Holden recently turned two and is proving himself to be the poster child for this infamous age of terror. He can literally scream for hours and frequently kicks and fist pounds the floor. During his astonishingly long and hysterical tantrums, Holden often gets into such a frenzy that he makes himself puke in order to receive extra attention.
While his determination is impressive, Sam and I are pretty unnerved by these outbursts. We feel especially stressed about Holden’s fits since we are always wondering if he is exhibiting typical two year-old behavior or if these extraordinary outbursts are actually related to complications from Pfeiffer syndrome.
When it comes to Holden, anything that seems slightly off makes Sam and I worry that it is a bigger problem than just “The Terrible Twos.” We ache for the day he has the vocabulary to tell us what is really going on.
When it comes to Holdens tantrums, we are concerned that he could be having behavioral problems because he is not getting good sleep. If you have followed Holden’s journey, you know that he suffers from sleep apnea and getting him to wear his CPAP has been a huge challenge.
For the better part of a year, I was tyrannical about forcing Holden to wear his breathing machine. I went as far as to tape his pajamas over his hands so he couldn’t rip his mask off in the middle of the night. For a long time, my tough love seemed to be working.
Then we had his annual sleep study and the boy went crazy. He ripped off every electrode they hooked him up to as soon as it landed on his head. The technicians tried to put his arms in restraints and he proceeded to immediately hyperventilate and throw up.
That night, we opted not to go through with the study and he has refused to wear his CPAP ever since.
Since then, we have met with Holden’s pediatric pulmonologist and have a plan in place to address his sleep issues. In the meantime, since Holden is not wearing his CPAP, anytime he has an apneic episode, the flow of oxygen to his brain is interrupted. This lack of restful sleep can lead to behavioral problems in children. So, Sam and I are left wondering, are these horrendous tantrums “The Terrible Twos” or are they related to Pfeiffer?
Part of our plan to alleviate Holden’s sleep apnea is to have his tonsils and adenoids removed. The hope is that by removing these his airways will be less constricted and he will breathe better at night.
We also recently met with Holden’s ENT and learned that he needs new tubes in his ears. This was no big surprise. Then he had his hearing checked and a new worry set in.
I sat in a little booth with Holden while the audiologist ran various tests to monitor his hearing. I could immediately tell that he wasn’t responding to the stimuli as quickly as I was.
The test results indicated Holden still suffers from mild hearing loss, which I naively thought had been resolved the first time tubes were placed in his ears. When I learned this, my mama brain started spinning and thinking about his speech. Once again, is it the age or the syndrome? Is Holden’s pronunciation and gibberish normal 2 year-old babble or is he distorting words because he can’t hear as well as the average child.
In order to determine the extent of his hearing loss, Holden until will undergo an Auditory Brainstem Response (ABR) Test. The ABR uses a special computer to measure how the hearing nerves and brain respond to sound. For children Holden’s age, an ABR has to be done under anesthesia.
Our goal is to anesthetize Holden as few times as possible over his lifetime, so his ENT will perform all of the procedures I have mentioned so far back-to-back later this year - adenoids, tonsils, tubes and the ABR.
Another aspect of Holden’s development that concerns Sam and me is his coordination. This is another area where I am unsure whether to attribute his clumsiness to Pfeiffer syndrome or the delightful age of two.
Holden can’t seem to master going down the stairs at our house and the other day I watched him run straight into a wall. I recall Everett being particularly ungainly around the age of two so I think, maybe Holden is just learning his physical limits. Yet, I know from his pediatric neuro ophthalmologist that Holden is missing one or more muscles in his eyes and this causes his vision to be misaligned. He also likely has problems with depth perception. Yet again I wonder, is it the age or the syndrome?
To address the issues with his eyes, Holden will also be undergoing surgery to fix his hypertropia and improve his vision. In my ideal world, we will be able to pair this surgery with the procedures the ENT will perform. It comes down to how long he can be safely be anesthetized as to whether this will be able to come to fruition.
While we wait to find out the answers to all of these questions regarding Holden’s development, I find a strange comfort in my son’s epic tantrums. Trust me, most of the time I want to rip my hair out while I am trying to dress him and he repeatedly kicks me in the face. But, when I think of Holden’s fierce, determined spirit I am relieved he has this spunk. The road ahead is not going to be easy, but Holden Smith is going to barrel down it with incredible strength and resolve.