Chapter 37 Care of pt w/ Hematologic Problems Flashcards
Exam 2
anemia
a reduction in the number of red blood cells (RBCs), the amount of hemoglobin, or the hematocrit (percentage of packed RBCs per deciliter of blood).
apheresis
the withdrawal of whole blood and removal of some of the patient’s blood components followed by reinfusion of the plasma back into the patient
blast phase cells
immature white cells that are dividing rapidly
engraftment
the successful “take” of the stem cells transplanted into the recipient
erythrocytes
red blood cells (RBCs)
glottitis
a smooth, beefy-red tongue
Hemoglobin A (HbA)
normal adult hemoglobin with two normal A chains and two normal B chains
Hemoglobin F (HbF)
the main type of hemoglobin in the fetus, having two normal A chains and two normal gamma chains that bind oxygen more tightly than does hemoglobin A or S
Hemoglobin S (HbS)
the hemoglobin of sickle cell disease in which there are two normal A chains and two abnormal beta chains that fold poorly, causing the red blood cell to assume a sickle shape under low-oxygen conditions
Hemolytic
blood destroying
hypercellularity
cellular excess in the peripheral blood
indolent
slow growing or slow to progress
intrinsic factor
a substance normally secreted by the gastric mucosa that is needed for intestinal absorption of Vitamin B12
leukemia
blood cancer that results from a loss of normal cellular regulation, leading to uncontrolled production of immature WBCs (“blast” cells) in the bone marrow
leukocytes
white blood cells (WBCs)
leukopenia
reduction in the circulating number of white blood cells (WBCs)
lymphomas
cancers of the lymphoid cells and tissues with loss of cellular regulation and abnormal overgrowth of lymphocytes
malignant
cancerous
multiple myeloma (MM)
a white blood cell cancer of mature-B lymphocytes called plasma cells that secrete antibodies
nadir
the period after chemotherapy in which bone marrow suppression is the most severe
pancytopenia
a condition of low circulating numbers of all blood cell types
perfusion
adequate arterial blood flow through the tissues (peripheral perfusion) and blood that is pumped by the heart (central perfusion) to oxygenate body tissues
m
peripheral blood stem cells (PBSCs)
stem cells that have been released from the bone marrow and circulate within the peripheral blood
pernicious anemia
anemia resulting from failure to absorb Vitamin B12, caused by a deficiency of intrinsic factor (a substance normally secreted by the gastric mucosa), which is needed for intestinal absorption of Vitamin B12
Philadelphia Chromosome
an abnormal chromosome often associated with chronic myelogenous leukemia caused by a translocation of the ABL gene from chromosome 9 onto the BCR gene of chromesome 22
Polycythemia Vera (PV)
one of the chronic myeloproliferative neoplasms (MPNs) in which there is loss of cellular regulation and excessive proliferation of specific groups of abnormal myeloid cells that have decreased function
SCD crisis
episodes of extensive cellular sickling that obstruct perfusion, causing tissue hypoxia and severe pain
Sickle Cell Disease (SCD)
a genetic disorder in which a mutation in the gene for the beta chains of hemoglobin causes chronic anemia, pain, disability, organ damage, increased risk for infection, and early death as a result of poor blood perfusion
Stomatitis
mouth sores
Teratogen
an agent that can cause birth defects
Thrombocytopenia
a reduction in the number of circulating platelets from reduced platelet production
Thrombocytopenia Purpura
the destructive reduction of circulating platelets after normal platelets production
hematologic
of or relating to blood; the dx, treatment, and prevention of disease of the blood and bone marrow as well as the immunolgic, hemostatic (blood clotting) and vascular systems
What is the function of RBCs in the body?
