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Taking Care Of A Child With Cystic Fibrosis

Added: 10/08/2007

Cystic Fibrosis is usually a hereditary disease that allows for a very short life span. Thirty three years old is the best statistic for today. However, with new medical advances, it may be possible for children with Cystic Fibrosis to live way beyond that number. This disease is known by its heavy mucous that clogs up the lungs and stomach. Kids have to be really careful when they have this disease.

Cystic Fibrosis is caused by a different coding in the CFTR (Cystic Fibrosis Transmembrane Conductance Regulator)gene. This can be tested for prenatally. Usually because the testing for this disease is so expensive, they will test one parent and if they test positive, they will test the other to see if they are a carrier or have a recessive gene. Only one gene is needed to develop Cystic Fibrosis. About 1 in 3800 children will develop it. There is no cure. It can get so bad that a lung transplant is the only answer.

Lung transplants can be quite expensive and you almost always have to replace both lungs. If one is clogged up, then th other will most assuredly get clogged as well. SO this leaves little room for error during the surgery. It is suggested that maybe the pancreas and/or liver could be transplanted at the same time to have everything done at once.

The main thought is that with an increase in Sodium and Chloride, there is an increase in mucous output due to more water than usual being absorbed into the cells. This leads to a buildup of mucous that can cause problems in the lungs, stomach, and pancreas. This disease is more common in Jewish people and people of European heritage.

Some tests that can be done are sweat tests, glucose testing, pulmonary function testing, examination of sputum, and lab tests of Chloride and Sodium. Other lab tests include checking for vitamin deficiencies and abnormal values of amylase and lipase.

Bacteria is a large breeder in the mucous pileup in the respiratory system. Staphylococcus Aureus, Hemophilus Influenzae, and Pseudomonas are attracted to this type of buildup. An infection is the worst thing that a Cystic Fibrosis patient could have. These types of infections are hard to fight because the bacterias become very resistant to antibiotics.

On kids, the best treatment thus far is doses of antibiotics such as Vancomycin and Ciprofloxin. They also do a procedure called Intrapulmonary Percussion. This is where the child lays down on the bed facing down with head over the side of the bed and a therapist beats on his back for about twenty minutes. He may cough a lot during the procedure and that is what they want. They have this done two to three times a day at first. It can increase to four to five times a day. The other end of the spectrum is they are placed on a breathing machine to help them rest a little so they are not working so hard to breathe all the time.

Since their digestive system is screwed up, these kids must additional vitamin supplements as well as extra of vitamins A, D, E, and K. They also must make sure they eat high calorie meals every day. The pancreas can also become clogged and stop producing insulin from the Islet of Langerhans cells. This will make the child become diabetic. They will placed on insulin to help keep glucose levels in a normal range.

A lot of support and communication are important for the child with Cystic Fibrosis. They can lead very difficult lives and need their parents to monitor their health when they can't. It can be difficult to make hard choices when they are very ill.


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