Saturday, December 28, 2013

Our Appreciation .....




Zachary’s Cakes for Christmas Fundraiser was a huge success! The generosity of others was absolutely overwhelming. We earned near $3,000.00 thanks to all of our family, friends and the community. We would like to recognize all the businesses that donated goods and services to our fundraiser.
Beltone, for giving Zachary a free pair of very nice hearing aids. Zachary now has total control of the sounds that enter into his ears and an FM System that is built right in for him to use while in school. And now the funds raised can be set aside for the Cochlear Implant that will be needed for Zachary’s right ear.
B’s Cheesecakes, Martha’s Chapel Rd, Cunningham
Canyon Road Steakhouse & Beer Garden, Wilma Rudolf Blvd.
Chick-fil-A - for sponsoring Zachary’s cause in a fundraiser held at the Wilma Rudolf location.
Cracker Barrel, Cracker Barrel Dr.
Dairy Queen, Riverside Dr.
George the Cake Guy, Strawberry Alley
GFS, Wilma Rudolf Blvd
Great American Cookie Company, Wilma Rudolf Blvd.
Kroger Bakery, Madison St.
O’Charly’s, Riverside Dr.
O’Charly’s, Wilma Rudolf Blvd.
Rafferty’s, Wilma Rudolf Blvd.
Sam’s Bakery, Veterans Pkwy
Silke’s Old World Bakery, College St.
Union Station Hotel, Broadway, Nashville
... a special thank you to my family in Texas, who rallied together to help our son by having a benefit concert and fundraising Christmas parties.
... and last but certainly not least, thank you Christie Wall for helping us get the word out and getting the media involved.

Again, we can not express the gratitude our family feels for all those involved. Thank you all for your continued support and prayers.

With Much Appreciation
The Huguley Family

Friday, December 27, 2013

An Adjustment



Zachary was having a little trouble with the fitting of the ear molds and Beltone graciously adjusted it for him. Just over the past few months I've seen the Lord's hand in our lives, He is ever mindful!!

Tuesday, December 24, 2013

Merry Christmas!!



Zachary received his new hearing aids today. We are forever grateful for the generosity Beltone has shown Zachary. He is so happy and now has total control of what sounds he hears. The simple gift of hearing independence has brought our son joy in a time of adversity. We can not express it enough..... Our Father in Heaven is so ever mindful of each of our needs. He knew that I needed to see that Z can still become and do anything he desires and with this simple blessing I was able to see not only that but I also see that in times of adversity he brings blessings to help us press forward.

Friday, December 20, 2013

Another Frequency Gone ...sniff

Took Z to the Audiologist yesterday, she said he is getting close to meeting the requirements for a cochlear implant in his right ear. He has lost yet another frequency.  The first test was done on the 2nd of December and the other just yesterday.


Friday, December 13, 2013

A Huge Success!!

The fundraiser last night was a HUGE success!! It was amazing!! Tears welled up on several occasions and the generosity of others was absolutely overwhelming. We earned a total of $2,455. Again, we can not express the gratitude our family feels for all those involved. 
Thank you all for your continued support and prayers. 
With Love
The Huguley's

Tuesday, December 10, 2013

A Fundraiser for "The Gift Of Sound"

We are trying to raise funds for Zachary in order to provide hearing aids for him. Please follow the link below if you would like to give Zachary the gift of sound before Christmas.

LINK: The Gift of Sound

Thank you in advance,
The Huguley Family



Information About the Fundraiser

Cakes for Christmas ... and other things
Gift Basket Silent Auction
Helen's Art - www.capturedmiracles.org
Photography Mini Sessions
Christmas Cards & Paper Crafts
Jewelry
Pampered Chef
Doterra - Essential Oils
Z’s Friends - Artwork from Z’s friends

Cake and Gift Basket Donation Drop Off 
is from 10am - 2:30pm 
December 12th 
at the Smith Trahern Mansion 
If you have any questions you can message me at the email that is listed in the flyer.




Monday, December 9, 2013

Hearing Aids .... Check!! Cochlear Implants???

