“Hey, you guys drive standing up, right?” Hardee Harr Harr. *Eyes Rolling*
When I check my email today, I found Candi left this message:
We are having an MRI done today at 3:00. Tonya - we will call you
tonite and let you know what the results are. Bryan’s dwarfism specialist
came back into town after the holiday and said we made the right
decision…and he ordered the MRI today. So hopefully we will know something
soon.
Candi
YaYa to Bryan
This has been the LONGEST weekend! Between being anxious about what is going on with Bryan and then our own drama, finally good news!
Over the weekend I talked to Brandy several times and she expressed concerns most of us parent’s with dwarf children echo at some point in their lives. Medical specialists who are so proficient in what they do that they are unwilling too look at any other option or talk to anyone else with more knowledge.
Understand, I am not underestimating these doctor’s abilities. In the end, Bryan may need surgery, at some point. But looking at the information Brandy gave me about Bryan’s head size and demeanor over the last six months, had those of been Knoah’s, I would have rejected the surgery. Bryan’s head size hasn’t jumped percentile’s, he is staying on his curve for Achondroplastic children. Today, he still remains in the 48th percentile. He isn’t showing any signs of Hydrocephalus as far as his behavior. His fontanel is soft, they can feel Bryan’s heartbeat through it. All good signs.
I was told to watch for a jump in Knoah’s head size. For example, at 6 months, Knoah was at 50cms, today he is at 54cms. A slight increase but not alarming. He remains within his curve, although he is moving into another percentile. This is why we have to watch it.
Some of the signs you watch for are irritability, headaches, sleepiness, bulging fontanel (soft spot), sunsetting of the eyes (basically, the child will move their eyes downward and they may or may not move back into a normal position).
Some Neurosurgeon’s see a large head and it causes concern, as it should, but in Achondroplastic individuals, a large head is the hallmark for Achondroplasia. Each person is different. Some people will have a smaller cranium that other person, but it will still be larger than an average height person.
That is why we use a different growth chart to measure their progress. If you click this highlight, there is a chart that has the head size growth chart. PRINT IT OUT, if you don’t have one! It also has growth charts for boys and girls as far as height, weight, etc. Print the whole booklet out. Give it too all of your child’s doctor’s. Even the geneticists if they don’t have one.
You are the parent. You are your child’s advocate!
An old wives saying is “Babies are not born with a book.” Well, in our case, they were! That works to our advantage!! We know when to expect certain milestones, we know what is abnormal and what is common. We know what medical complications can arise and what to look for. Read it over and over again!
Bryan avoided surgery because his mother and Ya Ya knew what the doctor said to them didn’t seem right. Brain surgery! This isn’t just removing a splinter, it’s Brain surgery!
When Bryan has his MRI, one of the things his doctor is going to look for is Stenosis of the Foreman Magnum (FM). If Bryan’s FM is narrower than most, the surgery he will need is called Decompression. Decompression surgery is one of the most common surgery individuals with Achondroplasia have.
In stenosis of the FM or narrowing of the FM, the FM looks like an old fashion key hole. Larger on one end than the other. In people, particularly infants with Achondroplasia, 10% of infants do not survive their first year due to compression of the FM. The reason for this is that the spinal cord in ALL people run through this hole.
*Project* Take your right hand and make an OK sign with your thumb and index finger touching. Take take your left index finger and put it in the center of the OK sign. It looks like a round circle. This is what your FM looks like. Your left index finger represents your spinal cord. The right hand represents the base of your skull. Now, take your right hand and push your thumb and index finger together even more. It should look like a tear drop. This is what the FM looks like in people with Achondroplasia. Put your left index finger back into the center. This is Stenosis of the FM.
If the spinal cord becomes trapped or pinched in the smaller part of the FM, it cuts off the brain’s signal to continue breathing. Thus, infants die due to lack of oxygen. The body doesn’t respond to wanting to breath because it doesn’t know it should.
The FM can also cause an increase in fluid in the brain. The fluid everyone has is spinal fluid. It’s main purpose is to bathe the brain. If the FM is too narrow, fluid can back up and increase the head size. It doesn’t have anywhere to go and isn’t able to be reabsorbed by the body. The head size increases and mimics Hydrocephalus if the FM hasn’t been checked.
Another indication of compression of the FM is extreme hypotonia. In achon’s that exhibit compression of the FM, they will be even farther behind in their gross motor development. Where the average achon usually walks alone without support about 22 months, a child with stenosis of the FM will still be crawling at 28 months. They will be behind.
That is why is EXTREMELY important to read everything that deals with your child’s dwarfism, they are very special child, in more ways than one!
