More Hurdles For Our Sweet Girl: Part Two

{Read Part One}

 

 

Although we do not think that Bella has currently lost any hearing, we are not positive. There are so many levels of hearing loss that it is hard to tell on a 1 year old. Like I mentioned before, unfortunately, we were not able to preform the standard hearing test at Dr. Yuksel's office that day during our appointment because we could not get Bella to calm down enough.

 

The Audiologist said that we need to do an ABR test (Auditory Brain stem Response) which we have decided to preform the next time that Bella is under anesthesia. We are pretty certain this will be when we do her Temporal CT scan sometime at the beginning of 2013. This test will give us a baseline of where Bella's hearing is now. We will also be able to know when she is starting to have hearing loss, if it has not started yet, and we will be able to tell what type of hearing loss and to what degree it is. We will have several of these tests over the next several years which will let us monitor her hearing in depth.

 

Along with significant hearing loss, Bella's characteristics of Mondini Deformity is commonly associated with Pendred Syndrome. Pendred Syndrome is progressive bilateral sensorinueral hearing loss (usually beginning before age 3) and goiter which can also be associated with hypothyroidism. Children with Pendred Syndrome usually have balance problems as well. Dr. Yuksel has referred us to a Pediatric Endocrinologist to further test for the Pendred Syndrome. I am actually glad that he has referred us to one of his colleagues with UT physicians because Bella will need an Endocrinologist in the near future for her SMMCI syndrome as well. We love that all of her specialists are with UT and they all work together and know each other fairly well!

 

We know that Bella has balance problems. We have been working on that quite fiercely in PT and OT. When we discussed this with Dr. Yuksel he said that this is most definitely due to the way her ears are formed. We also asked if there is anything that we can do about this. Sadly, there is nothing that can be done. He said that, based on the anatomy of her ears, he suspects her to be fairly dizzy, sometimes more than others and also have some significant balance issues. He also said that the brain is very smart. In most children, the brain finds ways to compensate for the vestibular loss and use other organs of the body to overcome these problems.

There are no medications or anything that can be done to make the vestibular problems better, we just have to let the brain do what it does best and continue with intense physical/occupational therapy to help train the brain. Mike and I are very optimistic in the fact that the stronger Bella's legs and trunk get, the more balance she will have and that her body will be strong enough to compensate for this dysfunction.

In addition to the Mondini Deformity, we had to discuss Bella's Pyriform Aperture Stenosis too. We have been aware, since Bella's surgery in December 2011 that Dr. Yuksel was not able to drill enough space in her nose to make her breathe easily through it. She is still a "mouth breather" and it seems to actually have gotten worse over time. She CAN breathe through her nose when she is totally calm and relaxed, but even then I would say she still only uses her nose a fraction of the time.

It is so noticeable that Kallie has decided Bella is a puppy! When Bella is active, Kallie always looks at me and says, "Look mommy, Bella is a puppy!" She literally sounds just like a puppy does when it is panting.

Dr. Yuksel said that we are going to have to repeat the surgery soon, as it is not healthy or proper for her to be breathing like that from her mouth. While in his office, he witnessed her mouth and noisy breathing. He agreed that it is not getting any better and, actually, may be getting worse.

We are not going to do the surgery immediately, but will evaluate it again in December. Bella has learned to adapt to, basically, not having a nose and has actually become a good mouth breather, but it is greatly affecting her feeding and drinking.

 If we do the surgery now, there will be a very high chance of having to do a 3rd or possibly even a 4th. Bella's face is so small that he will only be able to take out a few more millimeters which will not be enough for a significant change. We are tying to avoid more nasal surgeries (after this one) to prevent scars and scar tissue build up, which could actually cause more airway obstruction.

If we can wait for another 6 -9 months for her surgery then her face will have hopefully grown enough for the surgery to make a significant change in her breathing . If we end up having to do a 3rd or 4th surgery, in the same area of her nasal passages, we WILL have scars and scar tissue that will change her appearance. We are trying as hard as possible to steer away from having any appearance changes due to these surgeries. Of course, if her breathing continues to get worse over the next few months, we will have to do the surgery sooner than we are hoping for.

When we do Bella's CT scan for her ears, we will also do one for her nose and see how much bone he would be able to drill (at that time) and how much it would widen her nasal passages. This will also help us figure out how much longer we need to wait to repeat the surgery.

