Cystic Fibrosis varies widely in individuals from just requiring clinic follow-up to recurrent chest infections or gastro-intestinal problems needing extensive treatment and, or frequent hospitalisation.
Cystic Fibrosis is a progressive condition, with careful management children generally have good health, with increasing symptoms showing as they progress into adulthood. However, some children may have advanced chest disease whilst some adults remain very well.
Chest infections often require a two week course of intravenous antibiotics through a cannula placed into the vein or via an implanted device in the chest wall (portacath) which gives venous access. Antibiotics can also be given through a nebuliser, which may be long term. Other oral, nebulised and inhaled drugs help reduce inflammation in the lungs, ‘open up’ the airways and make it easier to cough up very sticky secretions from the lungs.
Repeated infections over time can cause the lungs to become scarred, which in turn reduces the normal function of the lungs. Patients may suffer from breathlessness and loss of energy and some may need oxygen during an infection or on a longer term basis. Some may continue to deteriorate to the point where lung transplantation is considered.
Other complications may include CF related diabetes, liver disease, gall stones, sinus problems, nasal polyps, blockage of the gut and joint problems. However it must be emphasised that not every patient will experience all or any of these complications.
Most women with Cystic Fibrosis are able to have children, with the majority of males being infertile but with a technique known as sperm aspiration, plus IVF for their partner, they may be able to become fathers.