Jack & Kenz Ryan Jack & Kenz attack HLH!!

First post: Apr 30, 2019 Latest post: Sep 27, 2022
Bare with me... this will be wordy but I promise all my posts won’t be like this. I want to give Jack’s back story to those who don’t know it.

“Jack’s Crappy Adventure ” (his choice on the name, except he said Jack’s C word Adventure) began on January 3rd, 2019. He called me to pick him up from school, he was in the nurses office with a headache and dizziness. At this time Kenz was fighting a pretty bad virus so everyone thought Jack was fighting off the same thing. He didn’t have further complaints until the weekend of January 18th when he said he started having double vision. That Monday morning I was waiting to get in touch with his pediatrician when I got a call that Jack was in the nurses office again with a headache and dizziness. I picked him up and made an appt with his doctor for later that day. When walking into the appt I noticed Jack slightly dragging his feet. He said it was due to wearing winter boots all weekend at dad’s but I knew that was not common for Jack. He passed the neuro exam and eye exam. They told me to document any possible neurological concerns for the week and we could get an MRI if needed. That Wednesday, January 23rd, jack mentioned his fingers felt a little numb and tingly when he did his homework. The next day he said his leg felt a little stiff. Friday, January 25th, we came home from school and he was walking in front of me up the stairs. He swung his foot forward to step and began walking on the inside of his foot. I immediately knew he needed to go straight to the ED at Children’s.

We we got to CHW and he limped all the way to the door. The strong willed, stubborn kiddo refused to let me help him. We spent 10 hours in the ED before being admitted to W7. He had an MRI and lumber puncture done along with tons of labs. We were told that his MRI looked so bad that they would assume he would be intubated and paralyzed and they couldn’t believe he looked as great as he did. Jack had lesions in his cerebellum and spinal cord. Two large areas in his cervical spine and one in his thoracic spine. He also had double vision, weakness to his right sided extremities, trouble with fine motor to his right hand, and was unable to walk well. He would trip frequently and drag his feet.

Jack was started on high dose IV steroids for 5 days. He started responding almost immediately to the steroids and improved dramatically each day. His MRI on discharge looked better but not great. At this point we were hoping it was a post viral, one and done type thing but they were trying to rule out autoimmune as well. He went home on a month long oral steroid taper.

Jack started physical therapy and started showing some improvements slowly. He continued to have difficulty walking at times, especially when he had a long day. Two days before his taper was completed, we were at Children’s for an appt and he began tripping down the skywalk. The next morning he fell in the kitchen before school, began having increased weakness in his right hand and his left hand began feeling weak. Also, we noticed his pupils weren’t equal. We packed up and went to Children’s knowing we’d be admitted again.

Jack went back to W7, had another MRI, and another lumbar puncture (without sedation! He’s a rockstar!!) This MRI looked worse than the one on the first admission! I should mention that everything has been coming back negative so all we kept hearing is how atypical Jack is presenting and he’s a mystery. Again he gets the 5 days of high dose IV steroids and again he responds AMAZING! Drastic improvements each day. He has a liver and spleen ultrasound, chest x Ray, bone marrow aspirate and biopsy. Then, we go home on 30 mg prednisone and DO NOT wean this time.

He has been doing physical therapy 3 times a week, occupational therapy once a week and many follow up appts in between. His follow up MRI didn’t show much change but no new lesions which is great. This poor kiddo has had so many tests done, for the WEIRDEST STUFF! And all negative. The neurologist was stumped and even suggested we get an opinion from Boston Children’s.
Jack has been doing great, especially recently. He is moving around more quickly, jumping and some jogging in place during therapy. His fine motor is better too as he has been doing a great job cutting things and has strength to open bottles. His need for independence and his determination has always amazed me.

This past week, there was one last lab out there that came back positive. HLH... Jack has the familial version on the UNC13D gene. I will share a video from Cincinnati Children’s that explains it. Jack does not present like most kids with HLH. Many have very high fevers, multi system organ failure and are very sick. Also, it is typically triggered when they are much younger than Jack. We feel very fortunate that Jack is not sick like many of these children and he is older which can help him be stronger to fight through this. Jack presented with the CNS issues and a slightly low white count, platelets and H&H initially but his labs have since been in a normal range.

The plan: Jack is on dexamethasone, fluconazole and Bactrim and will need to stay as healthy as possible for the next 8 weeks. During this time the dexamethasone will help with any inflammation and they will also work on getting him a bone marrow match. For now, he has to go to children’s for weekly visits and lab draws. In 2 weeks he has another MRI and lumbar puncture. We should find out later this week if Kenzie is a match. Then, he will need 7-10 days of chemo prior to the transplant. If all goes well, he will spend approx 6 weeks in the hospital.
They do not want him in school at this point because he needs to stay healthy.

We have a long road ahead of us and we are so thankful to have the love and support of all of you. I am overwhelmed by all the love we already feel. You are all amazing! ❤️

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