Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
226 Cards in this Set
- Front
- Back
When is sickle cell disease diagnosed?
|
After age 4 months
|
|
What type of hemoglobin do well people have?
|
HgbA
|
|
What type of hemoglobin do infants have?
|
HgbF
|
|
What type of hemoglobin is found in a person who has sickle cell disease?
|
HgbS
|
|
Explain why sickle cell disease affects males and females alike:
|
It is autosomal: the affected gene is located on one of the 1st 22 pairs of chromosomes that do not determine gender
|
|
If a child inherits only one abnormal "sickle cell" gene, does he or she have the disease?
|
No; only the trait
|
|
What type of disorder is sickle cell disease classified as?
|
Autosomal, recessive disorder
|
|
In order for a child to inherit sickle cell disease, what must happen?
|
Both parents must have the defective genes
|
|
What is the lifespan of a sickled red blood cell?
|
7 - 20 days
|
|
What is the lifespan of a normal red blood cell?
|
120 days
|
|
What can cause sickling of the RBC's?
|
*Dehydration
*Acidosis *Infection *Hypoxia *Venous stasis *Strenuous exercise *Anesthesia |
|
What are the symptoms of sickle cell?
|
*Growth retardation
*Chronic anemia *Susceptibility to sepsis *Delayed sexual maturation *Fever *Pain |
|
What is the most common and most distressing symptom of sickle cell?
|
Pain
|
|
What is priapism?
|
A nocturnal penile erection that lasts as long as 4 or more hours. Very painful
|
|
How is priapism treated?
|
*Estrogens
*Vasodilators |
|
What are reticulocytes?
|
Immature RBC's
|
|
What is the WBC count like in a patient with sickle cell?
|
Elevated
|
|
What is the total bili count like in a patient with sickle cell?
|
Elevated
|
|
What is the sickle cell turbidity test?
|
A screening tool only
|
|
How is sickle cell disease determined for sure?
|
Hemoglobin electrophoresis
|
|
How are results from a hemoglobin electrophoresis test interpreted?
|
*Less than 40% HgbS = Trait
*More than 40% HgbS = Disease |
|
What is the most common sickle cell crisis?
|
Vaso-occlusive crisis
|
|
Which sickle cell crisis causes lung/chest pain in the elderly?
|
Vaso-occlusive crisis
|
|
Which sickle cell crisis causes painful swelling of the hands/ feet and extreme pain in infants?
|
Vaso-occlusive crisis
|
|
Which sickle cell crisis commonly affects the knees/back of adults, and can cause infarcts of the small bones?
|
Vaso-occlusive crisis
|
|
Which sickle cell crisis is also known as splenic sequestration?
|
Sequestration crisis
|
|
Describe sequestration crisis:
|
*Blood is blocked into spleen
*Spleen enlarges *Spleen can become scarred/useless |
|
Why can a patient become "shocky" during a sequestration crisis?
|
Blood is trapped in the spleen and therefore not circulating throughout the body
|
|
In patients with sequestration crisis, what can happen by age 1?
|
Spleen can lose up to 29% of function
|
|
In patients with sequestration crisis, what can happen by age 2?
|
Spleen can lose greater than 40% of functionality
|
|
What is G6PD stand for?
|
Glucose 6 Phosphate Deficiency
|
|
What type of sickle cell crisis is caused by G6PD?
|
Hyperhemolytic Crisis
|
|
What does G6PD's function in the body?
|
It is an enzyme that stimulates the RBC's (gives them energy)
|
|
How much fluid intake is encouraged for patients who have sickle cell disease?
|
4-6 quarts per day
|
|
What is G6PD stand for?
|
Glucose 6 Phosphate Deficiency
|
|
How much fluid intake is encouraged for patients who are in sickle cell CRISIS?
|
6-8 quarts per day
|
|
What type of sickle cell crisis is caused by G6PD?
