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Hemophilia

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This document provides information about hemophilia, including: - Hemophilia is an inherited bleeding disorder caused by deficiencies in specific clotting factors like VIII or IX. - There are three main types - A, B, and C - determined by which clotting factor is deficient. - It is passed from parents to children via genes on the X chromosome. - Symptoms include bruising and bleeding, especially into joints or muscles. Treatment aims to prevent bleeding complications through blood transfusions or replacement of the deficient clotting factor.

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HEMOPHILI A PRESENTED BY MR. SAURABH SHARMA NURSING TUTOR SSIN BHAVNAGAR
DEFINITION Hemophilia is an inherited bleeding disorder. Children with hemophilia cant stop bleeding because they dont have enough clotting factor in their blood. Clotting factors are needed for blood to clot. Blood clots to prevent excessive bleeding. There are many blood clotting factors involved in the forming of clots to stop bleeding. There are 2 common factors that affect blood clotting. These are factor VIII and factor IX.
TYPES OF HEMOPHILIA The 3 main forms of hemophilia include: Hemophilia A. This is caused by a lack of the blood clotting factor VIII. About 9 out of 10 people with hemophilia have type A disease. This is also referred to as classic hemophilia or factor VIII deficiency. Hemophilia B. This is caused by a deficiency of factor IX. This is also called Christmas disease or factor IX deficiency. Hemophilia C. Some doctors use this term to refer to a lack of clotting factor XI.
CAUSES Hemophilia types A and B are inherited diseases. They are passed on from parents to children through a gene on the X chromosome. Females have 2 X chromosomes, while males have 1 X and 1 Y chromosome. A female carrier has the hemophilia gene on 1 of her X chromosomes. When a hemophilia carrier female is pregnant, there is a 50/50 chance that the hemophilia gene will be passed on to the baby. If the gene is passed on to a son, he will have the disease. If the gene is passed on to a daughter, she will be a carrier. If the father has hemophilia but the mother does not carry the hemophilia gene, then none of the sons will have hemophilia disease, but all of the daughters will be carriers. In about 1/3 of the children with hemophilia, there is no family history of the disorder. In these cases, its believed that the disorder could be related to a new gene flaw.
Hemophilia
CLINICAL MENIFESTSATION Symptoms may include: Bruising Bleeding in the brain Bleeding into the muscles Bleeding into a joint Easy bleeding Other bleeding
DIAGNOSTIC TEST Complete blood count (CBC) Bleeding times Genetic or DNA testing Clotting factors
TREATMENT Treatment will depend on symptoms, age, and general health. It will also depend on how severe the condition is. Treatment for hemophilia is aimed at preventing bleeding complications (mainly head and joint bleeds). Treatment may include: Bleeding in the joint may need surgery or immobilization. Your child may need rehab of the affected joint. This may include physical therapy and exercise to strengthen the muscles around the area. Blood transfusions may be needed if major blood loss has occurred. This is when your child gets donated blood. Self-infused factor VIII or IX can allow a child with hemophilia to lead a near normal lifestyle.
COMPLICATIONS Complications of hemophilia can include: Bleeding in the joints or muscles Inflammation of the joint lining Long-term joint problems Very serious tumor-like enlargements, of the muscle and bone Development of antibodies against clotting factors Infections from transfusions
CARE OF A HEMMOPHILIC CHILD Managing your child's hemophilia may include: Letting your child take part in activities and exercise, but not ones that may cause injury. These include soccer, rugby, wrestling, motocross, and skiing. Preventing dental and gum problems with good dental hygiene. Getting vaccines under the skin instead of in the muscle to prevent bleeding in the muscle. Not giving your child aspirin or other nonsteroidal anti-inflammatory drugs (NSAIDs). Wearing medical identification (ID) in the case of an emergency.
Key points about hemophilia in children Hemophilia is an inherited bleeding disorder. It causes an affected child to have low levels of blood clotting factors. The most common symptom of hemophilia is increased, uncontrollable bleeding. Giving factor VIII or IX can allow a child with hemophilia to lead a near normal lifestyle.
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Hemophilia

