How CF affects Multiple Organ Systems

Normally, in a person without cystic fibrosis,  all organ systems work together to help carry out important processes within the body. However, in a CF patient, the organ systems are damaged by the excessive mucus buildup.  The most common systems that CF affects is the respiratory, digestive, and reproductive systems.

The respiratory system is responsible for bringing oxygen into the body and expelling carbon dioxide out of the body.  It is responsible for bringing in an essential nutrient and getting rid of a harmful waste product, so the correct functioning of this system is extremely important.  For CF patients, this system is affected severely.  One way that CF affects the respiratory system is by bronchiectasis.  Bronchiectasis occurs when airways are damaged due to the large amount of mucus present.  This makes breathing difficult for the CF patient.  CF can also lead to chronic lung infections, because mucus is a breeding ground for bacteria. 

Another system that is affected by CF is the digestive system.  In a normal human body, the digestive system helps break down food, absorb water and nutrients, and get rid of waste.  When CF is present, several problems can occur in the digestive system.  One problem is nutritional deficiencies.  Mucus block the tubes which digestive enzymes move through from the pancreas to the intestines.  This leads to inefficient absorbance of nutrients needed by the body.  Mucus can also block the bile ducts, resulting in liver problems and gallstones.  Rectal prolapse is also common is CF patients.  Rectal prolapse occurs when internal rectal tissue comes out of the anus, due to frequent coughing. 

The reproductive system also has some serious effects due to CF, especially infertility.  Mucus can block the vas deferens, which connects the testes and the prostate.  This results in the process of making sperm to be destroyed.  Most men with CF are infertile, but women can conceive and have children. 

Resources:  http://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/basics/complications/con-20013731

Treatment Options for Cystic Fibrosis

Although living with cystic fibrosis seems difficult and overwhelming, there are several treatment options that are available.  In this blog post, I will be focusing in on some of the treatment options and how they work.

One of the devices used to treat CF is called a chest clapper.  A chest clapper is a handheld device that mimics the effect of cupped hands that are over the chest.  It is used to loosen mucus in the airways.  It literally claps on the person's chest.

http://openi.nlm.nih.gov/detailedresult.php?img=3094358_1745-9974-7-2-1&req=4



 Another treatment option available to CF patients is an inflatable chest.  This is a device that is worn around the chest that vibrates at high frequencies.  Like the chest clapper, it also tries to loosen mucus from the airways.

http://sunsci.wikispaces.com/Cystic+Fibrosis

Lastly, mucus-thinning drugs can be used to help treat CF.  These drugs help thin the mucus in the airways so it can be coughed up.  All of these treatment options are usually used together to provide the best possibilities of treating cystic fibrosis.

Here is the website for Pulmozyme, which is a drug that is commonly used to treat CF. It has an interactive video that shows how it works.
 http://www.pulmozyme.com/how-pz-works.html

Resources used: 
http://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/basics/treatment/con-20013731

How the pathophysiology is accquired

Cystic fibrosis is a genetic disease.  This means that the basis for developing CF is inherited from parents. As stated in earlier posts, a defect in the CFTR gene is what causes the development of CF.  Remember that the CFTR gene codes for the protein that controls ion, salt, and water movement out of the body's cells.

Each child receives two CFTR genes, one from each parent.  If the child receives only one faulty CFTR gene, they are considered a carrier. Carriers usually do not have any symptoms of CF, but they have the risk of passing it on to their children one day.  If the child inherits two faulty CFTR genes, the child will have cystic fibrosis.  This is called an autosomal recessive disease.  For the person to develop the disease, both copies of the defective gene must be present. 

This picture shows how the genes can be passed on from the parents.  There is a 25% chance (1 out of 4) that the child can inherit two normal genes or two faulty genes, and there is a 50% chance (2 out of 4) that can inherit one faulty and one normal gene.

Resources:
http://hihg.med.miami.edu/code/http/modules/education/Design/Print.asp?CourseNum=1&LessonNum=3
http://www.nhlbi.nih.gov/health/health-topics/topics/cf/causes.html