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Doctor insights on: How Did Cystic Fibrosis Get Its Name

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Dr. William Walsh
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Cystic Fibrosis (Definition)

A genetic disorder of the lungs and digestive tract. It causes recurrent lung infections and inhibits growth in patients and average life expectancy of patients with cystic fibrosis ...Read more


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L am 58 can I get cystic fibrosis at that age.

L am 58 can I get cystic fibrosis at that age.

No: You are born with cystic fibrosis, you don't "get" it later in life. It may not be diagnosed right away depending on the type and severity of symptoms. For example, a newborn with an intestinal blockage would be picked up quickly, but an older child or even an adult who presented with mostly sinus symptoms might not get diagnosed until later.

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Do males or females get cystic fibrosis and why?

Do males or females get cystic fibrosis and why?

Equal: More than 10 million americans are unknowing, symptomless carriers of the defective cystic fibrosis gene. In order to develop cf, an individual must inherit a defective gene from each parent. Since the CF gene is not on a sex chromosome (x or y), males and females are equally affected.

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How can you get easily checked up for cystic fibrosis?

Sweat chloride test: CF is an abnormality of chloride channels. A simple test where they tape a special paper to your arm and measure the amount of chloride in your sweat can help rule in or out cf.

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I'm worried I have cystic fibrosis. How do I get tested?

I'm worried I have cystic fibrosis. How do I get tested?

Sweat test: Cystic fibrosis is diagnosed by an abnormal sweat test and clinical symptoms that match the diagnosis. Genetic tests can also be done to determine if there are any mutations of the cftr gene that are known to cause cystic fibrosis.

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Do lots of people get cystic fibrosis, or is it unusual?

Do lots of people get cystic fibrosis, or is it unusual?

Depends on ethnicity: CF is a genetic condition inherited from carrier parent. Carrier frequency depends on the ethnic background. 1/25 caucasians have one copy of normal, one copy of a mutated CF gene. Both genes have to be mutated to have cf. Hispanic carrier frequency 1/50, africanam 1/70, asian 1/90. 1/5000 babies are born with cf, this may be decreasing, since pregnent females are now screened for CF

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What is a good way to get checked up for cystic fibrosis?

What is a good way to get checked up for cystic fibrosis?

Sweat chloride test: Talk to your physician who can direct you and order a test, if indicated. The gold standard test is the sweat chloride test. Blood testing may also be available, again, if indicated.

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What happens to me if I get diagnosed with cystic fibrosis?

Cystic fibrosis: Early diagnosis and management with likely increase your live expectancy.

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Help please! Is cystic fibrosis something you can get in childhood?

Words matter: CF will be recognized or diagnosed in childhood, so in a way you could say you get it then. However, the word use is confusing. You don't "get it" like you get a strep throat, you are born with the abnormal gene information that causes the disease.

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Can someone get cystic fibrosis as a teenager?

Can someone get cystic fibrosis as a teenager?

Yes: A small segment of patients affected by cystic fibrosis does not show typical symptoms until teenage and apparently has very mild disease. This depends upon the mutation involving cftr gene (homozygous vs heretozygous so on).

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What percentage of female va male fetuses get cystic fibrosis?

50/50: It is about 50/50; there is no real difference in the incidence of cystic fibrosis between males and females.

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Is it bad to get x-rays done when you have cystic fibrosis?

Is it bad to get x-rays done when you have cystic fibrosis?

No: This will not cause a problem, and may help diagnosis a new issue (although not always).

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How noticeable is the salty skin that people get with cystic fibrosis?

Depends on Severity: This would depend on the severity of the disease state in the individual, but by and large, it's enough of a difference from "normal" sweat to make one take notice. Clinically, the sweat chloride level is measured twice to make the diagnosis of cystic fibrosis (cf). The cutoff level to make the diagnosis of CF is 60 milliequivalents per liter of sweat chloride.

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Can you explain if it's possible to get cystic fibrosis without carrying the genes for it?

No: Cystic fibrosis (CF) is a genetically inherited disease, so CF or cf-related disease (atypical cf, crms) rely on having at least 1 inherited gene. The other part of CF diagnosis is having an elevated sweat chloride. It is possible to have a positive sweat chloride due to other circumstances (thyroid imbalance, trisomy 21), but without CF genetics, there cannot be a diagnosis of cf.

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In cystic fibrosis kids, how do you get them to swallow the enzymes?

Open the capsules: Enzyme capsules can be opened and the beads sprinkled on food or mixed with a food such as applesauce. This works well as long as the food is not acidic and the child doesn't chew the beads. There are also cups with a ledge to hold a pill or capsule that help a child learn to swallow pills with liquids. Teaching a child to swallow jelly beans can also work.

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For what reasons might the pancreas get blocked by mucus in cystic fibrosis patients?

Thick: The secretions in cystic fibrosis are more viscous (thicker) than normal and interfere with normal drainage resulting in blockages of the bronchial tubes of the lung with resultant pneumonia and lung injury. Similar issues involve the pancreas. Newborns with the disease frequently have difficulty passing their bowel movements.

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Do both parents have to be cystic fibrosis carriers for a child to get it?

Yes: Cystic fibrosis is an autosomal recessive disease which means that a person needs to inherit 2 abnormal genes in order to have the disease. Those 2 abnormal copies come one each from the 2 parents, so both parents need to be at least carriers.

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My child as cystic fibrosis, how likely will he get pseudomonas aeruginosa?

Later in life: Patients with cystic fibrosis often get these bacteria in their airways by adulthood. The vast majority of adult CF patients have airways colonized with pseudomonas aeruginosa (pa). Younger patients (kids) tend not to have pa in their airways, but that can vary.

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What can I do if my brother has a mild case of cystic fibrosis and my parents are making me get tested?

Cystic fibrosis: Good to know if you have the gene because sometimes clinical symptoms do not develope early. Also knowing if you are a one gene carrier (but not affected) helps with family planning in the future.

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