|
Hyperhemolytic Crisis
|
|
List some drugs that are used to manage pain in sickle cell patients:
|
*Methadone
*Morphine *Hydroxyuria *Dilaudid *Fentanyl patch |
|
What does G6PD's function in the body?
|
It is an enzyme that stimulates the RBC's (gives them energy)
|
|
What treatment for sickle cell disease is aimed at diluting the HgbS levels?
|
Chronic transfusions
|
|
How much fluid intake is encouraged for patients who have sickle cell disease?
|
4-6 quarts per day
|
|
What lab levels should be monitored in a patient who is undergoing chronic transfusions?
|
*Iron
*TIBC *Ferritin |
|
How much fluid intake is encouraged for patients who are in sickle cell CRISIS?
|
6-8 quarts per day
|
|
What should be avoided in patients with sickle cell disease?
|
*Caffeine
*Cold liquids |
|
List some drugs that are used to manage pain in sickle cell patients:
|
*Methadone
*Morphine *Hydroxyuria *Dilaudid *Fentanyl patch |
|
In the pathophysiology of sickle cell disease, what happens to the Hgb S cell?
|
It assumes a sickled appearance
|
|
What happens to the blood's viscosity when the RBC's sickle?
|
The viscosity is increased
|
|
True or False: sickled cells increase RBC destruction
|
True
|
|
What treatment for sickle cell disease is aimed at diluting the HgbS levels?
|
Chronic transfusions
|
|
What lab levels should be monitored in a patient who is undergoing chronic transfusions?
|
*Iron
*TIBC *Ferritin |
|
What should be avoided in patients with sickle cell disease?
|
*Caffeine
*Cold liquids |
|
In the pathophysiology of sickle cell disease, what happens to the Hgb S cell?
|
It assumes a sickled appearance
|
|
What happens to the blood's viscosity when the RBC's sickle?
|
The viscosity is increased
|
|
What happens to the oxygen carrying capacity of red blood cells when they are sickled?
|
It is decreased
|
|
True or False: sickled cells increase RBC destruction
|
True
|
|
What happens to circulation when the RBC's are sickled?
|
Circulatory stasis occurs
|
|
What happens to the oxygen carrying capacity of red blood cells when they are sickled?
|
It is decreased
|
|
What is a critical intervention for a patient in sickle cell crisis?
|
Bedrest
|
|
What happens to circulation when the RBC's are sickled?
|
Circulatory stasis occurs
|
|
What is a critical intervention for a patient in sickle cell crisis?
|
Bedrest
|
|
What kind of gene is affected in hemophilia A?
|
Sex-linked recessive gene
|
|
Why is almost 100% of hemophilia A found in men?
|
The gene is linked to the X chromosome, and males only have one X chromosome.
|
|
What clotting factor is deficient in hemophilia A?
|
Factor VIII
|
|
What is the preventive treatment for children with sickle cell disease?
|
*Penicillin at age 3 mo.
*Pneumococcal vaccine @ 2, 4 & 6 mo. *Flu shot @ 6 mo. |
|
What should be taught to parents of sickle cell patients?
|
*Recognizing s/sx
*Charting growth/development *Give folic acid early *Give protein supplements *Get eyes tested early |
|
What is Partial chimerism?
|
A transfusion in which a donor's bone marrow is mixed with the patient's bone marrow and administered to the patient
|
|
What clotting factor is deficient in hemophilia B?
|
Factor IX
|
|
What clotting factor is deficient in hemophilia C?
|
Factor XI
|
|
What is the difference between hemophilia and DIC?
|
Hemophilia: involves only intrinsic clotting factors
DIC: involves both intrinsic and extrinsic clotting factors |
|
What is a good physical activity to suggest for a patient with hemophilia?
|
Swimming
|
|
When is hemophilia usually diagnosed?
|
In early childhood (when the child begins to walk)
|
|
What is the usual treatment for hemophilia?
|
Replacement of coagulation factor that is affected
|
|
What type of deformities are a complication of hemophilia?
