Hematologic Disorders Flashcards
Anemia
RBC count is low
Polycythemia
RBC count is high
Agranulocytes
Lymphocytes: increased with infections
Monocytes: in chronic conditions
Granulocytes
Neutrophils: acute inflammation, bacterial infection
PMN: indicates infections
Bands: immature neutrophils
Eosinophil: allergic reactions
Basophils: healing phase of inflammation and hay fever
Platelets
Used to stop bleeding Made in bone marrow Stored in spleen Life span 8-10 days 150,000 - 300,000 is normal
Thrombocytopenia
Decreased platelet count from a bleeding disorder
Intrinsic pathway
Released by plasma
PTT measures abnormalities for pts on heparin
Extrinsic Pathway
Released by the injured tissue
PT measures abnormalities for pts on warfarin
Immunoglobulins
IgM - First responder, 2-3 days
IgG - Day 4-10, last for a new weeks
IgA - Located in GI tract, respiratory tract, and GU tract. Prevents adherence
IgE - Allergic response
T-Helper (CD4)
Involved in immune surveillance, stimulate B- lymphocyte production
CD4 count - HIV
T-Suppressor
Supresses production of immunoglobulins against specific antigens and prevents overproduction
Body fails to recognize self
T-Cytoxic
Aid in destroying viral, fungal, protozoan and some bacteria
Goes out and eats the cells
Natural Killer
Migrate to blood and spleen and aid the Tcells
Risk for Infection
Good hand washing, monitor VS, antibiotics, G-CSF to stimulate granulocytes
Aseptic technique for all procedures, insure good nutrition, and monitor CBC and WBC
Teach prevention!
Imbalanced Nutrition: Less Than
Promote nutrition with small high calorie feeds and maintain fluid balance
Dietary management, iron fortified formula for infants
Older child with PICA – eat ice
Risk for Injury: Bleeding
SAFETY!
Assess CNS, joints, GI/GU bleeding. Recognize and control bleeding
NO ASA or NSAIDs, IM injections
Give factor replacement
Platelets <50-100,00 limit contact sports, no bike riding, skateboarding, or climbing
Encourage quiet activity
Activity Intolerance
Assess color of skin and mucus membranes
Allow periods of rest
In bed activities
Good nutrition
Risk for Disturbed Body Image
Provide emotional support and allow them to discuss feelings
Have teens talk to someone with their condition
Impaired Physical Mobility
Promoting mobility is key
Physical therapy!!
Encourage ADL’s
Protect the joint
Caregiver Role Strain
May need to evaluate family coping
Provide emotional support and allow families to verbalize feelings
Parents may have guilt for being a carrier
Knowledge Deficit
Use a family approach
Teach disease process
Identify and avoid triggers
Treatment regimen
3 Types of Anemia
Microcytic and Hypochromic Normocytic and Normochromic Macrocytic -- Rare MCV- the size MCH- the color, dependent on amount oh Hgb
3 Causes of Anemia
Increased RBC destruction
Increased RBC loss
Decreased RBC production (aplastic anemia)
Iron Deficiency Anemia
Low MCV, MCH
Serum Feritin is low
Fatigue, pale, irritable
Underweight or overweight from too much milk
Increased RBC Destruction S/Sx
Pallor Tachycardia, Heart Murmur Fatigue, HA Muscle Weakness Frontal Bossing
Increased RBC Loss S/Sx
Jaundice Fatigue, HA Dark Urine Enlarged spleen and liver Low BP
Decreased RBC Production S/Sx
Pallor Fatigue, HA Muscle Weakness Cool skin Tachycardia Decreased peripheral pulses
Iron Deficient Anemia Causes
Cows milk is a poor source of iron
Iron supplements can begin at 2 months for preterm, 4-6 months for term babies
Appears around 9 to 12 months due to loss of iron stores from birth
In adolescents from poor diet and menstruating girls
Iron Deficient Anemia Nursing Care
Prevention and Education
High risk- WIC children and low income families
Administer medication via syringe at back of the mouth, or drink through a straw
Take with something acidic, Orange juice
Encourage a rich diet of leafy green veggies
Medication causes constipation and tarry stool!
Sickle Cell Anemia
A condition of normocytic, normochromis anemia with increased RBC destruction
Normal Hgb A is replaced with abnormal Hgb S
Cells are sickled
Autosomal recessive!
Girls > Boys
African American >
Newborn screening
Sickle Cell Assessment Questions
Ask about past crisis
Ask about triggers
Past medical treatment and home management
Assess psychosocial
Sickle Cell Signs and Symptoms
Asymptomatic until 4-6 months
Sickle cells cant flow easily
Clogged up – Trigger pain crisis
Blindness, enlarged liver and spleen, chest crisis
Joint swelling – also in penis and testes
Possible stroke, paralysis and death
Sickle Cell Head to Toe Assessment
See Box 35-4
Page 1437
Sickle Cell Treatment
Limit activity to reduce energy expenditure
Improve oxygenation
HYDRATION! I/O’s
Reduce Metabolic Acidosis – from hypoxia
PAIN MANAGEMENT!
