Sickle cell disease is a genetic blood disorder that affects hemoglobin, causing red blood cells to take on a sickle shape. It occurs most often in those with African or Hispanic ancestry. The most severe type is sickle cell anemia, where two copies of the sickle cell gene are inherited. Symptoms include pain crises when sickled cells block blood vessels. Treatments aim to reduce sickling through hydration, antibiotics for infection, and hydroxyurea to boost fetal hemoglobin levels.
Hydroxyurea therapy increases fetal hemoglobin levels and reduces vaso-occlusive crises in sickle cell disease by inhibiting ribonucleotide reductase and altering cell adhesion molecules, with common side effects being leukopenia and neutropenia. Guidelines recommend hydroxyurea for patients with frequent pain episodes or a history of acute chest syndrome or anemia to reduce complications. Treatment involves monitoring blood counts and titrating dosage up or down based on response and side effects.
This document provides an overview of sickle cell disease (SCD). It discusses the pathogenesis, presentation, complications, diagnosis, and management of SCD. Key points include:
- SCD results from polymerization of abnormal hemoglobin S, causing red blood cell sickling and hemolysis. It most commonly affects those of African descent.
- Presentation varies widely but includes painful vaso-occlusive crises, acute chest syndrome, organ damage to lungs, brain, liver and more. Complications increase mortality.
- Diagnosis involves clinical assessment, screening tests like solubility testing, and definitive testing with hemoglobin electrophoresis. Differential diagnoses include other hemoglobinopathies.
-
Sickle cell disease is a genetic blood disorder characterized by abnormal, rigid red blood cells that can cause painful crises and organ damage. The document discusses the clinical features, pathophysiology, epidemiology, predictors of complications, and considerations for preoperative preparation and anesthesia management in patients with sickle cell disease undergoing surgery. Managing risks like dehydration, hypoxia, acidosis and low temperature is important to prevent sickle cell crises in the perioperative period.
Sickle cell anemia is a genetic blood disorder that affects red blood cells. It is most common among those of African descent. The disease causes red blood cells to become rigid and sickle-shaped, which can cause anemia, pain crises, and other serious complications. Symptoms vary from person to person but often include fatigue, shortness of breath, and pain. The disease is inherited when both parents carry the sickle cell trait, resulting in a 25% chance their child will have the disease.
1. Sickle-cell anemia is a genetic blood disorder caused by a mutation in the hemoglobin gene, resulting in rigid, sickle-shaped red blood cells and chronic hemolysis. It is characterized by severe pain crises, anemia, and life expectancy is shortened.
2. Complications include organ damage from blocked blood flow, infections due to spleen dysfunction, stroke, and bone/joint problems. Painful vaso-occlusive crises are the most common acute symptom.
3. Management involves folic acid, antibiotics, pain medications, blood transfusions, and hydroxyurea. The heterozygous form known as sickle cell trait does not cause symptoms.
This 15-year-old African American female presented with bilateral thigh and hip pain that was worsening over one day. Her symptoms were consistent with a sickle cell disease pain crisis and a urinary tract infection. Sickle cell disease is caused by a single amino acid substitution that causes hemoglobin to polymerize and distort red blood cells into a sickle shape under low oxygen conditions, leading to vaso-occlusive crises and tissue damage. Treatment involves pain medications, fluids, antibiotics, transfusions, and potentially stem cell transplant.
This document provides an overview of sickle cell disease. It defines sickle cell disease as a hereditary form of anemia caused by a mutation in the hemoglobin gene. The presentation summarizes epidemiology, etiology, pathophysiology, signs and symptoms, syndromes, diagnosis, treatment, complications, and prognosis of sickle cell disease. It also provides responses to sample patient case questions.
Hydroxyurea therapy increases fetal hemoglobin levels and reduces vaso-occlusive crises in sickle cell disease by inhibiting ribonucleotide reductase and altering cell adhesion molecules, with common side effects being leukopenia and neutropenia. Guidelines recommend hydroxyurea for patients with frequent pain episodes or a history of acute chest syndrome or anemia to reduce complications. Treatment involves monitoring blood counts and titrating dosage up or down based on response and side effects.
This document provides an overview of sickle cell disease (SCD). It discusses the pathogenesis, presentation, complications, diagnosis, and management of SCD. Key points include:
- SCD results from polymerization of abnormal hemoglobin S, causing red blood cell sickling and hemolysis. It most commonly affects those of African descent.
- Presentation varies widely but includes painful vaso-occlusive crises, acute chest syndrome, organ damage to lungs, brain, liver and more. Complications increase mortality.
- Diagnosis involves clinical assessment, screening tests like solubility testing, and definitive testing with hemoglobin electrophoresis. Differential diagnoses include other hemoglobinopathies.
-
Sickle cell disease is a genetic blood disorder characterized by abnormal, rigid red blood cells that can cause painful crises and organ damage. The document discusses the clinical features, pathophysiology, epidemiology, predictors of complications, and considerations for preoperative preparation and anesthesia management in patients with sickle cell disease undergoing surgery. Managing risks like dehydration, hypoxia, acidosis and low temperature is important to prevent sickle cell crises in the perioperative period.
Sickle cell anemia is a genetic blood disorder that affects red blood cells. It is most common among those of African descent. The disease causes red blood cells to become rigid and sickle-shaped, which can cause anemia, pain crises, and other serious complications. Symptoms vary from person to person but often include fatigue, shortness of breath, and pain. The disease is inherited when both parents carry the sickle cell trait, resulting in a 25% chance their child will have the disease.
1. Sickle-cell anemia is a genetic blood disorder caused by a mutation in the hemoglobin gene, resulting in rigid, sickle-shaped red blood cells and chronic hemolysis. It is characterized by severe pain crises, anemia, and life expectancy is shortened.
2. Complications include organ damage from blocked blood flow, infections due to spleen dysfunction, stroke, and bone/joint problems. Painful vaso-occlusive crises are the most common acute symptom.
3. Management involves folic acid, antibiotics, pain medications, blood transfusions, and hydroxyurea. The heterozygous form known as sickle cell trait does not cause symptoms.
This 15-year-old African American female presented with bilateral thigh and hip pain that was worsening over one day. Her symptoms were consistent with a sickle cell disease pain crisis and a urinary tract infection. Sickle cell disease is caused by a single amino acid substitution that causes hemoglobin to polymerize and distort red blood cells into a sickle shape under low oxygen conditions, leading to vaso-occlusive crises and tissue damage. Treatment involves pain medications, fluids, antibiotics, transfusions, and potentially stem cell transplant.
