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Sickle-cell disease (SCD), or sickle-cell anaemia (or anemia; SCA) or drepanocytosis, is an autosomal recessive genetic blood disorder with incomplete dominance, characterized by red blood cells that assume an abnormal, rigid, sickle shape. Sickling decreases the cells' flexibility and results in a risk of various complications. The sickling occurs because of a mutation in the hemoglobin gene. Life expectancy is shortened, with studies reporting an average life expectancy of 42 in males and 48 in females. Background of the Disease

Sickle cell anemia is one type of anemia. Anemia is a condition in which your blood has a lower than normal number of red blood cells. This condition also can occur if your red blood cells don't contain enough hemoglobin. Red blood cells are made in the spongy marrow inside the large bones of the body. Bone marrow is always making new red blood cells to replace old ones. Normal red blood cells live about 120 days in the bloodstream and then die. They carry oxygen and remove carbon dioxide (a waste product) from your body. In sickle cell anemia, the number of red blood cells is low because sickle cells don't last very long. Sickle cells usually die after only about 10 to 20 days. The bone marrow can't make new red blood cells fast enough to replace the dying ones. Sickle cell anemia is an inherited, lifelong disease. People who have the disease are born with it. They inherit two genes for sickle hemoglobinone from each parent. People who inherit a sickle hemoglobin gene from one parent and a normal gene from the other parent have a condition called sickle cell trait. Sickle cell trait is different than sickle cell anemia. People who have sickle cell trait don't have the disease, but they have one of the genes that cause it. Like people who have sickle cell anemia, people who have sickle cell trait can pass the sickle hemoglobin gene on to their children.

Outlook and Epidemiology Sickle-cell disease, usually presenting in childhood, occurs more commonly in people (or their descendants) from parts of tropical and sub-tropical regions where malaria is or was common. One-third of all indigenous inhabitants of Sub-Saharan Africa carry the gene, because in areas where malaria is common, there is a fitness benefit in carrying only a single sickle-cell gene (sickle cell trait). Those with only one of the two alleles of the sickle-cell disease, while not totally resistant, are more tolerant to the infection and thus show less severe symptoms when infected. The prevalence of the disease in the United States is approximately 1 in 5,000, mostly affecting Americans of Sub-Saharan African descent, according to the National Institutes of Health. In the United States, about 1 out of 500 African-American children born will have sickle-cell anemia. Sickle cell anemia has no widely available cure. However, treatments can help with the symptoms and complications of the disease. Blood and marrow stem cell transplants may offer a cure for a small number of people. Over the past 100 years, doctors have learned a great deal about sickle cell anemia. They know its causes, how it affects the body, and how to treat many of its complications. Sickle cell anemia varies from person to person. Some people who have the disease have chronic (long-term) pain or fatigue (tiredness). However, with proper care and treatment, many people who have the disease can have improved quality of life and reasonable health much of the time. Due to improved treatments and care, people who have sickle cell anemia are now living into their forties or fifties, or longer.

PREDISPOSING ANG PRECIPITATING FACTORS Pre-Disposing Factor y Race (Most sub-saharan people have this condition) y Gender (male have higher rate than female) y Heredity y Extreme Low or extreme high environmental Temperature Precipitating Factor y autosomal recessive inheritance of two genes alleles for hemoglobin synthesis

DIAGNOSTIC PROCEDURE A blood test can check for hemoglobin S the defective form of hemoglobin that underlies sickle cell anemia. In most states in the United States, this blood test is part of routine newborn screening done at the hospital. But older children and adults can be tested too. In adults, a blood sample is drawn from a vein in the arm. In young children and babies, the blood sample is usually collected from a finger or heel. The sample is then sent to a laboratory, where a technician can screen for hemoglobin S. If the screening test is negative, there is no sickle cell gene present. If the screening test is positive, the technician will conduct further tests to determine whether one or two sickle cell genes are present. People who have one gene sickle cell trait have a fairly small percentage of hemoglobin S. People with two genes sickle cell disease have a much larger percentage of the defective hemoglobin. In HbSS, the full blood count reveals hemoglobin levels in the range of 68 g/dL with a high reticulocyte count (as the bone marrow compensates for the destruction of sickle cells by producing more red blood cells). In other forms of sickle-cell disease, Hb levels tend to be higher. A blood film may show features of hyposplenism (target cells and Howell-Jolly bodies). Sickling of the red blood cells, on a blood film, can be induced by the addition of sodium metabisulfite. The presence of sickle haemoglobin can also be demonstrated with the "sickle solubility test". A mixture of haemoglobin S (Hb S) in a reducing solution (such as sodium dithionite) gives a turbid appearance, whereas normal Hb gives a clear solution. Abnormal haemoglobin forms can be detected on haemoglobin electrophoresis, a form of gel electrophoresis on which the various types of haemoglobin move at varying speeds. Sickle-cell haemoglobin (HgbS) and haemoglobin C with sickling (HgbSC)the two most common formscan be identified from there. The diagnosis can be confirmed with high-performance liquid chromatography (HPLC). Genetic testing is rarely performed, as other investigations are highly specific for HbS and HbC. An acute sickle-cell crisis is often precipitated by infection. Therefore, a urinalysis to detect an occult urinary tract infection, and chest X-ray to look for occult pneumonia should be routinely performed. People who are known carriers of the disease often undergo genetic counselling before they have a child. A test to see if an unborn child has the disease takes either a blood sample from the fetus or a sample of amniotic fluid. Since taking a blood sample from a fetus has greater risks, the latter test is usually used.

SIGNS AND SYMPTOMS People with sickle cell trait have one gene for the disease. They don't develop the disease and usually have no signs and symptoms. Approximately one in 12 black Americans has sickle cell trait. People with sickle cell anemia have two genes for the disease one from each parent. They usually show some signs and symptoms after 4 months of age. Some people with sickle cell anemia have few symptoms. For others, the disease is more severe and they may require repeated hospitalizations.

Signs and symptoms of sickle cell anemia include:  Anemia. Sickle cells are fragile. They break apart easily and die, leaving you chronically short on red blood cells to carry oxygen to your tissues a condition known as anemia. Without enough red blood cells in circulation, your body can't get the oxygen it needs to feel energized. That's why anemia causes fatigue.  Episodes of pain. Periodic episodes of pain, called crises, are a major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to your chest, abdomen and joints. Pain can also occur in your bones. The pain may vary in intensity and can last for a few hours to a few weeks. Some people experience only a few episodes of pain. Others experience a dozen or more crises a year. If a crisis is severe enough, you may need hospitalization so that painkillers can be injected into your veins (intravenously)  Hand-foot syndrome. Swollen hands and feet are often the first signs of sickle cell anemia in babies. The swelling is caused by sickle-shaped red blood cells blocking blood flow out of the hands and feet.  Jaundice. Jaundice is a yellowing of the skin and eyes that occurs because of liver damage or dysfunction. Occasionally, people who have sickle cell

anemia have some degree of jaundice because the liver, which filters harmful substances from the blood, is overwhelmed by the rapid breakdown of red blood cells. In people with dark skin, jaundice is visible mostly as yellowing of the whites of their eyes. Frequent infections. Sickle cells can damage your spleen, an organ that fights infection. This may make you more vulnerable to infections. Doctors commonly give infants and children with sickle cell anemia antibiotics to prevent potentially life-threatening infections, such as pneumonia. Stunted growth. Red blood cells provide your body with the oxygen and nutrients you need for growth. A shortage of healthy red blood cells can slow growth in infants and children and delay puberty in teenagers. Vision problems. Some people with sickle cell anemia experience vision problems. Tiny blood vessels that supply your eyes may become plugged with sickle cells. This can damage the retina the portion of each eye that processes visual images. Other symptoms may include Attacks of abdominal pain, Bone pain, Breathlessness, Rapid heart rate, Ulcers on the lower legs (in adolescents and adults), Yellowing of the eyes and skin (jaundice), Chest pain, Excessive thirst, Frequent urination; Painful and prolonged erection (priapism - occurs in 10 - 40% of men with the disease)

