I thought I would do a post to answer some of the most common questions that we get asked about Anderson and his condition(s). Most people have never heard of his condition before and therefore don't really understand it. Some have asked these questions, while others I'm sure have simply wondered but not asked. Those of you who know me, know that I can explain things much better in writing than with words. So this post is dedicated to answering some of the most frequently asked/wondered questions about Anderson.
1. What exactly does he have?Anderson actually has 3 different diagnoses: Agenesis of the Corpus Callosum, Hydrocephalus, and a large interhemispheric arachnoid cyst.
2. What does all of that mean?Agenesis of the Corpus Callosum means that the piece of the brain that connects the 2 hemispheres and allows the right side of the brain to communicate with the left side of the brain (the corpus callosum) did not develop during pregnancy. Therefore, it is absent or missing (agenesis).
Hydrocephalus is a condition in which the amount of CSF (cerebrospinal fluid) in the brain is increased. It means that the body is not absorbing it like it should. Every person's body creates about 5 pints of CSF fluid daily, but it is then absorbed by your body. If your body doesn't absorb the fluid, for any reason, then it builds up inside the brain and can cause lots of damage due to increased pressure inside the skull.
Anderson's hydrocephalus, however, is not in a ventricle in his brain like most hydrocephalus. His is contained in a large arachnoid cyst. The doctors think that this large cyst grew and developed in place of part of his right hemisphere of his brain. The cyst is connected to his brain system, so it is not a self-contained cyst that can just be drained and be gone. It acts as part of his brain and collects CSF fluid daily.
3. So why does he have a shunt?Anderson's shunt is placed into the large cyst. When the pressure inside the cyst gets to a certain level from the CSF fluid, a valve on the shunt opens. When the valve opens, it allows the built up fluid to drain through a tube that runs down into his abdomen. The fluid is then absorbed by his body. The shunt does not drain the cyst completely. Once the pressure in the cyst goes back down to an appropriate level, the valve shuts off and quits draining the fluid. So the hydrocephalus and cyst are both the reasons for his shunt.
4. When you talk about shunt malfunction, what exactly does that mean?Whenever Anderson's shunt is not working properly, it's considered a malfunction. This could mean the tubing or the valve has become clogged with cells and tissue in the CSF fluid; it could mean that there is a kink in the tubing; it could be that a piece of the shunt has broken and is not working properly; or in Anderson's case it usually means that the catheter that is stuck into the cyst has come out of the cyst and is no longer collecting fluid like it should. You see, the catheter is kind of like a straw that they poke into the cyst- if the 'straw's" position moves to a certain angle, it is no longer able to do it's job. At that point, the fluide begins to increase and the cyst grows and begins to put pressure on his brain.
5. How do you know when the shunt has malfunctioned?Well, this part is hard. And I still questions myself every time. You see, when you have a child with a shunt, they don't let you leave the hospital until you fully understand the signs and symptoms of a shunt malfunction or infection. So, why is is so hard? Well, since Anderson's shunt is in a cyst and not in a ventrical in his brain (like they usually are), his symptoms don't exactly match the "typical' symptoms. So the doctors have left the responsibility up to Jamie and myself. "You know him. You know when something is just not right." Yes, it's a little scary, okay it's a lot scary. Because if it is malfunctioned, time is of the essence. Every day that we wait to decide if it's the shunt or not, that fluid is continuing to build up and increase pressure.
Also the symptoms in an infant are different than those in a toddler/child. So basically anytime that Jamie and I have that "gut" feeling that something is not right, we have to go through the ER at Vanderbilt to have the series of tests that he needs to confirm or deny our suspicions.
Also, if he is running a fever over 101 and his pediatrician cannot find the cause, then we have to go to the ER to check for infection. Although infection is usually only a threat within the 6 weeks after a shunt surgery.
6. What tests do they do in the ER to diagnose the malfunction?He starts by having a shunt series of XRAYS, which takes xrays of the entire shunt system from top to bottom to check for kinks or for the shunt to be broken somewhere.
Next he gets either an MRI or a CT scan. Usually they do the MRI. This gives them the pictures of the cyst. They are then able to measure the cyst to see if it's size has grown. It also gives them a good picture of the placement of the shunt catheter to see if it is still inside the cyst or if it has been displaced.
