This is a common question. What is Kyphosis? In medical terms it is this:

Thoraco-Lumbar kyphosis in achondroplasia. Most babies with achondroplasia develop a ‘hump’ in their lower back (sometimes referred to as a gibbus). For most of these that early kyphosis will go away by itself without any symptoms resulting. An x-ray of early thoraco lumbar kyphosis in a baby with achondroplasia is shown in Figure 4. In few percent of individuals with achondroplasia, however, that kyphosis will become stiff and angulated. And in those individuals, nerve damage can arise later on in life that, if not cared for, can result in paralysis. Rather than having to treat the kyphosis at that stage, what we would like to be able to do is prevent those severe kinds. Evaluations done here (and similar assessments carried out at other centers) suggest that some simple interventions may be effective in preventing those complications. It seems that if one can prevent gravity from exerting abnormal pressures on the bones of this region then much less frequently will the severe curves develop. How can those effects of gravity be minimized? It seems that prohibiting any unsupported sitting in the first year of life and always providing good back support markedly decreases the risk of serious and rigid curves developing. About 85% of all babies develop some kyphosis. With careful prohibition of such unsupported sitting all but about 15% of those will take care of themselves. In our clinic the remainder are braced for a time and in that group none have gone on to have worrisome curves.

For those of us who read this and said “huh?” it is a curvature of the lower spine. To easily explain this, place your hand in the middle of your back. The part that curves in right above your pelvic bone. That is a normal spine. With Kyphosis, the spine actually curves outward.

This is a visual time line of Knoah’s spine.

January 05, 2007

May 23, 2007

January 21, 2008

This is the reason we need to find another Orthopedic Surgeon. As of today’s date, Knoah’s Kyphotic curve is 74 degrees.

In a majority of children with Achondroplasia, 15% of children will end up being braced. A large number of those children will be braced after the age of 3. Very few (Ain’t we the lucky ones) will be braced before their 1st birthday.

Knoah’s current Ortho seems unwilling to work with or even contact Dr. Pauli. Why, I don’t know. It would seem to me that she wants to be a “SPIT-R” aka the smartest person in the room. Because of that, Knoah’s spine has gotten worse, despite bracing.

It is widely accepted that the following will minimize the degree of Kyphosis:

Most infants with achondroplasia develop a thoracolumbar kyphosis. More severe kyphosis is associated with unsupported sitting before there is adequate trunk muscle strength (Hall, 1988; Pauli et al., 1997). Parents should be counseled to avoid unsupported sitting and to avoid devices that cause curved sitting or “C sitting,” such as “umbrella-style” strollers and soft canvas seats during the first year of life. Use of feeder seats for upright positioning should be recommended. If severe kyphosis appears to be developing, consider a pediatric orthopedic surgical assessment to determine if bracing is needed (Pauli et al., 1997).

In other words, NO UNSUPPORTED SITTING! None! It is best to keep the baby flat on either his/her back or tummy for the first year of life. Encouraging early sitting is asking for trouble.

Now that I have said that, you ask “Why is Knoah’s back so bad? Don’t you write a blog about raising an Achondroplastic child? What did you do wrong?”

The answer is nothing. I did everything that is recommended. For his stroller, I used a Pram. His car seat was padded with extra padding. I never let him sit unsupported, until he started sitting on his own. Still, his Kyphosis has increased. It happens. In talking with Dr. Pauli at our July meeting in Grand Rapids, I asked him this question. Why? Unfortunately, in some children, there will be about 2% of Achon’s who develop a progressive kyphosis despite doing everything right. Again, lucky us.

I know it is causing him pain at this point. When he gets into a sitting position, he doesn’t stay in it long. I try to massage the sides of his spine, why, I don’t know. I am hoping it relives some of the pressure off his muscles.

Casey gave me a few names of doctor’s who work with Pauli. Their in Grand Rapids and in Madison, Wisconsin. I don’t know how my insurance will feel about it, but I have started the process.

With a progressive Kyphosis, damage can occur to the vertebrae. This is called Anterior Wedging. When the vertebrae start to hit each other, bone fragments start to break off. This leads to symptoms such as back pain, respiratory dysfunction, neurologic involvement, or symptoms of spinal stenosis.