We can not express the gratitude we have for all who are helping us raise funds for Zachary. After the newspaper article ran in the Leaf Chronicle, I received a call from the journalist who asked me to call Andy Stewart with Belltone Hearing. I was informed by him that his company would like to donate hearing aids for Zachary for Christmas. I was in shock and could not believe it. I held back from sharing this because I wanted to be sure I wasn't dreaming and it wasn't some sort of gimmick. After talking with Dr. Levy I found that they were very serious and want to help Zachary by donating hearing aids that will work for him. We are truly blessed!!
Where are we now? We are still trying to raise funds for Z and bring awareness to this debilitating disease, 95% of those who suffer with Superficial Siderosis lose their hearing completely and end up with Cochlear Implants. Our focus now is to raise money for that. Zachary is losing the hearing in his right ear very quickly and hearing aids will be of no use to him, I predict very soon.


Hearing Test from December 2nd. The red line is the right ear the blue line is the left ear.


Dr. Levy putting Silicone in Z's ear so a mold can be made for his hearing aids


New earbud style? Maybe? Ummm? Maybe not ...

Saturday, November 23, 2013

A Tough Day for Z ...

I'd like to call this one of my most difficult and heartbreaking days. Today we sat our son down and explained the ins and outs of Superficial Siderosis. We explained what was going on in his tiny little body, what exactly it was that was causing his hearing loss and what the future holds for him. I've had to go through and do a lot of hard things but I can not think of anything that comes even close to this. As we sat and talked and discussed these things I could see him trying hard to hold back the tears. I watched as they welled up in his eyes. When the conversation was done and his questions were all answered. We hugged it out and he went off to do his chores. I went down shortly after him and noticed he had taken his clean basket of clothes into his room. When I opened his door he sat knelt over the basket with his head in his clothes crying. I went in sat closely next to him and just held him for a while. Then I told him how special he really is. I told him that his Father in Heaven loves him and is always with him. I told him that He never brings him to something without bringing him through it. And I reminded him that he has family and friends who are all pulling for him and praying for him. He just wept, it absolutely broke my heart. 
I'm thankful for the love of our Father, who knows our strengths and our weaknesses. He knows what we can and can not handle. I felt His warm embrace as I sat there with His son in my arms crying at the reality of his disease. HE LOVES US and is ALWAYS WITH US and for this I am truly grateful.

Tuesday, November 12, 2013

The Sound is Back

Today is the day Zachary gets fitted with hearing aids his appointment is at 9:00. Dr. Smith is letting us use a trial pair for a little while. 


The Audiologist appointment went ... well, I won't say great as great would mean there would be no bad news. We took the donated hearing aids to the doctor to see if they could be used but unfortunately they are to powerful for Z right now and could damage the hearing he has left. The doctor advised we hold onto them as Z might could use them at a later date. We also found out that Zachary's hearing has gotten worse. The right ear more so than the left. 

We are very grateful that hearing aids will still be helpful to Zachary, however, we are concerned that his hearing will continue to decline.


A moment I never want to forget, the moment when he hears himself breath, priceless!

I took a video of Z clicking his tongue, very cute, but can not get it to load.


All the clicking and tapping continued all the way home. He played with his jacket zipper, tapped on a football, scuffed his feet across the floor, waved his hand about, knocked on doors and objects, even threw things in the air and hand smacked them. He is amazed at all the different sounds he has missed. We aren't sure when his hearing started to go away we only know when it started to affect us here at home (Sept). Zachary's teachers haven't noticed anything and his grades haven't changed at all. He is a very smart child to be able to keep up, even though his hearing was falling behind.

We are so blessed! 

Zachary has been given a pair of loaner hearing aids to try out for a little while. 
Even though our trip to the Audiologist came with some good news there was sad news to be reported as well. We found out that the hearing in his right ear has taken a plummet to the bottom of the hearing chart and that change occurred in only a mere 7 weeks. But watching our son hear himself breath for the first time was priceless. We are so grateful that Zachary is able to hear with the aids. What an amazing blessing that is. 
I'm learning that with every trial there are blessings that follow, maybe not the kind of blessings we would ultimately hope for, but blessings they are and I'll take what He is willing to give.

I called the school today and spoke with the guidance counselor about setting up the FM System for Zachary at school. Left him the Audiologists number so that they could get together and set up the FM System for Zachary. I also let him know that Zachary would be in school tomorrow with hearing aids on. 

Monday, November 11, 2013

An Answered Question


I wrote Dave Hill (runs the SS support group) a letter to get an idea of how quickly the loss of hearing goes. 