Finally, they come in and tap the shunt. This involves a very small needle that they put into the reservoir of his shunt and draw out fluid. This allows them to test the amount of pressure that is built up. It also tells them that the shunt is not working properly if they are not able to draw any fluid. If they do get fluid, it is sent off to a lab to be analyzed for infection and a host of other things.
Once all 3 of these tests have been completed, it is then determined whether surgery is necessary.
7. Will he always have the shunt or will he eventually outgrow it?He will most likely always NEED the shunt. But, if for some reason, it was no longer necessary, they would not remove it. WIth the amount of scar tissue that builds up around the tubing, removing it would be a bigger pain than simply leaving it in. Also, it would already be in place if he needed it to work again at some point.
8. So what about the Agenesis of the Corpus Callosum? How does it affect anything?The Agenesis of the Corpus Callosum (ACC) is what what diagnosed in our 20 week ultrasound. They were able to see that this piece was missing. ACC is actually more common than most people think. There are many people around the world who have the same (or similar) condition. With the increase in technology, it is becoming diagnosed more now during pregnancy than ever before.
ACC has a spectrum of symptoms almost as wide as the Autism spectrum. The symptoms range from mild to severe, and some have no symptoms at all. In fact there are adults who have an MRI or CT for some other problem, only to find out they have ACC their entire lives and never knew it.
Some ACCers are able to fully function without any major issues, while others are never able to do daily tasks such as self-feeding, walking, toileting, speaking, etc. The range of symptoms is mostly due to the different conditions and disorders that can accompany ACC. It also depends on if there is a chromosome deletion or abnormality.
9. Why were you told that Anderson would have no quality of life after he was born?Along with the ACC and hydrocephalus, which have many effects neurologically themselves, they were also able to see the large cyst right after he was born. The cyst was completely covering the majority of his right hemisphere of his brain. So much, in fact, that they felt that only a small portion of that right hemisphere had even developed. Basically, they initially told us that about 1/4 of his right side was brain tissue and the other 3/4 was this cyst that had grown in it's place. They knew that medically, only having 1/4 of one side of your brain, plus the ACC, plus the hydrocephalus, should equal MAJOR delays. What they didn't know (or express) is how big our GOD is, and how he is the GREAT PHYSICIAN.
So when I call Anderson our MIRACLE child, I am not just saying that. Medically, he shouldn't be able to do half of the things that he does. We still love when new doctors come in to see him after only reading his chart...it's priceless.
10. So has any of this affected him at all?Yes. Of course he had to have the shunt due to the Hydrocephalus and cyst. Along with the shunt comes all of the problems, frustrations, and worries with one of those. Anderson also struggles with his gross motor skills. He started Physical Therapy once a week at 6 months old, and just recently was released.
Why? Because doing activities like walking/crawling/etc you are moving both sides of your body at the same time..this requires the 2 sides of your brain to work together. But when the bridge that allows that communication is missing, it makes this much more difficult (the ACC). The brain is a wonderous organ, which can create new paths when needed. With a lot of repetition of movements/activities the brain creates new pathways to get the information from one side to the other.
Anderson has also been a part of the Tennessee Early Intervention System since he was a month old. I know that part of why he is doing so good is due to early intervention. Instead of waiting for him to be behind, we are able to be proactive. They keep a close watch on his developmental skills and milestones. We also have an Early Intervention teacher who comes to our house once a month to do activities with him to help him meet milestones. She also works with Jamie and I on things to do to develop needed skills. They are a great resource to have.
11. What does the future hold? Only one person truly knows that answer. But this much I do know...Anderson will be in the Early Intervention program until he turns 3. At that point, he will transition into a "Special Pre-K" class in the public school system, which he will be a part of until he goes to Kindergarten.
I also know we will continue with all of the issues that come with his shunt. I have come to accept that these ER visits will be part of our new normal. At 20 months old, he has had the initial shunt placement and 4 revisions. While I'm hopeful that this newest shunt system works for a long time, the longest we've gone so far with the same shunt is 11 months.
Beyond that, I really don't know. We don't know if he will have learning problems when he gets to school, or anything past that point. So we take everyday as it comes. We celebrate each small milestone as a great accomplishment. We are thankful each day for how well he is doing. We work hard each night on certain skills (right now it's speech). And other than that, we leave the rest of his story up to God. In my heart, I feel God has big plans for Anderson as he gets older, and I can't wait to sit back and watch the story unfold.
**Thanks for reading. If there are any questions that I didn't answer, feel free to leave those in the comments section and I will get to them. ***