When the Kyphosis starts to become fixed (unable to reduce the Kyphosis by placing gentle pressure on that part of the spine), surgery becomes the only option to stabilize the vertebrae.

We DO NOT WANT THIS! So, we are on our next journey to hunt for an Orthopedic Surgeon.

Hallelujah! He’s drinking!

I have been pumping him full of the pain medication. After 30 minutes, he’s good to drink! I have been alternating between his formula (Prosobee) and Pediasure. The pediasure has 250 calories per serving, so I figure if he drinks a majority of it, he’ll of compensated for the lost caloric intake. It’s not like he’s anywhere being overweight, yet. He only weighs 18 pounds.

Let’s keep it up big boy!!

I just want to say, Thank you for all the support we have been given during this latest journey! Our life can get overwhelming and this blog helps me vent. I so appreciate everyone who prays for us! I sometimes don’t pray because I know I get tired of hearing me ask the good Lord for a miracle, so I figure he must get really tired of me ask.

A all too common question in my house is “Hey, is he breathing?” The response is always “Yeah, why?” My response is “Just double checking”.

I am glad God has placed this blessing on me. I struggle, but I am glad. I just hope, he is.

It has been a long day!

Knoah decided he was going to stop drinking last night. I called the peds today to find out how long he should go without drinking. He advised me that it is common for children to not want to drink because of the pain. He said that if he doesn’t start to drink by Friday to call the Ortho or bring him in to him.

I went to the store and bought him a bunch of Italian Ice, Pediasure Popsicles and mini-ice cream bowls. He ate a bit of the ice cream, had two tablespoons of rice and 4 ounces of formula.

Will tried to give him some mashed sweet potatoes a while ago and throw it up. Wow! Did it stink! Luckily dad was holding him…snicker.

I am hoping this is just a minor set back and he will resume drinking again. I can’t threaten him with an IV, because well, he wouldn’t understand. I could walk around with a white jacket, but that would cause him to start crying uncontrollably.

He is asleep now, so I will place a bottle in his playpen (that’s where he is right now) and maybe he’ll drink and sleep. He’s known to do that.

As I mentioned early, Knoah’s surgrey was flawless! The ENT said that his Tonsils and Adenoids came out without a problem. He did shorten Knoah’s Uvula* and didn’t have to touch his palate!

Definition of Uvula

Uvula: The small piece of soft tissue that can be seen dangling down from the soft palate over the back of the tongue. The uvula is described variously shaped like a U, a tear or a grape. Its name comes from the Latin word for “grape,” uva.

In recovery, they made the decision to put him in Acute Care instead of a general room, because his O2 levels were dropping.

Last night was hard as Knoah was in a great deal of pain and his perscribed medication wasn’t helping, so they put him on a Morphine drip in addition to his meds. He recieved two rounds through out the night and it helped.

Knoah had Teddy there and it was a comfort to see his buddy with him. At one point, I pressed Teddy’s nose and Knoah, although not feeling well, smiled.

The ENT said that by removing them it has given Knoah 4 or 5 times more breathing room! You can tell. He sounds like a windtunnel instead of a snorting pig. The full results should be seen in about 4 weeks, but I think the surgrey was a success!!

Thank you all for the prayers and well wishes!! You mean everything to me!!

I’ll leave you with pics of Knoah’s T&A journey!

I told you he was flexible…

Yes, he even had ice cream!

*Funny Story:
Hannah says to Will one day (she saw Monster House, he didn’t) “Daddy, do you want to see my Uvula”? Will looks stunned and says “No Hannah! You don’t show anyone that! Those are your private parts!!! WHY ARE YOU (looking at me) LAUGHING?”
Hannah looking confused, looks to me and I asked Will if he knew what her Uvula was. He says quietly “Yes, it’s her… (Vulva) but how does she know?” I hugged him and said, “A Uvula is that thing in the back of throat. Uvula, Vulva, they use the same letters, two different parts of the body, babe!” Will, quite red by this time says, “Yes, Hannah, go ahead and show me”.

We are back home now. I am exhausted, Knoah is in pain. I will update later tonight, but all in all, the surgrey went very well!!

