Teagan Paige’s Story

Site created on June 14, 2005

(/visit/teaganpaige/mystory/)

(/visit/teaganpaige/mystory/)

Teagan was born 6 Sept 2004 and diagnosed with Hurler Syndrome (MPS-1) on 2 May 2005. She received a bone marrow transplant on 29 July 2005 at Cook Children's Medical Center in Fort Worth, Texas. Her 4 year old brother, Riley was her selfless donor.

Teagan was born on 6 September 2004 three weeks early at a healthy weight of 9 pounds 3 ounces (pictures (http://www.kodakgallery.com/PhotoView.jsp?&collid=778254858103&photoid=640064858103)). She had aspirated mechonium into her lungs when she was born and had a low pulse ox. Her delivery was very quick and she didn't get that extra 'squeeze' that most babies get during birth. She had to stay a few extra days in the hospital getting antibiotics and oxygen but, was able to go home at 3 days old. From that point on we always noticed that she experienced heavy breathing and had a constant snotty nose. I was using the bulb syringe on a daily basis. But, when I brought it up to the docs they assured me she would grow out of it. She also had a patch of hair on her lower back. An ultrasound was ordered to test for abnormalities but came back as fine.

In April of 2005 (7 months old) we went to the doctors to see about an ear infection she was getting over. We had just moved back to the State from 4 years in Japan. Our new Doc (Dr. Herman Shafer) looked thru her records and asked about her breathing issues. I told him what she had been experiencing. I also asked him about a small bump we had noticed on her lower back. We had been traveling a lot seeing family and I wondered if it had anything to do with her being in her carseat for extended periods of time. He didn't think so and ordered Xrays. The Xrays showed she definately had a 'gibbus deformity' on her back. After doing more research he came up with someting called 'Hurler syndrome'. He had seen it only once in his 30 years of practice, oddly enough in a patient a month before (Katelyn Van Ghle (/visit/katelynvanghle)).





On 2 May at 8 months of age and after a confirmation blood test (that took 3 weeks to get), she was diagnosed as having Hurler's syndrome (http://peds.wustl.edu/lsdc/mt.html) (MPS-1).

It was the single worst thing that any parent could ever imagine. When the doctor was explaining the disorder to us, all I could do was cry. He said the only thing that could help her live a some what normal life would be a bone marrow transplant (http://www.bmtinfonet.org/)... my heart sunk.

So, Teagan's journey began...



Signs of Hurler's that Teagan experienced

*very heavy breathing

*failed hearing tests

*constant snotty nose

*outtie belly button

*slow development

*patch of hair in lower back which turned into a small bump (Gibbus-Kyphosis)) by 6 months old

*'different' fingers

*'different' eyes

*an ear infection

*when she got sick... she got very sick and hard for her to breath

*stiff joints (couldn't put her arms above her head)



What is Hurler syndrome?

*Affecting 1 in 100,000 children, Hurler syndrome is a rare genetic disorder where the enzyme (alpha-L-iduronidase), which normally breaks down the mucopolysaccharides dermatan and heparin sulphate, is missing. These mucopolysaccharides build up in all tissues in the body causing progressive deterioration and eventual death.


*Hurler's children will, without therapy, experience profound mental retardation, coarse facial features and excessive hair growth, vision problems (due to clouded cornea), and severe heart problems. Other symptoms may include carpal tunnel syndrome, curvature of the spine, frequent runny nose, hernias, and hearing loss. Most patients die of heart failure between the ages of 5 and 10.

