachycardia, tachypnea, bleeding
- S/s of suppression of blood cells ‐ infection
what are the diagnostic tests for aplastic anemia?
- Labs ‐ CBC, iron, ferritin studies
- Bone marrow aspiration/biopsy
- ↑ yellow marrow, hypo cellular
what is the tx for aplastic anemia?
- ID and remove causative factor if possible
- Supportive care (fluids, O2, blood transplant)
- Bone marrow stimulant meds – Filgrastim, Epoetin - helps body create RBCs
- HSCT – bone marrow transplant
- Immunosuppressive therapy possible
- Corticosteroids
what are the complications of aplastic anemia?
- infection
- bleeding
- hypoxic organ damage (RBC carry o2 → ↓ RBC → necrosis)
what is hemophilia?
inherited disorder of clotting: X-link recessive
Hemophilia A+B transmitted by ______
female
• 50% of sons have the disease
• 50% of daughters are carriers of disease
• Daughters with the disease usually have a milder case
hemophilia requires a defective or deficient _____ -_____
coagulation factor
what are the 3 different coagulation factors that can be defective or deficient?
- A: 85‐90% ‐ missing Factor VIII
- B: 10‐15% ‐ missing Factor IX
- von Willebrand – missing von Willebrand factor
what is the most common congenital bleeding disorder??
von Willebrand
what are the sxs of hemophilia?
Bleeding: • Slow persistent oozing • Delayed bleeding • Uncontrolled hemorrhage • Bleeding in GI tract (dark tarry stools), nose, kidneys, brain, joints, or skin
what are the diagnostic tests for hemophilia?
- Clotting Factors VIII, IX, X, XII, vWF
* Platelets, PT, PTT, Thrombin, Bleeding time
what are the complications of hemophilia?
allergic or transfusion related reactions, clotting
problems, acquiring disease from blood transfusion
what is the treatment for hemophilia?
Prevent and treat bleeding, prevent complications
what is acute intervention for any bleeding in hemophilia pts?
- First aid for minor cuts
- Replace deficient clotting factors during a bleeding episode
- If joint bleeding occurs, pack it in ice and rest it
what is the preferred tx for hemophilia patients?
Factor Replacement Therapy • Plasma derived or recombinant products • DDAVP – Vasopressin – increases VIII, and vWF • Prophylaxis vs “on demand” • Decreased use of FFP nowadays • Antifibrinolytic therapy • Monoclonal antibodies
what is multiple myeloma?
malignant cells infiltrate bone marrow and destroy bone
what is the cause of multiple myeloma?
unknown cause, may be exposure to chemicals, radiation, or genetic factors
what is the pathophys of mulitiple myeloma?
- excess plasma B cells - produce abnormal antibodies (ineffective, don’t fight infection, infiltrate the bone marrow, show up in the urine (bence-jones proteins)
- excess cytokine production destroys bone as well
- develops slowly (usually not diagnosed until disease is advanced)
- bone destruction → calcium is released → worried about kidneys
TP: what are the simple nursing interventions that will help the kidneys in multiple myeloma?
- hydrate to make sure to flush the kidneys
- ambulate to use the bone
what are the clinical manifestations of multiple myeloma?
• skeletal pain • bone degeneration - osteoporosis - pathological fractures - hypercalcemia (bone loss) - renal, GI, and neuro manifestations
what are the diagnostics for multiple myeloma?
- labs (pancytopenia, ↑ Ca2+)
- radiological studies
- bone marrow biopsy
what is the disease tx for multiple myeloma?
- watchful watching
- corticosteroids
- chemotherapy
- targeted therapy
- HSCT
what is the symptom treatment for multiple myeloma?
- hypercalcemia (fluids and ambulation)
- analgesics, radiation therapy, skeletal support)
T/F Multiple myeloma is seldom cured , but treatment can relieve sxs, produce remission, and prolong life-preventing complications a major focus
TRUE
what are the complications of multiple myeloma?
- uric acid accumulation (gout)
- renal tubular necrosis
- pathological fractures
- pain and peripheral neuropathy
- infection (d/t ↓ antibodies TP: shouldn’t be around )
- depression and psychological stress
- death
what is disseminated intravascular coagulation?
not a disease, a physiological response to disease, infection, or insult
- abnormally initiated and accelerated clotting
- after clotting factors depleted, unstoppable bleeding
- can be acute or chronic situation
what risk factors for disseminated intravascular coagulation?
sepsis, shock, birth, cancer, burns, etc
what are the CLOTTING manifestations of disseminated intravascular coagulation?
cyanosis, hemorrhagic necrosis, ↓ urine output, sxs of PE, MI, DVT, stroke
what are the BLEEDING manifestations of disseminated intravascular coagulation?
petechiae, purpura, pallor, oozing blood through skin and orifices, hematoma, sxs of hypovolemia, visual changes, joint pian, hematuria, rectal bleeding, ALOC
TP: what is the hallmark sxs of DIC?
bleeding without a reason
what are the diagnostic tests for DIC?
- PT, INR, PTT
- thrombin time
- fibrin split products (FSP)
- fibrinogen
- platelets
- D-Dimer level TP
what are the complications of DIC?
organ ischemia or infarction, hemorrhage, ARDS, multiple organ dysfunction syndrome (MODS), death
what is the tx for DIC?
• frequent assessments - early diagnosis
• supportive care (blood products, plts, cryoprecipitate, FFP)
• anticoags (IV heparin)
- FIX the underlying cause (sepsis? ATB)