|
Joint deformities
|
|
What are the signs/symptoms of hemophilia?
|
*Prolonged bleeding anywhere in the body
*Bruising *Hematuria |
|
What laboratory finding will be abnormal in a patient with hemophilia?
|
PTT
|
|
Will the PT lab value be normal or abnormal in a patient with hemophilia?
|
Normal
|
|
What is DIC?
|
Disseminated Intravascular Coagulation
|
|
How will the PT / INR reflect in lab reports of a patient with DIC?
|
Prolonged
|
|
How will the activated partial thromboplastin time (APTT) reflect in lab reports of a patient with DIC?
|
Prolonged
|
|
How will the platelet count reflect in lab reports of a patient with DIC?
|
Decreased
|
|
How will the fibrinogen levels reflect in lab reports of a patient with DIC?
|
Decreased
|
|
How will the fibrinogen degradation product levels reflect in lab reports of a patient with DIC?
|
Increased
|
|
How will the D-Dimer test reflect in lab reports of a patient with DIC?
|
Increased
|
|
What is the cure for DIC?
|
Treat the cause
|
|
True or False: Once you have DIC, you are prone to get it again
|
False
|
|
What are some nursing priorities in patients with DIC?
|
*Maintain fluid balance
*Control bleeding *Restore normal clotting factors |
|
What are some important things to remember when caring for a DIC patient?
|
*No blood pressure cuffs
*Turn carefully *Be careful with catheters, ET tubes, etc. |
|
What level of D-Dimer proteins indicate DIC?
|
Greater than 250 per ml
|
|
Explain erythropoesis:
|
Kidneys respond to low O2 and release erythropoetin, which travels to the bone marrow to stimulate RBC production
|
|
What are the 5 types of WBC's?
|
*Neutrophils
*Lymphocytes *Eosinophils *Monocytes *Basophils |
|
What is the largest components of white blood cells?
|
Neutrophils
|
|
In regards to WBC's, what indicates a patient's ability to fight infection?
|
The number and percentage of neutrophils
|
|
How is the absolute neutrophil count calculated?
|
*Add the neutrophils plus the bands
*Write the sum as a decimal or percentage *Multiply this number by the total WBC number |
|
With regards to WBC's, what is a "left shift?"
|
The number of bands is greater than everything else
|
|
What do the granules of basophils and mast cells release?
|
Histamines
|
|
What happens to the lungs when histamines are released?
|
Airways swell
|
|
What happens in the nasal passages when histamines are released?
|
*Vasodilation
*Stuffy, runny nose |
|
What is the normal range for platelets?
|
150-440
|
|
What happens to platelets in a patient with sickle cell disease?
|
They are increased
|
|
When is AIDS diagnosed?
|
When the body loses the ability to fight opportunistic infections
|
|
What must the CD4 count be in order to be classified as AIDS?
|
Below 200
|
|
What does the HIV retrovirus have that helps viral replication?
|
Reverse transcriptase (RT)
|
|
What does Reverse Transcriptase do?
|
Forces DNA to use the virus as a "pattern"
|
|
After the Reverse Transcriptase forces the body's DNA to use HIV as a pattern, what takes place?
|
The new viral DNA gets into the body's DNA
|
|
In AIDS, how many viral particles per day can synthesize?
|
2 billion
|
|
What does the HIV virus do to the CD4 receptors?
|
Attaches to, infects and kills all immune cells with the CD4 receptors
|
|
What is significant of AIDS as opposed to HIV?
|
Profound immunodeficiency
|
|
What is the most common life threatening opportunistic infection in AIDS patients?
|
Pneumocystis Carinii (PCP)
|
|
What type of infection is pneumocystis carinii?
|
Protozoa and fungal infection
|
|
How is pneumocystis carinii (PCP) transmitted?
|
Via air, food, water
|
|
What percentage of AIDS patients have PCP as their first bout of AIDS-related opportunistic infection?