Sickle cell crisis y Vaso-occlusive crisis: The vaso-occlusive crisis is caused by sickle-shaped red blood cells that obstruct capillaries and restrict blood flow to an organ, resulting in ischaemia, pain, necrosis and often organ damage. The frequency, severity, and duration of these crises vary considerably. Painful crises are treated with hydration, analgesics, and blood transfusion; pain management requires opioid administration at regular intervals until the crisis has settled. For milder crises, a subgroup of patients manage on NSAIDs (such as diclofenac or naproxen). For more severe crises, most patients require inpatient management for intravenous opioids; patient-controlled analgesia (PCA) devices are commonly used in this setting. Vaso-occlusive crisis involving organs such as the penis or lungs are considered an emergency and treated with red-blood cell transfusions. Diphenhydramine is sometimes effective for the itching associated with the opioid use. Incentive spirometry, a technique to encourage deep breathing to minimise the development of atelectasis, is recommended. Splenic sequestration crisis: Because of its narrow vessels and function in clearing defective red blood cells, the spleen is frequently affected. It is usually infarcted before the end of childhood in individuals suffering from sickle-cell anaemia. This autosplenectomy increases the risk of infection from encapsulated

organisms; preventive antibiotics and vaccinations are recommended for those with such asplenia. Aplastic crisis: Aplastic crises are acute worsenings of the patient's baseline anaemia, producing pallor, tachycardia, and fatigue. This crisis is triggered by parvovirus B19, which directly affects erythropoiesis (production of red blood cells) by invading the red cell precursors and multiplying in them and destroying them. Parvovirus infection nearly completely prevents red blood cell production for two to three days. In normal individuals, this is of little consequence, but the shortened red cell life of sickle-cell patients results in an abrupt, life-threatening situation. Reticulocyte counts drop dramatically during the disease (causing reticulocytopenia), and the rapid turnover of red cells leads to the drop in haemoglobin. This crisis takes 4 days to one week to disappear. Most patients can be managed supportively; some need blood transfusion. Haemolytic crisis: Haemolytic crises are acute accelerated drops in haemoglobin level. The red blood cells break down at a faster rate. This is particularly common in patients with co-existent G6PD deficiency. Management is supportive, sometimes with blood transfusions. Other: One of the earliest clinical manifestations is dactylitis, presenting as early as six months of age, and may occur in children with sickle trait. The crisis can last up to a month. Another recognised type of sickle crisis is the acute chest syndrome, a condition characterised by fever, chest pain, difficulty breathing, and pulmonary infiltrate on a chest X-ray. Given that pneumonia and sickling in the lung can both produce these symptoms; the patient is treated for both conditions. It can be triggered by painful crisis, respiratory infection, bonemarrow embolisation, or possibly by atelectasis, opiate administration, or surgery.

Acute and Chronic Complications y Acute chest syndrome. This life-threatening complication of sickle cell anemia cause chest pain, fever and difficulty breathing. Acute chest syndrome is similar to pneumonia, but is caused by a lung infection or trapped sickle cells in the blood vessels of your lungs. It requires emergency medical treatment with antibiotics, blood transfusions and drugs that open up airways in your lungs. Recurrent attacks can damage your lungs. Organ damage. Sickle cells can block blood flow through blood vessels, immediately depriving an organ of blood and oxygen. In sickle cell anemia, blood is also chronically low on oxygen. Chronic deprivation of oxygen-rich blood can damage nerves and organs in your body, including your kidneys, liver and spleen. Organ damage can be fatal.

Blindness. Tiny blood vessels that supply your eyes can get plugged with sickle cells. Over time, this can damage the retina the portion of each eye that processes visual images and lead to blindness. Other complications. Sickle cell anemia can cause open sores, called ulcers, on your legs. Sickle cells can block blood vessels that nourish your skin, causing skin cells to die. Once skin is damaged, sores can develop. Gallstones also are a possible complication. The breakdown of red blood cells produces a substance called bilirubin. Bilirubin is responsible for yellowing of the skin and eyes (jaundice) in people with sickle cell anemia. A high level of bilirubin in your body can also lead to gallstones. Men with sickle cell anemia may experience painful erections, a condition calledp riap is m. Sickle cells can prevent blood flow out of an erect penis. Over time, priapism can damage the penis and lead toi mp ot en ce in men with sickle cell anemia.


Predisposing Factor -race -gender -Heredity -extreme low or high environmental temperature Precipitating Factor -autosomal recessive inheritance of two alleles gene for hemoglobin synthesis

Fathers gene alleles for the beta chain of hemoglobin (carriersickle cell trait)

Mothers gene alleles for the beta chain of hemoglobin (carriersickle cell trait)





HbA-HbA Completely unaffected

HbA-HbS Carrier of sickle cell trait TRAIT

HbS-HbA Carrier of sickle cell trait TRAIT

HbS-HbS AFFECTED of sickle cell trait TRAIT

Client has possessed 40% of abnormality at beta chain of hemoglobin

Formation of HbS

Factors that decreases oxygen conc. To sicklecell disease patients

Sensitivity to changes in oxygen content in RBC

y Insufficient oxygen inhalation y Dehydration y Infection y Venous stasis y Extreme low environmental temperature y Acidosis y Strenuous activities y anesthesia

if exposed to decrease oxygen concentration

Abnormal chains contract pile together

Assumes sickle-shape

Becomes rigid and clamp together

Client becomes oxygenated


Further decrease oxygen

RBC goes back to normal cell

Vision problems Masses of RBC formed

Blockage at the retina

Blockage of blood flow


Further exposure to Hypoxia


Can cause further RBC breakdown

Ischemia to the extremities

Decrease oxygen concentration at the chest/coronary area

Liver becomes overwhelmed by the rapid breakdown of red blood cells

Blockage of sickled RBC mass

Chest pain

Swollen hands and feet Too high level of bilirubin

Shortage of healthy red blood cells

Further Decrease oxygen concentration at the chest/coronary arteries

Can cause gallstone Stunted growth

Damage to spleen

Risk for infection

Further ischemia to tissues and organs

Organ damage


MEDICAL MANAGEMENT Bone marrow transplant offers the only potential cure for sickle cell anemia. But very few people have a suitable donor for transplant. As a result, treatment for sickle cell anemia is usually aimed at avoiding crises, relieving symptoms and preventing complications. If you have sickle cell anemia, you'll need to make regular visits to your doctor to check your red blood cell count and monitor your health. You may also require treatment from specialists at a hospital or sickle cell anemia clinic. Treatments may include medications to reduce pain and prevent complications, blood transfusions and supplemental oxygen, as well as bone marrow transplant. Medications y Antibiotics. Children with sickle cell anemia usually begin taking the antibiotic penicillin when they're about 2 months of age and continue until they're 5 years old. Doing so helps prevent infections, such as pneumonia, which can be lifethreatening to an infant or child with sickle cell anemia. Antibiotics may also help adults with sickle cell anemia fight certain infections. Pain-relieving medications. To relieve pain during a sickle crisis, your doctor may advise over-the-counter pain relievers and application of heat to the affected area. You may also need stronger prescription painkillers. Hydroxyurea (Droxia, Hydrea). This prescription drug, normally used to treat cancer, may be helpful for adults with severe disease. When taken daily, it reduces the frequency of painful crises and may reduce the need for blood transfusions. It seems to work by stimulating production of fetal hemoglobin a type of hemoglobin found in newborns that helps prevent the formation of sickle cells. There is some concern about the possibility that long-term use of this drug may cause tumors or leukemia in certain people. Your doctor can help you determine if this drug may be beneficial for you.

Blood transfusions In a red blood cell transfusion, red blood cells are removed from a supply of donated blood. These donated cells are then given intravenously to a person with sickle cell anemia. Blood transfusions increase the number of normal red blood cells in circulation, helping to relieve anemia. In children with sickle cell anemia at high risk of stroke, regular blood transfusions can decrease their risk of stroke. Blood transfusions carry some risk. Blood contains iron. Regular blood transfusions cause an excess amount of iron to build up in your body. Because excess iron can damage your heart, liver and other organs, people who undergo regular transfusions must often receive treatment to reduce iron levels. In 2005, the Food and

Drug Administration approved deferasirox (Exjade), the first oral medication that can reduce excess iron levels, for use in people older than the age of 2. Supplemental oxygen Breathing supplemental oxygen through a breathing mask adds oxygen to your blood and helps you breathe easier. It may be helpful if you have acute chest syndrome or a sickle cell crisis. Bone marrow transplant This procedure allows people with sickle cell anemia to replace their bone marrow and its sickle-shaped red blood cells with healthy bone marrow from a donor who doesn't have the disease. It can be a cure, but the procedure is risky, and it's difficult to find suitable donors. Researchers are still studying bone marrow transplants for people with sickle cell anemia. Currently, the procedure is recommended only for people who have significant symptoms and problems from sickle cell anemia. In a bone marrow transplant, your bone marrow is first destroyed using chemotherapyor radiation. A matched donor's healthy bone marrow is removed using a minorsurgical technique. You then receive a transfusion of that healthy marrow, and thehealthy marrow may start producing normal blood cells. Sometimes, however, the transplant doesn't work or the recipient's body rejects the new marrow. The procedure requires a lengthy hospital stay. After the transplant, you'll need drugs to help prevent rejection of the donated marrow. Treating complications Doctors treat most complications of sickle cell anemia as they occur. Treatment may include antibiotics, blood transfusions, pain-relieving medicines, other medications and possibly surgery, such as to correct vision problems or to remove a damaged spleen. Experimental treatments Scientists continue to gain new insights into the symptoms and causes of sickle cell anemia. Some possible new treatments researchers are studying include: y Gene therapy. Because sickle cell anemia is caused by a defective gene, researchers are exploring whether correcting this gene and inserting it into the bone marrow of people with sickle cell anemia will result in the production of normal hemoglobin. Scientists are also exploring the possibility of turning off the defective gene while reactivating another gene responsible for the production of fetal hemoglobin a type of hemoglobin found in newborns that prevents sickle cells from forming. Butyric acid. Some studies show that this commonly used food additive may increase the amount of fetal hemoglobin in the blood.

