Time:Managing HPN is time-consuming. Each day, we spend time taking down her HPN, prepping her HPN, hooking her up to HPN. Each one of those things is a clean procedure requiring several steps. In the hospital, it’s one of the things that puts nurses “in the weeds”. We change her dressing weekly, which is a sterile procedure. Sterile gloves, sterile dressing kit, sterile gauze, sterile water, sterile work surface, masks, alcohol wipes, and a bevy of other accoutrements that we use to get the job done. It’s easily one of the most time-consuming parts of our week, taking a few hours to remove all of her GRIP-LOK, prep the old dressing, give her a bath, and put on a new dressing. Even with two of us, it isn’t easy. If she gets wet, soils her dressing, or it starts to come off because she’s an active toddler, we need to change it again. In Puerto Rico, we had to change her dressing almost every night. You spend time ordering and organizing crates of supplies every week. You have weekly nursing visits for weight checks, drawing blood for labs, vitals, etc. You spend time communicating anything that happens with her line with providers. Last week she had a clot, so I was on the horn with her entire team several times in one day, explaining what was going on. Her HPN physically runs for 12 hours a day, so half of each day Adeline is anchored to her pump and getting her IV nutrition. This is actually a reduction from when she first started, when she was connected for 18 hours a day. HPN rules our home and comes before everything else. There’s many nights that we want to just read her a story and put her to bed, but can’t because we have all of the prep work ahead of us. And now, because Boston will be managing her HPN, we need to travel back and forth to Boston from Baltimore at least every other month.
Bathing & Swimming:Her dressing can’t get wet, so this really makes swimming and bathing…fun. Some families choose to never let their child bathe or swim, it’s a personal choice. If you do let them, it requires preparation. For our family, since we spend summers at the Jersey Shore, we knew the beach would be a big part of her life, so we decided to let her wade in the ocean. She doesn’t swim in public pools or go to amusement parks and just kind of puts her feet in the river across the street. Untrusted water sources can mean infection if the wrong thing gets up under the dressing or into the line. To prepare for swimming, we cover her line with sterile gauze and tegaderm tape. And then we do a dressing change as soon as the day is over or if her dressing comes off during the day. The cover isn’t perfect and definitely not bullet [water] proof. Even the stuff made for getting wet (like Aqua Guard) isn’t fool proof. For bathing, we only let Addie take one bath a week. We plan bath night around dressing/cap change night so we’re not as concerned if it gets wet while she splashes around with her dinosaurs. It can be difficult during the summer if friends invite us over to swim or even to just play in a water table or run in the sprinkler. We’ve tried to make it so Addie can participate as much as possible while keeping her safe. This kid loves the water, and it makes the work to prep her worth it to watch her splash around like a maniac.
Physical Growth:HPN is the only thing that has helped Addie grow. Since before she was born, she’s struggled with growth. While pregnant, I was diagnosed with IUGR (Intrauterine Growth Restriction) and throughout my pregnancy, she measured in only the 1st percentile. Prior to starting PN, her growth was flat, trending downward. Before PN, she was gaining [at most] 2-3 ounces a month. After starting PN, she was growing at a rate of a full pound a month. She went from a dot stranded in a distant galaxy below the bottom line on the WHO chart all the way to the 3rd percentile in less than a year. This summer, we tried to wean some of her PN calories while adding more into her feeding tube. Since the end of May, she’s essentially stopped growing again. Boston’s plan is to basically triple her PN to help her to start packing on the pounds.
Travel:I’d say, “holy moly is our travel impacted by life with HPN” if I wasn’t afraid that John would never let me live down saying “holy moly” in the year 2017. But holy moly is it ever. This past May, we took a trip to Puerto Rico and even our medical team didn’t want us to go. There are so many considerations to make when you travel with HPN. You need to find a good hospital wherever you go and make sure they have experience with central lines. You need to work closely with your current team and plan for all possible scenarios. Medical supplies must be sent, and you need to figure out the logistics of traveling with HPN; getting through security, understanding airline regulations, packing everything securely, etc. We made sure we had a clean place to do her medical cares, a dedicated place to store her medications, and access to clean water. And like we said about time & swimming, each night while our friends went out to dinner, we did dressing changes and medical cares. Holy moly things are different for our family.
