Nursing Care of H&L Disorders Flashcards
S&S of anemia
Extreme weakness Dyspnea Fever Hypoxia Weight loss Slight jaundice with pallor Lower extremity edema Intermittent constipation & diarrhea Flatulence, N, and indigestion Palpitations Dysphagia Hand/feet tingling Partial or total paralysis
What is anemia?
Deficiency of RBCs, hemoglobin, or both
What is the pathophysiology of:
Aplastic anemia
Iron-deficiency anemia
Pernicious anemia
Impaired production of RBCs
What is the pathophysiology of:
Sickle cell anemia
Thalassemia
Increased destruction of RBCs
What is the pathophysiology of anemia r/t:
Trauma- hypovolemia
Chronic infection
Renal failure
Massive or chronic blood loss
Aplastic anemia
Decrease of bone marrow function
Depression of erythrocyte production, which results in lowerd hgb and RBCs
All formed elements of blood are defective
What may develop due to Aplastic anemia?
Leukopenia and thrombocytopenia
Primary causes of aplastic anemia
Congenital
Secondary causes of aplastic anemia
Viral invasion
Meds
Chemicals
Radiation/Chemo
Diagnostic tests r/t aplastic anemia
CBC- all values low (except RBCs- they have longer life span)
Bone marrow aspiration- pale,yellow, fatty marrow
Total Iron-binding capacity
Serum iron level
Peripheral blood smears
Medical management of aplastic anemia
Bone Marrow and Stem Cell transplant
Blood transfusions
Antibiotics
Immunosuppressant agents (if autoimmune) -
-Antithymocyte globulin, Cyclosporine,
Methyprednisolone
Medications that stimulate bone marrow
-Colony-stimulating factors, Epogen-RBCs,
Neupogen- WBCs
Nursing interventions for aplastic anemia
Prevent infection
Neutropenic precautions
Hand hygiene
Avoid contact with those who have an infection
Bleeding precautions
Eliminate intake of raw meats, fruits, or veggies
Report S&S of infection or bleeding
Observe for blood in urine/stool, or petechiae
Use soft toothbrush or swab mouth
Avoid enemas or other rectal insertions
No nose picking or blowing nose forcefully
Avoid IMs
Avoid trauma, falls, bumps, and cuts
Avoid contact sports
Avoid aspirin
Use electric razor
Use lubrication and gentleness during intercourse
Nutrients needed for RBC formation
Iron- meats, green leafy vegetables, seafood
Folic acid- dark green, leafy veggies, citrus foods, avocados, seeds, nuts
Vitamin B12- shellfish, cheese, eggs, veggie burgers
Dietary deficiencies that lead to impaired RBC production
Iron
Folic acid
B12
Pernicious anemia is a lack of what vitamin?
Vitamin B12
Another term for pernicious anemia
Macrocytic anemia (large cells)
Pernicious Anemia
Autoimmune dx
Abscence of the intrinsic factor due to destruction of parietal cells
-essential for absorptoin of vitamin B12, transport of vitamin B12 is prevented, and affects the frowth and maturation of RBCs
Pernicious anemia has a insidious onset after what age?
40; higher in women
Perniciuos anemia has highest incidence in what descent?
African
Scandinavian
What can you get pernicious anemia from?
Gastrectomy
Bowel resection
Crohn’s
Other autoimmune diseases
What kind of diet is required with pernicious anemia?
Diet that includes :
Citrus fruits
Leafy green vegetables
Fortified cereals
S&S of pernicious anemia
Soreness/burning of the tongue
Chronic B12 deficiency can cause irreparable nerve damage (B12 is needed for production of myelin)
Diagnositc tests for pernicious anemia
Schilling test
Serum megaloblastic anemia profile
Bone marrow aspiration (revelas abnormal RBC development)
Gastric analysis (decreased hydrochloric acid secretion)
Medical management of pernicious anemia
Lifelong B12 injections, folic acid supplements, and iron supplements
-1000 mg vitamin B12 IM for life
*Daily for 2 weeks, weekly for 1 month, monthly
for life
-Intra-nasal form of B12 once a week
PRBCs if anemia is severe
CBC- every 3-6 months for life
Death can occur in 1-3 years if no treatment
Who is at risk for Vitamin B12 deficiency?