|
60%
|
|
What are the symptoms of pneumocystis carinii (PCP)?
|
*Fever
*Fatigue *Weight loss *SOB *Crackles *Cough |
|
What is the treatment for pneumocystis carinii (PCP)?
|
*Pentamidine (aerosol)
*Mechanical ventilation *aerosolized bronchodilators *TMP-SMX (sulfa compound) *Steroids |
|
What is a serious side effect of TMP-SMX?
|
Nephrotoxicity
|
|
What is the most common malignancy in HIV patients?
|
Kaposi's Sarcoma
|
|
Besides skin lesions, what are some other symptoms of Kaposi's Sarcoma?
|
*Diarrhea
*Blockage of lymph leading to edema of face or extremities *Respiratory distress *Cognitive changes |
|
What is the treatment for Kaposi's Sarcoma?
|
*Radiation
*Chemotherapy *Interferon |
|
What is Cryptosporidium?
|
A parasite
|
|
How is Cryptosporidiosis transmitted?
|
Animal to human or human to human
|
|
What is the most common site of cryptosporidiosis infection?
|
Small intestine
|
|
What is the most distressing symptom of cryptosporidiosis?
|
Copious explosive diarrhea (15-20 liters/day)
|
|
True or False: cryptosporidium can be killed by bleach
|
False
|
|
By what cycle is cryptosporidium transmitted?
|
Fecal-oral cycle
|
|
Besides the diarrhea, what are some other symptoms of cryptosporidiosis?
|
*Weight loss
*Dehydration |
|
Is there an effective treatment for cryptosporidiosis?
|
No
|
|
What drug can be given to patients with cryptosporidiosis?
|
Octreotide (to lessen diarrhea)
|
|
What is significant of Octreotide?
|
It has lots of side effects
|
|
What is the screening test that measures HIV antibodies?
|
ELISA
|
|
What is the confirming test for HIV?
|
Western Blot
|
|
Why is the CD4 count measured in HIV patients?
|
This is used to measure the progression of the disease and the effectivity of the medication
|
|
How often should an HIV patient have CD4 counts measured initially?
|
Every 3-6 months
|
|
What is the WBC count like in a patient with HIV/AIDS?
|
Low
|
|
What does the Viral culture/Viral Load Test measure?
|
The genetic material of HIV; this indicates amount of disease progression and effectivity of medication
|
|
How often should an AIDS patient have a Viral Load Test?
|
Every 2-8 weeks
|
|
What is the drug regimen for HIV patients who are pregnant?
|
ZDV (a combination of 3 drugs)
|
|
When is ZDV therapy started in a pregnant woman with HIV?
|
14-34 weeks gestation
|
|
When is ZDV administered IV in a pregnant woman with HIV?
|
During delivery
|
|
ZDV is given in liquid form to babies of HIV mothers at what frequency and duration?
|
*Every 6 hours
*For the 1st 6 weeks of life |
|
How are babies tested for HIV?
|
By looking for the virus itself (not the antibodies as in adults)
|
|
ZDV therapy in mothers with HIV is shown to reduce the risk of passing HIV to the baby by what percentage?
|
70%
|
|
When is a c-section delivery recommended for pregnant women with HIV?
|
*If viral load is greater than 1000
*If membranes are ruptured *If no HIV drug therapy *if no prenatal care |
|
How often should a room/bathroom be cleaned for immunocompromised patients?
|
Every day
|
|
How often should the vital signs be checked in an immunocompromised patient?
|
Every 4 hours
|
|
A minor elevation in temperature for an immunocompromised patient can indicate what?
|
Sepsis
|
|
How often should the mouth and skin of an immunocompromised patient be assessed?
|
Every 8 hours
|
|
How often should open areas/IV sites, etc. be assessed in an immunocompromised patient?
|
Every 4 hours
|
|
How often should IV tubing be changed for an immunocompromised patient?
