Sickle cell disease (SCD) is highly prevalent in sub-Saharan Africa, including Lesotho where it accounts for substantial morbidity and mortality. SCD is a life-threatening genetic disorder that is best managed when diagnosed early by newborn screening. The incidence and cases are not reported in Lesotho. Newborn screening which is paramount for early diagnosis and enrolment of affected children into a comprehensive care programme is not available in Lesotho. Up to now, this strategy has been greatly impaired in resource-poor countries, because screening methods are technologically and financially intensive; affordable, reliable, and accurate methods are needed. We aimed to test the feasibility of implementing a sickle cell disease screening programme using innovative point-of-care test devices into existing immunization programmes in primary health-care settings.
Goal; increase sickle cell disease awareness, diagnosis and management in Lesotho from 0- 20% by 25%
Mission: To improve care of all Sickle Cell Disease patients for their better future and to lower the prevalence of the disease through multi=sectoral, multi-faced coordinated approach towards screening and awareness strategies
Vision: Eliminate sickle cell disease as a public health problem in Lesotho. There is need for increasing the awareness about the disease in the community, implementation of mass screening activities for early identification, building a strong network of diagnosis and linkages, implementing robust monitoring system, strengthening the existing primary health care mechanism to incorporate SCD related strategies, capacity building of primary, secondary and tertiary health care teams and building cost-effective intensive interventions at higher care facilities.
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
This document discusses haemoglobinopathies, which are inherited disorders of haemoglobin synthesis or structure that cause significant morbidity and mortality worldwide. It focuses on sickle cell disorders and thalassaemias, which result from errors in the oxygen-carrying capacity of haemoglobin. Carriers are healthy but if two carriers have a child, there is a risk the child will have a clinically manifested haemoglobinopathy. National control programs aim to screen for carriers and provide treatment and counselling. Pneumococcal vaccination is important to prevent serious infections in children with sickle cell disorders.
This document provides an overview of sickle cell disease (SCD), including its definition, epidemiology, pathogenesis, clinical presentation, diagnosis, treatment, and counseling. SCD is caused by a genetic mutation resulting in abnormal hemoglobin called HbS. It most commonly presents as painful vasoclusive crises, acute chest syndrome, splenic sequestration, or aplastic crises. Treatment involves management of symptoms, antibiotics, hydroxyurea, blood transfusions, and counseling to enable informed family planning decisions.
The document discusses haemoglobinopathies, which are inherited disorders of haemoglobin synthesis or structure that cause significant health issues worldwide. It defines key haemoglobinopathies like sickle cell disease and thalassaemia, how they are inherited, symptoms, prevalence around the world, and importance of national control programs for screening and managing the conditions.
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.
This document provides an introduction and background to a study on sickle cell anemia (SCA) in Jos North, Nigeria. It discusses SCA as a genetic blood disorder with high morbidity and mortality. The incidence, prevalence, diagnosis and treatment of SCA in Jos North is examined. Improving outcomes for children with SCA through early diagnosis, prophylactic therapy and bone marrow transplants is discussed. The research problem is identified as a lack of knowledge among residents about SCA implications and the need for screening before marriage. The study aims to educate residents on SCA and identify carriers to reduce disease transmission risk. The scope covers selected primary health centers in Jos North.
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.
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
This document discusses haemoglobinopathies, which are inherited disorders of haemoglobin synthesis or structure that cause significant morbidity and mortality worldwide. It focuses on sickle cell disorders and thalassaemias, which result from errors in the oxygen-carrying capacity of haemoglobin. Carriers are healthy but if two carriers have a child, there is a risk the child will have a clinically manifested haemoglobinopathy. National control programs aim to screen for carriers and provide treatment and counselling. Pneumococcal vaccination is important to prevent serious infections in children with sickle cell disorders.
This document provides an overview of sickle cell disease (SCD), including its definition, epidemiology, pathogenesis, clinical presentation, diagnosis, treatment, and counseling. SCD is caused by a genetic mutation resulting in abnormal hemoglobin called HbS. It most commonly presents as painful vasoclusive crises, acute chest syndrome, splenic sequestration, or aplastic crises. Treatment involves management of symptoms, antibiotics, hydroxyurea, blood transfusions, and counseling to enable informed family planning decisions.
The document discusses haemoglobinopathies, which are inherited disorders of haemoglobin synthesis or structure that cause significant health issues worldwide. It defines key haemoglobinopathies like sickle cell disease and thalassaemia, how they are inherited, symptoms, prevalence around the world, and importance of national control programs for screening and managing the conditions.
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.
This document provides an introduction and background to a study on sickle cell anemia (SCA) in Jos North, Nigeria. It discusses SCA as a genetic blood disorder with high morbidity and mortality. The incidence, prevalence, diagnosis and treatment of SCA in Jos North is examined. Improving outcomes for children with SCA through early diagnosis, prophylactic therapy and bone marrow transplants is discussed. The research problem is identified as a lack of knowledge among residents about SCA implications and the need for screening before marriage. The study aims to educate residents on SCA and identify carriers to reduce disease transmission risk. The scope covers selected primary health centers in Jos North.
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.
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.
The document provides information about nephrotic syndrome in children, including:
- A case presentation of a 2-year-old male toddler with nephrotic syndrome presenting with fever, cough, facial swelling, decreased urine output, and hypoalbuminemia.
- Definitions of nephrotic syndrome as characterized by massive proteinuria, hypoalbuminemia, edema, and hyperlipidemia. The most common cause is idiopathic nephrotic syndrome.
- Histologic classifications of nephrotic syndrome including minimal change disease, focal segmental glomerulosclerosis, mesangial proliferative glomerulonephritis, and membranoproliferative
1. This document discusses Hepatitis B, including its causes, transmission, prevalence in Yemen, clinical features, diagnosis, and prevention.
2. Yemen has an intermediate prevalence of Hepatitis B, between 2-7% of the population, with mortality from viral hepatitis being 22% of deaths.
3. The disease is caused by the Hepatitis B virus and transmitted through blood or bodily fluids. It can range from acute to chronic infection leading to cirrhosis or liver cancer. Prevention focuses on vaccination, sterilization of medical equipment, and screening blood donors.
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.
HOW COMMON IS SICKLE CELL?
Sickle cell is most common among populations with ancestry in
sub-Saharan Africa; Spanish-speaking regions in the Western Hemisphere (South America, the Caribbean, and Central America); Saudi Arabia; India; and Mediterranean countries such as Turkey, Greece, and Italy. The CDC estimates that in the U.S.
SCD affects 90,000 to 100,000 Americans.
So this ppt will provide adequate knowledge to know about the disease sickle cell anemia.
Thanks
This document discusses sickle cell anemia, including its causes, inheritance pattern, symptoms, diagnosis, and treatment options. Specifically, it is an inherited blood disorder caused by a mutation in the hemoglobin gene that results in abnormal hemoglobin called hemoglobin S. The condition is inherited in an autosomal recessive pattern. Symptoms can include anemia, painful episodes, swelling of hands and feet, infections, and organ damage. Diagnosis involves blood tests. Treatments may include medications, transfusions, hydroxyurea therapy, bone marrow transplantation, and gene therapy.
Sickle Cell Disease (SCD) is major health problem in
Tanzania. Every year, approximately 11000 babies are born
with SCD1, and this number is expected to double by the year
2050. Tanzania has the fourth greatest number of annual
SCD births in all of Africa, and the fifth greatest in the world.
In addition, almost 20% of the Tanzanian population carries a
copy of the sickle gene in a form of sickle cell trait (AS).
Despite these staggering statistics, Tanzania has made
progress in the fight against SCD over the past decade. In
2008, the Ministry of Health and Social Welfare by then,
recognized SCD as a priority disease in the National strategy
for Non-communicable disease 2009- 2015, calling for all
sector to cooperate in combating the disease. A chapter on
SCD was also included in the national Non-communicable
Disease Treatment Manual.
Hydroxyurea is useful in the management of individuals with SCD. It reduces the complications
of SCD in infants, children and adults based on its ability to:
o Increase haemoglobin F levels
o Increase steady state hemoglobin counts
o Lower WBC and PLTs hence moderate the chronic inflammation state in SCD
Indications for starting hydroxyurea:
All children 9 months and above with proven SCD
Adolescents and adults with the following:
o Recurrent VOC ( 3 or more severe episodes requiring admission in the last 12
months)
o Severe and/or recurrent ACS (2 or more episodes in a lifetime)
o History of stroke or abnormal TCD (≥199cm/sec)
o Severe symptomatic chronic anemia that interferes with daily activities or quality of
life
o To reduce the risk of new or recurrent stroke where chronic transfusion therapy is not
feasible.
o Recurrent priapism
o Patient with chronic kidney disease on erythropoietin to improve anemia
#SickleCell disease#Indications for Hydroxyurea#Hamisi Mkindi#CKD#Investigations:
FBP - absolute neutrophil count (ANC) > 1,500/µl, platelet > 100,000/ul, Hb> 6g/dl.
