Sickle cell anemia is a global public health disorder that affects millions of people across the globe. It is an inherited autosomal recessive hemoglobin condition caused by a beta-globin gene mutation that results in an aberrant sickle cell hemoglobin molecule with glutamic acid replaced by valine at position six of the beta-globin chain. Sickle cell anemia is a hereditary illness characterized by persistent microvascular blockage by sickle-shaped cells, chronic hemolytic anemia, and tissue destruction. Red-cell dehydration, hypoxemia, metabolic acidosis, and hyperthermia are typical vaso-occlusive triggers. Children with sickle cell anemia frequently have poor growth and nutritional status, increased protein turnover, decreased lean body mass, and elevated resting and lower activity energy expenditures. These clinical findings present a problem for recommending exercise when taken as a whole, while engaging in physical activity increases the chance of issues like a "painful episode,". Children with sickle cell anemia miss out on the favorable health effects of regular physical activity, such as the growth of lean body mass, strength, and fitness. Vaso-occlusive events are recurring and prevalent in sickle cell anemia-related morbidity. Patients also have a higher chance of developing central nervous system conditions such cerebral vasculopathy and overt and covert stroke. It has been noted that the pathophysiological symptoms of SCA decrease skeletal muscle function. In fact, it has been demonstrated that individuals with SCA have diminished forearm and respiratory muscular maximal strength as well as peak locomotory muscle power. Vaso-occlusion, chronic hemolytic anemia, and vasculopathy form the core of sickle cell anemia pathophysiology and result in both acute and chronic consequences that have a significant negative influence on quality of life QoL. Even though clinical care for children with SCA has significantly improved in developed countries over the past few decades, children and adolescents with SCA still have a lower quality of life than their peers due to painful vaso-occlusive crises, frequent hospital admissions, and lengthy hospital stays. Generic and disease-specific measures of quality of life have been developed and validated in chronic illnesses to evaluate the influence of health on functional domains such physical and mental well-being, exhaustion, pain, social engagement, relationships, and emotional distress. Examples include the Child Health Questionnaire, Pediatric Quality of Life Sickle Cell Disease Module PedsQL SCD, and Patient-Reported Outcomes Measurement Information System PROMIS, all of which have been used to study SCA and have revealed various degrees of quality-of-life impairment, primarily in the United States. Health-related quality of life refers to the patient's assessment of how his or her well-being and level of functioning, in comparison to the perceived ideal, are influenced by personal health. A health-related quality of life assessment often considers physical, social, and emotional functioning in addition to academic and occupational performance. Studies on the quality of life for people with SCA have significantly risen during the previous five years. Measurement of health-related quality of life is beneficial for many reasons. Health-related quality of life can be used to measure the efficacy and effectiveness of treatment interventions, to predict outcomes and resource use, and to direct therapy. More importantly, the measurement of health-related quality of life aids in understanding the burden of disease that patients experience.
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assessing hand grip strength
Timeframe: 3 months
assess weight
Timeframe: 3 months
assessing Lower limb, trunk muscles strength.
Timeframe: 3 months
determine the child's standing height in centimeters (cm).
Timeframe: 3 months
assessing Health related quality of life
Timeframe: 3 months