This past week, Mike and I have both done a lot of research on the type of deformities Bella has in her ears. We were terrified of the fact that she will loose significant hearing, but we have, surprisingly, become calm and accepting of what her future may be like. We watched so many videos of children getting their Cochlear implants "activated" and it was one of the most touching and happy moments to see their faces light up with so much joy. It is amazing what technology can do to help people in so many ways! We feel ready for whatever God may present us with in Bella's future :)
 



More Hurdles For Our Sweet Girl: Part One

Exactly one week ago today, we got MANY of our questions answered from Bella's ENT team. We thought we were prepared for that appointment, but came to realize we were no where near as ready as we thought we were. We were devastated leaving the his office that day and it has taken me a week to feel emotionally stable enough to write about our visit that day.

We are feeling MUCH better about things and have really looked to God this past week for guidance. He has definitely told us that we are going to be able to handle what comes our way and no matter what, Bella is our Bella forever! We were born to be her parents and we feel so special to call her our daughter.

Going into her appointment last week we had a lot of concerns. The Mondini Deformity (and what it means in Bella's case), her yellow tongue, the continued/worsened mouth breathing and her balance problems.

The appointment was at 1 pm and oddly enough they called us back pretty quickly. Usually when were are at the medical center, it is about a 45 minute wait until we even get to move back to Dr. Yuksel's waiting area. He is with the UT Physicians Otorhinolaryngology department and they have several doctors on that floor. They have one big waiting room in the front then each doctor has their own mini waiting area by their rooms.

When we saw Dr. Yuksel's nurse she said that he was already looking at her previous CT scan and would be in with his team in a few minutes. When he walked in he commented on how big and beautiful Bella had gotten and then apologized over and over again that he did not catch the noted Mondini Deformity on her CT the last time we were at his office.

The day of Bella's CT (in march) we were down stairs in the hospital for the scan and then went right up to his office. He just pulled up the scan and we looked only at the nasal / sinus region to see how much more breathing room she has since the surgery. When you do a CT scan of the sinuses, you also see parts of the ears. The radiologist must look at all aspects and "note" anything that they see. The radiologist saw all of her nasal deformities, but also noted this Mondini Deformity. Mike, I and Dr. Yuksel, did not read the "results" from the radiologist that day and thus missed this all together... Fortunately, we still LOVE Dr. Yuksel and think he is an AWESOME doctor!

After his apologies, Dr. Yuksel sat down and pulled up Bella's CT scan on one screen and Google on the other. He started showing us all of the different deformities of Bella's inner ear, which is bilateral (in both ears). She has a deformed Cochlea, enlarged vestibular aqueduct, enlarged sac, deformities of her semicircular canals and a few other dilated areas. He might as well have just said that every part of her inner ear is deformed in some way!

After a long time of explaining and showing us a normal CT of the ears, and Bella's ears, I finally said, "Okay, I get it. She has a lot of things wrong with her ears. What does this mean for her? What can you tell me is going to happen or not happen to her?" His exact words were "You need to be prepared for severe to profound hearing loss even deafness, that will progress in her early childhood." GULP. For the next several minutes, while I was spitting out questions. I was trying to not fall to the floor crying. I had this MASSIVE lump in my throat and I finally thought to myself, listen! You need to listen to what he is saying and make sure you ask all of your questions!

 He explained to us that typically Mondini Deformity is found when a child comes in with complaints of hearing loss, then they usually do a hearing test and ct scans... Blah blah blah. Well in Bella's case, we found the Mondini Deformity prior to noticing any hearing loss. So now I feel like it is just a waiting game. How are you supposed to go through life not knowing when or how much of your child's hearing will be compromised.

He also explained to us that the hearing loss can happen in stages, or gradually. It can happen from the most simple things, like a bump on her head. We were told to keep her from hitting her self in the head (while playing), keep her from falling and hitting her head and also were told that she will not be able to play any type of contact sports or strenuous activities that involve her head when older, such as soccer, cheer leading, football, band and many others.

I have realized this past week that keeping Bella from accidentally hitting her head, is more stressful that I would have ever thought. I also realized that Bella is a 1 year old, and she needs to be a 1 year old. We have decided instead of stressing over it, put it in God's hands. If Bella looses significant hearing after bumping her head on the coffee table, then that is what God had planned for her. We already know that EVENTUALLY the loss is going to happen, whether she hits her head or not... It is just a matter or when.