If Hb is less than 6gm/dl do Reticulocyte Count[Do not start hydroxyurea in patients with
Hgb< 6 g/dl AND absolute reticulocyte count (ARC)<100,000/µL]
Serum Creatinine - should be within normal range,
Serum ALT – should not be greater than twice the upper limit of normal,
Bilirubin Total and direct
Urine Pregnancy Test in women
HPLC - Quantification of HbF (if this test cannot be done, Hydroxyurea should be prescribed
nevertheless and an elevated baseline HbF should not affect the decision to initiate
hydroxyurea)
Acute lymphocytic leukemia (ALL) is a cancer of the lymphoid cells in the bone marrow. Early symptoms include fever, fatigue, and enlarged lymph nodes. Diagnostic tests include blood tests, bone marrow biopsies, imaging scans, and spinal fluid tests to determine the extent of the cancer's spread. Treatment involves induction therapy to eliminate leukemia cells, consolidation therapy to destroy remaining cells, and maintenance therapy to prevent regrowth of the cancer.
Thalassemia is a genetically transmitted blood disorder caused by mutations in genes that produce hemoglobin. It affects approximately 7% of the global population and causes 50,000 to 100,000 deaths annually. There are two primary types - alpha thalassemia affects alpha globin genes and beta thalassemia affects beta globin genes. Current treatments include blood transfusions, iron chelation therapy, and bone marrow transplants, but these have complications. Gene therapy is a promising new treatment that involves using a lentiviral vector to insert a normal globin gene into a patient's stem cells and could potentially cure thalassemia without lifelong treatments. The first successful human trial occurred in 2007 in France and showed sustained
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.
The document is a report on a study of sickle cell anemia. It includes an introduction, literature review, discussion, summary, conclusion, and references sections. The report was submitted by Suhaila Abdulla Seif to partially fulfill requirements for a Bachelor of Pharmacy degree. It was conducted under the supervision of Ms. Ramandeep Kaur Sandhu at the University College of Pharmacy, Guru Kashi University in Talwandi Sabo, Punjab, India.
This document discusses the relationship between COVID-19 infection and preeclampsia in pregnant women. It suggests that COVID-19 may increase the risk of developing preeclampsia due to the virus' ability to downregulate ACE2 expression in the placenta. There are immunological and pathological similarities between the two conditions, including endothelial dysfunction. Studies have found higher rates of hypertensive disorders of pregnancy in women infected with COVID-19. Close monitoring is recommended for pregnant COVID-19 patients.
This document discusses acute kidney injury (AKI) in patients with COVID-19. It first defines AKI and describes its stages of severity. It then reviews the epidemiology of AKI in COVID-19, finding incidence rates ranging from 5-57% depending on location and study. The main proposed mechanisms of AKI in these patients include direct viral infection of kidney cells, cytokine storm effects, AKI related to acute respiratory distress syndrome, and kidney-lung crosstalk exacerbating injury. Risk factors for developing AKI include older age, male sex, hypertension, diabetes and pre-existing kidney disease. Treatment involves preventing AKI through fluids and avoiding nephrotoxins, with renal replacement therapy used in severe cases
This document discusses disseminated intravascular coagulation (CID), providing definitions, pathophysiology, risk factors, signs and symptoms, treatment options, and examples of research articles on the topic. Specifically, it defines CID as an alteration in coagulation factor V and VII that results in the excessive production of small blood clots within blood vessels. This causes thrombi to form in small blood vessels throughout the peripheral parts of the body. Risk factors include bacteria, viruses, and congenital factors. Signs may include purpura, microangiopathy, and cardiac congestion. Treatment involves blood transfusions, anticoagulants, and plasmapheresis. Examples of relevant research articles are also provided.
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.
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.
1) The document discusses presentation on leukemia by Qinying Wang. It provides an overview of leukemia including a survey of statistics on prevalence, new cases, deaths and survival rates.
2) The presentation introduces the different types of leukemia, their symptoms, risk factors, methods of testing and stages of progression. Common treatments for leukemia including chemotherapy, biological therapy, targeted therapy and radiation therapy are discussed along with their side effects.
3) Supportive treatments to manage side effects from cancer therapies are also covered. The presentation provides hope by noting that survival rates have improved for some types of leukemia through advances in treatments like stem cell transplants.
Primary glomerular diseases include various forms of glomerulonephritis and nephrotic syndrome. Acute glomerulonephritis is defined by the sudden onset of hematuria, proteinuria, and red blood cell casts. It is caused by an immunological reaction, often due to a streptococcal infection, that results in inflammation and proliferation in the glomerulus. Symptoms include edema, hypertension, and decreased kidney function. Treatment involves a low sodium diet, fluid restriction, antibiotics, corticosteroids, diuretics, and dialysis if needed. With treatment, most cases of acute glomerulonephritis resolve though some may progress to chronic kidney disease.
1. The patient presented with headache and right-sided hemiplegia, consistent with an acute stroke.
2. Strokes in sickle cell disease are most commonly ischemic and occur bimodally, peaking in younger children and those over 30 years old. Hemorrhagic strokes are rarer and peak during the second decade.
3. Diagnostic workup includes blood tests and neuroimaging to determine if the stroke is ischemic or hemorrhagic, as management differs between the two.
This study evaluated the prevalence of acute kidney injury (AKI) in 120 patients with confirmed dengue fever over one year at a hospital in India. The prevalence of AKI among these patients was found to be 27.5%. Several factors were analyzed to identify predictors of AKI in dengue patients, including demographics, severity of illness, laboratory values, and presence of complications. The majority of patients recovered and were discharged, while mortality was observed in 16.7% of cases. This research helps address the lack of data on renal involvement and AKI in dengue virus infection.
OVEARVIEW OF NON-COMMUNICABLE DISEASES IN LEOTHO SEJOJO PHAAROE
Chronic non infectious diseases that are silent and persecute mankind . non-communicable disease (NCD) is a disease that is not transmissible directly from one person to another. NCDs include Parkinson's disease, autoimmune diseases, strokes, heart diseases, cancers, diabetes, chronic kidney disease, osteoarthritis, osteoporosis, Alzheimer's disease, cataracts, and other
Demands for Haemophilia tratment centres to fullfull universal health access...SEJOJO PHAAROE
Haemophilia ia a rare disease that affect 1: 10 000 people. Demands for services, medication and access to cheap effective clotting factors is a human right for people living with haemophilia. A haemophilia registry is a demand from the Government at large .. Essential drug list should also contain haemophilia medication.
we need to be contacted for more information
www.thinktankent.com
More Related Content
Similar to SICKELE CELL DISEASE MANAGEMENT INITIATIVE FOR LESOTHO
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.
The document provides information about nephrotic syndrome in children, including:
- A case presentation of a 2-year-old male toddler with nephrotic syndrome presenting with fever, cough, facial swelling, decreased urine output, and hypoalbuminemia.
- Definitions of nephrotic syndrome as characterized by massive proteinuria, hypoalbuminemia, edema, and hyperlipidemia. The most common cause is idiopathic nephrotic syndrome.
- Histologic classifications of nephrotic syndrome including minimal change disease, focal segmental glomerulosclerosis, mesangial proliferative glomerulonephritis, and membranoproliferative
1. This document discusses Hepatitis B, including its causes, transmission, prevalence in Yemen, clinical features, diagnosis, and prevention.
2. Yemen has an intermediate prevalence of Hepatitis B, between 2-7% of the population, with mortality from viral hepatitis being 22% of deaths.
3. The disease is caused by the Hepatitis B virus and transmitted through blood or bodily fluids. It can range from acute to chronic infection leading to cirrhosis or liver cancer. Prevention focuses on vaccination, sterilization of medical equipment, and screening blood donors.
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.
HOW COMMON IS SICKLE CELL?
Sickle cell is most common among populations with ancestry in
sub-Saharan Africa; Spanish-speaking regions in the Western Hemisphere (South America, the Caribbean, and Central America); Saudi Arabia; India; and Mediterranean countries such as Turkey, Greece, and Italy. The CDC estimates that in the U.S.
SCD affects 90,000 to 100,000 Americans.
So this ppt will provide adequate knowledge to know about the disease sickle cell anemia.
Thanks
This document discusses sickle cell anemia, including its causes, inheritance pattern, symptoms, diagnosis, and treatment options. Specifically, it is an inherited blood disorder caused by a mutation in the hemoglobin gene that results in abnormal hemoglobin called hemoglobin S. The condition is inherited in an autosomal recessive pattern. Symptoms can include anemia, painful episodes, swelling of hands and feet, infections, and organ damage. Diagnosis involves blood tests. Treatments may include medications, transfusions, hydroxyurea therapy, bone marrow transplantation, and gene therapy.
