Kylie Anne Lether was born August 8, 1997 in Salt Lake City, Utah. She weighed 6lbs. 10oz. and was 21 ½ inches long. She was born with Infantile Idiopathic Scoliosis and Developmental Hip Dysplasia. Her back had a big hump on the left side, which was her spine lifting her shoulder blade up. Her hip sockets were straight instead of curved and there were no femoral heads. Doctors are sure both of these problems were caused by the way she was positioned and had ran out of room in utero. Kylie was full term, breech and on a diagonal facing outwards. I was told that she had to have been in that position for quite some time to have caused such damage.
Because she had both problems that couldn’t be focused on at the same time, her orthopedic doctor focused on the most severe, which were the hips. We were told that if the hips were not corrected as early as possible, she would have to undergo many surgeries involving cutting of the hip sockets, metal plates and screws. There would also be the possibility that she wouldn’t walk normal or even at all. In the mean time, her scoliosis was progressing. It was already in the high 20’s.
At two weeks old, she was placed in a Pavlik harness brace and then when she was four months old, she had her first of many Spica casts applied. After five weeks in her first Spica cast, she was hospitalized for two weeks with bacterial meningitis from an ear infection that didn’t respond to antibiotics. She was also misdiagnosed many times. When she was finally diagnosed, we were told that they didn’t think she was going to make it. If she did make it through, she would be mentally retarded, blind, deaf, and not live a long life. To treat her meningitis, doctors had to cut the Spica cast off. While being treated for the meningitis, her right leg had become dislocated and she needed surgery to get it back into the socket. During this time, we learned that she had a Duplex Kidney, which is a kidney that measures a half length longer than the other. She underwent testing to determine if the kidney was functioning normal, which it was. We also learned that she was deaf in her right ear. We all assumed that it was caused from the meningitis; we later learned through a MRI that her ear bones in the right ear had abnormalities from birth.
Kylie is a fighter!! She pulled through the meningitis with some possible nerve damage that makes her muscles weak and almost impossible to build strength. The left side of her body is affected more than her right side. She ended up being in Spica casts for about five months. Her hip sockets developed normally and the femoral heads developed, as well.
Finally, her doctor was able to focus on her spine. By this time, her curve was 45 degrees. She was immediately placed in a TLSO brace. The brace corrected the curve to 34 degrees. We would go to regular follow up appointments and the curve always stayed 34 degrees. When she was 3 years old, her curve started progressing. It was progressing very quickly. Her curve was now 52 degrees. Her doctor discussed Risser casting with us and we agreed to try it. She wore two different casts for three months and her curve was back down to 34 degrees. After the Risser casts, she went back into a TLSO brace. At that time, we started discussing with her doctor some of the other non-surgical options because we knew it was just a matter of time before her curve would progress. He told us about the Halo Traction procedure. The Halo Traction is the gentle pulling of the soft tissue (joints and muscles) to help straighten the spine. Weights are hung from a rope woven through a pulley system to a carabineer attached to the top of the Halo. The traction gently pulls against the child’s body weight to straighten the spine, gently and nonsurgiclly. The Halo Traction Treatment for progressive infantile scoliosis was a new procedure to the U.S. and at the time was only available at Shriner’s Intermountain Hospital in Salt Lake City, Utah. Fortunately, that was the Shriner’s Kylie went to and we only lived 20 minutes away.
We read a booklet published by Shriner’s Intermountain about the Halo Traction procedure that showed pictures and told us how the whole thing worked. We also watched the video with Olivia and Breann, two of the first children in SLC to benefit from this non surgical procedure. After reading and watching, I FREAKED!! I knew that one day she would have to have this procedure done, but I didn’t think I could put her through that after all she had already been through. My husband and I decided that we would allow her doctor to do the procedure when it became necessary. We knew it was the only way to avoid early fusing of the spine and we figured that we would have enough time to get use to the whole idea, before she had to have the Halo. Five months later her curve was at 70 degrees, HALO TIME! I was so upset and couldn’t stop crying. Kylie walked up to me and asked why I was crying and then said, “Oh, it’s because my back got worse huh? Well that’s ok mom, because the front of me is not broke.” And then went back to playing with some toys. That’s when I realized that I needed to pull it together. My child was stronger than me!
Kylie started the Halo Traction in May 2002. She had to stay at Shriner’s with her Halo for 2 months. She was unbelievable! She never complained or wanted her halo off. She loved it and couldn’t wait to show it to everyone and tell them that she was an angel. She never wanted her doctor to take it off.