|
Every day
|
|
How often should wound dressings be changed in patients who are immunocompromised?
|
Every day
|
|
How often should WBC's (esp. ANC) be monitored in immunocompromised patients?
|
Every day
|
|
Immunocompromised patients should not drink water that has been standing for how long?
|
Longer than 15 minutes
|
|
What do the initials HAART stand for?
|
Highly Active Antiretroviral Therapy
|
|
What is HAART?
|
A combination of 3 or more anti-HIV medications in a daily regimen
|
|
How do the Nonnucleoside Reverse Transcriptase Inhibitors (NNRTI's) work?
|
Bind to and disable reverse transcriptase
|
|
How do Nucleoside Reverse Transcriptase Inhibitors (NRTI's) work?
|
*NRTI's are faulty versions of building blocks HIV needs to make more copies of itself
*When HIV uses an NRTI instead of a normal building block, reproduction of the virus is stalled |
|
How do Protease Inhibitors (PI's) work?
|
Disable protease, a protein that HIV needs to make more copies of itself
|
|
How do Fusion Inhibitors work?
|
By blocking HIV entry into cells
|
|
What does HIV do to CD4 cells?
|
Destroys them
|
|
In the morphology of leukemia, what does "lympho-" indicate?
|
Originating from lymphoid or lymphatic system
|
|
In the morphology of leukemia, what does "Myelo-" indicate?
|
Originating from bone marrow
|
|
In the morphology of leukemia, what is indicated by the words "blastic" and "acute"?
|
Sudden; involves immature white cells
|
|
In the morphology of leukemia, what is indicated by the words "cytic" and "chronic?"
|
Over time; involves mature white cells
|
|
To what area/body system is leukemia prone to travel, yet chemotherapy does not reach this area/body system?
|
Brain/CNS
|
|
From what cells does Acute Lymphocytic Leukemia (ALL) arise?
|
Lymphoblasts
|
|
80% of ALL diagnoses are what age group
|
children
|
|
What is the peak age of diagnosis of ALL?
|
2-4 years
|
|
There is a sharp decline in diagnosis of ALL after what age?
|
10 years
|
|
What percentage of children with ALL can be cured?
|
50%
|
|
ALL is more common in what gender?
|
Males
|
|
In patients with ALL, a higher WBC indicates what?
|
A poorer prognosis
|
|
In regards to ALL prognosis, which diagnosis age groups do better?
|
*Diagnosed at age 2-9: better prognosis
*Diagnosed before age 2 or after age 10, poorer prognosis |
|
Which gender has a better prognosis with ALL?
|
Females
|
|
What should be done for patients who have an ANC of less than 500?
|
*Neutrapenic precautions
*Reverse isolation |
|
What is a normal ANC level?
|
Greater than 500
|
|
From what kind of cells does Acute Myelogenous Leukemia (AML) arise?
|
Single myeloid stem cell (myeloblast)
|
|
What is a myeloblast?
|
Precursor to a granulocyte
|
|
There is a sharp decline in diagnosis of ALL after what age?
|
10 years
|
|
What percentage of children with ALL can be cured?
|
50%
|
|
AML causes the development of what in the bone marrow?
|
Immature myeloblasts
|
|
At what age does AML occur more frequently?
|
*In adolescence (12-20 years)
*After age 55 |
|
ALL is more common in what gender?
|
Males
|
|
What is the common treatment for AML?
|
Bone marrow transplants
|
|
In patients with ALL, a higher WBC indicates what?
|
A poorer prognosis
|
|
From what type of cells does Chronic Lymphocytic Leukemia (CLL) arise?
|
Lymphocytes
|
|
In regards to ALL prognosis, which diagnosis age groups do better?
|
*Diagnosed at age 2-9: better prognosis
*Diagnosed before age 2 or after age 10, poorer prognosis |
|
Which gender has a better prognosis with ALL?
|
Females
|
|
CLL is more common in what age group?