Sickle Cell Disease (SCD) is major health problem in
Tanzania. Every year, approximately 11000 babies are born
with SCD1, and this number is expected to double by the year
2050. Tanzania has the fourth greatest number of annual
SCD births in all of Africa, and the fifth greatest in the world.
In addition, almost 20% of the Tanzanian population carries a
copy of the sickle gene in a form of sickle cell trait (AS).
Despite these staggering statistics, Tanzania has made
progress in the fight against SCD over the past decade. In
2008, the Ministry of Health and Social Welfare by then,
recognized SCD as a priority disease in the National strategy
for Non-communicable disease 2009- 2015, calling for all
sector to cooperate in combating the disease. A chapter on
SCD was also included in the national Non-communicable
Disease Treatment Manual.
Hydroxyurea is useful in the management of individuals with SCD. It reduces the complications
of SCD in infants, children and adults based on its ability to:
o Increase haemoglobin F levels
o Increase steady state hemoglobin counts
o Lower WBC and PLTs hence moderate the chronic inflammation state in SCD
Indications for starting hydroxyurea:
All children 9 months and above with proven SCD
Adolescents and adults with the following:
o Recurrent VOC ( 3 or more severe episodes requiring admission in the last 12
months)
o Severe and/or recurrent ACS (2 or more episodes in a lifetime)
o History of stroke or abnormal TCD (≥199cm/sec)
o Severe symptomatic chronic anemia that interferes with daily activities or quality of
life
o To reduce the risk of new or recurrent stroke where chronic transfusion therapy is not
feasible.
o Recurrent priapism
o Patient with chronic kidney disease on erythropoietin to improve anemia
#SickleCell disease#Indications for Hydroxyurea#Hamisi Mkindi#CKD#Investigations:
FBP - absolute neutrophil count (ANC) > 1,500/µl, platelet > 100,000/ul, Hb> 6g/dl.
If Hb is less than 6gm/dl do Reticulocyte Count[Do not start hydroxyurea in patients with
Hgb< 6 g/dl AND absolute reticulocyte count (ARC)<100,000/µL]
Serum Creatinine - should be within normal range,
Serum ALT – should not be greater than twice the upper limit of normal,
Bilirubin Total and direct
Urine Pregnancy Test in women
HPLC - Quantification of HbF (if this test cannot be done, Hydroxyurea should be prescribed
nevertheless and an elevated baseline HbF should not affect the decision to initiate
hydroxyurea)
Acute lymphocytic leukemia (ALL) is a cancer of the lymphoid cells in the bone marrow. Early symptoms include fever, fatigue, and enlarged lymph nodes. Diagnostic tests include blood tests, bone marrow biopsies, imaging scans, and spinal fluid tests to determine the extent of the cancer's spread. Treatment involves induction therapy to eliminate leukemia cells, consolidation therapy to destroy remaining cells, and maintenance therapy to prevent regrowth of the cancer.
Thalassemia is a genetically transmitted blood disorder caused by mutations in genes that produce hemoglobin. It affects approximately 7% of the global population and causes 50,000 to 100,000 deaths annually. There are two primary types - alpha thalassemia affects alpha globin genes and beta thalassemia affects beta globin genes. Current treatments include blood transfusions, iron chelation therapy, and bone marrow transplants, but these have complications. Gene therapy is a promising new treatment that involves using a lentiviral vector to insert a normal globin gene into a patient's stem cells and could potentially cure thalassemia without lifelong treatments. The first successful human trial occurred in 2007 in France and showed sustained
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.
The document is a report on a study of sickle cell anemia. It includes an introduction, literature review, discussion, summary, conclusion, and references sections. The report was submitted by Suhaila Abdulla Seif to partially fulfill requirements for a Bachelor of Pharmacy degree. It was conducted under the supervision of Ms. Ramandeep Kaur Sandhu at the University College of Pharmacy, Guru Kashi University in Talwandi Sabo, Punjab, India.
This document discusses the relationship between COVID-19 infection and preeclampsia in pregnant women. It suggests that COVID-19 may increase the risk of developing preeclampsia due to the virus' ability to downregulate ACE2 expression in the placenta. There are immunological and pathological similarities between the two conditions, including endothelial dysfunction. Studies have found higher rates of hypertensive disorders of pregnancy in women infected with COVID-19. Close monitoring is recommended for pregnant COVID-19 patients.
This document discusses acute kidney injury (AKI) in patients with COVID-19. It first defines AKI and describes its stages of severity. It then reviews the epidemiology of AKI in COVID-19, finding incidence rates ranging from 5-57% depending on location and study. The main proposed mechanisms of AKI in these patients include direct viral infection of kidney cells, cytokine storm effects, AKI related to acute respiratory distress syndrome, and kidney-lung crosstalk exacerbating injury. Risk factors for developing AKI include older age, male sex, hypertension, diabetes and pre-existing kidney disease. Treatment involves preventing AKI through fluids and avoiding nephrotoxins, with renal replacement therapy used in severe cases
This document discusses disseminated intravascular coagulation (CID), providing definitions, pathophysiology, risk factors, signs and symptoms, treatment options, and examples of research articles on the topic. Specifically, it defines CID as an alteration in coagulation factor V and VII that results in the excessive production of small blood clots within blood vessels. This causes thrombi to form in small blood vessels throughout the peripheral parts of the body. Risk factors include bacteria, viruses, and congenital factors. Signs may include purpura, microangiopathy, and cardiac congestion. Treatment involves blood transfusions, anticoagulants, and plasmapheresis. Examples of relevant research articles are also provided.
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.
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.
1) The document discusses presentation on leukemia by Qinying Wang. It provides an overview of leukemia including a survey of statistics on prevalence, new cases, deaths and survival rates.
2) The presentation introduces the different types of leukemia, their symptoms, risk factors, methods of testing and stages of progression. Common treatments for leukemia including chemotherapy, biological therapy, targeted therapy and radiation therapy are discussed along with their side effects.
3) Supportive treatments to manage side effects from cancer therapies are also covered. The presentation provides hope by noting that survival rates have improved for some types of leukemia through advances in treatments like stem cell transplants.
Primary glomerular diseases include various forms of glomerulonephritis and nephrotic syndrome. Acute glomerulonephritis is defined by the sudden onset of hematuria, proteinuria, and red blood cell casts. It is caused by an immunological reaction, often due to a streptococcal infection, that results in inflammation and proliferation in the glomerulus. Symptoms include edema, hypertension, and decreased kidney function. Treatment involves a low sodium diet, fluid restriction, antibiotics, corticosteroids, diuretics, and dialysis if needed. With treatment, most cases of acute glomerulonephritis resolve though some may progress to chronic kidney disease.
1. The patient presented with headache and right-sided hemiplegia, consistent with an acute stroke.
2. Strokes in sickle cell disease are most commonly ischemic and occur bimodally, peaking in younger children and those over 30 years old. Hemorrhagic strokes are rarer and peak during the second decade.
3. Diagnostic workup includes blood tests and neuroimaging to determine if the stroke is ischemic or hemorrhagic, as management differs between the two.
This study evaluated the prevalence of acute kidney injury (AKI) in 120 patients with confirmed dengue fever over one year at a hospital in India. The prevalence of AKI among these patients was found to be 27.5%. Several factors were analyzed to identify predictors of AKI in dengue patients, including demographics, severity of illness, laboratory values, and presence of complications. The majority of patients recovered and were discharged, while mortality was observed in 16.7% of cases. This research helps address the lack of data on renal involvement and AKI in dengue virus infection.
Similar to SICKELE CELL DISEASE MANAGEMENT INITIATIVE FOR LESOTHO (20)
OVEARVIEW OF NON-COMMUNICABLE DISEASES IN LEOTHO SEJOJO PHAAROE
Chronic non infectious diseases that are silent and persecute mankind . non-communicable disease (NCD) is a disease that is not transmissible directly from one person to another. NCDs include Parkinson's disease, autoimmune diseases, strokes, heart diseases, cancers, diabetes, chronic kidney disease, osteoarthritis, osteoporosis, Alzheimer's disease, cataracts, and other
Demands for Haemophilia tratment centres to fullfull universal health access...SEJOJO PHAAROE
Haemophilia ia a rare disease that affect 1: 10 000 people. Demands for services, medication and access to cheap effective clotting factors is a human right for people living with haemophilia. A haemophilia registry is a demand from the Government at large .. Essential drug list should also contain haemophilia medication.
we need to be contacted for more information
www.thinktankent.com
resilience is the order of the day when life knocks you down
If you have been experiencing some of the following signs and symptoms most of the day, nearly every day, for at least two weeks, you may be suffering from depression:
Persistent sad, anxious, or “empty” mood
Feelings of hopelessness, or pessimism
Irritability
Feelings of guilt, worthlessness, or helplessness
Loss of interest or pleasure in hobbies and activities
Decreased energy or fatigue
Moving or talking more slowly
Feeling restless or having trouble sitting still
Difficulty concentrating, remembering, or making decisions
Difficulty sleeping, early-morning awakening, or oversleeping
Appetite and/or weight changes
Thoughts of death or suicide, or suicide attempts
Aches or pains, headaches, cramps, or digestive problems without a clear physical cause and/or that do not ease even with treatment
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‘Freedom from pain should be seen as a right of every cancer patient and access to pain therapy as a measure of respect for the right in Lesotho
There are several barriers to effective pain control in both A focus on essential pain medicationaccessibility and Pain management cancer and / or HIV/AIDS. Such barriers could be patient related; clinician-related; societal/health system; and political and/or legal-related.
