{"id":259,"date":"2022-07-07T08:34:01","date_gmt":"2022-07-07T08:34:01","guid":{"rendered":"https:\/\/mayhospitalshendam.com\/?p=259"},"modified":"2022-07-11T07:09:44","modified_gmt":"2022-07-11T07:09:44","slug":"sickle-cell-disease-disorder","status":"publish","type":"post","link":"https:\/\/mayhospitalshendam.com\/?p=259","title":{"rendered":"SICKLE CELL DISEASE\/DISORDER"},"content":{"rendered":"
\"\"

Sickle Cell Anemia Red cells in Blood Stream<\/p><\/div>SICKLE CELL DISEASE\/DISORDER
\nBy Dr B. M. L. Yarima
\nINTRODUCTION
\nSickle cell disease and its variants are genetic disorders resulting from the presence of a mutated form of haemoglobin, haemoglobin S (Hb S).
\nSickle cell disease\/disorder causes significant morbidity and mortality, particularly in people of African and Mediterranean ancestry.
\nMorbidity, frequency of crisis, degree of anaemia, and the organ systems involved vary considerably from individual to individual.<\/p>\n

ETIOPATHOGENESIS
\nSickle cell disease (SCD) is a group of inherited red blood cell disorders. Red blood cells contain haemoglobin, a protein that carries oxygen to tissues for normal cell function. Healthy red blood cells are round , and they move through small blood vessels to carry oxygen to all parts of the body.
\nIn someone who has SCD, the haemoglobin is abnormal, which causes the red blood cells to become hard and sticky and look like a C-sharped farm tool called a \u201csickle.\u201d The sickle cells die early, which causes a constant shortage of red blood cells. Also, when they travel through small blood vessels, they get stuck and clog the blood flow. This can cause pain and other serious complications (health problems) such as infection, acute chest syndrome and stroke.
\nNOTE- SCD is a genetic condition that is present at birth. It is inherited when a child receives two genes- one from each parent- that code for abnormal haemoglobin.<\/p>\n

TYPES OF SCD
\n SCD denotes all genotypes containing at least one sickle gene, in which HbS makes up at least half the haemoglobin present.
\nThere are several types of SCD. The specific type of SCD a person has depends on the genes they inherited from their parents. People with SCD inherit genes that contain instructions, or code, for abnormal haemoglobin.
\nMajor sickle genotypes described so far include the following:
\n\uf0a7\tHbSS disease
\nPeople who have this form of SCD inherit two genes, one from each parent, that code haemoglobin \u201cS\u201d. This is commonly called sickle cell anaemia and is usually the most severe form of the disease.
\n\uf0a7\tHbSC
\nPeople who have this form of SCD inherit a haemoglobin \u201cS\u201d gene from one parent and a gene for a different type of abnormal haemoglobin called \u201cC\u201d from the other parent. This is usually a milder form a SCD.
\n\uf0a7\tHbS beta thalassaemia
\nPeople who have this form of SCD inherit a haemoglobin \u201cS\u201d gene from one parent and a gene for beta thalassaemia, another type of haemoglobin abnormality, from the other parent. There are two types of beta thalassaemia:
\n– beta thalassaemia \u201czero\u201d (HbS beta 0)
\n – beta thalassaemia \u201cplus\u201d (Hb beta +)
\nThose with HbS beta 0 thalassaemia usually have a severe form of SCD clinically indistintiguishable from SCA. People with HbS beta +-thalassaemia tend to have a milder form of SCD. <\/p>\n

\uf0a7\tHbS\/hereditary persistence of fetal haemoglobin- very mild or asymptomatic phenotype.
\n\uf0a7\tHbS\/HbE syndrome- very rare with a phenotype similar to HbS\/b+ thalassaemia
\n\uf0a7\tRare combinations of HbS with other abnormal haemoglobinopathies such as HbD Los Angeles, G-Philadelphia, HbO Arab, and others.
\n\uf0a7\tSickle cell trait (SCT) or carrier state, HbAS
\nPeople who have a sickle cell trait inherit a haemoglobin \u201cS\u201d from one parent and a normal gene (one that codes for haemoglobin \u201cA\u201d) from the other parent. People with SCT usually do not have any signs of the disease. However, in rare cases, a person with SCT may develop health problems; this occurs most often when there are other stresses on the body, such as when a person becomes dehydrated or exercises strenuously. Additionally, people who have SCT can pass the abnormal haemoglobin \u201cS\u201d gene on to their children.<\/p>\n

