Knoah had his Orthopedist appointment today. We drove to Ann Arbor alone. It was a nice, quiet (yeah right) ride. Knoah sat in back just yelling and screaming. He wasn’t upset, he was talking to me.

We get there and I am told to go down to have X-Rays taken of his spine.

It was horrible! You should have seen this contraption they put him in. The nurse says to me before hand, “It is safe, you might find it scary though”. I am thinking, how bad could it be?

Well, let me just tell you, they sit him on a piece of wood about 4 foot up in the air. His legs are dangling down and they strap him in with a large Velcro belt. This isn’t so bad.

Then the other nurse comes over and take Knoah’s arm and straps each one into it’s own little Velcro thing. Knoah looks like he is waiting in the dungeon, chained to the wall. It as terrible. I, being emotional anyways, start nearly crying because he, looks so sad. He looked like he was waiting for the executioner and not a simple X-Ray.

We try to get his attention by calling his name. I, of coarse am wiping tears away, smiling as big as I can to keep him facing us.

Pathetic sight really.

We get done with the appointment and we walk back to the Center.

The verdict on Knoah’s kyphosis. Bracing. Knoah is going into a TLSO brace. We found out that he also has scoliosis. That one shocked me. I didn’t expect it to be a bad as it was.

His curvature for his Kyphosis is 53 degrees. His Scoliosis is 23 and 21 degrees. I called to make the appointment to get his brace. We have to go to a place in Toledo.

I am praying the insurance company pays it. We have a 50% co-pay and I did a quick search….$2000 for the brace! The way I am reading it, he will have to be in it until he starts walking, at minimum. Most kids have to wear it until they stop growing.

Huh, well we won’t have to wait that long. Snicker. The only issue with this type of bracing is, is that a new one has to be made as he grows. It is not adjustable.

We will have no choice but to get air for the house now. The poor kid will have a big, plastic clamp around him 23 hours a day. More fun for mom!

Though I hear most kids learn to adapt to it in weeks. It will be helpful to Knoah when he starts sitting up and crawling. The brace will support his spine instead of himself. A friend of mine, who I met through POLP, has a son who had to wear a brace when he was younger. I will have to email her specific questions I have.

The Joys of Motherhood!

I have to make this a ranting post since I haven’t had one in a while. It will probably be rather long. Apologies!

Mostly everyone at this point has heard about the Chris Benoit tragedy.

In one of the news reports I read said that their son, Daniel was given growth hormones because he had dwarfism.

Obviously this peeked my interest as to why they said ‘he had dwarfism’. Yesterday morning, on 1130AM The Fan (Sports Radio) they started talking about the Benoit Family. They again mention Daniel and say he had dwarfism. Since this was local and I knew who to contact, I did. When I got on the air I basically said:

“I have a concern with this, they have said that Daniel had dwarfism and that is the reason his parents were giving him growth hormones. It needs to be made clear that there are over 200 types of dwarfism and not all require giving a child growth hormones. My youngest son has a form of dwarfism call Achondroplasia and I could give him growth hormones till he is 100 years old and it would literally only add about 4 inches onto his final height. I think we as a society have place so many expectations on what a child is suppose to be that when a family is given a child with a difference, they somehow need to find a cure for them. My understanding is that the child was small. Some children are just naturally small and that alone is not a reason for giving them growth hormones. We as people need to become educated about our children. I think that Benoit may have been giving his son the growth hormones because as a wrestler, he felt he need to have a boy-boy. That he had to conform to the image of the all American boy and because he felt he didn’t, he was bulking him to fit that image. We need to learn that the public perception of the all American boy is not real and that Daniel was an all American boy, my son is an all American boy. They are not something to be ashamed of.”

Then it comes out that Daniel suffered from Fragile X.

In layman’s terms, Fragile X is an inherited mental impairment.
Features usually include:

mental impairment, ranging from learning disabilities to mental retardation
attention deficit and hyperactivity
anxiety and unstable mood
autistic behaviors
long face, large ears, flat feet
hyperextensible joints, especially fingers
Seizures (epilepsy) affect about 25% of people with fragile X

Boys are typically more severely affected than girls. While most boys have mental retardation, only one-third to one-half of girls have significant intellectual impairment; the rest have either normal IQ or learning disabilities. Math is often a particular challenge for girls. Emotional and behavioral problems are common in both sexes.

