Jonathan’s Pemphigus Treatment

Hi, my name is Rachel, and I created this fundraiser to help fund my husband's medical treatments for Pemphigus Vulgaris. To help everyone understand how easily illnesses can go undiagnosed, and help you understand this illness, here's a bit of background on our experience with this disease for the last few years:

Beginning around mid-2018, a few "bumps" would appear sporadically on Jonathan's scalp. We simply attributed it to him working out in the sun and heat all day, or an allergic reaction. We went to get him checked a few times, but the only diagnosis given was an unspecified form of dermatitis. We didn't make too much of it, because his "bumps" didn't seem to cause him any trouble.

Then, in 2020, blisters began to appear in his mouth. They were painful but still went undiagnosed because by the time we went to the doctor to get them checked out, they had disappeared. After the mouth blisters, small blisters on his back began to appear. Again, we associated them with heat, sweat or an allergic reaction. And again, they were diagnosed as an "unspecified form of dermatitis."

At first, we didn't think the blisters were a big problem because they didn't hurt and didn't seem to cause any problem with his everyday life. However, early this year, the blisters began to appear at an exponential rate, and in April 2022, after a biopsy on one intact blister, he was diagnosed with a rare auto-immune disorder called Pemphigus Vulgaris.

Pemphigus Vulgaris is a rare lifelong condition caused by a problem with the immune system. Normally, your immune system produces antibodies to fight off harmful invaders, such as viruses and bacteria. But with pemphigus, the body produces antibodies that attack the proteins that bind your skin cells and mucous membranes. This causes a buildup of fluid between the skin cells, which results in the formation of blisters. Blisters can be inside the mouth and all over the skin/body. PV can be life-threatening if left untreated; in some cases, it can lead to a blood disorder (leukemia). Currently, there is no cure for this disease, but there are a few treatments that may help a patient reach remission.

Unfortunately, at this time, Jonathan has exhausted all of the oral treatment options (immunosuppressants) for PV. I won't give out too many details or post actual pics of his current condition because it is now severe. I can tell you that he is in pain; he can't make sudden movements, showers hurt like a mf, and if his t-shirt gets stuck to his skin (which happens all the time), it feels as if his skin is being peeled off. His dermatologist has put in an urgent referral to Baylor College of Medicine to evaluate and administer one of the following two treatment options:

1) IV infusion of Rituximab administered over several hours at various intervals. B-cells are responsible for producing antibodies for the body, and Rituximab works as an immunosuppressant that destroys all of the B-cells of the immune system. A course of rituximab is administered with the hope that it will destroy all the B-cells that make the antibodies in pemphigus attack skin cells. Retreatment with Rituximab may be required, usually at six months or longer after the initial treatment.

2) Intravenous Immunoglobulin (IVIG) therapy. This treatment is done by extracting the plasma from human blood. IVIG is given intravenously, under the skin via a syringe or catheter. The doses can be as high as 2000mg/kg to treat PV. Because doses are more elevated, infusions are administered over the course of up to five days. This treatment can become a lifetime commitment, or the condition may be resolved, and IVIG can be discontinued.

Both of the remaining two options will be a slow and tedious road to possible remission. At this time, we still don't know which option Jonathan will begin or the side effects he may experience after receiving either treatment option. Both treatments are similar to chemotherapy and will take a toll on him. If option 1 is attempted, he will have zero immunity to anything. He will slowly have to be revaccinated for every single vaccine he’s ever received, so we’ll have to be extra careful to try not to get sick and try not to get him sick because even a cold can cause serious damage (just like with cancer/chemo patients).

If any of you know me, you know I'm the type of person who always thinks of every possible thing that can and will go wrong, and I always try to plan in anticipation for "worst-case scenarios". (Anxiety much?) My husband and I are 100% not the type of people that ever ask for anything from anyone. And we especially do not like to share or put our business all up on social media. However, at this time, we are putting our pride aside because we do not know what's in store for us. We don’t know if the possible treatments will help him reach remission. We don’t know if he will have to receive treatments every 6 months for the rest of his life.

We are sharing our story with the hope that we, at the very least, help spread awareness about Pemphigus and other rare autoimmune disorders. Also, and most importantly, to help remind everyone to please not dismiss anything you feel is off or wrong with your body, even if it seems like no big deal, even if that something doesn't hurt or bother you, please get it checked out. I know, especially in the Latin/Hispanic community, we basically don't complain or go to the doctor until we're at like a 1000000% pain level until we literally can't walk or move anymore. We need to take better care of ourselves, even if it means taking time off from work.

I will personally be making Pemphigus Awareness shirts for anyone who can donate at least $20 to help fund Jonathan's future treatments. Please DM me after your donation with a unisex tee size and address and a possible pickup location in the 77065 area. If you're unable to contribute, I sincerely hope you can share this page in the hopes that we can help spread PV awareness and be able to fund more research to possibly find a cure for this and other rare auto-immune disorders. From the bottom of our hearts, we sincerely appreciate all of you during this trying time.

08/10/22 update:

We had our visit with specialist at Baylor last week and doctor ordered various labs and a bone density scan to ensure high dose steroids are not harming Jonathan’s bones. We may have to redo a couple labs due to issues with specimen obtained, but so far everything seems okay. Will follow up with Dr. in 3 weeks. At that time, after reviewing Jonathan’s lab and exams, and everything seems okay, the Dr expects to put in an order for Rituxan infusion treatment. 

Jonathan’s eye irritation/watering/redness has increased so we will be seeing an ophthalmologist the end of this week. Because pemphigus affects mucous membranes, which includes your eyes, it can cause severe damage to your eyes and vision. Inflammatory lesions on the ocular surface may result in scarring, loss of tear film, adhesions of the lids to the eyeball, corneal ulcers, and perforation. The frequent scarring that often occurs in this condition can lead to severe vision loss. We’re hoping that this is not the case with Jonathan. We’re not sure what kind of exams/tests will have to be performed, but we will find out in a few days.