![]() ![]() Chrispin-Norman score: is used to provide a summative assessment of structural lung changes in patients.Brasfield scoring system: can be used to score disease severity on plain film.pulmonary arterial enlargement due to pulmonary arterial hypertension in patients with long-standing disease.Plain radiographĬhest radiographs are insensitive to the early changes of cystic fibrosis, with changes seen on HRCT in 65% of patients with CF and normal chest radiographs 6. Other features to be sought include hyperinflation, regions of consolidation, lymph node enlargement, pneumothorax and pulmonary arterial hypertension. The intervening lung is often densely fibrotic and retracted 3.Īlthough the entire lung is affected, there is a predilection for: These begin as cylindrical and progress through varicoid to cystic forms. The cardinal finding of later stages of cystic fibrosis is the presence of thick-walled bronchiectasis. The bronchial submucosal arteries then become hypertrophied and, given the chronic inflammation, also more fragile, making them more susceptible to bleeding in the airways 9. These chronic bronchial injuries also lead to bronchial angiogenesis and vascular hypertrophy of the bronchial arteries, sometimes with the recruitment of additional aortobronchial collaterals. Over time, these weaken the bronchial walls, leading to bronchiectasis 3. Haemophilus influenzae: especially in the first 6 months of lifeĪs a result of repeated and chronic infections, there is a marked increase in the number of polymorphonuclear (PMN) leukocytes and associated inflammatory agents, including elastase and collagenase.Staphylococcus aureus: especially in the first 6 months of life 1.The organisms most responsible for pulmonary infections are: The result is iso-osmotic, but low volume, secretions, which tend to dry out, or be thick as they still contain all the other constituents.Ībnormal CFTR function has other effects 1,3, e.g: In CF patients, too little chloride is pumped out, too much sodium is reabsorbed resulting in osmotic re-absorption of water from the lumen. Therefore, under normal circumstances, salt and chloride remain in the lumen and keep water there osmotically. In the lung, the cystic fibrosis transmembrane regulator (CFTR) is a protein responsible for efflux of chloride and inhibition of the sodium channel's activity which controls the influx of sodium. Later in the disease, larger volume hemoptysis, which may be life-threatening, as well as pneumothoraces become more common 2. Patients have a chronic cough and expectorate copious quantities of sputum, frequently blood stained and containing mucous plugs 2,7. With that in mind we have compiled a short list of conditions which share co-morbidities with CF and important overlapping risks.Clinical presentation is with the expected recurrent bacterial infections and hemoptysis. One of the more troubling complications of CF in the lungs is that respiratory complications can be exacerbated by the constant inhalation of bile that cannot be cleared from the lungs because of the initial mucosal build-up, resulting in a kind of cyclical damage. This not only helps the CF community to stay abreast of important discoveries and emerging technologies, it also helps us to expand our advocacy potential by forging relationships with those who experience similar obstacles in life. However, we can also benefit by placing CF respiratory complications in a wider context to understand which conditions can exacerbate CF symptoms and which disease groups exhibit meaningful similarities with CF. Individuals managing CF are therefore not only more vulnerable to infections they contract but also more likely to contract them initially.Ĭystic Fibrosis Australia (CFA) fights hard to make sure that the effects of CF on the lungs are well understood and we advocate for universal access to the life saving medications that help relieve this strain on one of the body’s most crucial organ systems. Systems of the body affected by CF on a cellular level will also face comorbidities related to inflammation because the tissue will be more prone to aggravation in the first place.Īt the same time, blockages in the lungs can pose a risk for infection because stagnant material is unable to be cleared out. ![]() Pneumothorax and other severe complications can also result from long term strain on the lungs in people with CF. Lungs that are clogged with mucus will also exhibit a decreased blood flow, leading to increased blood pressure and possibly pulmonary hypertension. This not only clogs airways but can also result in bronchitis, leading to wheezing, shortness of breath and coughing. For individuals living with cystic fibrosis (CF), airflow in the lungs can be hindered by the production of thick, sticky mucus. ![]()
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