Cystic Fibrosis – Know it all?

[Lazy Nite]

Hey ppl

I really need help with this topic…

I understand that cystic fibrosis patients require:

1. Long-term nebulised saline to clear the airways on a regular basis.
2. Long-term IV/Oral antibiotics to prevent infection
3. Enzymes such as creon, due to their pancreatin deficiency
4. Ongoing care such as physiotherapy

What other things would I need to look into…? Any good reading sources anyone could recommend?

Any suggestions please?

[Nik]

I know it's an American source but Merck Manual of Diagnosis and Therapy ?

[lamzee]

http://www.bestofhealthsec.nhs.uk/pr...guidelines.asp

Consensus Guidelines [Cystic Fibrosis Trust]

One of the places I worked in a while back had two CF patients. Both on bucketloads of creon, also calogen to put on weight. They got 100 vials colomycin 1 megaunit per month(nebulised four times a day), and occasionally tobramycin. One got put on 7% hypertonic saline to be nebulised at one stage--10x10ml mini-plasco water for injection added to 3x10ml martindale NaCl 30% (I think--you can re-check the figures!) mixed herself at home and decanted into a 150ml bottle. She also was on pulmozyme amps (nebulised) one a day, and had zithromax almost on demand from the doc. They both got unlicenced vitamins called "adeks" containing vitamins A D E K , which CF sufferers find hard to absorb due to abnormal mucus in the intestines, and also took 40mg omeprazole bd, and carbocistine syrup for mucus (which probably didn't help the stomach lining much, being a mucolytic!)

One major problem for CF patients is that they have to go to hospital rather a lot, but this is where they are most vulnerable to picking up infections, so it is best to have a dedicated CF unit/ward, rather than just a chest clinic.

Hope this helps

[funkeeduck]

worth checking back issues of Hospital Pharmacy magazine (see PJ website), as they had a special feature on CF within the last couple of years I think.
I'll try and find the link, bear with me!

[funkeeduck]

http://www.pharmj.com/pdf/hp/200506/...06_disease.pdf

http://www.pharmj.com/pdf/hp/200506/..._treatment.pdf

Gosh, bit older now than I'd realised, how time flies... Should be a good start point though.

[Jeff]

1. Long-term nebulised saline to clear the airways on a regular basis.

A quick transfer of info from PrivateRx - with thanks to John Latona

A new entry to the Drug Tarif

In section IX A appliances under inhalation solutions is a product called
"sodium chloride 6% solution-sterile"

It has been used in hospital to treat the lungs of patients with Cystic Fibrosis and now that it's in the drug tarif expect prescriptions.

The details below might be helpful to obtain it as none of the
wholesalers are currently stocking it.

Product name - Mucoclear 6%
pack sizes - 20x4mls and 60x4mls

distributor - pari medical ltd west byfleet

Tel- 01932 341122

Jeff

[Lazy Nite]

Thank you so much to everyone who replied my post….I found the links very useful

Does anyone know which bacterium comes first in cystic fibrosis?

1. Staph 2. Haemophilus 3. Pseudomonas ……is that the correct order?

[Gowan]

See the Cystic fibrosis entry at the foot of this page on the old PJ Online site. It contains the HP links you already have plus other articles

Site map/index for PJ Online (Careers in pharmacy...Charity page...Contact the PJ...Cystic fibrosis)

[Shwampa]

J. B. Lyczak, C. L. Cannon, and G. B. Pier
Lung Infections Associated with Cystic Fibrosis
Clin. Microbiol. Rev., April 1, 2002; 15(2): 194 - 222.

http://cmr.asm.org/cgi/reprint/15/2/194.pdf