Siobhan was born June 9th 1999 in Vancouver, Canada, 4 days late. Her birth was normal. We noticed at about 4-6 months old that she didn’t bear weight and, when her feet were on a flat surface, they pronated (rolled inwards) quite severely.
Our family doctor sent her to see an orthopedist at our Children’s Hospital, who explained that she would need orthotics when she started walking. Then, upon closer examination, he remarked how long her fingers and toes were. He referred us to a genetic doctor to have Siobhan checked for the Marfan syndrome.
The genetic doctor sent us to an Ophthalmologist for a ‘slit lamp’ eye exam and to a Cardiologist for an echocardiogram and x-rays of the hands, feet and spine. During this spine x-ray, Siobhan’s scoliosis was detected. She was 20 months old, and her COBB measured 23 degrees lumbar and 17 thoracic. Our orthopedist at the time felt it would get worse as she grew, but recommended the “wait and see” approach. Siobhan had an x-ray every 3 months for 9 months with no change. It was then recommended that we cut down on the x-ray exposure, and she would be seen every 6 months.
About two months after that decision, I noticed in a recent photograph that one of Siobhan’s shoulders was markedly “dropped” (uneven). We decided not to wait for another 4 months, and took her back for another x-ray. Her lumbar curve had increased to 35 degrees and her thoracic to 26 degrees. That’s when she went into her Boston TLSO, which she wore 23/7 for 9 months, with her curves maintaining at approximately 35°/26°.
But by one year later, her lumbar curve was 45°, and I was growing very concerned. Our orthopedist had originally told us that a spinal fusion would probably be necessary when she reached 12 years old, but later mentioned that it could be necessary when Siobhan was 10 years old. By our last visit, when Siobhan had faithfully worn the brace for 23 hours a day, 7 days a week, he was suggesting fusing her spine at 8 years old.
This is when we looked into serial casting as an option. Thanks to the Infantile Scoliosis Outreach Program’s website and the information I learned through Heather Hyatt, we found the only doctor, Jacques D’Astous, MD, who was willing to cast her in a plaster of paris cast (POP jacket). He applied Siobhan’s first plaster cast in June, 2004, at Intermountain Shriners Hospital in Salt Lake City, Utah, when she was 4 years old. At that time, her curves were measured at 32° lumbar/20° thoracic (in the cast). Her second cast was applied at the end of August 2004, reducing her lumbar curve to 8°/thoracic 17°. Her third cast saw her lumbar curve at zero/thoracic 25°, (measured on x-rays while in the cast.)
She is currently back in a Boston TLSO, which she wears 16-20 hours a day, and is getting lots of physiotherapy and swimming. Dr D’Astous will continue to follow Siobhan every 3-4 months and, when he feels her curves are increasing, will apply another series of POP jackets to buy as much time as possible for her grow.
Siobhan’s diagnosis is a “connective tissue disorder/possible emerging marfans/possible MASS phenotype.” We all know this means that her spine may just keep on curving, but we will all do everything we can to stall any surgery until she is older, much older.
Thank you for reading my little girl’s story. And if you have any questions please feel free to email me at firstname.lastname@example.org
January 2008 Update
Siobhan continued to wear a brace 16 hours a day, until November 2005, when her doctor suggested she be evaluated for VEPTR (Vertical Expandable Prosthetic Titanium Rib).
We’d done a lot of research and believed that this fairly new procedure would help Siobhan, and improve the quality of her life even more.
The Mehta casting had bought us time, reduced Siobhan’s curves, and had helped correct her rotation, but her connective tissue disorder meant that we needed something more. Her doctor explained that these VEPTR’s would literally act as an “internal brace”.
We knew this surgery would be invasive in the short term, and a major commitment in the long term. But we trusted Siobhan’s Orthopedist.
In May 2006, Siobhan was the first child to receive the VEPTR dual hybrid (attached to her iliac crests) at the Shriners, Salt Lake City. Her curves went from 34 degrees thoracic/ 49 degrees lumbar to 0* thoracic/ 12* lumbar – All the doctors were very excited! Us too!
Siobhan received a confirmed diagnosis of “Emerging Marfan Syndrome”, by the Genetics Dept of our Children’s Hospital in January 2006.
Her first expansion of her dual VEPTR’s in November 2006 went smoothly – 6 months after the initial implants. But by January of 2007 we started to notice that Siobhan seemed unable to stand straight anymore – She’d acquired a “stoop” that she didn’t have before.
We emailed back and forth to her doctors in Salt Lake City, who informed us that we may need to look at moving these 2 VEPTR’s from the pelvis, as for some children, this stooping was becoming a problem. Both doctors agreed that relocating them to the laminae should help Siobhan.
The laminae are two flattened plates of bone extending medially from the pedicles to form the posterior wall of the vertebral foramen
Intermountain Shriners found Siobhan an appointment at the end of April 2007, where her surgeon moved the pelvic devices. Surgery was harder than expected, but as always within a couple of weeks, Siobhan bounced back!
Siobhan is doing well in Grade 3, tap dancing and playing the piano – Having the wonderful childhood she deserves.
Jacki, Siobhan’s mum