SickleCellAnemia：镰状细胞性贫血.pptVIP

Mechanism -HbS When sickle haemoglobin (HbS) gives up its oxygen to the tissues, HbS sticks together Forms long rods form inside RBC RBC become rigid, inflexible, and sickle-shaped Unable to squeeze through small blood vessels, instead blocks small blood vessels Less oxygen to tissues of body RBCs containing HbS have a shorter lifespan Normally 120 days Chronic state of anaemia Other Sickling Disorders Other types of Hb combine with sickle Hb Hemoglobin S-C disease Sickle haemoglobin (HbS) + (HbC) Hemoglobin S-Beta thalassemia Beta thalassaemia gene reduces the amount of HbA that can be made Sickle haemoglobin (HbS) + reduced HbA Milder form of Sickle Cell Disorder than sickle cell anemia Serious Complications Leg ulcers Seen in patients older than 10 years of age Resistant to therapy and cause significant morbidity Ophthalmic complications Proliferative retinopathy, vitreous hemorrhage, retinal detachment Priapism Distressing complication that occurs at all ages Difficult to treat Causes a high incidence of impotence Chronic Anemia Associated with fatigue, irritability, jaundice, pain, delayed puberty, leg sores, eye problems, gum disease Child Specific Issues: Coping with Pain Pain happens more often On an average of one third of all days Lasts longer Generally all day, even if not continuously all day Associated with great tiredness about half the time Causes them to spend significant time in bed On average the time spent wholly or partly in bed adds up to about a week of every school term. Psychosocial Issues Variability and Unpredictability Some are mildly affected and largely free from pain, while others have frequent and severe pain Most children go through good and bad patches Doctors cannot predict who will be severely affected. No easily overt detectable signs of sickle pain So children known to have sickle cell disorder who say they are in pain must be trusted If they can rely on the adults around them to take them seriously, they a