Adam and Mary on Fox 13 news at the Primary Children's Hospital and Huntsman Cancer Center Childhood Cancer Fundraiser walk in Sugarhouse Park.

Anthony got to go on the stage and he got a medal.  Adam said Anthony was the
ONLY bald child there, so Adam thinks the other cancer patients on chemo therapy
right now didn't go.

You can see the back of  Adam carrying Noah, just before the break where they
talk.  Adam is wearing orange and blue sweat pants with a white t-shirt.

Mary and Anthony are in the crowd in FRONT of Adam and Noah.  I can't find
them in this video.

Fox 13 Now news

I just found this email from Uncle Guy. He sent it to my Hotmail on September 8, 2014

(Daddy, Mark, hasn't been tested for this recently, last test 15 years ago)

Guy

Guy Burdett (guyburdett@gmail.com) Add to contacts 9/08/14

To: peggy burdett

Hi Peggy

I just found out I have hereditary hemochromatosis. I am sure you guys would know by now if Mark had it. My ferritins levels were 1036 which is pretty high I will be giving blood every week for the next year. 

Hope you all are doing well  

--

Guy Burdett

801-874-6194

mcmwrestling.blogspot.com

mcmwrestling.com

Heavy rains flood Phoenix--Note- they didn't cancel classes until AFTER Jacob took his test at 6:30 a.m. the day of the floods!

                                The top 3 photos are from Bing images in Phoenix and the bottom
                               photo is also from Bing, but in Nevada.

PHOENIX (AP) — The remnants of Hurricane Norbert pushed into the desert Southwest and swamped Phoenix with record rainfall for a single day, turning freeways into small lakes and sending rescuers scrambling to get drivers out of inundated cars.

At least two people died in the flooding, including a woman who was swept away in her car by rushing water and became trapped against a bridge. In addition, a 76-year-old woman drowned in floodwaters.

By Monday evening, floodwaters were threatening up to 100 homes in suburban Mesa after retention basins and channels along the U.S. 60 freeway reached or exceeded capacity, allowing water to flow into some neighborhoods, city officials said.

Crews were working to disconnect power to submerged transformers, provide sandbags to threatened homes, and pump water from affected areas. A temporary shelter was being set up at a recreation center.

The flooding was caused by heavy thunderstorms and showers associated with Norbert after it was downgraded to a tropical depression.

Sections of the two main east-west and north-south freeways through Phoenix — Interstates 10 and 17 — were closed during the morning commute, snarling traffic across the metro area.

Cars and SUVs sat in water up to their hoods on Interstate 10, while dozens of motorists parked on its wide, banked borders to stay clear of the water. A state Department of Public Safety officer used the roof of his SUV to carry three stranded motorists from a flooded area of I-10.

Joseph Friend was driving onto the freeway at 43rd Avenue about 4:15 a.m. when a passing big rig ruined his day.

"A big tidal wave just came up and totally took me out, came over the hood of my truck," Friend said.

With water filling his vehicle, he climbed out and walked up the freeway embankment to wait it out. His pickup truck was barely visible at the peak of the flooding.

Other drivers were stranded in the median. After the highway was shut down, a woman on top of her car in the median called for help, so Friend waded out and led her to safety.

"She was asking for help and nobody went out there, so I went out there and helped her out," Friend said. "I was already soaked anyway."

By late morning, the water on I-10 had receded, allowing trucks to take away several dozen vehicles that had been swamped and stranded.

The National Weather Service recorded 3.29 inches of rain at the Phoenix airport, by far the most precipitation ever received in one day in the city. The previous record was 2.91 inches in 1939.

MSN news

Marilee doesn't know whether her mother or father carried the recessive Gene!

Rachel Burdett, Dan's daughter, ask Marilee which of her parents carried the gene on Facebook.
I have posted Marilee's answer first, Rachel's question next.  I can't believe they haven't
thought of this.  How many of Guy's four children carry the gene?  I think Guy has more
grandchildren than we do (8) and Amy is pregnant expecting in February.

