Case Study - Hemophilia Final [PDF]

Zitiervorschau

Republic of the Philippines Department of Education Region IV-A CALABARZON

Tomas Claudio Colleges Taghangin Morong, Rizal

Hemophilia Case Study

Cielo Louise R. Dominguez Charles Andrew V. Sacramento Kate A. Boca Ryan I. Abdullah Kalvin James L. Dinampo Karylle Charmie S. Sumido Hannah Grace SJ. Yim Kim Eliz Q. Ulic-Ulic James Vincent P. Matus

Description Majority of the people will just ignore having a wound that causes a spontaneous bleeding. Some will just easily clean it with soap and water, alcohol and betadine or cover it with bandages, not knowing that those simple cuts and wounds are just a cover of a very serious condition that may cause death. Do you ever experience nose bleeding with an unknown cause? Do you ever had a big bruise even if no one beat you up? Have you ever experienced vomiting, double vision sight and supreme sleepiness after bumping your head on a hard object? If you answer yes on those cite questions, then you must continue reading this study which tackles about hemophilia. Hemophilia or haemophilia is a very frightening bleeding problem. People with hemophilia do not bleed any faster than normal people do but they can bleed for a long period of time. Their blood does not have enough clotting factor or it doesn’t have enough supply of protein in blood that controls bleeding because of some defective factors (World Federation of Hemophilia). Having a defective factor means that the body of person has no means to repair itself when there is damage in the blood vessel or injured tissue that can lead to excessive bleeding and internal hemorrhaging. This disorder was hereditary or can be pass to the other generations and mostly males are the one who is being diagnosed with this type of disease.

Hemophilia can be classified into two types; Hemophilia A and Hemophilia B. Hemophilia A or Classic Hemophilia is caused by a lack or decrease of clotting factor VIII while Hemophilia B or Christmas Disease is caused by a lack or decrease of clotting factor IX (CDC and Prevention). Hemophilia B with 1 out of 25,000 causes of male birth is much rarer than Hemophilia A with an approximate number of 1-5,000 causes of male birth. A CDC study conducted in six states in 1994 estimated that about 17,000 people had hemophilia at that time. Currently, the number of people with hemophilia in the United States is estimated to be about 20,000, based on expected births and deaths since 1994. Here in the Philippines, the World Hemophilia Federation estimates a ten thousand (10,000) hemophilia cases which quite low but still, a law was built to create a full country awareness for this disorder. Senate Bill 1335 authored by Sen. Joel Villanueva and coauthored by Sen. Nancy Binay, and Senate Bill 1389 authored by Sen. JV Ejercito both entitled “Bleeding Disorders Standard of Care Bill,” also seeks to give free treatment for persons with bleeding disorders such as hemophilia, von Willebrand Disease and other inherited bleeding disorders. It is stated on Senate Bill 1335 Section 2. Declaration of Policy that- - It is hereby declared the policy of the State: “ (1) To ensure the adequate treatment of hemophilia at the lowest possible 10 cost and endeavor to make them available for free to indigent patients; 11 (2) To ensure the establishment of treatment centers in public hospitals; and (3) To establish a standard of care so that patients with severe bleeding disorders can receive necessary and appropriate medical care. “

This Senate Bill promotes a battle between the Filipinos and hemophilia (and/or other bleeding disorders). They had given so much privileges for those who was diagnosed with this disorder and it also give rights for medical care and expenses. Overall, continuous bleeding and pains that you are feeling was not just a simple or natural effect of having a clot. So before treating yourself with so much medicines and

ways try to consult your reliable doctor or have a background check of your family history of hemophilia.

Diagnostic Most cases that are diagnosed hemophilic (a person with hemophilia) are gained from the genes of their parents or ancestors (hereditary or inborn). About two-third of person with this disease have a positive track of the same illness on their afore generations. Any family history of bleeding, such as following surgery or injury, or unexplained deaths among brothers, sisters, or other male relatives such as maternal uncles, grandfathers, or cousins should be discussed with a doctor to see if hemophilia was a cause. A doctor often will get a thorough family history to find out if a bleeding disorder exists in the family.

Many people who have or have had family members with hemophilia will ask that their baby boys get tested soon after birth. In the best of cases, testing for hemophilia is planned before the baby’s delivery so that a sample of blood can be drawn from the umbilical cord (which connects the mother and baby before birth) immediately after birth and tested to determine the level of the clotting factors. Umbilical cord blood testing is better at finding low levels of factor VIII (8) than it is at finding low levels of factor IX (9). This is because factor IX (9) levels take more time to develop and are not at a normal level until a baby is at least 6 months of age. Therefore, a mildly low level of factor IX (9)

at birth does not necessarily mean that the baby has hemophilia B. A repeat test when the baby is older might be needed in some cases. But still, humans without a family track of hemophilia must not ignore their bleeding because one-third of those hemophilic are also acquired or the symptoms and illness will occur while growing up.

