Flood

Floods are often used metaphorically. In literature, flooding is a tool that indicates a need for a new start, a beautiful and ethereal destroyer. It’s something everyone can easily envision. We can all relate to the need to breathe and the fear of dark and rising water.

My city is underwater. This flood is not metaphorical; it is real and devastating and historic. There is currently no way to get in or out of my town. Historically high tides are running through the streets. Just a few blocks from my home, people are being rescued from several feet of water by front end loaders. A fire truck had to be towed after getting stuck. Just Revelations level insanity. The craziest shit you’ve ever seen. Except I’m not there to see it.

People who have been following MastAttack for a while will know that I spend a fair amount of time in Florida. Three of my best friends, all of whom have mast cell disease, live in different cities dotting up the gulf coast of the state. Nicole has a horse farm in the middle of nowhere with no wifi and shitty reception. It’s the perfect place to lay low and duck out of life for a bit. I flew down the Wednesday before New Year’s with a return trip booked for yesterday. But I didn’t make that flight and I don’t yet know what flight I will make because I have been dealing with my own Revelations level insanity down here.

Last Saturday, Nicole and I drove from Ocala to Sarasota to visit my dear Kristina and her fantastic parents. On the way, we stopped and got breakfast. I got something I eat regularly without trouble. Thirty minutes after I ate, I knew something was wrong. The situation quickly evolved from GI cramping to excruciating epigastric pain. I took meds and applied Benadryl liberally.

We visited with Kristina and her family all afternoon which was great and not Revelations level insanity. For people who don’t know, Kristina is a mast cell patient who had a stroke in her brainstem in October 2015. The stroke caused Locked In Syndrome, a condition in which the patient is completely aware and cognitively normal but is unable to move or speak.

Kristina’s family was told that she would never recover any function. The good news is that that was a bunch of garbage because she’s regaining function and body control every day. It is an incredibly slow process but she is doing it. She now gets all her nutrition by mouth instead of via G tube. She is stable without IV meds. She is building core strength and working on standing. She communicates by a special computer that will read aloud what she types. She is able to leave the house more now and attends church regularly.

My GI tract was pretty sore when we left Kristina’s house and I realized shortly after that I had a GI bleed. This is not unusual or impressive for me; I bleed more often than I don’t at this point. I figured taking it easy and eating minimal solids for a few days would resolve it. It didn’t.

I woke up on New Year’s Eve feeling very sore but otherwise okay. I went for a walk around the farm. I did some yoga. Nicole and I went to her parents’ place for dinner. By the time dinner rolled around, I was feeling pretty nasty. I went back to the farm to medicate heavily and go to bed.

I had been puking and having diarrhea for a couple of hours before I started to think something was very wrong. I was sure that this was not a reaction and figured I had picked up a stomach bug somewhere. I was shivering and achy and unable to get warm. I called Nicole in the middle of the night and she came over with a thermometer and BP cuff. I had a fever of 102.5.

When you have a central line, every fever is scary. I don’t get them a lot and it really scares the shit out of me when I do. A line infection can be fatal. Even when it’s not, it can takes months to recover from one. I knew I needed to go to a hospital 1000 miles from home where no one knew me. I was scared of a line infection. But I was more scared of being subjected to ineffective care from providers who didn’t understand my disease and wouldn’t listen to me.

I remember getting into the ambulance. I do not remember arriving to the ER. I was super tachy with high blood pressure and a screaming high fever. We all immediately assumed this was sepsis. They didn’t fight me about my mast cell needs which is lucky because I doubt I would have been able to do anything about it anyway. I was hallucinating. The GI symptoms continued when a vengeance. They got my records from Boston and admitted me later that day.

The following day, I tested positive for CDiff, a severe GI infection that is almost always caused by recent hospitalization or recent antibiotic use, neither of which applied to me. I recovered so quickly it was almost shocking. I was discharged last night.

I had to reschedule my flight home because of the hospital admission. I was not healthy enough to travel. Boston was forecast to suffer an unreal storm the following day. In New England, the storm doesn’t always deliver the fury promised by meteorologists. This storm delivered. My flight for tomorrow was cancelled. It looks like I’ll get back to Boston Monday or Tuesday.

I am very paranoid about getting stuck somewhere without adequate medication or supplies. I am so paranoid about it that I have an excel spreadsheet that tells me how much to bring of everything based upon 150% of expected use. Fortunately, this means that I am pretty well stocked and can afford to wait out a few extra days. There are a few things I didn’t pack enough of. After a lot of anxiety and fretting, I have managed to cobble some of it together with the help of local patients. We will to figure out the rest of it tomorrow.

