Cystic Fibrosis

by Natalie Nguyen, Olivia Larkin

More Symptoms

Clubbed fingers and toes

  • the ends of fingers and toes become swollen and rounded


Polyps in nostrils

-soft growths in nasal passages that are caused by inflammation in the nose


Odorous Stools

-Mucus buildup in digestions


-Salty Skin


-Coughing with phlegm


-lung infections


-wheezing

Cellular Problem

Cystic Fibrosis is a disease that is caused by the mutation of the Cystic Fibrosis Transmembrane Regulator gene. CFTR is plasma membrane protein, the outermost layer of the cell which allows things to go in and out.The mutation affects the tertiary structure of CFTR.The organelles that work incorrectly are the cell membranes, in peroxisomes, and in lysosomes. The cell membrane allows things to travel in and out of the cell. The peroxisomes are organelles that contain enzymes that assist in metabolic reactions. Lysosomes also contain enzymes that help breakdown many different polymers. Any needed proteins fail to transport through the membrane. With Cystic Fibrosis patients, their cells trap salt in the cells and the moisture from the surrounding tissue is drawn and taken to the salty interior of the cell. This dries out the mucus making it thick and sticky, causing organs to clog.

This disease causes severe damage to the respiratory and the digestive system.

Cystic Fibrosis affects the respiratory system. The lungs are a pair of air filled organs that are located on both sides of the chest. You inhale air through your trachea (windpipe) and then through tubular branches called the bronchi. It then gets dispersed through even smaller branches called bronchioles.

Normally, bronchi are covered with cells that produce a thin layer of mucus. Cilia (little hairs) on the surface of the breathing tubes catch any foreign particles and send them away. Cystic fibrosis causes the mucus to become so sticky and thick that the cilia cannot sweep out the germs and other particles. Because of the infection, your immune system sends out white blood cells for an attempt to remove the infection. After white blood cells attack bacteria, they die and add on to the thickening layer of mucus.

Symptoms:

  • respiratory system

  1. abnormally thick and sticky mucus develops

  2. the abnormal mucus can clog the airways

  3. leads to bacterial infections and serious breathing

  4. bacterial infections cause coughing,wheezing, and inflammation

  5. over time bacterial infections and mucus buildup causes serious lung damage

  6. scar tissue and cysts develop in the lungs
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Cystic fibrosis also affects the digestive system. Your digestive system breaks down food, absorbs the needed nutrients, and gets rid of waste.

Cystic fibrosis causes mucus to fill the pancreas whose job is to produce enzymes that break down food in the intestines, letting nutrients be absorbed by your body. Food is not properly digested and causes malnutrition and will then lead to difficulty in maintaining weight.

Diabetes can also be an effect from cystic fibrosis. The pancreas also produces a hormone that helps control blood sugar. When clogged with mucus, the abnormal buildup of hormones start to break down the pancreatic tissue which stops insulin production causing diabetes.

Symptoms for the digestive system


  1. abnormally thick and sticky mucus builds up in pancreas

  2. mucus blocks ducks in pancreas

  3. this reduces the production of insulin and prevents enzymes from aiding digestion in the intestines

  4. leads to diarrhea, malnutrition, poor growth, and weight loss

  5. this shortage of insulin can lead to a form of diabetes, especially in adolescents and adults (cystic fibrosis-related diabetes mellitus

-Clubbed fingers and toes
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Transmission:

Cystic fibrosis is a genetic disorder that is transmitted to a child when both parents of a child have the recessive gene. (Note they do not have the disease.) There is a 25% that their child will develop CF, 25% that the child will not develop CF, and 50% that the child will receive the gene but not have CF.The individual inherits the disease in an autosomal recessive pattern (both copies of the gene in each cell have a mutation )


Tests:

Genetic tests-This can be done by taking a blood sample from the mothers womb before birth.

Sweat Test: They measure the level of salt that is in your sweat. People who are diagnosed with CF have an abnormal amount of sweat in their sweat.

Immunoreactive trypsinogen: This test is a standard tests used on newborns to scan for CF. If there is a high level of IRT detected that suggest CF further testing on the newborn will be done.
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Treatment

There is no cure for Cystic Fibrosis. Although, there are treatments that have helped the symptoms.

-Antibiotics are used to help prevent and treat lung infections and sinus infections.

  1. Taken by mouth

  2. Given in veins

  3. Breathing Treatments

Lifespan of those treated who are children live to about their early 50’s lifespan of adults who are treated live to about mid 40’s.


Untreated: Cystic Fibrosis is common to be untreated in adults. If untreated in children, a severe chest infection and chronic diarrhea can occur. Due to CF the child would be very weak and unable to absorb fat and protein. In 98% of cases, the pancreatic gland does not work properly and the gut is affected. Due to a constant battle between germs, patients can also develop arthritis.

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