SIGNS AND SYMPTOMS
\n Screening for HbS at birth is currently mandatory in the United States. For the first 6 months of life, infants are protected largely by elevated levels of foetal haemoglobin (Hb F). Sickle cell disease (SCD) usually manifests early in childhood, in the following ways:
\n\uf075\tAcute and chronic pain: The most common clinical manifestation of SCD is vaso-occlusive crisis; pain crises are the most distinguishing clinical feature of SCD
\n\uf075\tBone pain: Often seen in long bones of extremities, primarily, due to bone marrow infarction.
\n\uf075\tAnaemia: Usually present, chronic, and haemolytic in nature.
\n\uf075\tAplastic crisis: serious complication due to infection with parvovirus B19 (B19V).
\n\uf075\tSplenic sequestion: Characterized by the onset of life-threatening anaemia with rapid enlargement of the spleen and high reticulocyte count.
\n\uf075\tInfection: Organisms, that pose the greatest danger include encapsulated respiratory bacteria, particularly Streptococcus pneumoniae; adult infections are predominantly with gram-negative
\n organisms, especially Salmonella.
\n\uf075\tGrowth retardation, delayed sexual maturation, being underweight.
\n\uf075\tHand-foot-syndrome: This is a dactylitis presenting as bilateral painful and swollen hands and\/or feet in children.
\n\uf075\tAcute chest syndrome: Young children present with chest pain, fever, cough, tachypnoea, leukocytosis, and pulmonary infiltrates in the upper lobes; adults are usually afebrile, adults are usually afebrile, dyspnoiec with severe chest pain, with multilobar\/lower lobe disease.
\n\uf075\tPulmonary hypertension: increasingly recognized as a serious complication of SCD.
\n\uf075\tAvascular necrosis of the femoral or humeral head: Due to vascular occlusion
\n\uf075\tCentral nervous system (CNS) involvement: Most severe manifestation is stroke
\n\uf075\tOpthalmic involvement: Ptosis, retinal vascular changes, proliferative retinitis
\n\uf075\tCardiac involvement: dilatation of both ventricles and the left atrium
\n\uf075\tGastrointestinal involvement: cholecystitis is common in children; liver may become involved
\n\uf075\tGenitourinary involvement: kidneys lose concentration capacity; priapism is a well-recognized complication of SCD.
\n\uf075\tDermatologic involvement: leg ulcers are a chronic painful problem.
\nAppropriately half the individuals with homozygous Hb S disease experience vaso-occlusive crises. The frequency of crises is extremely variable. Some individuals have as many as 6 or more episodes annually, whereas others may have episodes only at great intervals or none at all. Each individual typically has a consistent pattern for crisis frequency.
\nTriggers of vaso-occlusive crises include the following:
\n – hypoxaemia\/ hypoxia: may be due to acute chest syndrome or respiratory complications, or flying in unpressurized aircraft.
\n – dehydration: especially from exertion or warm weather or vomiting and diarrhoea
\n – Infection
\n – Cold weather due to vasospasm
\n–\tEmotional stress
\n–\tPregnancy <\/p>\n

DIAGNOSIS
\nSCD is suggested by the typical clinical picture of chronic haemolytic anaemia and vaso-occlusive crisis.
\nLaboratory tests used in patients with SCD include the following:
\n\uf0a7\tHaemoglobin electrophoresis- electrophoresis confirms the diagnosis with the presence of homozygous HbS and can also document other haemoglobinothies (e.g. HbSC, HbS-beta+ thalassaemia)
\n\uf0a7\tComplete Blood Count
\n\uf0a7\tSerum electrolytes
\n\uf0a7\tPeripheral blood smear
\n\uf075\tRenal function (creatinine, BUN, urinalysis)
\n\uf075\tBlood culture
\nImaging Studies:
\no\tRadiography- CXRs should be performed in patients with respiratory symptoms. Pelvic x-ray- for Avascular necrosis of mainly femoral head.
\no\tAbdominal USS- to measure or assess spleen and liver sizes. It may be used to rule out cholecystitis or cholelithiasis.
\no\tCT scanning: may demonstrate subtle regions of osteonecrosis not apparent on plain radiographs in patients who are unable to have MRI.
\no\tEchocardiography: Identifies patients with portal hypertension
\no\tTranscranial doppler ultrasonography: can identify children with SCD at risk for stroke. <\/p>\n