The role of the government in strengthening accreditation readySEJOJO PHAAROE
June 9, 2015 marks World Accreditation Day as a global initiative, jointly established by the International Accreditation Forum (IAF) and the International Laboratory Accreditation Cooperation (ILAC), to raise awareness of the importance of accreditation.
This year’s theme focuses on how accreditation can support the delivery of health and social care.
the day was celebrated across the world with the hosting of major national events, seminars, and press and media coverage, to communicate the value of accreditation to Government, Regulators and the leaders of the business community.
What international support for quality improvement is available to Lesotho national health care initiatives?
• To what extent do national governments around the world specify quality improvement in legislation and published policy?
• What are the distinguishing structures and activities of national approaches to quality improvement within countries?
• What resources (in the form of organizations, funding, training and information) are available nationally?
What maintenance or implementation pathways are available , to prove to the world that Lesotho health care services are of excellence???
Vector borne infectious diseases in the face of climate changeSEJOJO PHAAROE
To understand how climate might affect the incidence of vector-borne diseases, one must first examine the life cycles of the diseases and the environmental parameters associated with each stage
A vector-borne disease is one in which the pathogenic microorganism is transmitted from an infected individual to another individual by an arthropod or other agent, sometimes with other animals serving as intermediary hosts.
The transmission depends upon the attributes and requirements of at least three different living organisms:
- the pathologic agent,
-the vector, and the human host.
intermediary hosts such as domesticated and/or wild animals often serve as a reservoir for the pathogen until susceptible human populations are exposed
We recommend proactive planning
more surveillance of direct impacts, such as changes in the reproduction rate of the vector or the agent, the biting frequency of the vector, and the amount of time the host is exposed to the vector due to changes in temperature, rainfall, humidity, or storm patterns.
Even less information is available to evaluate the impacts of societal and individual activities on the transmission of vector-borne diseases.
Changes in hydrology, agriculture, forestry, and infrastructure in response to global warming may also indirectly affect the interrelationship among the disease agent, vectors, and hosts
Ist Think Tank Entreprenuers Forum in Lesotho ( invitation , call for papers ...SEJOJO PHAAROE
In front of the current main disruptive changes the world is facing, entrepreneurship, creating both wealth and social justice, is key for shaping the world.
It embraces the regionality of the entrepreneurial ecosystem
•Business Entrepreneurs
•Social Entrepreneurs
•Philosophers
•Policy Makers
•Experts
•Academics
•Youth empowerment
•Research scientists
•Health professionals
It is altogether a Think Tank and a Do Tank
•We will share the latest trends and issues about global entrepreneurship.
•We will develop ideas and recommendations aimed at promoting and spreading entrepreneurship regionally .
•We will facilitate and implement entrepreneurial initiatives on a local or global scale
•By Creating Innovative and High-Growth Companies, to generate millions of jobs around the planet;
•Developing Entrepreneurship at the Bottom of the Pyramid, to alleviate poverty and create new markets;
•Implementing Entrepreneurial Education, to disseminate entrepreneurial mindset, skills and competencies throughout society, on a lifelong basis
WE ARE CALLING FOR PAPERS, AND USE THE REGISTRATION FORM ATTACHED FOR YOUR TRAVEL , AND LEETES OF RELEASE AND ACCOMODATION
Post exposure prophylaxis- HEALTH SECTOR WELLNESS SERVICES SEJOJO PHAAROE
HIV and HIV transmission
Indicators for PEP
Pre—requisite for PEP
-baseline and follow up tests
Pre-requisite for PEP Provision
PEP package
ARV- Treatment and adherence
Formative study on hiv workplace for health workers - copySEJOJO PHAAROE
Heterogeneity of the HIV epidemic in Lesotho
Formative Assessment: MOHSW
SECTORAL RESPONSE -MOHSW
ACTIONS TAKEN AND TOOLS AVAILABLE - TO DATE
DISSEMINATION- tools
ADVOCACY FOR BUY IN- - PPP
WELLNESS CHAMPIONS AND STRUCTURES
ADVOCACY-WELLNESS ACTIVITIES
M/E Tools
Cost benefit analysis
Learning and sharing
Action Research : Sejojo Phaaroe
3D MEDIA
Intorduction of work place policy lesotho sensitization paperSEJOJO PHAAROE
This document provides background on the development of a workplace HIV/AIDS policy for health sector facilities in Lesotho. It discusses findings from a formative assessment that identified high rates of HIV infection vulnerability among health workers. The policy aims to manage HIV/AIDS among health sector employees through strengthening prevention, treatment, care and support. It outlines key principles like non-discrimination and confidentiality. The roles and responsibilities of employees, management and affiliated associations in implementing HIV/AIDS programs are also defined.
Management and managing aspects of a wellness service in a health systemSEJOJO PHAAROE
This document discusses management and managing aspects of wellness services in health systems. It describes management as planning, organizing, staffing, leading, and controlling to accomplish goals, while managing refers to putting management theories into practice through business and organizational activities. Good management requires understanding people as coaches and mentors. Key components of an effective management system include policies, systems, programs, procedures, and instructions. Specific personnel are needed to perform tasks like sampling, testing, keeping records, and issuing reports. Managers require knowledge of psychology, business, organizations, and coaching to be effective.
The document summarizes the cytopathology report from March 2013. It outlines achievements, challenges, and the way forward for the cytology section. Key achievements included reducing turnaround time with a new staining machine. Challenges involved poor quality smears from some areas, lack of funding for external quality assurance, and lack of equipment like laptops needed for training. The way forward includes improving client satisfaction surveys, training for smear takers, purchasing laptops for training, providing refresher courses for cytology staff, and evaluating national cancer guidelines. The report calls for strengthening management systems and defining career paths for cytologists.
Overview of medical laboratory regulatory council, objectives 2013SEJOJO PHAAROE
There is an urgent need for professional regulation in the region , because of staff mobilty, and professional demands and aspirations for continous professional development and harmony in ethical practices. Lesotho Medical Regulatory Council is advocated , and this will operate within the Legal Framework of the Health Professional Council
This document discusses cervical cancer prevention strategies in Lesotho. It provides an overview of international conventions on cervical cancer and Lesotho's response. Key points include high rates of cervical cancer in Lesotho compared to other Southern African countries. Prevention strategies discussed include HPV vaccination, screening programs like VIA and Pap smears, and education initiatives targeting policymakers, health workers, and communities. Human papillomavirus (HPV) is identified as the cause of most cervical cancer cases worldwide.
Salivary Gland Cytology case of adenoid cyst carcinoma SEJOJO PHAAROE
This document discusses a case of adenoid cystic carcinoma in the parotid gland based on cytology findings. It describes the smear as showing cellular pleomorphism, anisonucleosis, nuclear notches, pulled out tadpole shapes and chromatin granulation. Syncytial groups and rosettes were also seen. The diagnosis was adenoid cystic carcinoma. The discussion section provides overview information on salivary gland tumors, including classification of malignant and benign tumors, epidemiology, risk factors, presentation and signs of salivary gland masses.
This document describes a case of adenoid cystic carcinoma in the parotid gland of a 52-year-old male based on cytology findings. Cytology of the fine needle aspirate showed cellular pleomorphism, anisonucleosis, nuclear notches, pulled out tadpole cells and coarse chromatin with mitotic figures. The final diagnosis was malignant adenoid cystic carcinoma.
Innovative Medicines Initiative, Call for Proposal 2013 for Health , Pharmacueticals, Nursing , and Laboratory Medicines in Lesotho- EU Horizon 2020 and FP7 Funding
General intro-presentation-of-2013-call-orientations en sejojo disseminateSEJOJO PHAAROE
The document outlines the European Commission's 2013 work programme for collaborative health research, including two calls focusing on innovation to translate research into new therapies, diagnostics, and medical technologies. Key areas of focus include brain diseases, antimicrobial resistance, and comparative effectiveness research. The programme has a budget of over €800 million and encourages cross-border collaboration between researchers, industries, and small- and medium-sized enterprises.