  • 1. HEMOPHILI A PRESENTED BY MR. SAURABH SHARMA NURSING TUTOR SSIN BHAVNAGAR
  • 2. DEFINITION Hemophilia is an inherited bleeding disorder. Children with hemophilia cant stop bleeding because they dont have enough clotting factor in their blood. Clotting factors are needed for blood to clot. Blood clots to prevent excessive bleeding. There are many blood clotting factors involved in the forming of clots to stop bleeding. There are 2 common factors that affect blood clotting. These are factor VIII and factor IX.
  • 3. TYPES OF HEMOPHILIA The 3 main forms of hemophilia include: Hemophilia A. This is caused by a lack of the blood clotting factor VIII. About 9 out of 10 people with hemophilia have type A disease. This is also referred to as classic hemophilia or factor VIII deficiency. Hemophilia B. This is caused by a deficiency of factor IX. This is also called Christmas disease or factor IX deficiency. Hemophilia C. Some doctors use this term to refer to a lack of clotting factor XI.
  • 4. CAUSES Hemophilia types A and B are inherited diseases. They are passed on from parents to children through a gene on the X chromosome. Females have 2 X chromosomes, while males have 1 X and 1 Y chromosome. A female carrier has the hemophilia gene on 1 of her X chromosomes. When a hemophilia carrier female is pregnant, there is a 50/50 chance that the hemophilia gene will be passed on to the baby. If the gene is passed on to a son, he will have the disease. If the gene is passed on to a daughter, she will be a carrier. If the father has hemophilia but the mother does not carry the hemophilia gene, then none of the sons will have hemophilia disease, but all of the daughters will be carriers. In about 1/3 of the children with hemophilia, there is no family history of the disorder. In these cases, its believed that the disorder could be related to a new gene flaw.
  • 6. CLINICAL MENIFESTSATION Symptoms may include: Bruising Bleeding in the brain Bleeding into the muscles Bleeding into a joint Easy bleeding Other bleeding
  • 7. DIAGNOSTIC TEST Complete blood count (CBC) Bleeding times Genetic or DNA testing Clotting factors
  • 8. TREATMENT Treatment will depend on symptoms, age, and general health. It will also depend on how severe the condition is. Treatment for hemophilia is aimed at preventing bleeding complications (mainly head and joint bleeds). Treatment may include: Bleeding in the joint may need surgery or immobilization. Your child may need rehab of the affected joint. This may include physical therapy and exercise to strengthen the muscles around the area. Blood transfusions may be needed if major blood loss has occurred. This is when your child gets donated blood. Self-infused factor VIII or IX can allow a child with hemophilia to lead a near normal lifestyle.
  • 9. COMPLICATIONS Complications of hemophilia can include: Bleeding in the joints or muscles Inflammation of the joint lining Long-term joint problems Very serious tumor-like enlargements, of the muscle and bone Development of antibodies against clotting factors Infections from transfusions
  • 10. CARE OF A HEMMOPHILIC CHILD Managing your child's hemophilia may include: Letting your child take part in activities and exercise, but not ones that may cause injury. These include soccer, rugby, wrestling, motocross, and skiing. Preventing dental and gum problems with good dental hygiene. Getting vaccines under the skin instead of in the muscle to prevent bleeding in the muscle. Not giving your child aspirin or other nonsteroidal anti-inflammatory drugs (NSAIDs). Wearing medical identification (ID) in the case of an emergency.
  • 11. Key points about hemophilia in children Hemophilia is an inherited bleeding disorder. It causes an affected child to have low levels of blood clotting factors. The most common symptom of hemophilia is increased, uncontrollable bleeding. Giving factor VIII or IX can allow a child with hemophilia to lead a near normal lifestyle.