About 20% of boys with fragile X meet full criteria for autism. Most boys and some girls have some symptoms of autism, but many tend to be very social and interested in other people.

Height in children with Fragile X is normal.

Notice I said ‘height is normal’. That would be the only reason you would give a child growth hormones, is if growth was affected. His height, his natural height was not going to be affect. Could Daniel have just been small because his parents were not big?

Sometimes, no matter how hard we try, there will be things we don’t understand. That is just the way it is.

Summer is in full heat and Knoah is miserable. He even thinks about breathing and he breaks out into a sweat. His poor little head yesterday was nothing but beads of sweat. I tried to keep him cool, but it didn’t work. When we went out to his PT appointment, I was asking the therapist about where to go to find help about getting an air condition for the house. We don’t have central air or an air conditioner and it gets extremely hot in the house. I am amazed how warm it gets. The other night, at about 11 pm, it was 88 degrees. In the house! The poor kid has to sleep with a fan in his bed. (No, he can’t reach it)

So she suggest I go over to MCOP. I am just curious to find out if there is a program that will help families pay the electric bill for an air conditioner. That is what is preventing me from getting one. I don’t want the temptation of having it in the window, using it and then face that bill at the end of the month.

I believe it would be considered a medical necessity for us to have one for him. Not only is his own thermostat broken, but he is also on the verge of having allergies and possibly Asthma. I know an air conditioner will help alleviate some of his symptoms.

So, I am going to look into it today. I don’t know if it will help, but I will try. My other issue with doing this is this. Many of the Directors and community agencies set up to help low income families are the same people that my husband works with on a daily basis. So, I don’t want to approach one of these agencies and ask for help and then have them attend a meeting with my husband the next hour. I know I have heard that it must be that I think we are better than other people or that I think we are entitled because of my husband’s position. Whatever! It has nothing to do with that. It has everything to do with pride. It has everything to do with self sufficiency.

A few years ago we were self sufficient. We could afford to buy the kids a new toy or I could go buy us new clothes.

New, now has new meaning. New means we didn’t own it beforehand. New means it was brought into the house for the first time. I haven’t bought a new outfit in a year. I haven’t gone shopping for clothing since Knoah was born. I don’t want to spend the money on me. I know there are sacrifices I will have to make because we have a child that requires extensive medical care. The kids need to see a dentist because they haven’t been to the dentist in over 3 years. Even the dentist that has the sliding fee is too much.

What do you do when people want money and you still have to eat? Where do you turn when you want to be self sufficient and do what your suppose to.

I need to find a job. I don’t have anyone I can rely on to babysit. I am going to stop because this rant will continue on and on…NEE. Our life is a NEE (Never Ending Evaulation).

Like a test in which there is truely no end. A determination that honesty will never come. A paycheck that is really not enough.

It has been one of those few days. I can try to keep a stiff upper lip, I just can’t do it alone.

I need to have a little faith, but what does that really mean?

Faith that it will be okay? Faith that the gas company will work with you and not shut off your gas? Faith that in the end, as long as your not a jerk, you will be rewarded and be given a chance? Problem I see is that most of the jerks get ahead. The people who are just decent, want to be left alone and live their lives are the ones who get shafted.

Chris Benoit. Perfect example. Here is a man who is given the oppurtunity to not only make a difference on a world wide scale. Had millions of dollars to not only support his family, give them shelter, a decent life, but he was also given a son who he could have used his own experiences to educate the world. Decided that it wasn’t perfect enough for him and killed his family. For what? He had it all. Or so I think. Maybe he didn’t. He didn’t even see the chance he was given. I see my chance.

My chance though doesn’t pay the bills. AAHHGG! Vicious cycle, don’t ever fall into.

