Doctor insights on:
Oldest Person With Cystic Fibrosis
Cystic fibrosis is a genetic disorder involving lungs & digestive tract, causing recurrent lung infections due to thick inspissated mucus & poor growth in majority of patients due to malabsorption. Treatment involves inhaled respiratory therapies to prevent lung disease & pancreatic enzymes to enable absorption of nutrients. Average life expectancy is near 40 years, ...Read more
Varies: Not everyone has a similar experience post lung transplant. In general, respiratory symptoms and breathing, overall improve, and people are able to do activities they were not able to do before as easily. Not all respiratory symptoms go away. Various medications are required and close follow up is mandatory. Survival after lung transplantation is ~ 50% at 5 years. ...Read moreSee 1 more doctor answer
Varies: Newborns can present with bowel obstruction from lthickened meconium , early stool, from lack of normal enzymes.Sometimes bowel atresia and twisted bowel in utero are other manifestations. In child, respiratory symptoms may result with over aeration and asthmatic symptoms from mucus plugging in lungs. Malabsorption, fatty liver, and anemia in early childhood. Some mild forms do not show until later. ...Read more
Extra loss: Cystic fibrosis is caused by a genetic mutation of cftr protein that moves chloride ions out of cells in respiratory tract, but into the cells in sweat gland ducts. Since cftr protein is not functional in CF pts, excess chloride gets secreted in their sweat, which causes the sweat to have higher salt concentration. That is why a sweat test measuring chloride is used as a diagnostic test for cf. ...Read more
Pancreatic insuffic: The pancrease is the organ that (among other things) secretes enzymes that help the body absorb fat. In CF the pancrease can become clogged and fail to do this... So the patient needs to take enzymes orally. Additionally, when the lungs are injured enough, cfers will start having a lack of appetite as well, the cause of this is less clear. ...Read moreSee 2 more doctor answers
Is it possible for a person to have a "minor" form of cystic fibrosis so-to-speak? And if yes, how would it manifest?
Yes: I would rather use a term "mild" or "atypical" instead of minor form of cystic fibrosis. Cystic fibrosis is caused by mutation involving cftr gene, and there are a few hundred different mutations. Atypical or mild form of CF manifest at a later age than in infancy as often seen in classic form, and mild in symptoms. ...Read moreSee 1 more doctor answer
What happens if there is an intrapulmonary shunt or the person has cystic fibrosis, can they survive on a ventilator?
Yes: Shunts and CF do not preclude survival on a ventilator. It may be more difficult to clear secretions from CF on ventilator but can be done ...Read more
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