Pulmonology focuses on diagnosing and treating lung and airway conditions such as asthma, COPD, and pneumonia, as well as overall respiratory health.
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Managing cystic fibrosis takes a lot of effort and is a lifelong process. The main goal is to prevent damage before it happens, not just treat problems as they arise. Treatment can take 2 to 4 hours each day. At Liv Hospital, our team helps patients fit these treatments into their daily lives to get the best results. The main areas of care are clearing the airways, supporting nutrition, controlling infections, and treating the genetic problem.
Mechanical clearance of mucus is essential to prevent infection. ACTs are performed daily, typically twice a day when well and up to four times a day when sick.
To make airway clearance effective, inhaled medications are used to alter the properties of the mucus. These are typically taken via nebulizer before or during ACTs.
CFTR modulators are small-molecule drugs that target the root cause of the disease, the defective protein, rather than just the symptoms. They have transformed the landscape of CF care.
Controlling the bacterial load is critical.
Good nutrition is directly correlated with better lung function.
For patients with end-stage lung disease (FEV1 < 30%, rapid decline, or massive hemoptysis) who have exhausted medical options, double lung transplantation is the final therapeutic consideration. While it exchanges the chronic lung disease for the risks of immunosuppression and rejection, it can offer a dramatic improvement in quality of life and survival. Post-transplant care is complex, but the CF defect is removed from the respiratory system.
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They are not a cure because if you stop taking them, the protein dysfunction returns. However, they are highly effective functional treatments that treat the underlying cause.
Any food containing fat or protein requires enzymes for digestion. If you skip enzymes with snacks, you will not absorb those calories and may experience stomach pain and bloating.
No, airway clearance is preventative. Even when you feel well, mucus is being produced. Clearing it daily prevents it from building up and becoming infected.
Cycling (e.g., one month on, one month off) helps prevent the bacteria from developing resistance to the drug while still keeping the bacterial population suppressed.
Hypertonic saline draws water back into the airway surface liquid, rehydrating the dried-out mucus so the cilia can move it more effectively; it’s like watering a dry slide.
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