A Breath of Relief: Troy Splawn’s Story

Antlers is a small town in southeastern Oklahoma, known as the deer capital of the world. With a population less than 3,000, the town was built on a spring where Indians and traders would nail the antlers of large deer — trophies from their hunt — on the surrounding trees.

Troy Splawn has spent his life in Antlers. He built his family a two-story, 2,000-square-foot home on the land he owns. He has a few cows, enjoys building projects, and has always led an active life, despite a diagnosis with a rare genetic disease when he was a child.

When he was just 5-years-old, Troy was diagnosed with Mucopolysaccharidosis type 1 (MPS 1), which is one of a group of rare genetic diseases in which the body is missing, or does not produce enough of an enzyme that the body needs to break down long chains of sugar molecules called glycosaminoglycans. Because they cannot be broken down, these sugar molecules accumulate in the tissues, causing symptoms that range in severity. There are various types and subtypes. Troy was diagnosed with Scheie Syndrome, a subtype of MPS 1. Symptoms become increasingly worse as the disease advances.

Children who are born with MPS 1 rarely show symptoms at birth, with most showing signs later in childhood. Severe cases will usually manifest within the first year and can include a large head, skeletal and spinal deformities, intellectual abnormalities, developmental delays, vision and hearing loss, recurring respiratory infections, enlarged organs, heart disease, decreased height, hernias and carpal tunnel syndrome.

Troy’s sister was also diagnosed with the disease, and at the time of their diagnosis, there was no treatment.

The effects of MPS 1 on Troy’s everyday life have included a cloudy cornea — he’s had two corneal transplants — and he has a blind spot in each eye. The disease slows him down and he works at a slower pace than someone without it. As MPS 1 has progressed, his joints have become stiffer and he struggles with pain when he works a lot.

In order to keep moving more freely Troy takes regular walks and keeps himself busy.

“I have the attenuated form of MPS 1,” he explains. “My body makes some of the enzyme but not all of it. On the other end of the spectrum it effects kids drastically. I was able to work at the DHS working with disabled people for 17 years. I just feel better when I keep busy and keep moving.”

A few years ago, Troy felt as though his throat was closing up. He knew something was wrong, but he didn’t know what it was.

“Gradually, I just couldn’t breathe,” he said. “I couldn’t get any air and then I started coughing and couldn’t stop. I couldn’t eat, I couldn’t sleep, and after a few days of this, I started to hallucinate.”

Troy’s wife took him to the small local hospital, and while they helped him breathe, they were unable to find out what was going on. Troy was sent to a bigger town with a larger hospital and they gave him benzodiazepines to help slow down the cough. Unbeknown to anyone, Troy was allergic to benzodiazepines and his breathing continued to decline. He was intubated and transported to a hospital in Oklahoma City via a helicopter.

Over a three-week period, Troy was in four different hospitals. A CT scan showed that his airway had collapsed and the usual treatment was a tracheotomy. The pulmonary specialist looking after Troy had heard that Fawad Chaudry, M.D., OU Health pulmonologist and Director of Interventional Pulmonology at OU Health University of Oklahoma Medical Center, was performing a tracheal stent procedure that is far less invasive than a traditional tracheotomy.

Troy was transferred to OU Health University of Oklahoma Medical Center under the care of Dr. Chaudry.

Troy had severe tracheal stenosis and thickening of the trachea which had caused respiratory failure. The trachea, which is also known as the windpipe, is part of the airway between the voice box and the lungs. Tracheal stenosis occurs when the trachea narrows and restricts the ability to breathe normally.

The diameter of a trachea is usually between 16-18 millimeters. Troy’s trachea was measuring less than 5 millimeters and was completely blocked.

Dr. Chaudry performed a bronchoscopy on Troy in which a camera, called a rigid bronchoscope, was inserted through the mouth into his trachea. He dilated the trachea and placed a silicone stent, increasing the tracheal diameter from 5 to 14 millimeters. The procedure was a success, allowing Troy to breathe comfortably on his own.

“I tell Dr. Chaudry every time I see him that he saved my life,” said Troy. “You don’t realize until you can’t breathe, that I was going to die if somebody didn’t find it, if somebody didn’t do something. He’s a lifesaver — he’s a great doctor. He’s warm, patient, he listens carefully and then, as a team, we make a decision together.”

The procedure changed Troy’s life. He was able to go back to enjoying all of the activities he used to. However, even with the ability to breathe freely, the disease would continue to advance without treatment.

Troy started enzyme replacement which treats progression of the disease. The two-way therapy is a combination of enzyme replacement and tracheal interventions for respiratory failure, which help Troy lead an active and full life.

Scheie Syndrome affects 1 in 500,000 people. It’s not very common, but can be devastating as the disease progresses.

As a result of the life-saving procedure, Troy continues to lead an active life doing what he loves.

“I’m back to doing all sorts of stuff — feeding the cows and keeping busy,” he said. “I feel good!”

“This is the only fellowship-trained interventional pulmonary center in the state,” explains Dr. Chaudry. “We see a high volume of patients with airway diseases and tracheal complications. Many are different diseases that have similar tracheal complications like MPS 1. We are treating the rarest and most complex diagnoses with advanced technology and treatment methods. We are here to serve the community.”

OU Health provides a wide breadth of both benign and malignant airway diseases treatment. Explore your pulmonary and respiratory health options or get a second opinion from an OU Health pulmonologist or thoracic surgeon. Call (405) 271-7001