I’m thankful my son is overcoming his anxiety about an infusion port
My oldest boy, who has Pompe, has had therapy this way since he was a baby
Many folks don’t know what an implantable venous port is and what it’s used for. However, my 6-year-old son, Cayden, is very familiar with it and its purpose.
Cayden received a port when he was just 8 weeks old, which is atypically young for a child. He was having problems with IVs and even developed a blood clot in his leg from a peripherally inserted central catheter (PICC) line.
Cayden needs a port so that he can receive enzyme replacement therapy to treat his infantile-onset Pompe disease. These infusions are now the only treatment for the disease approved by the U.S. Food and Drug Administration.
A port is a tiny device about the size of a dime that’s surgically placed under the skin, typically in the upper chest. It has a tube, or catheter, that runs into a vein so patients can receive medications. That makes access much easier than having to find a good vein for an IV, especially in babies or young children whose veins are tiny.
For years, Cayden was good at having his port accessed. We always applied a bit of lidocaine to numb the area so that he wouldn’t feel the needle going in. This routine used to work wonders, but around age 4, he had a bad experience with it. A small blood clot developed on the end of the line for his port, which prevented the flow of medication — and the return of blood. What was supposed to be a typical infusion day descended into chaos.
Cayden had to go to the hospital to get everything figured out. Thankfully, the hospital staff was able to get a tiny bit of medication into the line to dissolve the clot. I was ecstatic, because the only other option would’ve been surgery to remove the nonfunctioning port and replace it with a new one. That’s the last thing I wanted.
Since that incident, Cayden has felt a lot of anxiety when his port had to be accessed. It’s made him cry. More recently, he’s said that he hates the needle and wants it to go away.
Hearing that broke my heart. As his mother, I have a hard time absorbing something like that while knowing that there’s nothing I can do to help. He needs these enzyme replacement infusions to live. Without them, infantile-onset Pompe disease is fatal.
A breakthrough finally happens
Knowing all of that dynamic made me nervous about something that occurred last week. I discovered that I’d misplaced his lidocaine cream, so he’d have to access his port without it. That hadn’t happened in years, as it typically occurs only during emergencies when medical workers need to access his port quickly.
I let Cayden know that his home infusion nurse would have to access his port without the numbing cream. I figured he’d take the news hard, but to my surprise, he wasn’t even fazed. Instead, he managed to get through the process without crying or fighting. He just lay there, a little nervous but mostly calm, and let the nurse do her thing. He still noted that he hates the needle, but I couldn’t believe how well he handled it all.
I’d been waiting for a breakthrough like this one. Sometimes Cayden gets worked up and cries a lot, which makes his chest move. When that’s happened, it’s been even harder to access his port. Thankfully, this nurse is quite familiar with Cayden, whom she’s been seeing since he was about 18 months old. She can typically access Cayden’s port without a problem.
I’m eager to see how Cayden’s next infusion goes. I’ll have more lidocaine cream for him by then, but I’ll be interested to see if he’ll hold it together as well as he did this past time. Either way, I’ll be there to hold his hand through it all.
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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