This document provides an overview of sickle cell disease. It defines sickle cell disease as a hereditary form of anemia caused by a mutation in the hemoglobin gene. The presentation summarizes epidemiology, etiology, pathophysiology, signs and symptoms, syndromes, diagnosis, treatment, complications, and prognosis of sickle cell disease. It also provides responses to sample patient case questions.
Sickle cell disease is an inherited blood disorder caused by a mutation in the beta-globin gene. It most commonly presents with episodes of pain due to vaso-occlusive crises. Other complications include anemia, infections, acute chest syndrome, and stroke. Management involves prevention of complications, treatment of crises, immunizations, penicillin prophylaxis in children, and hydroxyurea therapy in patients with frequent crises to reduce symptoms. Patients require lifelong follow-up to monitor for complications through screening tests and imaging.
As long as knowledge on inherence, diagnosis and preventive measures are limited to very few people, it is difficult to control the spread of the genetic anomaly in our population. Apart from lack of comprehensive knowledge, The findings in this study showed a high level of general awareness about the existence of SCD but comprehensive knowledge about the cause and prevention was low and associated with vast misconceptions. A large percentage did not see its importance in influencing their marital decisions. Perhaps simple interventions that worked in the western countries can also work in India.
The document provides an overview of anemia and iron deficiency anemia. It discusses the typical signs and symptoms of iron deficiency anemia including fatigue, pale skin, spoon-shaped nails, and glossitis. Laboratory findings often show microcytic hypochromic anemia, low serum ferritin, and iron deficiency in bone marrow smears. Treatment involves oral or parenteral iron supplementation for 3-6 months to replenish iron stores.
Sickle cell disease is an inherited red blood cell disorder where the red blood cells become rigid, sticky and form into a sickle shape. It is a lifelong condition that is most common among those with African ancestry. People with sickle cell disease experience chronic anemia, frequent pain crises, infections and potentially early death due to damage to major organs. There is no cure for sickle cell disease but treatment aims to manage pain and prevent complications.
Anemia is the blood disorder, characterized by the reduction in:
1. Red blood cell (RBC) count
2. Hemoglobin content
3. Packed cell volume (PVC).
This occurs because of
Decreased production of RBC
Increased destruction of RBC
Excess loss of blood from the body
Sickle cell disease is caused by a substitution in the beta globin chain that causes red blood cells to take on a sickle shape in low oxygen conditions. This leads to hemolysis and damage to organs over time. Patients experience painful vasoclusive crises. Management of anesthesia for patients with sickle cell disease requires preoperative correction of anemia, aggressive postoperative pain control to prevent sickling, and watchfulness for acute chest syndrome in the postoperative period. Regional anesthesia techniques are not contraindicated.
Sickle cell anemia is a genetic blood disorder caused by a mutation in the hemoglobin gene. It results in abnormal sickle-shaped red blood cells and causes episodes of pain. The disease was first reported in Africa in the 1870s and the genetic basis was discovered in the 1940s. Symptoms can include anemia, pain crises, infections, and organ damage over time. Treatment focuses on managing pain, preventing infections, and blood transfusions in severe cases.
Anemia, thalassemia and hemophilia in childrenNimmy Tomy
This document defines and discusses different types of anemia, including hemolytic anemia. It notes that anemia is a low red blood cell or hemoglobin level, reducing oxygen delivery. Causes include decreased or ineffective red blood cell production, increased red blood cell destruction (hemolytic anemia), and blood loss. Hemolytic anemia specifically refers to the premature breakdown of red blood cells, either inside or outside blood vessels. Causes can be genetic defects affecting red blood cells or acquired factors like immune system attacks or infections. Symptoms range from none in mild cases to fatigue, palpitations, and jaundice in more severe cases.
Haemoglobinopathies are inherited disorders of haemoglobin that cause significant health issues worldwide. The disorders include sickle cell disease and thalassemias, which result from errors in haemoglobin's oxygen carrying capacity. Carriers are healthy but if both parents carry a trait, there is a 25% risk in each pregnancy of a child having a clinically significant haemoglobinopathy. Haemoglobinopathies are most common in populations from Africa, the Mediterranean, Middle East and parts of Asia.
Sickle-cell anemia is a genetic blood disorder caused by an autosomal recessive trait where the normal hemoglobin A is replaced with abnormal hemoglobin S. Symptoms include anemia from the breakdown of red blood cells, frequent pain from blockages in blood vessels, jaundice from liver damage, and limited growth from low oxygen levels. Treatments involve folic acid, pain medications, and procedures for complications, but there is no cure. It predominantly affects those of African descent, and while carriers are resistant to malaria, full sickle-cell anemia can have severe symptoms.
1. The study examined gene frequencies and disease characteristics of sickle cell disease among tribal and Chetti communities in Wayanad district, Kerala, India.
2. Gene frequencies for the sickle cell trait ranged from 1.9-19.6% across communities, with the highest in Wayanadan Chettis. Many homozygotes were observed with the oldest being 48 years old.
3. The disease was mild in 52.2% of cases, with painful crises in 43.5% and splenomegaly and leg ulcers each in 4.3%. Survival of patients appears higher in Kerala than other states.
Sickle cell anemia is a genetic blood disorder where red blood cells become stiff and sickle-shaped. It is caused by abnormal hemoglobin which makes red blood cells sticky and prone to clumping in blood vessels, blocking blood flow. This can lead to pain, infections, organ damage. Symptoms include anemia, fatigue, pain crises. There is no cure, but treatments can help relieve symptoms and complications through medications, blood transfusions, hydroxyurea. Bone marrow transplants may cure some cases. Researchers are studying gene therapy and new drugs to better treat this inherited lifelong condition.
Sickle cell anemia is a genetic blood disorder where red blood cells become misshapen and can clog small blood vessels. It is inherited from both parents and most common in those with African or Mediterranean ancestry. Symptoms include painful episodes, fatigue, infections, and organ damage. Treatment focuses on managing pain, preventing infections, blood transfusions, and for some, medications like hydroxyurea. Complications can include strokes, organ failure, and death if not properly managed.
