Thursday, June 15, 2017

Last Trip to MN!!! Kind of =)

We just got back from my latest trip out to MN to see my mast cell specialist, Dr. Afrin (actually his PA this time). If you've been reading this blog for any length of time, then you know (1) Dr. Afrin is pretty much amazing as a doctor and researcher for the mast cell community, and (2) I go to MN every 6 months to follow-up with him (or his PA). Except not anymore...maybe...

Could I be any less clear? Probably =)

Leni just resting that heavy SD brain she's got =)
Anyway, the big headline news in the mast cell community of the past week is that Dr. Afrin is leaving the University of Minnesota where he has been for the past 3+ years. At first glance, I think pretty much everyone in the mast cell community was in disbelief after hearing the news. How could such an incredible researcher and advocate for his many rare disease patients be leaving when so many of us (and our doctors) rely on him for his vast understanding of mast cell mechanisms and disease processes?

The answer...he couldn't...So he's starting his own mast cell institute! 

There is already a Center of Excellence for Mastocytosis and Mast Cell Activation Disorders in Boston where there are a couple other mast cell specialists that many east coast patients see. But other than that cluster, the mast cell knowledge is very much sparsely spread out across the country. In addition, beyond the specialists in Boston, Dr. Afrin, and a couple of others, there are very few doctors who truly understand the complexity of mast cell diseases and have the experience and knowledge to effectively treat them. 

It is very exciting news that Dr. Afrin is heading off on his own to start another center for mast cell research and treatment! We hope that as he gets set up with his new institute, more doctors will be brought on that are familiar with mast cell diseases, can learn from Dr. Afrin, and become additional resources for the many patients who wait years to see some of these top specialists. We are hopeful that with this new institute, new research can be done, additional treatment options explored, and maybe some breakthroughs will finally happen for those of us living with these complex, rare diseases that right now are understood by so few!

We are hopeful that this could be an incredible crossroads for mast cell research and be one more step in the right direction toward better diagnostics, more effective treatment options, and someday, one day, a cure!

In the meantime, though, there's plenty to keep all of us busy...while this is exciting news and a chance for renewed hope, it doesn't solve any of our current challenges in living with mast cell disease. My gut is still not absorbing, my neurological symptoms have not disappeared, and I'm still fighting my mast cells every day. So in the meantime, we have some new options to consider and decide what the best plan of action is at the moment. 

Like I mentioned in my previous post about the end of my GI testing (you can read that here), we are at a tough point. With my GI tract not wanting to play nice, we are forced to get a bit creative in terms of treatment. Unfortunately, until we get my GI symptoms under control, trialing any new oral medications is just not an option. Which puts us in a tough spot of having to decide what to do to get me more stable. While I am using IV meds on a daily basis through Mort (my port), the meds I am using currently are only rescue medications and not regular everyday management medications (despite me needing them daily right now...). We have been doing everything we can to sort out my GI issues, because we do not want to start any long term IV medications for everyday management. While I am living with a needle in my chest 24/7 right now, this is not something we hope to have to deal with long term. Thankfully, after my appointment on Monday, we came up with a few more options to try!

As we have exhausted all possible explanations for my GI issues (although there is potentially an update here as well!?!?), we are realizing that my symptoms appear to be mast cell driven. Meaning nothing will change until we can get my mast cell more stabilized and we can't keep waiting and hoping things will just resolve on their own. And since those very symptoms make adding new oral meds not a good option, we are faced with having to get a bit creative.

We'll be significantly increasing my dosage for a couple of the oral meds that I am on to see if by adding in higher amounts of the meds, we can increase even the small amount of absorption I am currently having. We will be trying to switch formulations for a few of the meds to see if that may impact how well I absorb things. We are also changing some of my meds from just as needed to regularly scheduled use. The meds are doing this with are localized medications but have also been reported to have a systemic benefit. Meaning that even though one may be targeting mast cells in the lungs and the other in the eyes, some patients have still seen improvement in other areas when taking these meds. Right now any options that bypass the gut are high on our list of things to try, so we'll be seeing if I can get a systemic benefit from some of these meds, too.

