Cystic Fibrosis

Written & Researched by Asis Tung


What is Cystic Fibrosis?


Cystic fibrosis (CF) is a progressive and genetic lung disease that affects the breathing of patients of all ages. It directly affects the lungs and respiratory system.

  • CF will cause the CFTR proteins in the body to lose function and not be able to move chlorides, salts and other important minerals to the cell surface.

  • When this occurs in the lungs, thick mucus starts to fill various organs including the lungs, and it traps bacterias that causes infections, swelling and failure.

  • It starts collecting germs that should not be in contact with one's lungs or bodies and the ones that usually the body flushes out.

  • The buildup of mucus blocks the body's digestive system, and can create damage in the bile ducts which are crucial for digestion and processing minerals/nutrients.



What causes CF?


  • Cystic fibrosis is caused by a change or loss of a gene called CFTR which in full form is Cystic fibrosis transmembrane conductance regulator. This genetic cell is all about controlling flows of salt and fluids in/out and throughout your body.

  • Cystic fibrosis patients have usually caught on two copies of CFTR (one from each parent.)

  • If this gene cannot provide its proper functions to the body, it causes irregular motions in the systems leading to thick, sticky mucus.

  • Usually if the child only has one copy of the gene from one of their parents they will not have the disease, however they will become carriers of the disease.

  • This can later pass generation through generation to their own kids.

  • It is most popularly noticed in white people from Northern European ancestry and it is in the family history, passed down through generations. This is why it can usually be detected in newborn children and they can be prevented from too much pain.



Diagnosis


If two CF carriers have children the chances of the child having Cystic Fibrosis is as follows:

  • 25 percent of children will have CF

  • 50 percent of the children will be a carrier and not have CF

  • 25 percent will either be a carrier or have the disease.

  • In the world over 80,000 people are living with CF

  • 1000 new cases of CF are noticed each year

  • More than 75% of CF patients have their disease noticed before age 2

  • Diagnosis tests include sweat test, carrier test and screening in newborns.



What Are The Symptoms?


  • Salty tasting skin in mouth and near lips area

  • Continuous cough that produces thick mucus

  • Wheezing in throat and in daily life

  • Lung infections detected

  • Sinusitis (infected throat) stuffy nose or inflamed nasal passages.

  • Greasy and smelly stool

  • Poor weight gain and growth (Full stopped weight gain and growth)

  • Constipation and not being able to pass stool.

  • Shortness of breath



Life with CF


  • The severity and symptoms range from person to person. Lots of factors such as age and gender affect the individual's vitals during the disease.

  • CF care has added years and years onto people's lives. With CF curing technology, children can get married, have kids and live their dreams.



Complications?


  • Growths in the nose that block the nasal passages and gives difficulty breathing.

  • Coughing up blood

  • Air leaking into the lungs and causing sudden loss of breath and chest pain

  • Lung failure

  • Damaged airway leading to infections, inflammation and chronic infections.

  • Nutritional deficiencies, not being able to absorb protein, fats or nutrients.

  • Liver diseases

  • Diabetes is about a 20% higher chance when someone is diagnosed with CF.

  • Infertility in men is the most common complication for men and they cannot become biological fathers.

  • Reduced fertility in women.

  • Thinning bones and mental health problems.

  • Constipation



Rules and pathology


  • CF patients are told to wear masks in hospitals and places with germs to reduce more germs going into their bodies.

  • They should not come in contact with other CF patients.

  • Getting genetic tests to prevent CF.

  • Airway clearance helps to loosen mucus so that it can be coughed out and breathing function can be improved.

  • Clinical trials that are always occurring to help find cures to Cystic Fibrosis.

  • Lots and lots of medications to fight diseases and infections.

  • Lung transplants help to start a new lifestyle with a new pair of healthy lungs

  • Large shaking west that helps to clear out and loosen thick, stubborn, sticky mucus.


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