My presentation in the "CME on Sickle Cell Disease" at Government Medical College, Akola, Maharashtra, India on 19th December 2012 organized by MMC-CME Committee of GMC, Akola and the Department of Pediatrics, GMC, Akola.
Sickle cell disease is a genetic blood disorder caused by a mutation in the beta-globin gene. This mutation causes red blood cells to take on a rigid, sickle shape which can cause episodes of pain and organ damage. The disease manifestations can range from asymptomatic to potentially lethal. Common clinical features include painful vaso-occlusive crises affecting the bones and organs, acute chest syndrome, splenic sequestration, and chronic organ damage to tissues like the lungs, kidneys, and eyes. The inheritance of sickle cell disease and its variants depends on whether a person inherits one or two copies of the sickle beta-globin gene.
The document discusses sickle cell disease, which affects the production of red blood cells and causes them to take on a sickle shape. This can block blood vessels and cause complications like pain, organ damage, and infections. It is most common among those with ancestry from sub-Saharan Africa and other regions. Sickle cell disease is caused by a genetic mutation and can be diagnosed via blood tests. Treatments aim to manage symptoms but for severe cases a bone marrow transplant may potentially cure the condition.
Sickle cell anemia is a genetic blood disorder caused by a mutation in the hemoglobin gene. This mutation causes red blood cells to become rigid, sticky and sickle shaped. When these irregular cells block small blood vessels, it can cause pain, organ damage and other severe complications. Common symptoms include anemia, pain crises, susceptibility to infection. Diagnosis involves blood tests to identify abnormal hemoglobin S. Treatment focuses on pain management, antibiotics, blood transfusions, and hydroxyurea which can help reduce symptoms and increase life expectancy. Newborn screening and genetic counseling can help prevent sickle cell anemia.
Sickle cell anemia is a genetic blood disorder where red blood cells become stiff and sickle-shaped. It is inherited when both parents pass on the sickle cell gene. Sickle cells do not flow smoothly and can get stuck, causing pain crises. Common symptoms include fatigue, shortness of breath, and pain episodes. Complications involve damage to hands/feet, spleen, lungs and other organs. It is diagnosed via newborn screening and treated through medications, transfusions, and potentially a bone marrow transplant. Gene therapy and new drugs are being researched to cure sickle cell anemia.
Chapter 40 Care of Patients With Hematologic Problems IV.pdfKhawlaKhalaf2
This document discusses care of patients with hematologic problems. It focuses on two priority concepts: perfusion and immunity. As an exemplar of perfusion problems, it provides an in-depth overview of sickle cell disease, including its pathophysiology, genetics, incidence, and interprofessional management. It describes how sickle cell disease results in impaired perfusion through vaso-occlusive crises that block blood flow and oxygen delivery to tissues. Proper management requires a collaborative approach to address patients' chronic needs and acute complications.
This document discusses alterations in hematologic function and childhood malignancies. It covers the following key points:
1. It describes the functions of red blood cells, white blood cells, and platelets and how they differ in children compared to adults.
2. It discusses common pediatric hematologic disorders like sickle cell disease and hemophilia, outlining their pathophysiology, clinical manifestations, diagnosis, and treatment.
3. It provides an overview of childhood cancers, including incidence, causes, signs, diagnostic tests, treatment goals like chemotherapy and bone marrow transplant, and the most common types.
Sickle cell disease is an inherited blood disorder caused by a mutation in the beta-globin gene. It most commonly presents with episodes of pain due to vaso-occlusive crises. Other complications include anemia, infections, acute chest syndrome, and stroke. Management involves prevention of complications, treatment of crises, immunizations, penicillin prophylaxis in children, and hydroxyurea therapy in patients with frequent crises to reduce symptoms. Patients require lifelong follow-up to monitor for complications through screening tests and imaging.
As long as knowledge on inherence, diagnosis and preventive measures are limited to very few people, it is difficult to control the spread of the genetic anomaly in our population. Apart from lack of comprehensive knowledge, The findings in this study showed a high level of general awareness about the existence of SCD but comprehensive knowledge about the cause and prevention was low and associated with vast misconceptions. A large percentage did not see its importance in influencing their marital decisions. Perhaps simple interventions that worked in the western countries can also work in India.
The document provides an overview of anemia and iron deficiency anemia. It discusses the typical signs and symptoms of iron deficiency anemia including fatigue, pale skin, spoon-shaped nails, and glossitis. Laboratory findings often show microcytic hypochromic anemia, low serum ferritin, and iron deficiency in bone marrow smears. Treatment involves oral or parenteral iron supplementation for 3-6 months to replenish iron stores.
Sickle cell disease is an inherited red blood cell disorder where the red blood cells become rigid, sticky and form into a sickle shape. It is a lifelong condition that is most common among those with African ancestry. People with sickle cell disease experience chronic anemia, frequent pain crises, infections and potentially early death due to damage to major organs. There is no cure for sickle cell disease but treatment aims to manage pain and prevent complications.
Anemia is the blood disorder, characterized by the reduction in:
1. Red blood cell (RBC) count
2. Hemoglobin content
3. Packed cell volume (PVC).
This occurs because of
Decreased production of RBC
Increased destruction of RBC
Excess loss of blood from the body
Sickle cell disease is caused by a substitution in the beta globin chain that causes red blood cells to take on a sickle shape in low oxygen conditions. This leads to hemolysis and damage to organs over time. Patients experience painful vasoclusive crises. Management of anesthesia for patients with sickle cell disease requires preoperative correction of anemia, aggressive postoperative pain control to prevent sickling, and watchfulness for acute chest syndrome in the postoperative period. Regional anesthesia techniques are not contraindicated.
Sickle cell anemia is a genetic blood disorder caused by a mutation in the hemoglobin gene. It results in abnormal sickle-shaped red blood cells and causes episodes of pain. The disease was first reported in Africa in the 1870s and the genetic basis was discovered in the 1940s. Symptoms can include anemia, pain crises, infections, and organ damage over time. Treatment focuses on managing pain, preventing infections, and blood transfusions in severe cases.
Anemia, thalassemia and hemophilia in childrenNimmy Tomy
This document defines and discusses different types of anemia, including hemolytic anemia. It notes that anemia is a low red blood cell or hemoglobin level, reducing oxygen delivery. Causes include decreased or ineffective red blood cell production, increased red blood cell destruction (hemolytic anemia), and blood loss. Hemolytic anemia specifically refers to the premature breakdown of red blood cells, either inside or outside blood vessels. Causes can be genetic defects affecting red blood cells or acquired factors like immune system attacks or infections. Symptoms range from none in mild cases to fatigue, palpitations, and jaundice in more severe cases.
