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Objectives Image source: Microsoft clipart

Sickle Cell Disease Sickle cell disease Inherited disorder that affects red blood cells Sickle cell trait Mainly affects African Americans and Hispanic Americans Average life expectancy is about 45 years Sickle cell disease is an inherited disorder that affects red blood cells. Individuals who have sickle cell disease inherit two copies of the sickle cell gene—one from each parent. An individual who inherits a sickle cell gene from one parent and a normal gene from the other parent has sickle cell trait. An individual who has sickle cell trait does not have sickle cell disease, but he or she has one of the genes that cause the disease. Sickle cell disease mainly affects African Americans and Hispanic Americans. In the United States, the average life expectancy for an individual with sickle cell disease is about 45 years of age.

Normal Red Blood Cells Bone marrow produces red blood cells Smooth, round Move easily through blood vessels Live about 120 days Image source: Microsoft clipart

Sickle Cell Disease Body produces a defective hemoglobin molecule Red blood cells become stiff and shaped like a sickle, or “C” Sickled red blood cells usually die after about 10 to 40 days Sickle cell anemia In sickle cell disease, the body produces a defective hemoglobin molecule that causes red blood cells to become stiff and shaped like a sickle, or “C.” Unlike normal red blood cells, sickled red blood cells usually die after about 10 to 40 days. Because the bone marrow is unable to produce new red blood cells fast enough to replace the dying ones, a lower-than-normal number of red blood cells are present in the body, resulting in sickle cell anemia. Anemia is a condition in which the blood has less than the normal number of red blood cells or less than the normal quantity of hemoglobin in the blood. In either case, the oxygen-carrying capacity of the blood is decreased.

Sickle Cell Crisis Sickled red blood cells Carry less hemoglobin than normal RBCs Do not move easily through blood vessels Have a tendency to stick together and form clumps Sickle cell crisis Sickled red blood cells carry less hemoglobin than normal red blood cells and do not move easily through blood vessels. They are stiff and have a tendency to stick together and form clumps, subsequently getting stuck in the blood vessels. A sickle cell crisis occurs when clumps of sickled cells build up in the blood vessels, blocking blood flow to organs and limbs. Impaired circulation causes pain, swelling, infection, and organ damage and death. There is no cure for sickle cell disease at this time.

Assessment Findings and Symptoms Signs and symptoms usually do not develop until after age 4 months Image source: Microsoft clipart Although sickle cell anemia is present at birth, signs and symptoms of the disease usually do not develop until after age 4 months. This is because an infant’s blood has a large amount of fetal hemoglobin, and therefore a higher oxygen concentration, that protects the infant during this time and inhibits sickling.

Assessment Findings and Symptoms Fatigue Most common symptom of sickle cell anemia Pain of sickle cell crisis Acute (sudden) Mild to severe Usually lasts hours to several days Chronic (long-term) Typically lasts for weeks to months

Assessment Findings and Symptoms Hand-foot syndrome Results from the blockage of blood vessels in the hands or feet by sickle cells Painful swelling Affect one or both hands and/or feet at the same time Hand-foot syndrome is one of earliest changes of sickle cell disease and results from the blockage of blood vessels in the hands or feet by sickle cells. It usually starts with painful swelling, affecting one or both hands and/or feet at the same time. Swelling typically occurs on the back of the hands and tops of the feet and moves into the fingers and toes.

Assessment Findings and Symptoms Ulcers (sores) develop on the lower third of the leg in some patients with sickle cell disease. The cause is unknown. Ulcers (sores) develop on the lower third of the leg in some patients with sickle cell disease. The cause of leg ulcers is unknown.

Assessment Findings and Symptoms Acute chest syndrome Occurs because of an infection or blockage of the blood vessels in the lungs by blood clots or sickled red blood cells Signs and symptoms Fever Chest pain Coughing Difficulty breathing Acute chest syndrome is a complication of sickle cell disease that can occur because of an infection or blockage of the blood vessels in the lungs by blood clots or sickled red blood cells. Signs and symptoms are similar to those of pneumonia and include fever, chest pain, coughing, and difficulty breathing.

Assessment Findings and Symptoms Splenic crisis Sickled red blood cells become trapped in the spleen A leading cause of death in children with sickle cell disease

Assessment Findings and Symptoms Scene size-up Ensure your safety Put on appropriate PPE Request advanced life support personnel Conduct a scene size-up and ensure your safety. Put on appropriate PPE. Prehospital care for a patient experiencing a sickle cell crisis is supportive. Request advanced life support to the scene as soon as possible. If the patient’s vital signs are stable, ALS personnel will be able to administer medications to help ease the patient’s discomfort en route to the hospital.

