Cystic fibrosis is a genetic metabolic disease with reduced DNase I concentration that cause a reduction in secretions in various organs. The airways display the worst symptoms. The mucus becomes so thick and sticky, that it causes recurrent bacterial infections. The body does not have the ability to remove this mucus.
Dornase alpha (which is rhDNase I) inhalations hydrolyze the proteins in bronchial mucus and makes the mucus more fluid so that it can be more easily removed.
Also called hyaline membrane disease. It is found in premature babies when the surfactant that covers a normal person's airways which are responsible for gas exchange are not present (this surfactant is produced just before birth and are therefore not present in a premature baby). The lungs can fall flat (atelectasis) which is fatal.
Treatment strategies:
Monitoring respiratory and circulatory function, is essential.
Oxygen ensures oxygenation (but do not use too long because can cause retinal damage and blindness)
Ventilation ensures positive pressure
Exogenous surfactants such as beractant or poractant alpha which provides surfactant so that gas exchange can take place an the lungs don't collapse.
Corticosteroids such as betamethasone administered systemically (orally) prophylactically to the mother before labour to induce the surfactant reproduction of the baby/initiate the baby's surfactant reproduction.
It provides oxygenation which makes up for the impeding gas exchange.
Retinal damage that cause blindness.
The respiratory center in the medulla is not yet fully developed to stimulate continuous breathing in neonates and premature babies. Apnoea together with bradycardia happens longer than 15 seconds and repeatedly. This cause hypoxia and neural damage.
Methylxanthines (theophylline and caffeine IV for weeks) stimulate the CNS.