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Sickle Cell Anemia PowerPoint Presentation

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Description : This ppt covers latest uptodate guidelines on diagnosis, management , presentation and clinical findings as well as sickle cell crisis of SCA.

Tags : sickle cell anemia, sickle cell crisis,blood cell,blood

Published on : Jul 22, 2023 | Views : 2836 | Downloads : 6

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Overview: Most common group of inherited red blood cell disorders Change in hemoglobin: called hemoglobin S do not bend or move easily and can block blood flow lead to serious problems, including Stroke, eye problems, infections, and episodes of pain called pain crises. both parents must have either sickle cell disease (two sickle cell genes) or sickle cell trait (one sickle cell gene) In short, If you have sickle cell disease, you will pass one sickle cell gene to your children.
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Types
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HbSS (Called Sickle cell disease): - most severe anemia HbSC: milder anemia, S from one parent, C from another HbS-Beta thalassemia: inherit a hemoglobin “S” gene from one parent and a gene for beta thalassemia Few rare types: HbSD, HbSE, and HbSO SCD is an autosomal recessive trait. For the disease to manifest, there must be homozygosity for Hb S or compound heterozygosity SCD is clinically heterogeneous. Individuals with identical Hb genotypes can have different frequencies of vaso-occlusive pain, variable rates of hemolysis, and different spectra of chronic complications. These differences are due partly to the modulating effects of modifier genes.
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Sickle Cell Trait:
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8 percent of African-Americans carry only one defective gene and typically live normal lives extreme conditions such as severe dehydration and high-intensity physical activity can lead to serious health issues, including sudden death
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Risk Factors:
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ethnic groups: African-Americans Hispanic-Americans Middle Eastern, Asian, Indian, and Mediterranean Conditions that increase polymerisation ? What are those
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pathogenesis: Hb S forms insoluble polymers when deoxygenated. Increased 2,3-BPG favors Hb S polymerization in three ways: ●It lowers oxygen affinity, increasing deoxy Hb S. ●It reduces intracellular pH, increasing deoxy Hb S. ●It alters the conformation of deoxy Hb S. At concentrations above the solubility of deoxy Hb S that cause polymerization, the Hb-oxygen binding curve is progressively right shifted
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Polymerization is enhanced by the following Deoxygenation Higher Hb S concentration cellular dehydration sickle-beta+ thalassemia Acidosis ph below 7.4 Reducing polymerization: 1. Direct inhibition - increasing Hb-oxygen affinity (decreasing deoxy-Hb S) with medications like voxelotor Increased Hb F 2. Indirect inhibition- reduce the mean corpuscular hemoglobin concentration (MCHC): concomitant alpha thalassemia. Hydrating the sickle RBC using agents that inhibit cation and water loss.
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Effects: Hemolysis:- Extravascular > Intravascular best biomarker for hemolysis is RBC lifespan. The percentage of reticulocytes and the Hb F level correlate strongly with the RBC lifespan in contrast, the Hb and hematocrit are not correlated RBC lifespan Intravascular: LDH is the marker, urine hemglobin, haptoglobin decrease Extravascular: liver/spleen/bone marrow- Phagocytosis by macrophage-> excrete, High bilirubin 2. Vascular occlusion 3. Hypercoagulable state: due to increase inflammatory cytokines, activation of coagulation factors, platelet activation
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Signs and Symptoms: symptoms can begin by four months of age, early diagnosis is critical All newborns in the United States are now tested for the disease
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1st: Acute painful Episode (prev called Crisis): Acute painful episodes- not all patients are in true crisis, and pain should not be allowed to progress to the point of crisis for patients to receive appropriate analgesia including opioid analgesics if indicated pain can co-occur with (and mask) other potentially life-threatening complications of SCD Acute pain should be assessed rapidly so as not to delay analgesia