Clotrimazole. Normally used to treat fungal infections, this over-the-counter medication helps prevent a loss of water from red blood cells, which may reduce the number of sickle cells that form. Nitric oxide. People with sickle cell anemia have low levels of nitric oxide, a gas that helps keep blood vessels open and reduces the stickiness of red blood cells. Treatment with nitric oxide may prevent sickle cells from forming.

PREVENTION If you carry the sickle cell trait, you may wish to see a genetic counselor before trying to conceive a child. A genetic counselor can help you understand your risk of having a child with sickle cell anemia. He or she can also explain possible treatments, preventive measures and reproductive options. There is an in vitro fertilization procedure that improves the chances for parents who both carry the sickle cell gene to have a child with normal hemoglobin. This procedure is known as preimplantation genetic diagnosis. First, eggs are taken from the mother. Then, sperm is taken from the father. In a laboratory, the eggs are fertilized with the sperm. The fertilized eggs are then tested for the presence of the sickle cell gene. Fertilized eggs free of the sickle cell gene can be implanted into the mother for normal development. However, this procedure is expensive and not always successful. Lifestyle and home remedies Taking steps to stay healthy is critical for anyone with sickle cell anemia. Eating well, getting adequate rest and protecting yourself from infections are good ways to maintain your health and prevent crises. Infants and children with sickle cell disease need to receive regular childhood vaccinations. Children and adults with sickle cell anemia also should have a yearly flu shot and be immunized against pneumonia. If you or your child has sickle cell anemia, follow these suggestions to help stay healthy: y Take folic acid supplements daily, and eat a balanced diet. Bone marrow needs folic acid and other vitamins to make new red blood cells. y Drink plenty of water. Staying hydrated helps keep your blood diluted, which reduces the chance that sickle cells will form. y Avoid temperature extremes. Exposure to extreme heat or cold can trigger the formation of sickle cells. y Avoid stress. A sickle crisis can occur as a result of stress. y Exercise regularly, but don't overdo it. Talk with your doctor about how much exercise is right for you.

Fly on commercial airplanes with pressurized passenger cabins. Unpressurized aircraft cabins don't provide enough oxygen. Low oxygen levels can trigger a sickle crisis. Avoid high-altitude areas. Traveling to a high-altitude area may also trigger a crisis because of lower oxygen levels.

PRIORITY NURSING DIAGNOSES Nursing Diagnosis: Impaired gas exchange related to decreased oxygencarrying capacity of the blood, reduced RBC life span or premature destruction, abnormal RBC structure, sensitivity to low oxygen tension due to strenuous exercise, as evidenced by Dyspnea, use of accessory muscles, Restlessness, confusion Nursing Interventions Monitor respiratory rate and depth y and use of accessory respiratory muscles y Auscultate breath sounds, noting presence or absence, and adventitious sounds. y Monitor vital signs; note changes in cardiac rhythm. Investigate reports of chest pain y and increasing fatigue. Observe for signs of increased fever, cough, and adventitious breath sounds. Assess LOC and mentation y regularly. Rationale indicator of adequacy respiratory function Development of atelectasis and stasis of secretions can impair gas exchange. Changes in vital signs and development of dysrhythmias reflect effects of hypoxia on cardiovascular system. Reflective of developing acute chest syndrome, which increases the workload of the heart and oxygen demand. Brain tissue is very sensitive to decreases in oxygen, and changes in mentation may be an early indicator of developing hypoxia. For safety

Evaluate activity tolerance; limit y activities to those within clients tolerance or place client on bedrest. Assist with ADLs and mobility, as needed. Assist in turning, coughing, and y deep-breathing exercises.

Promotes optimal chest expansion, mobilization of secretions, and aeration of all lung fields; reduces risk of stasis of secretions and pneumonia.

Encourage client to alternate y periods of rest and activity. Schedule rest periods, as indicated. Demonstrate and encourage use of y relaxation techniques, such as guided imagery and visualization. Promote adequate fluid intake, such y as 2 to 3 L/day within cardiac tolerance.

Protects from excessive fatigue and reduces oxygen demands and degree of hypoxia. Relaxation decreases muscle tension and anxiety and, hence, the metabolic demand for oxygen. Sufficient hydration is necessary to provide for mobilization of secretions and to prevent hyperviscosity of blood with associated capillary occlusion.

Nursing Diagnosis: ineffective tissue Perfusion related to Vaso-occlusive nature of sickling, inflammatory response, Arteriovenous (AV) shunts in both pulmonary and peripheral circulation, Myocardial damage from small infarcts, iron deposits, and fibrosis as evidenced by Changes in vital signs, diminished peripheral pulses and capillary refill, general pallor Decreased mentation, restlessness Angina, palpitations Tingling in extremities, intermittent claudication, bone pain Transient visual disturbances Ulcerations of lower extremities, delayed healing. Nursing Interventions Rationale y Monitor vital signs carefully. Assess y Sludging and sickling in peripheral pulses for rate, rhythm, and vessels may lead to complete or volume. Note hypotension; rapid, partial obliteration of a vessel with weak, thready pulse; and tachypnea diminished perfusion to with shallow respirations. surrounding tissues. Sudden massive splenic sequestration of cells can lead to shock. y Assess skin for coolness, pallor, y Changes reflect diminished cyanosis, diaphoresis, and delayed circulation and hypoxia capillary refill. potentiating capillary occlusion y Note changes in LOC; reports of y Changes may reflect diminished headaches, dizziness; development perfusion to the CNS due to of sensory or motor deficits, such as ischemia or infarction (stroke). hemiparesis or paralysis; and seizure activity. y Maintain adequate fluid intake. y Dehydration not only causes Monitor urine output. hypovolemia but increases sickling and occlusion of capillaries. Decreased renal perfusion and

Assess lower extremities for skin texture, edema, and ulcerations, y especially of internal and external ankles. Investigate reports of change in y character of pain, or development of bone pain, angina, tingling of extremities, and eye pain or vision disturbances.

Maintain environmental y temperature and body warmth without overheating. Avoid hypothermia.

Evaluate for developing edema y including genitals in men.

Monitor laboratory studies, such as y the following: Blood gases, liver and kidney function tests

Administer hydroxyurea (Droxia) y or experimental antisickling agents, such as sodium cyanate, carefully and observe for possible lethal side effects.

failure may occur because of vascular occlusion. reduced peripheral circulation often leads to skin and underlying tissue changes and delayed healing. Changes may reflect increased sickling of cells and impaired circulation with further involvement of organs, such as myocardial infarction (MI), pulmonary infarction, or occlusion of vasculature of the eye. Prevents vasoconstriction, aids in maintaining circulation and perfusion. Excessive body heat may cause diaphoresis, adding to insensible fluid losses and risk of dehydration. Hypothermia may exacerbate cardiovascular compromise with severe anemia. Vaso-occlusion or circulatory stasis may lead to edema of extremities and priapism in men, potentiating risk of tissue ischemia and necrosis. Decreased tissue perfusion may lead to gradual infarction of organ tissues, such as the brain, liver, spleen, kidney, skeletal muscle, and so forth, with consequent release of intracellular enzymes. Hydroxyurea, a cytotoxic agent, dramatically decreases the number of sickle cell episodes, and is given to prevent crises. Antisickling agents currently under investigational use are aimed at prolonging erythrocyte survival and preventing sickling by affecting cell membrane changes.

Nursing Diagnosis: deficient knowledge regarding disease process, genetic factors, self-care and treatment medications related to unfamiliarity with the resources of the disease condition as manifested by questioning and statement of concern. Nursing Intervention Rationale y Observe client's ability and y For assessment and to know the readiness to learn (e.g., mental extent of intervention. Education in acuity, ability to see or hear, no self-care must take into account existing pain, emotional readiness, physical, sensory, mobility, sexual, absence of language or cultural and psychosocial changes related to barriers). age y Assess barriers to learning (e.g., y The client brings to the learning perceived change in lifestyle, situation a unique personality, financial concerns, cultural patterns, established social interaction lack of acceptance by peers or patterns, cultural norms and values, coworkers). and environmental influences. y Determine client's previous y New information is assimilated into knowledge of or skills related to his previous assumptions and facts and or her diagnosis and the influence may involve negotiating, on willingness to learn. transforming, or stalling. y Involve clients in writing specific y Client involvement improves outcomes for the teaching session, compliance with health regimen such as identifying what is most and makes teaching and learning a important to learn from their partnership. viewpoint and lifestyle. Objectives put the content into focus, provide a forum for evaluation outcomes, and ensure continuity. y When teaching, build on client's y For better comprehension. literacy skills. In patients with low literacy skills, materials should be short and have culturally sensitive illustrations. y Present material that is most y Information building begins with explaining simple concepts and significant to client first, such as moves on to explanations of how to give injections or change complex application situations. dressings; present additional material once client's most pressing educational needs have been met. y Help client identify community y To have an additional resources that he may know in managing the resources for continuing condition information and support. Learning occurs through imitation, so

persons who are currently involved in lifestyle changes can help the client anticipate adjustment issues. Community resources can offer financial and educational support. For example, role modeling and skill training have been used to monitor symptoms and solve asthma problems Evaluate client's learning through y return demonstrations, verbalizations, or the application of skills to new situations.