Well Dave,
I'm not just any other American, lol. I've got a lot going on with this fundraiser for Z and an interview this week with a local paper to express my concern that Superficial Siderosis is under diagnosed and I want to bring awareness to it so that more research can be done to find a cure.
When I spoke to Z's Audiologist she had never heard of it and spent 15 minutes researching while we waited to be called to the back for our appointment. She mentioned that there are a lot of children who are going deaf without any explanation. I believe SS could be one of the reasons.

The only thing I have right now is Z's online fundraising efforts which is located on his blogspot.

I do have a question tho ... I know that once hearing loss starts it goes fairly quickly. Do we have an estimated amount of time from start of hearing loss to complete hearing loss? Are there any of the members who might could share their experiences with us? 

Yes, it used to be said in medical SS write-ups that SS was not a child's thing but I think one day they'll clearly change their sayings.

If  I asked your question to Dr. Levy I know clearly he would say everyone is vastly  different, and we sure are. I can't even remember now how quickly I went down to minus  zero as my CI audiologist said I went to. My estimates are though it would be only a few months. Look back in early versions of newsletters for an accurate account from me.
Dave

I called the Audiologist in Goodlettsville and asked about testing Z again. She said that typically they don't test this quickly, right after testing, but I expressed my concern so they will be performing another test. I'm feeling like the Cochlears are going to be a part of Z's life a whole faster than we thought.

I try to make everything alright in my head, but this all stinks!! The feelings a person gets when she loses something precious is still there. Z is such a trooper and I thank my lucky stars that he is as laid back as he is. I feel like I'm having all the emotions of loss for him. My baby is quickly losing his ability to hear my voice.

Sunday, November 10, 2013

Sharing Big News

Hey there, Dave!

We hope that you are doing well and are keeping your spirits up. I wanted to give you an update on Zachary, as you already know he is now in dire need of hearing aids. Our family is large and the medical bills are mounting pretty high so I am putting together a fundraiser for Z, so that he can receive the hearing aids he needs. There is a link on Zachary’s blog spot, http://hug-z.blogspot.com. I already have a possible 3 interviews set up (they aren’t confirmed yet) with the local media and I will be sharing as much information as I can about Superficial Siderosis. The big news is that ... I'm planning to open up a foundation for Superficial Siderosis to bring awareness to a disease that I feel is very much under diagnosed. So if the funds over exceed the goal amount the extra will be applied to helping others with Superficial Siderosis and toward research to find a cure. As soon as Z is all set with his hearing aids I will be focusing my time and energy toward that.
Z is doing very well, he’s just happy that there is no pain affiliated with losing his hearing. Our family is taking an American Sign Language class together to prepare Z for the future of possibly needing the skill.

We pray that a cure will come very soon and this can be a disease of the past.

Michelle

You are doing marvellously well Michelle with great ambitions. Other Americans have claimed to be starting up similar but they never seemed to do so. If you have more details please send them on as I'll pen up something for the next newsletter.
Well done that lady

Dave

Thursday, October 24, 2013

Dr. Levy Clears Things Up


Hi Ms. Huguley,

The spinal fluid is made deep in the brain and circulates around the brain and spinal cord, as you said correctly, and then ultimately gets resorbed into the veins at the top of the skull. The total volume of spinal fluid is turned over 3 times every day so you can imagine the circulation is fairly brisk. That's why a bleed anywhere in the spinal fluid leads to exposure of the whole nervous system to the blood. As the blood breaks down, the blood iron products precipitate out and eventually settle down by gravity to the back of the head while sleeping and to the bottom of the spinal cord while awake. The reason hearing is affected is because the nerve that goes from the ear to the brain spends the majority of its time in the spinal fluid. All other nerves that come directly off the brain have < 1 mm of surface exposed to the spinal fluid. So the nerve to the ear is vulnerable to blood products circulating in the spinal fluid. Since that nerve has a large surface area relative to the volume of the nerve, a large percentage of the nerve is exposed to the iron. That's why hearing is usually the first symptom of superficial siderosis. 