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Patient (or family) states that the pain is atypical for their vaso-occlusive pain, in severity or location. Abnormal vital signs. Major changes from the baseline complete blood count (CBC) or reticulocyte count.,Leukopenia or leukocytosis may indicate infection, including acute chest syndrome, Worsening anemia may indicate bone marrow suppression (eg, from infection), hepatic or splenic sequestration of blood, Increased hemoglobin concentration may indicate severe dehydration, Decreased reticulocyte count from baseline, which usually would be elevated, may suggest bone marrow suppression. Markedly elevated reticulocyte count may suggest increased hemolysis or hepatic or splenic sequestration Pain associated with or acute cholecystitis. abdominal distention, which may indicate hepatic or splenic sequestration, Jaundice, which may indicate increased hemolysis or hepatobiliary disease. Hematuria, which may indicate renal pathology. New laboratory abnormalities such as increasing creatinine, liver function tests, lactate dehydrogenase Complications of SCD - other than vaso-occlusive pain:
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Management: no combination of clinical and laboratory findings that can be used to determine that an individual is or is not in pain gold standard for the assessment of pain is the patient’s report absence of hemolysis or the stability of the hemoglobin level cannot be used to justify withholding or underdosing of pain medication Management of acute pain is individualized. treating SCD pain similarly to cancer pain
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In ED: Options for opioid therapy include intravenous morphine, hydromorphone, intranasal fentanyl- max 2 doses, Ketamine for acute pain unresponsive to opioids, mitigate opioid tolerance, intranasally at 0.25 mg/kg to a maximum of 1 mg/kg or infusion of ketamine at approximately 0.3 mg/kg/hour (PRN) pain medication should not be used as the sole source of pain relief, because such a practice will ensure that the individual will have subtherapeutic pain medication levels resulting in unnecessary pain. When using PCA, where the majority of the opioid dose is given by a continuous intravenous route and demand dosing is provided every 20 minutes at a dose equivalent to one-sixth of the hourly infusion dose. The demand dose is used for breakthrough pain and is not intended to substitute for a continuous infusion. Treatment for breakthrough pain – It is critical to make additional treatment available for breakthrough pain. The best approach is to provide immediate access to pain medication without requiring the individual to call the nurse.. This is most easily achieved using PCA with a demand-dose option. Individuals receiving hydroxyurea prior to hospitalization should continue it at their regular dose unless there is a hydroxyurea-induced cytopenia, For individuals not receiving hydroxyurea at the time of hospitalization, the possibility of starting hydroxyurea to reduce future pain episodes is generally discussed on an outpatient basis For treatment of hypovolemia, we generally give an initial intravenous bolus of 0.5 to 1 L of normal saline.Thereafter, we use a maintenance rate of combined intravenous and oral fluid that contains at least one-quarter normal saline (roughly 2 L/m2 per day), targeted to a net even or slightly positive daily fluid balance Hypernatremia should be avoided because it induces red blood cell (RBC) dehydration and sickling VTE prophylaxis — All adults with SCD admitted to the hospital
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Therapies to avoid or use sparingly Never use placebo Avoid ice and cold compresses (may precipitate sickling) Avoid meperidine Avoid ketorolac, NSAIDS generally ineffective, however can use in childrens Use anxiolytics only if indicated for anxiety, not for pain Use oxygen only for hypoxia, not for routine pain Use transfusion only for indicated complications, not for uncomplicated vaso-occlusive pain. . If pain and a complication requiring RBC transfusion are present, pain control and transfusion should be administered concurrently. No benefit of Antiplatelet, Glucocorticoids, Magnesium