Rachel is probably thinking the same question Adam asked, if the gene is in the Burdett
line. . . . . . .If the gene came from Laurie, then hopefully it isn't in the Burdett line, only
the Litchfield line.  (Litchfield's, Laurie's parents had over 10 children so it probably
would have shown up in their grand children by now due to the Large number)

Marilee Burdett Leishman Oh and Rachel Burdett I am not sure if I got it from my Mother or Father, good question! That would be interesting to find out.

Rachel Burdett Thank you so much for sharing your knowledge and experience. I bet it helps you through your process to write down what you are going through and it also helps others learn about this, which is important My heart goes out to your family during these hard times, and always. Such a big hug and so much love goes out your way  Do you know if your mom or dad is the carrier of the disease?

Marilee Burdett Leishman's son has GM type 2 Gangliosidoses

Jacob looked this up in his medical books.  It is a genetically inherited disease.

How is GM1 gangliosidosis inherited?

GM1 gangliosidosis is inherited in an autosomal recessive manner.[1][2] Affected individuals inherit 2 mutated copies of the disease-causing gene, one from each parent. Carrier parents (with 1 normal copy and 1 mutated copy) typically are unaffected and do not have any signs or symptoms of the condition. When 2 carriers of an autosomal recessive condition have children, each child has a 25% (1 in 4) risk to have the condition, a 50% (1 in 2) risk to be a carrier like each of the parents, and a 25% risk to not have the condition and not be a carrier.

It is important to note that GM1 gangliosidosis is type-specific within families. This means that individuals with a family history of the condition are generally only at increased risk for the specific type of GM1 gangliosidosis in the family.[2     
NIH

This is Marilee's Facebook post:

Hi - I just found out a few days ago that my 5 year old son has Gm1 type two. We thought he had some sort of rare genetic disorder but didn't know what. I am trying to take it all in. Eli was pretty normal until about age 2, except for slight wobbliness and speech delay. Then at age 2 his eyes began to cross and since then he has lost much of his large and fine motor skills, developed kyphosis of the spine and had 3 eye surgeries. He did lose most of the little amount of speech skills he had. He can walk slowly, but tires very quickly, so he now has a walker, which he likes, but he gets frustrated with it, because he gets stuck all the time. And because he falls easily, he wears a helmet to protect his head. He also didn't grow height wise at all from age 4-5. He has thinned out a lot over the past two years, but he still seems to be hungry all the time. He loves food and wants to eat often, and doesn't really know when he's full. Does anyone else have similar experiences? . . . .

. . . . I did notice on here that a ketogenic diet was mentioned. Is that to help with seizures or for the disease? Just wondering if I need to have my son on a special diet. I am overwhelmed by it all....

I have a daughter who is 7 and fine and another boy who has just turned 2. We think he may have it as well, we will hopefully get him tested and know for sure within a few months. The doctor is pretty sure he has it, but I am not ready to deal with that until I know for sure. I have contacted MN to see if we can get HOA testing done. I don't know much about all of that, I know it must be a big ordeal.

Like

The birth of a child with a recessive condition is often a total surprise to a family, since in most cases, there is no previous family history of the condition. Many autosomal recessive conditions occur this way. It is estimated that all people carry about 20 recessive genes that cause genetic diseases or conditions. It is only when a person has a child with a partner that carries the same recessive gene mutation, that there is a chance of having a child with a recessive disorder.

GM1 gangliosidosis is one of over 50 different lysosomal storage diseases.  It is characterized by the lack of the enzyme, beta-galactisodase.. . . . .  The build up of the GM1 lipid disrupts the normal cell function and causes them to self-destruct; which in turn, leads to the degeneration of the central nervous system and ultimately, death of the affected person.

  Type 2 - Juvenile onset:  this sub-type is characterized by a later onset and typically presents between 1-3 years of age. The children affected have less coarse facial features, hepatosplenomegaly, and skeletal abnormalities.  Life expectancy varies, depending on onset, but many children with Juvenile GM1 gangliosidosis survive until teenage years.        http://gm1gangliosidosis.webs.com/aboutgm1.htm