I have struggled with my fear about my disease since the day I was diagnosed. It’s not rational but it’s real. I have literal nightmares about forgetting my medication or supplies when I travel. I have literal nightmares about getting sick far from home and ending up in a strange hospital that doesn’t believe what I tell them.

It used to seem to me that it should get easier to cope with this fear but it never has, at least not for me. I keep waiting and waiting to happen across the moment when I am not afraid. When I can take a full breath. When I don’t feel like I am being pushed into the ground. When something unexpected does not immediately signal emergency. But I never do because there is no moment. That moment does not exist.

I hope my city get its head above water before I get home. I hope I get my own head above water, too.

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 79

92. Why is ketotifen not FDA approved? How do I get it?

Ketotifen is a mast cell stabilizer that is also an H1 antihistamine. It is regularly cited by mast cell patients as one of the more effective meds for managing mast cell disease, especially food intolerance. But it can be tricky to get ahold of in the US.

Firstly, ketotifen actually is FDA approved. It is FDA approved in eye drops. However, the formulation typically used by mast cell patients is oral. Oral ketotifen has not been approved in the US, but it’s not because it’s dangerous. It’s because it was never submitted to the FDA for approval. And why was it not submitted? Again, not because it’s dangerous. At the time, the manufacturer did not feel that there was enough of a market to justify the time and expense of an FDA submission when there were so many other H1 antihistamines available both over the counter and with prescription. It’s that simple.

So how do you get ketotifen in the US? You can import it from abroad for personal use as a mast cell patient, but there is an easier way: ketotifen capsules can be bought through compounding pharmacies who order the powder and put it in capsules. The most common strength for capsules is 1mg. Your provider just writes a prescription for it and the compounding pharmacy puts it together for you. As a side note, insurance often does not cover compounded medications so be prepared for that.

Because there wasn’t an FDA submission, there is less safety and dosing information available. In adults, dosing typically starts at 2-3mg a day. Some providers use much higher doses, even going upwards of 20mg per day in some instances. Again, we don’t have study data on this drug in mast cell disease, so conservative dosing is common.

Ketotifen is available as a tablet without a prescription in many countries, including Canada.

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 78

91. How long should it take to know if a medication is working?

  • This topic is controversial and how long to trial meds is not agreed upon. It varies by provider. This is because there haven’t been many studies done on how long it takes to see therapeutic effects in mast cell patients.
  • Firstly, this question is not “how long does it take for a medication to become active after I take it.” This question is how long you should keep taking a new medication to see if it helps your disease.
  • Firstly, when you are trialing a new medication, or even a new medication dose, try as hard as possible to not change anything else at the same time. It is easier to do this for medication that has short term benefits. I realize this is not always possible, and when it is, it is still a pain.
  • However, you really do need to be able to tell if any changes that occur are from the medication change or not. For example, if you are trying a new antihistamine, and two days after you start it, you also increase your dose of another med, and two weeks later you feel better, you are going to have no idea if it was the new antihistamine or the dose increase of the other med that helped.
  • In my experience, this leads to people being on a ton of meds that don’t all help. Some of us are on a ton of meds that actually help and that can’t always be prevented, but a lot of people just keeping adding things on top of one other without being sure they help. This can really complicate things down the line.
  • How long I trial meds has always been determined by how long it takes for them to cause notable changes in clinical symptoms. Because there aren’t a lot of studies on this topic in mast cell patients, it is common to use recommended time frames found in literature for other cells or other diseases.
  • If they have immediate short term benefits, I trial them for two weeks. Medications that block mediators from acting, like antihistamines and leukotriene inhibitors, are in this group.
  • If they have moderate term benefits, I trial them for six weeks. Medications that prevent mediators from being made, like COX inhibitors for prostaglandins or 5-lipoxygenase inhibitors like zileuton, are in this group.
  • If they have long term benefits, I trial them for sixteen weeks. Mast cell stabilizers like cromolyn and ketotifen and biologics like anti-IgE therapies are in this group.
  • If meds have mixed term benefits (like short term and long term effects), I trial them for the longer term.
  • Please note that steroids are a special case here because they have so many effects that are short, moderate and long term. People generally see immediate relief from them but they really are not meds that should be taken regularly if it can be avoided due to the slew of dangerous side effects.
  • These time frames have been recommended to me by my care team but you will need to discuss this with your own care team. I have found literature supporting these time frames necessary to produce clinical changes in other cell types or diseases.
  • I would also like to mention that in the past, I thought that four weeks was the appropriate period for trialing meds with short term benefits like antihistamines. I now feel that a two week trial is sufficient to identify benefits from these meds.
  • Please also note that for advanced systemic mastocytosis, including aggressive systemic mastocytosis and mast cell leukemia, there have been studies that have identified optimal duration of therapy to see a response for interferon and chemotherapies.