Sickle Cell Disease
Pathophysiology Clinical Features
• Autosomal recessive gene Characterized by periodic exacerbations (ie crises)
- Both parents must have this gene • Vaso occlusive: painful distal ischemia (hands and feet)
to be able to pass it down • Sequestration crisis: pooling of blood in liver and spleen
• Alterations in hemoglobin occur • Hyper hemolytic crisis: marked anemia d/t massive
d/t certain conditions (eg destruction of RBCs
deoxygenation, dehydration)
• RBCs forced into stiffened and Assessment Findings
elongated crescents (ie sickles) • Tachycardia: d/t hypoxemia, pain and infection
• Sickled cells clump together → • Leukocytosis: occurs with inflammation of tissues injured by
occlude small blood vessels → ischemia (WBC >10,000)
ischemia and possible organ • Anemia: RBC destruction
damage • Increases reticulocyte (baby) count: indicative of RBC
destruction
Nursing Interventions Oxygenation
Diet • Prevent pulmonary complications and provide
• High-protein, high calorie diet in addition to a comfort
multivitamin without iron Pain control
- No iron because RBCs are breaking down and • Analgesics scheduled around the clock or
releasing iron into the blood continually (eg patient-controlled analgesia)
Folic acid rather than as needed, to prevent
• Stimulation of erythropoiesis, which is needed breakthrough pain
d/t increased hemolysis Tissue perfusion
Hydration • Monitor hemoglobin levels and administer
• Aggressive IV and oral hydration to reduce blood blood products as indicated
viscosity
Infection prevention
• Vaccinations
, Anaphylactic Shock
Pathophysiology Common Allergens Clinical Features
Life-threatening immediate • Foods • Skin: flushing,
hypersensitivity reaction • Peanuts urticaria, swollen lips
• Often occurs from a repeat exposure • Shellfish and tongue
to an allergen - Warm and wet rash
• Usually immunologic and igE- Food additives • Respiratory: dyspnea,
mediated • Red dyes stridor, wheezing
• Activation of eosimophils, mast cells, • Cardiac: hypotension,
basophils Insect stings arrhythmias, syncope
• Excessive release of cytokines • Bees • Abdominal: crampy
(histamine, nitric oxide) pain, diarrhea,
- Vasodilation Biologic materials vomiting
- Increases systemic capillary • Vaccines
permeability • Monoclonal antibodies
“Leaky hose” • Natural rubber latex
- Laryngospasm • Blood transfusion
- Bronchospasm
Nursing Interventions • Administer adjunctive medications
• Immediately stop infusion if client is - Albuterol (salbutamol)
receiving blood transfusion or medications - Corticosteroids (eg methylprednisolone)
• Activate emergency response team - Antihistamines (diphenhydramine)
• Administer IM epinephrine • Initiate continuous cardiac monitoring
- Inject into mid-outer thigh • Maintain NPO until symptoms resolve to prevent
- Repeat q5-15m until client responds aspiration
• Place client in recumbent position and • Encourage client to obtain an epinephrine
elevate lower extremities autoinjector for emergency use
• Administer oxygen via facemask PRN • Instruct client of proper use of epinephrine
• Administer IV 0.9% NaCl autoinjector
• Ensure intubation equipment is available • Ensure client knows signs/symptoms of anaphylaxis
and importance of contacting rapid response team
Epinephrine Autoinjector
Emergency treatment of anaphylaxis
• Administration
• Hold pen at 90 degree angle firmly in dominant hand
• Remove safety cap
• Press orange tip firmly into outer thigh
• Hold in place for 10s
Client education
• Each pen has one use
• Can be administered through clothing
• Store at room temperature
• Call 911 after use
Pathophysiology Clinical Features
• Autosomal recessive gene Characterized by periodic exacerbations (ie crises)
- Both parents must have this gene • Vaso occlusive: painful distal ischemia (hands and feet)
to be able to pass it down • Sequestration crisis: pooling of blood in liver and spleen
• Alterations in hemoglobin occur • Hyper hemolytic crisis: marked anemia d/t massive
d/t certain conditions (eg destruction of RBCs
deoxygenation, dehydration)
• RBCs forced into stiffened and Assessment Findings
elongated crescents (ie sickles) • Tachycardia: d/t hypoxemia, pain and infection
• Sickled cells clump together → • Leukocytosis: occurs with inflammation of tissues injured by
occlude small blood vessels → ischemia (WBC >10,000)
ischemia and possible organ • Anemia: RBC destruction
damage • Increases reticulocyte (baby) count: indicative of RBC
destruction
Nursing Interventions Oxygenation
Diet • Prevent pulmonary complications and provide
• High-protein, high calorie diet in addition to a comfort
multivitamin without iron Pain control
- No iron because RBCs are breaking down and • Analgesics scheduled around the clock or
releasing iron into the blood continually (eg patient-controlled analgesia)
Folic acid rather than as needed, to prevent
• Stimulation of erythropoiesis, which is needed breakthrough pain
d/t increased hemolysis Tissue perfusion
Hydration • Monitor hemoglobin levels and administer
• Aggressive IV and oral hydration to reduce blood blood products as indicated
viscosity
Infection prevention
• Vaccinations
, Anaphylactic Shock
Pathophysiology Common Allergens Clinical Features
Life-threatening immediate • Foods • Skin: flushing,
hypersensitivity reaction • Peanuts urticaria, swollen lips
• Often occurs from a repeat exposure • Shellfish and tongue
to an allergen - Warm and wet rash
• Usually immunologic and igE- Food additives • Respiratory: dyspnea,
mediated • Red dyes stridor, wheezing
• Activation of eosimophils, mast cells, • Cardiac: hypotension,
basophils Insect stings arrhythmias, syncope
• Excessive release of cytokines • Bees • Abdominal: crampy
(histamine, nitric oxide) pain, diarrhea,
- Vasodilation Biologic materials vomiting
- Increases systemic capillary • Vaccines
permeability • Monoclonal antibodies
“Leaky hose” • Natural rubber latex
- Laryngospasm • Blood transfusion
- Bronchospasm
Nursing Interventions • Administer adjunctive medications
• Immediately stop infusion if client is - Albuterol (salbutamol)
receiving blood transfusion or medications - Corticosteroids (eg methylprednisolone)
• Activate emergency response team - Antihistamines (diphenhydramine)
• Administer IM epinephrine • Initiate continuous cardiac monitoring
- Inject into mid-outer thigh • Maintain NPO until symptoms resolve to prevent
- Repeat q5-15m until client responds aspiration
• Place client in recumbent position and • Encourage client to obtain an epinephrine
elevate lower extremities autoinjector for emergency use
• Administer oxygen via facemask PRN • Instruct client of proper use of epinephrine
• Administer IV 0.9% NaCl autoinjector
• Ensure intubation equipment is available • Ensure client knows signs/symptoms of anaphylaxis
and importance of contacting rapid response team
Epinephrine Autoinjector
Emergency treatment of anaphylaxis
• Administration
• Hold pen at 90 degree angle firmly in dominant hand
• Remove safety cap
• Press orange tip firmly into outer thigh
• Hold in place for 10s
Client education
• Each pen has one use
• Can be administered through clothing
• Store at room temperature
• Call 911 after use