Haemoglobinopathies are inherited disorders of haemoglobin that cause significant health issues worldwide. The disorders include sickle cell disease and thalassemias, which result from errors in haemoglobin's oxygen carrying capacity. Carriers are healthy but if both parents carry a trait, there is a 25% risk in each pregnancy of a child having a clinically significant haemoglobinopathy. Haemoglobinopathies are most common in populations from Africa, the Mediterranean, Middle East and parts of Asia.
Sickle-cell anemia is a genetic blood disorder caused by an autosomal recessive trait where the normal hemoglobin A is replaced with abnormal hemoglobin S. Symptoms include anemia from the breakdown of red blood cells, frequent pain from blockages in blood vessels, jaundice from liver damage, and limited growth from low oxygen levels. Treatments involve folic acid, pain medications, and procedures for complications, but there is no cure. It predominantly affects those of African descent, and while carriers are resistant to malaria, full sickle-cell anemia can have severe symptoms.
1. The study examined gene frequencies and disease characteristics of sickle cell disease among tribal and Chetti communities in Wayanad district, Kerala, India.
2. Gene frequencies for the sickle cell trait ranged from 1.9-19.6% across communities, with the highest in Wayanadan Chettis. Many homozygotes were observed with the oldest being 48 years old.
3. The disease was mild in 52.2% of cases, with painful crises in 43.5% and splenomegaly and leg ulcers each in 4.3%. Survival of patients appears higher in Kerala than other states.
Sickle cell anemia is a genetic blood disorder where red blood cells become stiff and sickle-shaped. It is caused by abnormal hemoglobin which makes red blood cells sticky and prone to clumping in blood vessels, blocking blood flow. This can lead to pain, infections, organ damage. Symptoms include anemia, fatigue, pain crises. There is no cure, but treatments can help relieve symptoms and complications through medications, blood transfusions, hydroxyurea. Bone marrow transplants may cure some cases. Researchers are studying gene therapy and new drugs to better treat this inherited lifelong condition.
Sickle cell anemia is a genetic blood disorder where red blood cells become misshapen and can clog small blood vessels. It is inherited from both parents and most common in those with African or Mediterranean ancestry. Symptoms include painful episodes, fatigue, infections, and organ damage. Treatment focuses on managing pain, preventing infections, blood transfusions, and for some, medications like hydroxyurea. Complications can include strokes, organ failure, and death if not properly managed.
My presentation in the "CME on Sickle Cell Disease" at Government Medical College, Akola, Maharashtra, India on 19th December 2012 organized by MMC-CME Committee of GMC, Akola and the Department of Pediatrics, GMC, Akola.
Sickle cell disease is a genetic blood disorder caused by a mutation in the beta-globin gene. This mutation causes red blood cells to take on a rigid, sickle shape which can cause episodes of pain and organ damage. The disease manifestations can range from asymptomatic to potentially lethal. Common clinical features include painful vaso-occlusive crises affecting the bones and organs, acute chest syndrome, splenic sequestration, and chronic organ damage to tissues like the lungs, kidneys, and eyes. The inheritance of sickle cell disease and its variants depends on whether a person inherits one or two copies of the sickle beta-globin gene.
The document discusses sickle cell disease, which affects the production of red blood cells and causes them to take on a sickle shape. This can block blood vessels and cause complications like pain, organ damage, and infections. It is most common among those with ancestry from sub-Saharan Africa and other regions. Sickle cell disease is caused by a genetic mutation and can be diagnosed via blood tests. Treatments aim to manage symptoms but for severe cases a bone marrow transplant may potentially cure the condition.
Sickle cell anemia is a genetic blood disorder caused by a mutation in the hemoglobin gene. This mutation causes red blood cells to become rigid, sticky and sickle shaped. When these irregular cells block small blood vessels, it can cause pain, organ damage and other severe complications. Common symptoms include anemia, pain crises, susceptibility to infection. Diagnosis involves blood tests to identify abnormal hemoglobin S. Treatment focuses on pain management, antibiotics, blood transfusions, and hydroxyurea which can help reduce symptoms and increase life expectancy. Newborn screening and genetic counseling can help prevent sickle cell anemia.
Sickle cell anemia is a genetic blood disorder where red blood cells become stiff and sickle-shaped. It is inherited when both parents pass on the sickle cell gene. Sickle cells do not flow smoothly and can get stuck, causing pain crises. Common symptoms include fatigue, shortness of breath, and pain episodes. Complications involve damage to hands/feet, spleen, lungs and other organs. It is diagnosed via newborn screening and treated through medications, transfusions, and potentially a bone marrow transplant. Gene therapy and new drugs are being researched to cure sickle cell anemia.
Chapter 40 Care of Patients With Hematologic Problems IV.pdfKhawlaKhalaf2
This document discusses care of patients with hematologic problems. It focuses on two priority concepts: perfusion and immunity. As an exemplar of perfusion problems, it provides an in-depth overview of sickle cell disease, including its pathophysiology, genetics, incidence, and interprofessional management. It describes how sickle cell disease results in impaired perfusion through vaso-occlusive crises that block blood flow and oxygen delivery to tissues. Proper management requires a collaborative approach to address patients' chronic needs and acute complications.
This document discusses alterations in hematologic function and childhood malignancies. It covers the following key points:
1. It describes the functions of red blood cells, white blood cells, and platelets and how they differ in children compared to adults.
2. It discusses common pediatric hematologic disorders like sickle cell disease and hemophilia, outlining their pathophysiology, clinical manifestations, diagnosis, and treatment.
3. It provides an overview of childhood cancers, including incidence, causes, signs, diagnostic tests, treatment goals like chemotherapy and bone marrow transplant, and the most common types.
Hematology oncology-nurs 3340 fall 2017Shepard Joy
This document outlines learning outcomes and content related to alterations in hematologic function and childhood malignancies. It begins by describing the functions of red blood cells, white blood cells, and platelets. It then discusses differences in pediatric hematopoiesis compared to adults. Specific topics covered include anemia, sickle cell disease, hemophilia, and childhood cancers. Nursing care is discussed for conditions such as vaso-occlusive crises, bleeding episodes, and cancer diagnosis and treatment.
