Treatments for additional lung related problems

• Coughs and colds
• Asthma
• Severe Small Airways disease/Fixed Airways Obstruction or Bronchiolitis Obliterans
• Allergic Bronchopulmonary Aspergillosis (ABPA) or Aspergillosis
• Unusual organisms that colonise sputum
• Haemoptysis (coughing up blood)
• Pneumothorax
• Nasal polyps
• Sinusitis
• Home oxygen
• Lung transplant

Coughs and colds

These are caused by viruses but antibiotics are always given in cystic fibrosis to counteract the increased risk of secondary bacterial infection. Doses of prophylactic drugs are doubled or another antibiotic is prescribed such as:

• Augmentin if not taking already
• Azithromycin
• Ciprofloxacin

Asthma

Asthma is common in children with cystic fibrosis, as it is in children without cystic fibrosis. Treatment of asthma follows the British Thoracic Society guidelines. Consult the asthma guidance for details of asthma care requirements if the child has asthma in addition to cystic fibrosis. Dry powder inhalers are preferred for steroid inhalers and use of spacer devices is encouraged.

Severe Small Airways disease/Fixed Airways Obstruction or Bronchiolitis Obliterans

The terms listed above such as ‘severe small airways disease’ are used to describe a small number of children with the following ongoing symptoms:

• Little if any sputum production despite large amounts on the chest
• Wheezing
• Tight chest
• Severe obstructive pattern on spirometry testing
• Minimal bronchiectasis on CT scan chest
• Often IgE blood test >500iu/l

It is not known whether these children have a severe form of asthma or something else such as Allergic Bronchopulmoary Aspergillosis. Children are likely to be fully investigated with bronchoscopy and CT scan. Possible treatments include some of the following:

• Long acting beta agonists (LABA) e.g. salmeterol or formoterol inhaler
• Symbicort – combination of steroid and formoterol (LABA) inhaler
• High dose steroid inhaler
• Salbutamol or other reliever for symptoms - up to 10 puffs for symptoms
• Montelukast – leukotriene antagonist tablet
• Prednisolone – 2mg per kg body weight per day for 2-3 weeks followed by dose reduction
• IV immunoglobulin – this treatment is given in hospital once a month, there are no known long term side effects although during infusion severe headache and flushing may rarely occur.
• Azithromycin – oral antibiotic treatment
• Subcutaneous terbutaline pump – see asthma guidance for information about this. It is rarely used.
• Methotrexate – this is an immunosuppressant drug given once a week. Blood monitoring is required weekly.
• Itraconazole or Voriconazole – oral antifungal treatment
• IV liposomal amphotericin (Ambisome) – intravenous antifungal treatment given daily over 4-6 weeks.

Allergic Bronchopulmonary Aspergillosis (ABPA) or Aspergillosis

Aspergillus is a fungus that can grow at body temperature in the lungs. The fungus produces toxic and allergenic products. It has the potential to cause serious lung damage with 0.6 to 11% of children with cystic fibrosis affected. The mechanism of lung damage is severe allergic inflammation caused by the presence of Aspergillus and its products in the lungs. The inflammatory response related to the allergy to fungal products is much greater than what would be seen if the lungs were simply infected with the fungus. This is called Allergic Bronchopulmonary Aspergillosis (ABPA) or Aspergillosis.

Symptoms of aspergillosis include:

• Increased wheezing
• High temperature
• Tiredness
• Thick sputum with brown or black casts

It is treated with:

• Oral prednisolone (steroid) tablets
• Intravenous methylprednisolone
• Antifungal drugs such as Itraconazole or Voriconazole which are taken by mouth and or Amphotericin (nebulised antifungal)
• rhDNAse has also been used

See also the treatment of severe small airways disease some of which is caused by aspergillosis.

Unusual organisms that colonise sputum

Children will have their sputum cultured regularly at each contact or if there is a change in symptoms to see if organisms are present. This may be referred to in the evidence as positive cultures or microbiology results. Terms used:

• Colonisation – the organism is present in sputum and is not causing symptoms. Colonisation is often treated with antibiotics to prevent infection.
• Infection – loose term, may be used interchangeably with colonisation – i.e. the organism is present in the sputum (colonisation) or may be used to indicate that the organism is causing chest symptoms and the child is unwell.

Often both colonisation and infection will require treatment, some of the organisms, once present, indicate ongoing risk of infection with them and lifelong antibiotics are prescribed. Often these are oral or nebulised and they can easily be given at home. Use of daily oral and or nebulised antibiotics reduces the risk of acute infections and the need for repeated course of IV antibiotics. IV antibiotics tend to be given as a short course, usually 2 weeks. The treatment can be commenced in hospital for the first couple of days and completed at home; some have the first dose in hospital once the line is inserted and the remainder done at home. This all depends on their presentation/well being/ parents willingness and ability to take on the responsibility. Prolonged IV antibiotic treatment at home is very unusual unless there is severe functional restriction or infection with Mycobacterium abscessus.

