Introduction If you practice nursing on a pediatric unit, you are likely to encounter patients with sickle cell disease. It is important to understand the underlying causes and the complications of the disease. Acute chest syndrome is one of the leading causes of death associated with sickle cell disease. It is critical to understand how to care for these patients holistically. Pathophysiology Sickle cell disease is a genetic condition that causes a mutation in the hemoglobin molecule. Normal red blood cells are round and flexible. In a person with sickle cell anemia the blood cells are stiff and sticky. This causes the cells to be misshapen and can become stuck within blood vessels. The disease process also causes a lack of healthy red blood cells to carry adequate oxygen throughout the body (Porth, 2015). Acute chest syndrome is a type of pneumonia caused by these sickled cells blocking blood vessels in the lungs. The syndrome can cause chronic respiratory insufficiency and is a leading cause of death in sickle cell disease (Porth, 2015). Laboratory and Diagnostics Sickle cell anemia is diagnosed by performing a blood drawn to check for hemoglobin S, the defective form of hemoglobin. Blood cells can also be viewed using microscopy to view the sickled cells. Acute chest syndrome can be diagnosed by chest x-ray. The sickled cells that obstruct blood flow to the lungs manifests as infiltrates on the images. After diagnosis, hemoglobin and hematocrit levels are
Sickle Cell Anemia is a group of disorders that cause red blood cells to become misshapen and break down. Sickle Cell Anemia affects many people all over the world; Sickle cell disease is the most common inherited blood disorder in the United States, affecting 70,000 to 80,000 Americans (Ashley-Koch, Yang and Olney). Sickle Cell Anemia causes your red blood cells to be thin, stiff, and shaped like a sickle. But your red blood cells are supposed to be round and soft. When a person is diagnosed with sickle cell anemia the blood cells start to become clogged blood vessels, which can cause a great amount of health issues including: infections, stroke, and acute chest syndrome. People get sickle cell anemia by inheriting a mutated gene from both of their parents. On the down side, there is not currently an effective cure for sickle cell anemia, there are several different therapeutic approaches to treating and attempting to cure the disease and help people who are affected with sickle cell anemia better manage their symptoms. There have also been several great scientists that dedicated a lot of research to help find a cure for sickle cell anemia.
When the sickle cells go through they can block up blood vessels and cause severe pain, fatigue, paleness, rapid heart rate, shortness of breath, and/or yellowing of eyes and skin. This can also start in early childhood and cause you to have a low number of red blood cells. This disorder can affect major organs such as lungs, kidneys, spleen, and brain. Anemia can cause shortness of breath, fatigue and Delayed growth development. The severity of the pain varies from person to person. The pain is usually medium to
A blood test can check for hemoglobin S, the gene that causes Sickle Cell Anemia. Sickle Cell Anemia can also be diagnosed in an unborn baby by sampling some of the amniotic fluid to look for the sickle gene. In the United States, the test is part of a routine newborn screening, but older children and adults can be tested too. If the screening comes out positive, the doctors will perform a test to find out if the person has one or two sickle cell genes by determining how much of a percentage of gene is present. People with a small percentage of the gene present have one
People with sickle cell anemia also may have bouts of pain in the chest, stomach, arms, legs, or other parts of the body. This is caused by sickle cells blocking blood flow through the blood vessels. Feeling tired and having trouble fighting
Each year in the US, an average of 75,000 hospitalizations are due to sickle cell disease, costing approximately $475 million. Sickle cell disease is also associated with significant mortality. Among children, the primary causes of mortality are bacterial infections and stroke. In adults, it is more difficult to attribute specific causes to mortality, but it appears that individuals with more indicative disease are at risk for early mortality.
The main thing doctors do to treat sickle-cell anemia is to avoid complications. They measure the patient’s height and weight to make sure they are growing properly. The doctors also monitor blood pressure to prevent strokes. Doctors also do blood and urine tests to see if there is organ damage or kidney problems. They also do TCD testing or brain ultrasounds to prevent strokes. They also do eye examines to prevent loss of vision. Sickle-cell anemia is caused only by having two mutated hemoglobin genes.
The diagnosis for the disease is straightforward. All states screen newborns for sickle cell. This can be done by a simple blood test. The treatment for sickle cell anemia is minimal. There are no cures for the disease. However treatments and pain killers can help with some of the symptoms. There are a few environmental factors of sickle cell. For example people with the disease should avoid cold areas so that their blood flow stays normal. They should also make sure that they have good circulation at all times to ensure that the blood is moving. All in all, sickle cell is a simple disease that does not take much to deal
To properly treat patients with sickle cell disease who are suffering from acute chest syndrome, multiple precautions must be taken into account. First, broad-spectrum antibiotics should be administered due to the high chance that it is caused by a bacterial infection. Secondly, airway hyperreactivity should be assumed and checked for even if wheezing or coughing is not present. Thirdly, if possible, patients who suffer from anemia should seek blood transfusions before the onset of respiratory distress. Lastly, if the patient sees no improvement, they may be treated with alternative drugs such as nitric oxide or hydroxyurea, but this is only in severe cases.
