Good treatment, started early in life, can prevent complications. Sickle cell anemia, or homozygous sickle cell disease, results from the inheritance of a sickle cell gene from both parents. In the uk, sickle cell disease is found amongst the african and caribbean population nhs, 2016. Sickle cell disease and sickle cell anaemia sickle cell disease scd is a serious, inherited condition affecting the blood and various organs in the body. Index terms sickle cell anaemia, sickle cell disease, hospital admission, therapy, pain management i. Overview of the clinical manifestations of sickle cell disease. Sickle cell disease and anesthesia anesthesiology asa.
Introduction pathogenesis presentation complications diagnosis differential diagnoses investigations managementtreatment conclusion 2242015 2. This treatment is very safe when given by medical specialists experienced in caring for patients with scd. Median life expectancy is currently 4060 years in high income countries but much less in low income areas. Management of sickle cell disease in the community the bmj. If anaemia is particularly severe or persistent, treatment with blood transfusions or hydroxycarbamide may be necessary. Sickle cell diseasegenetics, pathophysiology, clinical. Patients with the disease often experience acute or chronic pain. Sickle cell anemia symptoms and causes mayo clinic. Anaemia caused by sickle cell disease is not the same as the more common iron deficiency anaemia. Ebeshi 2 1 department of medicinal chemistry and quality control, national institute for pharmaceutical research and development niprd, pmb 21, garki, idu industrial area, abuja, nigeria. A sickle cell day hospital approach, which features staffing by experienced sickle cell providers, is increasingly used and results in improved pain management, better patient satisfaction, and decreased rates of hospitalization for both adults and children with sca benjamin et al.
The term scd includes sickle cell anaemia hbss and the heterozygous conditions of haemoglobin s and other clinically. Sickle cell anemia is a disease passed down through families in which red blood cells form an abnormal sickle or crescent shape. This publication, which was developed by physicians, nurses, psychologists, and social workers who specialize in the care of children and adults with sickle cell disease, describes the current approach to counseling and also to management of many of the medical complications of sickle cell disease. In populations of african ethnic origin, sicklecell. Allogeneic stem cell transplantation is the only curative therapy available for sickle cell disease and has been used with some success 19, 20. Introduction ickle cell disease scd is one of the top leading diseases affecting the black and ethnic minority population. Traditional herbal management of sickle cell anemia. Sickle cell disease scd and its variants are genetic disorders resulting from the presence of a mutated form of hemoglobin, hemoglobin s hbsref1 see the image below.
Purpose and scope the purpose of this guideline is to describe the management of pregnant women with sickle cell disease scd. The pain varies in intensity and can last for a few hours to a few weeks. This leads to a rigid, sicklelike shape under certain circumstances. Management of sickle cell disease in pregnancy rcog. Scd results from any combination of the sickle cell gene with any other abnormal.
Understanding the complications of sickle cell disease. The most common type is known as sickle cell anaemia sca. Attitudes toward management of sickle cell disease and its complications. It results in an abnormality in the oxygencarrying protein haemoglobin found in red blood cells. Sicklecell anaemia is a common genetic condition due to a haemoglobin disorder inheritance of mutant haemoglobin genes from both parents. Normally, rbcs are shaped like discs, which gives them the flexibility to travel through even. These guidelines are intended for use by clinicians providing care to children with sickle cell disease scd who present to the emergency department, to ensure that these children are expediently and correctly. Hence those who are heterozygous for the sickle cell gene will have a selective advantage in regions where malaria is hyperendemic. Pain management guidelines for sickle cell disease sickle. Red blood cells carry oxygen to the body and are normally shaped like a disc. Sickle cell anemia sca is a disease that is caused by the formation of an abnormal hemoglobin type, which can bind with other abnormal hemoglobin molecules within the red blood cells rbcs to. Problems in sickle cell disease typically begin around 5 to 6. Valine can fit into the hydrophobic pocket of another haemoglobin molecule, causing the haemoglobin to polymerise within the red cell, thus forming long stiff fibres of haemoglobin tetramers. It will include preconceptual screening and antenatal, intrapartum and postnatal management.
Over the years, this program and others like the cooperative study of sickle cell disease csscd, established in 1979, has funded research that has elucidated much of what we know about the disease today 4. Pdf sickle cell disease scd predominates in subsaharan africa, east mediterranean areas, middle east, and india. Sickle cell disease is a term used for a group of conditions in which the pathology is due to the presence of hemoglobin s. Read more about these practice guidelines pdf icon external icon. In most countries where sicklecell anaemia is a major public health concern, its management has remained inadequate, national control. This student paper was written as an assignment in the. Complications and treatments of sickle cell disease cdc. In most countries where sickle cell anaemia is a major public health concern, its management has remained inadequate, national control programmes do not exist, the basic facilities to manage the patients are usually absent, systematic screening is not a common practice and the diagnosis is usually.
These protocols are guidelines in use at the sickle cell center at grady health system, and they are intended for use by heath care providers treating patients with sickle cell syndromes. Nov 08, 2012 traditional herbal management of sickle cell anemia. This fact may explain why the sickle cell gene remains common in these areas in spite of the elimination of genes in patients dying of sickle cell anaemia. Periodic episodes of pain, called pain crises, are a major symptom of sickle cell anemia. The management of sickle cell disease national institutes of health national heart, lung, and blood institute division of blood diseases and resources nih publication no. It is one of the most common inherited conditions, although its prevalence varies widely.