Presenting information along with with examples of how to apply the information has been found more successful than providing information alone in a home care setting.

Nursing Diagnosis: compromised family coping related to the chronic nature of the disease, family disorganization, presence of other crisis, lifestyle restriction and the family expressing pre-occupation. Nursing Interventions with Rationale Coping and support If you or someone in your family has sickle cell anemia, you may need help handling the stresses of coping with this lifelong disease. Your doctor can talk with you about your concerns. Sickle cell centers and clinics also can provide information and counseling. Many areas have sickle cell support groups for families affected by the disease. It's especially important to find ways to control and cope with pain. Different techniques work for different people, but it might be worth trying heating pads, hot baths, massages or physical therapy. Prayer, family and friends also can be a source of support.

Other Possible Nursing Diagnoses:

y y y y

Delayed growth and development related to limitation of physical condition as manifested by altered physical growth and delay in performing skills expected for the age group. Risk for sedentary lifestyle related to safety concerns and prone to injury. High risk for ineffective tissue perfusion R/T infarcts due to sickling. Altered health maintenance R/T lack of knowledge regarding long term care. Altered family process R/T having a child with potentially life threatening disease.

Health Teachings y y y y y y y Avoid strenuous activities. Protect from hypoxia avoid mountain climbing, travel in unpressurized plains, under water swimming. Promote hydration oral intake, observe for signs of dehydration, avoid extremes of climates. Prevent infections adequate nutrition, rest, good hygiene, immunization. Avoid exposure to infections Pain management inform doctor about childs condition, liberal use of drugs round the clock, Use hot compresses and avoid cold compresses. Regular follow up visits.

POISONING A poison is any substance that, when ingested, inhaled, absorbed, applied to the skin, or produced within the body in relatively small amounts, injures the body by its chemical action. Poisoning from inhalation and ingestion of toxic materials, both intentional and unintentional, constitutes a major health hazard and an emergency situation. Emergency treatment is initiated with the following goals: y y y y To remove or inactivate the poison before it is absorbed To provide supportive care in maintaining vital organ systems To administer a specific antidote to neutralize a specific poison To implement treatment that hastens the elimination of the absorbed poison

Types of poisoning: INGESTED POISONS Ingested poisons are those poisons which have been consumed, whether accidentally or intentionally, by the victim. Ingestion is the most common route of exposure to toxic materials in the home. The local actions of an ingested poison can have irritant, acidic (corrosive), or basic (caustic) effects at the site of contact.  Corrosive poisons include alkaline and acid agents that can cause tissue destruction after coming into contact with mucous membranes.  Alkaline products include lye, drain cleaners, bleach, nonphosphate detergents, oven cleaners, and button batteries (batteries used to power watches, calculators, or cameras).  Acid products include toilet bowl cleaners, pool cleaners, metal cleaners, rust removers, and battery acid. If a substance is ingested without intent to harm, it's assumed that it was an unintentional poisoning. This includes the consumption of drugs, medications, or other chemicals that are used in excessive amounts and result in an overdose. A poisoning is also considered unintentional when a substance is ingested accidentally and unknowingly, such as with children or older adults. When a substance is ingested with the intent to harm, it's considered an intentional poisoning, such as in a suicide attempt or assault on another person. When you can't make the distinction between the two because of circumstances, then the poisoning may be considered undetermined until proven otherwise. Substances most likely to be ingested include over-the-counter pain medicines, cleaning products, cosmetics, plants, prescription medications, and cough and cold preparations. Fatalities usually result from the ingestion of analgesics, sedative-

hypnotics, antipsychotics, antidepressants, illegal drugs, cardiovascular medications, or alcohol. The phases of poisoning are as follows: y y Preclinical phase-some signs and symptoms may not be evident during this phase; the priority is decontamination Toxic phase-signs and symptoms and lab changes are evident during this phase and guide treatment; the emphasis is on shortening the duration of poisoning and lessening the severity of toxicity Resolution phase-this phase encompasses peak toxicity to recovery; the goal is to shorten the duration of toxicity.

PREDISPOSING FACTORS/PRECIPITATING FACTORS Common substances that can cause poisoning in children include the following: y Automobile fluids (e.g., gasoline, antifreeze, windshield fluid) y Cosmetics and other personal care products y Household cleaning products (e.g., drain cleaners, dishwasher detergent) y Over-the-counter or prescription medications (e.g., analgesics such as acetaminophen, cough and cold medicines, vitamins) y Foreign objects (e.g., toys, batteries) y Paints and paint thinners y Pesticides (e.g., insecticides, weed killers, rodenticides) y Plants y Art supplies and office supplies y Alcohol y Food products y Herbal medicines Risk Factors of Acute Intentional Poisoning y More common in females than in males in all age groups. y Low socioeconomic status. y Self-poisoning is likely to be seen in men belonging to the unskilled and low socioeconomic groups than in professionals and more affluent groups. y The incidence of poisoning is rising in the those in the 20s and 30s age group as compared to elderly people. y There is higher risk amongst divorcees than in single, married, or widowed individuals. y Previous episodes of self-poisoning. These people are also more likely to try selfpoisoning again in the future. y Increased risk of subsequent suicide.

Other precipitating factors are: y Unemployment. y Financial distress. y Alcoholism. y Drug abuse. y Marital discord y Recent broken relationship. y Those with criminal records. y Family violence. y Losing a parent at an early age. y Victims of child abuse.

DIAGNOSTICS The diagnosis in a case of poisoning can be made from the 1) History 2) Physical Examination 3) Laboratory Evaluation and 4) Toxicological Screening 1. History y Most important indicator of toxic ingestion. Careful history regarding involved toxins, amount of drug and timing should be recorded. y Information regarding prescription medication, over the counter drugs and illicit substances of abuse should be obtained. y Friends, relatives and other involved healthcare providers should be questioned and medications identified. y Medication found on or near the patient should be examined and pharmacy on the medication label should be called to determine the status of all prescription medication. 2. Physical Examination y Evaluation of Airway patency, Respiration, y Circulation. y Rapid assessment of mental status, temperature, pupil size, muscle tone, reflexes, skin and peristaltic activity. 3. Labororatory evaluation y Clinical laboratory data include assessment of the three gaps of toxicology9 1. The Anion gap 2. The osmolal gap 3. The arterial oxygen saturation gap. Unexplained widening of the difference between calculated determination of these values raises the suspicion of toxic ingestion. and measured

4. Toxicological Screening It provides direct evidence of ingestions, but it rarely impacts initial management and initial supportive measures should never await results of such analysis. It is used to 1) provides ground for treatment with specific antidote or method for enhancing drug elimination and 2) also identifies drugs that should be quantified to guide subsequent management. Also look for characteristic signs of various kinds of poisoning while immediate treatment measures are being started.

SIGNS AND SYMPTOMS y Large (dilated) or small (constricted) pupils. y Slow or abnormal breathing, chemical odors and unusual breath. y Burns or stains around the mouth. y Nausea, vomiting and diarrhea. y Excessive salivation, sweating, and tear formation. y Convulsions or seizures. y Swallowing and breathing may be difficult, especially if any corrosive was aspirated into the lungs. y Stridor, a high-pitched sound coming from the upper airway, may be heard. y The abdomen may be tender and swollen with gas, and perforation of the esophagus or stomach may occur. y Abdominal pain

PATHOPHYSIOLOGY Ingested poisons include foods, alcohol, medication, household and garden items, and certain plants. Acids cause coagulation necrosis; an eschar forms, limiting further damage. Acids tend to affect the stomach more than the esophagus. Alkalis cause rapid liquefaction necrosis; no eschar forms, and damage continues until the alkali is neutralized or diluted. Alkalis tend to affect the esophagus more than the stomach, but ingestion of large quantities severely affects both. Solid products tend to leave particles that stick to and burn tissues, discouraging further ingestion and causing localized damage. Because liquid preparations do not stick, larger quantities are easily ingested, and damage may be widespread. Liquids may also be aspirated, leading to upper airway injury. MEDICAL MANAGEMENT If the toxin was recently ingested, absorption of the substance may be able to be decreased through gastric decontamination. This may be achieved using activated