*To introduce alpha-L-iduronidase into the body, marrow or cord blood transplants are being explored. Transplants are performed to provide cells producing enzyme through the growth of normal, healthy blood cells. These blood cells appear to provide enzyme to other cells of the brain, preserving neurologic function. Following successful transplant, patients do not suffer cardiac deterioration, and the accumulated mucopolysaccharides in the liver, lungs, and marrow slowly disappear.
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More picture links

(if you have time on your hands)

Pregnancy (http://www.kodakgallery.com/PhotoView.jsp?&collid=545877288103&photoid=445877288103)(9) Teagan (http://www.kodakgallery.com/PhotoView.jsp?&collid=778254858103&photoid=640064858103)(59) First Halloween (http://www.kodakgallery.com/PhotoView.jsp?Urefreshkey=1119590581480&collid=848555799103&photoid=648555799103)(24) Father's Day 2005 (http://www.kodakgallery.com/PhotoView.jsp?Urefreshkey=1119590581480&collid=299422506203&photoid=889422506203)(69)

Admit to Hosp (http://www.kodakgallery.com/PhotoView.jsp?Urefreshkey=1122090361576&collid=631288996203&photoid=531288996203) (31) Harvest/transplant (http://www.kodakgallery.com/PhotoView.jsp?Urefreshkey=1122090361576&collid=831202527203&photoid=731202527203) (52) Bald head (http://www.kodakgallery.com/PhotoView.jsp?&collid=223550147203&photoid=913550147203) (20)

Hospital & PICU (http://www.kodakgallery.com/PhotoView.jsp?&collid=433343208203&photoid=333343208203) (69) T's 1st Bday (http://www.kodakgallery.com/PhotoView.jsp?&collid=538782948203&photoid=438782948203) (39) (http://www.kodakgallery.com/PhotoView.jsp?collid=545669009203&photoid=945669009203&&refreshkey=1127669515642)Break Out (http://www.kodakgallery.com/PhotoView.jsp?collid=545669009203&photoid=945669009203&&refreshkey=1127669515642) (49)























RMH (http://www.kodakgallery.com/PhotoView.jsp?&collid=44062293103.195770489203.1131076537839&photoid=985770489203&view=1&page=1&sort_order=0&albumsperpage=12&navfolderid=2005) (52) Halloween '05 (http://www.kodakgallery.com/PhotoView.jsp?&collid=44062293103.638981040303.1132239431426&photoid=538981040303&view=1&page=1&sort_order=0&albumsperpage=12&navfolderid=2005) (56) Home (http://www.kodakgallery.com/Slideshow.jsp?&mode=fromsite&collid=44062293103.820959451303.1134059459733&conn_speed=1) (http://www.kodakgallery.com/Slideshow.jsp?&mode=fromsite&collid=44062293103.820959451303.1134059459733&conn_speed=1)(65)

Christmas (http://www.kodakgallery.com/Slideshow.jsp?Uc=k8pjuy7.7jd8u9j3&Uy=cvb2bb&Upost_signin=Slideshow.jsp%3Fmode%3Dfromshare&Ux=0&mode=fromshare&conn_speed=1) (79) January no more tubes (http://www.kodakgallery.com/Slideshow.jsp?&mode=fromsite&collid=44062293103.819558113303.1137739019559&conn_speed=1) (85)



Newest Update

Journal entry by Stacey Pevler

I realize I haven't updated in a very long time. So much has happened since the last update. Teagan successfully had both carpal tunnels surgeries along with all 10 trigger digits released. After surgery on her right hand, she had the hardest time waking up from anesthesia. She was sick for about a day too. This is the first time she felt this bad after going under. The surgery itself went well and she recovered very nicely. Daddy also officially retired from the Air Force. He was offered a job at Vance Air Force Base in Enid, Oklahoma. So, we made the move. The kids have all adjusted well and have become active in different activities. Teagan started first grade and has done well. She is on an IEP, but it's not very evasive. She is on consult PT, receives OT & Speech. She is in a main stream classroom without any stipulations. We work with her daily at home and she is really doing well. She's reading at grade level and getting all "A's". She does require extra work, but still doing well. She and sister, Bailey were a part of a cheer camp for 1-5 graders at the local high school. They cheered and did a dance routine at half time of the basketball game. Teagan did great - I'll share the video. http://www.youtube.com/watch?v=lsLTZ4yYmQc&feature=youtube_gdata_player Hope everyone is doing well :-) Love- Stacey
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