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