She had a wheelchair and a walker that she used with the Halo. When it was almost time for the Halo to be removed, we needed to decide on what was next for treatment. I was terrified to have the Halo removed because I knew that with the Halo on, her spine couldn’t get worse. The Halo traction corrected her curve from 70 degrees to 24 degrees. The last time her curve was close to that, was when she was about four months old. We knew that if we had her placed into a TLSO brace again, her curve would progress very quickly and we didn’t want to loose the great correction we gained from the Halo Traction. In talking with her doctor, we learned about Subcutaneous Growth Rods. We were very nervous to try the Growth Rods because we didn’t know what the outcome would be and knew the risk of spontaneous fusion to the spine at the attachment points. They would have to surgically lengthen them about every six months as she grew. We were also told that we could have the rods removed at any time if we decided to, and could go with some other treatment. We decided we would give them a try.
Kylie had her Halo removed and underwent Subcutaneous Growth Rod surgery. I was amazed at how quickly she recovered from the surgery and that she didn’t have much pain at all. She immediately took to her new rods, although she was sad to have her Halo taken away. She called them her special rods. When she had her Growth Rods lengthened for the first time, six months later, her curve was still 24 degrees. Every time the rods were lengthened, a couple of degrees would be lost. After two years with the Growth Rods, she started developing Kyphosis above the rods. The Kyphosis progressed very quickly, to 95 degrees. In May 2004, the Growth Rods were removed and she underwent the Halo Traction for the second time. Kylie was so happy to have her Halo back, and I have to admit that I was too. She did so well with the first one and the correction we got was great, how could I not be happy!
Kylie was in the second Halo for two months. Because she is older, her spine has become rigid, and Kyphosis is harder to correct. So, we didn’t get as much correction as last time. Her scoliosis was 45 degrees corrected to 40 degrees and the Kyphosis was 95 degrees corrected to 56 degrees. After the second Halo was removed, we decided to have her placed into a plaster Risser cast, this time with neck support to help with the Kyphosis.
She had her first Risser cast with neck support on for about ten weeks. We didn’t gain any correction in it. She had the second cast applied. We did gain some correction this time. The Kyphosis has been corrected to 45 degrees, 11 degrees of correction. She had that cast on until November 2004. When the cast was cut off, her spine instantly regressed back to 49 degrees. That same day, while we were waiting for her brace with neck support to be completely finished, her spine progressed from 49 to 60 degrees in just six hours.
In March 2005, she had her first orthopedic appointment for x-rays since she’d been wearing the new brace. She measured at 60 degrees, still. After her orthopedic appointment, we met with the neurologist at Shriner’s to have Kylie evaluated. She read her history and asked a lot of questions, then examined her. She then told me that she didn’t think Kylie’s physical disabilities and muscle weakness were caused by the meningitis she had at five months of age. She believes Kylie has a type of nonprogressive muscle myopathy, which she was born with. I didn’t know what to say, that wasn’t at all what I expected to hear. I couldn’t help thinking, why wasn’t this noticed earlier, like when she was born. From the time she was two weeks old, she had been in a brace or cast for her hips and then in a brace or plaster cast for her scoliosis. She was not able to physically develop when she was suppose to and then having meningitis at a very young age, no one ever noticed that something else was wrong with her. It was assumed, that the meningitis was the cause of her physical disabilities and lack of muscle tone.
The neurologist scheduled Kylie for a MRI of the brain, to see how much and how the meningitis had affected her brain. We received the MRI results that same day. The scans showed that her frontal lobe was affected the most. The frontal lobe was covered with many large white patches, which are basically dead parts of the grey matter due to lack of oxygen. Her entire brain is covered with smaller white spots. The doctor at Primary Children’s Hospital pulled up her past CAT scans to look at them. The first scan was when she was admitted with meningitis, had no white spots anywhere on the brain. The second set of scans were taken three days later, had all the white spotting. Fortunately, the damage wasn’t bad enough to have taken any functions away, just enough to alter some things a little. Kylie is a very smart kid. Sometimes she needs constant reminding to do certain things. She has a hard time concentrating at times. These are a few things she will have to deal with. Kylie will also undergo a muscle biopsy the next time she is sedated for a plaster cast or Halo traction, to determine what is exactly wrong with her muscles.
In May 2005, Kylie was one out of fifteen children selected to be a part of the first ever, Early Treatment Trial Project (ETTP) training conference, for progressive infantile scoliosis. The conference was held at Shriner’s Intermountain Hospital in Salt Lake City, Utah. It was sponsored by the Infantile Scoliosis Outreach Program, a service of Ability Connection Colorado. The conference was held to train doctors in the early treatment casting technique from the pioneer of this treatment, orthopedic physician, Miss Min H. Mehta, MD, FRCS. Miss Mehta retired from the Royal National Orthopedic Hospital in London and Stanmore, England.