Management of sickle cell disease in pregnancy this is the first edition of this guideline. Treatments might include medications and blood transfusions. In sickle cell anaemia, valine replaces glutamic acid at the sixth amino acid of the beta globin chain, as a result of a recessive single gene mutation. A national survey of academic family physicians external icon mainous ag 3rd, tanner rj, harle ca, baker r, shokar nk, hulihan mm.
The patient with sickle cell anemia usually has the following laboratory results. This result from single point replacement of glutamine by valine at position 6 of. The majority of treatments for sickle cell prevent or manage. The aim of treatment for patients with sickle cell disease is symptom control and the prevention or management of complications. The sickle cell membranes display abnormal charge topography, most probably caused by increased lipid peroxidation sickle reticulocytes show abnormaly high expression of antigen cd36 and integrin. In between episodes of sickling, people with scd are normally well. Ppt sickle cell disease powerpoint presentation free to. Sickle cell anaemia is a homozygous form of hbshbss. Sickle cell patient 36 year old black male diagnosed with sickle cell anemia at age 2 formerly had 1 painful crisis each year, but recently has had 3 4 per year last october, acute chest syndrome. Expert panel report epr, 2014 is to synthesize the available scientific evidence on sickle cell disease and offer guidance to busy primary care clinicians. Acute nursing care and management of patients with sickle cell.
Aug 16, 2002 evidencebased management of sickle cell disease. If you or your child has sickle cell anemia, your doctor might suggest additional tests to check for possible complications of the disease. Expert panel report, 2014 ix foreword the purpose of the evidence based management of sickle cell disease. Pdf sickle cell disease scd is a genetic disorder characterised by anaemia and sickling of red blood cells, leading to chronic haemolytic anaemia. Pdf management of sickle cell disease from childhood through. As described elsewhere 14, 16, 17, among the efik and ibibio, hausa, igbo, idoma, and yoruba.
Rods placed in both arms and legs show video treatment. Sickle cell disease and sickle cell anaemia st georges hospital. Pdf antioxidants in the management of sickle cell anaemia. Summary of the 2014 nhlbi guidelines to manage sickle cell. Sickle cell disease scd is a group of blood disorders typically inherited from a persons parents. Uptodate, rose, bd ed, uptodate, wellesley, ma, 2007. In this seminar, we mostly discuss sicklecell anaemia, because there is little evidence for the management of other types of sicklecell disease. Sep 24, 2017 sickle cell anemia is an inherited form of hemolytic anemia sickle cell anemia is a severe hemolytic anemia that results from inheritance of the sickle hemoglobin gene the sickle hemoglobin hbs gene is inherited in people of african descent and to a lesser extent in people from the middle east, the mediterranean area, and the aboriginal tribes in india.
The identification of affected infants by neonatal screening programs allows early initiation of prophylactic penicillin and pneumococcal immunizations, which help prevent overwhelming sepsis. The most common form of scd found in north america is homozygous hbs disease hbss, an autosomal recessive disorder first described by herrick in 1910. It affects the red blood cells, causing episodes of sickling, which produce episodes of pain and other symptoms. Pain develops when sickle shaped red blood cells block blood flow through tiny blood vessels to your chest, abdomen and joints. For some children and teenagers, a stem cell transplant might cure the disease. The most common types include sickle cell anemia hb ss, the sickle betathalassemias hb s. Sickle cell disease scd is a monogenetic disorder due to a. Sickle cell anemia diagnosis and treatment mayo clinic. Sickle cell disease scd and its variants are genetic disorders resulting from the presence of a mutated form of hemoglobin, hemoglobin s hbs see the image below. Sickle cell anemia is an inherited disease that is caused by an abnormal version of hemoglobin, known as hemoglobin s or sickle hemoglobin. Sicklecell anemia is caused by a point mutation at the sixth. Sickle cell disease scd and its variants are genetic disorders resulting from the presence of a mutated form of hemoglobin, hemoglobin s.
Management of sickle cell anemia is usually aimed at avoiding pain episodes, relieving symptoms and preventing complications. Traditional herbal approaches to sickle cell anemia in nigeria. Do not take iron supplements to treat it without seeking medical advice, as they could be dangerous. Sickle cell c disease tends to have a milder phenotypic expression than the homozygous scd because of the ameliorating presence of hemoglobin c. Sickle cell anemia, or sickle cell disease scd, is a genetic disease of the red blood cells rbcs. Patients with sickle cell disease can be afflicted with a multitude of acute and. Proper management of sickle cell anemia sca begins with establishing the correct diagnosis early in life, ideally during the newborn period. If anaemia is particularly severe or persistent, treatment with blood transfusions or. So, early diagnosis and specialist treatment are advised for scd. However, the side effects of taking hydroxyurea during. If you inherit the sickle cell gene from only one parent, you will have sickle cell trait. World health organization executive board eb11734 117th session 22 december 2005 provisional agenda item 4.
834 829 276 1386 219 341 1000 1275 1474 1199 570 231 1396 719 1265 471 5 53 556 993 34 831 964 1510 898 1005 1485 363 948 877 1095 579 617 688 308 735 500