GI scoping in mast cell patients

• Mast cell patients often need gastrointestinal scoping procedures to investigate the cause of dominant GI symptoms or see the full extent of GI organ inflammation, dysfunction or failure.

• GI scoping procedures for mast cell patients require thoughtful preparation due to the many triggers these procedures present. Overwhelmingly, GI scoping is performed safely in mast cell patients.

• An IV is placed before the start of the procedure. It is not unusual for mast cell patients to be “hard sticks”, meaning that it is hard to place an IV. There are several reasons that this happens.

• Mast cell disease causes significant third spacing, a phenomenon in which the fluid is the bloodstream falls out of the bloodstream and gets stuck in tissues. This means that mast cell patients may not have as much fluid in their bloodstream as they should, causing functional dehydration. Dehydration causes the blood vessels to be smaller and more tense.

• Mast cell inflammation is linked to hardening of blood vessels over time, making it harder to get an IV into the vessel.

• Many mast cell patients have connective tissue disorders like Ehler Danlos Syndrome. In these patients, their connective tissue may not properly hold the blood vessels in the right place, making it harder to get an IV into the vein.

• I have a weird observation to add to the “Reasons IV’s are difficult to place in mast cell patients” list. I have found that for the past fifteen years, anytime I had an IV removed, something weird happened. There was some kind of deposit at the IV site. It felt “sandy” and kind of “crunchy”. Whatever was there was solid as I was able to roll it up and down the blood vessel in my arm. I now refer to this as “mast cell deposition” for want of a better term. Once the deposit was gone, which would take weeks, I could no longer get an IV at that site or below it. They would try to place an IV in one of those spots and it hurt a lot and just wouldn’t work. It was bizarre. All of my doctors are stumped. I have two theories: local mast cells have a huge inflammatory response that attracts way more immune cells that normal; or,  that those little sandy bits are platelets all clumped together since mast cells release platelet activating factor. This is purely speculation. Does this happen to anyone else?

• If you are allergic to adhesives like Tegaderm, be aware at Tegaderm is what comes in IV kits to put over the IV once it is placed. If you react to Tegaderm, be sure to remind your nurse when placing the IV that you cannot use Tegaderm and will need another kind of dressing. 

Moist heat can help blood vessels to relax and become larger, making them easier to find and to place an IV there. What worked for me was running a facecloth under really hot water, wringing it out, and letting the facecloth sit on my arm for about ten minutes before attempting to draw blood.

Mast cells are involved in inflammation of the blood vessels. If the mast cells irritate the blood vessels enough, vasculitis can occur. This may be local (close to the site of the IV) or diffuse (more widespread and affection many blood vessels.) Mast cell patients may develop vasculitis from the IV.

GI scoping is performed with twilight sedation. Typically, IV medications are given to patients to help with the discomfort and anxiety associated with procedure. These medications including propofol, midazolam, and fentanyl. There are no particular concerns for the use of these medications in mast cell patient. (These are the meds I use when I get scoped.)

Mast cell patients should premedicate prior to GI scopes starting the day before the procedure. The general recommendation for premedication uses H1 and H2 antihistamines, leukotriene inhibitors, and corticosteroids. You can find this protocol here:
- Prednisone 50 mg orally (20mg for children under 12) 24 hours and 1-2 hours before procedure
- Diphenhydramine 25-50 mg orally (12.5 mg for children under twelve) OR hydroxyzine 25mg orally, 1 hour before procedure
- Ranitidine 150mg orally (20mg for children under 12) 1 hour before procedure
- Montelukast 10mg orally (5mg for children under 5) 1 hour prior to procedure

Premedication is given in addition to regular daily meds.