Thank you for the prayers and love! I know they have help him get through this!!

Monday morning Knoah will under go a Tonsillectomy and Adenoidectomy (T&A). In children with Achondroplasia, the Tonsils and Adenoids pose a breathing obstruction during sleep, AKA Obstructive Sleep Apnea or OSA.

Knoah’s last sleep study revealed he was still having a significant amount of OSA. This causes concern because while your sleeping, your breathing becomes shallower. When it becomes too shallow and you have OSA, your blood oxygen level decreases or you have Hypoxia. This can be life threatening if left untreated.

Oxygen is required for all humans to live. When your oxygen level decreases and your vital organs work harder to maintain your body. This will lower your bodies ability to recoup after an injury, illness or leave children mentally and physically delayed.

We are hoping that by removing Knoah’s tonsils and adenoids, he will no longer experience the apnea, he will sleep better and his physical activity will not exhaust him.

I had explained to the ENT that when Knoah is sleeping, it sounds like he is breathing against a wall. In effect he is. His tonsils are blocking about 50% of his throat. The ENT remarked that Knoah’s throat was narrower than most children with Achondroplasia and any illness or injury to that region of his body could be deadly. So, night after night, I listen to him sleep. Some nights he is so loud that I can hear him from the first floor. Which for me, is good, I know he is still breathing. For him, it’s bad, he isn’t getting a good night’s sleep. He wakes up right now on average of 4 or 5 times a night. It makes for a tired momma and crabby baby.

He has also had the recurrent sinus infections. With everything beening block in the sinus cavity, any virus he gets stays there and he isn’t able to get rid of it. I don’t mind Snot having a runny nose, but he has produced some of the biggest boogie’s I have every seen! His boggie’s hang out until the shear weight of them cause them to fall out and goodness, there HUGE! LOL

We have already received the Rx for the antibiotic and pain killer. So for the next several days, Knoah is going to be high. They are giving him Codeine. This should be fun for us. As if he’s not unstable enough.

I have been researching T&A and found it is the most common surgery ENT’s preform. It also carries a 1 in 250,000 risk of death. That’s the ideal rate!

Major concerns with a T&A are excessive bleeding requiring a blood transfusion, poor fluid intake and infection. About 4% of children will require additional hospital care and surgery because of uncontrolled bleeding.

So, about 11 am or so Monday morning forward, I will be a nervous wreck. I am relived to know that they will keep him for 23 hours or so after the surgery to watch his fluid intake. Thank God, because trying to get anything down him when he isn’t feeling well is like trying to remove the ginormous boogie’s. It’s not happening.

Please pray that everything goes as planned and he is as comfortable as possible.

I hope he makes a speedy recovery and is back to his self in no time.

Yesterday, while doing my usually running around, I decided to stop into Sensational Beginnings to use a gift certificate Knoah recieved for his birthday.

I had been browsing their website for the last few weeks trying to decide what to get him. I knew he really didn’t need any more toys (although I must add, SB has some REALLY cool toys!).

He recieved the gift certificate from an unnamed person and I didn’t just want to buy him anything. The person who gave us the gift certificate is very special to us, so I want to be able to say to Knoah in the future this item came from so and so.

Well, I bought his gift.

Knoah is getting his first step stool.

Standing at the counter yesterday, talking with the sales associate, I explained Knoah’s Achondroplasia and what I was wanting to do. In finally deciding to purchase the step stool, I became teary eyed.

It was that moment, it concreted to me that this step stool, Knoah will have the rest of his life. He will not simply reach into the cabinet to get his cereal. He will not reach over to turn on the facet to brush his teeth. This wooden stool will help him accomplish daily activities in life.

It was a moment of “it’s true”. I envisioned step stools strategically placed around the house. I envisioned wooded platforms to raise him to a level that he could live on his own.

As I stood at the counter and shed my tears, I am sure they looked at me like I was nuts. Buying a stool for a child’s 1st birthday isn’t something we parents think about doing and if we do, it’s temporary. His, is for life.

It is a bittersweet moment for me. Where Jewish boys have Bar Mitzvah, Knoah gets a stool. A rite of passage, into a life I couldn’t possibly know what it is like. A normalcy, he will have.

