Wednesday, February 14, 2018

Grumpy Valentine...

On Monday, Max made it through his entire school session.  This was the first time since around October that he was able to sit through all of school.  He had an idea of what he wanted to do and got to work on it!

He wanted to make Valentines!  He had in mind what colors he wanted, who he wanted to make them for, and what he wanted them to say.

His first heart was for Daddy.  He wanted to draw the heart, and then cut it out with his special cutter.  He wanted it to be a red heart on yellow paper.  He drew the first eye, them the second.  A little nose and then a frown. 

I asked him why his heart was sad.  He said it was funny.


But he seemed a little unsure of how his joke was going to play.  He asked "you think it's awesome it's funny" "after he saw a would idea bad joke" He was afraid Daddy wouldn't think it was a funny joke to have a frowning heart.  Mary and I made sure he knew it was HILARIOUS!



He said, " Not too many people like funny valentine."


He wanted to sign his Valentine, and let us in on the joke he was making up.  This was a Grumpy Valentine.


He said he wanted, "Grumpy Valentine Dad I love you."

After he wrote his card out to his dad, he said "color my black h-a-r-t." He was on a roll with the jokes...saying Daddy had a black heart, something Steve likes to joke about...he likes his coffee like his soul/heart. 


Next, Max wanted to make a Grumpy Valentine for his aunt.  He chose a black background and wanted three hearts for hers. Who knows at what point he started thinking it was funny to say Auntie Angie stinks, but this is how he came up with telling her she stinks. 




 Now he was on a roll with the jokes and was cracking himself up!!


This is an example of how he made all his hearts.  He wanted them to be "curly" and cut them out curly too.

This is Adeline's Valentine.  He came up with a great pun for her Valentine.  He said "Quack Duck", then said "Adeline, you crack me up." I asked him was he being silly and said "you quack me up?" and he said Yes!

 These were all he could finish in school.  When it was time for Speech Therapy, he told Robby he would watch a video with him, but "first Grumpy Valentine Uncle Dan."  He had one more to crank out before he watched a video with Robby.

 Uncle Dan's has glasses, like he and Uncle Dan wear.  He said this one needed to be purple and green and it was "most awful grumpy valentine".
His special message for Uncle Dan was to tell him to be quiet.  We've always had a suspicion he was being a bit cheeky when he would tell us to be quiet. 


Today, he was pretty low energy.  He was awake all night, but was determined to go to Denver to take his Valentine's to them.  He even came up with the sweet treat he wanted to get Addie - Booger Jelly Beans!




The picture is blurry and not the best, but we were doing our meet up in the van before they had to run and we had to hurry home for Max's nurse. He got a couple of special Valentines from them, and he got to give his Valentines to both of them too. 

It was so interesting watching him be able to get this idea out.  It was also interesting to watch him be unsure of how it was going to come across.  We've never seen him reach this way in trying to see if what he was saying was appropriate, or if it was coming across as funny like he meant it to be.  It was so cool to see him considering it all, and work so hard at getting his thoughts out.

He has had a really good week...just a little tired as he continues to come down from the steroid.  He really liked being the "Grumpy Valentine" this year.  It was a title he let us call him all week.  He's still my sweet, thoughtful boy...even if he's grumpy.

Tuesday, February 13, 2018

Week 5 and a roadblock...

We're onto week six of treatment for Max's acne fulminans. His skin has been looking better, but he's had a few areas flare up once he started to go down on the Prednisone dose 10 days ago.  




Last week the Prednisone wean was only a little difficult.  He had a hard time staying asleep, and was awake a lot during the nights.  He had a hard time calming down if he started getting fussy too. 

We ran into a roadblock at the end of last week in regards to the Isotretinoin (Accutane) treatment.  Accutane is no longer sold under that name, instead, all eight generic forms of the medicine are made by just seven manufacturers in the country for the ipledge program.  This is a risk management program to keep track of everyone on Isotretinoin.  We found out last week that the medicine is all manufactured to be in gelatin capsules.  Max is VERY allergic to gelatin.  We now have Allergy involved, along with Dermatology, Rheumatology, Gastroenterology, Endocrinology, Metabolics, and his PCP all working together trying to find a solution to this latest problem.
We took him in on Sunday to have an X-ray of his spine and pelvis to check for arthritis or bone inflammation - which can be caused by the acne fulminans. The X-rays do not show any signs of arthritis or bone inflammation. X-rays are not very sensitive at picking up these findings (although if the inflammation is longstanding they can see chronic changes sometimes) which is why we will be getting the MRI in the Spring.  The good news, of course is that he's not showing signs of arthritis or bone swelling.  This means we have likely avoided the long term arthritis treatment for now.  