Infection & Illness:One of the biggest risks of HPN is a central line infection. It can happen easily and degrade quickly if germs or bacteria get into the blood stream through the central line. It was once explained to us that they’re such a risk for infection because they’re a foreign body that blood doesn’t flow through. Normal antibodies that keep infections at bay don’t run through the line, making it a breeding ground for bacteria. Add to that the fact that you’re pushing massive amounts of sugar through the line, bacteria have a field day. Following strict clean and sterile procedures and being diligent, even when you don’t feel like it can help prevent them. But they’re not guaranteed protections. CLABSI (Central Line Associated Blood Stream Infection) is the primary cause of death in patients who receive PN. CLABSI can lead to sepsis, which can lead to organ failure, which can lead to death. Addie’s had 3 infections since the line was placed, the worst of which she got while inpatient. They thought the bacteria translocated from her gut into her bloodstream – making even our cleanest procedures futile. Every fever has to be treated like a central line infection. Anything over 100.4, she’s admitted and immediately put on a series of broad spectrum antibiotics while they wait for the results of the cultures. Doesn’t matter if it turns out that she’s has a virus or an ear infection, or if she’s just teething. 100.4 and we’re out the door and on the floor.
Cognitive Growth:When your body doesn’t grow, your brain doesn’t either. You see friends, contrary to what you may have heard, your brain is a part of your body. When Addie stopped growing, her learning slowed to a crawl. Physical brain development and early cognitive function are completely intertwined. Acute malnutrition, under-nutrition, and even iron or iodine deficiencies can greatly impact brain development. At 13 months old, Addie wasn’t talking and we had a speech and language evaluation done. At that time, she scored between at the 4-6 month old level. She started HPN shortly after that assessment and at 21 months old, she was tested again. This time, she scored in the 24- 36 month old range. 20-30 months growth in 8 months time. Math. While she was also receiving early intervention services and we worked ceaselessly on her verbal skills, the lesson here is that nutrition literally feeds the brain.
Stress:Being on HPN is a double-edged sword of stress. Relieving much but causing more. Having a central line is risky business. There are negative side effects and the HPN is hard on the liver. Each time Addie gets a fever, I’m a walking ball of anxiety. Afraid that she may have a line infection. If her dressing falls off or gets wet, I’m anxious about the risk of infection. If her line breaks (which it has, 5 times) I’m stressed about whether it can be fixed, if the break will cause an infection, or if it’ll need to be replaced. If she has it replaced, I’m worried about how that will go. She has a duplicate vascular system and it was really hard to place the first time; a procedure that normally takes 30 minutes and one incision took 3 hours and four incisions. If we’re invited to something as simple as a dinner, I start planning in my head about when we will do her cares, if there will be a clean place, will we connect her later, etc. Stress, stress, stress.
Socially:Being on HPN impacts your social life. For nearly a year, Addie was connected to her pump for 18 hours a day, leaving only 6 hours of wireless baby time. Of course, she can leave the house while connected, but it’s not an easy thing to do and it limits you. She wasn’t strong enough to carry her own pump and once she became mobile I’d constantly chase her around with the IV pole. Although we made a scooter for her that she can pull around the house, it wasn’t ideal to have in many social situations. John says it like this – anyone who’s ever owned one of those wired RC cars knows why it sucks. Worst. Toy. Ever. Her evening medical cares also take up time, and can’t be done just anywhere. While we make exceptions for special events, it’s just easier to just not have a life. It prevents you from being spontaneous. You can’t just take an impromptu trip or mini vacation without planning.
Life:Items 1 – 8 on this list represent our cross to bear. But bearing it means so much to us, and to Addie. HPN literally gives Addie life. The invention of it is considered one of the four most significant accomplishments in modern surgery. It has saved over 10 million newborn lives, including the life of our kid. She started her life on TPN and is now living her life on HPN. It really is amazing stuff. Even with everything we have to deal with, I recognize that Addie probably wouldn’t be here if it wasn’t for her HPN. That makes it worth it. Being able to receive parental nutrition in the home isn’t something that has always been a given and shouldn’t be taken for granted. While we’ve got some things that make us different, it has allowed us all to be “normal”. Addie can be a kid, she can play, she can be home with her parents, and she participate in family events and social experiences. With a bit of diligence, planning, and determination – you can make it work.