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Practice A Patient’s Journey

The missing vital sign

BMJ 2013; 347 doi: https://doi.org/10.1136/bmj.f4163 (Published 05 July 2013) Cite this as: BMJ 2013;347:f4163
  1. Christina Mangurian, assistant professor of clinical psychiatry, director1,
  2. Morton J Cowan, professor of paediatrics, chief2
  1. 1University of California, San Francisco, UCSF Public Psychiatry Fellowship, San Francisco General Hospital Department of Psychiatry, San Francisco, CA 94110, USA
  2. 2University of California San Francisco, Allergy Immunology and Blood and Marrow Transplant Division, UCSF Benioff Children’s Hospital, San Francisco, CA 94143, USA
  1. Correspondence to: C Mangurian Christina.Mangurian{at}ucsf.edu
  • Accepted 30 March 2013

The author catalogues the challenges to family, friends, and herself of managing her 10 month old son’s long hospital stay as he received a transplant to cure Wiskott-Aldrich syndrome

The paediatric bone marrow transplant team entered my 10 month old son’s isolation room for a second time one morning. It was supposed to be Anderson’s “day of rest” between chemotherapy and his transplant. As a physician, I knew something was wrong—busy doctors do not do rounds twice. They had returned to tell me that the umbilical cord blood needed to cure my son of his rare condition, Wiskott-Aldrich syndrome, had been thawed a day early. The doctor who had just finished his fellowship said that the early thawing would not affect Anderson. He then went on to blame the lab, saying that the “mistake probably happened because we don’t really do many cord blood transplants here.” The nurse practitioner told me that she thought it was likely a communication problem and promised that “this will never happen again.”

At 4 months, Anderson had been diagnosed with the most severe form of Wiskott-Aldrich syndrome, a genetically inherited, immunodeficiency disease affecting 1/250 000 men. Children with severe Wiskott-Aldrich syndrome die early of infection or haemorrhage if not treated with a bone marrow transplant (BMT). Children receiving a BMT from an unrelated donor or via cord blood donation can be hospitalised for up to three months—all in an isolation room because of the risk of infection. My family had already endured a three week hospitalisation when my son was only 1 month old, and I knew that I had to develop a plan to take care of not only Anderson, but also my 4 year old daughter, my husband, and myself, physically and emotionally, during this marathon.

In stereotypical doctor fashion, I do not …

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