Anne Casey was born with a congenital heart defect, and her doctors discovered she had pulmonary hypertension during heart surgery. The “sassy, spunky and fun” 4-year-old now needs oxygen when she sleeps or has a respiratory illness.

Lily Cipriani, 10, was diagnosed with PH in November 2022. She initially needed supplemental oxygen only at night, but after getting the flu in January 2024, her health rapidly declined. Since then, she needs one liter of oxygen a minute during the day and two liters a minute at night. She’s also listed for a transplant.

Both girls receive treatment at Cincinnati Children’s Hospital, a Pulmonary Hypertension Association-accredited PH Care Center. And their mothers are advocates for the Supplemental Oxygen Access Reform Act.

Last year, Meg Casey and Lindsay Cipriani met a legislative staffer for Rep. Greg Landsman (D-Ohio) in an online meeting spearheaded by Melissa Magness, a nurse practitioner specializing in pediatric cardiology at Cincinnati Children’s. The meeting included another parent, pediatric cardiologist Russel Hirsch, two other physicians and several members of the hospital staff.

The group asked Landsman to co-sponsor the SOAR Act, which would make it easier for patients to get the type and amount of supplemental oxygen they need. The bill also would make liquid oxygen more readily available.

Meg Casey said she was baffled that she and her family had never heard of liquid oxygen — despite having good insurance through her husband’s job with a large employer — until hearing from people with PH who had lost access to it.

Liquid oxygen has become nearly impossible to get in most parts of the U.S., but it can be a more manageable option for many oxygen users. Liquid oxygen is created by supercooling oxygen until the gas becomes a liquid, then storing it in a large metal reservoir tank. When exposed to room temperatures, the oxygen becomes a gas again. The reservoir supplies oxygen at home, and patients can fill smaller portable containers from it. In liquid form, it’s possible to store a larger volume of oxygen in a smaller device, making it easy to carry six or more hours’ supply in a light-weight container that could last a full school day.

Portability issues

The biggest challenge Magness sees for her patients is portability. The devices and types of oxygen delivery systems currently available are limited and can negatively affect kids’ quality of life.

Families struggle to find solutions to their children’s oxygen needs that don’t weigh them down or hold them back. Kids sometimes take off their oxygen cannulas when they shouldn’t because they want to do something that’s out of reach of their tubing or because they just want to run around without getting tangled up.

Kids have to work with long oxygen tubes because more portable options don’t work for them. Most portable oxygen concentrators that can be worn like a purse offer only the pulse-flow style of oxygen delivery, where oxygen is released only when a user takes a deep full breath.

Those models require more mindfulness and can be hard for kids to learn how to use properly, Magness says. Instead, most families use stationary oxygen concentrators and homefill systems that allow them to refill compressed oxygen tanks on their own.

The “portable” oxygen machine Casey’s insurance provided for her daughter has wheels, but it weighs more than Anne. Now that her current treatment regimen has helped her become mobile, Anne’s favorite thing to say is “Mom, I’m fine!” as she runs off to play. Casey says she can’t count the number of times Anne has gotten yanked back while she’s running around in her playroom and her oxygen cord gets hooked on something.

Portability was a concern for Lily as well. Cipriani fought with the insurance company to get another concentrator that could stay at school, rather than having to transport the heavy machine and spare tanks back and forth every day. She also had to fight to get more oxygen tanks, especially smaller tanks that Lily can carry.

When Lily first needed oxygen during the day, her private school was concerned about its ability to accommodate her needs. For two weeks, Cipriani had to go with her daughter to all her classes and manage her oxygen to help train the school nurse and her teachers on what was needed.

Safety and weight

The smallest oxygen tanks weigh 5 pounds and can be challenging for younger kids to wear on their backs for long periods. Because the tanks are smaller, they don’t hold much oxygen, so they run out faster. Each of Lily’s tanks takes about four hours to refill, so her family is constantly refilling them, causing high monthly energy bills.

Oxygen concentrators need electricity to function, unlike liquid oxygen. During storms, the Caseys stay awake all night in case the power goes out so they can safely switch Anne from her oxygen concentrator to her back-up tanks. If they lose power, they need to swap out tanks every few hours so Anne doesn’t run out of oxygen.

Although the family is on their utility company’s list to get power restored first, they have to take Anne to the hospital if they lose electricity for more than 12 hours. That’s the only way to ensure Anne will get the oxygen she needs. Casey also learned to safely store the back-up tanks, something she especially worries about.

Feeling ‘normal’

Lots of kids are concerned about looking different because of their supplemental oxygen, Magness says. The attention they get from using oxygen in public can be overwhelming and can add to the reasons kids don’t want to wear their oxygen. Both Casey and Cipriani say they often have to educate people when they’re out of the house, even while getting groceries.

Lily is an active kid who enjoys cheerleading. She uses her big oxygen concentrator during practices, and her team has gotten used to jumping over the 23-foot tubing. However, being tethered to the machine is frustrating. The Cipriani family has found ways so Lily can swim and go into the ocean, but water activities now take much more planning.

It’s been a challenging adjustment, since Lily remembers the activities she could do when she was healthier, and she has had to re-learn how to interact with her sibling and friends.

“She just wants to be a normal kid,” Cipriani says. “It’s taken a toll on her self-confidence.”