Miss Mehta examined each child with the doctors and practitioners observing her examination techniques. We learned a lot of new things about Kylie, some good things and some not so good. We learned that the only benefit Kylie will ever have from casting is to hold her curve where it is, no permanent correction. Also the casts can not untwist her severely twisted spine. Kylie’s spine is too rigid. That was very upsetting to hear. Miss Mehta was
however, able to give us great relief in knowing that our decision of growth rods did not cause Kylie to develop kyphosis. She told us that her vertebrae in
that area were already twisted and that she would have developed kyphosis anyway. Miss Mehta told us that because her spine is so rigid and it’s been like this for so long, it might start to self fuse. She highly recommended that Kylie needed to spend up to 4 hours a day out of her brace doing special exercises and stretching techniques which she showed us, in hope that it will loosen her spine and avoid self fusing. I was terrified to have her out of her brace that
long, she progresses very quickly. When her last cast was cut off and we were waiting for her brace to be finished, her spine went from 49 degrees to 60 degrees in just 6 hours. Kylie’s been out of her brace every day for 2 to 4 hours doing constant stretching and exercising.
Kylie had her orthopedic follow up at the beginning of June 2005. We were quite nervous for this appointment because the outcome of this appointment would determine if she would undergo her third Halo traction. Kylie doesn’t have to undergo a third Halo for now. At her appointment three months later, her spine measured at 60 degrees in the brace. In June 2005, her spine measured at 55 degrees out of brace! We were shocked!
I believe that the special exercises, stretching techniques, and spending that much time out of the brace to do them, is the reason she is at 55 degrees now. She has also been doing a lot of swimming in our pool. Kylie is now able to get herself off the ground with no help from anyone or anything. We were told she may never accomplish that, she did!
Kylie will continue to wear her brace with neck support and enjoy the summer in her swimming pool. Kylie has been wearing this brace since November 2004. It was believed that this brace would not hold her spine at all. This brace has helped her spine from progressing! Kylie has her next orthopedic appointment in October 2005.
2005-2007 Update:
Kylie had her orthopedic appointment in October 2005, her scoliosis measured 60 degrees. She also has a lot more rotation. On February 6, 2006, she underwent her third Halo Traction. She was very happy! She was also able to have a muscle biopsy. The muscle biopsy came back positive for myopathy, no specific type. Her myopathy is non-progressive, although if she doesn’t stay active and work her muscles, they will atrophy. She will be unable to regain the muscles. The muscle tone and strength she has now, is all she’ll ever have. It is uncertain at this time how this will affect her as she gets older and bigger.
Two weeks before her Halo was to be removed, she was approved to undergo a procedure called the VEPTR. (Vertical, Expandable, Prosthetic, Titanium Rib.) VEPTR is a surgical procedure that consists of two rods that are made of titanium. They are adjustable, and are placed on the outside of the rib cage. They are attached to a set of top ribs and to the pelvis, she has one on both sides. The VEPTR is internal bracing for the spine and must be surgically adjusted as she grows, approximately every six months.
This procedure couldn’t have come at a better time. We had been told that plaster casts were no longer an option, the Halo Traction was not correcting much at all, and back braces alone would never hold her spine. Eventually, her heart and lungs would suffer. At this time, a spinal fusion was not an option either. Kylie was nine years old, she is small for her age. Her Duplex kidney causes her internal organs on her left side to be crowded. The bottom part of her lung it folded under and her heart has been shifted out of its proper spot. If the growth of her torso is stopped, the internal organs will become even more crowded and this would be life threatening.
Kylie had her VEPTR dual hybrid surgery on April 3, 2006 at Primary Children’s Hospital in SLC. She was transferred there from Shriner’s Intermountain Hospital, in her Halo Traction. The co-inventor of the dual VEPTR hybrid performed her surgery with assistance from her orthopedic doctor, since birth. She did wonderful, everything went as planned. The VEPTR has done amazing correction for her. With her spine being so rigid, we were all surprised how much the VEPTR dual hybrid had corrected her spine. After two months in the Halo Traction and before the VEPTR, her scoliosis was at 63 degrees. After the VEPTR it was at 42 degrees. Her kyphosis was 70 degrees before the VEPTR, after it was ZERO! Her back was so flat, it wasn’t even that flat when she was born.
Four days after her surgery, she was transferred back to Shriner’s Hospital, where she had been staying for two months prior to surgery, to finish recovering. She was able to go home the following week. Every six months, she will return to have her VEPTR expanded by the brilliant surgeon surgeons in SLC..
In August 2006, four months after her VEPTR was implanted, the left side came off the rib. She went in for surgery later that week to have it re-implanted. Her doctor felt like that the reason for it coming off was that there was too much tension on the VEPTR in that area where the kyphosis was being held to zero degrees. During surgery, he released some of the kyphosis and moved the VEPTR down to the next rib. A week later the VEPTR on the right side came off and broke the rib. Her doctor re-implanted the rod that had eroded through the rib and reattached it on the rib below, like the other side had been. He also went ahead and expanded the VEPTR, she was only a month away from her expansion date.