In order to continue the Prednisone wean, until we have a solution to the Isotretinoin solution, Max has been put on a combination of Doxycycline and Adapalene and Benzoyl Peroxide Gel.  These are just temporary fixes...and won't cure the acne fulminans.  But we have to do something to treat the acne while coming off of the steroid so he doesn't have a huge flare. 

I've had several friends ask if we would consider medical cbd oil for Max to get through this, and yes - we would and have considered it especially since we have run into a few roadblocks with traditional treatment. However, no one is currently in agreement about how to go about it, as Max's neurologist of the past 15 years has moved to another state. This has left Max without a neurologist who knows anything about him. If we decide to go that route, we'll document it as well. But for now we're doing everything we know to do to help him.

Yesterday I was looking for the phone number to one of the clinic nurses who has helped us in the past.  I pulled out Max's notebooks to find it.  It was in the last book I looked in.  I saw my scribbles on the first day he was diagnosed, February 20, 2004.  I saw pages and pages and pages of seizure logs.  I saw notes from all of his surgeries. I saw notes on metabolic, neurology, eye, ENT, cardiology, orthopedics, urology, dental, endocrinology, nutrition, GI, and pediatrician appointments.  I have diagrams drawn by his doctors and notes from nurses written in his books. 





I looked through every page of those books and saw a history of Max's life. With all of the very rare diseases Max has had, I never thought ACNE would be the one to stump us all the most.   I was able to see in his notebooks through the years, we have found a solution to every problem so far, and got we will find a way with this too. 


Tuesday, February 6, 2018

Follow-Up with Dermatology


At the end of week 4 on prednisone, we had a follow-up appointment with Max's dermatologist.  We discussed starting the wean off of prednisone, but it will be slow. I think the end of it will be in mid-March.  

He's been handling the wean fairly well so far.  He has had some moments of rage and definitely having interrupted sleep, but those were also happening before we started the prednisone.  It's just now when he wakes in the middle of the night there's no chance of him getting back to sleep, and when a mood swing starts it always ends with him safely in his bed to kick it out. He told me in speech therapy yesterday that he likes to be awake at night because "best things happen at night. it's fun."  Oh boy.  

His skin continues to clear up with his face and chest looking the best.  His back is still pretty irritated, but he's always laying on his back in his chair.  At night he will let us lay him on his side, so he has some relief then. 

He's been on the naproxen for two weeks now and he is not complaining of the pain nearly as much.  On very cold days we will notice he's a lot tighter and guards his arms close to his body rather than moving them around. 

We got a long list of creams, lotions, sunscreens, eye drops, and body wash from dermatology.  The Accutane will cause his oil glands to shrink, so there is drying that will happen.  He's been really good about letting me put vaseline on his lips...I will report back on how putting thick cream all over him goes.  He's always been less than helpful when it comes to putting creams and lotions on.

After the dermatology appointment, we had to get a baseline blood draw to see what Max's metabolic levels are before starting the new medicine.  It's something they will watch since it's not a medicine that has been used before in his specific type of metabolic disease.  Forever the frontrunner, this one.

We are waiting to hear back from dermatology on when we will be starting the isotretinoin since the dose his dermatologist wants to start him at only comes in gel caps. When she said that we were both quick to point out that "gel" stands for gelatin -- which Max is extremely allergic to.  So, they're talking with the manufacturer to see if it's animal gelatin or vegetable gelatin.  It it's animal, we go to plan B...which is yet to be determined.

With the prospect of having to put cream on Max's scalp, I talked him into letting me give him a haircut.  He hasn't had one since around his birthday and he was starting to look a little shaggy!  But he had so many acne spots on his scalp that cutting his hair was out of the question.  The spots on his scalp are nearly gone so I had a go at it last night. 


I do love seeing those bright blue eyes of his! This makes it easier to see the spots and make sure it's all healing up well too.

The back of his head has quite a few scars now.  They'll get covered when his hair grows in a bit, and he's no stranger to scars.  I know it could have been much worse...just google images of acne fulminans...but I still hate to see how much of his head was covered.




I was running my hand over his big guy head this morning, fooling myself that he would actually go back to sleep, and remembered doing the same when he was a tiny baby.  Always stroking his hair, holding his head close to me.  It reminded me of this moment.  He was just 3 months old here.  His first trip to the ocean, in California.  We learned on that trip that he was having seizures.  I would hold him and stroke his fluffy ducky hair.

January 2004

He's always going to be my perfect little baby...scars and all. I'm hoping the steroid wean continues to be uneventful and we can start the acne medicine soon.