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Section 3

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The PICU stopped the request for newborn screening (NBS) values and continued to give the patient the harmful formula, Nutramigen.

Newborn Screening

On a baby’s NBS - an acylcarnitine profile using blood spots on Guthrie card, there are two reports: a regular report and a report with analyte values. When a patient shows symptoms, doctors should interpret the values using acylcarnitine reference ranges instead of checking a regular report, which only screens a few diseases. (WA doesn’t screen CPT-II deficiency.)

The two requests showed that SCH knew that a NBS can be used for diagnosis (PDF).

Unfortunately, the 1st NBS they received was a regular report. PICU asked again and received the same report.

1. On the night of Apr. 11, the father heard and saw that a male PICU staff member wrote down a request by Dr. Laura Chen, a resident doctor, asking for a NBS with values, not a regular report, to be requested from the DOH the first thing next morning. This request was evidently stopped as the DOH did not receive any requests after Apr. 11. The patient was then put on fasting by resident doctors based on a normal regular report when his attending physician on Apr 10, Lincoln S. Smith, MD was absent.

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2. After the SCH PICU stopped the NBS request and manipulated the acylcarnitine lab, they continued to give the patient Nutramigen formula, which derives 50% of its total calories from long chain triglycerides (LCT). However, the WA DOH requires <10% for infants with FAOD.

It resulted in increasing amount of ammonia in his plasma, which is toxic. The nurse who manipulated the blood specimen collected bags of dark brown urine -- which indicated that his kidney was failing. She said everybody’s urine would turn that color from oxidation.

3. The harm caused by the formula was stopped by the parents around 11:45pm on Apr 13 when they practically commanded the PICU to replace Nutramigen with IV glucose after Dr. Zimmerman did not show up for 7 hours to address the formula issue. (PICU has up to 60 hours over weekends, not 12 overnight, to harm a patient as shown below.)

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This action against the doctor’s order was not documented by Dr. Zimmerman. He did not speak to the family during his 3-day service. The patient’s family calls Jerry J Zimmerman, MD, PhD the 1st murderer in Seattle Children Hospital and the University of Washington.

The next morning, Apr 14, the parents asked SCH PICU to investigate FAOD before considering any other diseases and shared with them one paper. The parents also shared it with the new metabolic doctor, Sihoun Hahn, MD, PhD, who documented this (PDF). The PICU asked the parents to wait for Simon P. Horslen, MD.

Uncertain about the patient’s condition, the parents took his GI doctor’s advice, giving him EleCare, a formula that derives 30% of its total calories from LCT. The patient’s ammonia slowly climbed to 128, ~4x the normal maximum (33 mcmol/L), before a liver biopsy on Apr 18th.

Dr. Horslen, the ALF expert of SCH, promised that the biopsy would give an answer. He assured the parents that SCH’s most experienced pathologist would review the biopsy on Apr 15.

The CPT-II deficiency, based on the NBS marker C16, was confirmed by CPT-II enzyme activity values. Opinions of multiple professionals will be presented together.