I asked Dr. Yuksel, "Have you ever, in your entire career, seen a child with Mondini Deformity that did NOT have hearing loss?" His answer was, "No." In fact, it was not only no, but he almost immediately started talking to us about Cochlear implants for her future.. Not saying she needed one right away or anything, just preparing us for the future. He said, we need to do a few things today. We need to do a hearing test and tympanomety (test that checks for fluid on her ears). Bella is constantly having double ear infections and we have talked about tubes a few times now.

We were not able to preform the hearing test on Bella because she was way too upset. We were able to check for fluid though because crying does not interfere with that test. She did come back with fluid on her left ear so we are going to re-check in December.

Dr. Yuksel made it very clear that he is trying to steer away from putting tubes into Bella's ears as much as possible. When you insert tubes, it makes a very small hole in their ear. He said that when you put a Cochlear implant in, it seals MUCH better if the child has not had tubes.

This is when it made it much more clear to me about the severity of hearing loss associated with Mondini. Her doctor was sitting here telling us that he is trying hard not to do something right now so that it will be easier for him to help her hear better in the future. He seemed pretty determined to not do tubes unless it is 100% necessary because of something that we are not even sure that she will need. It seemed to me like he was pretty sure that implants are somewhere in her future.

The plan of action, for the moment, pertaining to the Mondini Deformity, is to have a Temporal CT scan done, meet with a pediatric endocrynologist, and continue speech, PT and OT. If we can get her talking prior to any hearing loss, then we are going to have a strong start :)

Pure joy!

This was a 3 hour appointment with Dr. Yuksel and there was so much more that happened during this visit, I am going to have to make a part two. Don't worry, I will have it posted soon :)

One Smart Cookie

Bella had physical therapy this morning and was actually in a decent mood. Lately, she has been hating therapy! She tends to get really mad when anyone touches her or "makes" her do things.

We have been focusing almost all of our attention on standing (unassisted). She stands GREAT and has lots on stamina, when she is against something. She will stand by the couch or coffee table for really long periods of time and play with toys, but she will not [for the life of her] remove both hands. It is has become almost a joke. She has found a way to do everything and anything she needs to with one hand. Bella's progress has been at a stand still for a few months now so her therapist have been really thinking outside the box to try and get her to the next phase.

Working on sensory processing!

The frustrating part is that if she can stand for that long that against the coffee table, we know that she will be able to stand alone, she just refuses. She has this fear that we can not seem to get her to overcome.

We walked into therapy this morning and Mustafa, her PT, was telling me that him and Farida, Bella's OT, have been brainstorming ways to get both of Bella's hands free at the same time, when she is in a standing position. We literally tried EVERYTHING today with no success. The girl is smart, and on to all of our tactics.

Besides the fact that we could not get her to stand alone, she had a pretty good session. We did a lot of standing, a little bit of walking with the chair, some sit to stand exercises and we worked on some sensory stuff too!

Big girl! - trying to walk with the stool :)

We were also supposed to have developmental therapy today at home with Claudia, but she never showed up! I really liked Claudia the first time she was here, and Bella did very well with her but we have had to reschedule every session since then. We have been told pretty much every excuse in the book from her (always within the hour that she is supposed to show up) and now today she didn't call or answer when I called her 30 minutes after she was supposed to be here.

Needless to say, I called ECI and explained to them that with Bella's current therapy schedule, me working part time and Bella's doctor appointments, we don't have time to try and reschedule all of these sessions every time. They totally agreed that this was unacceptable and we are going to be re-assigned a new developmental therapist :-)

Hopefully we will have more luck this go around!

Worn Out!

Why Is My Child's Tongue Yellow?

It sounds gross and looks pretty gross too- but it is not as scary as it might seem!

For several months we have noticed that Bella's tongue was always a yellow/ orange color. We used to think it was what she was eating, but realized that it was never going away and actually seemed to be getting worse.

We thought about thrush, but she didn't have bad breath and she had no other signs of having an infection. She was totally healthy in that aspect. Don't get me wrong, I thought to myself several times "does my daughter have some flesh eating infection attacking her tongue?" It totally freaked me out some days. I mean, how many yellow tongues have you seen?

Luckily, we found out that mouth breathing is the culprit of Bella's yellow tongue! We knew that Bella was still breathing from her mouth most of the time, even though she has had surgery to correct the stenosis, but did not realize the extent of her mouth breathing. This confirms that the surgery was not as successful as we had hoped for...