Michael Levy, MD, PhD
Assistant Professor, Johns Hopkins University
Director, Neuromyelitis Optica Clinic
1800 E. Orleans St.
Pathology 509
Baltimore, MD 21287
443-287-4412 phone
888-523-4168 fax

Tuesday, October 22, 2013

What is Superficial Siderosis? And a Letter to Dr. Jordan & Dr. Levy

This information is located earlier in this blog, I'm adding it again for those of you just visiting this page. It's a real quick overview of what Z has and what possible side effects he will endure in the future. 


What is Superficial Siderosis

Superficial siderosis (SS) of the central nervous system is a rare brain disorder resulting from chronic, and repeated, slow haemorrhaging into the subarachnoid space, and if accurate international figures were obtainable, would most certainly prove to be evenly spread amongst all nations around the world – the small country of New Zealand does appear to have more than its fair share of diagnosed survivors however. People diagnosed with SS range in age from just a couple of years old, right through to their 80s, though the most common age bracket is the 50s and 60s.
Despite being known for over 100 years, it is only in the past few years that SS has been widely acknowledged and studied. Previously known by several titles it was only in 1985 a definite title was applied to the disease. The disease is unusual in that it carries so many side-effects that a sufferer may experience. The symptoms are still being discovered, but currently number over 30, only some of which are common to all, or most, known survivors of the disease.
Prior to the introduction of MRI machines, diagnosis of the disease was carried out on the autopsy table.

Side effects and symptoms

  • The most common side effect is the lack of hearing, with 95% of those affected. What hearing may be retained, if any, is generally of no value. The hearing does deteriorate extremely quickly with SS and hearing aids provide only short term assistance. Cochlear implants currently appear to be the only permanent life buoy for this side effect.
  • Ataxia (lack of balance) and loss of co-ordination are the next most common and frustrating side-effects for those diagnosed. Around 85% of all cases are affected with these two debilitating side effects. Mobility aids, such as walking poles or canes, wheeled walking frames, and wheelchairs provide valuable assistance. Many with the ailment advance to a stage where they are bedridden.
  • Bowel and bladder complications are also commonly accepted complaints.
  • Side effects vary right down to only two known cases of the 8th cranial nerve being covered in hemosiderin, stopping the survivor from feeling any pain in their teeth.

Known SS symptoms

  • lack of hearing
  • imbalance
  • poor co-ordination
  • early dementia
  • bladder complications
  • bowel complications
  • headaches
  • migraines
  • severe tiredness
  • short term memory loss
  • loss of smell
  • loss of taste
  • muscle cramps
  • double vision and other vision complications
  • cranial nerve palsies
  • imaginary pains, smells, tastes and sounds
  • permanently bed bound
  • failure of the senses
  • one pupil larger than the other
  • double sciatica
  • mini strokes
  • seizures
  • speech difficulty
  • nystagmus
  • numbness and pain in parts of the body, especially feet
  • swallowing difficulties
  • failure to feel pain in the teeth
  • ‘Parkinsons’ type leg and hand wobbles
  • inability to control emotions – anger, sorrow, etc.
  • intolerance and uncertainty to heat and cold; may feel roasting hot, but are actually cold
  • failure of sexual organs – inability to arouse an erection, or no feeling of satisfaction for females






















Understanding How SS Affects the Hearing


I wanted to be able to understand more about how SS affects hearing so I wrote a letter to Dr. Jordan & Dr. Levy to get their expertise on the subject. 

Dr. Jordan and Dr. Levy

I’m sorry to be such a burden. I just want to be sure I'm understanding the CNS and the CSF flow correctly. I’m seriously trying my best to understand all of this. Just when I think I’ve got it, I don’t.
I understand that iron is being deposited on Zachary’s spinal cord. What I don’t understand is how does that affect his hearing? I guess I’m confused by the details of Superficial Siderosis and it's affects on the hearing. How does the iron affect his hearing? I want to know exactly how SS affects the hearing. Does it destroy the inner ear somehow? I don’t understand this part of it and because this seems to be the reason for Z’s hearing loss I’d really like to know more about how SS wreaks it’s havoc on this part of the body.
I would be most grateful if you could help me to understand. I’m all about researching and if there are reliable places for me to do this that you can send me to, I’d be greatly appreciative. I would really like to be as educated, as a mother who has a child with SS can be.

