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  • Strategies to reduce line infections in a small child with homozygous familial hypercholesterolaemia who cannot yet receive LDL apheresis

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Learning from errors
CASE REPORT
Strategies to reduce line infections in a small child with homozygous familial hypercholesterolaemia who cannot yet receive LDL apheresis
  1. Misan Lee1,
  2. Janet Barr1,
  3. Stewart Kribs2,
  4. Guido Filler1
  1. 1Department of Paediatrics, University of Western Ontario, Ontario, Canada
  2. 2Department of Radiology, University of Western Ontario, Ontario, Canada
  1. Correspondence to Dr Guido Filler, guido.filler{at}lhsc.on.ca

Summary

Patients with homozygous familial hypercholesterolaemia are optimally treated with low-density lipoprotein apheresis. Young patients who do not meet a weight threshold (25 kg) receive regular plasmapheresis. This approach may remove excessive immunoglobulins and vascular access set-up can be challenging. We report the case of a 4 year-old child who exhibited repeated septic infections (5 in 6 months) and had recurrent access issues before two interventions were implemented: (1) the percutaneous central venous line was modified to two implanted paediatric ports, and (2) the patient started receiving two bags of Octaplasma at the end of each plasmapheresis treatment to account for the excessive loss of immunoglobulins. For the paediatric plasmapheresis access port, a 19-gauge Huber needle had to be used for the arterial port to prevent the collapse of the extension. These two simple changes have left the patient infection-free for 9 months.

  • Lipid Disorders
  • Healthcare Improvement And Patient Safety
  • Congenital Disorders
  • Paediatrics

https://doi.org/10.1136/bcr-2017-219538

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    Footnotes

    • Contributors ML: acquired the data and drafted the article. SK: interpreted the data and critically revised the article for important intellectual content. JB: acquired the data and critically revised the article for important intellectual content. GF: conceived and designed the case report, interpreted the data and critically revised the article. All authors provided final approval of the version published, and all authors agree to be accountable for the article and will ensure that all questions regarding the accuracy or integrity of the article will have been investigated and resolved.

    • Competing interests None declared.

    • Patient consent Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.