charcoal, gastric lavage, whole bowel irrigation, or nasogastric aspiration. Routine use of emetics (syrup of Ipecac), cathartics or laxatives are no longer recommended. y Activated charcoal is the treatment of choice to prevent absorption of the poison. It is usually administered when the patient is in the emergency room or by a trained emergency healthcare provider such as a Paramedic or EMT. However, charcoal is ineffective against metals such as sodium, potassium, and lithium, and alcohols and glycols; it is also not recommended for ingestion of corrosive chemicals such as acids and alkalis. Whole bowel irrigation cleanses the bowel; this is achieved by giving the patient large amounts of a polyethylene glycol solution. The osmotically balanced polyethylene glycol solution is not absorbed into the body, having the effect of flushing out the entire gastrointestinal tract. Its major uses are following ingestion of sustained release drugs, toxins that are not absorbed by activated charcoal (i.e. lithium, iron), and for the removal of ingested packets of drugs (body packing/smuggling). Gastric lavage, commonly known as a stomach pump, is the insertion of a tube into the stomach, followed by administration of water or saline down the tube. The liquid is then removed along with the contents of the stomach. Lavage has been used for many years as a common treatment for poisoned patients. However, a recent review of the procedure in poisonings suggests no benefit.[8] It is still sometimes used if it can be performed within 1 hour of ingestion and the exposure is potentially life threatening. Nasogastric aspiration involves the placement of a tube via the nose down into the stomach, the stomach contents are then removed via suction. This procedure is mainly used for liquid ingestions where activated charcoal is ineffective, e.g. ethylene glycol poisoning. Emesis (i.e. induced by ipecac) is no longer recommended in poisoning situations, because vomiting is ineffective at removing poisons. Cathartics were postulated to decrease absorption by increasing the expulsion of the poison from the gastrointestinal tract. There are two types of cathartics used in poisoned patients; saline cathartics (sodium sulfate, magnesium citrate, magnesium sulfate) and saccharide cathartics (sorbitol). They do not appear to improve patient outcome and are no longer recommended. Analgesics are administered if the patient complains of pain. Diuresis for substance excreted by the kidney. Hemoperfusion involves detoxification of the blood by processing it through an extracorporeal circuit and an adsorbent cartridge containing charcoal or resin, after which the cleansed blood is returned to the patient.

y y

y y y

NURSING MANAGEMENT y Ensuring airway patency and assessing for the risk of aspiration, especially after any gastric decontamination process (make sure adequate suctioning equipment is readily available) y Checking vital signs and monitoring for hypotension or hypertension y Monitor ECG and neurologic status closely for changes. y An indwelling urinary catheter is inserted to monitor renal function. y Blood specimens are obtained to determine the concentration of drug or poison. y Determine what substance was ingested; the amount; the time since ingestion; signs and symptoms, such pain or burning sensations, any evidence of redness or burn in the mouth or throat, pain on swallowing, vomiting or drooling; age and wight of the patient and pertinent health history. y Ensuring telemetry monitoring is intact and active (check for ventricular tachycardias or bradyarrhythmias with hypotension) y Initiating seizures precautions if necessary. y The patient who ingested a corrosive poison is given water or milk to drink for dilution. y Dilution is not attempted if the patient has acute airway edema or obstruction or if there is clinical evidence of esophageal, gastric, or intestinal burn or perforation. y Do gastric empying procedures such as syrup of ipecac to induce vomiting (never use with corrosive poisons); gastric lavage; activated charcoal administration; and cathartic. y The specific chemical or physiologic antagonist is administered as early as possible to reverse or diminish the effect of toxin. NURSING DIAGNOSES y Imbalance nutrition less than body requirement y Impaired oral mucous membrane y Impaired swallowing y Risk for poisoning y Acute pain y Risk for injury

CARBON MONOXIDE POISONING Carbon monoxide is probably the common cause of inhalation injury, because it is a byproduct of the combustion of organic materials, therefore is present in smoke. It may occur as a result or household incidents or attempted suicide. It is implicated in more deaths than any other toxins except alcohol. Carbon monoxide exerts toxic effect by binding to circulating haemoglobin and thereby reducing the oxygen carrying capacity of the blood. Hemoglobin absorbs carbon monoxide 200 times more daily than it absorbs oxygen carbon monoxide bound haemoglobin called carboxyhemoglobin does not transport oxygen. PREDISPOSING FACTORS/PRECIPITATING FACTORS y Environment y Air pollutants y Age y Gender y Race DIAGNOSTICS y Serum carboxyhemoglobin levels y Arterial blood gas levels y Fiberoptic bronchoscopy y Xenon 133 (133Xe) ventilator perfusion scans SIGNS AND SYMPTOMS y Headache y Muscular weakness y Palpitation y Dizziness y Confusion y Coma y Skin color which range from pink or cherry red to cyanotic and pale PATHOPHYSIOLOGY y Carbon monoxide poisoning occur as a result of industrial or household incidents or attempted suicide. y Carbon monoxide exert its toxin effect by binding to circulating haemoglobin y Thus reducing the oxygen-carrying capacity of the blood. y Hemoglobin absorbs carbon monoxide 20 times more readily from it absorbs oxygen. y Carbon monoxide bound haemoglobin called carboxyhemoglobin does not transport oxygen.

MEDICAL MANAGEMENT y Administration of oxygen is essential to accelerate the removal of carbon monoxide from the haemoglobin molecule. y Intubation and mechanical ventilation with 100% oxygen. NURSING MANAGEMENT y Carry the patient to fresh air immediately open all doors and windows. y Loosen all tight clothing. y Initiate cardiopulmonary resuscitation if required. y Administer 100% oxygen. y Prevent chilling, wrap the patients in blankets. y Keep the patient as quiet as possible. y Do not give alcohol in any form or permit the patient to smoke. y Observe patient for respiratory complications. NURSING DIAGNOSES y Impaired ventilation on gas exchange y Airway cobstruction y Decreased lung compliance y Decreased arterial oxygen levels y Respiratory acidosis

SKIN CONTAMINATION POISONING (CHEMICAL BURNS) y y injuries from exposure to chemicals with large number of offending agents diverse action and metabolic effects the severity of chemical burn is determined by the mechanism of action, penetrating strength and concentration and amount and duration of exposure of the chemical to the skin

PREDISPOSING FACTORS/PRECIPITATING FACTORS y Because of their curiosity and ignorance, children are the most common poison victims. In fact, accidental poisoning, usually from the ingestion of salicylates (aspirin), cleaning agents, insecticides, paints, cosmetics, and plants, is the fourth leading cause of death in children. In adults, poisoning is most common among chemical company employees, particularly those in companies that use chlorine, carbon dioxide, hydrogen sulfide, nitrogen dioxide, and ammonia, and in compnies that ignore safety standards. Other causes of poisoning in adults include improper cooking, canning, and storage of food; ingestion of or skin contamination from plants (for example, dieffenbachia, mistletoe, azalea, and philodendron); and accidental or intentional drug overdose (usually barbiturates) or chemical ingestion.

DIAGNOSTICS y Toxicologic studies (including drug screens) of poison levels in the mouth, vomitus, urine, stool, or blood or on the victim's hands or clothing confirm the diagnosis. If possible, have the family or patient bring the container holding the poison to the emergency department for comparable study. In inhalation poisoning, chest X-rays may show pulmonary infiltrates or edema in petroleum distillate inhalation, X-rays may show aspiration pneumonia. Abdominal X-rays may reveal iron pills or other radiopaque substances.

SIGNS AND SYMPTOMS y presence of rash y dryness of the skin y corrosive y wound/lesion cause by the chemical PATHOPHYSIOLOGY Absorbed e.g. chemical splashes to the skin. Poisoning and envenomation can be life threatening and require rapid first aid management and urgent assistance from trained medical personnel.

MEDICAL MANAGEMENT y Antimicrobial treatment y Debridement y Tetanus prophylaxis as prescribed y Plastic surgery if required for further wound management y Reexamined the affected area at 24 and 72 hours and in 7 days due to the risk for understanding the extent and depth of these types of injuries NURSING MANAGEMENT y The skin should be drenched immediately with running water from shower, hose, or faucet y Water should not be applied to burns from lye or white phosphorus because of the potential for an explosion or deepening the burn y A constant stream of water should continue as the patients clothing is being removed y Prolonged lavage with generous amounts of tepid water is important y Identify and characteristics of the chemical agent for future treatment NURSING DIAGNOSIS y IMPAIRED SKIN INTEGRITY

FOOD POISONING Food poisoning refers to an acute illness caused by ingestion of food contaminated by bacteria, bacterial toxins, viruses, natural poisons, or harmful chemical substances. It is characterized by a short incubation period (1 wk or less). The symptoms, varying in degree and combination, include abdominal pain, vomiting, diarrhea, headache, and prostration; more serious cases can result in life- threatening neurologic, hepatic, and renal syndromes leading to permanent disability or death. Most of the illnesses are mild and improve without any specific treatment. Some patients have severe disease and require hospitalization, aggressive hydration, and antibiotic treatment. y y is sudden, explosive illness that may occur after ingestion of contaminated food or drink Botulism is a serious form of food poisoning that requires continual surveillance

PREDISPOSING FACTORS/PRECIPITATING FACTORS y More than 250 known diseases can be transmitted through food. The CDC estimates unknown or undiscovered agents cause 68% of all food-borne illnesses and related hospitalizations. Many cases of food poisoning are not reported because people suffer mild symptoms and recover quickly. Also, doctors do not test for a cause in every suspected case because it does not change the treatment or the outcome.


y y

The doctor may need to do a rectal examination. The doctor performs this test by inserting a lubricated and gloved finger gently into the rectum. The purpose is to make sure there are no breaks in the rectal wall. A sample of stool is taken and tested for blood and mucus. In some cases, a sample of stool or vomit can be sent to the laboratory for further testing to find out which toxin caused the illness. In a majority of cases, a specific cause is not found. A urine sample helps assess how dehydrated the patient is and may indicate possible kidney damage. Blood tests may be performed to determine the seriousness of the illness. An Xray of the abdomen or a CT scan may be taken if the doctor suspects the patient's symptoms may be caused by another illness. Gram staining and Loeffler methylene blue staining of the stool for WBCs helps to differentiate invasive disease from noninvasive disease. Microscopic examination of the stool for ova and parasites are an essential study. Bacterial culture for enteric pathogens such as Salmonella, Shigella, and Campylobacter organisms becomes mandatory if a stool sample shows positive results for WBCs or blood or if patients have fever or symptoms

persisting for longer than 3-4 days. If the patient is notably febrile, then blood cultures should be obtained to rule out bacteremia and sepsis. Other labs including CBC count with differential, serum electrolyte assessment, and BUN and creatinine levels help to assess the inflammatory response and the degree of dehydration. Assay for C difficile should be done to help rule out antibiotic-associated diarrhea in patients receiving antibiotics or those with a history of recent antibiotic use.