I'm trying to understand the flow of the Cerebrospinal Fluid ... Is it true that it is produced in the center of the brain and then travels to the ventricles then down the spinal cord and back? I just want to be sure I understand that it does go down the central canal of the spinal cord and then back up again to the brain, which means it must pick up some of that deposited blood/iron at the base of Z's spine and it drags it back to the brain, right?

Thank you so much for your time
Michelle Huguley

Wednesday, September 25, 2013

Another Visit to the Audiologist

Robert went to the Audiologist with me and Zachary and I recorded most of the appointment. Z was tested by Dr. Holly a pediatric audiologist He was tested a week or so ago by Dr. Smith. Today we are here to see if their findings match up and to find out exactly what's going on with Z's hearing.


The structure of the ears look nice and healthy, the ear drums are moving like they should and that looks great. There is a little tiny muscle in the middle ear behind the ear drum. When we have loud sounds they muscle is supposed to contract, now I did see one response but I wasn't able to get normal responses for that in either ear on all the different frequencies that we looked at, in and of itself it does not indicate that there is a problem there, however that wasn't completely normal.
Then we did or OAE testing and what we're looking at with OAE is some different tones, different pitches, or frequencies going into the ear. It travels down into the inner ear and that inner ear has little hair cells in it, now when those are functioning normally sound is going to come in and those little hair cells will send back an echo it's very tiny. There are a lot of things that could block that response from being gained and I did not get a normal response in either ear. The ear canals have no blockage and they aren't inflamed, the ear drum is fine. The organ called the Cochlea has thousands of tiny hair cells which change the vibrations into electrical signals that are sent to the brain through the hearing nerve. The brain tells you that you are hearing sound and what the sound is. So in the test given we found that the little hair cells in the Cochlea did not respond to the tones telling us that more likely than not the origin starts here (the cochlea). There are three nerves going to the brain, one is for balance, one is a facial nerve and the other is the hearing nerve. Are finding are that the damage is starting at the Cochlea
It sounds to me like with the tests that we both did. We are both definitely seeing damage start at the origin of the cochlea. That's the beginning of that neural response. We did not see any kind of issues that would relate this to any type of allergy or ear infection.

An Explanation of the Graph Below

This is an audiogram. This is a graph of the softest sounds that he's hearing. What we are looking at are the softest sounds you can hear. Across the top of the graph we've got base sounds, the boomy low sounds all the way to high pitch, high squeaky sounds. These are the sounds that are important for speech and language. Down the side we've got really soft quiet sounds all the way to very loud sounds. So, basically the farther down the graph you go the louder I had to turn the beep for Z to hear it so he would raise his hand. The red is for the right ear and the blue is for the left ear so you can see they kind of match up they have that same pattern, which is good. If there were a huge difference we might be concerned about other things, so it's good that they are matching up. the normal range of hearing is going to be 20 and above. So you can see that some of those responses come up to that normal range but for the most part they are below the normal. I had to turn up the beeps so they were a little louder for him to hear them. Now that is just hearing the sound. Is the sound there? or not there? That's the first part of hearing so we've got from a ..., and I kind of jotted it down out here so you can remember it later when you take this home, but this is kind of a mild hearing loss in the low frequencies and then it slops down here to a moderately severe in the left and severe in the right. The other things that are on this graph are just everyday sounds anything below your lines you can hear it because it's loud enough, but anything above you either can't hear it at all or you have a really difficult time hearing it. You can tell that the low frequency sounds of speech like vowel sounds those are very loud and boomy they give the volume to speech. Those are audible so he can hear them when you're talking but sometimes understanding what you say id hard because look at all these consonant sounds that give the clarity to speech. Those are above that line so Z can't hear them. He's not hearing a lot of those sounds at all. So that means that his hearing is very muffled, it's distorted and he's not understanding much of what is said in conversation. 