Assessment Findings and Symptoms Primary survey Position of comfort Perform a physical examination Vital signs, medical history Reassess as often as indicated Document Perform a primary survey to identify and treat any life-threatening conditions. Manage the patient’s airway and breathing. Allow the patient to assume a position of comfort and provide calm reassurance. Administer oxygen. If the patient’s breathing is inadequate, give positive-pressure ventilation with 100% oxygen. Assess the adequacy of the ventilations delivered. Perform a physical examination. Take the patient’s vital signs and gather the patient’s medical history. En route to the hospital, make the patient as comfortable as possible, provide reassurance, and keep him warm. Reassess as often as indicated until patient care is turned over to ALS personnel or medical personnel at the receiving facility. Record all patient care information, including the patient’s medical history and the emergency care provided, on a PCR.

Hemophilia

Hemophilia Hemophilia Inherited bleeding disorder Caused by an abnormality of a blood- clotting factor Can be acquired if an individual forms antibodies to the clotting factors in his or her bloodstream Usually occurs in males The normal blood clotting process involves a number of different clotting factors that work with platelets to stop bleeding. Hemophilia is an inherited bleeding disorder caused by an abnormality of a blood-clotting factor. Hemophilia also can be acquired if an individual forms antibodies to the clotting factors in his or her bloodstream. Hemophilia usually occurs in males, only rarely occurring in females.

Assessment Findings and Symptoms Primary signs and symptoms Easy bruising Excessive bleeding External Internal Signs and symptoms of hemophilia vary depending on the severity of clotting factor deficiency. The primary signs and symptoms of hemophilia are easy bruising and excessive bleeding. Bleeding may be external or internal. External bleeding can be seen. Possible causes of excessive external bleeding in the patient who has hemophilia include a nosebleed with no obvious cause, unexplained bleeding or bruising; or prolonged bleeding from a tooth extraction, cut, or after surgery.

Assessment Findings and Symptoms Internal bleeding Is not visible Occurs inside body tissues and cavities Bleeding in the brain Bleeding into the muscles and joints Internal bleeding is not visible because it occurs inside body tissues and cavities. Signs and symptoms of internal bleeding can include chest or abdominal pain, hematuria, hematemesis, or tarry stools. Bleeding in the brain is a complication of hemophilia. Signs and symptoms can include altered mental status, neck pain or stiffness, seizures, extreme fatigue, double vision, long-lasting headache, repeated vomiting, difficulty walking, or arm or leg weakness. Because bleeding into the knees, elbows, hips and shoulders, and muscles of the arms and legs can also occur, be sure to assess the length of each extremity carefully. Initially, bleeding into a joint may cause complaints of “tightness” in the joint, but no pain. As bleeding continues or when sudden bleeding occurs, the joint feels warm to the touch, swelling is present, and joint movement is impaired.

Emergency Care Scene size-up Ensure your safety Put on appropriate PPE Make sure suction is within arm’s reach at all times Conduct a scene size-up and ensure your safety. Put on appropriate PPE. Perform a primary survey to identify and treat any life-threatening conditions. Manage the patient’s airway and breathing. Allow the patient to assume a position of comfort and provide calm reassurance. Administer oxygen. If the patient’s breathing is inadequate, give positive-pressure ventilation with 100% oxygen. Assess the adequacy of the ventilations delivered. A patient with internal bleeding may vomit. Watch the patient closely to make sure his airway remains clear. Make sure that you have suction within arm’s reach at all times. Suction as needed.

Emergency Care Perform a physical examination Vital signs and medical history Assess carefully for signs of bruising, obvious bleeding, and signs that suggest internal bleeding Control external bleeding, if present. Reassess as often as indicated Record all patient care information on a PCR. Perform a physical examination. Take the patient’s vital signs and gather the patient’s medical history. Assess the patient carefully looking for signs of bruising, obvious bleeding, and signs that suggest internal bleeding. Control external bleeding, if present. Be alert for signs of recurring bleeding if bleeding has been controlled. If signs of shock develop, place the patient in a supine position and transport as soon as possible to the closest appropriate facility. En route to the hospital, make the patient as comfortable as possible, provide reassurance, and keep him warm. Reassess as often as indicated until patient care is turned over to ALS personnel or medical personnel at the receiving facility. Record all patient care information, including the patient’s medical history and the emergency care provided, on a PCR.

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