Cystic Fibrosis, second post


Cystic fibrosis is an inherited disorder that causes significant damage to an individual’s lungs, digestive system and other organs throughout the body, Cystic fibrosis affects cells that produce mucus, digestive fluids, and sweat. In normal individuals, these bodily fluids are thin and slippery; in individuals who have cystic fibrosis they are thick. This is due to a defective gene that causes these bodily secretions to be thick and sticky. As one would assume, having thick bodily secretions can cause problems such as obstruction. Instead of acting as a lubricant, the secretions plug passageways, tubes, and ducts especially in the pancreases and lungs.

The cause of cystic fibrosis is a mutation in the CFTR gene. The CFTR gene changes in a protein that regulates the movement of salt in and out of cells. In an individual who has cystic fibrosis, the CFTR gene inhibits the protein from functioning properly. The mutation in the CFTR gene is inherited. Each individual inherits a CFTR gene from both of their parents. The inheritance of two faulty CFTR gene is what causes cystic fibrosis. Those who only inherit one faulty CFTR gene and one normal CFTR gene are carriers for cystic fibrosis, Carrier experience no symptoms. Cystic fibrosis can occur in all races. However, it is most common in white Americans.

Those who suffer from cystic fibrosis experience several respiratory complications such as damaged airways, chronic infections, growth in the nasal passages referred to as nasal polyps, hemoptysis (coughing up blood), pneumothorax, respiratory failure, and acute exacerbations. They also experience complications in their digestive system. Complications such as nutritional deficiencies, diabetes, obstructed bile duct, and intestinal obstruction.

There is no cure for cystic fibrosis. However, there are designated programs that can help ease symptoms and reduce complications. Due to the complexity of the disease, early intervention is recommended. Individuals who have CF are often cared for by a team of medical providers who work together in order to prevent and control pulmonary infections, remove and loosen mucous from the lung, treat and prevent intestinal blocking, and provide adequate nutrition. There are also several antibiotics that can aid in the prevention and treatment of several pulmonary infections.


Comments

  1. I learned the basics of cystic fibrosis in biochemistry and we watched a video about people with this disorder. It was so hard to see what they have to go through because they have this disorder. I know that there are programs that can help ease the pain and symptoms, but I really hope that sometime in the future, there will be a permanent cure.

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  2. I feel like I hear a lot about this case in children. Which I think makes sense due to the underdevelopment of certain organs. As they grow maybe adults are able to better manage this due to treatments they receive throughout their lives, management, and more development.
    I also had an experience with a child who had an episode while at home and was affected by allergies as well. These two things did not add up well, but they made it out fine.

    I think it is interesting that now that we know more about this family take into account whether they should have children and risk the disease. This I think would be a great place for genetic therapy to take affect or be used as we move forward.

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  3. Wow. I feel really bad now. I also did my post over cystic fibrosis. I have been interested in this disorder since biochemistry. I have always thought it was so interesting how they use High-frequency chest wall oscillation. It is crazy to me that they came up with a vest to loosen mucus within the body.

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