Sickle Cell Anemia or Sickle Cell Disease (SCD) is a passed down blood disorder that attacks and destroys the red blood cells. This Disease was first discovered back in 1910. The disease was first described as a clinical entity by Herrick1 in 1910 (Anderson & Ware 1932). Sickle cell sickness is brought about by a change in the hemoglobin-Beta quality found on chromosome 11. Hemoglobin transports oxygen from the lungs to different parts of the body. Red platelets with typical (hemoglobin-An) are smooth and round and coast through veins. Typical red platelets are round like doughnuts, and they travel through little blood tubes in the body to convey oxygen. Sickle red platelets turn out to be hard, sticky and molded like sickles used to cut wheat. At times these blood cells can become crescent shaped and have a hard time passing through small blood vessels, this keeps red blood cells and the oxygen they carry from getting to all parts of the body. This causes less blood to reach the body at this point the tissues that have not received a normal blood flow will become damaged. Individuals who have sickle cell
Sickle cell disease is categorized as an autosomal recessive disease. That means in order for one to inherit the disease, they must inherit two recessive alleles for sickle cell disease, or hemoglobin S gene. Having only one recessive allele makes you a carrier. The disorder causes the affected person’s red blood cells to disfigure into a crescent or sickle, which differs from the normal red blood cells that shaped into a doughnut. Sometimes, cells in a sickle shape cannot get through the narrow passageways of the blood vessels as easily as normal red blood cells. This blocks blood from entering and oxygen can’t reach the affected person’s organs. It can also crystallize and block arteries and
Sickle Cell Disease, commonly referred to as Sickle Cell Anemia, is a blood disease which red blood cells form an abnormal sickle or crescent shape. Red blood cells carry oxygen to the body and are normally shaped like a disc. This disease is genetically inherited from both parents. If you inherit the sickle cell gene from only one parent, you will have sickle cell trait. People with sickle cell trait do not have the symptoms of sickle cell anemia.
The short life span of RBCs and the damage they cause to tissues can result in the following signs and symptoms: severe abdominal pain, priapism, tissue engorgement, painful swelling of joints and feet, fever, shock, profound anemia, and hypovolemia. Many organs can be affected to include the brain, eyes, bones, liver, spleen, kidneys, penis, extremities, and skin (Ball, J., & Bindler, R., 2015). Pain from sickle cell crises is the most common reasons for hospitalization in children with sickle cell disease. Pain typically affects the joints, chest, back and abdomen. Children may also experience acute chest syndrome (ACS), a life threatening complication requiring hospitalization. ACS is characterized by chest pain, fever, tachypnea, cough, shortness of breath, crackles and
The only way you get sickle cell is by inheritance from a family member, whether it be your mom or your dad. If a person inherits the sickle cell gene from only one parent will not develop the gene but will have something called sickle cell trait. People with sickle cell trait do not have the disease, but they might have a few sickle cell blood cells in their bodies. Although having sickle cell trait does not give them the disease they have the sickle cell gene and it will be passed down to their kids. Kids and parents may never know for a fact if they have the gene or not that is why doctors recommend getting checked out for sickle cell genes. To check for sickle cell genes doctors use a special blood test called a hemoglobin electrophoresis. All newborns get screened and tested for sickle disease. It is required in every state to have your newborn tested for sickle cell
Sickle Cell Disease is a genetic disease caused by a SNP (Single Nucleotide Polymorphism). Which means it is caused by a single letter gene mutation. Through the advent of CRISPR/Cas9 the disease might be cured. It is a serious reality and will enter medical trials within a handful of years. Sickle Cell Disease is an inherited disease that predominantly affects people of African descent. Red Blood cells become rigid and sickle shaped, causing blockages and pain crises. In cases of Sickle Cell Disease the Beta-Globin are mutated causing faulty and deformed Hemoglobin proteins thus creating sickled and short-lived Red Blood Cells.
Sickle Cell Anemia is a group of disorders that cause red blood cells to become misshapen and break down. Sickle cell disease is the most common inherited blood disorder in the United States, affecting 70,000 to 80,000 Americans (Ashley-Koch, Yang and Olney). Sickle Cell Anemia causes your red blood cells to be thin, stiff, and shaped like a sickle. But your red blood cells are supposed to be round and soft. A person that is diagnosed with sickle cell anemia, their blood cells start to become clogged, causing blood clots, which can cause a great amount of health issues including: infections, stroke, and acute chest syndrome. People get sickle cell anemia by inheriting a mutated gene from both of their parents. On the down side, there is not currently an effective cure for sickle cell anemia, there are several different therapeutic approaches to treating and attempting to cure the disease and help people who are affected with sickle cell anemia better manage their symptoms. There have also been several great scientists that dedicated a lot of research to help find a cure for sickle cell anemia.