*Edited to add: Now I feel like a big old jerk! My husband just came back this morning. He bought me two new outfits with the money from his mileage check. I told him he didn’t have too. He said that it’s not alot, but he wanted too. It is more than he even knows! I suck!

Will and I have talked about what having a child with a disability means to our family and life in general. Then I asked him this “How do you feel knowing you have a son who isn’t considered “The All American Boy”.

This entry is written from a Dad’s perspective:

Writing this is odd for me. I am never short on opinions nor am I ever short on what to write. The difference here is that now the opinion and ideals I am to share are about my son, my daughter, my son, my wife and ultimately about me.

Tonya has asked me what is it like to know that Knoah is not considered the “All American boy”. I don’t really think I can answer that question. I wish I could. There was a time when I could, but that time past shortly after Knoah’s birth.

I guess that I should explain something. I am a pretty basic person with relatively concrete beliefs and ideals. The quote that guides much of my thinking is, “It is not necessary to change. Survival is not mandatory.” (Dr. W. Edwards Deming 1900 - 1993). I know that to some, this quote is cold and uncaring, for me it is just the opposite. To me, this quote demands that each of us always evaluate, re-evaluate those situations in our life that are important - work, family, faith, community. So, it was with this mindset that I approached welcoming Knoah, as my son, into the world.

When Dr. Julie, the geneticist with Oakwood Hospital, first told us that something was wrong with Knoah I was scared. I had no possible idea what any of this would mean. I was not even sure if this would be something that I or my marriage was prepared for. What I knew was that I was getting close to forty (39 this past March) with two kids who were awesome, an old house that seemingly needed more work than Ty Pennington could do, a job that was becoming exhausting and a six month pregnant wife that was crushing me with, “I am getting fat aren’t I?” questions. Now, I was being told that this child, my son, would have a genetic disorder that would alter his life.

Preparing for Knoah was really no different, for me, than what it was like to prepare for Jacob or Hannah. I daydreamed about what it was going to be like to hold him and play with him. I thought about what it would be like as Knoah got older, would I be able to ride with him at Cedar Point when he was 17 and I was 57? Crazy stuff like that. Dad stuff like; will his hand be big enough to throw a football? Is he going to be able to take the car out on a car date? Will Knoah be teased by the other kids?

But all of this doesn’t answer the question of how I felt knowing Knoah would not be the prototype, “all American boy”. But, the problem is, I don’t have an answer. Like I wrote earlier, I wish I could. I want too, but no. See what happened is, God made a decision for me that I was unable or unwilling to do. I was “forced” to “change” my outlook at 1:15 am on November 15th, 2006.

Within moments of Knoah’s birth into this world by cesarean, I was confronted with reality. Tonya was groggy and not really “with it” and Knoah was - not breathing. When Dr. David pulled him from Tonya’s womb and handed him to the nurse, his body was limb. As the neonatal doctor began to work on him, it was clear that he was not well. I could see his completion was blue and the Doctor had begun to use the rescue breather.

I was torn more in those few moments than I have ever been in my life. Tonya, who’s hand I was still holding, could not see Knoah and she was wearily asking me, “how is Knoah”. I wanted to tell Tonya that he was fine but she could tell by my anxiety that something was wrong. Knoah was becoming more responsive but before he could be presented to us the Doctors rushed him to ICU.

I quickly followed Knoah. It was then at that moment, knowing my son had been so very close to death, that any thoughts or disappointments that I may have had about Knoah not being the, “all American boy” were gone. I simply wanting Knoah to be my son, alive.

It is true for me, just like all parents, that I love all of my children. It is also true for me, like all parents, that I want the world or my children. But, what I really want, deep down, is just for my children to be safe. What I really want is to be thankful just that they are here.

It does not matter to me what Jacob, Hannah or Knoah grow up to be as long as they are happy. It does not matter to me what cloths they want to wear. What music they want to listen to. Whatever. It’s all window dressing. See, for me, I have made the decision to “change” not just my outlook for my children’s life but for my relationships with them. I have made the decision to “change” how and where I place importance. Because, I know that I wish nothing more than to see Knoah survive to become a wonderful toddler, great tween, awesome teenager and a spectacular adult. I know this will happen because shortly after his birth, thanks to God, Knoah made a choice to no longer be blue and to stay with us, to survive.