TREATMENT
\nThe goals of treatment in SCD are symptom control and management of disease complications. Treatment strategies include the following goals:
\n1)\tManagement of vaso-occlusive crisis
\n2)\tManagement of chronic pain syndromes
\n3)\tManagement of chronic haemolytic anaemia
\n4)\tPrevention and treatment of infections
\n5)\tManagement of the complications and the various organ damage syndromes associated with the disease
\n 6) Prevention of stroke
\n 7) Detection and treatment of pulmonary hypertension.
\nPharmacotherapy:
\nSCD may be treated with the following medications:
\n\uf075\tOpioid analgesics (eg, oxycodone\/aspirin, methadone, morphine sulfate, oxycodone\/acetaminophen, fentanyl, codeine, acetaminophen\/codeine)
\n\uf075\tNonsteroidal analgesics (eg, ibuprofen, ketorolac, acetaminophen, aspirin)
\n\uf075\tAntibiotics (eg, cefuroxime, amoxicillin\/clavulanate, penicillin, ceftriazone, azithromycin, cefaclor)
\n\uf075\tAntimetabolites: Hydroxyurea
\n\uf075\tVitamins (eg folic acid)
\n\uf075\tTricyclic antidepressants (eg amitriptyline)
\n\uf075\tVaccines (eg, pneumococcal, meningococcal, influenza, and recommended scheduled childhood\/adult vaccinations)
\n\uf075\tOthers: haemoglobin oxygen-affinity modulators (eg, voxelotor), P-selectin inhibitors (eg, crizanlizumab), etc.
\nOther approaches to managing SCD include the following:
\n\uf0a7\tTransfusions: For severe anaemia due to acute splenic sequestration, parvovirus, or hyperhaemolytic crises
\n\uf0a7\tWound debridement
\n\uf0a7\tPhysical therapy
\n\uf0a7\tHeat and cold application
\n\uf0a7\tStem cell tranplantation: Can be curative
\nCombination pharmacotherapy and non-pharmacotherapy
\n\uf075\tVigorous hydration (plus analgesics): For vaso-occlusive crisis
\n\uf075\tOxygen, antibiotics, analgesics, transfusion, and bronchodilators: For treatment of acute chest syndrome.<\/p>\n

PREVENTION
\n\uf075\tPre-marital screening and counselling
\n\uf075\tPre-conception screening and counselling
\n\uf075\tIdentifying and avoiding triggers
\n – dehydration: ensure adequate hydration
\n – fever: antipyretics, prompt treatment of cause
\nREFERENCES
\n\uf075\tMedscape
\n\uf075\tCdc.gov\/ncbddd\/sicklecelldisease<\/p>\n","protected":false},"excerpt":{"rendered":"

SICKLE CELL DISEASE\/DISORDER By Dr B. M. L. Yarima INTRODUCTION Sickle cell disease and its variants…<\/p>\n","protected":false},"author":2,"featured_media":0,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"nf_dc_page":"","_monsterinsights_skip_tracking":false,"_monsterinsights_sitenote_active":false,"_monsterinsights_sitenote_note":"","_monsterinsights_sitenote_category":0,"_jetpack_memberships_contains_paid_content":false,"footnotes":""},"categories":[38],"tags":[51,50],"class_list":["post-259","post","type-post","status-publish","format-standard","hentry","category-journal","tag-haemoglobin-s-hb-s","tag-sickle-cell-disease-and-its-variants-are-genetic-disorders-resulting-from-the-presence-of-a-mutated-form-of-haemoglobin"],"yoast_head":"\nSICKLE CELL DISEASE\/DISORDER - May Hospital, Shendam<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/mayhospitalshendam.com\/?p=259\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"SICKLE CELL DISEASE\/DISORDER - May Hospital, Shendam\" \/>\n<meta property=\"og:description\" content=\"SICKLE CELL DISEASE\/DISORDER By Dr B. 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