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REGISTRATION AND TREATMENT PROTOCOL FOR THALASSEMIA MAJOR PATIENT


NEW PATIENTS
  1. Every new patient coming to Al-Fajar Foundation should be dealt with as follows.
    1. Filling up of Registration Form A
    2. Filling up of Clinical Evaluation Form B.
    3. Filling up of Laboratory Evaluation Form C.
    4. Treatment given & its entry in to the Treatment Form D.
    5. Genetic counseling explained to the parents & its entry in to the Counseling Form E.
    6. Follow up explained to the parents & filling of follow up forms on subsequent visit.

  2. REGISTRATION
    1. The receptionist should receive every new patient & attendants.
    2. A new file should be opened for every new patient which should contain all the Forms.
    3. Registration Form should be filled by the incharge registration by interviewing the parents.
    4. Patient should then be referred to the Medical Officer for Clinical Evaluation.
    5. After filling the Clinical Evaluation.
    6. After filling the Clinical Evaluation Form lab. Evaluation Form should be filled by performing the following lab. investigations:
      1. CBC
      2. Hb electrophoresis
      3. Serum ferritin level
      4. Liver function tests
      5. Fasting blood glucose level
      6. Growth hormone level
      7. HIV, HCV & HBs Ag.
      8. Blood grouping
      9. ECG.
      10. X.Ray Chest
      11. Urine examination
      12. BLOOD TRANSFUSION
      OBJECTIVES
      1. To keep the Hb within normal range.
      2. Maintain mean Hb at about 12.0 g/dl.
      3. Mean Hb is the average of pre & post transfusion Hb level.
      4. Post transfusion level not to rise above 15.5g/dl.
      WHEN TO START TRANSFUSION
      1. All homozygous beta-thalassemic cases whose Hb level falls below 7.0g/dl & remains so for a week or more.
      2. If patient shows the following, even if the Hb-level is more than 7.0g/dl:
        • Impaired growth
        • Bone changes.
        • Rapidly enlarging spleen.
      3. No transfusion should be given to a clinically well patient & Hb-level of more than 10.0g/dl. However a watch should be kept on the growth & development of the patient & transfusion should be started in case of impaired growth & development.
      TYPE OF BLOOD TO BE GIVEN
      1. Packed red blood cells should be given to all thalassemic patients with minimal white blood cells & plasma.
      2. Leuco poor blood is better if easily available.
      3. Special WBC removing filters should be used.
      QUANTITY OF BLOOD TO BE GIVEN
      1. Use the following formula to calculate the quantity of packed cells to be given. Give 10mls of packed cells for every kilogram of body weight.
      2. The desired Hb level may not be achieved initially; however with a regular transfusion the relationship between the desire and the achieved. Hb level becomes linear and thus achievable.
      PREPARATION OF BLOOD
      1. All common blood group antigens should be tested on the patients. These include:
        • ABO & Rh antigens.
        • • Kell, Duffy & Kidd.
      2. Carry out antibody screen on the patients blood using panel of three red cells.
      3. Carry out a cross match between donor cells and patients plasma using indirect coomb’s test. An auto control should also be set. [patients cells plus patients serum]
      4. Donor blood must be screened for HIV, HCV, HbsAg, syphilis & if possible for malaria.
      RATE OF BLOOD TRANSFUSION
      1. When patient has no cardiac problem.
      2. Administer 10-15mls per/kg of body weight/2hours.
      3. Larger volumes [20mls/kg] may be given at slower rate.
      4. Don’t prolong transfusion longer than 4 hours.
      2. If patient has cardiac problem.
      • Transfusion rate should not be more than 5mls/kg at one time.
      • The rate should not exceed 2mls/kg/hour.
      • Smaller amounts of blood at shorter interval may be given.
      • A diuretic [Lasix 1-2mg/kg] may be given parentally.

      FREQUENCY OF TRANSFUSION……
      1. An interval of 4-6 weeks is quite acceptable.
      2. Patients bad clinical condition require shorter interval.

