Four-year-old Thomas Onorato is a young zoologist at heart. Often seen with binoculars in hand, the adventurous preschooler is particularly drawn to bird watching. He enjoys talking about his feathery friends and studying their beauty and habitat.
Thomas’ love of animals runs so deep that he says he wants to be a veterinarian when he grows up. “Thomas is obsessed with animals. It’s his love,” says his mother, Melissa.
Beyond his quest to care for animals, Thomas has two other important missions — to manage the rare condition, microvillus inclusion disease (MVID) and receive the lifesaving parenteral nutrition (PN) support he needs to grow and thrive.
Microvillus inclusion disease is a genetic condition of the intestines that causes severe diarrhea and the inability to absorb nutrients. The inability to absorb nutrients is why Thomas receives long-term, home-based PN support.
“Thomas will unfortunately be in and out of the hospital for the rest of his life because there is no cure for microvillus inclusion disease,” says Melissa, who travels from Long Island, New York to Boston Children’s for Thomas’ care. “PN is one of the major reasons our precious son is alive today.”
Thomas currently receives Omegaven, a fish oil-based experimental drug developed by Boston Children’s Dr. Mark Puder, and medical support from the HPN team, including Dr. Bram Raphael, director of the Home Parenteral Nutrition Program. Thomas gets the nutrients he needs to grow, in the comfort of his own home.
Since his arrival at Boston Children’s, Melissa says her son has seen major improvement — his growth is age appropriate, his energy level is high and “he’s the best he has ever been.”
“We feel very blessed that Thomas has come as far as he has,” says Melissa. “The level of care he receives at Boston Children’s is hands-down the best.”
Melissa shares some of her family’s learnings and offers the following parent-to-parent tips to manage parenteral nutrition care at home.
1. Create emergency/back up HPN supply kits
Emergency supply kits include extra alcohol, gloves, antibacterial gel, saline, heparin, swab caps, central line dressing such as Tegaderm, injection caps, a plastic clamp and even tubing in various sizes. Store emergency supplies in a medium-size Ziploc bag so everything is visible. Keep an emergency kit in your car, diaper bag and child’s backpack. Also leave larger emergency kits at close family and friend’s houses that you frequent. Sometimes supplies have been defective (or dropped accidentally on the floor) when away from home. Boston’s HPN team has always stressed the importance of preparation and the critical importance of keeping supplies sterile and clean. This is especially important when you’re away from home and hooking up HPN.
2. The power of 2: Have two people present for sterile dressing changes
Sometimes there are emergencies and only one adult is present, but Boston’s HPN team encourages having two people present as standard practice. There have been several times when I forgot a supply after putting on my sterile gloves or a supply drops on the floor. Having an extra pair of hands is incredibly helpful. It is also nice to have someone entertain your child and make sure they keep their arms up away from the site until the dressing change is complete.
3. Make HPN functional for active toddlers
Thomas is now four years old and is more conscious that he is connected to tubing and a backpack containing HPN. But sometimes he forgets. I recommend using Vygon’s lectrospiral tubing. It resembles a telephone cord and allows further range of motion. Also, another precaution is to place a piece of medical tape near the end of the tubing where it connects to his broviac. If your child sprints while being hooked up there will be initial tension on the tubing not on the broviac.
4. Manage your HPN schedule
Don’t be afraid to ask family and friends to accommodate your HPN schedule in order to maximize quality time. Thomas has many cousins and it’s such a great feeling to watch him interact with them. He wants to be active and run around with them. If we can, we usually ask family and friends to spend time with us when he is off his infusion so we don’t have to hover over him while he wears his HPN backpack. More importantly, Thomas loves the freedom!
5. Remind others to include HPN kids at mealtime
I’ve noticed that teachers, family members and friends are always curious about mealtimes in our home and want to know how to include Thomas during birthday and holiday celebrations that focus on special meals. Mealtime is a social event across cultures. We include Thomas at the table and take advantage of that time to talk and laugh. Also, Thomas loves to cook! He might not be interested in the end result (eating a delicious meal), but he enjoys the process.
6. Educate others about HPN
It is very important to us to educate others in order to raise awareness. We enjoy sharing our story and educating others about our HPN lifestyle. I find that children will ask questions immediately if they see Thomas’ tubing, HPN backpack or his central line. Once they understand it’s how he gets his nutrition it’s no longer the elephant in the room. I even notice that children show compassion and increased tolerance in his presence, which is a beautiful thing to see in young people.
7. Stay connected
Having a child with a medical condition can be very isolating, especially if your child has a rare disease. Our family is very fortunate to be a part of a private Facebook page for families affected by microvillus inclusion disease and Boston Children’s HPN Group. To have a platform, to discuss a spectrum of topics from medical challenges to personal victories is a very powerful tool and resource.