THALASSEMIA.powerpoint, BLOOD DISORDER.pptx

THALASSEMIA, BLOOD DISORDER, A FAMILY GENETIC DISEASES.

1. THALASSEMIA BY: L eahnina Lopez, RN Pediatric Center

2. Introduction Kuwait Records 475 Cases Of Thalassemia KUWAIT CITY : The chairperson of Thalassemia Association affiliated to National Bank Hospital’s Pediatric Consultancy and Medical Society Maha Burseli said Thalassemia is among the most dangerous blood diseases, reports Al- Shahed daily . She stressed patients suffering from the disease have abnormal blood cells which prevent the rate of iron in the blood to survive beyond 22 days, and there is need for change of blood on regular basis or else it will be impossible to replace the blood.

3. She declared Kuwait has 475 cases of Thalassemia and it costs KD 20,000 to treat each patient in a year. She also said only KD 7 can prevent the disease through pre-marital medical test. She noted the best treatment for the disease is bone marrow transplant but the procedure is complicated and expensive, and it costs about KD 100,000, besides severe pain the patients undergo before and after the process.

4. objectives AT THE END OF MY LECTURE, WE WILL BE ABLE TO: DEFINE THALASSEMIA DESCRIBE SIGNS AND SYMPTOMS OF DIFFERENT TYPES OF THALASSEMIA KNOW OUR NURSING and MEDICAL MANAGEMENT

5. DEFINITION Thalassemia is a blood disorder passed down through families (inherited) in which the body makes an abnormal form of hemoglobin. Hemoglobin is the protein in red blood cells that carries oxygen. The disorder results in large numbers of red blood cells being destroyed, which leads to anemia. Hemoglobin is made of two proteins: Alpha globin and beta globin. Thalassemia occurs when there is a defect in a gene that helps control production of one of these proteins.

6. RISK FACTORS Thalassemia occurs when there is an abnormality or mutation in one of the genes involved in hemoglobin production. This genetic defect is inherited from your parents. If only one of your parents is a carrier for thalassemia, you may develop a form of the disease called “thalassemia minor.” If this occurs, you will probably not have symptoms, but you will be a carrier of the disease. Some people with thalassemia minor do develop minor symptoms. With both of your parents are carriers of thalassemia, you have a 25 percent chance of inheriting a more serious form of the disease. Thalassemia is most common in people from Southeast and Central Asia, the Mediterranean, the Middle East, India, and North Africa.aa

7. Signs and symptoms Thalassemia minor Thalassemia minor usually does not cause any symptoms. If it does, it causes only minor anemia. Beta thalassemia (Cooley’s anemia ) fussiness paleness frequent infections poor appetite failure to thrive jaundice (yellowing of the skin and whites of the eyes) enlarged organs This form of thalassemia is usually so severe that it requires regular blood transfusions.

8. Alpha thalassemia Alpha thalassemia also has two serious types : Hemoglobin H disease can cause bone issues. The cheeks, forehead, and jaw may all overgrow. In addition to the bone issues and the anemia associated with any kind of thalassemia, hemoglobin H disease can cause these other symptoms: jaundice extremely enlarged spleen malnourishment

9. Hydrops fetalis extremely severe form of thalassemia. It occurs before birth, and most individuals with this condition are either stillborn or die shortly after being born. Other symptoms can include: Bone deformities in the face Fatigue Growth failure Shortness of breath Yellow skin (jaundice) Persons with the minor form of alpha and beta thalassemia have small red blood cells but no symptoms.

10. MEDICAL MANAGEMENT General treatment: blood transfusions bone marrow transplant medications and supplements possible surgery to remove the spleen and/or gallbladder You may be instructed not to take vitamins or supplements containing iron. This is especially true if you require blood transfusions. People who receive blood transfusions receive extra iron that the body can’t easily get rid of and iron can accumulate in tissues, which can be potentially fatal.

11. C helation therapy R eceiving an injection of a chemical that binds with iron (and other heavy metals). This helps remove extra iron from your body.

12. NURSING CARE PLAN Activity intolerance related to imbalance of oxygen supply and consumption needs . Changes in tissue perfusion associated with reduced cellular components that are essential to deliver pure oxygen to the cells . Changes in nutrition less than body requirements related to lack of appetite.

13. NURSING MANAGEMENT Improve tissue oxygenation through monitoring of signs of hypoxia, cyanosis, hyperventilation, increased pulse rate, breath frequency and blood pressure . Provide frequent rest periods to reduce oxygen consumption. Monitor blood products, transfusion reactions like fever, chills, cardiac dysrhythmias, nausea, vomiting, chest pain, headache, back pain and or any signs of shock or renal failure. Monitor for signs of excess fluid circulation such as dyspnea, increased respiratory rate, cyanosis, chest pain, and dry cough. Minimize or eliminate pain .

14. Prevent infection. Assess signs of infection, fever , malaise, soft tissue and lymph nodes inflamed/swollen. Monitor for signs of complication : Vascular collapse and shock, splenomegaly, infarction of bones and joints, leg ulcers, stroke, blindness, chest pain, delayed growth and development. Provide age-appropriate explanations to children about procedures and hospitalizations. Give support to children and families. Encourage family members and family members screened.

15. CONCLUSION Severe thalassemia can cause early death (between ages 20 and 30) due to heart failure. Getting regular blood transfusions and therapy to remove iron from the body helps improve the outcome. Less severe forms of thalassemia often do not shorten lifespan. You may want to seek genetic counseling if you have a family history of the condition and are thinking of having children.

16. Thank you!