• A number of patients, including myself, find that using IV antihistamines and corticosteroids before the procedure works better for us. I personally find this to be the case for me. Patients should work with their care team to amend their individual premedication procedure if necessary. My premedication protocol is:
- Prednisone 50mg orally 24 hours before procedure
- Diphenhydramine 50mg IV 1 hour before procedure
- Famotidine 40mg IV 1 hour before procedure
- Solu-medrol 40mg IV 1 hour before procedure

• Patients should be aware that IV diphenhydramine (Benadryl) is sclerotic to blood vessels. This means that the use of IV Benadryl can irritate or damage blood vessels. If using the IV Benadryl in a regular peripheral IV, this could cause irritation of the blood vessels. Dilution of the medication and pushing it slowly through the IV can help to avoid this.

• I personally dilute IV Benadryl in saline (1mL of Benadryl to 9mL of normal saline) and push it through the port over five minutes. I then push the saline flush over five minutes. Last summer, I had a midline placed so that I could deaccess my port in the hopes the ulcer at my access site would heal. A midline is basically one step above a regular IV. They aren’t intended for long term use and they aren’t central lines. Medication pushed through it enters the body is a small vein. With central lines like ports, the medication enters the body into a very large vein that blood is moving through very quickly. I got a really nasty case of local vasculitis from pushing benadryl through the midline. I was diluting each dose 1mL of Benadryl to 50mL of saline and it still hurt. We had to pull the midline after only nine days and I had to go back to using my port. Patients should work with their care team to determine if dilution and slow pushing is necessary in their individual cases.

Touching the GI tract from the inside causes massive mast cell activation. This triggers huge degranulation of mast cells throughout the GI tract. The chemicals released can trigger the activation of mast cells in other parts of the body. The degranulation of mast cells in the GI tract also contributes to a condition called ileusPremedication helps to lessen the severity of activation and degranulation.

Patients should not have to discontinue mast cell medications prior to scoping. If patients are on NSAIDs to block prostaglandin production, like aspirin, the provider may request that this med be skipped on the day of the procedure. However, this is at the discretion of the provider and is a decision specific to each patient. (Author’s Note: Many thanks to MastAttack admin Pari who reminded me of an important note regarding meds and biopsies. A number of mast cell patients also have eosinophilic GI disease. When biopsying for EGID, use of steroids, which is part of the mast cell premed protocol, will skew the results. Mast cell patients who have EGID or who are suspected to have it should speak with their care team about whether or not they need to avoid steroids and for how long in advance of a scope.)

• Patients may find their symptoms are worse than baseline in the days following the procedure. Many people find that increasing antihistamines for a few days can help to mitigate these symptoms. For example, some people do a Benadryl taper. I used to do the same before I ended up taking Benadryl every day. It goes like this:

Day One: 50mg Benadryl ever 4 hours

-Day Two: 50mg Benadryl every 6 hours

-Day Three: 50mg Benadryl every 6 hours

-Day Four: 50mg Benadryl every 12 hours

-Day Five: 50mg Benadryl every 12 hours

Patients should discuss this with their care team to see if this is appropriate for them.

• For many patients, the hardest part of lower GI scoping is the bowel prep. Bowel preping is inherently mast cell activating. Everyone has mast cells in their GI tract. Mast cell patients often have more mast cells than usual in their GI tract. The bowel prep procedure increases GI motility, leading to mast cell activation. Patients should be aware that these increased symptoms, while unpleasant, are not generally dangerous. Patients should ask their care team whether or not they should discontinue the prep or go to the emergency department if certain severe symptoms occur.

• The standard prep for colonoscopies uses some version of polyethylene glycol, things like Miralax or Golytely. Like everything else, there is no way to predict whether or not a patient with react to it. There are alternative preparation protocols for people who can’t use polyethylene glycol. My prep plan is as follows:
 Two days before the scope: 1 bottle of magnesium sulfate, 600mg oral docusate sodium, consume clear fluids only
- One day before the scope: 1 bottle of magnesium sulfate, 600mg oral docusate sodium, consume clear fluids only
- The day of the scope: 2 saline enemas, the first one given two hours before leaving the house, the second one given one hour before leaving the house

• Biopsies should be taken during scopes. Mast cells can cause inflammation on the cellular level and the tissue may be inflamed despite looking normal during the scope.