Second type is the Clotting factor tests, also called factor assays, which required to diagnose a bleeding disorder. This blood test shows the type of hemophilia and the severity. It is important to know the type and severity in order to create the best treatment plan. Hemophilia is a serious disease that need to be prevented even in the first month of the baby. Diagnosed your kids and treat them now by engaging your babies with this test. Doctors prepared to give test for the babies if the blood are clotting properly. There are two test which are; screening test and blood clotting test. Screening tests are blood tests that show if the blood is clotting properly. The first test is also divided into four types which are; Prothrombin Time (PT) Test, Fibrinogen Test, Activated Partial Thromboplastin Time (APTT) Test and Complete Blood Count (CBC).

Complete Blood Count (CBC) is a common test which measures the amount of hemoglobin (the red pigment inside red blood cells that carries oxygen), the size and number of red blood cells and numbers of different types of white blood cells and platelets found in blood. The CBC is normal in people with hemophilia. However, if a person with hemophilia has unusually heavy bleeding or bleeds for a long time, the hemoglobin and

the red blood cell count can be low. Activated Partial Thromboplastin Time (APTT) Test is a test that measures how long it takes for blood to clot. It measures the clotting ability of factors VIII (8), IX (9), XI (11), and XII (12). If any of these clotting factors are too low, it takes longer than normal for the blood to clot. The results of this test will show a longer clotting time among people with hemophilia A or B. Prothrombin Time (PT) Test ehich also measures the time it takes for blood to clot. It measures primarily the clotting ability of factors I (1), II (2), V (5), VII (7), and X (10). If any of these factors are too low, it takes longer than normal for the blood to clot. The results of this test will be normal among most people with hemophilia A and B. Lastly, Fibrinogen Test that also helps the doctors assess a patient’s ability to form a blood clot. This test is ordered either along with other blood clotting tests or when a patient has an abnormal PT External or APTT test External result, or both. Fibrinogen is another name for clotting factor I (CDC and Prevention).

Assessment Severe hemophilia is when the factor VIII or IX is less than 1 percent. Bleeding can occur in these children, even with the minimal activities of daily life, and bleeding may also occur from no known injury. Bleeding most often occurs in the joints and in the head. Other symptoms of hemophilia include: Bruising. Bruises can occur from small accidents, which can result in a large hematoma (a collection of blood under the skin causing swelling). For this reason, most diagnoses occur around 12 to 18 months of age, when the child is becoming more active. Bleeds easily. A tendency to bleed from

the nose, mouth, and gums with minor trauma, teeth brushing, and/or dental work is often an indication of hemophilia. Bleeding into a joint. Hemarthrosis (bleeding into a joint) can cause pain, immobility, and eventually deformity if not medically managed properly. This is the most common site of complications due to hemophilia bleeding. These joint bleeds can lead to chronic, painful, arthritis, deformity, and crippling with repeated occurrences. Bleeding into the muscles. Bleeding into the muscles can cause swelling, pain, and redness. Swelling from excessive blood in these areas can cause increased pressure on tissues and nerves in the area, resulting in permanent damage and/or deformity. Bleeding in the brain from injury or spontaneously. Bleeding from injury, or spontaneously in the brain, is the most common cause of death in children with hemophilia and the most serious bleeding complication. A brain hemorrhage can occur from even a small bump on the head or a fall. Small bleeds in the brain can result in blindness, retardation, a variety of neurological deficits, and can lead to death if not recognized and treated immediately. Other sources of bleeding. Blood found in the urine or stool may also be a symptom of hemophilia. The symptoms of hemophilia may resemble other blood disorders or medical problems. Always consult your child's doctor for a diagnosis. Treatment

Treatments for hemophilia Specific treatment for hemophilia will be determined by your child's doctor based on: Your child's age, overall health, and medical history Extent of the disease Your child's tolerance for specific medications, procedures, or therapies Expectations for the course of the disease Your opinion or preference Treatment depends on the type and severity of the hemophilia. Treatment for

hemophilia is aimed at prevention of bleeding complications (primarily head and joint bleeds). Treatment may include: Immunizations may need to be given under the skin (subcutaneous) instead of in the muscle (intramuscular) to prevent deep muscle bleeds. Joint hemorrhages may require surgery and/or immobilization. Rehabilitation of the affected joint may include physical therapy and exercise to strengthen the muscles around the area. Before surgery, including dental work, your child's doctor may recommend factor replacement infusions to increase the child's clotting levels prior to the procedures. Your child may also receive the specific factor replacement infusions during and after the procedure to maintain the clotting factor levels and to improve healing and prevention of bleeding after the procedure. Your child's doctor may also recommend the discontinuation of aspirin, and aspirin-containing products, since these products have been linked to bleeding problems. Blood transfusions may be necessary if significant blood loss has occurred. Proper dental hygiene is a preventive measure. Prophylactic self-infusion of factor VIII or IX can allow a child with hemophilia to lead a near normal lifestyle. The use of gene therapy to replace the defective genes that cause hemophilia is under study, and clinical trials are being conducted, according to the National Heart, Lung, and Blood Institute.