Viewers Discretion Advised: This is a difficult video to watch. This is the worse that my child's seizures got. Please be aware it may not look like typical seizures but the damage done by the seizures is tremendous.
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.
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.
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.
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.
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. Supposedly people with seizures can 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.
I hope we never have to do this.
It scares me to death.
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. This is why we are so passionate about donating to the research! This poor children fight everyday and there has to be an answer out there. Please join us in this cause!
This is an edited page from my blog. Feel free to read more about this disease and my son's journey:
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.
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.
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.
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.
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. Supposedly people with seizures can 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.
I hope we never have to do this.
It scares me to death.
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. This is why we are so passionate about donating to the research! This poor children fight everyday and there has to be an answer out there. Please join us in this cause!
This is an edited page from my blog. Feel free to read more about this disease and my son's journey: