Revisiting the pros and cons of a port for my daughter with LEMS

Accessing her veins for infusions is always a challenge, this columnist says

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by Lori Dunham |

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Most people who have faced a health crisis understand the relief of finally receiving a diagnosis. Once this happens, a plan of action can be put into place.

When our daughter Grace was diagnosed with Lambert-Eaton myasthenic syndrome (LEMS) at 15 years old, I was definitely relieved to know what we were dealing with. Only then could we begin to discuss treatment options.

It’s now been more than three years since her diagnosis. Grace has tried multiple medications and treatment plans, which originally included intravenous immunoglobulin (IVIG) therapy. After months of trial and error, we settled on the medications Firdapse (amifampridine), Mestinon (pyridostigmine), and Rituxan (rituximab).

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Trouble with running an IV

Very early on, we knew Grace would most likely require infusions to combat her LEMS symptoms. Her doctor started her on IVIG therapy, and the infusion took two days a month. Grace didn’t like leaving her IV in overnight, so the nurses would end up running a new line each day. This posed a serious problem because we learned that Grace has deep and collapsing veins that are hard to access.

After attempting to run her IV four or five times, the nurses often had to call in a vascular access team to run it using ultrasound.

Deciding whether to get a port

At that point, Grace’s neurologist suggested a port, which would eliminate the painful process of running an IV twice a month. However, we didn’t pursue it because we didn’t think Grace was seeing significant benefits from IVIG therapy.

Grace no longer does monthly infusions, but she does receive Rituxan infusions every six months. This treatment has made the biggest difference for Grace.

The nurses couldn’t access Grace’s vein during her last infusion, so the vascular access team was called in. The topic of ports came up once again. Nurses discussed the pros and cons with us, but our consensus was that a port wouldn’t be a good fit for Grace, since she only receives infusions twice a year.

According to Grace’s rheumatologist, port placement reduces anxiety and simplifies the infusion process for many. It also makes it easier to do bloodwork and administer other medications. However, she advised against it for Grace.

First, Grace has an immune deficiency, which means she’s at a greater risk of infection when placing the port and keeping it clean. Furthermore, the doctor suggested that unless Grace has absolutely no vein access or receives another diagnosis that requires additional IV therapy on a regular basis, she wouldn’t recommend a port. Since Grace is down to two infusions a year, she’s not a good candidate at this time.

I know numerous people with LEMS do have a port, and I’m glad that it’s an option for those whose veins don’t cooperate during the infusion process.

Note: Lambert-Eaton 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 Lambert-Eaton News or its parent company, BioNews, and are intended to spark discussion about issues pertaining to Lambert-Eaton myasthenia syndrome.


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