Introduction
The widespread presence of CFTR protein throughout the body (and it’s absence in CF) explains why CF is a multisystem condition affecting many organs. The two major systems affected, however, are the respiratory and gastrointestinal systems. We will look at each of the systems in turn on the next four pages. A summary of complications is provided below.
Respiratory
- Pulmonary exacerbation
- Pulmonary infection
- Bronchiectasis
- Respiratory failure
- Pneumothorax
- Haemoptysis
- Allergic Bronchopulmonary Aspergillosis (ABPA)
- Asthma
- Sinusitis, nasal polyposis
Gastrointestinal
- Constipation
- Distal Intestinal Obstruction Syndrome (DIOS)
- Intussusception
- Pancreatitis (in pancreatic sufficient patients)
- Gastro Oesophageal Reflux Disease (GORD)
- Gastritis, peptic ulcer disease
- Biliary fibrosis, cirrhosis
- Gallstones
- Portal Hypertension, variceal bleeding
- Appendiceal disease
- GI cancer
- Colonic strictures, fibrosing colonopathy
- Rectal prolapse
- Clostridium difficile colitis
Musculoskeletal
- Osteoporosis
- Pathological fractures e.g. vertebrae, and ribs from coughing
- Arthropathy
Other
- Renal calculi (incidence increases with age and affects 1 in 20 adults)
- Renal failure—can be drug-related pre- and post-transplant
- CF Related Diabetes (CFRD)
- CF related arthritis
- Port (Totally Implantable Venous Access Device) problems
- 98% Male infertility, reduced female fertility
- Delayed puberty