This is defined as a "catastrophic childhood seizure." Although the name sounds simple and perhaps the child's spasms or seizures may seem minimal they are anything but that. These seizures are developmental. They start during a specific developmental stage of a child's life and continue until about the age of two and then turn into other epileptic issues. These seizures generally cause developmental regression in the child. Milestones they may have reached are lost and they quickly fall behind (Bridger could smile, bear weight, and lift his head up on his tummy before the seizures started).
The seizures are characterized by specific patterns seen on an EEG called hypsarrhythmia. This is known as chaotic brain waves. Often times the seizures will look like mere reflexes. The child will generally crunch forward or arch back and extend/stiffen his arms and legs. It will most likely happen in clusters and can have up to several a day (Bridger had up to ten a day with clusters of 45+).
Most children's (70%) spasms are symptomatic. This means that the seizures are tied to something else such as cerebral palsy, birth injury, brain injury, genetic disorders, autism, tuberous sclerosis etc...The outcome for this type depends a lot on the underlying disorder.
The other 30% are stated to by cryptogenic or idiopathic. I just discovered there are differences between these two categories.
Cryptogenic means that there are suspected underlying abnormalities but they are unable to identify it.
Idiopathic means that there is no known cause for the spasms. In other words you have sh*** luck.
The good thing about this however is that children who are cryptogenic or idiopathic have the greatest likelihood of developing normally and being seizure-free once the seizures are stopped.
Infantile Spasms Treatment
There are several options for seizure treatments but the only two products that have been FDA approved are ACTH (H.P Acthar Gel or Acthar) and Vigabatrin (Sabril). The problem with any of the treatments that are done is that they honestly have no clue why they work (when they work) and what exactly they are doing to the brain.
ACTH (Acthar)
This treatment is believed to possibly work by assisting the body in creating natural hormones and directly effecting the brain. The goal of this is to cease the spasms and improve the hypsarrhythmia patterns.
This treatment is given through injection (shots). Parent's are taught how to give the shots correctly and give them to their children 1-2 times a day. It is difficult to say how soon the treatment will work but many see results within the first three days and see a cessation of the spasms and hypsarrhythmia patterns by two weeks.
There are some promising studies about this treatment. The problem with many of the studies is that their study samples are not large or consistent which makes it difficult to say that this treatment is reliable.
Side effects include and are not limited to: crushing syndrome, obesity, hypertension, irritability, gastric ulcers, growth retardation, immune system suppression, and risk of infection.
We tried this but it did not work for Bridger. It has been the answer to many parents prayers though. Just because it does not work for our child does not mean it won't work for someone else.
Vigabatrin (Sabril)
This treatment was even more of a pain to figure out exactly what it might be doing to the brain. It is possibly affecting a chemical in your brain called gamma-aminobutyric acid (GABA). I haven't figured out if it increases GABA or just stabilizes it. Basically GABA is considered a never calming agent and is able to help keep the nerve activity in balance inside the brain. The idea is that Vigabatrin is able to calm down the nerve activity (the hysparrhythmia) in your brain. Doing this prevents the seizures from happening. Again many see results within the first three days (we saw results after the very first dose).
This is a very effective treatment for children with tuberous sclerosis. The same problem with ACTH though applies to Vigabatrin. Small study samples and inconsistent results.
Another problem with this treatment is the very likely side effects. 30% of participants that take Vigabatrin end up with vision problems. This includes loss of peripheral visions and blurry vision. This is the medication we chose after the prednisone treatment failed. They will send you lots of information and warnings about the risk of vision problems. Unfortunately you have to decide for yourself if the damage of Infantile Spasms cause out weigh the possible risk of vision loss (know that IS can or will eventually cause vision problems to your child if not stopped).
Other side effects include but are not limited to: sleepiness, weight gain, headaches, dizziness, confusion, speech problems, aggression, irritability, depression, paranoia, speech problems, flaccid muscles, and vomiting.
We used this treatment and it worked fairly quickly but Bridger's seizures broke through after 2 months. We were lucky enough to have a neurologist who let's us do whatever we want and by combining this with phenobarbital Bridger's seizures stopped again.
Other Treatments
Steroids
There are several oral steroid treatments that neurologists chose to use such as prednisone, prenisolone, and methylprednisolone. They have seen positive results in about 1/3 of children who have done this treatment. It is a good add on drug with another therapy such as Vigabatrin or ACTH.
This treatment did get rid of Bridger's spasms but it did make them less severe and less frequent. We chose to do combo of Vigabatrin and Prednisone. It seems to have worked so far!
Our neurologists chose to the prednisone because they thought he was not a severe case of infantile spasms and would be cured quickly. As I have said before Do Your Research! These is not an FDA approved treatment. It can work but I'm telling you if it has not worked within the first week or two try something else soon. If this treatment has worked for you congratulations. I envy you. You are sooo lucky!
The side effects for this are similar to the side effects included in the ACTH treatments. Minus infections though.
Ketogenic Diet
This is basically a high-protein, low-carb diet. It has been around for a very long time. People with seizures may respond very well to this especially when medical treatments don't work. It is a tough diet to stick to but it seems to be a great last resort for people. It can cause kidney stones and your child won't exactly grow like they need to (which makes sense you're only eating protein).
Surgery
A final option that can be considered is surgery. The surgeon would basically remove the brain abnormality in the child. This can potentially stop the seizures and improve the developmental outcome if the surgery can be done safely. It amazes me the things we can do with the technology and knowledge that we have.
Final Words
In a nutshell this is what Infantile Spasms is all about. There is not a whole lot of research on it and anything they do have is not considered reliable information. It is a lot of guessing and hoping for the best in the end. If there is any new information you would like to add to this feel free. If you ever need someone to talk to you or have questions you are more than welcome to email me at:
lucretiastallings@gmail.com
You are not alone.
Websites about Infantile Spasms
Understanding Infantile Spasms
Infantile Spasms Center
Websites about Vigabatrin
FDA Information on Vigabatrin
Sabirl (Vigabatrin)
Vigabatrin for Epilepsy
Sabirl (Vigabatrin) Website
Websites about ACTH
Official Acthar Website
Homecare Handbook on IS and ACTH
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