they carry oxygen to tissue which helps with perfusion
RBC disorders are problems that…
impair production of RBCs (either too little or too much), impaired function (for instance with SCD not enough functional Hgb to carry enough O2), and/ or abnormal destruction of RBCs
Conditions that cause low blood oxygen/ sickling
- hypoxia
- dehydration
- infection
- venous stasis
- pregnancy
- alcohol consumption
- high altitudes
- low or high environmental or body temperatures
- acidosis
- strenuous exercise
- emotional stress
- anesthesia
is when red blood cells, which are usually round and flexible, become hard and shaped like a crescent or a “sickle”; this happens because of a change in the hemoglobin, the protein inside red blood cells that carries oxygen; a change in the hemoglobin causes RBCs to sickle
sickle cell disease
Cells that do not move through blood vessels easily
sickle-shaped cells
characteristics of sickled cells
- become hard and shaped like a crescent moon or a “sickle”
- don’t move through blood vessels easily
- get stuck and block blood flow
- break apart faster than normal cells
Sickled cells breaking apart and blocking blood flow leads to what?
leads to problems like pain, tiredness, and damage to organs because the body isn’t getting enough oxygen
A clumped masses of sickled RBCs block blood flow and perfusion is known as what?
vaso-occlusive event (VOE)
VOC
(sudden onset) weekly or even yearly, conditions causing local or systemic hypoxemia; can refer to the overall crisis involving any issues arising from the blockage of blood flow
VOE
results in impaired perfusion with long term effects to tissues and organs (organs most affected spleen, liver, heart, kidney, brain, joints, bones and retina); typically emphasizes the painful experience of sickle cell patients during an episode
VOE/ VOC
in practice- the terms are used interchangeably
:is the protein in red blood cells that carries oxygen throughout the body
hemoglobin
when oxygen levels are low, HbS causes red blood cells to change shape from their normal round form to a rigid, sickle shape; these sickle-shaped cells do not flow well through blood vessels, leading to blockages and various complications
the sickling of red blood cells
genetic risks for SCD
- family history
- ethnicity
Who is at a higher risk of having children with SCD?
individuals with a famliy history of SCD or sickle cell trait
SCD is more prevalent in which populations?
- individuals of African descent
- people from the Mediterranean region
- individuals from parts of the Middle East and India
SCD is inherited in what manner?
autosomal recessive
What is autosomal recessive?
a person must inherit 2 copies of the mutated gene (one from each parent) to have the disease
What is an individual considered if they inherit only one copy of the mutated gene but usually do not experience symptoms ?
a carrier (having sickle cell trait)
What is carrier status?
if both parents are carriers of the sickle cell trait, there is a 25% change with each pregnancy that their child will inherit two copies of the mutated gene and have sickle cell disease; a 50% chance that the child will be a carrier; and a 25% chance that the child will have normal hemoglobin
There are approximately how many people living with SCD in the US?
100,000
Repeated VOEs cause what to tissues and organs, especially the spleen?
long-term damage
the spleen acts as a what for your blood?
filter
what does spleen do?
filters the blood; recognizes & removes old, malformed, or damaged RBCs; fights infection
How does the spleen get damaged when filtering the blood?
the spleen will destroy sickled cells but in the process becomes damaged; it fights infection if it becomes damaged it can lead to infection/ risk of infection
What is the trigger for RBCs to sickle?
low oxygen conditions
Where does WBC formation occur?
in the soft tissue inside of your bones (bone marrow)
What are the 2 types of WBCs?
- T cells
- B cells
What is another name for white blood cells?
lymphocytes
What is another name for red blood cells?
erythroctye
where does t cells grow?
thymus
What does the T cells do?
- wipe out infected or cancerous cells
- direct the immune response by helping B lymphocytes to eliminate invading pathogens
What does the B cells do?
create antibodies
B cell aka?
B lymphocyte
T cell aka?
T lymphocyte
:a fist-sized organ found in the upper left side of your abdomen, next to your stomach and behind your left ribs; an important part of your immune system but you can survice without it; part of your lymphatic and immune system
spleen
What does the spleen do?
