Ryker is a happy, loving, and sweet seven year old boy. He resides in Lincoln, Nebraska with his mom, Amy, his dad, Ross, and his sister, Mila (5). Ryker loves to listen to music, loves being around his peers, and loves swinging. Ryker has been on a challenging journey, but continues to find joy in everything around him. His story serves as a strong reminder to enjoy the moments you are given.
Amy had an uneventful pregnancy and delivery with Ryker. He thrived as a baby and grew into a healthy toddler. On Ryker’s fourth birthday the daycare he was attending completed a routine evaluation on him and reported to Amy that Ryker was unable to report his numbers and colors. These were facts that Ryker had known and should have been able to recite. Since it was a new daycare for Ryker, Amy and Ross thought maybe the transition was hard on him and caused him to forget the basics. Soon, Ryker started to have potty accidents at daycare even though he had been potty trained for about eight months at that point. Ryker also started to have issues with hitting at daycare, which was “so far beyond his personality. Ryker did not hit. That’s when I started to question,” Amy shares.
The daycare suggested that Amy and Ross reach out to the public schools for an evaluation since the schools could help place him in services if qualified. After the evaluation, Ryker was recommended for services and they began to work with him once a week. “Within a month they reported that they had documented loss of skills,” Amy explains. They encouraged Amy and Ross to take Ryker to the pediatrician. “It had been about four months since the pediatrician saw him and it was really eye opening for her. She said it was a completely different kid than she saw four months ago,” Amy says. The pediatrician put in a referral for them to see a pediatric neurologist and an early childhood development specialist. They were placed on the waiting list to see these specialists and it was months before they could be seen. While they were waiting to be seen, Ryker continued to lose abilities. He soon was unable to speak in complete sentences. Since they were unable to get into the specialist for quite some time the pediatrician ordered her own tests. An EEG came back normal and an MRI showed some fluid on the cerebellum. The radiologist tech that completed the MRI diagnosed Ryker with Dandy Walker syndrome, which is a congenital brain malformation involving the cerebellum and the fluid-filled spaces around it. They referred them right away to a pediatric neurosurgeon.
At the meeting with the neurosurgeon it was reported that Ryker did not have Dandy Walker syndrome and that the fluid that he saw should not be what was affecting his abilities. They were then referred to a metabolic specialist for more testing. They found that Ryker had very long chain fatty acids and that his body was not breaking down fatty acids the way it should. At this time, Amy was seeking out care on her own, doing anything she could to help her son since they were not getting into see the specialists they needed to see for some time. They began chiropractic care for Ryker and seeing a local holistic homeopathic doctor who was completing her own tests and providing them with remedies.
Ryker was eventually referred to a neuro-optometrist where they found that his retina was not functioning properly. They were not able to say for sure, but suspected that he could probably not see more than three feet in front of him and only able to make out silhouettes. This was the first time that they were aware that Ryker was losing his vision. “He was running into things and falling down the stairs because he couldn’t control his motor functions. We didn’t know at the time about his vision and all the other stuff. It was a hard time because we had to put his mattress on the floor. He was losing his neurological functioning. We couldn’t keep him in bed at night. He couldn’t control his movements. We didn’t want him to fall out of bed so he had a mattress on the floor, but then he would still get up and fall into the wall,” Amy shares. “We didn’t sleep. We always had to be listening to see if he got up.” Amy was connected with another mom through Ryker’s school when he started kindergarten. This woman’s daughter, who sadly passed away, had used a bed called an entrapment bed that had sides on it and was able to be raised and lowered. She offered it to Ryker, and “that changed our lives because we could all finally sleep again,” Amy says.
After the retinal scan they were able to get genetic testing covered by insurance. “Up until we got the diagnosis, you know you just think you’re going to find out what it is and you’re going to fix it,” Amy shares. They completed the genetic tests and it took seven weeks to receive the results. The doctor called and reported that the tests showed that Ryker had Batten disease and that there were no treatments or cures for it and it was fatal 100% of the time. Batten disease is a rare group of nervous system disorders called neuronal ceroid lipofuscinosis that get worse over time. The doctor reported that he did not know much about the disease and that Amy and Ross were probably going to find more information by finding other parents and children who have the same disease. The doctor was reading off a piece of paper when he was speaking to them over the phone and he actually told them the wrong type that Ryker had and said it was CLN2. There is actually an infusion you can get for CLN2 that was recently approved; it does not stop the disease, but it is able to slow the progression. Amy and Ross began looking into getting this done and other options for Ryker, but when she sent his lab results to the drug company that does the infusions they reported back that he did not have CLN2, but CLN1. Cases with CLN1 are not able to receive this infusion. “That was another gut punch because we thought there could at least be something we could be doing to help him, but there wasn’t,” Amy shares.
Since 2017, when Ryker was diagnosed, Amy and Ross are focusing on keeping up with his regression. In 2018 he received his first wheelchair and a walker. These items were wonderful to have for Ryker, but it was not even a year before he needed a different one because they no longer supported him and the needs he had due to his regression. The following year they got all the justifications and paperwork together for insurance to see, but it took eight months to get approved and receive the wheelchair that he needed. Insurance prefers for the families to have the wheelchair for at least five years before needing a new one. Insurance has denied equipment that is vital for Ryker and if it happens to be approved it takes far too long for them to receive. Ryker regresses so quickly that they need the equipment quickly or it doesn’t do any good.
Today Ryker is a joyful and loved little boy. His disease continues to progress, but he is still able to show love and happiness every day. Ryker’s school has been an incredible support to the family. Through the school he sees a physical therapist, an occupational therapist, and is provided with resource teachers. When he is in need of equipment that hasn’t been provided to him yet, they get creative and work to give him what he needs in order to be happy and thrive. “They made him a stander out of cardboard. It looks like a little rocket ship that he can stand in and stand up during the day for awhile. It’s super cool,” Amy explains. They’ve also been able to send some equipment home with Amy and Ross so that Ryker can be comfortable at home. “All the good in the world has definitely been brought to the forefront for us,” Amy shares when speaking of the community around her and how much they’ve shown up for their family and now it’s time for us to show up with them.
Time to get loud shouters!
Throughout the month of November we will be shouting love for Ryker and one other Lincoln family. It is a great honor to be able to tell Ryker’s story and we hope you will join us in getting to know them.