In a house on Mendenhall Pennisula, soft guitar music plays and a visiting baby gurgles his words and happily crawls on the floor, getting into things he shouldn’t like most babies. At 10 months old, Sylas Stutz has no trouble standing by holding onto furniture.
But unlike most babies, Sylas may lose one of the feet he’s just now learning to stand on.
Sylas was born with the fibular bone in his right leg 9 centimeters shorter than his left, and his right foot is missing two toes. To keep the foot will require years of painful, and costly, surgeries to lengthen the bone in his leg. Diagnosed with fibular hemimelia, a 1 in 40,000 chance, Sylas faces medical complications if his rare limb defect goes untreated.
Wrangell resident Krysta Gillen, 19, took her son Sylas to Shriner’s Hospital for Children in Portland, Oregon, when the doctors in Southeast Alaska couldn’t diagnose his condition. She had previously searched online with the help of her sister, Sara, for the answers to his limb defect. Fibular hemimelia matched Sylas’s condition, so the official diagnosis wasn’t much of a surprise. What was a surprise was the recommended treatment.
“What they mostly suggested was amputation… just leave his heel and cut the rest of his foot off and put on a prosthetic,” Krysta said. “They said he would be able to walk around without the prosthetic on his heel … when he’s not doing things he needs the rest of his leg for. They said that it would be quick and easy and painless. … I don’t see that being an option.”
Krysta learned about an alternative option to amputation called limb lengthening. The doctors at Shriner’s were hesitant to recommend it, however. The lengthening “would last till he is 20 or so,” Krysta said. “It would be a painful process and obviously we would be going back and forth for surgeries.”
She said there was also the option for reconstruction surgery to be done on Sylas’s foot to stabilize him when walking if limb lengthening was done.
However, Krysta said she was told if Sylas underwent limb lengthening, he would be psychologically damaged from the experience. To make matters harder, her only insurance is Alaska Medicaid. If she went with the limb lengthening procedure, she faces the potential of hundreds of thousands of dollars in medical and travel costs.
To pay for the best possible treatment and care, Krysta set up a Go Fund Me campaign for her son Sylas.
“Shriners is a great hospital but they’re not experts in Sylas’s condition.” Krysta said. “I don’t think I could ever imagine taking Sylas to Shriner’s to get his leg lengthened,” said Krysta. “I don’t think I could ever get (his foot) amputated, … He’s got his toes, a perfectly good foot.”
With much to think over, Krysta returned to Wrangell to consider her options. What she really wanted was a second opinion on Sylas’s condition so he could receive the best possible treatment.
Krysta’s sister found a Facebook page dedicated to fibular hemimelia, and the second opinion Krysta wanted — the Rubin Institute, a part of Sinai Hopsital in Baltimore, also called the International Limb Lengthening Center. The doctors there have performed thousands of limb lengthenings and are considered the leading experts.
Many people on the Fibular Hemimelia Facebook page, Krysta noted, took their children to the Rubin Institute. On that page, Krysta learned about a Southeast Alaska family who had gone to there for the same procedure Sylas needed.
Meet Keagan
While most people were counting down the minutes until the New Year on Dec. 31, 2014, Sue Walker had just managed to get her son Keagan, 16 at the time, to Sinai Hospital to undergo his 20th and final surgery to correct his proximal femoral focal deficiency, also a rare limb defect.
For them, it was the eve of a new chapter in Keagan’s life. He wouldn’t have to take frequent trips to Baltimore for treatment, and wouldn’t have to spend time in a wheelchair, cast or using crutches. He wouldn’t have interrupted school years, receive stares from strangers, awkward questions from classmates or have to go to the bathroom with the school nurse, like when he was in a cast from chest to feet.
His femur, which had been nearly 19 centimeters shorter than the other at birth, was now as long as his right leg due to the procedures. The long trips to Baltimore, the surgeries, therapies and stresses involved with correcting Keagan’s proximal femoral focal deficiency would finally be over.
Before his last surgery, Keagan was able to bike from his house on Mendenhall Pennisula to the West Glacier Trail, climb Mount McGinnis, hike back down, bike home and then go to work at the Rock Dump, where he also likes to climb. He has use of his own leg and foot, and puts it to good use thanks to the treatments.
