Wednesday, January 22, 2014

Do you hear what I hear?

Does Alex hear what we hear?  For two years we weren't sure.  It's very common for kids with Hunter Syndrome to have hearing loss; virtually all kids with Hunter Syndrome have hearing aids and get repeat PE tube placements.

Alex's hearing loss has occurred over a long, intermittent road.  At birth Alex failed his hearing test, and had to follow up with audiology after his release from the NICU.  After that initial test he passed his next hearing screening, but it's very hard to test an infant. They can't always get all frequencies tested.  They have to be sleeping but not snoring, basically the sun, moon and stars need to align.  We went every six months for audiology appointments in Chicago and then Cleveland, monitoring and looking for signs of hearing loss.  With every test there were results but then a shred of doubt, in that he wouldn't cooperate with a test or they/we couldn't keep him quiet for a frequency, etc.  Then we started also doing auditory brain stem response (ABR) tests that are in my view a little independent of behavior.  This basically tests your hearing while under anesthesia and when they get a hearing response to a beep, it means you can hear at that level. To what clarity it's unknown but you'll get a chart that shows you what your child can hear, and if amplification (hearing aids) can bring your hearing up to normal.

When we moved to Cleveland, Alex had a hearing test, an ABR, that basically said he couldn't hear anything below the decibels of a jack hammer.  He was diagnosed with severe to profound hearing loss.  I became loud Mommy trying to talk to him and then forgot to adjust my level when talking to others!  In the cul-du-sac I often hear my voice ricocheting off the windows of the other houses in the suburban sanctity of silence. We also found out while he exhibited no symptoms of ear infections, or fluid on his ears; there was intermittent evidence of fluid behind his ears.  Quite a complex puzzle.  This presence of fluid makes sound really muffled.  Unfortunately he had fluid in his ears when the ABR was done so we left with hearing aids turned up so high they could blow glass out of a window!  Then the fluid went away and they turned them down.  Up, then down, then down, and we thought gosh will we ever get this right?  It's also not fun at all making him wear them.  It's very, very difficult.  He watches the iPad or TV while we place them in his ears and basically have a tennis match defending the hearing aids in his ears!

We finally decided we need more help. We went to a hearing expert in Chicago in December.  Now we might have an idea regarding what Alex might be hearing but it took seeing an expert to get a solid plan in place.  His recent test showed that he has mild to moderate hearing loss, and we've had the hearing aids set to that level (it took 2 years!).  We're going to continue to work the plan and see how his hearing goes.  Losing his hearing has had so many effects on him from being withdrawn when he can't interact with anyone to being giggly and engaging the day after he gets PE tubes placed in his ears.

The million dollar question is why isn't he talking more in line with kids his age?  Is it because his hearing has been intermittent at best during those key development years? Or is it because he is suffering from the cognitive decline that takes so many of our MPS kids away?  As a Mom, I hold out hope based on facts and observations that it's his hearing.  We do need the intrathecal trial putting medicine into his brain in case I am not right about his hearing, and we really can't waste any time proving theories.    

We'd  also like to share what we learned about what challenges hearing impaired families go through!!  It's enlightening.

Learning #1, Hearing Aids are very expensive!  You are basically buying a mega-amplifier for a rock band that are two ears on your child's head.  You get to select from the equivalent of an amplifier Aerosmith would use down to I just want to hear anything for cheap!   They come with gadgets such as strapping yourself with a microphone to use in another room to call them in for dinner to Bluetooth that allows them to talk on a mobile phone.  Just astonishing!

Learning #2 Hearing aids are not widely covered by insurance. They're viewed as a nice to have and not a medical necessity.  Usually covered at 0%.  I know, shocking.  Some states like NC have great programs others not so much.  Our model cost $10K, just to give you some ballpark figures. Here's our car, I mean hearing aids.

Learning #3 When buying for a child, buy bright hearing aids that can be found anywhere when they throw them! Florescent, glow in the dark if possible!!  Do not buy for vanity or looks because at age 5 this child doesn't care, and you will never find them...and you will rip your hair out or your minivan apart for $10K!!!

Learning #4 - Even the Aerosmith model screeches.  How is this possible with innovation?  These babies deliver the loudest screeches on the planet. Even though the high end models have screech-o-matic management.  They are still terrible.  Don't sit too close or hug your child they will screech.

Learning #5 - If you see a child or family with a child with hearing aids, hug them but not too close, you don't want to make the aids screech. Or donate to a charity for deaf or hearing impaired children, it's not a nice road, it's hard and fraught with little support.

Learning #6 (FINAL) - Have a good speech therapist (special teams), ENT (the quarter back) and audiologist (the receiver), they are your life line.

Thanks for reading, and supporting!  Now go plug your iPhone (or phone of choice) into those hearing ears and enjoy the sound!

The Cherrstroms

Wednesday, January 1, 2014

Update from our Chicago Visit

Our little football team converged on Chicago in early December to participate in the Natural History Study clinical trial, see Genetics,see a hearing expert, get carpal tunnel testing, and followup with Neurosurgery. A full plate indeed. We literally all huddled together in Chicago, and didn't see any of our friends we miss so much. We drove by our old neighborhoods and Jack's old school.  Next time, we definitely want to see all the wonderful friends there, we love and cherish. This trip was different, it was hard, stressful, and our hearts need healing. Nicholas continues to fare better than Alex at this point. We were told that our boys likely have severe MPS II.  Generally kids who have severe MPS II, lose their speech by age 12 and pass away by age 15.  The message that Dan and I heard though is if you don't disrupt the course of the disease, if you don't do anything to save your children, that's their fate.  I walked away sad but VERY inspired to be better parents, better people, better workers, and give everything we have in our souls.  I can't live with myself if I don't do everything possible to help our kids.  Some of these hard messages just give us clarity to put a plan together and get moving on things we can impact and set aside those we can't.  I am also not ready to accept any dire outcome until I make sure that I help Alex with his ability to hear.

The outcome from the specialists is as follows: Nicholas will be followed for the syrinx issue with his spine but otherwise it's not going to impact any involvement in the intrathecal clinical trial.  In that trial, medicine is delivered directly to their spinal fluid and effects the brain and other organs IV infusion cannot effect at this point.  From other parents and children involved in the intrathecal trial, the effects are very promising and they're seeing reversal of the effects of Hunter Syndrome in some children . Again this is according to what we read from other parent posts and blogs.  We're not enrolled in that trial yet.  We're hoping to meet the trial criteria sometime in 2014.

It was one of the most difficult decisions to have Alex undergo spine surgery.  It paid off big time though.  In his MRI images, his spine looks beautiful!!  Best surgeon ever in Chicago, can't thank him enough. It doesn't mean he won't ever have other spine issues but the surgery ended up being the right thing to do.

Alex's special education teacher used a technique with him called a token board.  This approach paid off big time during this visit because he was able to sit and comply for most of his IQ testing, which is major progress!!

More updates to come in the New Year!

Here's a picture of the boys playing at Ann & Robert H. Lurie Children's Hospital in Chicago.

At the Chicago Children's Museum before our Genetics Appointment.