• Biopsies should be tested using immunohistochemistry (IHC) for the markers CD117, CD2, and CD25. CD117 will show any mast cells present. CD2 and CD25 are markers that are found on the mast cells of many patients with systemic mastocytosis.

• Sometimes providers order the lab to look for mast cells using regular microscopy staining instead of IHC. Toluidine blue and Giemsa-Wright are both stains that can show mast cells. However, IHC is much more accurate than using these stains. Mast cells could be missed by using these stains instead of IHC.

There is not usually enough mast cell DNA in GI biopsies to accurately test for CKIT D816V mutation, a DNA mutation that is associated with mastocytosis.

• You can find additional information on how to test these biopsies here.

There is not a universal way to report the number of mast cells seen with microscope in a GI biopsy that has been put on a slide. One of the more common ways to do this is to count the mast cells in five different high powered microscopy fields (hpf) and then average the counts.

There was an excellent paper published in 2014 called “Perioperative Management of Patients with Mastocytosis.” It is free and publicly available. You can find it here. I encourage you to bring this paper with you to the appointment. The paper discusses all the triggers we experience from surgery and how to medicate patients properly for the procedure. Even though GI scoping is not the same as surgery, the vast majority of advice on surgery in mast cell patients also applies to scoping procedures.

For further reading, please visit the following posts:

Premedication and surgical concerns in mast cell patients

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 5

Third spacing

Coast

When I was growing up, my family went camping every weekend from April to October and most of the summer. We had a trailer on a seasonal site in southern New Hampshire. There were tons of kids around my age and our parents were all friends so they often planned group outings for all of us. We went to a few different places but my favorite was Salisbury Beach.

Salisbury Beach in the 90s was the quintessential New England seaside town. There were several arcades across the street from the beach with lots of games to play. There were places to get fried dough. Salisbury Beach also boasted a relic pulled out of times long past: an oceanside amusement park with a wooden roller coaster called Pirate’s Park. Together with an annual trip to the newer Canobie Lake Park, it instilled in me an appreciation for roller coasters. You can’t worry about work or responsibilities when you are screaming and barreling down a steep incline. There are a handful of seconds when you are totally and completely free.

For this reason, I have always found amusement parks to be worthwhile distractions. In the spring of 2016, after a particularly stressful few weeks, I spontaneously booked a trip to Disney World for just me. I wanted to go swimming during the day and go to parks at night and just be by myself and not have to say the words “mast cell” for a week. I hid behind a huge black floppy hat and sunglasses for a few days. And of course, I stopped at all my favorite roller coasters.

Nicole and I have made plans to go to Universal Studios several times. Every time, I have been too sick to go. We planned to go this past Tuesday but I ended up in the hospital with CDiff. We resigned ourselves to the fact that this adventure would have to wait yet again but then the storm hit and my flight was cancelled. I couldn’t get a flight back until tomorrow. So when I seemed to be okay yesterday, we decided that we were going to Universal today. The Mast Cell Amusement Park Team was back in business.

It was cooler today than normal for Florida at this time of year, in the low 60s with a nice breeze. It felt like Boston in the fall and the weather could not have been more perfect for us. We got Express passes to get us to the front of all the lines and we went on every single roller coaster they had. I needed to take huge doses of prednisone today anyway to prepare for my flight tomorrow and I banked on that prednisone managing my symptoms enough to go on rides. I banked right. It was such a great day.

I can hold my disease back with medical intervention enough to do something like this but it is very temporary and never lasts more than four or five hours. As anticipated, I crashed around nightfall. I was already pretty sore when the day started and I am now in significant pain. Tomorrow will be rough. It will take days beyond that to get back to baseline. I don’t care. I made this choice understanding that this is part of the bargain. CDiff and the Bomb Cyclone blew epic craters in my plans. But we were able to pull this off, and that is worth celebrating.