I know that there will be difficult things ahead. Knoah will need surgery for tubes. He still requires more careful care (holding, playing with) then Jacob or Hannah did. Knoah will need a brace for his back and he may need surgery to correct this. His breathing remains more forced, “jackhammered”, than other children. Knoah is struggling trying to crawl. Knoah will have a hard time keeping up with other children his age. Knoah will, I am sure, be teased by others. I am sure that I will want to do everything I can to protect him, to save him. But, I have to always keep in mind that Knoah, without me, made the choice to stay even when he did not have to.

So, how do I answer Tonya’s question? I am really not sure. What I am sure of is that when I watch Knoah look at this older brother and sister with a smile that will melt your heart I couldn’t be happier. What I know is that when I see Knoah looking at Tonya as if she were Miss America and an Angel all in one, I smile. What I am sure of is when I see Knoah smile or hear him laugh, I couldn’t be more satisfied. What I am sure of is that I have three wonderful children with unique personalities and abilities. What I know is that I have a terrific wife. What I know is that “All American”, ideals are already here. My “All American” is a boy that loves running, soccer and reading (Jacob). A girl that love trees, bugs and animals (Hannah). A boy that has fought off death three times, learned to hold a bottle using his feet and was given a laugh that can brighten the darkest day (Knoah) and a wife that somehow is able to maintain all of these unique people while never losing who she is. I have my “All American”.

We have made it a few days without the monitor. I am getting use to not having it. Okay, not really, but I am realizing that it’s gone.
GONE! Meaning I have to rely on nothing to alert me to problems. GONE! Meaning I have to watch him that much more closely.
GONE!

He’s fine with it. He thinks he is wireless.

I have to of coarse add in a little humor and tell you a few advantages and disadvantages of having a short limbed child, as I have come to know.

1. Hugs and kisses are that much closer! Advantage
2. They stay in smaller clothing, longer. Advantage
3. People notice your child’s difference. Disadvantage
4. Little legs mean little feet can touch butt and get poo all up their legs. Disadvantage
5. Little arms and legs mean when he starts crawling, it is will be easier to catch him. Advantage
6. Little arms mean he hits himself in the head with his toys, ALOT! Disadvantage

I have been thinking about his pregnancy and some of the things we were told. There are over 200 different types of dwarfism known. At one point, the doctor’s thought he might have a form of dwarfism called OI. OI or Osteogenesis Imperfecta is a genetic disorder characterized by bones that break easily, often from little or no apparent cause.

With OI, there are five catorgories. People with OI usually are proportionate physically. Now a days, OI is usually diagnoised during the pregnancy or shortly after. Years ago, OI was diagnoised after the child had been brought into the emergency room screaming their little head off for what seemed no reason. Only then did the full body X-ray discover multiple fractures and broken bones and then the parents were told to speak to the police. Suspected Child Abuse.

It wasn’t until he was born that we got a DX. That was one of the reason’s they did a full body scan. They wanted to check for fractures and broken bones. They didn’t find any and the shape of his bones were Achondroplastic.

I am hoping to start adding in post about the different types of dwarfism. When we are educated, tolerance grows.

Well, Knoah’s surgery is canceled.

Remember I mentioned the article in my last post about Anesthetic and Pathophysiology in Dwarfs?

Well, my intention was to give the anesthesiologist the information so they are better prepared to handle Knoah. In a shorten version of the 17 page article. Yes, I read all of it. Three times.
It describes the physical characteristics in dwarfs compared to average height people. For instance if you have surgery, the anesthesiologist will place both hands under your head to straighten it. In dwarfs, do this simple movement while sedated can actually close off the airway. Another is, with some children developing Intracranial Pressure, such as Knoah. If he were to do this in surgery, we could have a huge problem on our hands.