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Taper opioids: Do not taper opioids in the first 24 hours unless there is respiratory depression or increased lethargy Taper opioids during the day when appropriate assessment of pain can be performed Taper 10 to 20 percent of the opioid dose at a time Taper opioids by decreasing the dose rather than by increasing the interval between doses Convert to oral pain medication when the intravenous dose is roughly equivalent to home doses Pain Prevention: Hydroxyurea:all ages including infants oral pill/liquid, once daily, :improve life expectancy, reduce complications :cause hair loss, GI symptoms, avoid prior to conception both males and females L- Glutamine: 5 yr old, BD oral : improve pain episodes, can combine with hydroxyurea Voxelotor: 4 yr old, OD oral : reduce pain episode, increase Hb, improve fatigue, can combine with hydroxyurea Crizanlizumab: IV every 4 weeks, good choice for severe pain and frequent episodes, can be combined Opioids Antidepressants: Duloxetine Discharge indication:
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Chronic transfusion may beneficial if not improve pain by analgesics and hydroxyurea. For 6 months Simple transfusions and phlebotomy may be used to maintain the hemoglobin S below 50 percent. If this is ineffective or associated with significant iron overload, red blood cell exchange transfusion may be substituted Refractory Pain:
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Management at home: Non-opioid therapy is used for mild pain Ice and cold compresses should be avoided pain severity increases, opioids of escalating strengths are used, if severe: use continues opiod therapy Individuals who require long-acting opioids on a regular basis should have additional short-acting medication for breakthrough pain such as morphine, oxycodone, or hydromorphone (Dilaudid)
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2nd complication: Infection
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Functional hyposplenism develops in early childhood around 4 months risk of serious infection with encapsulated organisms Viral infections may also be more virulent: Parvo, influenza, covid Common organisms: Bacteremia: Encapsulated organisms, especially Streptococcus pneumoniae and Haemophilus influenzae Meningitis: Encapsulated organisms, especially S. pneumoniae. H. influenzae Pneumonia: Mycoplasma pneumoniae, Chlamydia pneumoniae, Respiratory virus preventive care — routine use of prophylactic penicillin and vaccination against pneumococci and H. influenzae Prophylactic penicillin V — Beginning by two months of age and continuing until at least age five years Age 2 months to <3 years – 125 mg orally twice daily Age ≥3 years – 250 mg orally twice a daily Folic acid — Folic acid (400 micrograms to 1 mg daily) is often given to children How early and from what?
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3rd complication: Anemia Chronic compensated hemolytic anemiafollowing findings [63,67]: Hemoglobin level approximately 8 to 10 g/dL Hematocrit approximately 20 to 30 percent Reticulocytosis approximately 3 to 15 percent Sickled cells Howell Jolly bodies (nuclear remnants that have not been phagocytosed, due to reduced splenic function) Sickle cell HJ Body Nucleated RBC Other: Increase LDH, low haptoglobin, Pigmented gallstones anemia and markers of hemolysis may be less severe in some individuals, including those with concomitant alpha thalassemia, those undergoing chronic transfusion therapy, and those receiving hydroxyurea Fetal hemoglobin (Hb F) is mildly to moderately elevated (typical range, 1 to 4 percent), especially in individuals receiving hydroxyurea
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Routine Mgt of Anemia: Folic acid Monitor patient baseline: decline in the setting of suspected aplastic, splenic sequestration, or hyperhemolytic crises Evaluation of Iron def: S. ferritin < 25 Aplastic Crisis: Acute rapid and life-threatening drop in hemoglobin level caused by chronic hemolysis, transient arrest in erythropoiesis Retic <1%, absolute retic count <10,000 infections by Streptococcus pneumoniae, Salmonella, Children: Parvovirus Reappearance- 2-14 days Rx: Transfusion Hyperhemolysis: sudden exacerbation of hemolysis worsening anemia despite ongoing reticulocyte production Immune role: complement/macrophage mediated Rx: transfusion with compatible blood + IVIG 0.4g/kg 5d + IV methylpred 500mg/d 2d
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4th: Splenic Sequestration Crisis: life-threatening complication of SCD characterized by an acute drop in hemoglobin level, typically two g/dL below baseline. RBCs are captured and pool within the spleen present with a rapidly enlarging spleen and a marked decrease in hemoglobin level despite persistent reticulocytosis Absolute reticulocyte count (ARC) ≥300,000 splenectomy is often used after the first acute event to prevent recurrence not recommend regular blood transfusion therapy as a strategy to decrease splenic sequestration
