Respiratory Management

Oxygen Jedi: Equipment Sounds Like Darth Vader. Six-Year-Old Chooses R2-D2 System.

• By Elisha Bury
• Aug 08, 2007

NAME Ashley Frank
AGE 24 (6 at diagnosis)
DIAGNOSIS Chronic and obstructive sleep apnea; severe peripheral pulmonary artery stenosis
EQUIPMENT Liquid oxygen

HER STORY At age 6, Ashley was admitted to intensive care because of a series of peculiar pneumonias. While she was in intensive care, physicians observed that Ashley desaturated into the 70s, sometimes as low as the 60s while she slept. The doctors weren't sure how to treat her, so they kept her in intensive care for two weeks. The first idea for treatment was to bypass the obstruction with a trach. Ashley's mom, Peggy Frank, objected, so a test was performed to see if the trach would work. The test showed that the trach would not be effective, and the physicians ended up sending Ashley home with oxygen.

"Our first oxygen machine was Darth Vader," says Peggy. "Darth Vader was black and electric. … Darth Vader would go huff and puff all night long like Darth Vader because it was right after 'Star Wars.' "

A home-based sleep study showed that oxygen was helping.

"Then they changed us to R2D2, which was a silver and white oxygen thing that was liquid," says Peggy. "And we liked that one a whole lot better, and then they finally decided they would keep her on this at night permanently."

Because Ashley was petite, an adult cannula was too small for her. Peggy says she had to cut off the ends of the nasal cannula to fit Ashley. The family also purchased long cords so she could get up at night if needed.

Then six or seven years ago, Ashley was diagnosed with severe pulmonary artery stenosis — parts of her lungs were not getting enough blood supply. Though there is no treatment, physicians decided to put Ashley on oxygen around the clock. Finally, Ashley was able to have some surgical intervention to open up a sliver of the pulmonary arteries. After a couple of surgeries, she was able to quit using oxygen during the day.

BIGGEST CHALLENGE Training and helping school staff become comfortable with oxygen was one of the biggest challenges, says Peggy. The family lived in Northern California briefly, and the school retrofitted the bus and stored a large oxygen tank on school premises. The staff was trained to help Ashley refill her portable tank.

"She took her portable tank with the trolley, and as needed, they would fill it up there," says Peggy. "That worked fine. However, when we moved to Florida, they could not figure out how to retrofit the bus, and it caused a lot of problems."

Eventually, the school in Florida retrofitted the bus and implemented the large oxygen tank. An in-service helped the staff learn how to fill the tank.

Another challenge was coping with people staring at Ashley. In addition to using oxygen, she was also born with a partial deletion of the short arm of chromosome 20.

"She looks a little different to begin with," says Peggy. "And over the years, everybody stares at her. … A couple of years ago, we were out for sushi and Ashley was with us and she had her oxygen on. This little girl, little like 4 or 5, was staring at her. And instead of getting upset, I turned to the parents and the little girl and I said, 'Are you looking at this? Do you want to know what this is? See this little tube; this helps her breathe. You want to come touch it?' And she was so excited, and the parents were so into it. And it was really nice because we tend to stare at what we're uncomfortable with."

HOW EQUIPMENT HELPED Liquid oxygen has made all of the difference in Ashley's quality of life. Portable E tanks have enabled her to go to Disneyland, the beach and the movie theater. "She puts it on herself; she turns it on and does her little thing," says Peggy. "It's what saved her from staying in intensive care forever. She'd still be there otherwise."

HME INSPIRATION The family has a strong relationship with the oxygen provider. The provider follows up every Monday to make sure Peggy will be there for oxygen delivery. "My oxygen man comes once a week. He's very nice," says Peggy. "My little 3.5-pound dog barks at him all the way up and down the stairs."

Peggy says that respiratory therapists have been lifesavers as well. "Over the years, the respiratory people were my favorite people because (Ashley) had so many pneumonias. That whole field is just amazing. They're so compassionate. They saved me."