You might ask, how does a person's tongue turn yellow from mouth breathing? Well, when a person uses their mouth to breath, it causes inflammation of the papillae. When the papillae is inflamed it appears a yellow/orange color and easily catches bacteria which can aid in the color change.

We know that mouth breathing is not ideal and can cause health problems over time. We are going to talk to Bella's ENT about this more in depth and try and figure out when it is the right time to do the second surgery to try and better correct her Pyriform Aperture Stenosis. We are aware that there is a possibility of more surgeries for her sinuses, adenoids and tonsils in the future, but we don't know if it is ideal to wait till then to repeat the corrective nasal passage surgery.

Bella snores a lot at night, she always has, and it has been considered 'acceptable' for her condition in the past, but now her doctors are concerned about what will happen once the adenoids start to grow into the already narrowed nasal passages. We have been asked to watch her sleep more closely and hope that we do not see increased distress over the next several months! I am still hoping that we do not have to do another sleep study for a while and that her adenoids don't cause much concern in the near future, but I know that we are very limited on space in there...

For now, we will enjoy Bella's yellow tongue, and cherish it as one of her special characteristics ;)

Message Of Hope

This is such an awesome message for all of you other moms and dads struggling with the emotional and physical toll of having a child with special needs :)

This video is about how families, of children with disabilities, responded when they were asked about what they would have told themselves the day that their child was diagnosed...

You are not alone, there is HOPE!

 

This & That

This past week was supposed to be full of therapy, but ended being a quiet week as two of our therapist called in sick, and I came down with walking pneumonia (of all things) later in the week. I finally have some energy today, enough to sit on the couch and write a blog, at least ;)

We did make our physical therapy appointment in Cypress on Monday, which actually made me a little bit depressed. Both of our at home therapist for developmental and speech called in sick on Monday.

Physical therapy is hard sometimes. You see progress, then you don't. She does some things SO WELL, then others I feel like we are so far from (walking). She stands against furniture so good, but when we are in the middle of the room, she cripples. Her feet will barely even go on the floor. No balance, no weight, no trying...just fear. We talked about what happens when a child has balance issues and won't walk unassisted. We talked about using a walker (medical) around age two and praying that she will gain some balance in the near future. We are hoping that she will begin to show some signs of standing and walking independently soon, we just wont know until she does it.

The speech therapist ended up coming on Tuesday, which was just an evaluation for Bella. We have had a referral for speech for a few months and are finally getting to have an evaluation. The therapist was super nice and very interested in all of Bella's issues. Bella will be 14 months old this week and has been evaluated at an age level of 7-11 months. We knew that she was pretty far behind, she is actually at this same age level for physical therapy too. They have suggested that we start therapy at 2x per week for right now and increase more if we need too.

The therapist did note that Bella has some high tone in her face, which is what she also has in her trunk/ legs. She said that she has not seen high tone before but also does not think that it is going to affect her rotary chewing too much. She does have rotary movements, but not a lot. We will have to work on this as this is why she is not able to eat many table foods.

We are going to start with something called "food chaining" as well as some muscles exercises to try and stimulate more rotary chewing (where you chew from side to side in your mouth). We are also supposed to put the food into Bella's mouth on one side using our finger to stimulate the rotary movements.

Food chaining was explained to us with an example of a french fry, which is definitely not the healthiest of foods, but an easy example to follow.

When you begin food chaining, you pick one food to use as your "food chaining" food. Each day of the week you use your same food (french fry) and start the week by using a very soft fry. Let her eat it like that then the next day, make it a little bit more firm. By the ended of the week, she should be eating the french fry in a very crunchy form. This way, she is used to the food but gets it in a different texture every day, slowly getting her up to a very textured food.

We are hoping that this is going to help her tolerate other foods. Right now she can only tolerate soft breads, cheese, yogurt, and goldfish (at times). Hopefully in the next few weeks we will be able to say that we have successful introduced another food, or two!

We will also start to do transitional foods in the next few weeks. This is when you introduce a unfamiliar food, with a familiar food. She can only have the familiar food once she tries the unfamiliar, and so forth.

We all bribe our children with "once you eat the rest of this, you can have this", but when it has to do with an actual physical issue rather than just a personal dislike, it becomes more of a problem!

Along with all of the new feeding therapy, we will work on the speech (which will come more when she starts walking) and also start learning some sign language. Sign language is going to be great for Bella to start learning early on so that we will all be prepared if she does loose some of her hearing. It is also a good way for her to express herself until she is walking and talking more :)