I think about him getting his first step stool and I want to cry. Not because I am sad for him, because I’m not. I can’t put words too it.

I know this stool will liberate him. In some cases, he will not need me to help him. He will be able to do the same things his brother and sister does, only modified.

I don’t know if this was a premature purchase, it’s not like he’s going to use it the minute it ships in. It will probably sit in the corner of the room, quietly calling out, reminding me of his Achondroplasia. Everything I have done since the minute he was born has been because of his Achondroplasia. Holding him, buying clothes for him, feeding him. Always present, always reminding me. It is those spare quite days, when I forget and he becomes just my baby. Just as mischievous, funny and loving as the next child. A child who has just required a little bit more attention and medical care than the next. My son, who I am proud of, regardless of what he needs.

Knoah’s nickname…Snot. Doesn’t require an explanation.

Two years ago the thought of baby-proofing my home was a thought of the past.

“NO MORE CABINET LOCKS!”

“NO MORE BABY GATES!”

“NO MORE FEAR OF CHILDREN CLIMBING UP THE STAIRS AND FALLING DOWN REQUIRING UNSCHEDUALED TRIPS TO THE ER!!”
…well, ‘cept for maybe Hannah, we know how graceful she is…

My valiant cries are mute.

Snot decided that last night he was going to free himself from the confines of the 1st floor. Curiously, he looked around.

“It’s safe!” He thought, not realizing momma IS ALWAYS in ear shot of his pidder-patter.

Snot look at his conquest, determined to climb the “Mountain of Wood”. He prepares, breathing deeply as he raises his arm up.

Naysayers who came from around the world, expressed their feelings.

“He won’t do it, he’s too small!”

“Snot is not like us, he’s disadvantaged!”

“Look folks, I’ll be honest, he’s short. Everyone knows only tall people are successful!”

“I can do it” he babbled. “I can do…Oh MOMMA!”

Yes, momma was right behind him. He feared she would remove him from his flight of glory before he began, but momma encouraged him! “Go ahead, you can do it!” “You can do ANYTHING you set your heart too!!”

As he climbs each step, he realizes he will have had his workout for the day.

Momma realizes if she lets him do this, he will be a sleep early!

Climbing, breathing ever so deeply now, Snot thinks he has taken on too much.

“Go ahead Knoah, just a bit further”. Momma says.

Snot, getting a second wind after resting a moment, sees the summit!

“AGGHHHHH” he screams. It radiates through the house.

The crowd screams as Snot accomplished the “Mountain of Wood!”

“HE DID IT!” “HE REALLY DID IT!”

With rosy cheeks and heaving breaths, Snot looked at his fans and thanked them!

Then with a turn of events, Snot looked at the Naysayers and said….

“You may kiss me now!”

Snot, reaching up to momma for congradutory kisses and some of the required sponsored formula, gently lays back into his mother’s arms and breath a sigh of relief. He did it!

Momma wanting to see what he could do, knew he would do it. She also knew, it being close to bedtime, she would have a good night’s sleep!

*DISCLAIMER* All dust bunnies were killed with the making of the story!!

Knoah at 3 weeks old.

So, I ended my last post with “jealous yet?”

There was a hidden meaning…it appears that most of us moms with little ones (below average, shorter than standard, not on the charts children) have been going through a “phase”. I’ll call it a “phase” because frankly we will never grow out of it, we’ll just get use to it.

Explanation.

I was talking with my mom the other morning about my “jealousy”. Knoah is 14 months old. He says ma-ma, da-da and baba. He is not walking yet, he refuses to catch on to the idea of using a spoon and is simply delayed.

As a mother of a child with Achondroplasia, I expect this. It is “normal”.

As a mother of a child, it still doesn’t take away the fact that you want your child to be like everyone else.

I can live with the fact he is small. I can live with the fact it is ever increasingly evident. I can’t live with the fact that society will look at him as something is wrong. I, in fact sometimes look at average height (AH) children his age and think “what is wrong with them?”

