Parker's Path - Juvenile Batten Disease

I haven’t done an update in so so long!! Thankfully that means life has been mostly uneventful!!

Tonight we are celebrating STABLE brain scans!! Woohoo!! This is the best news we can get!! Extremely mild if any progression 🙌🏻

Luckily our children were able to go to school for most of the school year, benefits of being in a small school!!

Parker graduated 8th grade and will be moving on to high school. We have been so lucky to have the staff at GGS be apart of Parker’s life. I am so grateful that he was able to walk across that stage!!

Batten Disease showed its ugly side today and reminded us just how fast things can change. Just when you think maybe this is it, maybe his transplant worked and my boy will stay stable and get to live a somewhat normal life, just when you get comfortable and in a new normal...Bam! Batten disease shows up and reminds you who is in charge!

Parker had his first ever seizure this morning. It was a call I hoped I would never get (he was with his Dad). We rushed to the hospital as he was taken by ambulance and boy did I feel helpless not being right there for him. He was pretty confused and had a hard time finding his words for a bit, but he is feeling much better now.

We are currently at Lurie's to monitor him via EEG. He had some dinner and said he was feeling lots better after that. Started a movie for him and he is now sleeping very hard.

Please keep Sadie in your thoughts as well as she witnessed the whole thing, it broke my heart when she told me what happened to her Bubby. I have never questioned why I was chosen as her mother. She is the most compassionate and calm child I have ever seen in these types of situations. She is Parker's saving grace...she always knows just how to calm him or make him feel better. She is going to move mountains one day. She just makes life better!!

Thank you to everyone that was so caring today from hospital staff(Morris & Luries) to both sets of ambulance staff, and everyone who has called or texted.

For now hopefully he gets a good night of rest and we will meet with Dr's tomorrow.

It has been a very long time since I have updated on this page!! Which just means there is really nothing to report, Yay!!

Today Parker is celebrating his 12th birthday, he took treats to school, he requested tacos for dinner, and then ditched me for a sleepover with his cousin!

Parker started 6th grade this year and is loving junior high. I had conferences tonight and everyone said he is doing a great job and that he has really adjusted to all obstacles this year. We have had some aide changes and he just keeps going with the flow, which makes me so happy. If you know Parker he can tend to like things his way, but they rarely see that side of him at school. He really gives everything he has at school, and it makes me so proud!

Parker returned to Duke in August for his 3 year post transplant studies and everything is continuing to stay stable!! Best words we can hear!! He still needs to have an EEG to complete the studies but we will have that completed near home.

Overall Parker is doing incredible!!

Parker and Dr Prasad, one of our favorite people ever!! Parker's 2 year check up couldn't have been better! Dr Prasad could not have been happier with how Parker looked, he said you would never know he had a stem cell transplant 2 years ago! It can take some people up to 5 years to fully recover!! Parker has had ZERO progression in his disease for 2 years!! His MRI of the brain is stable, and stable is just what we want. This means his MRI he recently had shows no change compared to the one he had before transplant. All other tests looked great also. We are in the process of changing neurologists at home as ours (Which we loved) has moved onto something new. We have an appointment coming up with the new neurologist in July and then will return for an overnight EEG to make sure we are still seizure free. Hoping to see stable results on this test also.

With that being said, Dr Prasad feels we should put most of our focus on Parker' s visual needs since that is his most pressing medical issue. He feels we should find the best of the best neuroopthalmologists out of Chicago and get working on a way to find him an option to improve the small amount of vision he has left in his right eye. He said he may end up a good candidate for some up and coming eye technology! This would be such a blessing for us all!!

Thank you to everyone for your continued support of our family! It has been a crazy journey and one we couldn't have made it through without you all!!

Parker had a morning full of tests that he did well with. We went and fed the ducks and took a walk around Duke Gardens. Overall it has been a nice day and Parker is looking forward to seeing Dr Prasad tomorrow!!

Parker and I have made it to Durham for his 2 year transplant follow up!! It didn't take long for Parker to get comfy at the Ronald McDonald House!! He had an MRI early this morning and is done with tests for the day!

I haven't given an update on Parker for quite some time, which is a good thing because it simply means there is not much to report!!