Strict vegetarians
NIs for pernicious anemia
Mouth care- several times a day
High protein, vitamins, and minerals diet
Light weight blanket- cold sensitivity
Interventions should conserve energy and prevent injury
Teach about lifelong vitamin B12 treatment
Folic acid deficiency
Can occur in pregnancy
Can occur with sickle cell disease
ETOH can aggravate deficiency
Certain medications can cause it (ones used to treat cancer, RA, and seizures)
Amino acid sources
Eggs Meat Milk and milk products Poultry Fish Nuts Legumes
Folic acid sources
Green leafy veggies Asparugus, broccoli Organ meats Whole grain breads Enriched cereal Fish Legumes
Spina bifida
Linked to folic acid deficiency in the mother
Babies have early exposure to latex products during surgery and develop allegy
Another term for iron deficiency anemia
Microcytic anemia
smaller than normal cells
Iron deficiency anemia
Insufficent intake of iron, excess blood loss, or lack of stomach acid
What is the most common type of anemia in all ages?
Iron deficiency anemia
Infants and children who have a high milk intake are at a higher risk for what?
Iron deficiency anemia
What is the most common cause of iron deficiency anemia?
Excess iroin loss in adults
Inadequate intake of iron in children
What is a common source of iron deficiency anemia
Chronic intestinal or uterine bleeding
Iron deficiency anemia may be caused by
bleeding from gastric or duodenal ulcer Esophageal varices Hiatal hernias Colonic diverticulitis Tumors Celiac disease Sprue Malabsorption- less common
When can iron deficiency anemia occur?
When the body’s demand for iron exceeds it’s absorption
How much blood loss from upper GI is required for melena?
50-75 mLs
What is a common cause of iron deficiency anemia in young adult women?
Menstrual blood loss
Diagnositc tests for iron deficiency anemia
History- especially children Reiculocyte count CBC Serum iron levels- (Ferritin- major iron storage protein) \+ Direct Coombs antigobulin test
What does a direct coombs antiglobulin test look for?
Antibodies that may bind to RBCs and cause premature RBC destruction
Hemolysis
premature RBC destruction
Food sources of Iron
Organ meats Muscle meats Eggs Shellfish Whole-grain bread and cereal Iron-enriched breads and cereal Legumes Nuts Dried fruits Dark green vegetables Orange juice Grape juice
Medical management for iron deficiency anemia
Ferrous sulfate- 900 mg/day given with citrus fruits and juices;Oral or injection (z-track-staining) Iron administered orally or injection IM dextran or IV venofer Ascorbic acid Nutritional counseling Treatment of underlying conditions
NIs for iron deficiency anemia
Plan rest periods
Diet adequate in iron
Teach SE of iron therapy (dark, tarry stool)
Balance between rest/activites
Report N/D
Do not take with tetracycline antibiotics
Toxic to children
Teaching for iron deficiency anemia
Stew acidic foods in iron cookware
Can give iron-fortified formula
Pregnancy can deplete mother’s iron stores
For children, give liquid iron through a straw (avoids teeth staining)
Best solid food iron source for children is infant cereal
Infants need more than just milk
Erythropoietin
Protein made in the kidneys
Stimulates bone marrow to produce RBCs
Erythropoietin can:
Cause: hives, dyspnea, swelling of face, lips, tongue or throat
Increase: risk of blood clots
Hemolyteic anemias cause what?
Increased destruction of RBCs
Main types of hemolytic anemias
Sickle cell anemia
Thalassemia
More types of hemolytic anemias
G6PD deficiency Aquired hemolytic anemia Immune hemolytic anemia Malaria Tick-borne diseases Snake venom Toxic chemicals
Hemolytic anemia
Marrow produces enough RBCs, but they are destroyed
R/T defects of the cell membrane of RBC, inherited enzyme defects, or trauma
Causes of hemolytic anemia include
Infections
Drug reactions
Certain cancers
Delivery of a newborn with Rh+ blood while mom is Rh-
What is the most common genetic disorder?