Our hospital is a very good hospital. We have informed doctors. We have honest doctors. The ana who would have done Knoah admitted that they would not have felt comfortable doing this surgery. They admitted that if something were to have happened in surgery, they would not have been equipped to handle it. Knoah is a medically compromised child to begin with. You have to be prepared for anything.

They recommended that we get a referral to see an ana who is in a hospital equipped to handle him.

I thanked her for her honesty and candid conversation. I have found there are some doctors who are so proud, so…whatever that they will say they want to do something to add a notch into their belt. I know, some of the doctor’s Knoah has seen have been testers. In the medical profession, not many doctor’s have ever treated a dwarf. But my son will not be a guinea pig.

So we are getting the referral to see an ENT at U of M. I don’t like the drive, it is expensive to fill the car up. We are going to A2 at least 5 times in the next month.

I am hoping they can get him in sooner than August. The longer the fluid sits in his ears the more damage it can do. We don’t want him to get another infection!

Phew! I am glad that is over with!

He did very well. He might have slept better if I wasn’t bothering him throughout the night. I poked at him, touched him. Anything to make him move. I put his pacifier in his mouth. My thinking is there is research that suggests pacifiers reduces the risk of SIDS. Hey, anything helps!

I prayed and prayed. Later, I prayed again, trying to barter with God.

I did panic a bit, fought off sleep. Tried whatever I could to stay awake. In the end, I lost. My mind said ‘enough’ and I drifted off to sleep.

About 10 minutes later or so, I awoke and touched Knoah. Still breathing, GOOD!

I woke up this morning to the glorious sound of Knoah squelling. His high-pitched, whine. The sound that I heard yesterday morning and was asking him to be a little quieter, mommy was trying to sleep. Telling him the sun has only been up for 47 secs, just give me a little time.

He was awake, breathing, with me.

I am happy!

We also have his appointment with the ENT today. His pre-op appoinment. I have a copy of an article from the magazine ANESTHESIOLOGY to give to the doctor. It basically discusses Anesthetic and Pathophysiology in Dwarfs.

It lists common indicators for anatomic and physiologic abnormalities in dwarfism where anesthesia is concerned. Interesting reading really.

So, I better go, it’s almost time!

Doctor V: “Do you drink Margarita’s?”

Me: “Why, should I start?”

Doctor V: “Have yourself three tonight, we’re getting rid of the monitor.”

Me: silence

See Knoah’s face in this picture. This is how I am feeling.

We saw the Pulmonologist today and Knoah is…monitor free!.! No warning. No, brace yourself. Nope. Dr. V layed it on, thick, heavy and to the point.

BREATHE

Right now I am, okay. I cannot think about it too much, I start feeling anxious. Though he has done really well the past 60 days. He hasn’t had any alarms!

This is a bittersweet time. He finally *Praying* has gotten past his apnea. His body and brain have finally gotten the rhytem right. Breathing is automatic now. I hope.

This is were my faith in God reaches a all time high. Tonight, when I lay him down and my body finally gives into sleep. I will be praying to wake up to find him breathing. I will wake up to find him still here with us.

That monitor was my sanity. That monitor was my, friend. I trusted in it. I believed in it. I gave my faith to it, knowing it would let me know of any problems.

Now, it’s gone. As quick as it came into my life, it is gone.

I feel like I am mourning. I am, I guess. I knew it wasn’t going to be on him forever. I was just hoping it would be on him until, it was safe. When will it have been safe? I was cool with 80 or 85 years old.

He had lived his life, had two or three children. Maybe one or two great-grandchildren. Yeah, 85 would have been nice.

My job now, is getting him through the next 84.3 years. Alone. Without a monitor.

Do I drink Margarita’s? No. I do have some Wine in the fridge.

Remember…MODERATION!

I am amazed at the lack of sensitivity of some people.

I belong to a internet group for parents of LP children and we have been notified that our children’s photographs and images are being stolen and used on various websites. Obviously, these websites are promoting “Midget Bashing” and “Dwarf Tossing”. They are profiting from stolen photographs showing LP in costumes, sitting on barstools, even proclaiming “Midget’s are the new dog”.