SIGNS AND SYMPTOMS y Fluid and electrolyte imbalances y Lethargy y Rapid pulse rate y Fever y Oliguria y Anuria y Hypotension y Delirium y Hypovolemic shock PATHOPHYSIOLOGY The pathogenesis of diarrhea in food poisoning is classified broadly into either noninflammatory or inflammatory types. Noninflammatory diarrhea is caused by the action of enterotoxins on the secretory mechanisms of the mucosa of the small intestine, without invasion. This leads to watery diarrhea without the presence of leukocytes (white blood cells). The enterotoxins may be either preformed before ingestion or produced in the gut after ingestion. Examples of organisms causing noninflammatory diarrhea include Vibrio cholerae, enterotoxicEscherichia coli, Clostridium perfringens, Bacillus cereus, Staphylococcusorganisms, Giardia lamblia, Cryptosporidium, rotavirus, Norwalk virus, and adenovirus. Inflammatory diarrhea is caused by the action of cytotoxin on the mucosa, leading to its invasion and destruction. The colon or the distal small bowel commonly is involved. The diarrhea usually is bloody, with the presence pus comprised of leukocytes. Sometimes, the organisms penetrate the mucosa and proliferate in the local lymphatic tissue, followed by systemic dissemination and sepsis. Examples include Campylobacter jejuni, Vibrio parahaemolyticus, enterohemorrhagic and enteroinvasive E coli, Yersinia enterocolitica, Clostridium difficile, Entamoeba histolytica, and Salmonella and Shigella species. In some types of food poisoning (e.g., staphylococci, B cereus), vomiting is caused by a toxin acting on the central nervous system. For example, the clinical syndrome of botulism results from the inhibition of acetylcholine release in nerve endings by the botulinum. The pathophysiological mechanisms that result in acute gastrointestinal symptoms produced by some of the noninfectious causes of food poisoning (naturally occurring

substances [e.g., mushrooms, toadstools] and heavy metals [e.g., arsenic, mercury, lead]) are not well known. y y y Infectious agents include viruses, bacteria, and parasites. Toxic agents include poisonous mushrooms, improperly prepared exotic foods (such as barracuda - ciguatera toxin), or pesticides on fruits and vegetables. Food usually becomes contaminated from poor sanitation or preparation. Food handlers who do not wash their hands after using the bathroom or have infections themselves often cause contamination. Improperly packaged food stored at the wrong temperature also promotes contamination.

MEDICAL MANAGEMENT y Anti emetic medication is administered parenterally as prescribed if the patient cannot tolerate fluids or medication orally y Anti-vomiting and diarrhea medications may be given. y The doctor may also treat any fever to make the patient more comfortable. y Antibiotics are rarely needed for food poisoning. In some cases, antibiotics worsen the condition. Only a few specific causes of food poisoning are improved by using these medications. The length of illness with traveler's diarrhea (shigellae) can be decreased with antibiotics, but this specific illness usually runs its course and improves without treatment. y With mushroom poisoning or eating foods contaminated with pesticides, aggressive treatment may include intravenous (IV) fluids, emergency intervention for life-threatening symptoms, and giving medications such as antidotes, such as activated charcoal. These poisonings are very serious and may require intensive care in the hospital. NURSING MANAGEMENT y The key treatment is to determining the source and type of food poisoning y The suspected food should be brought to the medical facility and a history obtained from the patient or family y Food, gastric contents, vomitus, serum, and feces are collected for examination y Monitoring the patients respiration, blood pressure, sensorium, CVP( if indicated) and muscular activity y Measure to control nausea are also important to prevent vomiting y For mild nausea, the patient takes a sips of weak tea, carbonated drinks, or tap water y After 12 to 42 hours if nausea and vomiting subsides the diet gradually progress to a low-residue, blant diet NURSING DIAGNOSIS y FLUID VOLUME DEFICIT RELATED TO VOMITING


Shock is a serious medical condition where the tissue perfusion is insufficient to meet demand for oxygen and nutrients because the body is not getting enough blood flow. This can damage multiple organs and can get worse very rapidly. This hypoperfusional state is a life-threatening medical emergency and one of the leading causes of death for critically ill people. Shock affects all body systems. It may develop rapidly or slowly, depending on the underlying cause. During shocks the body struggles to survive, calling on all its homeostatic mechanism to restore blood flow and tissue perfusion. Any insult to the body can create a cascade of events resulting in poor tissue perfusion. Therefore, almost any patient with any disease state maybe at risk for developing shock. Regards of the initial cause of shock, certain physiologic responses are common to all types of shock. These physiologic responses include hypoperfusion of tissues, hypermetabolism, and activation of the inflammatory responses. The body responds to shock states by activating the sympathetic nervous system and mounting a hypermetabolic and inflammatory response. Once shock develops, the patients survival may have more to do with the bodys ability to effectively respond to it than with the initial cause of shock. Failure of compensatory mechanisms to effectively restore physiologic balance is the final pathway of all shock states and results in endorgan dysfunction and death. Nursing care of patients with shock requires ongoing systematic assessment. Many of the interventions required in caring for patients with shock call for close collaboration with other members of the health care team and rapid implementation of prescribed therapies. Nurses must anticipate these therapies because they need to be implemented with speed and accuracy.


(ARDS = adult respiratory distress syndrome, GI = gastrointestinal, IL = interleukin, TNF = tumor necrosis factor.)

PATHOPHYSIOLOGY Cellular Changes In shock, the cells lack an adequate blood supply and are deprived of oxygen and nutrients; therefore, they must produce energy through anaerobic metabolism. This results in low energy yields from nutrients and an acidotic intracellular environment. Because of these changes, normal cell function ceases. The cell swells and the cell membrane becomes more permeable, allowing electrolytes and fluids to seep out of and into the cell. The sodium-potassium pump becomes impaired; cell structures, primarily the mitochondria, are damaged; and death of the cell results. Glucose is the primary substance required for the production of cellular energy in the form of ATP. In stress states, catecholamines, cortisol, glucagons and inflammatory cytokines and mediators are released, causing hyperglycemia and insulin resistance to mobilize glucose for cellular metabolism. Activation of these substances promotes gluconeogenesis, which is the n formation of glucose from noncarbohydrate sources such as proteins and fats. Glycogen that has been stored in the liver is converted to glucose through glycogenolysis to meet metabolic needs, increasing the blood glucose concentration.

Continued activation of the stress response by shock states causes a depletion of glycogen stores, resulting in increased proteolysis and eventual organ failure. The inability of the body to have enough nutrients and oxygen for normal cellular metabolism causes a buildup of metabolic end products in the cells and interstitial spaces. Cellular metabolism is impaired, and a negative feedback loop is initiated. Vascular Responses Local regulatory mechanisms, referred to as autoregulation, stimulate vasodilation or vasoconstriction in response to biochemical mediators released by the cell, communicating the need for oxygen and nutrients. A biochemical mediator is a substance released by a cell or immune cells such as macrophages; the substance triggers an action at a cell site or travels in the bloodstream to a distant site, where it triggers action. Blood Pressure Regulation Three major components of the circulatory system --- blood volume, the cardiac pump, and the vasculature--- must respond effectively to complex neural, chemical, and hormonal feedback system to maintain an adequate blood pressure and perfuse body tissues. BP is regulated through a complex interaction of neural, chemical, and hormonal feedback systems affecting both cardiac output and peripheral resistance. Cardiac output is a product of the stroke volume and heart rate. Peripheral resistance is determined by the diameter of the arterioles. Tissue perfusion and organ perfusion depend on mean arterial pressure, or the average pressure at which blood moves through the vasculature. MAP must exceed 65 mmHg for the cells to receive the oxygen and nutrients needed to metabolize energy in amounts sufficient to sustain life. True MAP can be calculated only by complex methods. Frequently, Map is calculated by automatic blood pressure machines, however, the nurse must ensure accurate blood pressure measurements is obtained before interpretating data from automated vital sign equipment. BP is regulated by baroreceptors located in the carotid sinus and aortic arch. These pressure receptors are responsible for monitoring the circulatory volume and regulating neural and endocrine activities. When BP drops, catecholamines are released from the adrenal medulla. These increase heart rate and cause vasoconstriction, thus restoring BP. Chemoreceptors, also located in the aortic rate using much the same mechanism in response to changes in oxygen and carbon dioxide concentrations in the blood. These primary regulatory mechanisms can respond to changes in BP on a moment-to-moment basis. The kidneys regulate BP by releasing rennin, an enzyme needed for the conversion of angiotensin I to angiotensin II, a potent vasoconstrictor. This stimulation of the rennin-angiotensin mechanism and the resulting vasoconstriction indirectly lead to the release of aldosterone from the adrenal cortex, which promotes the retention of