After Keagan completed his surgery, he wanted to give back and help other children receive the same opportunity to receive treatment as he did. He participated in Save-A-Limb’s fundraiser this year and raised $1,620. The organization uses the money to fund travel and medical procedures for those who may otherwise be unable to receive treatment, like Sylas.
He also shared a personal essay he wrote about living with proximal femoral focal deficiency on the rare limb defects Facebook page. “I put it on there so they could see why their kid would go through all this,” Keagan said. “I know people who can do amazing stuff with a prosthetic, but I can do amazing stuff with my leg. I did all this to keep that leg.”
A day before Keagan and Sue were in Baltimore for Keagan’s final operation, Sylas was born at Bartlett Regional Hospital. Though the two share similar limb defects, their families don’t have the same financial means for treatment.
When Sue met Krysta over Facebook, she wanted to help the young mother who faced a hard road ahead. Sue knew all about the cost, the operations required and being the one to make the difficult choice of which treatment to consider.
When Krysta came to Juneau with Sylas for a medical appointment, the Walkers opened their home and the two families met for the first time. In the Walker home, the group sat down for an extended interview to talk about their experience with rare limb defects, possible treatments for Sylas and financial constraints in tow.
Both Sue’s and Krysta’s experiences were similar in that neither of them knew their children had a rare limb defect until after their babies were born. Rare limb defects such as proximal femoral focal deficiency and fibular hemimelia are non-genetic disorders, and doctors do not know what causes it, only how to correct it.
Sue explained just what limb lengthening entails.
“First there’s the extraction phase where they’re pulling the bone apart, put in more bone and that takes about two to three months,” Sue said. “Then there’s the consolidation phase because that bone is very soft, it’s like toothpicks. So the fixator, or the lengthening rod, stays in because that bone needs to harden. Then there’s the recovery phase where you have to regain your range of motion and strength. So the whole process takes year.”
The amount of time this is repeated differs between individuals, Sue said.
“It depends on how much length you need to make up,” she said. “If Sylas is only 9 centimeters, well, that’s providence. That’s probably no more than two lengthenings, maybe one. Keagan’s was almost 19 centimeters. When he was little he would need three or four (leg lengthenings). His ended up being just three. He also had to have 20 separate surgeries due to his condition.”
Seeing Keagan as a young adult helped Krysta decide what she wanted for her son. Now she just needs to find a way to pay for it.
“At first I was scared going to Shriner’s, when they said it would be really painful and he would be in pain all the time,” Krysta said about the lengthening procedure. “But now I’m meeting Keagan, … it was inconvenient having to leave all the time and it’s kind of painful going through surgery, but he doesn’t regret it and he’s happy.”
For Krysta, learning about Keagan’s success with limb lengthening and seeing firsthand how he has use of his leg to go skiing, rock climbing and biking, gave her some peace that going to the Rubin Institute would be the right choice.
“If I had the money I would just get on a plane … and go for it,” Krysta said. “But that’s not really an option.”
Financial challenges
Krysta already knew that going to Baltimore just to receive a consultation would be expensive. Krysta said she got quoted $800 for an initial consultation. But there are other costs to consider.
Sue said that with travel expenses such as airfare, car rental, lodging and food, the doctor’s visit would be closer to $3,000 to $3,500. And that doesn’t include the limb lengthening procedure or other surgeries needed to correct Sylas’s fibular hemimelia.
Krysta graduated high school and is now a full-time stay-at-home mom to care for Sylas. Her boyfriend, Sylas’ father, works in construction building roads, frequently traveling for work. Their medical insurance is through Medicaid and it won’t pay for procedures at Sinai Hospital. Sue knows this as well; Medicaid would only pay to fly her and Keagan to Baltimore and back, nothing else.
The overall cost for covering limb lengthening can be a hefty amount. For Keagan’s surgeries, it was huge.
“I figure he’s got a million dollar leg,” Sue said of Keagan, accounting for all medical procedures, therapies, travel costs and other miscellaneous expenses such as modified shoes with a built in lift to make up for the height discrepancy while his left leg was being lengthened.
Sue, who now has better insurance from her job as the regional hydropower coordinator with the National Marine Fisheries Service, said their insurance still only covered about 90 percent of the total cost. She and her husband, Dr. Vic Walker, a self-employed veterinarian, paid about $100,000 out of pocket.
