Taking a Look at Cystic Fibrosis

Introduction Cystic fibrosis (CF) is the most common autosomal recessive disorder with short life span. The Republic of Ireland has the highest incidence rate and is believed to be born at a rate of 1 in 1461 people. Improvement in quality and frequency will increase the prevalence of all ethnic groups. In the United States, CF affects 30 thousand adults and children and the median predicted survival rate for affected people is estimated to be 2, which is 36.8 years. Situation) 3 is love

Cystic fibrosis is an autosomal recessive disorder - that is, two defective genes (one for each parent) are needed to develop the disease. The gene thought to cause cystic fibrosis is called cystic fibrosis transmembrane conductance regulator or CFTR. This gene is on chromosome 7. The severity and extent of this disease vary widely among patients with cystic fibrosis. Typically, this condition results in an increase in the chloride transport defect and trauma across epithelial cells and the viscosity of body secretions, particularly secretions from the respiratory tract (ie, the lungs, throat) and pancreas. As a result, patients are prone to pancreatic dysfunction and repeated chest infections

Cystic fibrosis affects cells that produce mucus, sweat, digestive fluids. These secreted fluids are usually very thin and slippery. However, in people with cystic fibrosis, defective genes cause thickening of secretions. Instead of acting as a lubricant, secretions block tubes, ducts and ducts, especially in the lungs and pancreas. Cystic fibrosis requires routine care, but people with this disease can usually work at school and work, and in the past few decades more people with cystic fibrosis The quality of life is good. Improvements in screening and therapy mean that people on cystic fibrosis now live on average about 30 years of age, and some live in their 40s and 50s.

Marten deVlieger was initially diagnosed with cystic fibrosis. This is a degenerative and incurable disease affecting the whole body, especially the lungs. "Life with cystic fibrosis is a continuous task." Most of us are said to be not 2 years old, 10 years old, 21 years old after birth ... to the current average CF life The living person thought that he shared the expectation of 42 years old, "I thought it might be", "He shared it, but Marten dreams of independence. During adolescence he We organized local dumps, started looking for metal scraps and parts and used it to make coarse heavy chest equipment to reflect the traditional mucus relaxation method of CF patients. ChestMaster 5000 was born.