Asher Kenan was born in South Korea on April 7, 1995. He arrived in the United States that Labor Day and was placed into the arms of his adoptive family. We had read in his medical reports that his occipital area (the back of his head) was "flattened" and his foster mother had been instructed to position him on his sides when lying down. When he came, his face was absolutely adorable but when he turned sideways it was a startling sight. The back of his head was completely flat and seemed to end evenly with the back of his neck. Later that day we also discovered that he had no soft spot and that his skull seemed to be bulging out over the top of his ears. Still, we believe it was all due to the Korean practice of sleeping babies on their backs and that, in time, his head shape would normalize.
Four days after his arrival, we took Asher for a check up with our family doctor. I couldn’t help but notice the doctor’s worried look when he saw us in the hall. The doctor carefully examined Asher and then told me, "I think he is a brachy baby. We’ll need a skull x-ray to be sure". I reluctantly consented but asked few questions believing that the doctor was simply culturally biased and that the condition was purely positional. Late that Friday night, the doctor called us at home. His fears were valid. Both of the lambdoid sutures in Asher’s skull were fused. That was the first time I had ever heard the word "craniosynostosis".
Asher also underwent a CT which confirmed the diagnosis. We were referred to a pediatric cranio-facial team at Detroit’s Children’s Hospital of Michigan. We were scheduled to see pediatric neurosurgeons Dr. Canady and Dr. Ham and pediatric plastic surgeon Dr. Rozelle. In the mean time, we had so many questions. We did a lot of research, both on-line and in medical libraries so that we might better understand this condition, the treatments, and Asher’s prognosis. The information helped us a great deal, which is, in part, why we have put together this web site.
On November 17, 1995 at age 7 months, Asher underwent more than 8 long (especially for his parents!) hours of surgery. The cranio-facial team made an incision from ear to ear over the top of Asher’s head, like a head band. They only shaved a narrow strip of hair. Then, they removed the back two thirds of Asher’s skull, broke it into small pieces, and carefully repositioned them, mosaic fashion. After surgery, he went to the pediatric intensive care unit.
It was so hard to see that little boy with his head all wrapped up in a turban-like bandage, two drains, and all sorts of tubes and wires. He also required a blood transfusion. He was sedated and slept peacefully through most of the first night. The next 24 hours or so were difficult as Asher was very restless and had some pain. By the third day, however, Asher (now in a regular room) was once again playful and content. When they took the bulky bandages off, the change was remarkable! His head shape was so different! He had a very large soft spot, a rectangular shaped band along the upper part of the back of his head where there hadn’t been enough bone left to fill it in. But the back of his head was nice and round, with plenty of space for his brain to grow.
On the fifth day post-op, we were allowed to take Asher home for Thanksgiving. We had much to be thankful for. He continued to take antibiotics and pain medication for several days after discharge. His recovery was very swift. While I would have liked a helmet to protect his poor skull and soft spot, the surgeons said this would cause his head to take on the shape of the helmet and was not necessary. Asher had no complications from his operation. Asher’s skull still bulges out a little over each ear and his ears are somewhat displaced but he is extremely cute!
We had been told that there was a potential for developmental delays so we enrolled him in Michigan’s Early On program. Initially there was concern about his gross motor function because he also had anomalies involving the base of his skull and the top of his spine. Turns out, Asher is an exceptionally well coordinated child who is quite advanced physically. When his baby teeth came in, quite a few were malformed but functional. There are, however, two areas in which Asher has lagged behind: growth, and language development.
A hearing evaluation revealed a conductive hearing loss. Even though he had never had an ear infection, Asher's ears were found to be filled with fluid due to eustachian tube dysfunction. Our research reveals this is apparently not uncommon in children with craniosynostosis. On February 17. 1997, Asher had tubes placed in both ears and we were very hopeful that this would aid in his speech development. At that time, Asher had a spoken vocabulary of 4 words, but a sign language vocabulary of about 30 words. Signing was recommended to us as a bridge to spoken language and has been of enormous benefit in reducing frustration and establishing communication. He also began receiving speech therapy.
By June of 1997 Asher's hearing tested well within normal limits but his spoken vocabulary had increased by only 2 words (he could sign about 70). After extensive testing by several speech and language clinics, Asher was diagnosed with severe verbal and oral apraxia. Apraxia is a neurologically based speech disorder caused by subtle brain impairment or malfunctioning. For more information on apraxia see:
http://www.avenza.com/~apraxia/index.html . We were then referred to a pediatric neurologist, Dr. Koo, again at Children's Hospital. Since that time, Asher has also been found to have problems with sensory integration and complex partial seizures originating in the right parietal area and the right temporal lobe (areas that were affected by the craniosynostosis). A PET scan has confirmed significant damage in both the temporal cortex and the temporal lobe.Asher is now attending speech therapy four days a week and occupational therapy twice a week. He has made remarkable progress with his speech but it is becoming clear that this will be a life-long struggle for him. The OT seems to be helping with some of his sensory defensiveness. Asher began attending the Early Intervention Preschool in the fall of 1998. He was there for more than three months before he said a word. School is pretty overwhelming for him but he is slowly adjusting. He is very bright and a whiz at video games. Asher is still quite small for his age and very thin. The theory is that this may be due to an oral apraxia and sensory deficit based ambivalence towards eating.
The seizures were occurring up to 3 times a day but are now down to several a week. Asher was initially put on Tegretol which did control the seizures but caused him to lose his ability to speak for hours, sometimes days, at a time. He is currently on Neurontin which has been quite helpful. It looks like it will be a very long process of trial and error to find the right dose, combination, medication, etc.
After being so terrified at the initial diagnosis of craniosynostosis and so relieved after the surgery, we really thought we were finished dealing with craniosynostosis. In fact, we will be coping with craniosynostosis and its effects for a long, long time. Of course, this is not necessarily the case for all children with this medical condition. Asher's form of craniosynostosis accounts for less than 1% of all synostoses and so not that much is known about it.
In spite of everything he has been through, Asher is a joyful and clever child. He has the most wonderful laugh! He is that special kind of child that strangers seem compelled to make a fuss over. Asher is a true blessing in the lives of all who know him.
If you are reading this page, perhaps craniosynostosis is a part of your life too. We would love to hear from you!
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