The document discusses promoting EU funding for biomedical research through the HEALTH-NCP-NET project. It provides background on the project and role of National Contact Points (NCPs) in supporting researchers. It then describes guidelines and best practices discussed at a Romania workshop for NCPs, including on intellectual property rights. The workshop aimed to help NCPs provide more equal and effective support to researchers in applying for EU funding.
share - Lions, tigers, AI and health misinformation, oh my!.pptxTina Purnat
• Pitfalls and pivots needed to use AI effectively in public health
• Evidence-based strategies to address health misinformation effectively
• Building trust with communities online and offline
• Equipping health professionals to address questions, concerns and health misinformation
• Assessing risk and mitigating harm from adverse health narratives in communities, health workforce and health system
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These lecture slides, by Dr Sidra Arshad, offer a quick overview of the physiological basis of a normal electrocardiogram.
Learning objectives:
1. Define an electrocardiogram (ECG) and electrocardiography
2. Describe how dipoles generated by the heart produce the waveforms of the ECG
3. Describe the components of a normal electrocardiogram of a typical bipolar lead (limb II)
4. Differentiate between intervals and segments
5. Enlist some common indications for obtaining an ECG
6. Describe the flow of current around the heart during the cardiac cycle
7. Discuss the placement and polarity of the leads of electrocardiograph
8. Describe the normal electrocardiograms recorded from the limb leads and explain the physiological basis of the different records that are obtained
9. Define mean electrical vector (axis) of the heart and give the normal range
10. Define the mean QRS vector
11. Describe the axes of leads (hexagonal reference system)
12. Comprehend the vectorial analysis of the normal ECG
13. Determine the mean electrical axis of the ventricular QRS and appreciate the mean axis deviation
14. Explain the concepts of current of injury, J point, and their significance
Study Resources:
1. Chapter 11, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 9, Human Physiology - From Cells to Systems, Lauralee Sherwood, 9th edition
3. Chapter 29, Ganong’s Review of Medical Physiology, 26th edition
4. Electrocardiogram, StatPearls - https://www.ncbi.nlm.nih.gov/books/NBK549803/
5. ECG in Medical Practice by ABM Abdullah, 4th edition
6. Chapter 3, Cardiology Explained, https://www.ncbi.nlm.nih.gov/books/NBK2214/
7. ECG Basics, http://www.nataliescasebook.com/tag/e-c-g-basics
Integrating Ayurveda into Parkinson’s Management: A Holistic ApproachAyurveda ForAll
Explore the benefits of combining Ayurveda with conventional Parkinson's treatments. Learn how a holistic approach can manage symptoms, enhance well-being, and balance body energies. Discover the steps to safely integrate Ayurvedic practices into your Parkinson’s care plan, including expert guidance on diet, herbal remedies, and lifestyle modifications.
Basavarajeeyam is a Sreshta Sangraha grantha (Compiled book ), written by Neelkanta kotturu Basavaraja Virachita. It contains 25 Prakaranas, First 24 Chapters related to Rogas& 25th to Rasadravyas.
Here is the updated list of Top Best Ayurvedic medicine for Gas and Indigestion and those are Gas-O-Go Syp for Dyspepsia | Lavizyme Syrup for Acidity | Yumzyme Hepatoprotective Capsules etc
Histololgy of Female Reproductive System.pptxAyeshaZaid1
Dive into an in-depth exploration of the histological structure of female reproductive system with this comprehensive lecture. Presented by Dr. Ayesha Irfan, Assistant Professor of Anatomy, this presentation covers the Gross anatomy and functional histology of the female reproductive organs. Ideal for students, educators, and anyone interested in medical science, this lecture provides clear explanations, detailed diagrams, and valuable insights into female reproductive system. Enhance your knowledge and understanding of this essential aspect of human biology.
SICKELE CELL DISEASE MANAGEMENT INITIATIVE FOR LESOTHO
1. Sickle cell disease
Sejojo Phaaroe FIBMS(UK) ; MGSc: CT(IAC) : MLSc(CNAA ) Ie : AHMP(YALE);
+266 50468036
Registered Charted -Medical Scientist- UK
Cytologist - International Academy of Cytology #6467
NLP Practitioner (Neuroscience ) GSF
Lead , Technical Assessor and Master Trainer- ISO15189 SADC -EU.
SADCAS Technical Advisor- ISO17025
2. Learning Objectives
• At the end of this session participants are expected to be able to:
Describe sickle cell disease and types of sickle cell anaemia
Describe epidemiology, risk factors and pathogenesis of sickle cell disease
Recognize clinical presentation of sickle cell disease
Perform clinical and laboratory assessment of patients with sickle cell disease
Diagnose SCD including awareness of the clinical and laboratory features of
the various forms of SCD.
Guide SCD-affected families on how to maintain health and reduce the risk of
complications due to SCD.
3. Learning Objectives (2)
Manage complications of SCD such as pain, fever, acute chest
syndrome, stroke, etc.
Safely transfer those with severe complications to a higher level of
care
• Provide pharmacological and nonpharmacological treatment to
patients with sickle cell disease
• learn how to Implement referral pathway for patients with sickle
cell disease
• Conduct regular follow up monitoring to patients with sickle cell
• know why it is important to integrate sickle cell disease in to NCD
integration programs
• POLICIES AND REGULATORY FRAMEWORKS FOR NCDs INTEGRATION IN LESOTHO
6. Altered
amino acid (G-V)
altered protein
variant - betha
globulin
on chain
affected HGB
itseself
HB GENE
sickle cell
both alpha
betha chains
excess synthesis of
normal globulin
chain
disturbed globulin
sysnthesis rate
betha-globin gene
control production
of b globin
thalassimia
7. Definition of Sickle Cell Disease
• 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
8.
9.
10.
11.
12. cell Disease-(SCD) in short
• Sickle Cell Disease-(SCD) is one of the disorders in a broad group of
hemoglobinopathies
• People with this disease have atypical hemoglobin molecules called
hemoglobin S, which can distort red blood cells into a sickle, or
crescent, shape.
cells are
sticky
13. Hemoglobin
• Hemoglobin is a tetramer composed of two α-globin and two non- α -globin chains
working in conjunction with heme to transport oxygen in the blood
• Normal adult hemoglobin (HbA) is designated αA 2 : βA 2
• Variant hemoglobin is derived from gene abnormalities affecting the α-globin genes
(HBA1 or HBA2) or β-globin (HBB) structural genes (exons)
• More than a thousand hemoglobin variants have been identified relative to changes
in the globin chains. in genomics
• Qualitative changes correspond to amino acid substitutions resulting in
hemoglobinopathies. in proteonomics
• Quantitative changes like amino acid insertions, deletions or mutations in the
intervening sequences (introns) correspond to thalassemia and result in decreased
globin chain production -NGS ADVOCACY
14. Inheritance of sickle cell disease
• People who have sickle cell anemia inherit two faulty
hemoglobin genes
• — hemoglobin S — from each parent.
• Sickle cell disease can also occur when a child inherits one
hemoglobin S gene from one parent and another faulty
hemoglobin gene,
• such as beta (β) thalassemia,
• hemoglobin C,
• hemoglobin D, or
• hemoglobin E, from the second parent.
15. • A person has sickle cell trait when they inherit hemoglobin S
gene from one parent and a normal hemoglobin gene
(hemoglobin A ) from the other.
• They are a carrier of the hemoglobin S gene and can pass it
on when they have a child.
• People who have sickle cell trait are generally healthy.
18. Epidemiology
Sickle gene originates from Africa and India
Historically, it has been traced back to a Ghanaian family who suffered from signs and symptoms suggestive of SCD
in the year 1670 [1].
It was, however, not until 1910 when Dr. James B. Herrick brought attention to a peculiar case of a dental student
who had what is now known as SCD [2].
It is estimated that 5% of the world’s population carry genetic imprints responsible for hemoglobinopathies
(primarily sickle cell and thalassemia)
its now spreading worldwide (1 per 625 live births in America, Medscape)
Highest incidence are found in Subsaharan Africa, India and Middle East
There is sickle cell in Lesotho 1989 ( Sejojo Phaaroe etal) DANIDA/WHO
Median age to acquire renal failure is 23.1 years and life expectancy is 27 years regardless of dialysis (medscape) ie.
SCD
19.
20.
21. global burden
• Global Burden of Diseases, Injuries, and Risk Factors Study (GBD)
2021 --------assessment of prevalence of sickle cell disease and
mortality burden by age and sex for 204 countries 2000 to 2021.