This document provides guidance on managing sickle cell disease in community care settings. It discusses screening, preventing, and treating common complications of sickle cell disease. Key points include screening children for stroke risk with annual transcranial Doppler ultrasounds and treating vasoclusive crises and acute chest syndrome promptly with analgesics and antibiotics respectively. Immunizations against pneumonia and meningitis are also recommended.
PAIN MANAGEMENT IN SICKLE CELL DISEASE AND CRISIS.pptxCampusHub360
Sickle cell disease is a public health disease, it is not new to most of us, some of us have relatives, friends or even colleagues who suffer from sickle cell disease while some have nursed these people. Sickle cell disease poses significant challenges to the global population health. It contributes significantly to the morbidity and mortality of pediatric and adult population.
SICKELE CELL DISEASE MODULE 3 SEJOJO.pptx TO BE PRESENTED. IN TRAININGS.pptxSEJOJO PHAAROE
Sickle cell anemia is a genetic disorder whereby red blood cells are abnormally shaped, causing problems with the flow of blood through the body as well as transport of oxygen throughout the body
inheritance is Autosomal because its a blood disorder and systemic disorder
its caused by mutation on B-chain of the globulin chain , where red blood cells (RBCs) become sickle/crescent shaped
Cells get destroyed in narrowed thin blood capillaries , RE system and cause anaemia Blockage in thin layers body
The document discusses sickle cell disease (SCD) and its pathophysiology. Key points include:
1. The pathophysiology of SCD is complex and involves more than just red blood cell sickling - it also includes endothelial activation, enhanced adhesion of cells, neutrophil activation, polymerization of hemoglobin S, and chronic hemolysis.
2. Vaso-occlusion, the clinical hallmark of SCD, is caused by adhesion of sickled erythrocytes and leukocytes to the endothelium, resulting in vascular obstruction and tissue ischemia.
3. While painful vaso-occlusive crises (VOCs) are visible manifestations of SCD, subclinical ongoing vas
1) Hemoglobinopathies are inherited disorders affecting hemoglobin structure or production, ranging from asymptomatic to fatal. The most common types are sickle cell disease and thalassemias.
2) Thalassemias are caused by deficient production of globin chains, leading to imbalanced globin synthesis and red blood cell damage. Beta thalassemias result from low beta chain production while alpha thalassemias involve alpha chains.
3) Clinical features vary by specific disorder from mild anemia to transfusion-dependent anemia and organ damage. Management involves treatment of complications, transfusions, chelation therapy, and in severe cases, stem cell transplant.
Sickle cell disease is a genetic disorder caused by a mutation in the beta-globin chain of hemoglobin. This causes red blood cells to become rigid and sickle-shaped, leading to anemia, pain crises, and damage to organs over time. The most common acute complications are vaso-occlusive pain crises, acute chest syndrome, and stroke. Treatment focuses on prevention, management of pain and infections, and addressing chronic organ damage through screening and medications.
The document provides an overview of approach to anemia in children. It begins with definitions of anemia and discusses clinical features and etiologies. Common causes of anemia include impaired red blood cell production, increased red blood cell destruction, and blood loss. The document reviews physiological neonatal anemia and pathological neonatal anemia. It outlines the clinical approach including history, physical exam, and initial lab workup. The approach depends on red blood cell indices and reticulocyte count to guide further testing and diagnosis of the underlying cause.
1. Sickle cell disease is a group of inherited blood disorders caused by a mutation in the beta-globin gene resulting in abnormal hemoglobin S. This document discusses sickle cell anemia, its inheritance, pathophysiology, clinical manifestations, complications, and management.
2. Key symptoms include painful vaso-occlusive crises, acute chest syndrome, strokes, and susceptibility to infections. Management involves prevention, pain management, antibiotics, hydroxyurea, and blood transfusions.
3. Understanding the inheritance patterns and genetic counseling is important as sickle cell disease has a major health impact and often requires lifelong multidisciplinary care.
Thalassemia is a genetic blood disorder characterized by reduced or absent globin chains that make up hemoglobin. There are two main types: alpha thalassemia affects alpha globin production and beta thalassemia affects beta globin production. Thalassemia major occurs when defective genes are inherited from both parents and results in severe anemia requiring lifelong blood transfusions and iron chelation therapy to remove excess iron from transfusions. Management involves regular blood transfusions to maintain hemoglobin levels, chelation therapy to remove excess iron from transfusions, and potentially spleenectomy. Prognosis depends on treatment adherence but most patients can survive into their 30s with supportive care.
Sickle cell anemia management guidelines provide recommendations for screening, preventing complications, and treating acute issues and chronic conditions in patients with sickle cell disease (SCD). Key points include: screening children annually with transcranial Doppler ultrasound to prevent stroke; administering pneumococcal and meningococcal vaccines; using hydroxyurea or transfusions as disease-modifying therapies; and treating acute complications like vaso-occlusive crises, acute chest syndrome, fever, and splenic sequestration with hydration, antibiotics, analgesics, and transfusions. The guidelines aim to help community providers properly care for patients with SCD.
- Sickle cell disease is a genetic blood disorder caused by a mutation in the beta-globin gene, which results in abnormal hemoglobin called hemoglobin S.
- There are different types of sickle cell disease depending on the specific mutations present. The most severe forms are Hb SS and Hb Sβ0 thalassemia. Sickle cell disease results in hemolytic anemia and blockages in blood vessels.
- Clinical manifestations include pain crises, acute chest syndrome, strokes, and organ damage to the spleen, lungs, kidneys, and bones. Treatment involves pain management, antibiotics, transfusions, and hydroxyurea which can reduce complications by raising fetal hemoglobin levels.
This document discusses pernicious anemia, which results from vitamin B12 deficiency due to a lack of intrinsic factor. It is an autoimmune disease where the immune system destroys stomach cells, preventing vitamin B12 absorption. Symptoms include fatigue, neurological issues, and megaloblastic anemia. Testing includes blood work and biopsy, while treatment involves vitamin B12 therapy and blood transfusions.