Not every choice you make about your disease will be the right one. I struggle with this. With every wrong choice comes a rising sense of culpability. As if you are somehow complicit in your own disease, that you have caused this and deserve to suffer. As if your decision to eat a cheeseburger or go to an amusement park could possibly be responsible for all of the things inflicted upon you by mast cell disease. You are not that powerful. You do not have that much control. Mast cell disease is not something that you made happen. It is something that happened to you.

There are days when I am sure I have nothing left, that I’m hollow from the absence of all the things I have lost. But there are other days. On some of those days, I have roller coasters.

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 82

95. How do you take oral cromolyn?

  • Cromolyn is a mast cell stabilizer. Most mast cell patients are on cromolyn. Currently, it is taken orally for use in the GI tract, or it is taken nebulized for use in the lungs.
  • Cromolyn is an incredibly finicky substance. It sticks to everything. Your body barely uses it: only 2% of cromolyn is actually absorbed in the GI tract and only 5% in the lungs. The cromolyn that is absorbed is actually not the cromolyn that helps stabilize mast cells. The rest basically just sits on top of cells in the GI tract or lungs and stabilizes mast cells that way.
  • In order to maximize benefit from cromolyn, it is important that it not be taken when there is food or medications that cromolyn could stick to. This is mostly an issue for oral cromolyn used in the GI tract. You do not want to take other medications too close to taking cromolyn because the cromolyn may stick to the other med and not be available to stabilize mast cells. You do not want to eat too close to taking cromolyn because the food could stick to cromolyn, making it unavailable to stabilize mast cells, or the food could block the cromolyn from getting to the surface of the mast cells, preventing it from stabilizing them.
  • Oral cromolyn is usually taken 30 minutes before meals and at bedtime for a total of four times daily. Cromolyn should not be taken until two hours or more after eating the previous meal as this is about how long it takes for food to move out of the stomach. It is worth noting that many mast cell patients have gastroparesis or impaired GI motility which can cause food to stay in the stomach longer. There is no particular recommendation on what to do in this instance.
  • Ampules of cromolyn need to be stored at room temperature and protected from light. The ampules should not be taken out of the foil packs until you are using them. They should not be mixed ahead of time.
  • The intended dose for oral cromolyn in mast cell patients is usually 200 mg (two ampules) four times a day. Patients usually do better when they gradually increase the amount of cromolyn they are taking rather than starting at that dose. How slowly they increase varies widely. Patients should speak with their providers about an appropriate dosing schedule. There is lots of information about this in patient groups and forums.

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 81

94. How are mast cells involved in cancer?

  • Mast cells are very involved in cancer biology. They are frequently found in tumors. Tumors can trick mast cells into doing things they need to stay alive, like make blood vessels to supply the tumor with blood, and tissue remodeling, to push aside the healthy tissue and make room for the tumor.
  • Cancer is mast cell activating. All cancers. This is because cancers often trick the body into doing things that help the cancer and not the body, like I just described above. Having cancer frequently causes allergy symptoms because of mast cell activation.
  • Cancer can also cause the body to make more mast cells than normal, a condition called mast cell hyperplasia. This can happen because the body is trying to fight off the cancer with more immune cells or because it has been tricked by the cancer to make more mast cells to help the cancer.
  • Please note that mast cell hyperplasia is NOT the same as mastocytosis. Mast cell hyperplasia is too many healthy mast cells that function normally. Mastocytosis is too many aberrant mast cells that do not function normally. Cancer does not cause mastocytosis.
  • Long term inflammation increases future risk of cancer at the site of inflammation. This applies almost universally. Mast cells participate significantly in inflammation so they can contribute to the risk of cancer. For example, patients with long term colon inflammation, which may be caused by mast cells, are at increased risk of colon cancer.
  • Patients with mastocytosis have increased risk of developing cancer, especially those with systemic mastocytosis. As many as 40% of patients with systemic mastocytosis develop another blood disorder with too many broken cells. Frequently, the other blood disorder is a blood cancer like chronic myelogenous leukemia.
  • It is not yet known if mast cell activation carries an increased risk of developing cancer.
  • Two forms of systemic mastocytosis are cancerous, mast cell leukemia and mast cell sarcoma. These are both extremely rare and it is extremely rare for a person with a history of mast cell disease to develop either of these conditions.

