Parents Fight To Save Their Sons - Valley News Live - KVLY/KXJB - Fargo/Grand Forks

Parents Fight To Save Their Sons

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The Kulsruds look like your typical family, but parents Jay and Laura are fighting for their sons' lives. The three boys are fighting a very rare disease that will eventually kill them, unless the Federal Drug Administration quickly approves a drug that could potentially cure them. Valley News Team's Ashley Bishop traveled to Grace City, North Dakota to visit with the family about their plan of action.

Lane, Tanner and Ty Kulsrud suffer from a very rare genetic disease called Pantothenate Kinase-Associated Neurodegeneration, also known as PKAN. It's a disorder that causes the human body to block absorption of vitamin b5 which can cause a build up of iron on the brain.

"Because of the iron accumulating on the brain, it then causes neurological issues such as falling and speech issues," Laura Kulsruds, the boys' mother, said.

Their eldest son suffers from PKAN. In addition, his body spasms and makes it hard to do simple tasks. Laura and Jay are both carriers of the disease but did not know it. PKAN is a late onset disease and, as it progresses, it gets worse, eventually leading to an early death.  Two years ago, the Kulsrud founds out their now 13 year old son Lane has PKAN. They tested the two younger boys, Tanner and Ty, after Lane's diagnosis. Both tested positive.

"The chance of one having it is very rare and to find out all your sons have it is extremely rare," Laura Kulsrud said.

Lane and Tanner have shown symptoms of the disease. Tanner suffers with issues in his arms and has trouble writing.

"The biggest things that are a challenge for him are getting dressed, tying shoes putting a jacket on, eating, anything where his has to use his arms. It is hard. Where as Lane, his struggles are in his walking. So, his body has distorted from the dystonia," Laura said. 

Ty has not shown any symptoms yet, but is getting to the age where the disease begins to show. Laura says the boys don't normally complain and she tries to give them a normal childhood but some things they can't do.

"I can't do all the sports things that I want to do, like play football, basketball and baseball," Tanner Kulsrud said.
The boys do have daily tasks to help slow the disease. They use a machine called the "vibe". It uses weights on the body to vibrate and create different nerve paths, which helps with the spasms. The boys also use an elliptical bike.

"I just want to be a normal boy and grow up to be a normal boy," Lane Kulsrud said.

The Kulsruds were devastated and looked to friends and faith, but they were also determined to find a cure. 

"We were on a mission to find something to cure these boys cause how can you lose all of your kids? So we started searching," Laura said.

Laura and Jay scoured the internet for a cure. In the fall of 2013, they stumbled upon a drug company called "Retrophin".

"They had an article they just posted weeks before they completed a study about a drug called REO-24," she explained.

The drug was tested on an array of animals. Results showed the animals were cured after ingesting the drug.  

"I called our doctors both of them right away and I was thinking this is awesome, they couldn't believe it," she said.

The Kulsrud's doctors submitted an Emergency Physician Investigational New Drug Application to the Federal Drug Administration, which would allow the doctor to get the drug and try it on the boys.

"Within 24 hours the FDA denied that saying that the boys were not on the verge of death. Thankfully not yet. But it is a critical disease because each day we wait there are more things damaged in their bodies," Laura said.

The doctors applied again, but this time, the FDA said the risk to benefit ratio is too high.

"In our eyes the risk to benefit ratio is life or death. Without the drug there is nothing and they will die. With the drug there is a chance to be cured," she said.

Laura and Jay both said they would test the drug before the boys took it to prove it would not kill them but the FDA denied that. Laura has visited the FDA  and asked them why they are withholding a potential cure for their sons.

"They are so used to researching things to death, they research and research," she said.

With rare diseases, Laura says, they don't have time to study for years.

"Especially when the toxicology comes back okay. That is our main concern- that the drug will not kill them and I don't think there should be any other reason when your doctor, the drug company, your parents and even the boys feel they need this drug," Laura said.

"Give us the medicine right now we really really need it," Lane pleaded.
"Give me the medicine like right now...i would like it," Tanner added.

Last week in Europe REO-24 was approved. The Kulsruds are going to try get the drug here but if they can't in a few months they will travel to Europe to get it. FDA approval would make the the back-and-forth European appointments unnecessary. Laura has reached out to our congressional delegation and has been told they are working on it. We are also in touch with our congressman and senators and will continue to follow this story and let you know what happens.

 Currently in North Dakota the Kulsruds are the only family with this rare disease.

If you want to know more about the family, check out their Facebook page. Or if you would like to call the FDA, the number is below.

FDA: (1-888-463-6332)
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