• total births of babies with sickle cell disease increased globally by
13·7%
• The number of people living with sickle cell disease globally increased
by 41·4% (38·3–44·9), from 5·46 million (4·62–6·45) in 2000 to 7·74
million (6·51–9·2) in 2021
22. • COVID-19 has been a powerful reminder of the importance of integrated molecular disease surveillance systems
in advancing public health systems. Scientists from around the world have published open-source analyses of
next-generation sequencing data for SARS-CoV-2, the virus that causes COVID-19, collaborating to further track
the spread of the pathogen and to provide information for critical response efforts. In the coming months and
years, NGS will continue to contribute to better understanding of SARS-CoV-2 including how it may evolve over
time, which has important implications for vaccine and drug development.COVID-19 has been a powerful
reminder of the importance of integrated molecular disease surveillance systems in advancing public health
systems. Scientists from around the world have published open-source analyses of next-generation sequencing
data for SARS-CoV-2, the virus that causes COVID-19, collaborating to further track the spread of the pathogen
and to provide information for critical response efforts. In the coming months and years, NGS will continue to
contribute to better understanding of SARS-CoV-2 including how it may evolve over time, which has important
implications for vaccine and drug development. COVID-19 has been a powerful reminder of the importance of
integrated molecular disease surveillance systems in advancing public health systems. Scientists from around
the world have published open-source analyses of next-generation sequencing data for SARS-CoV-2, the virus
that causes COVID-19, collaborating to further track the spread of the pathogen and to provide information for
critical response efforts. In the coming months and years, NGS will continue to contribute to better
understanding of SARS-CoV-2 including how it may evolve over time, which has important implications for
vaccine and drug development.
24. efforts for Lesotho
• Sickle cell disease mortality burden is highest in children, especially in countries with
the greatest under-5 mortality rates
• establish comprehensive NCDs integration strategies to address morbidity and
mortality associated with sickle cell disease, attainment of SDG 3.1, 3.2, and 3.4
• provide community engagement strategies, health education, family pedigrams ,
multi-sectoral inteventions. eg in early childhood programs
• urgent need for routine and sustained surveillance efforts in Lesotho
• data management to fill Widespread data gaps
• Global organisational efforts to fight diseases, such as measles and tuberculosis,
have contributed to cause-specific mortality declines in children younger than 5
years, yet sickle cell disease has had no such global push
• further research to assess the contribution of conditions associated with sickle cell
disease, and widespread deployment of evidence-based prevention and treatment
for those with sickle cell disease
25. • Universal newborn screening combined with preventive treatment is
feasible and effective even in low-resource locations like Lesotho.
• Early diagnosis of sickle cell disease to allow for risk mitigation and
early treatment intervention, which most likely explains some
improvement in survival
26.
27. Pathogenesis 1
SCD is caused by a point mutation at position 6 of the beta haemoglobin
gene in which a hydrophilic glutamic acid is replaced by valine
The susceptible RBCs once subjected to an oxygen tension of <40mmHg
for about 2-4 minutes they become deoxygenated
HYPROXIA
28.
29.
30.
31. Deoxygenated haemoglobin undergoes hydrophobic interaction with adjacent sickle
haemoglobin forming large polymer and thus RBC becomes less deformable and acquire
sickle shape
Rigid RBCs obstruct microvasculature causing tissue hypoxia which precipitates further
sickling
Sickle cells rapidly haemolyze and have a life span of about 10- 20 days IN STEAD OF 120
DAYS
The initiation of polymerization may be incomplete and reversible if re-oxygenation occurs
early in the process
Repetitive exposure to alternating deoxygenated and oxygenated states can lead to
membrane distortion, oxidative damage and irreversible sickling
32. L
RAAS= regulates blood pressure by increasing sodium (salt) reabsorption,
water reabsorption (retention) and vascular tone (the degree to which your
blood vessels constrict, or narrow)
LOW O2 TENSION
STIMULATES
ERYTHOPOETIN
33.
34. Organ Systems Involved
The renin-angiotensin-aldosterone system is ubiquitous with the
involvement of multiple organ systems, especially the kidneys, lungs,
systemic vasculature, adrenal cortex, and brain
36. Signs and symptoms
• Signs and symptoms of sickle cell disease usually begin in early childhood.
• Characteristic features of this disorder include a low number of red blood
cells (anemia), low wwc
• periodic episodes of pain. --vascular integrity
• The severity of symptoms varies from person to person( mild , moderate ,
severe)
• It causes frequent infections,
• swelling in the hands and legs, pain,
• severe tiredness, and delayed growth or puberty.
37. signs and symptoms of sickle cell anemia vary
between individuals.
• Some of the commonly noted symptoms include:
• Irritability or fussiness in babies
• Severe fatigue or tiredness
• Frequent episodes of pain, also known as crises, in different parts of the body
• Swelling of hands and feet, associated with pain
• Frequent infections
• Delayed growth noted in children and adults
• Vision problems
38. Clinical Manifestation of Sickle Cell Disease
Chief cause for admission of patients is Sickle cell crises
This includes the following:
• Vaso-occlusive crisis
1. Pain Crisis---------------------pain
2. Splenic sequestration-----------------splenomegaly
3. Acute Chest Syndrome----------pain
4. Aplastic Crisis--------------------anaemia
5. Hemolytic Crisis------------------ blood fragidity , bilirubin ,
39. Clinical Manifestation of Sickle Cell (2)
The most common clinical manifestation is pain, which occurs unpredictably and is often excruciating
Acute manifestations that may rapidly become life-threatening include
bacterial sepsis ( b-hemolytic strep, stapplococus, other enterobacteriacae , )
severe anaemia,
acute chest syndrome,
and stroke
Other acute complications include
aplastic crises and
priapism ---
45. Pediatric Problems
• All children presenting with
unexplained acute illness
• acute neurological symptoms,
• loss of vision,
• collapse,
• respiratory symptoms,
• hepatosplenomegaly,
• jaundice,
• swollen limbs,
family history of SCD and sepsis
should be tested for SCD
50. - Causes of Nephrotic Syndrome - in SCD
• Usually caused by damage to glomeruli in the kidneys as a result
of certain diseases such as:
• Minimal change disease (a kidney disease)
• Focal segmental glomerulosclerosis
• Membranous nephropathy
• Diabetic kidney disease
• Systemic lupus erythematosus
• Amyloidosis
• Blood clot in a kidney vein
• Heart failure
51. The risk factors :
• Certain medical conditions such as diabetes, lupus, amyloidosis,
reflux nephropathy and other kidney diseases
• Certain infections such as HIV, hepatitis B, hepatitis C, COVID 19, SVD
and malaria.
• Certain medications such as nonsteroidal anti-inflammatory drugs
• alchohol, drugs that cause autosomal gene abberations in pregnancy
52. NCD’s INTEGRATION DEMAND FOR LESOTHO
• lobbying for Funding to conduct full scale prevelance survey , Awareness
and demand creation ( Family peridgrees and genomics) EU-AFRICA4 MED)
• develop screening protocols and integration in to STG
• capacity building and mentoship program of Health professionals and Med
LAB on SCD diagnosis and Management and monitoring treatment
• conduct Community-Based Chronic Disease Care integration of SCD
• Capacitating lay village health workers (VHWs) to deliver essential services
at the community level to improve access to and outcomes of NCD’s in
LMICs
• develop an Integrated SCD program Country wide
• conduct population NGS on SCD for Lesotho
53. • If untreated for a prolonged period it may lead to
• Blood clots-occur due to loss of proteins that prevent clotting
• High blood cholesterol and elevated blood triglycerides- due to
release of more cholesterol and triglycerides by the liver as it
produces albumin
• Poor nutrition-due to loss of proteins which can result in malnutrition
• High blood pressure- due to uremia
• Acute kidney failure
• Chronic kidney disease – leads to loss of kidney function gradually
54. Starts suddenly, very severe, and it may last for several hours
Commonly long bones are affected, but in the 1st 18 months can involve metatarsals and
metacarpals causing dactylitis (painfull swelling hands and feet)
Splenic sequestration
• Sequestration causes worsening of baseline anemia and increase risk of infection by
encapsulated organism
• This includes H. influenza, S. pneumoniae, S. typhi and N. meningitidis
Acute chest syndrome
o Commonly characterized by chest pain, fever, cough, tachypnea, pulmonary infiltrates,
hypoxemia
o Medical emergency is commonly precipitated by chest infections in children
55. Vaso-oclusive Crises cont
Due to obstruction of microvasculature by rigid sickle cells
Painful crises- can affect any part but is chiefly experienced in the
abdomen, bones, joints and soft tissues
56. Aplastic
Worsening of baseline anemia due to infection of parvovirus B-19
This is single stranded RNA virus that is acquired by respiratory
droplets and a special philia for erythrocytes progenitor
Thus, switches off RBC production and causes drastic drop of
reticulocyte counts
57. Hemolytic Crisi
Accelerated breakdown of RBC that occurs with co-existence of G6PD deficiency
G6PD is a recessive X linked disorder that results to deficiency of the enzyme
reqiured for formation glutathione in Pyruvate Phosphorylation pathway
Glutathione is required for clearing free radicals to prevent oxidative damage
58. Factors that can Precipitate SC -lifestyle
Infections
Low oxygen tension
Concomitant medical conditions (e.g., sarcoidosis, diabetes mellitus, herpes)
Dehydration
Acidosis
Extreme physical exercise
• Physical or psychologic stress
• Alcohol and drugs
• Pregnancy
• Cold weather
62. FREQUENT URINALYSIS IS GOLD STANDARD
Glucose
Bilirubin
Ketone
Specific Gravity
Blood
pH
Protein
Urobilinogen
Nitrite
Leukocyte Esterase
63. Diagnosis
Screening Tests Point of Care (POC)
tests
Confirmatory tests
Sickling Test (non-
diagnostic): All positive
sickling test must be
confirmed by the
approved point of care
tests.
tests released by Licensed
Medical Laboratory
Scientists
Sickle SCAN®
Hemotype SC™
Hemoglobin
Electrophoresis
Iso-Electric Focusing
(IEF)
High-Performance
Liquid
Chromatography
(HPLC).
