The many areas that may be affected by cystic fibrosis require a range of treatments. The NHS Direct site describes the aim of treatment to ease the symptoms and to make the condition easier to live with. Set out below are the main areas of the body that are affected and the treatments used to deal with them
The Lungs
Bronchodilator drugs help to open the airways in the lungs by relaxing the muscles in the lungs thus allowing the airways to widen and clearing thick secretions. This is achieved by accelerating the motion of the tiny hairs (cilia) that line the walls of the airways and help "sweep" the mucus away. Bronchodilators can be delivered by either inhaler or nebuliser.
Bronchodilators are most effectivie with people who have inflamed and narrow airways.
Side effects of bronchodilators can include anxiety, muslce tremors, increased heart rate.
There are two types of bronchodliators usually used for cystic fibrosis, beta-2 agonists and anticholinergics both of which are available in short and long-acting versions.
There is conflicting evidence with regard to the effectiveness of bronchodilators for people with cystic fibrosis. It would appear that beta-2 agonists are more effective than anticholinergics but it is important to note that is is based on fairly limited data.
Antibiotics are used to tackle infection in the lungs. Doctors will prescribe the type of antibiotic that is best able to deal with a particular kind of infection. he infection is identified by the analysis of sputum which is coughed up by the lungs.
Antibiotics are given via a nebuliser or intravenously if the infection is more severe.
For people with cystic fibrosis antibiotics are essential in minimising damage to the lungs and thus prolonging life but they do have side effects. The most common side effects are nausea and vomiting, mild diarrhoea, skin rashes and kidney problems. Hearing can also be affected by long term use which can also lead lead to fungal infections in the mouth,throat and lungs.
Long term use may also lead to the development of antibiotic-resistant bacteria in the lungs.
In the case of severe infection, people with cystic fibrosis may need to take two different types of antibiotics at the same time.
Corticosteroids are given in either tablet or inhaled form to reduce inflammation in the lungs. Corticosteroids mimic the effect of cortisol which is a hormone produced in the adrenal glands to help the body reduce inflammation.
Corticosteriods are not recommended for long term use as they have the following side effects-
Inhaled corticosteroids are considered to have a lesser risk of side effects than those given in tablet form.
DNase is inhaled and is used to reduce the stickiness of mucus in the lungs making it easier to cough up.
When bacteria builds up in the airways, these are attacked by white blood cells which release DNA into the mucus making it even thicker. DNase is an enzyme that fragments DNA thus reducing the viscosity of the mucus.
A commonly prescribed for of DNase is Pulmozyme. The manufacturer's patient information leaflet states that its safety has not been established in pregnant women and that it is not yet known whether Pulmozyme passes into breast milk. the usual dose is one ampoule per day although some adults over 21 may inhale two ampoules per day.
Pulmozyme should not be prescribed to children under the age of 5.
Side effects include irritation of the airways and sore throat. Less common side effects are conjunctivitis, indigestion, fever, shortness of breath, runny nose and sneezing, skin rashes and itchiness.
Lung transplantaion is considered when there is respiratory failure and all other treatments are no longer effective. A lung transplant (both lungs are replaced) is a complex surgical procedure and there are risks of infection and rejection but for severely ill patients a transplant offers the possibility of a much improved quality of life. An American study published in 2004 estimated that a lung transplant improved the life expectancy of people with CF by an average of 4.5 years.
There has been some recent debate in America as to whether those people who have a bacteria called Burkholderia cenocepacia in their lungs should be barred from receiving transplants. This is because this infection is related a poor post-operative survival rate.
The digestive system.
Pancreatic enzyme supplements are required by 95% of all cystic fibrosis patients who cannot adequately absorb and digest their own food. Enzymes are best taken with food, just before meals and snacks . Too high a dosage can result in constipation. The required dose should be calculated with the help of a dietitian according to fat intake.
Because the dose is individually calculated it should not be changed without prior discussion with your doctor.
Enzymes must be taken with all meals and snacks. The Cystic Fibrosis Foundation lists the following as foods that don't need enzymes- fruit, juice, juice drinks, soft drinks, sports drinks, infant rehydrating formulas, tea or coffee without cream, hard sweets, fruit snacks, jelly beans, chewing gum, freezer pops, flavoured ice.
The skeleton.
Bisphosphonates are prescribed to treat osteoporosis, a weakening of the bones that can be a side effect of cystic fibrosis. They increase bone mineral density and thus reduce the risk of fractures. A Cochrane Review found that bisphosphonates can cause severe bone pain in people who are not taking corticosteroids.
Oral biphosphonates should be taken in the morning on an empty stomach thirty minutes before breakfast with at least 240ml of water. This is to ensure that the medication is properly absorbed. Biphosphonates can also be given intravenously.
Biphosphonates are not licensed for use in children and are contra-indicated in pregnancy.
The Cystic Fibrosis Trust's consensus document states that biphosphonates may be beneficial in children with a history of fragility structure and those listed for/post transplantation.
Vitamin D supplementation may also be used to help maintain bone health.
The pancreas.
Over time, damage to the islets of Langerhans can halt the production of insulin which leads to cystic fibrosis-related diabetes (CFRD). According to the Cystic Fibrosis Foundation CFRD is found in 35% of adults aged between 20 and 29 and 43% of those over 30.
CFRD is a distinct type of diabetes with features of both Type 1 and Type 2 diabetes.
Early diagnosis of CFRD is important in maintaining health status. Delayed diagnosis can lead to a decline in lung function.
In order to maintain normal blood glucose levels at a normal level people with CFRD need to inject themselves with insulin every day.
Because diet has a direct effect on blood glucose levels, people with CFRD will be given nutritional advice according to their own circumstances and preferences. It is estimated that people with CFRD need 120-150% of the normal calorific requirement.
The Cystic Fibrosis Trust's (CFT) consensus document on treating CFRD stipulates that a diet should be high in energy, fat and with planned use of carbohydrates. The document also states that "conflicts between dietary therapy of cystic fibrosis and diabetes shoulld be resolved in favour of the cystic fibrosis diet and the insulin regimen adjusted to the diet rather than the diet to the insulin." The individual diet plan should be put together by a dietitian with specific expertise in Cystic Fibrosis.
It is recognised that children and adolescents may find it difficult to adhere to the required insulin and dietary regimes. In these cases the CFT consensus document recomends a simple regime rather than a complex one, accepting that a pragmatic approach should recognise that in some cases treatment and blood testing will be erratic.
Patient education and self-monitoring are viewed as very important elements in the treatment of CFRD. People with CFRD will need to be taught how to inject themselves with insulin and how to monitor their own blood glucose levels.
People with CFRD should not have more than two or three units of alcohol at any one time and should never drink alcohol on an empty stomach.
Appropriate psychological support should be made available for people with CFRD.
Physiotherapy
Chest physiotherapy is really important in helping to clear the thick mucus from the lungs. Physiotherapy should start as soon as cystic fibrosis is diagnosed.
Parents are taught how to administer this to their child. From the age of nine most children with cystic fibrosis should be able to start doing part of their physiotherapy for themselves. Most teenagers achieve complete independence and only need help if they have increased secretions.
The duration of physiotherapy sessions varies according to the amount of mucus in the lungs. If the lungs a relatively free of mucus then the session should only last for 10 or 15 minutes, if there is a lot of mucus then a session can last up to an hour. Most people have two sessions per day but this can rise to four if the need rquires.