So, I have to add copyright information to my blog. I have to instruct people to NOT use my images or any images contained on this site for ANY PURPOSE!

How sad is this? People think it is acceptable to refer to LP’s as the “M” word. It is okay to make fun of, point at and crack jokes about someone with a disability.

Here is a site that explains exactly what I am talking about.

The old adage “Take my picture, it will last longer” has taken on a whole new meaning in the days of the internet.

I freely place pictures of Knoah on my blog because I understand people are curious. I understand that all of our readers what to see how Knoah is changing. We all enjoy watching our children grow. I understand that a mother, somewhere out there may have just found out her child has a skeletal dysplasia and is terrified about it. She wants to be reassured that her child is going to look like every other baby. With skin, eyes and all. I know I did.

I realize that having a “dwarf” in the family is now considered “cool”. Okay, I don’t get it but whatever.

I just wanted people to respect our photo’s.

*EDITED TO ADD:* None of Knoah’s pictures have been taken to my knowledge. I look just about everyday. I will be changing the photo’s on this site to add a watermark with my name and a DO NOT COPY.

The Places Knoah has been!

He has seen Turkeys running wild on the Plains!

He has seen fish swimming in the Pacific Ocean!

He has taken on and beat animals 1000 times his size. See, he’s try to eat it and it’s still stuffed!

All of this without leaving the county! I am impressed!!

He has been to 13 States and London! He is enjoying his travels!
He wants to Thank everyone who has let him visit!

THANK YOU!

It seems it is in the process of ending, but never gets completed.

We saw the ENT today and he confirmed the diagnosis. Knoah has Serous Otitis Media.

This is Dr. Ryan Meredith. He is a Clinical Audiologist. He works in the office with Dr. George Levy. Dr. Meredith is preforming a Tympanogram. It measures the pressure in the ear and detects the movement.

Kudos to Dr. Meredith! He was very gentle with Knoah, even though this test does not hurt. He talked to Knoah and reassured him he was alright. I also got the impression Dr. Meredith did some research about Achondroplasia. That is Number 1 in my book! I REALLY appreciated it! Thanks!

Children with Achondroplasia are highly susceptible to ear infections. This is due to smaller than average passage ways in the mid face region.

The passage way or Blockage of the Eustachian tube during a cold, allergy, or upper respiratory infection and the presence of bacteria or viruses lead to the accumulation of fluid (a build-up of pus and mucus) behind the eardrum. This is the infection called acute otitis media. The build up of pressurized pus in the middle ear causes earache, swelling, and redness. Since the eardrum cannot vibrate properly, you or your child may have hearing problems.

Sometimes the eardrum ruptures, and pus drains out of the ear. But more commonly, the pus and mucus remain in the middle ear due to the swollen and inflamed Eustachian tube. This is called middle ear effusion or serous otitis media. Often after the acute infection has passed, the effusion remains and becomes chronic, lasting for weeks, months, or even years. This condition makes one subject to frequent recurrences of the acute infection and may cause difficulty in hearing.

So the remedy…tubes.

They are going to call me tomorrow to let me know when the surgery is.

I need not say, I am very scared. I will probably be a bumbling, blubbering bag of tears when they take him from me. Not one of my children have ever had surgery. Receive stitches, yes, broken nose, yep, receive a life threatening knee injury that required 10 band-aids. Seen it all.

I guess this is the least of my concerns, right. I mean, were only talking ear tubes. How many surgeries are preform everyday on children? Thousands! So what is my problem? I saw it coming, but somehow, I feel as if I was just hit in the face.

Shock.

I am petrified at the thought of them putting him under. I am worried about what could go wrong. That Knoah will stop breathing. That his airway will close. That his neck will get hyper-extended.

This is where the perils of being educated about Achondroplasia become a conflict with normal medical practices.

I am ashamed of myself. I mean, come on Tonya, really. Knoah could face more serious surgery’s than tubes. I am thinking about the Occipital bone issue. That is next to his spinal cord. Definitely a bigger surgical concern there.

These are just tubes, but this is still my baby.