Sickle cell anemia
What is sickle cell?
Abnormal crescent/sickle shape RBC containing hemoglobin S
Sickle cell anemia is a _____, _______, ______ condition
Severe;chronic;incurable
The sickle cell cases the RBC to be …..
Unable to carry as much O2, which causes pt. to become more sickled due to lack of O2
Crises cna be life threatening- Hypoxia and ischemia
Sickle cell anemia is seen in descendants from?
People of: Africa Saudi arabia India Meditarranean area
1 in _____ african americans have sickle cell anemia
400
Sickle cell trait occcurs in the person wtih…
One defective gene and one normal one
Often the carrier of ____________ shows no S&S
sickle cell anemia
Infants with sickle cell anemia do not show S&S until what age?
> 6 months
Sickles RBCs live how many days?
10-20 days
Sickle cell anemia crisis lasts how many days?
4-6 days; may come in clusters or far apart
What happens in sickle cell disease?
When O2 is released to the tissues, the abnormal hemoglobin becomes more viscous and crystallizes, causing the erythrocyte to change from a round shape to an elongated crescent shape.
As the sickles cells clump together, circulations slows, resulting in obstruction with severe hypoxia and necrosis
Precipitated by infection, fever, hypoxemia, dehydration, high altitudes, cold, or emotional stress
G6PD deficiency
Glucose-6-phosphate dehydrogenase
Genetic disorder
In affected individuals, a defect in the enzyme G6PD causes RBCs to break down prematurely.
G6PD deficiency occurs most often in?
Males
S&S of sickle cell anemia
Asymptomatic until a crisis Pain in joints and back Loss of appetite Dehydration Irritability with weakness Fatigue Fever Dyspnea Cough Strokes- in children Joint deformity Abdominal elargement (pooling in liver and spleen) Jaundice Extremity edema Skin irritation and ulceration Priapism
Complications of sickle cell anemia
Multisystem failure
Infarctions
Hemorrhage
Retinal damage
Diagnostic tests for sickle cell anemia
Hemoglobin electrophoresis (7-10g/dL)
Peripheral blood smear (shows sickled cells)
CBC
MRI
X-rays
Sickledex test (along with hgb electrophoresis, can determine whether trait or disease)
Medical management of sickle cell anemia
Supportive care
Alleviate S&S
Antibiotics
Pneumovax, haemophilus influenza and hepatits immunizations
Medical management of sickle cell anemia during a crisis
O2 Rest IVFs Pain management RBC exchanges (NOT TRANSFUSIONS) Folic acid Hydroxyurea therpay (reduces hemolysis) Bone marrow transplant
NIs for sickle cell anemia
Supportive care Hydration Frequent VS and O2 sats Analgesia Watch for fever and infection Blow nose gently Genetic counseling Good diet (increased need for folic acid) I&O
Evaluation of NIs for sickle cell anemia
Activity levels increase
Client reports no SOB on exertoin
Client explains treatment plan, meds, and diet
Client remains free of injury
Client teaching to avoid sickle cell crisis
Avoid high altitudes and air travel Increase fluid intake, avoid dehydration Notify MD of D/V High risk for pregnant women to develop pulmonary and/or renal conditions ROM, encourage physical activity as tolerated (but not during a crisis) Avoid trauma Avoid extreme temperatures Avoid ETOH and smoking Wear loose clothing Genetic counseling
Effects of sickle cell disease on the brain
Thrombosis
Hemorrhage
Stroke
Effects of sickle cell disease on the heart
Failure
Effects of sickle cell disease on the abdominal organs
Hepatomegaly
Gallstones (cholethiasis)
Splenic enlargement
Splenic infarction
Effects of sickle cell disease on the bones and joints
Hand and foot syndrome
Effects of sickle cell disease on the eye
Retinal or conjunctival hemorrhage
Blindness
Effects of sickle cell disease on the lungs
Atelectasis
Infarctoin
Pneumonia
Effects of sickle cell disease on the kidney
Dilute urine
Diuresis
Hematuria
Effects of sickle cell disease on the penis
Priapism
Effects of sickle cell disease on the skin
Stasis ulcers
Sickle cell crisis: Acute attack
What does it cause?