On October 30, 2006, Kylie underwent soft tissue releasing of the hips, back of both knees, and her left shoulder. With myopathy of the muscles, her soft tissues don’t grow at the same rate as her bones. The soft tissues become contracted like tight rubber bands and require to be released with surgery. This is something she will have to do again until she is done growing.
Kylie hit another obstacle with her VEPTR. Her kyphosis had drastically progressed right above where the VEPTR is implanted to the ribs. Her upper back and neck had completely collapsed. This was a complication due to her myopathy. She doesn’t have the muscle strength to hold up any unsupported parts of her spine, therefore that part of her spine collapses. Both of her doctors agreed that she needed to wear a brace with neck support so her neck didn’t worsen and get her on the schedule at Shriner’s for another Halo Traction as soon as possible.
On January 8, 2007, she underwent Halo Traction number four. She was able to keep the VEPTR in while being in traction because the area that needed to be straightened was above the VEPTR. This was the first time a Halo Traction was done with the VEPTR still implanted. Everything went very well. Her upper back and neck were corrected back to the way it was before collapsing.
Now the plan was to expand her VEPTR and move it to higher ribs to get better control, put her in a Halo Brace for two months while she healed from the surgery, then put her in a brace with neck support.
On February 8, 2007, she had her VEPTR expansion and revision surgery. Things did not go as planned. Kylies surgeon was unable to move the VEPTR up any higher. Her kyphosis would not allow it. He couldn’t get the left side to the next rib and the right side he couldn’t get it to stay on the rib. It immediately popped off and broke the rib. The VEPTR was put back on the original ribs as before. Kylie stayed in traction with only half of the weights as before, until the end of February. Then the Halo Brace was put on and she went home.
Kylie was in the Halo Brace for three weeks, we noticed that her VEPTR on the left side was starting to bulge out more than normal. I took her to get an x-ray to see if the VEPTR was coming off or if it was already off. The VEPTR was still on, but barely. This was happening because her upper back and neck, once again, was collapsing. No one could believe that it had collapsed with a Halo Brace on.
On March 26, 2007, Kylie underwent her fifth Halo Traction to correct her collapsed spine once again. She was very happy to have her Halo Traction back. Kylie told us that the reason she loves being in the Halo Traction, was because it allows her to move and do things she can’t do without it, because of her weak muscles. She is very proud that she holds the record for having the most Halo Tractions for spine straightening in the United States, by a long shot.
On April 23, 2007, we had no other options but to have the upper part of her spine that continued to collapse, fused. She underwent a fusion from T1 to T5. Her surgeon since birth performed the surgery. The area was reinforced with wire and she has a rod that runs alongside it. Her skin was closed with forty staples. Her skin has been cut so many times in that area that it is solid scar tissue. The staples hold the skin together better. The Halo Traction was left on for two more weeks with half the weights.
Kylie’s recovery was amazing! She was up and on the go four days after the surgery. She finally was able to go home on, May 11, 2007.
Since being home and recovered, she is a lot weaker than she was before. Although she was very active in her Halo, being in traction for so long had allowed muscles that couldn’t be used, weaken. She can no longer do some of the things she used to do before the last two Halo Tractions. This is very devastating to us. She continues to do therapy on a daily basis, to maintain what muscle strength she has left.
On September 17, 2007, she had her VEPTR expansion and another revision of the left side. The VEPTR on the left side continued to migrate and start to come off the rib. Kylies doctors tried to attach a growth rod and move the left side off the rib and over to the area of her spine that had been fused. They were unable too, they didn’t have the right size of connector, so they re-implanted it back on the rib in a different spot. A week later the bulge was coming back more and more each day. By the end of the week, the left VEPTR had come off once again. October 1, 2007, she underwent revision surgery again. This time her doctor had the right connectors. He removed the left VEPTR, connected a growth rod to the part of the device that is attached to the pelvis, attached it over to the bottom of the rod that runs along the fused area of her spine.
So far, things seem to be working! She is scheduled for a VEPTR/Growth Rod expansion in April 2008.
Kylie’s doctors have done so much for her. There are so many doctors out there who would have never of done half of what these two have done for her. They are both determined to do whatever they need to do to allow her to get the growth she needs, before she undergoes a full spinal fusion.
We’re not sure what the future will bring for Kylie, she is fighter. She has overcome so much in her ten years of life. I have no doubts in my mind that she will continue to successfully overcome what ever life throws her way!
She is our precious little angel!!!
Thank you for reading Kylie’s story. We will keep you updated. Feel free to e-mail me at Nlether@yahoo.com with any questions regarding Kylie’s story.
Sincerely,
Natalie Lether