Seeing Knoah on a daily bases, he looks like a baby should, but then I get him out into the public and see AH children and this is what I see:

Their heads are too small, their arms are really long (they kinda of remind me of monkeys), their legs are long and skinny. They look like walking mini adults.

Then I realize, people are looking at Knoah thinking, he looks funny. His head is almost the size of mine, his arms look like nubs sprouting out from the sides of his body and his legs are just too short. He looks like a star. All sides even with exception to his head.

Then I see AH children doing and have things Knoah does not.

I want to see him walking, I want to hear him talking. I want to find clothing for him that reflects his age and not his stature. I want to look at other children his age and not wish he could compare to them. When we were at the doctor’s office one day, there was a little boy who was 2 months older than Knoah. This child looked like a giant standing over Knoah. He kept repeating “Baby, Baby”. I smiled. The child’s mother said “Be careful, he’s a baby”. I wanted to say he’s not a baby. He’s almost a year and a half, but there lay Knoah on the floor, probably praying the kid doesn’t step on him.

Visually, I see a baby. He’s no bigger than a 4 month old infant. Common things the other two were doing, he is no where near doing yet. Jacob knew his colors by 15 months. I struggle getting Knoah to say ma-ma.

How do you help yourself in the transition from what you expected to
reality? What things do you worry about with Noah? Any advice or
suggestions are appreciated.

This question was asked in one of the comments.

That’s a loaded question. How do you transition from what you expected to reality? I don’t know that you can. I think we parents will always look to other kids and mourn what our kids won’t do.

It’s not that we lost our dream of the perfect child, but we have a different sense of what perfect is.

Mentally, I know Knoah is a dwarf. I can see it. I am okay with it. Then I see a dwarf adult and my mind scrambles. It is like it comes to a screeching halt, trying to understand what I am seeing. Then it hits me that this is what he will look like as an adult. He will look different from Jacob and Hannah, he will look different than I or Will. He will face challenges we don’t think about, yet, I already know this, but it is still shocking.

As humans, we need to know “it will all be okay.” It will be for Knoah, it will be for us, as a family.

We need to know our children will want for nothing. That our children will do what they want to in life. That if they set their hearts on it, they will accomplish it.

Will and I have already started the process of modifying the house for Knoah’s needs. In the next few weeks, we are going to be installing a bar, just above the base board so Knoah can safely pull himself up an walk along the wall. It’s not that we have to do this, but we want him to experience “cruising” like any other child, without retching his head back to pull himself up on the furniture.

I have already purchased Knoah’s big boy bed. Which is funny, because he hasn’t slept a night in his crib yet. He is still sleeping in our room. The bed is an antique 1880’s child’s bed. We have to have a mattress made for it because a standard mattress will not fit in it, but when it is put together, Knoah will be able to crawl into bed without assistance. This bed, I hope, he will use till he gets married.

Still, I see an adult LP and reality sets in. He will eventually move out of our house and face the AH world. Terror sets into my mind. What is he going to do if I am not there?

He will have to make do. That’s the reality.

I have always said the human mind is an amazing thing and we tend to make things worse than it appears. He will go to school with AH children, he will experience bigotry and hatred. He will experience love and joy. He will come home from school with smiles and stories of rewards, he will come home from schools with tears and stories of defeats. That is life. We all have them. People who intentional hurt others are out there, we can’t escape them. We can’t make them get to know us or see what is in our hearts. They will make blanket assumptions about who we are without sincerely sitting down with us and talking with us. That is their loss. People of that caliber are far and few between and the majority of the world is compassionate, kind, sincere and trustworthy.

I am as new to this as the next parent. I will fall at times and make nasty statements to the nasty remarks that are made about Knoah and I will be able to walk away from others. I will defend my decision to give birth to him till the day I die and I will tell him I love him the same.

We (parents of special needs children, here and in heaven) were chosen for a blessing. A blessing, that is tiresome and joyful, filled with specialists appointments, surgeries, consults and second opinions, but we, the lucky few, were given second sight into a world we would have walked past and just quietly said “that’s too bad” and made our way through life.

We have to learn too accept our own feelings and fears about our child not being “normal” before we can expect other’s too.

Though, as I have said “What is normal?”

This is what he is doing today.

Jealous yet?