Case Study: Copper Deficiency in a Young Patient With Short Bowel Syndrome

By Andrea Adler, RD and Kadakkal Radhakrishnan, MD

Many nutrients are considered essential for newborns and throughout life. Copper is one of those essential nutrients. Copper is needed for enzymes in the body that affect many metabolic processes. Some of the metabolic processes include oxidation of ferrous iron to bone marrow for hemoglobin synthesis, collagen and elastin in connective tissue, the conversion of dopamine to norepinephrine in the brain, free radical scavengers, serotonin synthesis and melanin synthesis.

Copper is mainly absorbed in the small intestine and is excreted mostly in bile. While copper toxicity can cause abdominal pain, kidney and liver failure and can lead to death, copper deficiency can lead to anemia, leukopenia and bone abnormalities, including osteoporosis and, rarely, neuromuscular weakness.

Patients with short bowel syndrome require prolonged parenteral nutrition (PN) support as their GI tract may not function appropriately to optimize growth, absorption and hydration, which may then lead to pediatric intestinal failure. PN includes macronutrients (dextrose, protein and fat) in addition to electrolytes, vitamins, minerals and trace elements.

Navigating parenteral nutrition shortages

As healthcare providers, we all have run into medication, supplies or nutrition shortages. The American Society for Parenteral and Enteral Nutrition (ASPEN) has recommendations on how to optimize and prioritize parenteral nutrition shortages. PN shortages have occurred since the 1980s, however, since 2010, almost every component of PN has had a shortage at least once. Specific recommendations for trace element shortages were created and approved by the ASPEN Clinical Practice Committee and Board of Directors on July 20, 2016.

A case-based scenario

A patient was transferred to Cleveland Clinic Children’s Hospital Pediatric Intestinal Failure and Rehabilitation program around 4 years of age with complications related to short bowel syndrome. Though the patient had a significantly long past medical history, only information pertinent to the case study is discussed here.

The patient had a past medical history, including esophageal atresia with stricture following reconnection, microgastria (stomach capacity of 40 ml), which required a G-tube placement at birth, and development of necrotizing enterocolitis (NEC), which required a partial jejunal and ileal resection with temporary diverting ileostomy at 1 month of age. Subsequent reconnection left approximately 35 cm of small bowel with ileocecal valve to the entire colon (except for the transverse and descending colon, which was used later for the colonic interposition).

He then underwent gastro-cutaneous fistula at prior G tube site, esophageal reconstruction with substernal colonic interposition (using transverse and descending colon), pyloroplasty, and replacement of G-tube. Around 2 ½-years-old, the patient had the G-tube changed to a G-J tube.

The patient had required PN support since birth.

Uncovering the cause of severe anemia

At the time of transfer, the patient was receiving continuous feeds of semi-elemental pediatric formula at 57ml/kg via G-J-tube in addition to PN support. All the nutrients in PN from the outside hospital were thoroughly reviewed including trace elements. The patient’s PN order on transfer contained 10 mcg Cu/kg, which is within ASPEN standards of 5-20 mcg/kg for patients’ weight.

Due to national copper shortages, home care didn’t have Cu as an additive to the PN. The patient was admitted at the age of 4 years and 7 months due to severe anemia requiring two blood transfusions and multiple outpatient blood transfusions. He had borderline low iron levels, not severe enough to explain his severe anemia. After investigating the cause of anemia, the patient’s home PN was discovered to be without copper for seven months. Copper was initiated in the PN at a dosage of 30 mcg/kg, which was above ASPEN standards for weight, however, due to prolonged omission from PN because of the national shortage and the patient’s admission for anemia, a higher dosage was initiated.

The patient was discharged home at 4 years and 8 months with a dosage of 30 mcg Cu/kg in the PN order. Patient had improved energy level after 24 hours suggesting replenishment of his mitochondrial function and increased energy production. His hemoglobin continued to improve and he did not require further transfusions. His Cu levels were monitored serially outpatient and eventually normalized, as noted in the table below.

The take-home message

1. Home PN patients require lab monitoring, including trace elements and vitamins.
2. Knowing the signs and symptoms of micronutrient deficiencies helps early diagnosis and improve care of patients with intestinal failure.
3. When a nutrient shortage has resolved, go back to the previous standard of care (i.e. add nutrient back into the PN mixture immediately).

About the authors. Dr. Radhakrishnan is Director of Nutrition and Intestinal Rehabilitation at Cleveland Clinic Children’s. Ms. Adler is an advanced practice pediatric dietitian at Cleveland Clinic Children’s with interest in intestinal failure.