Nursing issues delayed my son’s enzyme replacement infusion

A columnist struggles to find a nurse to administer her son's Pompe treatment

Keara Engle avatar

by Keara Engle |

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Every other week, my son Cayden, who’s almost 5, receives an enzyme replacement infusion as part of his treatment for infantile-onset Pompe disease. The infusion typically takes about six hours, and although we’re used to the routine, it does make for a very long day.

Cayden has been receiving these infusions since he was 1 month old. He’s only missed one when he was sick and developing a fever. To receive enzyme replacement therapy, a patient must be fever-free for 24 hours. Even in these situations, we make sure he receives his treatment as soon as possible.

Recently, there’s been a delay in his infusions. Cayden has been getting them at home since he was 18 months old and always has the same infusion nurse. She’s become family to us at this point.

Unfortunately, there was a recent death in her family, so she needed to take some vacation time. While this is completely deserved and understood, it put a halt to Cayden’s infusions.

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Finding a replacement

The nursing agency we use tried their hardest to find another nurse who could step in and administer the infusion, but a widespread nursing shortage made it difficult. The agency eventually had to reach out to the pharmacy that supplies the medication, and they were able to find us a nurse.

However, her schedule was jampacked, resulting in even more of a delay. She was finally able to come about three weeks after Cayden’s most recent infusion. Although his infusion was a week late, I’m just thankful that she was able to find time to squeeze him into her already hectic schedule.

The new nurse was very nice, but Cayden is wary when it comes to new people, especially in medical situations. Plus, he gets anxious when someone needs to access his port. He’s so used to his regular nurse and didn’t enjoy letting someone else access him, especially after a scary incident in the fall when we had trouble accessing his port due to a blood clot.

However, all went well in the end. His regular nurse has been coming consistently for years, so her vacation time was much-needed. Delaying Cayden’s infusion from time to time isn’t the end of the world. As I often say, “Better late than never.”

Note: Pompe Disease News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pompe Disease News or its parent company, BioNews, and are intended to spark discussion about issues pertaining to Pompe disease.


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