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Sickle Cell Anemia Sickle Cell Anemia Picture, ` - Coggle Diagram

- - - - If Hemoglobin S becomes deoxygenated the molecule will undergo polymerization become crystallized. The crystallization causes a separation of long polymers that leads to the deformation of the erythrocytes in dense sickle shapes.
        
        Due to the dense sickle shape, erythrocytes become inflexible which then leads to their premature destruction.
        
        As there is a decreased amount of erythrocytes, the affected's hemoglobin levels are also decreased thus resulting in anemia.
    - - Sickle celled erythrocyte shapes injure the blood vessel walls resulting in activation of endothelial cells. Blood vessel injuries lead to inflammation which leads to endothelial adhesion.
        
        Endothelial Cells interaction w/ RBCs
        
        RBCs adhere directly to activated endothelial cells, platelets, and WBCs
        
        Endothelial Cells interaction w/ WBCs
        
        WBCs adhere to inflamed endothelium of the blood vessel wall and RBCs which leads to further inflammation
        
        Multicellular adhesion reduces and blocks blood flow to organs and thus leads to vaso-occlusion of blood vessels.
        
        Endothelial Cells interaction w/Platelets
        
        Platelet adhesion is activated by sickle cell disease which leads to platelet bondage to WBCs, endothelial cells, and RBCs
- - - - Greece
      - Turkey
      - Italy
  - - - 50% Chance of a Normal Child
      - 50% Chance of Child Being Born with Sickle Cell Trait
    - - 25% Chance of Child Being Born with Sickle Cell Trait
      - 50% Chance of Child Being Born with Sickle Cell Trait
      - 25% Chance of Child Being Born with Sickle Cell Disease
    - - 100% Chance of Child Being Born with Sickle Cell Disease
    - - 100% Chance of Child Being Born with Sickle Cell Trait
- - - - Administration
        
        Do Not Cut, Crush, or Chew
        
        Take Once a Day at the Same Time
        
        Take PO With or Without Food
        
        Store at around 70 Degrees Farenheit
      - Therapeutic Advantages
        
        Reduces Pain Crises
        
        Might Reduce Blood Transfusion Needs
      - Adverse Effects
        
        Increase Risk of Infections
        
        Increased Risk of Developing Skin Cancer
        
        Constipation or Diarrhea
        
        Mouth Sores
        
        Low Blood Cell Counts
        
        Loss of Apetite
        
        Nausea and Vomiting
      - Dosage
        
        Tablet
        
        Starting: 20 mg per kg per day
        
        Max: 35 mg per kg per day
        
        Dosage Increase Determined by Physician
        
        Capsule
        
        Starting: 15 mg per kg per day
        
        Max: 35 mg per kg per day
        
        Dosage Increase Determined by Physician
    - - Therapeutic Advantages
        
        Reduces Pain Crises
        
        Increases the proportion of nicotinamide adenine dinucleotides in sickle cell erythrocytes which reduces oxidative stress
      - Adminstration
        
        Mix Powder with a Liquid or Soft Wet Food
        
        Taken PO Twice a Day at the Same Times
        
        Store at around 70 Degrees Farenheit
      - Dosage
        
        Recommended: 5 - 15 grams per dose mixed with 8 ox of cold or room temperature beverage or 4 - 6 oz of food before ingestion
        
        Dosage Based on Body Weight
      - Adverse Effects
        
        Constipation
        
        Nausea
        
        Headache
        
        Abdominal Pain
        
        Cough
        
        Back, Leg, Feet, Arm, or Hand Pain
    - - Administration
        
        Delivered by Intravenous Infusion
        
        Average 30 Minutes for Infusion
        
        Delivered Once Every 2 - 4 Weeks
        
        Delivered in a Medical Setting
        
        Stored at 36 - 46 Degrees Farenheit
        
        Do Not Freeze/Shake
      - Dosage
        
        Adult (18 Years or Older)
        
        Initial: 5 mg/kg via IV infusion every 2 weeks for 2 doses
        
        Maintenance: 5 mg/kg via IV infusion every 4 weeks
        
        Pediatrics (16 Years or Older)
        
        Initial: 5 mg/kg via IV infusion every 2 weeks for 2 doses
        
        Maintenance: 5 mg/kg via IV infusion every 4 weeks
      - Therapeutic Advantages
        
        Reduces Vaso-Occlusive Crises
        
        Inhibits the production of the P-selectin glycoprotein preventing the adhesion molecule process that leads to blood flow occlusion
      - Adverse Effect
        
        Dizziness
        
        Nausea
        
        Lethargy
        
        Itchiness
        
        Chills
        
        Fever/Sweats
        
        Wheezing/SOB
        
        Joint Pain
    - - Adverse Effects
        
        Generalized Rash
        
        Uticaria
        
        Mild Shortness of Breath
        
        Mild Facial Swelling
        
        Eosinophilia
      - Administration
        
        Take PO Once a Day
        
        Take With or Without Food
        
        Do Not Cut, Crush, or Chew
        
        Store Below 86 Degrees Farenheit
      - Therapeutic Advantages
        
        Increases Affinity Between Hemoglobin and Oxygen
        
        Inhibits Sickling of Red Blood Cells
      - Dosage
        
        1500 mg Oral Dose
        
        1000 mg Oral Dose if Severe Hepatic Impairment
- - - - Assessing stroke risk at the age of two using ultrasound technology to determine blood flow continuity