Nurses and patients work together to make home infusions successful
We have been blessed to be cared for by compassionate, capable personnel
The two clinical sites where our kids began their enzyme replacement therapy (ERT) for Fabry disease were staffed by top-notch nurses. My kids enjoyed snacks and entertainment while skilled hands worked IVs into their veins. Making the decision to do home infusions was a natural choice, however. The kids couldn’t wait for the day when they could stay in their pajamas and get their infusions in the comfort of their own home.
I’ve written about our complete confidence in the specialist who first prescribed ERT for my twin sons, Michael and Anthony, at the time of their diagnosis. I once naively asked Dr. Raphael Schiffmann if we could administer ourselves the ERT that would generate the missing enzyme caused by the twins’ genetic mutation. I’d seen diabetics give themselves insulin and wondered if there was a similar system for ERT. It didn’t take long to realize there is a reason why Fabry patients don’t infuse themselves. While being completely dependent on specialized pharmacists, nurses, and doctors, patients undergoing home infusions must be cooperative and creative to get the best results.
My now 21-year-old twins and their sister, Marisa, 13, have together had more than 200 infusions in the past four years as part of their treatment for Fabry disease, and it’s fair to say no two infusions have been the same. Even the setting has changed — major hospitals, small student exam rooms on college campuses, outpatient clinics, dorm rooms, and our living room couch. Once, an infusion was finished up at a restaurant.
Our family has been so blessed to be in the care of many compassionate and capable medical personnel. We were delighted to kick-start the twins’ treatment under the supervision of a very experienced nurse who assists in Fabry research. Being the first nurse to explain everything to us, she set a positive tone for my sons’ treatment journey and put us all at ease, happily answering our questions. She has continued to follow up with them for the past four years.
Making the home infusion experience the best it can be
Leaving the security of hospital infusions can be difficult. When problems arise during the infusion process at home, we must collaborate with and rely on the nurse. Since different nurses come into and out of our lives, we must work to build rapport with each new one.
Thankfully, our experience has been overwhelmingly positive, but we’ve been thrown a few curveballs. On those occasions, we’ve developed a few strategies for partnering with our nurses for success.
For instance, it can be frustrating if the nurse is very “by the book,” which might prolong the infusion. When this happens, it helps to focus on the positive: diligence and carefulness reduce risk. The nurse who takes extra time to cover every precaution is acting in the patient’s best interest and we can rest assured that we are in good hands.
Sometimes technical difficulties happen and can become quite stressful for everyone. We’ve found that taking a step back can lead to peace and creative solutions. On several occasions, we’ve had a malfunctioning pump. Thankfully, things were resolved after pausing, praying, and being willing to innovate, even if it meant rigging up a drip using items found around the house.
If a patient is a “hard stick,” it’s important that the nurse be understanding and willing to explore new ways to find the right vein and start the IV. The key is not to panic or place blame when attempts are unsuccessful. Tension can often cause veins to constrict. We’ve learned that taking a break is helpful. Then, with a sense of calm, the nurse and patient can discuss what strategies have worked in the past or agree to try something totally new.
An infusion-associated reaction without hospital or clinical staff around can be scary. My son once had a severe and unexpected reaction in his dorm room and the medication he should have had on hand was in a different building. In moments like that, it’s important to think fast. Together with his nurse, he was able to find a solution using what was available to them.
Communication is indispensable. In order to have a smooth infusion, it’s necessary to have all the right supplies in place. Making sure the nurse knows the correct size and quantity of everything that must be ordered is important. And let’s not take for granted the pharmacists and home health professionals who are available to answer questions and help finalize orders.
Home infusions can be comfortable and convenient for Fabry families. Positive nurse-patient relationships are crucial to the success of the treatment. We are grateful for all the medical personnel who’ve understood how complex treatment can be and have the expertise to make ERT available to Fabry patients at home.
Note: Fabry 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 Fabry Disease News or its parent company, Bionews, and are intended to spark discussion about issues pertaining to Fabry disease.
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