Sickle cell anemia is a genetic blood disorder where red blood cells become rigid and sticky and are shaped like sickles or crescents. This causes them to get stuck in small blood vessels and block blood flow, leading to pain crises. It is caused by a mutation where glutamic acid is replaced by valine in the beta hemoglobin gene. Treatments aim to prevent crises and complications through antibiotics to prevent infection, pain management, oxygen therapy, hydration, transfusions, and hydroxyurea which can increase fetal hemoglobin production and decrease attacks. The only potential cure is a bone marrow transplant but it has high risks.
Leukemia is a cancer of the blood and bone marrow that results in the overproduction of immature white blood cells. The exact causes are unknown but risk factors include genetic, environmental, and viral factors as well as radiation exposure. Leukemias are classified as either acute or chronic depending on the speed of progression, and as affecting myeloid or lymphoid cells. Common symptoms include fatigue, fever, bruising, and infections. Diagnosis involves blood tests and bone marrow biopsy showing abnormal white blood cells. Treatment involves chemotherapy, radiation, steroids, stem cell transplant, or targeted therapies to destroy leukemia cells.
This document provides an overview of hemolytic anemias, with a focus on sickle cell disease. It describes the general diagnostic findings of hemolytic anemia and classifies hemolytic anemias as either hereditary (defects within red blood cells) or acquired (external causes). For sickle cell disease specifically, it identifies complications, diagnostic findings, and treatments including health care maintenance and management of painful crises.
Sickle cell anemia is a genetic blood disorder where red blood cells become rigid and sickle-shaped. It is caused by a mutation in the hemoglobin gene. Symptoms include anemia, episodes of pain, frequent infections, and potentially serious complications affecting organs and tissues. Diagnosis is made via a blood test. Treatment focuses on pain management, antibiotics, hydroxyurea, blood transfusions, and potentially a bone marrow transplant to cure the disease.
NURSING MANAGEMENT OF PATIENT WITH EMPHYSEMA .PPTblessyjannu21
Prepared by Prof. BLESSY THOMAS, VICE PRINCIPAL, FNCON, SPN.
Emphysema is a disease condition of respiratory system.
Emphysema is an abnormal permanent enlargement of the air spaces distal to terminal bronchioles, accompanied by destruction of their walls and without obvious fibrosis.
Emphysema of lung is defined as hyper inflation of the lung ais spaces due to obstruction of non respiratory bronchioles as due to loss of elasticity of alveoli.
It is a type of chronic obstructive
pulmonary disease.
It is a progressive disease of lungs.
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Certain chemicals, such as phthalates and parabens, can disrupt the body's hormones and have significant effects on health. According to data, hormone-related health issues such as uterine fibroids, infertility, early puberty and more aggressive forms of breast and endometrial cancers disproportionately affect Black women. Our guest speaker, Jasmine A. McDonald, PhD, an Assistant Professor in the Department of Epidemiology at Columbia University in New York City, discusses the scientific reasons why Black women should pay attention to specific chemicals in their personal care products, like hair care, and ways to minimize their exposure.
2. Sickle Cell Disease (SCD) is a life long
condition with no known cure. It is an
autosomal recessively inherited condition that
affects the structure of the hemoglobin. Within
the United States, SCD occurs most often in
Black American and Hispanic individuals (Brown,
2012; Gill, Lavin, & Sim, 2010).
3. Introduction (continued)
SCD is a group of disorders:
*Sickle Cell Trait (SCT)
*Sickle Cell Anemia (SCA)-most
common
*Sickle Cell Hemoglobin C
*Sickle Cell-Thalassemia
(McCance, 2010)
4. Incidence
*1- 400 to 1-500 live births among blacks
*1 of every 36,000 Hispanic births
*In the general population, the risk of two black
parents having a child with SCD is 0.7%
*According to National Heart, Lung, and Blood
Institute, SCD affects an estimated 70,000 to
100,000 people in the United States (Gill, Lavin, & Sim, 2010).
5. Prevalence
*Tends to occur in people with origins in
equatorial countries, particularly central Africa,
Near East, Mediterranean area, and parts of
India
*Occurs in 7% to 13% of blacks in the
United States
*As high as 45% in East Africans, those
who have the trait, not necessarily the disease
(Brown, 2012).
*Average life span 45-65 years
6. SCT – The individual inherits HbS (hemoglobin S)
from one parent and HbA (normal hemoglobin) from the
other (AKA: the sickle cell carrier)
SCA – The individual has two HbS genes (HbSS), the
most severe type of SCD, and homozygous form of SCD
(65% of SCD)
Sickle cell HB C disease - heterozygous form in
which the child will inherit another type of abnormal
hemoglobin from one parent (25% of SCD)
(Gill, Lavin, Sim, 2010)
7. Characteristics (continued)
Sickle Cell Thalassemia – Individual inherits one
copy of HbS gene and one copy of the Hb beta-thalassemia
gene.
*Beta Thalassemia genes result in reduced or
lack of expression of normal HbA
*Individual thus has only HbS or mostly HbS
with very small percentage of HbA (Gill, Lavin, & Sim, 2010)
*Life span of sickled cell—10 to 20 days,
normal RBC—120 days (Brown, 2012)
8. NOTE: Hemoglobin polymerization,
leading to erythrocyte rigidity and vaso-occlusion
is central to the pathophysiology
of Sickle Cell Disease (Rees, Williams, & Gladwin, 2010).
