My nephew has Cystic Fibrosis, he's gotten psudemonas and they said they ca!


Question: My nephew has Cystic Fibrosis, he's gotten psudemonas and they said they can't treat it, what does that mean?
Answers:

(the other 2 answerers were quite complete on info on CF, but that wasn't your question)

It sounds like your nephew has a particularly resistant form of pseudomonas.
What that means is that the bacteria that is causing it is proving to be resistant to most or all of the available medications.

It doesn't mean that he will die, it means that they might need a different approach. Inhaled medications vs medications that go through the blood stream, for example. Infectious disease docs have a lot of tricks up their sleeves.

In the mean time, they will continue to give him the medication that appears to work the best, and will continue Chest PT, etc.

I wish him the best.

Occupational Therapist



Cystic Fibrosis is genetic disorder which, causes in a thick sticky mucus being developed in the airways, because of a mutation in the genes, which causes the CFTR protein not being developed. Without this protein in the cells, normal transport of water (osmosis) between the cells and its surroundings does not happen, what happens is that chloride ions cannot leave the cells because of there not being the CFTR protein, this causes the water outside of the cells to go in to the cells, leading to less water outside the cells. Therefore the mucus outside the cells is not runny, and is sticky and thick because of a lack of water outside the cells. Know this thick and sticky mucus is built up in the airways as the cilia (tiny hairs that transport the mucus) cannot transport the mucus due to it being too sticky and thick. The mucus which is built up around the airways due to it being thick and sticky, provides great conditions for pathogens (diseases like pseudemonas) to be developed, because the white blood cells (cells that destroy pathogens and diseases) cannot affect the diseases growing on the mucus. Therefore they may say you cannot treat it, but there are possible cures.

Know you can do physiotherapy on your nephew, this will try and remove as much of the thick and sticky mucus from the lungs as possible. This will make it easier for him to breathe and take in enough oxygen, but also reduces the risks of lung infections like pseudomonas. You'll probably have to do it a couple of times a day.

Antibiotics, could be used to destroy the bacteria causing the lung infections. However it would be difficult to getting the antibiotics to the tissue where they are needed, however aerosols (inhalers) could be used to breathe them deep into the lungs.

Mucolytics, are drugs that make the mucus more runny making it easier for the cilia to move out and let your nephew cough the mucus out getting rid of any infections on the mucus like pseudomonas.

Similarly DNAase enzymes make the mucus thinner and therefore easier to cough up the mucus, and again getting rid of any infections on the mucus like pseudomonas.

A more major and expensive treatment could be a lung transplant. The new organ would not be affected by cystic fibrosis, however the body will still be affected. Your nephew will need to take immunosuppresent drugs for the rest of his life to prevent his body reacting against the new organ and rejecting it, however these drugs weaken the immune system and make it easier for other diseases to develop however with cystic fibrosis who have the lung transplant do generally well.

Hare Krishna and good luck to your nephew.

NERD!!



Cystic fibrosis (CF) has no cure. However, treatments have greatly improved in recent years. The goals of cystic fibrosis treatment are to:
* Prevent and control lung infections
* Loosen and remove thick, sticky mucus from the lungs
* Prevent or treat blockages in the intestines
* Provide enough nutrition
* Prevent dehydration (a condition in which the body doesn't have enough fluids)
Depending on how severe the disease is, you or your child may be treated in a hospital.
Pseudomonas aeruginosa is a highly relevant opportunistic human pathogen. One of the most worrying characteristics of P. aeruginosa is its low antibiotic susceptibility.
The following organizations are good resources for information on cystic fibrosis:
* Cystic Fibrosis Foundation - www.cff.org
* March of Dimes - www.marchofdimes.com
* National Heart, Lung, and Blood Institute - www.nhlbi.nih.gov/health/dci/Diseases/cf…

http://en.wikipedia.org/wiki/Cystic_fibr…
http://www.medicinenet.com/cystic_fibros…
http://www.mayoclinic.com/health/cystic-…
http://www.nlm.nih.gov/medlineplus/ency/…
http://www.nlm.nih.gov/medlineplus/ency/…
http://www.merckmanuals.com/professional…
http://en.wikipedia.org/wiki/Pseudomonas…




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