Transfuse packed RBCs, Antibiotic – If needed
Home care: Penicillin K, multivitamin, folic acid
B- Thalassemia Major
Cooley's Anemia Autosomal Recessive Defective in the synthesis of hgb A Microcytic and hypochromic Leads to increased RBC destruction Increased serum feratin EXTRA Iron- overload Mediterranean Middle Eastern Decent
B- Thalassemia Major Treatment
PRBC transfusions – prevent bone marrow expansion
Keep Hgb >9.5
Take Desferal (Deferoxamine)
Diet low in iron
Prophylactic antibiotics
Spleenectomy to decrease discomfort and rate of RBC destruction
B- Thalassemia Major Nursing Care
Support family and child
Body Image disturbance – bony changes, decreased growth and sexual immaturity
Aplastic Anemia
Formed elements of the blood are low, RBC, WBC and Platelets
Sudden onset
Life threatening
Autosomal recessive- primary or congenital
Aplastic Anemia Acquired Causes
HPV, Hepatitis, an overwhelming infection
Immune disorders
Drugs, household chemicals
Aplastic Anemia Congenital Cause
Fanconi’s Anemia
Patchy brown areas of skin discoloration
Genetic counseling is needed!
Autosomal Recessive Syndrome
Aplastic Anemia Diagnosis
CBC is first step
Bone marrow aspirate– appear yello and fatty instead od red
Aplastic Anemia Treatment
Immunosuppressant to limit aplasia
Stem Cell transplant
Treatment is supportive
Hemophillia A
X-linked recessive disorder of Factor 8 deficiency
Boys > girls
Mild, moderate and severe
Mild Hemophillia A
Bleeding with trauma or surgery
Moderate Hemophillia A
Bleeding with trauma
Severe Hemophilla A
Spontaneous bleeding without trauma
More common
Hemophilla Concerns
Bleeding into the tissue –
Neck or thorax can impair breathing
Intracranial can be fatal
Retro peritoneal is an issue to due large space for blood accumulate
Hematoma on spinal cord = paralysis
Hemoarthosis in the joint– lead to loss of function
Hemophilla Treatment
Factor 8 Replacement as an infusion Goal is to stop[ bleeding Permanent IV access R.I.C.E. Parents can administer -- then child
Hemophilla B
Christmas Disease
Sex linked Recessive trait
Factor IX deficiency
Treatment with replacement therapy
Idiopathic Thrombocytopenia Purpura (ITP)
Autoimmune disease
Excessive destruction of platelets
Usually resolves within 6 months
ITP Symptoms
Bruising, purpura
Bleeding that wont stop
RISK FOR INJURY
Wear helmets A LOT
ITP Treatment
Supportive and observe if mild
Symptomatic – give IV IgG to raise platelet count
Prednisone up to 3 weeks
HIV Etiology
Vertical Transmission - perinatal from mother – leading cause in children
Horizontal from sex or parental contact with blood or body fluid
Vertical # 1 way
Look at CD4 count
HIV Diagnosis
Done by ELISA and Western Blot
Positive in infants with HIV+ mothers, maternal antibodies last 18 months
PCR x2 for proviral DNA – early as 1 to 3 months
CDC surveillance for AIDS
HIV Treatment Goals
Slow growth of HIV Virus
Promote normal growth and development
Decrease infections and cancers
Improve quality of life and prolong survival
HIV Treatment
Antiretroviral agents AZT first then HAART Bactrim starting at 4 - 6 weeks MMR and Varicella as scheduled Monitor viral load and CD4 Standard isolation techniques
HIV Prognosis
Earlier presentation of AIDS = Poor, esp 100,000 and CD4 < 15%
SCIDS
Congenital X-linked
Autosomal Recessive
Prents the formatio nof T-lymphocytes
Affects maturation of B-lymphocytes
SCIDS S/Sx
Early infection (oral candidiasis) Failure to thrive Chronic diarrhea Otitis media Pneumonia Failure to recover from an infection Low or absent B & T lymphocytes
SCIDS Treatment
Goal: Restore immune function Give IV Ig Possible Stem cell transplant HIGH RISK FOR GRAFT vs HOST Prevent and promptly treat infections
SLE
Girls > Boys
Chronic inflammatory disease with an unknown origin
Characterized by exacerbation and remission
Involves more than one body system
SLE S/Sx
Nephritis
Arthritis or vasculitis
Butterfly rash
See pg 1683
SLE Treatment
Goal is remission of symptoms and prevent complications
May need steroids
Anti-malarials help skin, renal and arthritis
NSAIDs for pain
SLE Nursing Care
Education and compliance with medication Understanding triggers and episodes Positive self- image r/t weight -- buffalo hump and moon shaped face Sunscreen and protective clothing Support groups
Anaphylaxis
Clinical syndrome resulting from exposure to an allergen
Result of IgE release – histamine release – vasodilaion – multi-system involvement
MEDICAL EMERGENCY
EPIPEN!