Causes occlusion and ichemia of distal blood vessels
Sickle cell crisis: Acute attack
Where is the pain felt?
Pain in hands and feet
Sickle cell crisis: Acute attack
What physical and emotional factors trigger an attack?
Dehydration Change in O2 tension (changes in altitude) Infection Fever V/D Overexertion Changes in climate (especially the cold) ETOH Smoking Stress
Sickle cell crisis: Aplastic crisis
What is it caused by?
Premature destruction of RBCs, = profound anemia
Sickle cell crisis: Aplastic crisis
Medical management
Transfusions of PRBCs
NOTE: watch for iron build up
Sickle cell crisis:Vaso-occlusive
Obstruction of the small blood vessels in the hands and feet, resulting in edema, impaired ROM, and pain
Sickle cell crisis:Sequestration
Blood pools in the spleen and liver resulting in hepatosplenomegaly; can progress to CV collapse and death
Sickle cell crisis:Vaso-occlusive
Palliative Tx:
Pain meds
O2
Sickle cell crisis:Sequestration
Treatment
Pain meds
Volume expanders
Transfusions
Splenectomy
Sickle cell crisis nursing Dx of pain
Pain r/t thrombotic crisis
Sickle cell crisis nursing Dx of pain interventions
Position in alignment Protect joints Position with slow, gentle movements Apply warm, moist compresses to relieve discomfort Administer and monitor pain meds
Sickle cell crisis nursing Dx of impaired skin integrity
Impaired skin integrity r/t alteration in circulation to tissues, resulting in hypoxia and inadequate nurtition
Sickle cell crisis nursing Dx of impaired skin integrity interventions
Remove constrictive clothing
Maintain room and body warmth
ROM
Inspect extremities for adequate circulation
Monitor gases for indictors of adeqaute tissue perfusion
Thalassemia
Insufficient production of normal Hgb - abnormal synthesis
Recessive genetic disorder
Thalassemia affects what ethnic groups?
Ethnic groups with origins near the mediterranean area
Thalassemia has what kind of forms
Major and minor
Thalassemia is AKA..
Cooley’s anemia
Treatment for major thalassemia
Blood transfusions
Folate therapy
Chelation therapy
Bone marrow transplant- may help
Treatment for minor thalassemia
None-
Body adapts to reduction of normal Hgb
Anemia of Chronic Infection/Disease
ACI or ACD
Human body limits the amount of iron available when potentially harmful things get into our body
Bacteria and cancer cells depend upon iron to sustain life
Iron gets shuttle to ferritin so the harmful invader cannot get the iron
ACI or ACD low normal range
9.5-10.5 g/dL
Anemia of Chronic Renal Failure
Kidneys are unable to secrete the hormone erythropoietin
Toxins may also depress bone marrow
Aluminum may accumulate in the bone marrow of dialysis patient and can contribute to anemia
Disseminated Intravascular Coagulation (DIC)
Overstimulation of clotting and anti-clotting processes in response to disease and injury
Clotting and hemorrhage issues
DIC is caused by
Infection (sepsis) Obstetric complications Neoplastic disorders Hematological disorders Trauma
Clots normally lyse in ______ while in DIC clots lyse_____
7-10 days; immediately
S&S of DIC
Bleeding GI bleeding Hemoptysis Hematuria Dyspnea Pulmonary edema Diaphoresis PE Cold, mottled digits Purpura on the chest and abdomen- 1st sign Petechiae Hypotension Abdominal tenderness Tachycardia Absent peripheral pulses Restlessnes Confusion Seizures Coma
Diagnostic tests for DIC
Decreased fibrinogen levels
Increased PT and PTT
Increased fibrin split products
Increased D-Dlmers
Treatment of DIC- Supportive
Administer blood FFP Platelets Vitamin K Cryoprecipitate
Treatment of DIC- controversial
IV heparin
Nurse teaching for DIC
Involve the family
Critical situation= IVs, N/G, Foley
Emotional support
Thrombocytopenia
Idiopatic Thrombocytopenia Purpura (ITP)
Platelets reduced below 150,000/mm3; may be due to decreased production or decreased survival
Autoimmune disease, platelets are coated with antibodies. Although they function normally, are destroyed by macrophages in the spleen
ITP is assosciated with…
Aplastic anemia Leukemia Tumors Chemo Infection Antibody destruction Viral overload Drug induced
ITP chronic form in common in who?
