Monday, December 24, 2018

Skin update...

It has been almost a year since Max was diagnosed with acne fulminans.  Last Christmas he was so miserably sick - but we didn't know what was making him so sick.  At the beginning of January this year we learned it was another rare disease called acne fulminans.  It's an auto-inflammatory disease which causes systemic acne.  This means his whole body was fighting against the large painful cysts forming all over the outside of his body because of the inflammation that was happening inside his body.

You can read about the initial diagnosis here:

Since January we have been trying to find the right treatment for Max.  He was started on high doses of prednisone, which immediately started to help the inflammation and made the daily high fevers go away.  We started into the process of getting the known treatment on board for him - isotretinoin - but hit a roadblock when we realized the only form this medicine comes in is by gelatin capsule. Max is extremely allergic to gelatin.  This realization kicked off a lot of other doctor visits, with more tests to see if there was any way at all we could administer the medicine and not cause anaphylaxis in Max.

It's taken nearly all year, but in November we finally were able to do a drug trial to see if he would be able to take the medicine without reaction.

We had to do the trial in the hospital, with crash cart and intubation kit ready.  It was possibly the most uneventful hospital/doctor visit he's ever had.  The head of allergy has been Max's allergist since he was a baby, and was the one who wanted to administer the medicine.  They had a few nurses on hand to help if needed.  And Max was the only patient in clinic that day.

We gave the first dose of isotretinoin per the instructions of St. Jude's hospital for administering through g-tube.  Then waited for 3 hours to see if he would have a reaction.  He was happy to hang out and watch videos with Daddy, and then we came home with a new treatment option. 


We haven't seen many side effects from the medicine, other than it makes him sleepy so we give it at night.  And it makes him itchy.  He told his doctor his skin, "feel better but ich".  We are using a lot of lotion and vaseline for his lips so they don't crack. 

All in all, it's working.

The picture on the left is the day we started.  On the right is at one month of treatment.  He had an appointment with the Dermatologist last week and she was really pleased with the progress.  We've increased the dose and we'll see how his skin is doing in a month. 

He has to be seen by his doctor every month as long as he's on the istretinoin because it's a part of the ipledge program. He has to be seen in person monthly before they can write the prescription for the next month. We're going slow with it because it can actually cause his skin to have an overreaction. 

At the appointment the diagnosis has now changed from Acne Fulminans to Acne Conglobata, since he no longer is showing signs of systemic fevers and joint swelling.  We are still trying to get him off of the Prednisone that was keeping it all in check while we were waiting to trial the isotretinoin.  We will be weaning that down for months still so his body can recover from not having the natural adrenal function for so long. 

It's been a long, trying time getting from diagnosis to treatment but hopefully we will only continue to see him feeling better as time goes on.

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