*Deoxygenation - the most important variable
in determining the occurrence of sickling
(McCance, 2010; Rees, Williams, & Gladwin, 2010)
10. *Mutation of the 6th amino acid produces a hydrophobic
motif in the deoxygenated HbS tetramer
*Flexible, oxygenated, HbS-containing red blood cells
(SRBC) traverse the capillaries and release oxygen
*Once oxygen released, polymerization begins and the
SRBC becomes rigid
(Sangkatumvong et al., 2011)
*Crystallization produces polymer nucleus, growing and
filling the erythrocyte
11. *Architecture and flexibility of erythrocyte disrupted,
promoting cellular dehydration
(Rees, Williams, & Gladwin, 2010)
*Increased osmolality of plasma draws water out of
erythrocyte
*Decreased plasma volume occurs in states of dehydration
causing increased viscosity of blood
*Cell stretched to elongated crescent
(www.bydeway.com)
McCance, 2012)
12. Pathophysiology (continued)
*HbS not bound with oxygen forms aggregates of
semisolid gel that become stacked within the
erythrocyte
*Cell changed from flexible, nourishing cell to an
inflexible obstacle that starves and damages tissues
(Sangkatumvong et al., 2011)
*SRBC may become lodged if failing to escape
microvasculature
*Microvascular bed becomes obstructed
(Designerget.com)
13. Pathophysiology (continued)
(Prathama.org/tbds.php)
*Increased viscosity of blood--the final common pathway
leading to multiple pathologic effects
*Viscous blood flows slowly, promotes vascular
obstruction by increasing opportunities for sickling while
decreasing opportunities for reoxygenation in lungs (McCance,
2010)
14. Pathophysiology (continued)
*An oxygenated sickle-shaped red blood cell
(RBC) returns to biconcave shape…however…
*Repeated cycles of sickling tires the RBC
*RBCs eventually become irreversibly sickle
shaped
*Rigid sickled RBCs easily trapped, circulatory
survival shortened, and chronic hemolysis is end
result (Wells, DiPiro, Schwinghammer, & DiPiro, 2012)
15. *Autosomal recessive
genetic disorder
*For disease to manifest,
one must inherit two copies
of sickle cell gene (one from
each parent)
*Results from an amino acid
substitution in the Beta
globin chain of hemoglobin
(medical-dictionary.thefreedictionary.com/sickle+cell+disease)
16. Manifestations of SCD
*General: pallor, fatigue, jaundice, and irritability
*Acute: Crises (4 types)
1. Vascular/thrombotic – sickling in microcirculation, vasospasm
and “logjam” effect, extreme pain
2. Aplastic – transient cessation of red blood cell production,
acute anemia, extremely low reticulocyte count (result of viral
infection)
3. Sequestration – large amounts of blood pooled in liver/spleen
(seen only in a young child)
4. Hyperhemolytic (rare) – accelerated rate of red blood cell
destruction usually in association with certain drugs and
infection (McCance, 2010)
18. Complications of SCD
(medical-dictionary.thefreedictionary.com/sickle+cell+disease)
Complications (examples of
those visualized)
Acute chest syndrome
-New pulmonary infiltrate with chest
pain, temperature >38.5° C,
tachypnea, and cough
-Lung becomes spleen-like, sickled
red cells attach to endothelium, fail
to become reoxygenated, increased
inflammation, lung infarcts
-Poor prognosis, leading cause of
morbidity, 25% of all deaths in those
with SCD (McCance, 2010)
19. Complication Examples (continued)
Glomerular Disease
-Characterized by damage to glomeruli
-Protein and often red blood cells allowed to leak into
urine
-Caused by sickled cells in the kidney
-Results in nephropathy progressing to renal failure
Cholecystitis
-An inflammation of the gall bladder from a gallstone in
cystic duct
-Can be caused by hemolysis resulting in increased
bilirubin leading to gallstone formation (McCance,2010)
20. Complication Examples (continued)
Cerebral Injury
-Common in SCD
-Approximately 45% of patients with SCD have
cerebral infarcts
-One study showed 37% patients had a silent stroke by
age 18
-Result of vascular congestion of sickled cells
(Sangkatumvong, 2011)
21. Precipitating / Sickle Triggering
Factors
One or more of the following stressors:
*Hypoxemia
*Increased hydrogen ion concentration in
blood, low pH (decreases hemoglobin’s
affinity for oxygen)
*Increased plasma osmolality
*Decreased plasma volume – dehydration
*Low temperature (McCance, 2010)
*Infection
*Pregnancy (growing fetus causes strain,
can lead to crisis)
*Physical/Mental stress (Brown, 2012)
22. Pharmacologic Treatment of SCD
Note: Pharmacologic treatment is based primarily on symptoms
presented, no know cure for SCD. Dosages of medications are
patient specific (age, weight)
GENERAL
-SCD patients should receive routine immunizations, plus
influenza, meningococcal, and pneumococcal vaccinations
(Kiera105.wordpress.com)
-Children are recommended (up to age of 5) to be placed on
prophylactic Penicillin by 2 months of age
Pen VK (Penicillin V Potassium) 125 mg. orally twice daily
until age 3 then 250 mg. twice daily until age 5 (Wells et al., 2012)
23. Pharmacologic Treatment of SCD
(continued)
GENERAL (continued)
-Or Benzathine penicillin 600,000 units IM (Intramuscularly)
every 4 weeks from 6 months to 6 years old
-Adults, pregnant women and all patients with chronic hemolysis
should take Folic acid 1 mg. daily (Wells et al., 2012)
SPECIFIC
1. Pain (severe)
Opioids: Morphine or Dilaudid – first line of management – PCA
(patient-controlled analgesia) appropriate method for continuous
dose of analgesic, reducing peaks and troughs of pain
24. Pharmacologic Treatment of SCD
(continued)
Other analgesics: Fentanyl, Oxycodone (2nd line opioids)
Non-steroidal anti-inflammatory: Ibuprofen, Ketorolac
(Brown, 2012)
2. Pain (moderate)
Weak Opioid: Codeine or Hydrocodone (Wells et al., 2012)
Mild Analgesic: Acetaminophen
3. Dehydration
3-4 liters of 0.9% Normal Saline for the adult with accurate
fluid monitoring to avoid overload (Brown, 2102)
25. Pharmacologic Treatment of SCD
(continued)
4. After 3 or more vaso-occlusive pain crises/acute chest
syndrome/severe symptomatic anemia
Hydroxyurea - chemotherapeutic agent, stimulates HbF (fetal
hemoglobin) which correlates with decreased RBC sickling
and adhesion
-Dosage begins at 10-15 mg/kg daily as single dose,
can be increased 5 mg/kg/day every 8-12 weeks
Maximum dosage 35 mg/kg/day (Wells et al., 2012)
5. Acute Chest Syndrome
Broad-spectrum antibiotics
26. Pharmacologic Treatment of SCD
(continued)
Macrolide (Azithromycin 500 mg twice daily,
Clarithromycin 500 mg twice daily, Erythromycin 500 mg
twice daily)
Quinolone (Ciproflaxin 400 mg IV every 8-12 hrs.