64. Precautionary Blood test and genetic tests
• If you do not know whether you make sickle hemoglobin, you can find out
by having your blood tested.
• You may also have a genetic test. This way, you can learn whether you
carry a genes — or have the trait — for sickle hemoglobin that you could
pass on to a child.
• Cytogenetics can help determine which type of sickle cell disease you
have or confirm a diagnosis if results from blood tests are not clear.
• Genetic testing can also tell whether you have one or two copies of the
sickle hemoglobin gene.
65. Prenatal screening- protocol
• Healthcare providers can also diagnose sickle cell disease before a
baby is born.
• This is done using a sample of either amniotic fluid (the liquid in the
sac surrounding a growing embryo) or tissue taken from the placenta
(the organ that attaches the umbilical cord to the womb).
• Testing before birth can be done as early as 8 to 10 weeks into the
pregnancy. This testing looks for the sickle hemoglobin gene rather
than the abnormal hemoglobin itself.
• This testing cannot predict the severity of the disease.
66. Newborn screening
• In newborn screening programs, drops of blood from a heel prick are
collected on a special type of paper.
• The hemoglobin from this blood is then tested in a lab. Newborn screening
results are sent to the provider who ordered the test and to your child’s
healthcare provider.
• Providers from a special follow-up newborn screening team will contact
you directly if your child has sickle cell disease. Your child’s providers will
then retest your child to make sure the diagnosis is correct.
• Newborn screening programs also find out whether your baby has
the sickle cell traitexternal link and is a carrier.
• If this is the case, counseling will be offered. Remember that when a child
has sickle cell trait or sickle cell disease, their future siblings or future
children may be at risk.
67. Clinical investigations
• Attacks are diagnosed clinically, i.e. there is no gold standard
diagnostic test
• Hemolysis (anemia and jaundice) is often present, although for
painful crises the diagnosis depends essentially on how the patient
describes the pain
68. Lab Investigations
• For Screening, Diagnosis and Confirmation
Sickling test (Hemoglobin S solubility test and sodium metabisulfite test), not
for infants. Useful after 6 month from birth
Newborn Screening: HPLC fractionation (High performance liquid
Chromatography) and thin Layer/Isoeletric focusing
69.
70.
71. Lab Investigation (2)
Abnormal hemoglobin forms are detected on hemoglobin electrophoresis a form of gel electrophoresis on
which the various types of hemoglobin move at varying speed
Sickle cell hemoglobin (HbSS) and Hemoglobin C with sickling (HbSC) the two most common forms can be
identified from there
urinalysiis - BIOCHEMISTRY , MICROSCOPY CULTURE AND SENSITIVITY
FBC- CBC----blood films including Malaria, Howel joel bodies, reticulocyte count
direct combs test, aminoscenthesis
LFTSs, KFT’s, Cadiac FTs, Endocrinology ( s’e-aldosterone, T4/T3, ca+,mg+, renin, angitensin
Kidney function tests including renin, angitension1, 11 aldosterone , tryptophan and NSEs
Cultures and sensitivities including TORCH,
Cytogenetics. immunocytochemistry ( perl perressioan blue, NSE, silver stains,amyloid, reticulin etc)
NGS
PRECISION MEDICINE -
72. • DNA analysis ---------cytogenetics
• Other tests that may be used to help evaluate someone who is suspected of having or who is known
to have sickle cell trait or disease include:
• Complete blood Count (CBC)
• Blood Smear differential and malaria - (era of climate change and travel episodes )
• Neurological tests SILVER STAINS, (agentaffin and argirophill cells) , TRYPTOPHAN , NSE
• blood gases and Electrolytes- always when infussions and transfusions are done
• Chest X-ray in Acute Chest Syndrome
• Trans-Cranial Doppler Ultrasound (TCD),MRI (with or without angiography) and Neuro-
psychometric studies (NPM)
73. RETICULOCYTES COUNT- in peripheral hospitals
• To perform a reticulocyte count, you can follow these steps123:
• Collect a few drops of blood in EDTA.
• Incubate the blood with new methylene blue solution.
• Prepare a thin smear on a glass slide from the mixture.
• Count the reticulocytes under the microscope.
• Alternatively, you can perform a reticulocyte count with an automated
instrument (hematology analyzer)4.
74.
75.
76.
77.
78.
79.
80.
81.
82.
83.
84. TREATMENT
• aimed at avoiding pain episodes, relieving symptoms, and preventing complications.
• Treatments might include medications and blood transfusions.
• For some children and teenagers, a stem cell transplant might cure the disease .
• Hydroxyurea is a medication that can reduce the frequency of painful crises and might reduce
the need for blood transfusions and hospitalizations .
• glutamine oral powder is another drug that has been recently approved by the FDA for the
treatment of sickle cell anemia. It helps in reducing the frequency of pain crises 1.
• Crizanlizumab is a drug that can help reduce the frequency of pain crises in adults and children
older than 16 1. otherwise use WHO pain step ladder for opioids -PALLIATIVE CARE
• Voxelotor is another drug that can lower the risk of anemia and improve blood flow throughout
the body 1.
• Children with sickle cell anemia might receive penicillin between the ages of about 2 months old
until at least age 5 years. Doing so helps prevent infections, such as pneumonia, which can be
life-threatening to children with sickle cell anemia
86. Follow up Patients with Sickle Cell Disease
• Take history and family gengogram/ pedigre
• Full and thorough Examination
• Blood tests: at each visit (already said visits are yearly for older well children
• Provide / Organise / Prescribe / Check
• USE MED LAB MEDLAB MED LAB
• Follow-up appointment to be booked
88. Routine Medications for SCD
Drug Dose: Adult Dose: Child
Penicillin V Not given <1yr: 62.5mg BD
1-3yrs: 125mg BD
>3yrs: 250mg
> 5yrs: not usually given as prophylaxis
Folic acid 5mg or 1mg as available OD <1yr: 1.25mg OD
1-3yrs: 2.5mg OD
>3yrs: 5mg OD
Analgesia 2-week supply for PRN use Paracetamol 1g QDS, Ibuprofen 400mg
QDS
Paracetamol 15mg/kg QDS,
Ibuprofen 5mg/kg TDS
Ferrous sulphate if MCH<25pg 200mg TDS 2mg/kg TDS for 6 weeks, then check Hb level and ferritin.
Mebendazole after every 3 months 200mg
400mg stat dose
1-2years
2years
Malaria prophylaxis Low transmission
Use insecticide treated nets
Prompt diagnosis and treatment
89. Next Genomic sequencing
• Next-generation sequencing (NGS) is a new technology used for DNA
and RNA sequencing and variant/mutation detection.
• NGS can sequence hundreds and thousands of genes or whole
genome in a short period of time.
• The sequence variants/mutations detected by NGS have been widely
used for disease diagnosis, prognosis, therapeutic decision, and
follow up of patients.
• The capacity of its massive parallel sequencing offers new
opportunities for personalized precision medicine.
90. testing platforms
•Space capacity is minimal, test menus
are multi-pex multi-integrated for EID,
Viral load, HIV,, MTB and HPV SVD,
COVID 19 and other infectious
diseases.
•GENOMIC TESTING IN CANCER AND
OTHER NCDs
91. Population genomics
• large-scale application of genomic technologies to study
populations of individuals. For example, population
genomics research can be used to study human ancestry,
migrations and health conditions .
• Population Genomics. Basically, population genomics
allows Medical scientists to understand how groups are
related over time.
• It also allows scientists to understand our ancestral
backgrounds and how that ancestral background has been
shaped over time, in terms of the environment that our
ancestors have lived their lives.
93. Process or algorithms for follow-up Affected
individuals
• : Notification of a family about a (presumptive) serious health
problem in an otherwise healthy appearing newborn is not a trivial
task. Ideally, notification should be done in person or by live phone
call.
• The initial conversation should be expected to raise anxiety, and
cause disappointment and sadness at a time of joy;
• it may also stir anger or denial.
94. cont
• The messenger should be someone familiar with the clinical course of SCD
in infants, young children and adults, and be able to answer basic questions
about SCD and ready to provide reassurance and support for the family.
