MARCH MADNESS

I know what you're thinking. Why is she writing about basketball on a blog about her son? Well, I have no idea what's going on in the world of college basketball but I can tell you about the "MADNESS" going on in the world of Caden.

As you read in my last post "One Day At A Time", Caden has been hospitalized for his usual, neurological and G.I. issues.  He had surgery last Thursday and then the madness began (not that the past 3 and a half years with Caden and his brother and sister haven't been madness as well).

The night after the procedures Caden did nothing but sleep, wake up and scream in high pitched shrills for a few minutes and then crash back to sleep. This occurred every twenty minutes to a half hour over the next three days. All he did was sleep, wake up and scream, and sleep. His Neurosurgeon ordered three CT Scans which all showed no change. This prompted them to tell me his issues were due to the anesthesia and not the shunt tie off. I insisted otherwise. After much arguing with various neurosurgery residents and fellows, I convinced the department to tap Caden's shunt to see if his pressure was building up. And guess what...I was right. Almost instantly after they drew 20 cc's of fluid off his brain, he felt better, woke up and was alert for the first time in days.

Neurosurgery agreed that his pressure was increasing and they needed to do somewhat emergent surgery to externalize his shunt and measure his intracranial pressure (ICP).  Monday morning, surgeons went in and externalized his shunt. While under anesthesia, they also placed the Broviac so he could begin TPN for nutrition and calories.

Shortly after surgery, we saw improvement in Caden's pain levels, alertness and overall neurological status. Today, Caden spent half the day upright and half the day with his head lower than his legs to see how his pressure changes in various positions. This was done because the biggest medical mystery with Caden is the fact that despite having hydrocephalus and severe reflux, he prefers to lay flat or hang upside down. Doctors from the three best children's hospitals in the country (Philadelphia, Boston and Cincinnati) do not understand why Caden prefers these positions.

Here's a video from last year when he was at Cincinnati Children's Hospital.  It's a bit long but shows how different he is when laying flat as opposed to upright (please ignore my annoying voice:)

Youtube video: Caden's Changing Moods

I have my theory and although I am not a doctor, I spend more time with Caden and have done far more research in trying to solve the "mystery" of Caden. I have presented my theory to many doctors with no luck. Doctors disagree with my ideas because it does not make sense to be happening to a three year old boy. Yet, these are the same doctors that say they have never seen a boy like Caden.  Doesn't it make sense that if these brilliant doctors can not find a reason for my son's bizarre positional preferences, to assume it may be something slightly out of the ordinary? Isn't this why he's been coined a "medical unicorn"?

So here is my theory...

Caden has been diagnosed with a connective tissue disorder called Ehlers-Danlos Hypermobility.  This disorder affects the collagen (glue that basically holds the body together). Many people, mostly adults, with EDS have symptoms similar to Caden's due to changes in posturing. When upright, many people with EDS have brain stem crowding or various other fancy medical terms I will not bore you with. Those that are symptomatic from these issues feel relief from the symptoms when laying down. The cervical issues which present with EDS usually only occur in adults who have suffered from EDS much of their life. It is rarely if ever seen on a three year old.

The picture below shows how the angle changes in someone with EDS 

when they are upright. Notice the little bone pressing on the brain stem.

                  

Upright-Health.com

But Caden is not like every other three year old.  He had a brain injury at birth which caused a bleed causing brain damage. This damage to his brain left him with very low muscle tone.  Now if you add the ligament laxity and connective tissue defects from EDS and the low muscle tone from his brain injury, wouldn't it be possible when Caden is upright for his posturing to be so poor and weak that it may cause the top of his spine to move up compressing the brain stem and causing blood and fluid restriction to the brain. Compression on the brain stem causes something called dysautonomia which presents with many of the symptoms Caden has (irregular/fast heart rate, irregular blood pressure, temperature regulating issues, G.I. issues, etc.), particularly when he is upright.

I know this is a lot to wrap your brain around and I have included some links if you want to play "Dr. House" and investigate further:

Medical Zebras

Autonomic Events and Crisis

Please feel free to ask any questions in the comments. Believe me, I am not a doctor. I just play one in real life.