Below is a list of organisms that may be found on sputum culture, their significance and their likely treatments:

Staphylococcus aureus - Common organism – treated by increasing dose of oral antibiotics. May require intravenous antibiotics

Haemophilus influenzae (H influenzae or Hib) - Common organism – treated by increasing dose of oral antibiotics. May require intravenous antibiotics

Pseudomonas aeruginosa (Pseudomonas or P aeruginosa)

Children may be chronically colonised or infected with this. Presence of this organism accelerates lung damage and it is always aggressively treated in an attempt to eradicate it. If treatment is successful and sputum is clear ‘re-eradication’ may be tried if it comes back, this means a further course of treatment.

Treatments include:

• Oral ciprofloxacillin and
• Nebulised TOBI (tobramycin an antibiotic), or
• Nebulised Colistin (polymixin E an antibiotic), or
• Nebulised Gentamicin and Colistin (antibiotics, gentamicin and polymixin E), or
• 2 week course of intravenous antibiotics

If pseudomonas is persistently present in sputum lifelong nebulised antibiotics are prescribed, these are:

• Gentamicin and Colistin (antibiotics, gentamicin and polymixin E), or
• Double dose Colistin

Multi-resistant gram negative organisms

Long term oral antibiotics are used to treat these. IV antibiotics may be used.

Stenotrophomonas maltophilia

May not be treated because it is expected to clear by itself or oral antibiotics if child is unwell.

Burkholderia cepacia (cepacia)

This is a potentially life threatening organism and will be aggressively treated with oral or IV antibiotics possibly at home and for extended periods. Children with this organism cannot be seen in the usual CF clinic and must maintain strict isolation from others with cystic fibrosis if admitted to hospital. This may have psychological implications for the child and their family.

Non-tuberculous mycobacteria (NTM)

These may or may not be treated. Mycobacterium abscessus, one type of NTM has a particularly bad prognosis and will require long term treatment with 3 or 4 antibiotics over at least a 12-18 month period. Nebulised amikacin or meropenem may be used.

Candida

Candida is the fungus that causes thrush; this can affect the mouth and is treated if it is causing symptoms.

MRSA (Methicillin resistant staphylococcus aureas)

May or may not be treated with oral and or IV antibiotics. But patients harbouring MRSA are seen separately or at the end of a CF clinic and must maintain strict isolation from others with cystic fibrosis if admitted to hospital. This may have psychological implications for the child and their family.

Haemoptysis (coughing up blood)

Small amounts of haemoptysis are common in chronic infective lung conditions. Large bleeds may occur in cystic fibrosis, these will always be treated in hospital. Children prone to recurrent large bleeds may be treated with oral tranexamic acid ( a drug that improves blood clotting) and in very severe cases embolisation (occlusion of the bleeding blood vessel) or surgical removal of the affected part of the lung may be carried out.

Pneumothorax

This is an acute condition where air from the lung escapes in to the space between the lung and the chest wall and the lung collapses. A Pneumothorax is a marker for severe lung disease in cystic fibrosis. Surgery may be used to prevent recurrence of Pneumothorax but this type of surgery can make subsequent lung transplant more complicated.

Nasal polyps

These may be annoying and block the nose but in most cases there are no symptoms. Nasal polyps can impact significantly on quality of life in some patients. Rhinitis (inflammation in the nose) that leads to polyps can be treated with:

• Steroid nasal spray or drops e.g. Flixonase or Nasonex
• Surgery – polyps can be removed but they often recur
• Oral steroids may be used in severe cases

Sinusitis

Almost all children with cystic fibrosis have sinusitis but only about 1% have symptoms. Chronic sinusitis is associated with polyps. Treatments include:

• Long term oral antibiotics e.g. for 3-6 weeks
• Surgery – drainage procedures may rarely be used to prevent the build of mucus in the sinuses

Home oxygen

Initially this is almost always used only at night. It is given when overnight oxygen monitoring shows that oxygen saturation levels are less then 90% for more than 5% of the time. There may be associated symptoms including waking with a headache and tiredness. Oxygen cylinders are not used, oxygen concentrators are used which concentrate oxygen from room air so multiple deliveries of oxygen tanks to the home are not required. The concentrator will be maintained under contract during this period. Portable oxygen is used to enable some activities of daily living.

Lung transplant

Obviously lung transplant is only carried out in end stage respiratory failure. Only a small number of children reach this stage in childhood. Only 6 children received lung transplants in the year to March 2009 and not all of these will have been for cystic fibrosis. Not every child on the transplant list gets a transplant and having new lungs is not a cure for the chronic chest problems associated with cystic fibrosis. After lung transplant many of the problems associated with impaired oxygenation such as fatigue, inability to walk and weight loss will improve dramatically. Children will be able to walk and run around; will feel better and be able to go back to school. However pancreatic insufficiency continues and enzymes and vitamins need to be taken. Any other non-lung complications will remain the same – e.g. liver disease, diabetes. Diabetes can get worse after transplant. The new lungs will function very well at first but because of chronic colonisation of the sinuses with unusual and drug resistant bacteria infection and deterioration of the new lungs often occurs. A chronic process called obliterative bronchiolitis occurs which results in a progressive decline in lung function. The regimen of physiotherapy often needs to continue and there may be gradual deterioration of the new lungs despite this. In addition immunosuppressant drugs to suppress transplant rejection need to be taken every day, these have side effects of their own. One report of survival after lung transplant reported:

• 1 year survival 86%
• 3 year survival 65%
• 5 year survival 50%

Lung transplant is not a cure for cystic fibrosis but can significantly improve mobility needs.