sodium and water. The increased concentration of sodium in the blood stimulates the release of antidiuretic hormone (ADH) by the pituitary gland. ADh causes the kidneys to retain water further in an effort to raise blood volume and BP. These secondary regulatory mechanisms may take hours or days to respond to changes in BP. DIAGNOSTICS Diagnosis of shock is based on the patient's symptoms, as well as criteria including a significant drop in blood pressure, extremely low urine output, and blood tests that reveal overly acidic blood with a low circulating concentration of carbon dioxide. Other tests are performed, as appropriate, to try to determine the underlying condition responsible for the patient's state of shock.

Three Stages of Shock: There are three stages of shock: Stage I (also called compensated, or nonprogressive), Stage II (also called decompensated or progressive), and Stage III (also called irreversible). In Stage I of shock, when low blood flow (perfusion) is first detected, a number of systems are activated in order to maintain/restore perfusion. The result is that the heart beats faster, the blood vessels throughout the body become slightly smaller in diameter, and the kidney works to retain fluid in the circulatory system. All this serves to maximize blood flow to the most important organs and systems in the body. The patient in this stage of shock has very few symptoms, and treatment can completely halt any progression. Medical Management Medical treatment is directed toward identifying the cause of the shocks, correcting the underlying disorder so that shocks does not progress, and supporting those physiologic processes that thus far have responded successfully to the threat. Nursing Management o monitoring tissue perfusion o reducing anxiety o promoting safety In Stage II of shock, these methods of compensation begin to fail. The systems of the body are unable to improve perfusion any longer, and the patient's symptoms reflect that fact. Oxygen deprivation in the brain causes the patient to become confused and disoriented, while oxygen deprivation in the heart may cause chest pain. With quick and appropriate treatment, this stage of shock can be reversed.

Medical Management These include use of appropriate intravenous fluids and medications to restore tissue perfusion by: o optimizing intravascular volume o supporting the pumping action of the heart o improving the competence of the vascular system Nursing Management o preventing complications o promoting rest and comport o supporting family members In Stage III of shock, the length of time that poor perfusion has existed begins to take a permanent toll on the body's organs and tissues. The heart's functioning continues to spiral downward, and the kidneys usually shut down completely. Cells in organs and tissues throughout the body are injured and dying. The endpoint of Stage III shock is the patient's death. Medical Management During the irreversible stage of shock is usually the same as for the progressive stage. Although the patients conditions may have progressed from the progressive to the irreversible stage, the judgment that the shocks is irreversible can be made of only retrospectively on the basis of the patients failure to respond to treatment. Nursing Management The nurse focuses on carrying out prescribed treatments, monitoring the patients, preventing complications, protecting the patients from injury, and providing comport. Offering brief explanations to the patients about what is happening is essential even if there is no certainty that the patients hears or understand what is being said.

TYPES OF CIRCULATORY SHOCK There are five main types: Hypovolemic (low-volume) shock occurs whenever there is insufficient blood to fill the circulatory system. Neurogenic shock is due to disorders of the nervous system. Anaphylactic (allergic) shock and septic shock are both due to reactions that impair the muscular functioning of the blood vessels. And cardiogenic shock is caused by impaired function of the heart.

HYPOVOLEMIC SHOCK y A shock resulting from insufficient blood volume for the maintenance of adequate cardiac output, blood pressure, and tissue perfusion. Without modification the term refers to absolute hypovolemic shock caused by acute hemorrhage or excessive fluid loss. This is a common type that happens when blood or plasma is lost in such quantities that the remaining blood cannot fill the circulatory system despite constriction of the blood vessels. The blood loss may be external, as when a vessel is severed by an injury, or the blood may be lost into spaces inside the body where it is no longer accessible to the circulatory system, as in severe gastrointestinal bleeding from ulcers, fractures of large bones with hemorrhage into surrounding tissues, or major burns that attract large quantities of blood fluids to the burn site outside blood vessels and capillaries. The treatment of hypovolemic shock requires replacement of the lost volume.

RISK FACTORS o External: trauma, surgery, vomiting, diarrhea, dieresis, diabetes insipidus o Internal: hemorrhage, burns, ascites, dehydration SIGNS AND SYMPTOMS
 Anxiety, restlessness, altered mental state due to decreased cerebral perfusion

and subsequent hypoxia.

 Hypotension due to decrease in circulatory volume.  A rapid, weak, thready pulse due to decreased blood flow combined with

 Cool, clammy skin due to vasoconstriction and stimulation of vasoconstriction.  Rapid and deep respirations due to sympathetic nervous system stimulation and    

acidosis. Hypothermia due to decreased perfusion and evaporation of sweat. Thirst and dry mouth, due to fluid depletion. Fatigue due to inadequate oxygenation. Cold and mottled skin (cutis marmorata), especially extremities, due to insufficient perfusion of the skin.

 Maintain or increase intravascular volume, In hypovolaemic shock, caused by

bleeding, it is necessary to immediately control the bleeding and restore the victim's blood volume by giving infusions of balanced salt solutions. Blood transfusions are necessary for loss of large amounts of blood (e.g. greater than

20% of blood volume), but can be avoided in smaller and slower losses. Hypovolaemia due to burns, diarrhoea, vomiting, etc. is treated with infusions of electrolyte solutions that balance the nature of the fluid lost.  Decrease any future fluid loss via I.V fluid regimen  Give supplementary O2 therapy to commence replacement of fluids via the intravenous route. NURSING MANAGEMENT  Check for patent airway and adequate circulation.  Begin an I.V. infusion with normal saline solution or lactated Ringers solution delivered through a large bore.  Help insert a central venous line and pulmonary artery catheter for hemodynamic monitoring.  Insert an indwelling urinary catheter.  Draw an arterial blood sample to measure ABG levels.  Obtain and record the patients blood pressure, pulse and respiratory rates, and peripheral pulse rates.  Monitor the patients CVP, right arterial pressure, pulmonary artery pressure, and cardiac output atleast hourly as ordered.  Measure the patients urine output hourly.  Monitor the patients ABG and electrolyte levels frequently as ordered.  Watch for signs of impending coagulopathy such as petechiae, bruising, bleeding or oozing from guns or venipuncture site.  Explain all procedures and their purposes to ease the patients anxiety.  Discuss the risk associated with blood transfusions to the patient and his family.

CARDIOGENIC SHOCK y y Is a disease state where the heart is damaged enough that it is unable to supply sufficient blood to the body. Most common causes are: a). acute myocardial infarction b). dilated cardiomyopathy, This is a serious disease in which the heart muscle becomes inflamed (enlarged and stretched) and doesn't work as well as it should. c). acute myocarditis d). arrhythmias

RISK FACTORS  Being age 65 or older  Having a history of heart failure or previous heart attack  Having blockages (coronary artery disease) in several of your heart's main arteries

SIGNS AND SYMPTOMS o Similar to hypovolaemic shock but in addition:  Distended jugular veins due to increased jugular venous pressure.  Absent pulse due to tachyarrhythmia. MEDICAL MANAGEMENT o The main goals of the treatment of cardiogenic shock are the reestablishment of circulation to the myocardium, minimising heart muscle damage and improving the heart's effectiveness as a pump.  Oxygen (O2) therapy to reduces the workload of the heart by reducing tissue demands for blood flow.  Administration of cardiac drugs  Increase hearts pumping action through medication such as Dopamine, dobutamine, epinephrine, norepinephrine, amrinone NURSING MANAGEMENT  Administer oxygen by face mask or artificial airway to ensure adequate oxygenation of tissues.  Adjust the oxygen flow rate to higher or lower level, as blood gas measurements indicate.  Administer an osmotic diuretic, such as mannitol, if ordered to increase renal blood flow and urine output.  Never flex the patients ballooned leg at the hip because this may displace or fracture catheter.  To ease emotional stress, allow frequent rest periods as possible.  Allow family members to visit and comfort the patient as much as possible.  Monitor and record blood pressure, pulse, respiratory rate, and peripheral pulse every 1 to 5 minutes until the patient stabilizes.  Record hemodynamic pressure readings every 15 minutes.  Monitor ABG values, complete blood count, and electrolyte levels.  During therapy assess skin color and temperature and note any changes. Cold and clammy skin may be a sign of continuing peripheral vascular constriction, indicating progressive shock.

