Concept Map Sickle Cell Anemia

RISK FACTORS (Lifestyle & Medical) PATHO: Describe the Patient Condition  African American  Sickle cell disease is an inherited red blood cell disorder (autosomal recessive). People who have sickle cell disease  have an abnormal protein in their red blood cells. In the US, most people who have sickle cell disease are of African   Hispanic background descent, but the condition is also common in those with a Hispanic background.  Family history of the disorder (genetic)  If you have the trait but not the full blown disease, visiting high altitudes (e.g. Denver, CO) or being in extreme hot or cold temperatures can trigger a crisis Sickle cell disease is caused by several mutations in the β-globin gene. These mutations cause the 6th amino acid to be changed from glutamic acid to valine. The hemoglobin that’s left (called HbS) has abnormal properties. The hemoglobin, which is supposed to be round in shape, becomes a sickle or C-shape. These abnormally shaped cells do not hold enough oxygen to sustain what the body needs. The sickle cells also cannot pass through the person’s capillaries (especially the really small ones in their hands and feet) which causes them to clump together and cause vaso-occlusion. This causes extreme pain for the patient, due to the anoxia in the tissue. This pain crisis can be triggered by things such as stress, high altitudes, extreme hot and cold, and most commonly, dehydration.      DIAGNOSTICS / LABS & RATIONALE     Hemoglobin electrophoresis - a blood test that Sickled RBCs can appear normal in shape after the blood oxygen level is normalized, however the damage is not reversed. As a result, a sickle cell survives about 20 days, whereas a normal RBC lives up to 120 days. can detect different types of hemoglobin High performance liquid chromatography (HPLC) - identifies which type of hemoglobin (Ward, pages 193, 373) is present; to confirm the results of HPLC, a genetic test may be done A SickleDex test – tests for the presence of sickling hemoglobins; cannot distinguish PAST MEDICAL HISTORY: Related to Primary Diagnosis sickle cell trait from sickle cell disease CBC – indicates the number of red blood cells as well as the amount of hemoglobin and will evaluate the size and shape of the RBCs present o Reticulocyte count will be high o WBCs may be elevated TREATMENT & RATIONALE         There is no cure, just management options Pain-relieving medications - to relieve pain during a sickle cell crisis o Opioids o NSAIDS Advise the patient to drink lots of water to stay hydrated – this helps prevent pain crisis episodes Antibiotics - children with sickle cell may begin taking the antibiotic penicillin when they're about 2 months old and continue taking it until they're at least 5 years old (because they have most vaccines by then) Hydroxyurea (Droxia, Hydrea) – daily medication that works for some sickle cell patients; stimulates production of fetal hemoglobin Keeping children UTD on all vaccines to help prevent infection Blood transfusions - they increase the number of normal RBCs in circulation, helping to relieve anemia Bone marrow transplant – not as common unless the patient has significant symptoms; healthy donor stem cells are injected IV into the bloodstream, where they migrate to the bone marrow and begin generating new blood cells     NURSING DIAGNOSES (Top Three)  Acute pain R/T intravascular sickling with localized stasis and occlusion AEB facial grimacing and patient verbally states pain is 10/10.  Impaired gas exchange R/T abnormal RBC structure AEB dyspnea and use of accessory muscles.  Risk for deficient fluid volume R/T increased fluid needs (i.e. – hyper-metabolic state/fever, inflammatory processes) POTENTIAL COMPLICATIONS Pain crisis Splenic Sequestration Damage the heart, brain, lungs, kidney, spleen, and other organs of the body  Jaundice  Wounds that won’t heal  Death    NURSING CARE/ INTERVENTIONS/EDUCATION (As Related to the Top Three Nursing Diagnoses) Priority Medical Diagnosis Sickle Cell Disease     SIGNS & SYMPTOMS Fatigue and anemia Pain Dactylitis (swelling and inflammation of the hands and/or feet) and arthritis Bacterial infections Sudden pooling of blood in the spleen and liver congestion Lung and heart injury Leg ulcers Aseptic necrosis and bone infarcts (death of portions of bone) Dark urine Stunted growth Stroke Eye damage               Assess for pain. Note location, duration, and intensity (scale of 0-10). Observe nonverbal pain cues: gait disturbances, positioning of the body, guarding behavior, facial grimacing, reluctance to move, and physiological manifestations of acute pain (increased BP, tachycardia, increased RR). Discuss what pain relief measures were effective in the past. Involve the patient and family in care. Teach and discuss alternative pain relief measures: relaxation techniques, biofeedback, yoga, meditation, progressive relaxation techniques, distraction techniques, guided imagery and breathing techniques. Apply warm, moist compresses to affected joints and other painful areas. Avoid use of ice or cold compresses. Maintain adequate fluid intake; Administer IV fluids as indicated. Monitor respiratory rate, depth, use of accessory muscles, and areas of cyanosis. Check thoroughly reports of chest pain and increasing fatigue. Monitor for signs of fever, couch, and adventitious breath sounds. Regularly assess level of consciousness. Schedule rest periods and encourage patient to alternate rests and activity. Administer supplemental humidified oxygen as indicated. Administer packed RBCs or exchange transfusion as ordered. Administer medications as indicated Maintain accurate I&O and weigh daily. Note urine characteristics and specific gravity. Monitor vital signs, comparing with patient’s previous readings. Measure BP in lying, sitting, and standing positions if possible. Assess patient for fever, changes in level of consciousness, poor skin turgor, dryness of skin and mucous membranes, pain. Monitor laboratory studies: Hgb/Hct, serum and urine electrolytes.