Landen's Update: Day at the park a much needed break

Friday, April 27, 2012

Day at the park a much needed break

So I feel like I'm about to loose it to the stress! I'm knee deep in fundraising stuff combined with the hours of therapy and one doctor appointment after another and trip after trip to Flagstaff... and when I'm not in the doctors office or the car I'm at home just trying to keep it all together. Granted I know that there are those of you reading this who feel like I'm just complaining but I'm so close to burn out. I needed a break I needed to get out of the house! So on this beautiful cloudy day I decided to take the boys to the park. Between naps, meals, therapy appointments and the fact its shopping day I could only squeeze in a short 30 minute trip. But on the way there I just was DONE my head hurt and I was tired! I wasn't going home and I wasn't going to the store I was going to sit in the grass with my boys. I was going to enjoy some time doing nothing more then have fun with my boys. So I stopped and grabbed some lunch and we went to the park.

We ended up staying for over three hours. The boys got to swing, Brayden got to run, Landen got to walk and all I had to be was mom. It was even nice to be the only people at the park. For once I can get out Landen's walker and watch him play and explore without other kids there reminding me what Landen could have right now. Or them following him around asking why does he have that walker and why doesn't he talk to them. Not that kids aren't wonderful but they aren't always the most tactful. So was nice until Landen decided he didn't like anything but to sit in my arms. Don't get me wrong I love snuggling my Little Landen but I had to focus all of my attention on Landen leaving Brayden to play all by himself. It hit me hard... poor Brayden. It suddenly made me realize why I have such an independent little boy. Brayden is so often left to entertain himself while I'm taking care of Landen. I feel so bad for him I want so badly to be able to chase him around the park and sit and read with him all the time but its just not always that way poor Brayden doesn't get as much mommy as he deserves. I guess that's why Heavenly Father made him so darn freaken CUTE! He's always able to get attention when he needs it. The day got better after a quick 20 minute snuggle nap in my arms Landen was up and ready to go! So glad I decided to stay I really think both my boys enjoyed the day too!

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About Landen

Landen is SIX years old! He loves loves loves his daddy (and his mommy too). After a long struggle he has learned to walk with the help of a walker. Landen was wearing hearing aids since he was 2.5 months old and did very well with them. But eventually he lost the last of his limited hearing. In March 2013 he received a cochlear implant that helps him to hear again and he loves it! This disease has kept him smaller then most babies his age but he has the personality and love to make up for it! His little brother passed him up long ago.

He loves to close his eyes and feel things I don't know if its because of the issues with his eyesight or a personality thing. He is not talking at all yet. Even though he doesn't have words or signs yet he knows what he wants and will tell you in other ways. I guess because I am his mommy and spend every minute of his life with him I understand him. I get to work as his interpreter with others. He is strong willed when he is trying to learn something and doesn't give up on something he has already put his mind to. Landen is a loving child who loves to cuddle. He will sit on your lap for hours is you are willing to play patty cake with him! I often come in late at night or early in the morning to see him lying next to his daddy stroking his arm. My favorite is when he sneaks up behind me to play with my hair! I've never met such a snuggle bug! We Love our Little Landen so much! He may not be destined for a long life but it will be a happy life!

About PBDs

Peroxisomal Biogenesis Disorders are complicated genetic conditions with many big words I will try my best to explain some basic concepts and include links to some websites to help you understand it. I'm explaining it the way I understand it please forgive me if I make a mistake this is a learning process with a disease that is rare and therefor hard to find information on. I will correct things if I come across new information.

Peroxisomal Biogenisis Disorders include three disorders Infantile Refsum Disease (IRD), Neonatal Adrenoleukodystrophy (NALD) and Zellweger Syndrome (ZS). The distinction between the disorders is based on severity Zellweger Syndrome being the most severe and Infantile Refsum Disease the least severe. But it is often impossible to put a child solely in one of these diseases. Landen is thought to be on the IRD end of the scale but he has also had blood work suggesting he may be more severe. For that reason we just like to say he has a PBD.

Peroxisomal Biogenesis Disorders affect every part of Landen's body. PBDs are caused when the part of the cell that breaks down substances the peroxisome does not work properly. Peroxisomes break down substances like long chain fatty acids. In Landen's case his cells are unable to break down these substances. These substances build up in the blood stream and can be deposited in various tissues and nerves causing damage.
Symptoms of this disease include hearing loss, retinitis pigmentosa, nystagmus, mental retardation, loss of reflexes, muscle weakness, poor balance and retarded growth. These symptoms vary from patient to patient. Some babies learn to walk, talk and read while others are severely cognitively delayed and confined to wheelchairs. Our Landen has shown signs of severe cognitive delay and muscle weakness but he is determined to move and learn
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PBD is genetic and is inherit recessive meaning both Scott and I are carriers of a rare mutated gene. Landen has received one from each of us and is now is affected by PBD. Brayden or youngest shows no signs of PBD but still has a 50% chance of being a carrier a 25% chance of having two normal genes and a 25% chance of having PBD or both genes are mutated like Landen. Although Brayden's development and growth suggest that he is unaffected. Any future children we may or may not choose to have will also have a 25% chance of inheriting this disorder.

PBDs have no cure and is eventually fatal but we are doing our part to control it and treat the symptoms. First we try to control it through diet. One of the most dangerous and common substances that build up is phytanic acid. It is a by-product of photosynthesis and is only made in the green leafy part of plants. So Landen doesn't eat any animal product from animals that eat leafy greens AKA no beef, lamb, fish or dairy products. Symptoms are addressed and treated as they appear. He has a cochlear implant. His vision problems are uncorrectable with glasses but we are closely monitoring for change. Physical and Occupational therapies are key in helping him to grow and learn. Landen has shown great improvement since starting these services.

Although this disease will eventually take our son we will have some warning before it is time for him to go. His motor and cognitive functions will regress as more nerve damage occurs.
We love our baby boy and refuse to treat him like all is lost. We will teach him all that he wants to learn, give him all we can and love him forever! We appreciate the love and support of our family and friends as we start this journey together.

Landen's Update

My Little Landen

Friday, April 27, 2012

Day at the park a much needed break

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