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5th: Stroke/TIA Ischemic stroke is more common in children, hemorrhagic stroke is more common in adults 14-fold increased risk of overt stroke Primary prevention to reduce the risk of a first stroke is based on the use of regular transcranial Doppler measurements and selective screening with magnetic resonance angiography. Extracranial internal carotid artery (eICA) stenosis is relatively common and requires close monitoring At-risk children are treated with chronic prophylactic transfusion+ hydroxyurea Individuals who have had a stroke are treated with chronic prophylactic transfusions to prevent recurrent stroke
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Feeling Sick-le in the Chest
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6th: Pulmonary: Acute Chest Syndrome: syndrome of fever, chest pain, hypoxemia, wheezing, cough, or respiratory distress in the setting of a new pulmonary infiltrate Pain precede ACS episode ACS suspicion should be present from day of admission Occur in 50% Patients, m/c cause of hospitalization and mortality cause is often multifactorial and includes infection, vaso-occlusion, hypoventilation, atelectasis, thrombosis or thromboembolism Can progress rapidly to Multi-organ failure: only predictor is decline in platelet counts compared at presentation
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Management: Pain control Ketorolac as non-sedating analgesic used in childrens, not adults O2- baseline SpO2 >95% keep strictly!, NC, Bipapap, CPAP <96% SpO2 ass with stroke/ vaso-occlusive episodes Intubation, bronchoscopy to clear secretions, ECMO if refractory Bronchodilators use Incentive spirometry Antibiotics: MAcrolide+3rd gen Cephalo/Moxiflx Transfusion: MAINSTAY Rx in all: can be simple or exchange Fluids: typical regimen is 1.5 times maintenance fluids of D5 in one-half normal saline for the first 24 to 48 hours Maintain Euvolumia, if overload: Rx Furesemide VTE prophylaxis of more than 18 years, not in younger Glucocorticods: Not used! Use only if asthma, or rapidly progressive ACS Inhaled NO- no benefit
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On Discharge: Minimize future ACS risk Prevent infection by Penicillin upto 5 years, and vaccination Asthma monitoring and suspected nocturnal hypoxemia if SpO2 <93%- consider polysomnography Reduce vasocclusion by Disease modifying drugs: like hydroxyurea Chronic transfusion for 6 months if hydroxyurea is not effective HSCT in patient with multiple life threatening ACS Periodic spiromerty
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7th: Skeletal- Dactylitis and AVN Dactylitis: Dactylitis is vaso-occlusive pain in the small bones of the hands and feet occurs in infants and children with SCD up to approximately four years of age: as marrow replace by fat diagnosis is generally made by history and physical examination, as radiography typically does not show any changes repeated episodes of dactylitis will lead to a mottled appearance However, virtually any bony structure containing red (erythropoietic) marrow can be affected Challenging to d/d Osteomyelitis: vague bone pain, inability to ambulate, soft tissue edema, skin erythema, and point tenderness to palpation Osteonecrosis (avascular necrosis): Patho: bone ischemia due to occlusion of blood vessel-> microfractures, collapse of cancellous bone, and finally collapsed articular surface Femur head, humerus head and distal tibia unique feature of AVN in SCD is involvement of the entire epiphysis; in most other conditions associated with AVN, only weight-bearing areas are affected
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C/F of AVN: AVN often affects more than one joint in the same individual back, buttock, groin, or knee pain present with chronic pain or recurrent pain in the same area that occurs over the course of weeks to months decreased range of motion and pain associated with ambulation decreased ability to abduct the hip relative to the contralateral Dx: MRI in all with SCD + intermittent/chronic hip pain or unexplained and recurrent pain Rx: - Pain control - Rx concomitant osteomyelitis if on MRI or septic arthritis - hydration, crutch weight bearing - Core decompression: remove necrotic bone and put bone graft - Joint replacement if conservative donot help - Regular blood transfusion not recommended
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Special Thank you to Dr. Rupali Sangrampurkar for the opportunity! Any Questions or Anything to add?