The next part of the testing that we did is involving speech because we want to know how soft does he understand speech and then if speech is loud enough it should be clear enough to hear. How well does he repeat back? That's where he started having some trouble. When we did the speech testing a lot of interesting things happened. Now, we know he's going to miss some things because we now know he is missing all those consonant sounds but I also noticed ... it was a little out of the ordinary not just missing those sounds. You know missing the /p/ sound at the end or the /s/ on the end of the word. It's hard to describe this but it's like he would say a completely different word or just say something that was not your typical response you would see. It was really out of the ordinary. Another observation he kind of twitchy or moving a little bit. The scores were stable from last time to this time which is a very good thing that his understanding of speech did not drop between the two test times. He is understanding speech better on his right side than on his left. Another interesting thing is when we turned up the volume. I went ahead and repeated the right ear I didn't do the left ear because, I mean, the right ear did the same thing where when we turn it up a little bit louder you should still be fine. You should hear it even better, it wasn't to an uncomfortable degree I just turned it up a little bit extra, so you would think, that's easier to hear that word. No, he actually did slightly worse. So he didn't do as well as we thought he would when we turned up the volume, that's out of the ordinary, not something we come across very often. Again goes along with damager in the cochlea or the inner ear. How is he doing in school? 

"He's an A,B student and the teachers haven't noticed anything". 

It's very subtle, because he has that drop where he's got a mild loss and then he slopes down in those high frequencies and you can kind of see on this graph. If the lines above that sound, that's typically that you should be able to hear those sounds pretty well so these consonant sounds he's going to struggle here because his line drops below, but the vowel sounds he should hear pretty well. But you can see where it's borderline right here. I think he's getting by on having a little bit better hearing in the low so when you talk about amplification or hearing aids. It kind of helps span that gap and gives you a little bit of a boost where you need it and a lot more boost where you need it there. Unlike testing under headphones or listening to TV or something like that. He may turn the TV up here to try and grab these. Well it going to be way to loud for right there. (The Dr. is pointing at the above chart as she describes this to us). So if he's listening to the TV or to music or something like he may really try to jack it up so he's catching those consonant sounds, but when he does that it over corrects on those low sounds and could cause further damage. 

 

"How would hearing aids fix that"?

Hearing aids? We can't say that they totally correct your hearing like a pair of glasses corrects your vision. It's not quiet the same but hearing aids amplify or give us a chance at being able to modify so that ..., basically where he doesn't need as much power we don't give him as much and where he needs more we can give him more. A basic hearing aid even the good basic you may only have four sections where we can actually make changes to it. So, it gets kind of dicey where he goes from a mild and slopes pretty quickly to a severe to moderately severe level. Thats one reason why I would plan on the total cost being in that 4 - 5 thousand dollar price point. I can extend a little bit of a discount and we will work with your insurance on that, but you should plan on there being some out of pocket expense. We will try to get the best basic aide that we can but its going to be a lot harder to fit his hearing loss and also get a hearing aid that will function well in the school type setting. You really want something now that manages that speech and noise.
You can compare the different levels of hearing aids to cells phones take technology for instance you take like an old basic cell phone vs. a smart phone. It's a computer chip on the inside that's what we're dealing with and so the smart phone computer chip and the processing is happening quickly it can do a bunch of things automatically running the background. Whereas the other one you literally have to push buttons each time to do what you want it to do. So, hearing aids are based on a computer chip and so when you go top of the line everything is more automatic, it's a faster processing it a more sophisticated of a noise manager so someone who has a more active life style if you think about it he will be going into different environments, for example he will be going into this noisy classroom, and then into a cafeteria where it's this big open space with a lot of noise and then he will be going home where it's very quiet and he will be focusing on mom or a TV. 
"So the hearing aid automatically adjusts the volume" 
Yes, exactly! It's automatically going to detect noisy rooms from quiet ones. Let's say I'm in a coffee shop and there's a guy talking over there while I'm trying to listen to my mom the hearing aide is going to alleviate that noise so that I can hear my mom or it will lower the range of where you are listening. With a basic hearing aid you have to physically look at the person you're talking to and it's only going to lower the volume from the back or behind you, so it's just very basic. And then you have technologies that are between there. 

So a top tier, it used to eliminate back noise or it would minimize or eliminate what was going on behind you. Now it actually will kind of zoom back if like someone was behind you telling you to do something it would recognize that there is a prominent noise from behind and it would zoom to the back, whereas before you would have to turn so you could see who was talking to you. They are always going to work better with anything within 6 feet and more face to face but that's just an example of the newest offering in the high or top tier level. The other thing I would consider for Zachary is doing the water proof or water resistant it's a little bit bigger casing but its going to be a little more durable for a child. Given to how we are uncertain to how this will progress I would put him in a Rick that way we have a lot of flexibility for the life expectancy of that hearing aide. If we start seeing his hearing change then we're not coming back in a couple of years and saying we need to get a different hearing aide. If his hearing progresses to where it is off this graph then we would be talking about cochlear implants. Hopefully we wont see it progress rapidly like that, but it is something we will want to watch for. I would recommend we have him tested every 6 months for the first couple of years and if it remains stable then we can see him once a year. 