Women aged 20-40
Acute form common in children (ITP)
Bleeding beneath the skin
Follows viral infection
S&S of ITP
Petechiae Ecchymosis Bleeding from mucous membranes Splenomegaly from entrapment of blood in the spleen Bleeding in brain Nosebleeds
Diagnostic tests for ITP
CBC
Peripheral blood smear
Coags
Bone marrow aspiration (may show that precursor cells for platelets, the magarkaryocytes, are adequate)(Platelets live for a few hours rather than 7-10 days)
Medical management of ITP
Corticosteroid therapy (prolongs platelet life) Splenectomy IV immunoglobulin Immunosuppressive drugs PRBCs to treat hemorrhage Platelet transfusions (counts <20K) Vitamin K Anti-D antibody therapy (new)
Meds assosciated with thrombocytopenia
ASA Digitalis Lasix NSAIDs ORal hypoglycemics PCN Quinidine Rifampicin Sulfonamides Thiazides
NIs for thrombocytopenia
Meticulous aseptic technique
Careful positioning and moving of client
Watch for S&S of increased ICP
Bleeding precautions
*Avoid trauma, soft toothbrush, high fiber diet
blow nose gently
Transfusions- monitor for reactionHeparin Induced Thrombocytopenia (HIT)
Development of thrombocytopenia due to heparin administration
HIT can predispose to thrombosis
What is HIT caused by?
Abnormal antibodies that activate platelets
Hemophilia
Hereditary coagulation disorder
Distrubance of clotting factor
2 types- A and B
X-linked hereditary trait (only affects males, women are carriers though)
Hemophilia A
most common
Absence of factor VIII
Hemophilia B
Christmas disease
Deficiency in factor IX
Von Wilebrand’s disases
Mild factor VIII
Hemophilia- what happends after repeated bleeds?
Muscles become weak, scarred, and shorter than normal (sometimes permanently)
They can no longer protect the joints
Hemophilia- joints above and below the muscle cannot move prperly. They may ______
bleed more often
Hemophilia- what happends if nerves are damaged during muscle bleeds?
Muscle may become weake or paralyzed
Diagnositc tests for hemophilia
Normal INR and PT
Prolonged aPTT
Factors VIII and IX are absent
S&S of hemophilia
Internal & External bleeding
Ecchymoses from slight trauma
Hemarhrosis (indicates severe disease; seen in knees ankles and elbow; exibits pain, edema, erythema and fever)
Excessive blood loss from small cuts and dental procedures
Medical management of hemophilia
Minimize bleeding- avoid trauma Relieve pain- no aspirin Minor bleeds- ice packs Transfusions (child needs Hep B vaccine) Factor VIII or IX concentrate Cryoprecipitate (rich in factor VIII) Manufactured VIII or IX (less risk of viral transmission)
Treatment for Hemophilia A
Inhalation of desmopressin (DDAVP) which can stimulate body to release more clotting factors
Treatment for hemophilia B
Transfuse factor IX
NIs for hempophilia
Apply pressure & cold (ice) Control hemorrhages Educate family Monitor transfusions Pain management Genetic counseling No ASA products Avoid injury Medic alert bracelet Diet Dental care & preventive dental care Non-contact sports Soft, age appropriate toys Immobilize affect part Notify MD Teach good diet to avoid obesity Avoid over protection
Von Willebrand’s disease
Inherited bleeding disorder
Characterized by abnormally slow coagulation of blod
Mild deficiency of factor VIII
Common in post-partum period
How is Von Willebrand’s disease different from hemophilia?
