Teegan

Propionic Acidemia

Note: Teegan’s mom and dad have loved, lived with and cared for this little boy; I get the joy of being his grandma. This is written from my perspective.)
 
Teegan Keith was born on July 3, 2002, in Idaho Falls, Idaho. At four weeks old, Teegan and a group of us headed for a weekend retreat in Park City, Utah. He never fussed, never woke up, didn’t eat, didn’t wet. The ideal baby! Things just didn’t seem right though, and we took Teegan to the local medical clinic. The doctor said Teegan was dehydrated and thought the iron in his formula was the cause. He suggested we get him to his regular doctor as soon as we returned home.

Back in Idaho Falls, we called his doctor’s office. The response was that it wasn’t unusual for a baby to not have bowel movements for several days and if it continued, to come on in. It continued and he was taken to the hospital where placement of an IV in his little body proved fruitless. The diagnosis – leukemia. He was prepared for his first ambulance ride to Primary Children’s Medical Center in Salt Lake City, Utah, and assigned to the bone marrow unit.

After five days of testing and probing – one of the genetic specialists, Dr. Nicola Longo, made the resulting diagnosis: Propionic Acidemia or PPA. I will never forget the big, burly nurse who’s eyes were brimming over as he realized what all the “normal” feedings had been doing to Teegan.

Teegan was moved to ICU where he and his parents spent the next 40 plus days of his life. They arrived back in Idaho Falls with this tiny, little boy with tubes running down his nose – my first introduction to a NG tube. His mom informed us of all the medical terms, the treatments and instructed us in taking care of this doll.

It seemed like each day was now up and down, crisis and calm. As in a lot of communities in the US, PPA was a foreign visitor in Idaho Falls. His primary care physician had never heard of PPA, but tried his best to come up to speed on this condition. The Idaho Falls hospital staff was clueless without the prior experience of a case of PPA. New-born screening was not part of the norm in Idaho Falls.

A series of emergency hospital trips were required during this time and each trip brought more and more frustration. From the inability of the medical staff to find an adequate vein to hydrate his body, to the looks of empty stares when you told them he had PPA, each visit was a long drawn out process of educating those who we thought should already know.  The only oasis of hope we had for Teegan was 350 miles away in Salt Lake City.

At 8 months, Teegan went into convulsions and lost consciousness. Once again he was rushed to the Idaho Falls hospital to endure hours of indecisiveness; sticking of impotent IV probes into his arms, his feet, his head, his hands; and precious time ticking away. When a willing vein was finally discovered, I remember the thick consistency of his miniscule blood sample that was extracted. After more than five hours of just waiting for some kind of answer to reverse this situation, Teegan and his mom were once again put into an ambulance to send them back to Primary’s in Salt Lake. Once there, Teegan was found to have a buildup of ketones. Teegan’s reflux condition was also a factor; he was slowly starving as well as developing higher and higher levels of ketones. The insertion of a nissen ring, medically known as a nissen fundoplication, was completed following Teegan’s recovery from pneumonia and a staff infection.

During the next year, two more emergency trips were made to Salt Lake; one via Life Flight. Trips were also made every few weeks for check-ups and blood tests because there were no facilities to complete them in Idaho Falls. Receipt of formula in Idaho Falls has also been a problem, and one emergency trip had to be made to Salt Lake to pick up formula from the hospital so Teegan wouldn’t miss his feedings.

Two to three times per week, therapists would come to the house. Teegan was also given swimming therapy. Because of the late diagnosis of PPA, Teegan’s muscles have gotten a slow start and he continues to work hard at making progress.

Teegan’s last trip to the hospital was four months ago after experiencing convulsions again. It took five days to stabilize his metabolic counts and get him home.

Teegan is such a joy, and because of his condition, we have grown stronger as a family. I know, as his grandma, the amount of love generated from this little boy and want to publicly tell my son and daughter-in-law THANK YOU for all you have done for Teegan – you both are wonderful! Discovery of Teegan’s condition has also prompted more new-born screening and has helped other families with early diagnosis.

Teegan’s new life in Phoenix will bring new challenges for his mom and dad.  Arrangements have been made for contact with a new doctor with the help of Primary’s in Salt Lake. He’ll be back in Idaho Falls for his third birthday – and what a great day that will be!!

Pat
Idaho Falls, Idaho
From the Summer 2005 OAA Newsletter