|
50-70 years old
|
|
What should be done for patients who have an ANC of less than 500?
|
*Neutrapenic precautions
*Reverse isolation |
|
What is a normal ANC level?
|
Greater than 500
|
|
From what kind of cells does Acute Myelogenous Leukemia (AML) arise?
|
Single myeloid stem cell (myeloblast)
|
|
What is a myeloblast?
|
Precursor to a granulocyte
|
|
AML causes the development of what in the bone marrow?
|
Immature myeloblasts
|
|
At what age does AML occur more frequently?
|
*In adolescence (12-20 years)
*After age 55 |
|
What is the common treatment for AML?
|
Bone marrow transplants
|
|
From what type of cells does Chronic Lymphocytic Leukemia (CLL) arise?
|
Lymphocytes
|
|
CLL is more common in what age group?
|
50-70 years old
|
|
CLL is more common in which gender?
|
Males
|
|
What are the WBC levels like in a patient with CLL?
|
High
|
|
What is the survival rate for those diagnosed with CLL?
|
4-5 years
|
|
From what cells does Chronic Myelogenous Leukemia (CML) arise?
|
Granulocytes
|
|
Which leukemia has the Philadelphia chromosome marker?
|
CML
|
|
What is the survival rate for those with CML?
|
5-6 years
|
|
Which leukemia is associated with "blast crisis"?
|
CML
|
|
What is the prognosis for a CML patient in blast crisis?
|
2-4 months
|
|
What is the treatment for CML patients?
|
Bone marrow transplant
|
|
What are some symptoms of CML?
|
*Fatigue
*Anorexia *Splenomegaly *Weakness *Weight Loss |
|
How should the nurse explain the pain of bone marrow aspiration procedure?
|
"you will experience a brief, sharp pain"
|
|
What is involved in the diagnosis of CML?
|
*Elevated WBC (15,000-500,000)
*Granulocytic hyperplasia in the bone marrow |
|
What should the nurse monitor in leukemia patients in regards to oxygenation and circulation?
|
*Anemia
*Hemorrhage |
|
What is the hallmark for Hodgkin's Disease?
|
Reed-Sternberg cell
|
|
What age groups are affected by Hodgkin's Disease?
|
Any age group
|
|
What are the major symptoms of Hodgkin's Disease?
|
*Painless enlargement of a lymph node
*Fatigue *Anorexia *Unexplained fever *Night sweats |
|
What are some diagnostic tools for Hodgkin's disease?
|
*Lymph node biopsy
*Chest X-Ray *CT scan |
|
What is Multiple Myeloma?
|
Bone cancer; cancer of the plasma cells
|
|
Multiple myeloma is a WBC cancer that involves what kind of cells?
|
More mature cells
|
|
What is the onset of multiple myeloma like?
|
Slow and insiduous
|
|
What are the main problems with multiple myeloma?
|
*Pathologic bone fractures
*Renal failure |
|
What are some other symptoms of multiple myeloma?
|
*Back pain
*Paralysis *Constipation *Hypercalcemia *Hyperuricemia *URI |
|
Is multiple myeloma common?
|
No; very uncommon
|
|
At what age is multiple myeloma usually diagnosed?
|
Around age 50
|
|
What is a very important treatment for people with multiple myeloma?
|
Hydration: approx. 3000 cc's per day
|
|
Besides hydration, what are some other treatment measures for multiple myeloma?
|
*Glucocorticoids
*Calcitonin *Chemotherapy *Radiation *Bone marrow transplant |
|
Which multiple myeloma treatment has a poor prognosis?
|
Bone marrow transplant
|
|
What is the percentage of remission with multiple myeloma?
|
50%
|
|
What is the hallmark of multiple myeloma?
|
Bence Jones protein
|
|
What type of diet is recommended to prevent calculus formation in a multiple myeloma patient?
|
Low calcium
|
|
What lab values should be checked often in multiple myeloma patients?
|
BUN/Creatinine
|