VW’s disease is not limited to males like hemophilia
S&S of von willebrand’s disease
Spontaneous episodes of:
GI bleeding
Epistaxis
Gingival bleeding
Primary polycythemia
Polycythemia Vera (PV)
How are primary and secondary polycythemia different and similiar?
Etiologies and pathophysiology differe although S&S and compliactions are similiar
Polycythemia Vera
Myeloproliferative- characterized by excessive bone marrow production
S&S of PV
Increase in erythrocytes, granulocytes, and platelets
Stem cell abnormality
Pathologic response is a malignancy of blodd cells
Secondary polycythemia
Caused by hypoxia
Hypoxia stimulates erythropoietin in the kidneys, which in turn stimulates erythrocyte production (people who live in mountains, COPD)
S&S of secondary polycythemia
Gradual onset Mainly affects men in middle age Venous distention and platelet dysfunction can cause organ infarction Florid or dark, flushed face Esophageal varices Epistaxis GI bleeding Petechiae
Medical management of polycythemia
Repeated phlebotomy
Myelosuppressive agents, such as Myleran, Hydrea, and radioactive phosphorus are often given to inhibit bone marrow activity
Main cause of morbidity and mortality is thrombosis
Nursing actions for polycythemia
Controlling chronic pulmonary disease and avoiding high altitudes (secondary)
Proper hydration to avoid fluid overload
NIs for the nursing DX Ineffective tissue perfusion r/t polycythemia
Comfy position
Provide passive ROM q2-4 hours
Report S&S of thrombosis
Avoid trauma
NIs for the nursing DX activity inolerance r/t polycythemia
Avoid high NA+ food
Encourage adequate exercises
Explain disease course
Lymphadenopathy
Swelling of lymph glands
Lymphagenopathy causes
Infection
Cancer
Autoimmune disease
Lymphangitis
Inflammation of one or more lymphatic vessels
S&Sof lymphangitis
Clinical symptom of infection important* Fine red streaks from affected area Groin or axilla Not localized- edema is diffuse Chills Fever Local pain H/A Myalgia
Medical management/NIs of lymphadenopathy/lymphangitis?
PCN or other antibiotics
Moist heat to area
Elevate extremity
Use aseptic technique
Lymphedema
Primary or secondary disorder
Accumulation of lymph in soft tissue due to obstruction, increased amount of lymph in area of removal of nodes(usually occurs for strep or staph infection in an extremity)
Extreme edema &tightness resulting in pain and pressure
Often a result of mastectomies
Lypmphedema- extreme edema & tightness in affected extremity resulting in pain and pressure is aggravated by?
Standing pressure
Pregnancy
Obesity
Warm environments
Medical management/NIs for lymphedema
Diuretics Antibiotics SCDs Elastic stocking or sleep Elevate extremity Restricted Na+ diet Avoid constrictive clothing/trauma Meticulous skin care Be supportive- body image disturbance may occur
Nursing problems for blood and lymphatic disorders
Risk for infection Risk for injury (trauma, bleeding, falls) Knowledge deficit Pain (acute,chronic) Impaired gas exchange Activity intolerance Ineffective coping Impaied skin integrity Disturbed body image
Splenomegaly
Enlarged spleen
May not enlarge, but still destroys RBC and platelets?
Splenectomy problems
Bleeding Pneumonia Atelectasis Pancreatitis Fistulas OPSI- overwhelming post-op infection (1 week - 20 years)
Cullen’s sign
Sign of retroperitoneal bleeding/hemorrhage
Superficial edema and bruising in the subQ fatty tissue around the umbilicus
Grey-Turner’s sign
Sign of retroperitoneal bleeding/hemorrhage
Takes 24-48 hours to appear
Bruising/blue discolaration of the flanks
***A flank = the side of a person’s body between ribs and hip! Who knew?! Not me!
Kehr’s sign
Acute pain in the tip of the shoulder due to the presence of blood or other irritants in the peritoneal cavity when a person is lying down with the legs elevated
**Pain in left shoulder is sign of a ruptured spleen
Neutropenic precautions
Reverse isolation No raw meats, veggies, or fruit No fresh flowers Visitors= cannot show any S&S of infection; not a lot of visitors Good handwashing
