ArtsAutosBooksBusinessEducationEntertainmentFamilyFashionFoodGamesGenderHealthHolidaysHomeHubPagesPersonal FinancePetsPoliticsReligionSportsTechnologyTravel

More On Treatment And Prophylaxis Of Hemophilia A And The Christmas Disease (Hemophilia B)

Updated on February 5, 2014

Treating Hemophilia

Source

A General Overview

Freeze dried AHG is available from several laboratories. Each vial contains 250 to 1000 units. It is more concentrated and purified and it remains potent for 18 months. In the ordinary case, the initial dose should be 250- 1000 units depending on the severity of the bleeding. Larger initial doses are more effective. A formula has been used to calculate the requirement of factor VIII concentrates.

K (rise in factor VIII concentration in the patient’s plasma produced for each unit/Kg of infused material) = Patient’s weight (Kg) X desired rise in factor VIII (%)/Units of factor VIII in the dose administered.

Factor VIII inhibitors may develop as a result of repeated administration of AHG. These neutralize factor VIII:C and render administered factor VIII concentrates ineffective. In such subjects, AHG made from porcine plasma is effective and nonantigenic. At present, prothrombin complex concentrates (PCC) are available. These have factor VIII bypassing activity and they may be effective even in those having antibodies to factor VIII:C. Their use is still controversial. Recent reports suggest the possible role of repeated administration of AHG concentrates in conferring acquired immunodeficiency state (AIDS) in hemophilics. Cryoprecipitate is devoid of this risk. If factor VIII concentrates are not available, fresh frozen plasma is the next best alternative. Fresh blood should be used to replace the lost blood.

Antifibrinolytic drugs like epsilon-aminocaproic acid (EACA) given in doses of 24g daily or tranexamic acid 10 mg have been used along with AHG to prevent dissolution of the clots formed as a result of therapy. Mild cases of hemophilia respond to 1-deamino-8-D- arginine vasopressin (DDAVP) and this form of therapy is gaining acceptance. Intravenous administration of DDAVP in a dose of 0.3 ug/Kg slowly in patients with factor VIII:C levels of 10% or more is advised. If the factor VIII:C level is lower, DDAVP is not useful.

Hemophilia Foot

Source

Manifestations Of Hemophilia B

Source

Prophylaxis

All hemophilics should carry identification cards showing their blood groups to enable emergency treatment without delay. They should be made aware of the nature of the disease and its management. Children should attend ordinary schools but should avoid games and physical training. Special care should be taken to avoid all trauma. Intramuscular injections and antiplatelet drugs should be acoided in all hemophilics. Dental care should be instituted to avoid dental caries and the need for extractions.

Several blood transfusion centers have adopted home treatment programmes. The patient or his near relative is instructed to identify bleeding episodes and self-administer AHG early. This prevents further bleeding and joint deformity.

Prenatal diagnosis of hemophilia is possible by estimating factor VIII:C in fetal blood obtained by fetoscopy. Affected pregnancy can be aborted. Carrier females can be detected by VIII:C assay. Such females show only half, the VIII:C activity expected for the level of VIII.R.AG.

Occasionally, a non-hemophilic subject may acquire antibodies against factor VIII:C as in systemic lupus erythematosus. Such a subject develops bleeding tendency as a hemophilic.

Christmas Disease (Hemophilia B)

This also is an X-liked recessive disorder transmitted by females and manifested in males. The genetic defect is located in the X-chromosome and it is different from that of hemophilia A. The manifestations resemble those of mild hemophilia. This disease accounts for 20-25% of the inherited coagulation defects in India. Factor IX is absent or functionally defective. Diagnosis can be made by factor IX assay. Thromboplastin generation test reveals a defect in patient’s serum. Treatment is to give fresh frozen plasma once in 24 hours.

© 2014 Funom Theophilus Makama

Comments

    0 of 8192 characters used
    Post Comment

    No comments yet.

    working

    This website uses cookies

    As a user in the EEA, your approval is needed on a few things. To provide a better website experience, hubpages.com uses cookies (and other similar technologies) and may collect, process, and share personal data. Please choose which areas of our service you consent to our doing so.

