A lady 52 yrs old bed ridden and on o2 for last 30 yrs and was getting treatment for non responding asthma by various doctors and health centres.she was in severe respiratory distress and Needed o2 3l/ min to maintain spo2 above 90%.she was on steroids largedose and various brochodilators for several yrs still didn't respond to any such therapy.
Chest xray revealed bronchopneumonia and sputum grew pseudomonas and staph.chest had crackles and use to expectorate copious viscid sputum.I strongly doubted cystic fibrosis and did sweat chloride test which was above 60.trysinogen test too came+ve. so mutational gene study was sent.delta 508 mutation came +ve and thus cystic fibrosis was proved and then accordingly management begun by proper antibiotics along with prophylaxis by bronchodiltors.Colistin sulfate and DNASE and acetylcystiene nebulizations and other supportive care.for last 3 yrs she is much relieved with infrequent exacerbations and mostly is managed without o2.
Her mother and one sister had similar problem and expired at very young age.so when one gets such family history of non responding asthma one should doubt about cystic fibrosis and other chronic lung diseases like interstitial lung disease.
Dr s kumar
Child specialist.rainbow child hospital.
Beside NIFFT gate.hatia.ranchi.jharkhand.
7250725044. 7004780897.
Chest xray revealed bronchopneumonia and sputum grew pseudomonas and staph.chest had crackles and use to expectorate copious viscid sputum.I strongly doubted cystic fibrosis and did sweat chloride test which was above 60.trysinogen test too came+ve. so mutational gene study was sent.delta 508 mutation came +ve and thus cystic fibrosis was proved and then accordingly management begun by proper antibiotics along with prophylaxis by bronchodiltors.Colistin sulfate and DNASE and acetylcystiene nebulizations and other supportive care.for last 3 yrs she is much relieved with infrequent exacerbations and mostly is managed without o2.
Her mother and one sister had similar problem and expired at very young age.so when one gets such family history of non responding asthma one should doubt about cystic fibrosis and other chronic lung diseases like interstitial lung disease.
Dr s kumar
Child specialist.rainbow child hospital.
Beside NIFFT gate.hatia.ranchi.jharkhand.
7250725044. 7004780897.
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