- it fights any invading germs in the blood (the spleen contains infection-fighting white blood cells/ antibodies)
- it controls the level of blood cells (WBC, RBC and platelets)
:small cells that form blood clots
platelets
What screens the blood and removes any old or damaged red blood cells?
spleen
If the spleen does not work properly, it may start to remove what? What can this lead to?
health blood cells; anemia- from a reduced number of RBCs; an increased risk of infection- from a reduced number of WBCs; bleeding or bruising - from a reduced number of platelets
Sickle Cell Disease Assessment Questions
History
* ask about previous crisises, what let to the crisis, severity, and usual management
* explore recent contact with ill people and activities to determine what caused the current crisis; ask about signs and symptoms of infection
* review all activities and events during the past 24hrs, including food and fluid intake, exposure to temperature extremes, drugs taken, exercise, trauma, stress, recent airplane travel, and use of alcohol, tobacco, or other recreational drugs
* ask about changes in sleep and rest patterns, ability to climb stairs, and any activity that induces shortness of breath
* assess the patient’s perceived energy level using a scale ranging from 0 to 10 (0=not tired with plenty of energy; 10= total exhaustion) to assess the degree of fatigue
What is the most common symptom of SCD crisis?
pain
Psychosocial Assessement: SCD
- often cognitive and behavioral changes are early indications of cerebral hypoxia from poor perfusion
- assess the patient and document mental status exam results
hematocrtit of patients with SCD
is low (between 20%- 30%) because of RBC shortened lifespan and destruction; the value decreases even more during a crisis or stress (aplastic crisis)
the reticulocyte count of SCD patients is
is high, indicating anemia of long duration
the total bilirubin of a SCD patient may be
high because damaged RBCs release iron and bilirubin
a SCD patient’s WBC count is
usually high, the elevation is r/t chronic inflammation caused by tissue hypoxia and ischemia
Why would patients with SCD have bone changes?
from a result of chronically stimulated marrow and low bone oxygen levels
Why may a SCD patient’s skull on an xray look like a “crew cut”?
bone surface cell destruction and new growth giving the skull a “crew cut” appearance
What may joints show on an xray of a SCD patient?
necrosis and destruction
What may ultrasonography, CT, positron emission tomography (PET), and a MRI show of a SCD patient?
soft-tissue and organ changes from poor perfusion and chronic inflammation
What do ECG changes show in a patient with SCD? specific ECG?
changes indicating cardiac infarcts and tissue damage; related to the area of the heart damaged
Echocardiograms may show what in SCD patients?
cardiomyopathy and decreased cardiac output (low ejection fraction)
Infection prevention in SCD patients:
- spleen plays a big role in immune system
- patient with scd at risk for infection
- watch closely for sepsis
- prophylactic antibodies often given
- recommend annual flu shot and pneumonia vaccine
- strict asepsis technique for all invasic procedures
- HANDWASHING!
What procedure is helpful for SCD patients?
transfusions with RBCs; must use cautiously to avoid iron overload from repeated transfusions; will see transfusions used pretty commonly with goal being to avoid stroke
what labwork would you expect to see ordered for a SCD patient?
- CBC
- H&H
- ABGs
H.O.P. (SCD interventions)
- Hydration
- Oxygenation
- Pain relief
Pain management for patients with SCD experiencing a acute crisis usually starts with what?
at least 48 hrs of IV pain meds
What percentage of SCD patients suffer from an opioid addiction?
2%-5%
Concerns about substance abuse can lead to what?
inadequate pain treatment in SCD patients, as they are often treated as drug seekers
CHECK YOUR BIAS!
SCD patient: pain management
Morphine and Dilaudid often given as
via IV or PCA pump
Avoid what concerning pain meds with SCD patients?
PRN schedule
A SCD patient is usually dehydrated when they come in for a crisis…
hydration through both oral and IV routes
Hydration is important in SCD patients experiening a crisis, what should we encourage?
the patient to drink fluids- water, juices
Because the SCD patiet is dehydrated their blood is…
what fluids are given?
usually hypertonic; hypotonic fluids given IV (usually 1/2 NS infused, often initial bolus at 250mL/hr & then reduced to maintenance rate)
SCD
In a crisis, IV intake should be at least what rate?
200mL/ hr
Why is oxygen given to a SCD patient during a crisis?
a lack of oxygen is the main cause of sickling; ensure the oxygen is humidified
Complementary and altenative therapies: SCD patients/ crisis
- keep the room warm
- use distraction
- relaxation techniques
- positioning
- warm soaks
chimerism
presence of donor cells