Parker has been doing great from a transplant point of view. We haven't had any concerns and haven't needed a doctor visit since our last trip to Duke in September. He has a few minor daily struggles since transplant which include some skin irritation in certain areas, he still struggles with endurance and tires quickly (which could also be a Batten issue), and his gut still isn't quite the same since receiving chemo.

Batten Disease, ugh I hate this disease! While Parker has remained what would be considered stable, Batten Disease is still very much affecting him. He seems to be giving his all at school, and is continuing to make educational gains, which is wonderful! He is working hard with his white cane and becoming more comfortable with it. He has recently started bringing it home from school and has consistently used it anytime we have left the house without me even asking him to use it. This is huge because he has never been a fan of his cane!! So besides being blind what other things has Batten Disease affected... Parker does have some mild dementia. For example he will ask me 3 times in 15 minutes what we are having for dinner. I will ask him if he can try to remember what I just told him, but most of the time he simply just cannot remember. He also has some behavioral issues, which have been better than they were before transplant, but they are definitely still a challenge. Anything can send him into a meltdown. It is very much his way or no way, which is typical for a juvenile Batten Disease child. His behaviors are also not age appropriate, there are days I would swear I am dealing with a 5 year old. Seizures...oh the dreaded seizures! Well I honestly don't know if Parker is having them. His neurologist suspects he is having them in his sleep, which I believe is true. I am pretty confident I witnessed him have an absence seizure a few weeks ago when he was woken up from sleeping. So far we have been lucky enough to avoid being on seizure meds which makes me very happy. Then the lack of endurance, his legs get sore very quickly with any type of activity. I actually had the wheelchair talk with his palliative care nurse the other day. Not for every day use but for events that would require more than 45 minutes to an hour of walking. Once his legs are tired it is constant complaining and honestly can ruin everyone else's day because he can get so upset over it. So I am going to test it out, there will be rules with it like he can't use it until he has been walking for at least an hour because let's be honest Parker can be lazy! Hoping it will make outings a little less stressful for everyone!

Overall Parker is doing well, he enjoys going to school and being around his classmates. He is becoming a little more aware of his memory issues, which helps him not get so frustrated when he can't remember. I will be honest Batten Disease is exhausting even with his minor issues, but I am beyond thankful we haven't seen too much progression since transplant!

Thank you to everyone for your continued support!

HAPPY 10th BIRTHDAY to my favorite boy!!! What a difference a year makes, especially after transplant! I added a photo of last years birthday as a comparison, it is amazing how far he has come since then! Parker had a wonderful pool party with classmates and family, he had a ton of fun!! He is celebrating at school today with donuts and juice boxes. Parker is doing wonderful! He is thriving at school and loves being there! He has been able to keep up in all regular education classes (except for math) with the help of his aide. We are all so amazed at how well he has done at school, I am so proud of him!! Take that Batten Disease! We will fight you with all our might!!

Parker had his 18 month check up at Duke (a month early because I goofed!)!! We flew in yesterday morning and will be flying back in a few hours!!

Dr Prasad was so happy with everything and just couldn't believe how well he is doing. All of his tests came back great and his labs were the best they had ever been!! He said Parker is 100% recovered from transplant which is a huge deal for us! Now we just have to monitor him neurologically over the next 3 1/2 years to determine if they feel the transplant has worked to either slow or stop progression of the disease.

So where does this leave us...we just wait!! Wait to see if anything comes up whether it be seizures, decline in mobility...I am constantly on alert but really hopeful that we will never see any of these symptoms.

Where does he stand medically in terms of Batten disease...
At this time Parker has lost almost all of his vision, he has a small amount remaining in his right eye. He goes through periods of sleep problems and periods of emotional outbursts. He also has a hard time remembering certain things, so that is a sign of some mild dementia. These were all present before transplant and the only thing that has worsened considerably is his vision. The emotional outbursts have actually improved.

Thanks for all of the continued support!!

Parker's Make A Wish is complete!! He is very very happy with it and has played most of the evening on it!! Thank you to Make A Wish and Pirate Playships for this amazing wish!! Parker has already asked me to buy pirate hats, swords....so him and his friends can play on it!! According to him the swords we have are knight swords not pirate swords, so those just won't work, haha!! He is something else!

It will be done tomorrow morning but Parker checked it out a little tonight! Pirate ship is almost done, still needs slide, swings, and a few little things. He is so so excited!! Thank you Make A Wish!!