CAPPS CranioKid Sierra

When I was five months pregnant with Sierra I had an ultrasound and was told that her head was lemon-shaped by the radiologist. We then went back to my OB and he did another ultrasound. He said my baby's head was cloverleaf shaped and probably had Craniosynostosis, I was shocked to say the least. He then sent us to a geneticist who said it wasn't cloverleaf, but she did have Craniosynostosis and there was nothing they could do until after she was born. This is when I started to search for information about Craniosynostosis and found capps. This site was a Godsend since CAPPS provided more information than most doctors.

Sierra is my second child. Her older brother, Ryan, is four years old. She was born on November 28, 2000 (her due date) and weighed in at 5 lbs. 3 ozs and was 18 inches long. As soon as she was born we knew something was wrong. The doctors immediately took her even before we had a chance to see her. She had trouble breathing, her eyes were bulging, and her head wasn't round. It looked like someone had put a clamp over her head from ear to ear. Sierra was immediately taken to the NICU because she couldn't breathe on her own, her apgar was a two at one minute, four at five minutes, and five at ten minutes.

Sierra was transferred to the Dayton Children' Medical Center that same day. I was only able to hold her about ten minutes before they took her away. Sierra was put on a ventilator until she had her tracheostomy (first surgery at ten days old). That same day, her eyelids got stuck under her eyeballs so she had to be rushed to Children's Hospital in Cincinnati where they stitched her eyelids partially closed (her second surgery in two weeks) to create artificial eye sockets. While Sierra was in the NICU she was tested for the cause of her problem, which turned out to be Crouzon's Syndrome. Sierra ended up staying in the NICU for three weeks until we were trained to take care of her at home. She was finally released on December 20, 2000. Her first night home she threw up in her trach, which scared us to death.

We returned to Children's Hospital in Cincinnati on December 27, 2000 to have Sierra's first cranial surgery (her third one in a month). The hardest part is letting the doctor's take away your baby for surgery, it doesn't get any easier. The only way you get through it is knowing that this surgery will help your child. Sierra's Craniectomy took a little less than two hours. We had friends from our church come while Sierra was in surgery, it helped passed the time. We were able to see Sierra about a half hour after surgery in the recovery room before she was taken up to the PICU, her eyes were still open and she looked like she was wearing a turban. After 24 in the PICU, Sierra was transferred to the NICU (we had asked if she could go back there since we were familiar with the nurses, since she had only been gone one week). Sierra's eyes only swelled shut for a day before the swelling starts to go down. She was given morphine the first day for pain and then Tylenol after that. Sierra recovery went smoothly and we were released on January 1, 2001.

Sierra went back to Children's Hospital for another Cranial surgery on June 4, 2001 (her fourth surgery in six months). She had a CVR/FOA which went well. The surgery lasted 5 !/2 hours. This time around we knew a little more of what to expect. Sierra's eyes swelled shut for a whole week (swelling increases more with additional surgeries). Sierra had swelled up so much we had to delay her trach change a couple of days. Sierra seemed to be in more pain after this surgery. Every time anyone would touch her in the first four days she would cry. Finally after four days I was able to hold my baby again. We expected the same rapid recovery as last time, but there was several complications (fever for nine days, blood in her stool, vomiting, low sodium levels, high white blood count. She had all the symptoms of an infection, but the doctors could not determine its source. Sierra ended up staying in the hospital for 21 days (seven days in the PICU). On day 15, they finally found the infection after she had to go through X-Rays, CT's, and a Spinal Tap (to rule out meningitis). She had caught a bacterial infection called "pseudomonas" (not related to her surgery). The infection was in her head, so she was taken to emergency surgery (number five in six months) to clean up the abscess. Thankfully Sierra's eyes didn't swell shut the this time, praise God. We were very happy to go home on June 25, 2001.

Sierra did much better at home. She recently had surgery (#7) to partially open up her eyelids, and in about six weeks she will have another to fully open up her eyelids, hopefully before Christmas. Sierra has been in and out of the hospital 14 weeks out of 11 months, but she has proven that she is a fighter. God has watched over our little girl and been with us every step of the way.

In January of 2002 Sierra had her final bilateral tarrosaphy (unstitch her eyelids) done on January 16th (surgery #8). The following week she had her tonsils and adenoids taken out, an ABR and a Bronchoscopy (surgery # 9). We received more bad news the ABR confirmed that Sierra had severe to profound hearing loss and would need hearing aids forever. This is one area where the doctors would not be able to fix the problem.

In April after a rough winter and several ear infections her ENT decided it was time for tubes in her ears (surgery #10). Sierra had a really good summer and managed to stay out of the hospital for the entire summer. Three months was the longest stretch Sierra had made it out of the hospital. We were still dealing with all the reflux problems and the medicine not helping so we went back to talk to her doctors and decided the best thing was to have a fundoplication done to help protect her airway from all the acid. Her nissan fundoplication was done on November fourth (surgery #11). Since the Nissan was done we have managed to keep Sierra out of the hospital. Sierra is scheduled to have a midface advancement (Lefort III with internal distracters) done on July 7, 2003 (surgery #12). This surgery will help open up her airways and create deeper eye sockets and help align her jaws. We are hoping that in the summer of 2004 after an airway reconstructive surgery Sierra will be able to get rid of her trach.