Background

Copper is an essential micronutrient, vital for various enzymatic functions. It is generally absorbed from stomach and duodenum with normal daily requirement of 0.2-0.5mg in children. Malnutrition and short bowel syndrome are known risk factors for copper deficiency, manifesting as myelodysplasia and neurological disorders.

Objectives

To raise awareness regarding copper deficiency in children with transpyloric feeds and highlight management challenges in paediatric intensive care.

Methods

A 2 years old boy with X-linked myotubular myopathy and severe gastro-oesophageal reflux had three admissions to PICU within 2 weeks due to respiratory distress and persistent tachycardia requiring invasive ventilation. There were no obvious reasons for the deterioration apart from severe anaemia Hb 69g/dL and severe copper deficiency with levels 0.4mmol/L (Normal values: 11-22).

Results

Intravenous copper replacement 0.2mg/day for 14 days, resulted in transient improvement in Copper levels to 14.7mmol/L, dropping to 5.9mmol/L once treatment was stopped and IV replacement was repeated. His tachycardia and respiratory deterioration had a temporal correlation with the copper deficiency and improved after supplementation. Copper replacement in this case was very challenging due to difficult intravenous access, risk of anaesthesia and inability to use gastric route due to severe reflux and risk of aspiration.

After multiple discussions, he was commenced on high dose Copper replacement via jejunal route i.e. 1.67mg/day that included his usual feeds + supplements ‘Seravit’. His Copper levels gradually improved to 23 and supplementation was adjusted.

Conclusion

Copper deficiency should be considered in patients on long-term trans-pyloric feeds and high dose jejunal replacements could be attempted in complex cases.