Jackson's new chair

Yesterday we went and picked up Jackson's convaid cruiser. Which is a adaptive stroller/wheelchair. Jackson immediately got in and said he loved it!! It looks so comfy and I know its going to be such a big help. It's been a big process in getting it so its a huge relief to have it now. A little over a year ago we started looking into getting one of these for Jackson. We knew there was a process and would take some time to get. At the time Jackson was 3.5 years old and was having a lot problems walking and fatigue. With the way he was growing we need we didn't have much longer in any regular strollers. We talked to his doctors and they all agreed he would need one. We first got the referral to see the right physical therapist. She wanted to have several visits with him to see what his needs,strengths and weaknesses where. After a few months of seeing her she sent her recommendation to the seating clinic and we got an appointment there. That was a couple months wait. During that appointment we went over everything and the different options there where. Jackson got to pick out the color of his chair and out of the 5 colors he picked blue. Jackson has 2 different insurance companies so we submitted to the primary first and they completely denied it(this is very typical, I've even heard of children that can't walk at all being denied wheelchairs, its sad). After waiting for them to say it was denied we then submitted to secondary. We heard back from them and they approved it but we had to a good amount of out of pocket expense. After the insurance came through and we paid our portion they where able to order the parts and have it made. After a year of requesting his chair they called on monday to say it was in and we could pick it up this week. I was thrilled!

For those wondering why Jackson needs a wheelchair I'll explain a little. Jackson has low muscle tone and its much harder for him to do things requiring his muscles. He also has a lot of fatigue and very quickly wears out. He can walk and some days more then others. On days he's weaker he can't walk farther 20 yards before falling and needing to be carried. He also has other issues like a lot of pain and other various medical problems that having the chair can help. Now at almost 5 there is no way he can fit in a regular stroller at all and wouldn't support him very well in areas where is muscle tone is affected more. Since he has a mitochondrial disease,which is a progressive disease, he will continue to have problems with increasing fatigue and muscle weakness. As he gets older he maybe able to walk more but his tone and fatigue will not improve and most likely get worse. This chair has a lot of growth room in it and hoping it will last him about 5 years. That will be about when he's 8 or 9 that we would have to start looking where he's at then and seeing if he will need a bigger one or not.

This chair will be such a great help to us and him. Since he can't walk very far he gets carried a lot. He's a solid built boy weighing in at 48 lbs. Thats a lot to be carrying around every where. Also when we go anywhere like stores and such he has to be in the cart. Its difficult for him because its very uncomfortable for him and does not support him at all. He's really big to riding in the grocery cart. Plus its hard for me to lift him in and out of it. Then with 2 kids in the cart there's no room for groceries and such. Jackson will be able to do a lot more, go a lot more places, and will be a lot better with less fatigue and pain. He'll also be able to take this to school and hopefully he'll do better and last longer at school. He'll feel a lot better being in something that is supportive of him properly.

I only have one concern now that we have this. Any time we go out Jackson will need to be in his chair. I'll have to other children as well. The new baby I'll most likely carry in a wrap, sling or baby carrier. Then I'll have Noah who's 2, very active, and likes to run around. I'm not sure what to do with him. I fear if he's walking next to us that he'll run off and I'll have a hard time catching him running with a baby and pushing a wheelchair. As he gets older this won't be as much of an issue because he'll understand more. But at 2 I can't expect him to never walk off. Hopefully we'll figure out something.

Jackson's chair from the side with his tray on.

Jackson in his chair with the shade on it

Mito

Jackson's been having a lot of health issues lately and we've been seeing some new doctors as well. I wanted to update family and friends on how he's doing but it's difficult when most don't understand whats going on. Also some people have been asking questions about what exactly is going on with Jackson and why things are the way they are, so I wanted to explain a little bit about what it is Jackson is dealing with.

Ever since Jackson was born he's had a variety of symptoms, seen countless doctors, had lots of tests and procedures. His current main diagnosis is mitochondrial disease. Mito is a difficult thing to diagnose and, for many people, requires many tests over long period of time to find out for certain if they have it. A lot of doctors say that it's "as clear as mud" in trying to figure things out. For Jackson we are still in the process of finding the exact cause, so he has a "clinical" mito diagnosis. We haven't found the exact genetic mutation, but the doctors feel Jackson meets the clinical picture, and many tests point to mito being what he has. Because of the nature of this disease we are treating him as if we know for sure he has it.

I wanted to explain a little bit about what mitochondrial disease is.

What is Mitochondrial Disease
Mitochondrial diseases result from failures of the mitochondria, specialized compartments present in every cell of the body except red blood cells. Mitochondria are responsible for creating more than 90% of the energy needed by the body to sustain life and support growth. When they fail, less and less energy is generated within the cell. Cell injury and even cell death follow. If this process is repeated throughout the body, whole systems begin to fail, and the life of the person in whom this is happening is severely compromised. The disease primarily affects children, but adult onset is becoming more and more common.

Diseases of the mitochondria appear to cause the most damage to cells of the brain, heart, liver, skeletal muscles, kidney and the endocrine and respiratory systems.

Depending on which cells are affected, symptoms may include loss of motor control, muscle weakness and pain, gastro-intestinal disorders and swallowing difficulties, poor growth, cardiac disease, liver disease, diabetes, respiratory complications, seizures, visual/hearing problems, lactic acidosis, developmental delays and susceptibility to infection

Prognosis

As more research dollars are raised to find more effective treatments and ultimately a cure, some of the affected children and adults are living fairly normal lives with mitochondrial disease. At the opposite end of the spectrum, many are severely affected, and some children do not live past their teenage years.

When are they at greatest risk?

The child or adult is at risk for neurological or organ damage during and for the two weeks following an illness. Therefore even a simple flu or cold virus can have devastating effects on the patient, even death. Any illness must be treated immediately with medical interventions, like IV fluids and IV antibiotics.

You can find out more about mito on the website umdf.org

There is no cure for mitochondrial disease. The only treatments are treating symptoms that can be managed and taking the "mito cocktail". The cocktail is group of supplements that help the body improve function and may slow the progression of the disease. Jackson has recently started taking some of the supplements and we've some great improvements.

Some of the symptoms that Jackson deals with that are common in mito:
Developmental delays, autistic features, dysautonomia(temperature,blood pressure and blood sugars mostly), seizures, Weakness, cramping, dysmotility, hypotonia, muscle pain, diarrhea/constipation, fatigue, cyclic vomiting syndrome and other various symptoms.

Jackson is getting a stroller/wheelchair very soon. This is very common for children that have mito. It's mostly because of how quickly he fatigues and gets weak. Its also for his pain. Jackson's fatigue varies day to day. Some days he's able to walk and play then there's days that he needs help walking more then 10 yards. The stroller will allow him to do more on the days he's to weak to walk and also on the days that aren't too bad preserve his energy for more important things.