Here’s some more good news on the treatment of neonatal hemochromatosis (NH).

Over at the Macclesfield Hospital in Cheshire, England, little William celebrated his first birthday back in February this year. William’s parents lost their daughter, Elizabeth, to NH even though she received an emergency liver transplant. Like Mary, William’s mum underwent the IVIg treatment through her doctors at the hospital and with the help of Dr Whitington. William’s now doing fine thanks to the determination of his parents and the success of the treatment. Click here to read the complete article from the Wilmslow Express.

A similarly successful outcome was also published online in the Journal Prenatal Diagnosis last month. Venkat-Raman et al (see link below) report on the successful maternal intravenous immunoglobulin treatment of a woman with a previous history of NH. In this case the woman had two successive previous pregnancies where the babies had died from NH, which causes severe liver damage and excessive iron deposition in other cells in the body. Like Mary’s experience, the woman started the IVIg treatment for her latest pregnancy at week 18 and continued until an elective caesarean section at 38 weeks. The baby boy was born with elevated ferritin levels (the protein associated with iron storage) and some signs of decreased liver and coagulation function. However, all these indicators returned to normal within a couple of months and the child made an “excellent recovery”. It’s interesting that the mother received the same ‘brand’ (Sandoglobulin) of immunoglobulin as Mary and the dosage rates (6% solution at a maximum rate of 150mL/h) were also the same, hence the poor mum would have been receiving the infusion for at least 9h!. We have heard from mums where the rates of infusion are much higher hence the time taken for infusions can be as short as 3-4h.

Venkat-Raman et al concluded that the recurrence risk of NH is high but that the immunoglobulin treatment “appears to alter the course of the disease with better infant survival”.

References

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