Thalassemia DEFINITION Thalassemia is an inherited blood disorder
Thalassemia
DEFINITION Thalassemia is an inherited blood disorder characterised by less oxygencarrying protein (haemoglobin) and fewer red blood cells in the body than normal.
Causes of thalassemia Thalassemia occurs when there’s an abnormality or mutation in one of the genes involved in hemoglobin production. You inherit this genetic abnormality from your parents. If only one of your parents is a carrier for thalassemia, you may develop a form of the disease known as thalassemia minor. If this occurs, you probably won’t have symptoms, but you’ll be a carrier. Some people with thalassemia
Symptoms of thalassemia The symptoms of thalassemia can vary. Some of the most common ones include: bone deformities, especially in the face dark urine delayed growth and development excessive tiredness and fatigue yellow or pale skin
Different types of thalassemia There are two main types of thalassemia: Alpha thalassemia occurs when a gene or genes related to the alpha globin protein are missing or changed (mutated). Beta thalassemia occurs when similar gene defects affect production of the beta globin protein.
Diagnosis Thalassemia can be diagnosed via a complete blood count, hemoglobin electrophoresis, and DNA testing.
Treatment Some of the treatments include: blood transfusion Medications (Hydroxyurea, Hydroxycarbamide, Deferiprone, Deferisirox, Butyrate) and folic supplements possible surgery to remove the spleen or gallbladder
Nursing Interventions Activity. Assist the client in planning and prioritizing activities of daily living. Health education. Explain the importance of the diagnostic procedures (such as complete blood count), bone marrow aspiration and a possible referral to a hematologist; Prevent infection. Assess for local or systemic signs of infection, such as fever, chills, swelling, pain, and body malaise; instruct the client to avoid contact with people with existing infections; instruct the client to avoid eating raw fruits and vegetables and uncooked meat; stress the importance of daily hygiene, mouth care, and perineal care; and teach the client and visitors the proper hand washing. Prevent bleeding. Assess for any frank bleeding from the nose,
NURSING DIAGNOSIS Ineffective tissue perfusion related to reduced cellular components that are essential to deliver pure oxygen to the cells. Activity intolerance related to imbalance of oxygen supply and consumption needs. Imbalanced nutrition: less than body requirements related to lack of appetite. Ineffective family coping related to impact of the disease to family functioning.
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