• The processes and algorithms for notification and follow-up often place the
primary care service as the medical home,
• presuming that staff is able to interpret screening results and take
appropriate actions when a baby with a positive screen is reported.
• Reporting individuals with abnormal screening results to a dedicated
follow-up agency and/or sickle cell treatment center will increase the
timeliness of appropriate care
95.
96. Pharmacological Treatment
• Febrile illness
Children with fever are screened for bacteraemia i.e. complete blood count,
reticulocyte count and blood culture taken
Younger children (varies from center to center) are admitted for intravenous
antibiotics while older children with reassuring white cell counts are managed at
home with oral antibiotics
Children with previous bacteraemic episodes should be admitted
97. Pharmacological Treatment (2)
Painful (vaso-occlusive) crises
Most patients with sickle cell disease have intensely painful episodes called
vasoocclusive crises
Painful crises are treated symptomatically with analgesics; pain management
requires opioid administration at regular intervals until the crisis has settled
98. Pharmacological Treatment (3)
Painful (vaso-occlusive) crises
For milder crises a subgroup of patients are managed by NSAIDs (such as
diclofenac or naproxen)
For more severe crises most patients require inpatient management for
intravenous opioids; patient-controlled analgesia (PCA)
99. Pharmacological Treatment (4)
Painful (vaso-occlusive) crises
Diphenhydramine is effective for the itching associated with the opioid use
NB: analgesia, Oxygen, hydration and warmth
100. Pharmacological Treatment (5)
Acute chest crises
• Management is similar to vaso-occlusive crises with the addition of antibiotics
(usually a quinolone or macrolide)
• When the pulmonary infiltrates worsen or the oxygen requirement increases,
simple blood transfusion or exchange transfusion is indicated
101. Pharmacological Treatment (6)
Acute chest crises
• Exchange transfusion involves the exchange of a significant portion of the patients red cell mass for normal
red cells, which decreases the percent hemoglobin S in the patient's blood
Hydroxyurea
• The first approved drug for the causative treatment of sickle cell anemia
102. Pharmacological Treatment (7)
• Hydroxyurea had previously been used as a chemotherapy agent, and
there is some concern that long-term use may be harmful
• it is likely that the benefits outweigh the risk
Splenic Sequestration Crisis
Treatment includes early intervention and maintenance of hemodynamic
stability using isotonic fluid or blood transfusions.
103. Pharmacological Treatment (8)
• Splenic Sequestration Crisis
• Careful blood transfusions with red blood cells are recommended to treat
both the sequestration and the resultant anemia
Blood transfusion aborts the red blood cell sickling in the spleen and allows
release of the patient’s blood cells that have become sequestered, often
raising the hemoglobin above baseline values
104. Pharmacological Treatment (9)
• Typically recommend only 5 mL/kg of red blood cells because the goal is to prevent
hypovolemia
• Blood transfusion that results in hemoglobin levels above 10 g/dL may put the
patient at risk for hyperviscosity syndrome because of the risk that that patient may
release the blood within the spleen
• If there is recurrent episode Prophylactic splenectomy is the only choice for
preventing future life threating episode.
105. Pharmacological Treatment (10)
Priapism
The optimal treatment for acute priapism is unknown. Acutely, supportive therapy, such as a hot
shower, short aerobic exercise, or pain medication, is commonly used by patients at home
Urology consultation is required to initiate this procedure, with appropriate input from a
hematologist
• A prolonged episode lasting >4 hrs should be treated by aspiration of blood from the corpora
cavernosa followed by irrigation with dilute epinephrine to produce immediate and sustained
detumescence
106. Pharmacological Treatment (12)
• Penicillin prophylaxis
• The most important intervention in the routine management of children with
SCD is penicillin prophylaxis to prevent pneumococcal infection, which
justifies newborn screening
• Penicillin is given twice daily from as 2months of age
107. Pharmacological Treatment (13)
• Children with SCD-SS are given Penicillin VK: 125mg by mouth twice
daily for those under 3yrs of age and 250mg twice daily for those 3
and older
• An alternative to oral penicillin is an injection IM of 1.2 million units
of long acting Bicillin (Penicillin G Benzathine) every 3 weeks
108. Pain Management
Pain is the commonest acute complication in SCD
Mild to Moderate Pain
Use oral analgesics Paracetamol, Ibuprofen or diclofenac
Encourage excessive oral fluids intake
109. latest treatments of sickle cell disease
• According to the National Heart, Lung, and Blood Institute (NHLBI), researchers are studying new
ways to prevent blood cells from sickling, which could help alleviate anemia and reduce the
frequency and severity of pain crises in people living with sickle cell disease
• One such study involves activating a protein in red blood cells called pyruvate kinase (PKR), which
supports cellular health and metabolism. Researchers found that stimulating PKR activity may
prevent or alleviate severe pain crises in people with sickle cell disease
• Another treatment option for sickle cell anemia is bone marrow or stem cell transplantation,
which can cure the disease in some patients
• However, these procedures are risky and are typically reserved for people with severe disease.
Gene therapy is another promising treatment option that may become available in the future 3.
• It’s important to note that treatment for sickle cell anemia is highly individualized and depends on
the severity of the disease and the patient’s overall health. If you or someone you know has sickle
cell anemia, it’s best to consult with a healthcare professional to determine the best course of
treatment.
111. Key Points
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
Sickle cell anemia exists in Lesotho
112. Key Points (2)
Clinical presentation of Sickle cell crises includes the following:
• Vaso-occlusive crisis
• Aplastic Crisis
• Hemolytic Crisis
• Treatment include pharmacological and non-pharmacological interventions
• Follow up monitoring have to be done closely by doing complete assessment and
treatment and counselling of parents
•
113. Population health and NGS
• We see population health as a broad concept encompassing not only
the elimination of diseases and injuries, but also the intersecting and
overlapping factors that influence health.
• These influencing factors include the environment, education,
mobility, policy and governance, poverty, racism, infrastructure,
access to technology, urban planning and many more.
• Together, these issues revolve around three major pillars — human
health, environmental resilience and social and economic equity —
that affect the lives of billions of people around the world.
114. Network
• Governing bodies must work with industry to maximise the
public benefits of precision medicine.
• This vital line of communication accelerates innovation by
ensuring that bold ideas fit within safety standards imposed
by regulatory agencies, before companies set on product
development and testing.
REVIEW learning objectives with participants.
CLARIFY any questions they may have before moving on.
EXPLAIN: Sickle cell anemia is an inherited disorder of hemoglobin associated with abnormally shaped red blood cells, causing problems with the flow of blood through the body and the resulting transport of oxygen throughout the body.
MENTION: Pulmonary hypertension is a risk factor for early death
EXPLAIN to participants other clinical manifestations.
Other clinical manifestations:
CNS
major devastating manifestation is stroke that is normally ischemic but can be hemorrhagic in adults after rupture of aneurysm.
convulsions occurs concurrently with stroke but can occur isolated or with acute chest syndrome.
EYE-Para orbital infarction may lead to ptosis and also retinal vascular manifestation.
PRIAPISM- Sustained, painful and unwanted penile erection in SCD. Recurrent episodes may lead to fibrosis and impotence.
LEG ULCERS- Due to minor injuries especially on the malleoli. And because poor circulation healing is delayed and infections are established.
CHOLELITHIASIS- This is due to rapid hemolysis of RBC causing hyperalbuminaemia that causes formation of bile stones.
RENAL MANIFESTATIONS- Vasooclusion of renal small vessels causes ischemic injury and hence renal failure insues.
VIRAL HEPATITIS- Due to multiple blood transfusion.
NOTE: The results from Point of care rapid tests are reliable and final, unless they are inconclusive
PROVIDE Explanation of each bullet using notes in the Facilitators’ Guide
REFER to desk guide page 51: Sickle cell information
EXPLAIN
Not all of the following need to be done on each attendance. Always check that the child has had everything done on the new-patient list; due to the nature of a new-patient attendance it is not always possible to have achieved everything at that first appointment.
EXPLAIN hydroxyurea, was shown to decrease the number and severity of attacks in a study in 1995 (Charache et al) and shown to increase survival time in a study in 2003
This is achieved by reactivating fetal hemoglobin production in place of the hemoglobin S that causes sickle cell anemia
EXPLAIN
A study in children older than 5 years of age, found no clinical benefit of penicillin prophylaxis compared with placebo, indicating that treatment may be stopped at that age.
For those allergic to penicillin, Erythromycin ethyl succinate (20mg/kg) divided into 2 daily doses can provide adequate prophylaxis.
The importance of prophylactic antibiotics should be emphasized at all visits because parents may become noncompliant with this essential treatment.
Activity: Brainstorming (5 minutes)
ASK participants to brainstorm on the following question:
How do you follow up and monitor a child with sickle cell disease?
ALLOW few participants to respond
WRITE their responses on the flip chart/ board
CLARIFY and SUMMARISE by using the content below