“Other people are going on vacations, they’re saving for college and we’re buying a leg,” Sue said. “It’s worth every penny, and he uses it well. But it’s expensive stuff.”
Sue said she and Keagan stayed in one lodging right across from the hospital, and though it was subsidized, it was still fifty dollars a night.
“When you’re there for three or four months, that’s a lot.” Sue said. “Our last lodging bill was $6,500.
“Why should Keagan get these treatments and Sylas doesn’t? Just because I have insurance and you don’t?” she said to Krysta. “In one of the most developed countries in the world …that’s not fair.”
The alternative treatment, amputation, is not without its costs, according to Sue, who pointed out that children grow and continually need a new prosthesis. Also, children need a variety of shoes for day-to-day life, which also must be changed as they grow – gym shoes, rain boots, snow boots, tennis shoes, etc. Each pair must be made by a cobbler, which becomes a huge expense over time.
Amputation isn’t an unusual procedure to deal with limb defects, however.
In an interview done with Medical Breakthroughs, Dr. Dror Paley, a surgeon who specializes in treating rare limb defects, said that amputation has been a common treatment for fibular hemimelia. Amputation is seen as a simple solution because it requires one surgery, and a prosthesis can be replaced to accommodate a growing foot.
“Most orthopedic surgeons can reliably do an amputation,” said Dr. Paley. “…The problem is most pediatric orthopedic surgeons might see one or two of these types of patients a year. That’s not enough to get good at (leg lengthening), and yet, to be good at amputation doesn’t take a lot of experience.”
Dr. Paley also said he and his team “found no adverse effects of the lengthening on their (patients’) psyche. While it a challenging process, these challenges actually enhance patient confidence while teaching patients to be goal oriented.”
Weighing the options
Sylas not undergoing either treatment isn’t a realistic option. Besides having a leg that is nine centimeters shorter than the other, which already would make walking difficult, Sylas has an unbalanced foot. Over time, he could face serious medical complications.
According to the Save-A-Limb Fund, the non-profit created by the Rubin Institute to help children with limb defects, “one in 125 children is born with a limb deficiency and many others suffer from limb deformities. Some children undergo amputation because they do not have the resources to travel to a specialist and pay for limb lengthening or reconstruction surgery. Over time, many people’s joints wear down creating painful conditions that make simply walking to the door an excruciating task. Whether from degeneration, arthritis or injury, some people are unable to receive treatment because they cannot afford the cost of surgery or physical therapy.”
After Krysta returned to Wrangell, she participated in an online group chat hosted by Dr. Shawn Standard and Dr. John Herzenberg of the Rubin Institute to discuss Sylas’s condition. The doctors told her they would provide a free consultation if she could make it to Baltimore to determine the best treatment option for Sylas. He wouldn’t have to come in until after he’s one year old, and wouldn’t have surgery until he is three. Based on the x-rays Krysta provided, the doctors confirmed Sylas only has moderate fibular hemimelia and will need some of his leg lengthened but mostly reconstruction on his ankle and foot for proper support.
After Sylas’s birthday Dec. 30, Krysta wants to take him to Baltimore to receive a detailed medical plan on all the surgeries and treatments he will need to move normally on his own foot.
With her Go Fund Me at $2,750 Krysta will be able to go to Baltimore for the free consultation offered by Standard and Herzenberg. With a $30,000 goal, anything more she receives will go to medical costs and other expenses.
“ … I was told in the hospital that they have kids whom they amputate all the time. They run and play and walk and do everything with their prosthetic,” Krysta said. “I just couldn’t imagine doing that.”
• Contact Clara Miller at 523-2243 or at clara.miller@juneauempire.com.
To make a donation
Sylas’s Go Fund Me page, “Sylas’s Lucky Fin Project,” can be found at: www.gofundme.com/pz4zw4pc.
For more information on Save-A-Limb and to donate visit savealimb.org.
Keagan Walker, 17, right, watches as Krysta Gillen of Wrangell plays with her 10-month-old son, Sylas, during a visit in September. Keagan, born with a short femur called Proximal Femoral Focal Deficiency, has gone through nearly 20 surgeries and three limb lengthenings to his femur to make his legs the same length. Sylas was born with a shortened lower leg, called Fibular Hemimelia, and Gillen was visiting the Walker family to learn more.