      IRON CHELATION THERAPY

      INDICATIONS:
      1. It should be started after 10-15 blood transfusions have been given.
      2. Serum ferritin level of more than 10000ug/l.
      3. Serum ferritin level of 2000ug/l in case of Thalasemia intermedia.

      CHOICE OF DRUG FOR IRON CHELATION:
      1. Dessferal injections [Desferroxamine] are the drug of choice.
      2. A number of oral chelating agents are under trial.
      L-1 is one of the oral chelators.
      3. Vitamin C increases Iron Chelation if given along with dessferal.

      DESSFERAL THERAPY:
      1. One vial [500mls] of dessferal s dissolved in 5.0mls of distilled water.
      2. It should be discarded if discoloured, opaque of cloudy.

      ROUTE OF ADMINISTRATION:
      1. Subcutaneous route is the best route.
      2. It should be given in the form of an infusion subcutaneously over a period of 8-10 hours through a special purpose buile pump.
      3. The administration should be for 5 days a week.
      4. Change the site of prick for administration regularly [usually over the abdomen].
      INTRAVENOUS ROUTE:
      1. This route is a used in-patient with cardiac problems due to secondary iron-overload.
      2. Bolus dose may produce unpleasant flushing & so it should be avoided.
      DOSE OF DESSFERAL.
      1. Dose of dessferal is proportional to the extent of iron
      overland [level of serum ferritin]
      2. Dose is as follows:

      LEVEL OF FERRITIN. DOSE OF DESSFERAL.

      < 2000ug/L. 25mg/KG/Day
      20003000ug/L 35mg/KG/Day
      3000 & more ug/L 45mg/Kg/Day
      3. Do not exceed 50mg/KG day unless extensive chelation is required.
      4. 50mg/KG day may be given by continuous I/V infusion in cardiac patients.
      5. Tea drinking may be encouraged as tea inhibits iron absorption.

      ORAL DRUGS FOR IRON CHELATION.

      1. L 1 one of under trial oral chelating agent & has following dosage schedule:
      75mg/KG/day orally.
      2. Side effects of L1 are:
      Agranulocytosis.
      Arthropaty.
      G.I distrubances.
      Zink deficiency.
      3. This drug is still experimental & should only be used in properly planned & executed trials by a properly qualified person.

      VITAMIN C

      1. It increases iron chelation if given with dessferal therapy.
      2. Dose is as follows:
      Children’s under 10 years = 50mg/day orally.
      Children’s over 10 years = 100mg/day orally.
      3. Usually started 01 month after dessferal therapy is started.

      FOLIC ACID.

      1. Only given if patients are under transfusion.
      2. Dose is 5mg tablet orally per day.

      SUPPORTIVE TREATMENT FOR THALASSEMIA MAJOR PATIENTS.

      MANAGEMENT OF INFECTIONS
      1. Most common infections in these patients are:-
      Upper respiratory tract infections.
      Urinary tract infections.

      2. These infections should be treated promptly if present.
      3. Usual drugs used are:-
      Oral ampicillin ……………………..50-100mg/KG/6 hourly.
      Oral amoxil………………………….50-100KG/8 hourly
      Oral augmentin……………………..50-100mg/KG/8hourly
      (commonly we give syp. Augmentin/amoxil 1 tsf-tds for at least 5-7 days along with any anti pyretic, like syp. Paracetamol 1 tsf – sos to treat the fever of infection)
      4. Patients on desferal therapy are frequently suscebtible to yersinia infections, if that happens then stop desferal immediately give combination tharapy of Gentamycin & Cephalosporin.

      CONGESTIVE CARDIAC FAILURE (CCF.) IN THALASSEMIA MAJOR & ITS MANAGEMENT
      1. Usually it is due to cardiac siderosis in Thalassemia.
      2. Severe anemia & increased need for transfusion make patients more susceptible to CCF.
      3. Transfusion rate in these patients should not be more than 2mls/KG/hours.
      4. Inj Lasix 1-2 mg/KG should be given parenterally in these patients.
      5. Continuous I/V infusion of desferal not more than 50mg/KG/day should be given.

       
 
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