Sickle Cell Anemia
Care and Treatment
Although contacts with Sickle Cell Anemia patients are rare it's important to know a few things about this chronic condition that can insure that we give the best patient care possible when the need arises.
Take time to review this short tutorial and complete the test for credit.
Facts about Sickle Cell Anemia
Definition
Sickle Cell Anemia is a genetic disorder caused from an abnormal type of hemoglobin molecule. Hemoglobin that doesn't bind with oxygen thickens in the cell and stretches or elongates it. This is called "sickling". Sickling occurs when oxygen is decreased in the blood due to stressors. The sickled cells stiffen and cannot pass through the body's circulation.. This causes plugging of the blood vessels leading to numerous complications such as vascular occlusion, pain, organ infarction from lack of oxygen, skin ulcerations and cronic kidney disease.
Diagnosis
Sickle Cell Anemia is genetic so the baby is tested at birth to six months.
Maintenance
Routine blood work- CBC ( complete blood count) for hemoglobin level to rule out anemia, creatine level for kidney function, and liver function test. Urinalysis and chest x-ray to rule out infiltrates.
Prevention
Avoid infection. Receive immunizations such as flu/pneumonia shots, and antibiotics. Control fever with acetaminophen rather than aspirin ( to avoid metabolic acidosis ) Fluids to avoid dehydration . Avoid constricting clothing as it may decrease circulation. Avoid cold temperatures as it may decrease circulation. Nutritional supplements- vitamins, folic acid, and iron (to prevent anemia) health teaching to the patient family.
Types of Sickle Cell Crisis
- Vasooclusive - Blood flow obstructs small blood vessels by sickled cells. The obstruction causes less blood flow leading to tissue death from lack of oxygen. Causes consist of exposure to the cold, dehydration, and lack of oxygen (hypoxia) signs and symptoms consist of joint swelling, priapism, weakness/pralysis (stroke)
- Sequestation - Blood pools in the liver and the spleen. Sickled cells go to the spleen for hemolisis, but build up causes pooling and eventually infarction of the spleenic vessels. Cause is dehydration therefore treatment includes rehydtration as well as blood transfusion, and spleenectomy.
- Aplastic - Anemia from decreased hemoglobin with hypo plastic bone marrow. Lost blood cells are not replenished. Cause is viral
- Hyperhemolytic - Increased rate of red blood cell destruction causes decreased hemoglobin and hematicrit ( % of hemoglobin in blood). Causes are infevtion and certain medications.
Chronic Symptoms
Blindness, pulmonary hypertension, tachypnea, CHF, cardiac ischemia, priapism, skin ulcers, osteoporosis.
Treatment on the Scene
- Pt’s chief complaint and duration of.
- Determine type of Sickle Cell crisis
- Patient’s history, recent hospitalizations, current meds
- Head to toe assessment- nuerological-lethargy, weakness, paralysis,( may have a hemorrhage or thrombosis)
- Cardiovascular - vital signs, EKG. Obtain IV access
- Pulmonary - respiratory distress, hypoxia, SAo2 administer oxygen (rule out pneumonia, or pneumothorax-.
- Gastrointestinal - check for liver and spleen enlargment
- Genitourinary - Blood in urine
- Muscloskeletal - bone/joint swelling( Skin assess for leg ulcers)
- Pain Assessment - narcotics for pain control, also replenish fluids without over hydrating.