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There was a case of Spinal Muscular Atrophy SMA on my ward last week.
He was type 2 SMA, cos he had normal motor developmental milestones up until about twelve months. This was an interesting case, cos he had two other siblings with the same condition. Both parents are alive and well - it is an autosomal recessive inheritance. Patient is very intelligent and has normal social, language and behavioural development.
The boy presented with cough, hematemesis and chest pain. ( was not pleuritic) On examination, The patient was cachectic, had a scapoid abdomen, and bilateral talipes equinus varus. he was flaring his alae nasi, there was subcostal and intercostal recession ( obviously in respiratory distress, clinically) he was tachypnoeic. On auscultation, breath sounds were bronchial in the lower lobes. bilateral crepitations were heard on the mid and lower zones.
He was admitted, but unfortunately the houseman on call failed to pick up the respiratory infection, cos he reported in the patients notes that the breath sounds were vesicular, no added sounds heard.
The patients respiratory embarrassment was thought to be due to terminal effects of his weak respiratory muscles. i guess.
In my view the patient should have been put on antibiotics, knowing very well that he was a case of SMA-II. Cos these patients usually die from respiratory infections and its complications. There should have been suction, cos the patient was drooling. he should have been placed on a ventilator. He should have been observed closely - assessing blood gas levels. All these were not done.
I feel so down, cos i failed to report my findings on chest examination to a resident or even my consultant. He could still have died, even if antibiotics had been started, cos they usually die from resp infections. But in this case he was not started on immediate antibiotic therapy.
I am disappointed in myself, cos i am part of the team as such i believe its all my fault.
reason for respiratory complications of SMA
1) WEAK RESP MUSCLES. this gives a poor cough reflex, as such patients aspirate secretions and cannot cough out irritants which are inhaled
He was type 2 SMA, cos he had normal motor developmental milestones up until about twelve months. This was an interesting case, cos he had two other siblings with the same condition. Both parents are alive and well - it is an autosomal recessive inheritance. Patient is very intelligent and has normal social, language and behavioural development.
The boy presented with cough, hematemesis and chest pain. ( was not pleuritic) On examination, The patient was cachectic, had a scapoid abdomen, and bilateral talipes equinus varus. he was flaring his alae nasi, there was subcostal and intercostal recession ( obviously in respiratory distress, clinically) he was tachypnoeic. On auscultation, breath sounds were bronchial in the lower lobes. bilateral crepitations were heard on the mid and lower zones.
He was admitted, but unfortunately the houseman on call failed to pick up the respiratory infection, cos he reported in the patients notes that the breath sounds were vesicular, no added sounds heard.
The patients respiratory embarrassment was thought to be due to terminal effects of his weak respiratory muscles. i guess.
In my view the patient should have been put on antibiotics, knowing very well that he was a case of SMA-II. Cos these patients usually die from respiratory infections and its complications. There should have been suction, cos the patient was drooling. he should have been placed on a ventilator. He should have been observed closely - assessing blood gas levels. All these were not done.
I feel so down, cos i failed to report my findings on chest examination to a resident or even my consultant. He could still have died, even if antibiotics had been started, cos they usually die from resp infections. But in this case he was not started on immediate antibiotic therapy.
I am disappointed in myself, cos i am part of the team as such i believe its all my fault.
reason for respiratory complications of SMA
1) WEAK RESP MUSCLES. this gives a poor cough reflex, as such patients aspirate secretions and cannot cough out irritants which are inhaled