4. Haemophilia And Royal Families

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Haemophilia is a genetic bleeding disorder that affects the blood's ability to clot. It was introduced to European royal families through Queen Victoria of England, who had one son and two daughters that were carriers. This spread haemophilia to the royal families of Russia, Germany, and Spain through marriages and descendants. The disease weakened royal rule and damaged public perceptions of affected royal families.

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Haemophilia and the royal families of Europe

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Hemophilia is a common hereditary coagulation disorder due to deficiency or reduce activity of clotting factor VIII or clotting factor IX. This disorder is a X- linked recessive disorder. Types: Hemophilia A- deficiency of clotting factors VIII Hemophilia B- deficiency of clotting factors IX Hemophilia C- deficiency of clotting factors XI Parahaemophilia- deficiency of clotting factor V Causes of hemophilia Hemophilia has a sex-linked recessive inheritance. In most cases Hemophilia caused by a mutation in a gene that encodes for one of the clotting factors . Since the hemophilia gene is located on the X chromosome, Hemophilia usually occurs in males, and Female is the carrier of hemophilia. Diagnosis Complete blood cell count Coagulation studies FVIII assay Normal values for FVIII assays are 50-150%. Values in hemophilia are as follows: Mild: >5% Moderate: 1-5% Severe: <1% Treatment of Hemophilia Other Types of Treatment Desmopressin (DDAVP) Antifibrinolytic Medicines Vaccinations- hepatitis A and B. Gene Therapy Gene Therapy New Drugs for Hemophilia treatment New Drugs for Hemophilia treatment Bangladesh perspectives Bangladesh would have 10800 hemophiliacs. But, initially the patients does not concern about hemophilia. Patients are usually diagnosed only after bleeding episode and sometimes the episode are causes serious consequences. Conclusion Primary diagnosis with the success of gene therapy and availability of the new bioengineered products the prospect of the hemophiliacs will be brighter in near future.

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