Essentially Zachary is going to need some support in the classroom. Being moved to the front of the classroom and the FM System we discussed last time we met and possibly a note taker as he gets older if he needs it.

Zachary do you have ringing in your ears? yes 
Are you in any pain today? No 

I just wanted to be sure pain wasn't a factor as sometimes if a child is in pain they won't be as focused during the testing.

"Does the constant tone in his ear have any affect in him hearing some of the higher pitched sounds?" 

No it will not create a problem for our testing, but it can be a little disruptive throughout the day. Sometimes it can have an impact on sleeping, getting to sleep at night, because its kind of annoying. We look at that too when we're managing that hearing loss.





Monday, September 16, 2013

Definitely Experiencing Hearing Loss

Audiologist said that Zachary is definitely experiencing hearing loss and wants us to bring him back to be tested again by a pediatric audiologist that she works with to make sure they are seeing the same thing and then wants to test nerve function with a machine she shares with another audiologist. She'd like to have this done before the end of the month. She was an awesome doctor. I told her about the Superficial Siderosis. She totally looked it up and studied it for about 15 minutes. She said she had never heard of it before. I like doctors that listen and take the time to study up on their patients.

Zachary was at a really good part of his book he didn't want to be left with a cliffhanger when the doctor called him back.


Z being tested, I asked him if he was ready and if he studied he said he didn't get a study guide.


Into the silent room he goes! 



The Results! 
  

Dr. Beth Smith advised me to go to the school and ask about their hearing loss services and reccommend an FM System for Zachary to use. I got the ball rolling over at the school today.
Hearing loss is the most common symptom associated with Superficial Siderosis. The research that I've done, hearing loss doesn't occur until age 40 plus, however we are in uncharted territory as Zachary is the only documented child case with SS. 


You know with the news of Z losing his hearing, there were some pretty serious emotions going on today, mostly from me. Zachary? He's so laid back and just goes with the flow, he doesn't really seem to concerned about it. Then today after years of trying to ride a bike. Today, the Lord sent us a Tender Mercy! Z was able to balance himself enough to ride a bike. Whoo Hoo! I just couldn't hold back the tears. Nothing is more rewarding than to see your child that happy. I'd like to pocket this memory, for it was definitely a good one. 





Wednesday, September 11, 2013

The Run Around

Went to Dr. Westerkamm (the ENT doctor) today and he did the exact same thing that our family doctor did. He put a cone shaped cup onto a light and looked in both ears. We were in and out of the room in less than 5 minutes. Guess What? Z has no blockage! What? You're kidding me! You mean my family doctor got it right? Shocking!! It aggravates me to no end when doctors give you the run around and charge you all kinds of money. Seriously I didn't see any since in that appointment at all. We totally could have skipped that one and went straight to the Audiologist.


Z walked out with the biggest toy from the toy chest. That toy will only cost us a couple hundred dollars.

I just get so aggravated when we are handed the run around. One, I don't have the time for it and two, I don't have the money for it.

Friday, September 6, 2013

Reply from Dr. Levy & Dr. Jordan


Hello Ms. Huguley,

Thanks for the update. I'm glad to hear that Zachary is doing so well. I will let Lori Jordan know.

It will be important to track the hearing loss over time. If Zach continues to lose hearing year after year, you may want to think about iron chelation therapy. 

Michael Levy, MD, PhD
Assistant Professor, Johns Hopkins University
Director, Neuromyelitis Optica Clinic
1800 E. Orleans St.
Pathology 509
Baltimore, MD 21287


So glad to have this update Mrs. Huguley.  I'm glad that  Zachary is overall functioning so well. Though I am sorry to hear that he has some hearing loss. Hopefully it stays minor. 
I am here and am always happy to see Zachary if neurology care is helpful. 
Best, 

Lori Jordan MD, PhD
Department of Neurology, Divisions of Pediatric Neurology and Stroke
Vanderbilt University School of Medicine
2200 Children's Way, DOT 11-242
Nashville, TN 37232