depending on severity of infection, or Levoflaxin 500 mg IV
daily)
6. Infection-Fever >38.5°C (Empiric antibiotic therapy with
coverage against encapsulated organisms recommended)
Cefotaxime 1-2 gms every 8 hours IV for moderate to severe
infections (for inpatients)
Ceftriaxone 1-2 gms daily IM or IV (for outpatients)
(Wells et al., 2012; Alexander et al., 2012)
27. Pharmacologic Treatment of SCD
(continued)
OTHER
1. Antipruritic & Antiemetics: Hydroxyzine, Compazine
2. Opioid Antagonist: Naloxone (to counteract effects of
respiratory depression caused by opioids)
3. Laxative: (reduce constipation due to opioid usage)
Docusate, Lactulose, Senna (Brown, 2012)
28. Non-Pharmacological Interventions
*Heat application to areas of pain
*Limiting movement of painful extremity
*Regular check ups with eye doctor, watching for vessel
damage to vessels in eye and retina
*Oxygen therapy when hospitalized
*Hydration
29. Non-Pharmacological Interventions
(continued)
(Sciencephoto.com)
*Blood transfusions – in children to maintain HbS <30%
and prevent stroke and recurrence of stroke
(Wells et al., 2012)
*Well-balanced diet high in protein and fiber (will
facilitate tissue repair and decrease risk of constipation)
*Distraction Therapy – television, music (Brown, 2012)
30. Experimental Treatments
*Gene Therapy – Researchers exploring possibility of
inserting normal gene into bone marrow of children
with SCD to promote production of normal HgB
*Butyric Acid – A food additive that increases amount
of fetal hemoglobin (HbF) in blood
*Clotrimazole (Mycelex) – An OTC medication used
to treat fungal infections helps prevent loss of water
from RBCs and may reduce formation of sickle cells
31. Experimental Treatments
(continued)
*Nitric Oxide – Helps keep blood vessels open and
reduces stickiness of RBCs, those with SCD have
lower levels of nitric oxide
*Nicosan – Herb used in Nigeria to prevent episodes
of sickle cell crisis
*Decitidine – A medication that increases the HgF
levels
32. Experimental Treatments
(continued)
*GMI-1070 – Experimental pan-selection inhibitor
that treats vaso-occlusive crises, inhibiting a key
early step in the inflammatory process involved
with cell adhesion
*Bone Marrow Transplant – A potentially curative
treatment, must come from matched donor, usually
a family member who does not have SCD
(Gill, Lavin, & Sim, 2010)
33. Nursing Implications & Key Points
*Education (Personal)
- Important due to misconceptions about
patients with SCD, usually perceived as “drug-seeking”
- Pain management (i.e.: Morphine vs. Demerol)
- Health maintenance/promotion
- Assessment skills for signs & symptoms of crisis
- Psychological factors and SCD (Valentine et al., 2010)
34. Nursing Implications & Key Points
(continued)
*Care of the Patient with SCD
- Adequate pain management
- Enhanced communication skills
- Familiarity of cultural dynamics
- Utilization of the nursing process (assessment,
intervention, re-assessment, and evaluation)
35. Nursing Implications & Key Points
(continued)
- Recognition of SCD patient in crisis
(cardiovascular accident, acute chest syndrome,
organ damage from SCD)
- Monitoring labs (CBC, Fe, TIBC, renal function
electrolytes, bilirubin, ALT)
- Vital Sign monitoring (for early detection of
respiratory failure, shock, cardiovascular
accident, acute chest syndrome)
36. Nursing Implications & Key Points
(continued)
- Neurologic assessment (noting any changes
in behavior, slurred speech, headaches,
vomiting)
- Infectious screening (mid-stream urine,
sputum
culture) Note: infections are common
precipitants of acute vaso-occlusive crises
- Promotion of well-balanced diet--high in
protein and fiber
37. Nursing Implications & Key Points
(continued)
- Psychological support--patients experience
fear, anxiety, and stress caused by having an
unpredictable condition
- Fluid monitoring (Accurate intake & output)
- Empathy, compassion, a non-judgmental
attitude, and taking time to listen are extremely
helpful in assisting a patient’s recovery
38. Nursing Implications & Key Points
(continued)
- Identify patient’s mental capacity and
willingness to adjust when changes in health
circumstances make that necessary
- Patient education—medications, coping
mechanisms for pain, early recognition of crisis
- Advocate for patient in accessing other services
(Social Services, Occupational Therapy, Support
groups)
39. Nursing Implications & Key Points
(continued)
- Discharge planning (rest until energy levels
increase, pain management, hydrate, well-balanced
diet {dietician may be advantageous
in relation to cultural foods}, review of
medications, follow-up appointments with
primary physician and/or hematologist)
- Achievable goal setting for patient (pain
management, coping strategies)
(Brown, 2012)
40. Conclusion
SCD is a very complex genetic condition,
showcased by episodes of excruciating unpredictable
pain. Many people can and do lead active lives and
cope well with their illness, having developed a
number of coping strategies. “Patients can live with
their condition and not live by it” and should be
encouraged to do so (Brown, 2012, p. 96).
41. References
Alexander, J. et al. (2012). Drug information handbook
for advanced practice nursing (13th). Hudson,
OH: Lexicomp.
Brown, M. (2012). Managing the acutely ill adult with
sickle cell disease. British Journal of Nursing,
21(2), 90-96.
Gill, V., Lavin, J., & Sim, M. (2010). Managing sickle.
Nursing Made Incredibly Easy, 24-31.
doi:10.1097/01.NME.0000388522.79370.d2
42. References (continued)
McCance, S. (2010). Pathophysiology: The biologic basis for
disease in adults and children (6th). Maryland Heights,
MO: Mosby.
Rees, D., Williams, T., & Gladwin, M. (2010). Sickle-cell
disease. Lancet, 376, 2018-2031.
doi:10.1016/S0140-6736(10)61029-X
Sangkatomvong, S., Khoo, M., Kato, R., Detterich, J., Bush, A.,
Keens, T., Meiselman, H.,…Coates, T. (2011).
Peripheral vasoconstriction and abnormal parasympa-thetic
response to sighs and transient hypoxia in sickle
cell disease. American Journal of Respiratory and
Critical Care Medicine, 184, 474-481.
doi:10.1164/rccm.201103-05370C
43. References (continued)
Valente, S., Alexander, J., Blount, M., Fair, J., Goldsmith, C., &
Williams, L. (2010). Sickle cell disease in emergency
department: Education for emergency nurses. JOCEPS:
The Journal of Chi Eta Phi Sorority, 54(1), 11-14.
Wells, B., DiPiro, J., Schwinghammer, T., & DiPiro, C. (2013).
Pharmacotherapy handbook (8th). New York:McGraw-
Hill.