Infantile Spasms seems innocent enough, at first. Most people don't think of epilepsy when they first hear of "Infantile Spasms" because  the name is very deceiving. Infantile Spasms is considered a catastrophic seizure disorder that is often resistant to medicine therapy, and usually results in mental retardation.

Infantile Spasms is the only seizure disorder where the seizures occur in clusters. They typically affect children from 4-7 months of age, but can occur earlier or later in life as well. These spasms can look either like an exaggerated startle reflex - where the arms flail outward - or a "salam bow" where the trunk flexes forward and the arms and legs draw inward. In Cody's case, they looked more like gentle "waves" were washing up under his torso - his arms would "flow" up on each side and his head would tilt to the left. The videos of his seizures on this site shows how hard to detect they can be. There is an extremely mild seizure + a classic cluster of seizures.  The "clusters" lasted 3-5 minutes with 40+ individual seizures in that time frame. Cody averaged 9-12 clusters daily. Infantile Spasms usually has a characteristic EEG pattern called Hypsarrhythmia. The photo above is Cody having an EEG where they discovered Hypsarrthythmia all over the place.  Now it is gone, thankfully!

In terms of the cause of these seizures - children are deemed either "symptomatic" or "idiopathic." The symptomatic children make up the majority of all kids with i.s. Symptomatic means "known cause" such as brain malformation, brain tumor, chromosonal issue, enzyme deficiency, etc. A child who is symptomatic has a 95% chance of retardation. Only a small percentage of children with i.s. are idiopathic or "no known cause." Of the children who are idiopathic, 40% will turn out "normal" - 60% will have developmental delay, retardation, etc.   Cody began "idiopathic" until we discovered a brain abnormality that made him "symptomatic." 

The seizures typically occur when waking up or falling asleep. No one knows why. They also occur throughtout the day - but most often are triggered by drowsiness. The greatest goal with infantile spasms is gaining seizure control which comes differently to every child.  The most common first line of treatment is ACTH steroids. Vigabatrin is popular in other countries, but is not FDA approved in the U.S. yet.  After that, common meds used are zonegran, depakote, topomax, and lamictal. The ketogenic diet also sometimes works for seizure control (it's a high protein, low carb diet for kids.)

This disorder is mysterious - there are no rules or formulas. Little is know about i.s. - what causes it or what cures it. Basically, it is trial and error, and some patients will never achieve real seizure control.  If seizure control is achieved, some children can re-gain what they've lost developmentally due to the seizures...some stay frozen.  The outcome depends largely on what caused the spasms.  For Cody, the brain abnormality could get better or worse over time.  Some children can function WITH such an abnormality, others cannot.  An added variable is how much brain damage the seizures do before seizure control is gained.   Luckily children's brains are incredibly resilient so there is hope that some of that damage can be reversed - if we ever gain seizure control.  One other variable is the anti seizure meds that children have to remain on (usually for up to 2 years) after seizure control is gained.  Those drugs affect development greatly...their side effects include lack of coordination, sedation, hyperactivity...the list goes on and on.  Cody has also suffered from many movement disorders as side effects of these drugs.  It seems every week he leaves one "tic" behind and picks up another.  Each one requires a new EEG to make sure they are not seizure related.  So far, we've chalked up all his funny movements to the meds.  He's gone through eye squinting, constant head turns to the left, and more.  He is a handful and is in constant motion.  He has trouble sleeping and as a result - we don't much sleep either.  Luckily, he has an angelic disposition despite all these obstacles and has never had a tantrum or outburst.  He is affectionate and a dear little soul.  His seizures are now in 2 varities:  "small ones" are about 15 seconds long and are called "tonic" where he stiffens all his limbs, his eyes roll back, and then it's over and he is just a bit disconcerted for about 15 minutes.  "Big ones" are up to 2.5 minutes long - he goes tonic - shakes - his eyes roll - he screams - then stops breathing and turns blue.  We can't do anything during these horrific events but hold his stiff little hands and pray for him or sing to him.  After he collapses for about an hour in our laps.  These big ones wipe him out for about a half day - and on days when he has 4 or 5, he just lays on our laps the whole time.  When he has more than a few that cluster close together, we have to give him rectal diastat (valium) to keep them from gaining too much steam. 

For children like Cody, the prognosis is a question mark.  We have to wait, watch and see.  Will  he continue to develop?  Will the brain abnormality wreak havoc?  Did the seizures do too much damage to ever achieve "normalcy?"  Cody had i.s. for a year - then at the 1 year mark he went seizure free for 3 months.  They slowly returned, but not as infantile spasms clusters.  They came back as startle seizures - then morphed into what he has now - tonic seizures.  This is often the case - a child has i.s. for a year or two, then it settles into a more garden variety seizure type.  Hyppsarythmia stops and a more normalized EEG replaces it - although still usually quite abnormal compared to "typical" kids.  The new seizure types are most often less catastrophic than the infantile spasms clusters.  However, without seizure control, development is almost always hindered.  Many children who start with i.s. go on to be plagued by other seizure types their whole lives.  The lucky ones find seizure control early on and can progress normally. 

Right now, at almost 4 years of age, Cody has never gone backward.  He has always inched along - and at times - really made great developmental gains (for him.)  Cody was diagnosed with autism at the age of 2 - autistic characteristics often are present with severe developmental delay.  That triggered a lot of research on my part toward therapies that work for autistic kids.  We began a home ABA (applied behavioral analysis) program for Cody in April of 2008.  For Cody, ABA therapy really works given it's high repetition and reward system.  We partner that with a heavy Floortime approach (Floortime is another common autism therapy.)  We also have a private speech pathologist and occupational therapist.  Cody's school provides a portion of his therapy in our home because his seizure condition prevents him from being educated in a public classroom.  We supplement the rest of the hours of intensive therapy out of pocket - costing tens of thousands of dollars per year.  We shoot for 35 hours of therapy per week.  That may sound like a lot of 'work' for him - but most of his therapy is play-based so he doesn't even know he's 'working.'  Though the cost is great, the results are wonderful for us.  Cody uses about 8 signs, can now match objects & pictures, understands activating many cause & effect toys, is more engaged and content than he has been in a long time. 

We still battle many behavioral issues.  He is still self-injurious - eye poking, biting, etc.  He feels very little pain so often he's hurt and we don't know it.  He's chewed holes in all of our furniture.  He is constantly moving and is nearly impossible to get to bed.  He is extremely oral and needs something to chew or bite non-stop.  Because he's non-verbal and has limited motor abilities to sign - he often becomes frustrated that he cannot express his wants & needs.  But despite daily seizures and all these issues - he still progresses and develops - and he still works like a champ in his therapies to move forward.  We are so proud of him.