Erin’s Story

by mmcnett | Oct 20, 2014 | Congenital Scoliosis, Infantile Scoliosis, Infantile Scoliosis Blog, Life with Mehta Casts, Our Stories

Erin was born on February 2, 2002. It would have been her Maternal Great Grandmother’s 95th birthday, and Erin was the first girl on the paternal side of the family in 90 years!

At approximately 37-38 weeks, I had an ultrasound to make sure things were on track. There was a question raised from that ultrasound as to whether or not the baby had both of his/her kidneys. I was instructed to mention this to the L&D nurses after the birth of the child. We did not know until the birth whether it was a boy or a girl.

I was induced on Feb 1 at about 10:45 pm. This was due to my blood pressure going up and the baby was due on the 2nd anyway. L&D went fine, although the epidural was a waste of time – again. The same thing had happened with the birth of my son.

When Erin entered the world, and Dr. Lee announced “IT’S A GIRL!” John, my husband, said “Are you SURE?” Yeah, they were sure! After a few minutes of cuddling, the nurses took her to be weighed, measured and cleaned up. They all knew there was a question of a missing kidney, so while the nurse was cleaning her up, she said “She’s peeing!” What great news.

The next day, after an uneventful night, Erin was whisked away for an ultrasound. This confirmed, that in fact, she did only have one kidney. When they brought her back, I was filling out all the paperwork about lead, and hearing etc. The nurse said that even though we didn’t fall into the criteria for the newborn hearing test screening, we should probably have it done since kidneys, ears etc all form at the same time. We did and her hearing was fine.

At her 3 month checkup, her pediatrician noticed that her head would tilt to the right, he suspected torticollis, but wanted to wait till the 4 month check up because babies have such wobbly heads to begin with. At 4 months it was confirmed. Erin also had hip xrays because there was concern that her hips were not in alignment because one leg appeared longer than the other. Her hips were fine.

We started physical therapy for the torticollis, and when Erin was about a year old, her PT was rubbing her back and thought she felt a curvature of the spine. After xrays, we went to an Orthopedist who confirmed that Erin had congenital Scoliosis with hemivertabrae. After hearing the diagnosis, we switched orthos to Dr. Alvin Crawford (Dr. C), department head of the Ortho dept. at Cincinnati Children’s Hospital Medical Center (CCHMC). For about a year, we did the wait and see with xrays every 4-6 months. Erin was also seen by the Human Genetics dr, but it was discovered that none of this could be tied to Genetics. Dr. C also said Erin had Klippel Feil Syndrome, which is characterized by a low hairline and shortened cervical vertebrae.

When Erin was 2, Dr.C decided to put her in a Modified TLSO brace with Cervical Head Collar. this was not to help her spine, it was to stop the facial dysplacia that was occurring due to the torticollis. During this time, Dr. C explained what our options would most likely be: fusion, rods, or a new procedure that was still in clinical trials, the Vertical Expandable Prosthetic Titanium Rib or VEPTR. At this point we also discovered that Erin had fused ribs.

John and I were not thrilled with the idea of fusion and rods seemed so old fashioned. So I began trying to find out anything and everything that I could about the VEPTR. I found out that one of the trial sites was Boston’s Children’s Hospital. So I made a few phone calls and got Erin an appointment with Dr. John Emans. She and I flew to Boston with all her xrays and scans and met Dr. Emans in May of 2004. Based on what I had, Erin was not a candidate for the trials. But Dr. Emans did say he wanted to see her again if we ever visited (my family lives in Massachusetts).

When we returned, Dr. C was glad I had checked it out, but we were still looking for the best option for Erin. In 2004, Dr. C presented her case to three (3) different symposiums in different parts of the country to get other suggestions, advice, recommendations. In September 2004, the VEPTR was approved by the FDA!!!

In January of 2005, Erin had another 3-D CT scan. This one, Dr. C sent to Dr. Robert Campbell, co-creator of the VEPTR in San Antonio, TX. Dr. Campbell got back with Dr. C and said Erin would be a “picture perfect” candidate for the VEPTR, and would be perfect to be the first patient to receive it at CCHMC!!

On March 14, 2005, while I was on a cruise ship heading to Cozumel, Dr. C’s office called with the news that Dr. Campbell would be in town on March 22 to do Erin’s surgery!! John said yes – and did try to reach me on the ship to no avail. When I got back to my sister’s in Tampa, there was a message for me to call home. I was absolutely shocked when John told me the news. I flew home and spent the next week getting things set up for Erin’s surgery – having pre-op work done, getting coverage for me at work, finding someone to watch Patrick etc.

Everything worked out and Erin had her surgery and she came through it with flying colors!!! Protocol says the child will be on a ventilator for a day or two – Erin came out of surgery off the vent. . Protocol says the child will be in the hospital for 14 days or so, but we were only there 10. She was in PICU for 2 days then on the regular floor for 8 more. She was also a celebrity as the Cincinnati Enquirer did an article on her and we posted it on her door.