    For more information on managing or withdrawing consents and how we handle data, visit our Privacy Policy at: "https://hubpages.com/privacy-policy#gdpr"

    Show Details
    Necessary
    HubPages Device IDThis is used to identify particular browsers or devices when the access the service, and is used for security reasons.
    LoginThis is necessary to sign in to the HubPages Service.
    Google RecaptchaThis is used to prevent bots and spam. (Privacy Policy)
    AkismetThis is used to detect comment spam. (Privacy Policy)
    HubPages Google AnalyticsThis is used to provide data on traffic to our website, all personally identifyable data is anonymized. (Privacy Policy)
    HubPages Traffic PixelThis is used to collect data on traffic to articles and other pages on our site. Unless you are signed in to a HubPages account, all personally identifiable information is anonymized.
    Amazon Web ServicesThis is a cloud services platform that we used to host our service. (Privacy Policy)
    CloudflareThis is a cloud CDN service that we use to efficiently deliver files required for our service to operate such as javascript, cascading style sheets, images, and videos. (Privacy Policy)
    Google Hosted LibrariesJavascript software libraries such as jQuery are loaded at endpoints on the googleapis.com or gstatic.com domains, for performance and efficiency reasons. (Privacy Policy)
    Features
    Google Custom SearchThis is feature allows you to search the site. (Privacy Policy)
    Google MapsSome articles have Google Maps embedded in them. (Privacy Policy)
    Google ChartsThis is used to display charts and graphs on articles and the author center. (Privacy Policy)
    Google AdSense Host APIThis service allows you to sign up for or associate a Google AdSense account with HubPages, so that you can earn money from ads on your articles. No data is shared unless you engage with this feature. (Privacy Policy)
    Google YouTubeSome articles have YouTube videos embedded in them. (Privacy Policy)
    VimeoSome articles have Vimeo videos embedded in them. (Privacy Policy)
    PaypalThis is used for a registered author who enrolls in the HubPages Earnings program and requests to be paid via PayPal. No data is shared with Paypal unless you engage with this feature. (Privacy Policy)
    Facebook LoginYou can use this to streamline signing up for, or signing in to your Hubpages account. No data is shared with Facebook unless you engage with this feature. (Privacy Policy)
    MavenThis supports the Maven widget and search functionality. (Privacy Policy)
    Marketing
    Google AdSenseThis is an ad network. (Privacy Policy)
    Google DoubleClickGoogle provides ad serving technology and runs an ad network. (Privacy Policy)
    Index ExchangeThis is an ad network. (Privacy Policy)
    SovrnThis is an ad network. (Privacy Policy)
    Facebook AdsThis is an ad network. (Privacy Policy)
    Amazon Unified Ad MarketplaceThis is an ad network. (Privacy Policy)
    AppNexusThis is an ad network. (Privacy Policy)
    OpenxThis is an ad network. (Privacy Policy)
    Rubicon ProjectThis is an ad network. (Privacy Policy)
    TripleLiftThis is an ad network. (Privacy Policy)
    Say MediaWe partner with Say Media to deliver ad campaigns on our sites. (Privacy Policy)
    Remarketing PixelsWe may use remarketing pixels from advertising networks such as Google AdWords, Bing Ads, and Facebook in order to advertise the HubPages Service to people that have visited our sites.
    Conversion Tracking PixelsWe may use conversion tracking pixels from advertising networks such as Google AdWords, Bing Ads, and Facebook in order to identify when an advertisement has successfully resulted in the desired action, such as signing up for the HubPages Service or publishing an article on the HubPages Service.
    Statistics
    Author Google AnalyticsThis is used to provide traffic data and reports to the authors of articles on the HubPages Service. (Privacy Policy)
    ComscoreComScore is a media measurement and analytics company providing marketing data and analytics to enterprises, media and advertising agencies, and publishers. Non-consent will result in ComScore only processing obfuscated personal data. (Privacy Policy)
    Amazon Tracking PixelSome articles display amazon products as part of the Amazon Affiliate program, this pixel provides traffic statistics for those products (Privacy Policy)