We also discussed (at length...) my Xolair situation. It's a bit tricky. If you read my previous post, then you saw I both passed out in my doctor's office AND went into anaphylaxis (warranting 2 separate ER trips) within 8 hours of my injection last week. This is not the first time I have had a severe reaction or anaphylaxis within 24 hours of getting Xolair which is one of the black box warnings for these injections. HOWEVER, I've also managed to have an anaphylactic reaction to absolutely nothing while sitting in my room at home without having had anything to eat or drink or done anything that could have possibly triggered a it's not really clear cut if I have started reacting to the Xolair...We know that when I started it over a year ago, it made a HUGE difference for me. I was able to begin eating again without reacting severely to digestion and that benefit has continued. Even though my gut is not playing nice in many ways, I am still able to eat some solid food on good days without risking anaphylaxis. I could possibly lose this again if we stop Xolair. We just don't know. And unfortunately, no really does. Dr. Afrin's protocol is usually to stop Xolair after the first anaphylactic reaction, but yet again, I seem to have found a gray area in an already not well understood rare disease.

We have decided to give it a few more months. We are going to be a bit more aggressive with pre-treating prior to the injection AND for the first 24-48 hours after I get it with the hope that being a bit more pro-active in preventing issues may help avoid any potential reactions to the injections. We'll be adding in a few new meds during that 48 hour window and...just see what happens...Always said I'd love to donate my body to research, but somehow I didn't think that meant while I was still alive...

To be honest, I'm very torn about this. There is a part of me that wants to run in the opposite direction of any potential anaphylaxis trigger...who wouldn't want to do everything possible to avoid that?? But at the same time, I know how much benefit we have seen from the Xolair...that we KNOW is from the Xolair...I desperately don't want to lose what has been one of the most effective treatments for me! Since we're not 100% sure the reactions are directly caused by the injections...we keep going, keep trying, and I keep pushing the fear from my hands into God's and trusting Him since I definitely don't have any control over this...

Photo on the art museum in Minneapolis. Somehow
I don't think it's the same type of POTS... =)
Please keep us in your prayers for these next few weeks as we attempt to put together a plan for the next few months. Our trip to MN was good to get some new suggestions and discuss options which I will be discussing more with my my local allergist and GI at my appointments later this month. Like I alluded to earlier in this post, we also have a new potential direction to head with my GI issues that we are praying may be the breakthrough we have been looking for. We still need to go ahead with this new treatment plan for mast cell issues since any testing and treatment would be a potentially lengthy process, but we have another small glimmer of hope that we are pursuing for one more chance that there is something we might actually be able to address in my GI tract. Please pray for ease of scheduling and discernment for my doctors as they review my testing and history and help decide on the best plan of action for me and my nasty masties. And as always, pray for smiles, joy, and laughter and that we are able to glorify God no matter what my mast cells may be doing. 


  1. For your absorption issues.. Have your tried oral cromolyn sodium. My wife is on the waiting list for Dr. Afrin, but her immunologist suggested we try this. It had a profound effect, for years she had been taking oral vitamin d3 (same brand, same doseage) in huge iu's because blood tests would constantly show low levels. After 3 months on oral cromolyn sodium those blood test numbers shot up so high that she had to temporarily stop the d3. Nothing else had changed, and as you know with this disorder you keep a tight watch on your routine.

    Once thing that she has learned over the years is that for her, she has to start with very small amounts of everything. The same held true for cromolyn sodium. I would suggest starting out with one vial in 4oz of water. Then take a literal baby sip, and do it 30 min before you try to eat. My wife found that she cannot tolerate over 1-1.5 vials per day at her maximum amount. But it took a several weeks to reach that level. Good luck to you

  2. Thank you for the comment! I have tried oral cromolyn, and it has been one of the most effective GI meds for me. I think I've been on it 5 or 6 years now.