NEUROGENIC SHOCK y A shock caused by the sudden loss of the sympathetic nervous system signals to the smooth muscle in vessel walls. This can result from severe central nervous system (brain and spinal cord) damage. With the sudden loss of background sympathetic stimulation, the vessels suddenly relax resulting in a sudden decrease in peripheral vascular resistance and decreased blood pressure. This type, often accompanied by fainting, may be brought on by severe pain, fright, unpleasant sights, or other strong stimuli that overwhelm the usual regulatory capacity of the nervous system. The diameter of the blood vessels increases, the heart slows, and the blood pressure falls to the point where the supply of oxygen carried by the blood to the brain is insufficient, which can bring on fainting. Placing the head lower than the body is usually sufficient to relieve this form of shock.

RISK FACTORS     Spinal cord injury Spinal; anesthesia Depressant action of medications Glucose deficiency

SIGNS AND SYMPTOMS o Similar to hypovolemic shock except in the skin's characteristics. In neurogenic shock, the skin is warm and dry. MEDICAL MANAGEMENT
 Large volumes of fluid may be needed to restore normal hemodynamics  Vasopressors (Norepinephrine)  Atropine (speeds up heart rate and Cardiac Output)

NURSING MANAGEMENT  Elevate and maintain the HOB at least 30 degrees when a patient receives spinal or epidural anesthesia.  If suspected spinal cord injury, immobilize the patient to prevent further damage to the spinal cord.  Applying anti-embolism stockings and elevating the foot of the bed may minimize pooling of blood in the legs.  Passive range of motion of the immobilize extremities helps promote circulation.

 Monitor the patient for signs of internal bleeding that could lead to hypovolemic shock.

ANAPHYLACTIC SHOCK y Is a severe, whole-body allergic reaction. After an initial exposure to a substance like bee sting toxin, the person's immune system becomes sensitized to that allergen. On a subsequent exposure, an allergic reaction occurs. This reaction is sudden, severe, and involves the whole body. Common causes include insect bites/stings, horse serum (used in some vaccines), food allergies, and drug allergies. This type is a rare phenomenon that occurs when a person receives an injection of a foreign protein but is highly sensitive to it. The blood vessels and other tissues are affected directly by the allergic reaction. Within a few minutes, the blood pressure falls and severe dyspnea develops. The sudden deaths that in rare cases follow bee stings or injection of certain medicines are due to anaphylactic reactions.

RISK FACTORS      Penicillin sensitivity Transfusion reaction Bee sting allergy Latex sensitivity Severe allergy to some foods or medications

SIGNS AND SYMPTOMS o Symptoms of anaphylaxis are related to the action of Immunoglobulin E and other anaphylatoxins, which act to release histamine and other mediator substances from mast cells (degranulation). In addition to other effects, histamine induces vasodilation of arterioles and constriction of bronchioles in the lungs, also known as bronchospasm (constriction of the airways). o Symptoms can include the following: Polyuria, respiratory distress, hypotension (low blood pressure), encephalitis, fainting, unconsciousness, urticaria (hives), flushed appearance, angioedema (swelling of the lips, face, neck and throat), tears (due to angioedema and stress), vomiting, itching, diarrhea, abdominal pain, anxiety, and impending sense of doom.

 Identify and remove causative antigen  Administer counter-mediators such as anti-histamine  Oxygen therapy and I.V fluid replacement

NURSING MANAGEMENT  Provide supplemental oxygen and observe. If hypoxia continues, prepare to help insert an artificial airway.  Insert an I.V. line for giving emergency drugs and volume expanders.  Continually reassure the patient and explain all tests and treatments to reduce fear and anxiety.  If the patient undergoes skin or scratch testing. Keep emergency resuscitation equipment nearby during and after the test.  Continuously assess the patients response to treatment.  Monitor vital signs and cardiopulmonary and neurologic function.  Observe for complications associated with anaphylaxis, such as vascular collapse and acute respiratory insufficiency or obstruction.  Closely observe a patient with known allergies for anaphylaxis when giving a drug with high anaphylactic potential.

SEPTIC SHOCK y A shock associated with overwhelming infection, usually by gram-negative bacteria, although it may be produced by other bacteria, viruses, fungi, and protozoa. It is thought to result from the action of endotoxins or other products of the infectious agent on the vascular system causing large volumes of blood to be sequestered in the capillaries and veins; activation of the complement and kinin systems and the release of histamine, cytokines, prostaglandins, and other mediators may be involved. This type, resulting from bacterial infection, is being recognized with increasing frequency. Certain organisms contain a toxin that seems to act on the blood vessels when it is released into the bloodstream. The blood eventually pools within parts of the circulatory system that expand easily, causing the blood pressure to drop sharply. Gram-negative shock is a form of septic shock due to infection with gram-negative bacteria. Most common of this case may its happened to the patients with Meningococcemia, Waterhouse-Friderichsen syndrome, DIC (disseminated intravascular coagulation), Multiple organ dysfunction syndrome (MODS), Acute Respiratory Distress Syndrome (ARDS).

RISK FACTORS      Immunosuppression Extremes of age (<1 yr and >65 yrs) Malnourishment Chronic illness Invasive procedures

SIGNS AND SYMPTOMS o Similar to hypovolemic shock except in the first stages:  Pyrexia and fever, or hyperthermia, due to overwhelming bacterial infection.  Vasodilation and increased cardiac output due to sepsis.  initial chills and fever, warm flushed skin, increased cardiac output, and a lesser degree of hypotension than with hypovolemic shock; if therapy is ineffective, it may progress to the clinical picture associated with hypovolemic shock. MEDICAL MANAGEMENT

Restore intravascular volume via I.V fluid Give supplemental O2 therapy Identify and control source of infection Administer antibiotic Remove risk factor for infection

NURSING MANAGEMENT  All invasive procedures must be carried out with aseptic technique after careful hand hygiene.  Monitor signs of infections on IV lines, arterial and venous puncture sites, surgical incisions, traumatic wounds, urinary catheters and pressure ulcers.  Obtain appropriate specimens for culture and sensitivity.  Fever is common, administer acetaminophen or applying a hypothermia blanket to reduce the temperature.  Monitor blood levels components because they may cause increase and produce toxic effects; and reports changes to the physicians.  Monitor patients hemodynamic status, fluid intake and output, and nutritional status.  Daily weights and monitoring of serum albumin and prealbumin levels help determine the patients protein needs.

GENERAL TREATMENT Fluid Replacement These are administered in all types of shocks. The type of fluids administered and the speed of delivery vary, but fluids are given to improve cardiac and tissue oxygenation, may include crystalloids (electrolyte solutions that move freely between intravascular and interstitial space), colloids (large-molecule intravenous solutions) or blood components. Crystalloid and Solutions The best fluid to treat shocks remains controversial. In emergencies, the best fluid that is readily available. Both crystalloids and the colloids can be given to restore intravascular volume. Blood component therapy is used most frequently in hypovolemic shock. Complications of Fluid Administration Close monitoring of the patients during fluid is necessary to identify side effects and complications. The most common and serious side effect of fluid replacement is cardiovascular overload and pulmonary edema. Vasoactive Medication Therapy Administered in all forms of shocks to improve the patients hemodynamic stability when fluid therapy alone cannot maintain adequate MAP. Vasoactive medications are selected for their action on receptors of the sympathetic nervous system. When vasoactive medications are administered, vital sign must be monitored frequently (at least every 15min until stable or more often if indicated) Nutritional Support Nutritional support is an important aspect of care for the patient with shocks. Increased metabolic rates during shock increase energy requirements. The patient in shocks requires more than 3000 calories daily. The release of catecholamine early in the shocks continuum causes glycogen stores to the depleted in about 8 to 10 hours. y The most important goals in the treatment of shock include: quickly diagnosing the patient's state of shock; quickly intervening to halt the underlying condition (stopping bleeding, re-starting the heart, giving antibiotics to combat an infection, etc.); treating the effects of shock (low oxygen, increased acid in the blood, activation of the blood clotting system); and supporting vital functions (blood pressure, urine flow, heart function). Treatment includes keeping the patient warm, with legs raised and head down to improve blood flow to the brain, putting a needle in a vein in order to give fluids or

blood transfusions, as necessary; giving the patient extra oxygen to breathe and medications to improve the heart's functioning; and treating the underlying condition which led to shock. PROGNOSIS The prognosis of an individual patient in shock depends on the stage of shock when treatment was begun, the underlying condition causing shock, and the general medical state of the patient. PREVENTION The most preventable type of shock is caused by dehydration during illnesses with severe vomiting or diarrhea. Shock can be avoided by recognizing that a patient who is unable to drink in order to replace lost fluids needs to be given fluids intravenously (through a needle in a vein). Other types of shock are only preventable insofar as one can prevent their underlying conditions, or can monitor and manage those conditions well enough so that they never progress to the point of shock.