Pseudomonas aeruginosa in cystic fibrosis

What is pseudomonas aeruginosa?

Pseudomonas aeruginosa or ‘P. aeruginosa’ is the most common organism causing chronic lung disease in patients with cystic fibrosis. Chronic infection with P. aeruginosa can increase the number of chest infections and in some people, can accelerates the rate of lung decline. It is important to try and completely get rid of infection early on.

P. aeruginosa is a bacterium and is found in many natural and domestic environments.

These include: plants, soil, surface water (stagnant pools of water) and warm moist environments containing organic material or contaminated by human or animal waste.

Hydrotherapy pools and jacuzzis can be a risk for people with cystic fibrosis because the combination of water, warmth, aeration and human contamination can make trying to keep the water clean difficult. It can be the ideal conditions for P. aeruginosa.

The sea is not a risk factor as P. aeruginosa cannot survive in the high salt concentration.

How can P. aeruginosa infection be prevented?

A few strategies are useful in reducing pseudomonas infection:

• Regular mucus, snot samples and cough swab samples should be taken (we do this at every clinic visit and each time your child has new chest symptoms).
• Good hand hygiene by staff, patients and families when in clinic.
• Separating those children with P. aeruginosa from those who do not have P. aeruginosa in their airway.
• Clinic rooms, toys, equipment and the spirometry room are cleaned in between patient use.
• Children admitted to the ward have their own cubicle and they are told not to wander around the ward but to stay in their cubicle.
• Patients with cystic fibrosis should not mix with each other in hospital and are advised not to mix socially.

How is P. aeruginosa treated?

1st time P. aeruginosa is found

If your child is well (they do not have any chest symptoms such as a cough) then their treatment is:

• 3 months of nebulised colomycin and 3 months oral ciprofloxacin. The 1st dose of colomycin is given on the cystic fibrosis unit. This is because it can sometimes irritate the airway and it is important this is checked for. If your child has a reaction to the nebulised colomycin (such as a fall in lung function, chest feels tight or wheezy), we can use Salbutamol to open up the airway and relieve the symptoms, or try an alternative nebulised antibiotic.
• If your child has a chest infection when P. aeruginosa is found, a 2 week course of intravenous antibiotics will be given followed by the above treatment.
• If your child is under 6 months when they 1st have P. aeruginosa, we will give 2 weeks of intravenous (IV) antibiotics.
• Mucus, snot samples and cough swab samples should be taken every month to check that P. aeruginosa does not to grow.
• If after 3 months of treatment your child has remained symptom free and P. aeruginosa has not come back, treatment can stop.
• If your child develop a chest infection whilst on treatment, a 2 week course of intravenous antibiotics may be needed. The colomycin and ciprofloxacin will then continue until complete.
• If during the 3 month period or at the end of treatment your child still has P. aeruginosa, a 2 week course of intravenous antibiotics will be needed.

If P. aeruginosa comes back

Depending on how long it is before P. aeruginosa comes back, different treatment strategies may be recommended. This may be a course of intravenous (IV) antibiotics or an alternative nebulised antibiotic (such as tobramycin, also called Bramitob or Tobi).

If P. aeruginosa keeps coming back

If your child continues to have P. aeruginosa, despite all the therapy we can do, we will assume the bacteria is permanently in their lungs. A long-term nebulised antibiotic will be prescribed. The aim of this treatment is to suppress the bacteria and reduce the number of chest infections.

As well as this an antibiotic, azithromycin will be prescribed. This should be given once daily for 3 days per week (usually Monday, Wednesday and Friday). This antibiotic reduces inflammation in the lung.

Whilst we try to clear P. aeruginosa from your child’s lungs, you will be seen at the beginning of a pseudomonas clinic. Your child will continue to be seen in this clinic up to 3 months after treatment is complete (6 months in total).

Important additional information

There is a number of pseudomonas types such as pudita and fluorescens. These are not associated with causing lung damage in the way that aeruginosa is. Because of this, we rarely treat these if found on mucus, snot samples or cough swab samples.

Nebuliser and physiotherapy equipment should be cleaned after each time it is used. Please ask the physiotherapist for further details.

Nebulised antibiotics should be given 2 times daily, usually after chest physiotherapy. Chest physiotherapy clears mucus out of the airway and the antibiotic lines the airway to fight the bacteria. The cystic fibrosis physiotherapist will explain this in more detail.

Oral ciprofloxacin should be given 2 times daily. Please read the instructions on the drug packet carefully. This medicine needs to be given 1 hour before or 2 hours after milk or milk products. When taking this medicine the skin becomes sensitive to the sun (even on cloudy days) so sun block is recommended, otherwise the skin will burn.

Sheffield Children’s Hospital will provide the full 3 months of nebulised colomycin (as you cannot get this from the GP) and 1 month’s ciprofloxacin. You can get the rest of the course of ciprofloxacin from the GP.

If you have any questions after reading this resource please ask a member of your cystic fibrosis team.