Her recovery has been great and it is amazing how tall and straight she stands. She will have to have expansion surgery every 4-6 months, but that should go just as well as the initial surgery. She is not able to play contact sports, but if you saw her on the playground with her friends, you would never know that she has had spinal surgery!
Gail

Devyn’s Story

by mmcnett | Sep 20, 2014 | Congenital Scoliosis, Infantile Scoliosis, Infantile Scoliosis Blog, Life with Mehta Casts, Our Stories

Devyn was born October 3rd, 2005. The pregnancy and delivery were anything but normal. I had a sub-chorionic bleed until my 5th month of pregnancy that almost caused me to miscarry. The delivery started out normal until I started to push…. He got stuck twice and then was not breathing when he was born. After they resuscitated him he was fine except for his purple and blue bruised face.

Devyn seemed very healthy as a baby except for a milk allergy. He rolled over early, sat up at 5 to 6 months, and started crawling at 9 months, and walking at a year. It was then when we noticed something alarming. He stood in front of me one day in just his diaper, when I noticed that his back looked different. I mentioned it to my husband who knew it was there, but found it easier to pretend there was nothing wrong. We had a family get together for Thanksgiving, and I asked my mom and sister what they thought. They too said it was not normal and something was wrong.

The first week in December we had an appointment with his pediatrician for his shots, and I asked her to look at his back. She said that it was curved and that we needed x-rays and to see an orthopedist. Sure enough, the x-rays showed that he had scoliosis with a 24-degree curve in the lumbar area. The Orthopedist wanted an MRI to see if the spine was tethered at the base. So on January 11th we had the MRI done, what an experience—that drained us all. The results showed that the chord was not tethered and he had abnormally shaped cerebellar tonsils. The Orthopedist said that the pediatrician needed to refer us to a neurosurgeon and that she didn’t want to do anything at the time for the scoliosis, and to come back and see her in 4 months. So we saw our pediatrician again and she said we were not going to watch and wait. She sent us to Children’s Hospital in Denver to see an orthopedic surgeon for the scoliosis and a neurosurgeon.

We saw the orthopedic surgeons PA on February 2nd, 2007. He told us that he could definitely do something to help correct the scoliosis from progressing. He explained the early treatment process with the POP jackets and how it works. He said that we had to see the neurosurgeon first to make sure there were no neurological problems that would be affected by the casts.

March 7th we saw the neurosurgeon, who informed us that Devyn had Chiari Malformation along with his progressive infantile scoliosis. Chiari Malformation is where the lower part of the back of the skull does not form properly and is flat rather than curved. This pushes on the cerebellum (part of the brain) and causes the cerebellar tonsils at the base of the brain to descend downward into the spinal canal instead of under the skull and can cause pressure. This can cause the balance to be very unsteady, headaches, numbness, and in severe cases paralysis. He did not believe that the two were related because the Chiari malformation was not severe and not causing pressure on the spine. He told us at first that he wasn’t sure that surgery for the Chiari was necessary. He then asked if he could keep the MRI and share it with his colleagues to get their opinion. Of course, we had no problems with that—I want the best for my baby. The week after our appointment I received a call from the doctor himself. He explained that he shared the films with the other doctors and they decided that the surgery was necessary to make sure the two were not related.

On March 29th, 2007 Devyn had decompression surgery for the Chiari Malformation. They opened up the back of his neck and cut out half of the C1 vertebrate, and took out the left cellebellar tonsil, and cut the right one back. They also opened the Dura, which is on the back of the brain. He spent four days in the hospital, and is doing great. We have noticed small improvements of things we never knew were wrong with him. His balance has improved greatly; he used to scream in the car non-stop and now is not bothered by car rides. These are things we didn’t know because he was too young to tell us.

Although these things are better his scoliosis had not improved. They did x-rays on March 7th after his appointment with the neurosurgeon. His curve had progressed to 37-degrees and his rotation was at 30 degrees. His right foot turned in like a pigeon when he walked and seemed worse every day. His curve also looked like it was getting worse.

We were finally cleared for Devyn to get his first POP jacket on May 9th 2007. They did not do x-rays when they casted. The doctor tries to expose them to as little radiation, as possible. They were figuring the curve at a little over 40 degrees at the time he got his cast. He did better than we had expected in the hospital, a little mad at first but got used to the idea that it wasn’t coming off. The first week was a little rough as he had a hard time adjusting and moving in the cast. He couldn’t bend over or sit on the floor and sleeping was really hard at first for him, as well. After the first week he improved, and he learned how to accomplish something new every day. After two weeks he was back to his old self, getting into everything and climbing on everything.

On June 8th we went in for his first x-ray in cast. The doctor walked in and said, “refresh my memory, what did we start at?”   He handed us the x-ray and told us to guess….. I was amazed, the x-ray looked almost straight! He said it measured 15 degrees and hopefully one more cast and a good growth spurt should do the trick. On July 6th Devyn received his second cast.  They didnt x-ray on the day of casting this time either, they used the numbers from his last x-ray. On August 9th they took his in cast x-ray. This time it measured 10 degrees. As you can imagine we were very happy. The doctor said lets do one more cast to see if we can go any lower or if we hold this degree.

On September 10th Devyn received his third cast. We followed up on October 5th for his x-ray. The curve was holding at 10 degrees. We are still so happy because it’s better then when we started! On October 27th they took off Devyn’s cast and molded him for a brace. He received his first brace on October 31st. They did an x-ray to make sure the brace was adjusted correctly. He still measured 10 degrees. He wears his brace all the time except for bath’s and swimming. We went in on November 30th for his first x-ray with the brace, this time they had us take an out of brace x-ray. He is still holding at 10 degrees.