For further reading, please visit the following post:

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 48

Mast cells in the GI tract: How many is too many? (Part Three)

An open letter to healthcare providers about pain medication

Dear Health Care Providers,

My name is Lisa Klimas. I am a 34 year old research scientist and a subject matter expert in mast cell diseases. I have a rare blood disorder called systemic mastocytosis.

The hallmark of mast cell disease is severe allergic reactions or anaphylaxis to things we’re not actually allergic to. Constant mast cell activation can affect every system in the body. Organ damage from inflammation is not unusual. I myself have had significant organ damage, most specifically in my GI tract. Additionally, mast cell reactions and anaphylaxis can occur at any time and without warning. Living with this disease is unpredictable in the extreme.

Mast cell activation under a variety of circumstances has been linked to pain. I have pain. I have bone pain in the legs and pelvis. I have costochondroitis that keeps me awake. I have significant GI pain. My stomach is paralyzed. My colon is heavily damaged and has been operated on twice. I had a colostomy for over two years. My colon has herniated multiple times.

I deal every day with the consequences of this disease. I deal every day with the pain it causes me and will always cause me. Since pain is part of my life, so is pain medication. Since pain medication is part of my life, so is abuse and judgment by providers I don’t know. It is so prevalent and requires so much energy that mistreatment by health care professionals is almost a symptom of this disease.

Every good story has an antagonist. You need someone to root for and someone to despise in order to drive the narrative. This also plays out in real life. Shared disdain provides a reassuring connection to others who share your views. There is always a good guy and a bad guy. And in the story that the healthcare establishment tells itself, patients who need pain meds are the antagonists. We are the bad guys.

Healthcare providers often feel that it is not just allowed, but expected, that patients in need of pain medication are treated as if they were seeking these medications for recreational use. We know those people exist. But we are not those people. We are people who are damaged and hurting. Pain is crushing. It takes so much from you. It takes things from you that you can never get back. For many of us, the only way to control the pain is with narcotics.

We have legitimate medical needs for pain management medications. We do not get high from them. We do not enjoy them. For many of us, pain medication is the difference between functioning and not. We are just trying to live our lives to the best of our ability and pain medication makes that possible.

Please recognize this situation for what it is. Please stop conflating pain patients with drug addicts. We are not scamming you. We are coming to you for help. Please treat us the way you would treat anyone else who came to you for help.

This story you are telling yourself is wrong. We are not your enemies. We are not antagonists. We are people. Help us.

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 80

93. How is adrenal insufficiency related to mast cell disease?

Adrenal insufficiency is when the adrenal glands do not make enough cortisol, a stress hormone your body needs to help your body respond to the things happening inside and around it. Not having enough cortisol is dangerous and can be fatal.

Adrenal insufficiency is not the same as adrenal fatigue. Patients with adrenal insufficiency demonstrate lower than normal levels of cortisol. Adrenal fatigue is a term that is used to describe a similar constellation of symptoms as seen in adrenal insufficiency but without the lower than normal serum cortisol level when tested. Adrenal fatigue is not well accepted in main stream medicine.

There are several steps involved in making cortisol. These steps use hormones to tell the body to make other hormones until cortisol is finally made. The molecules that are involved in getting the body to make cortisol are collectively called the HPA axis.

Mast cells interact with the HPA axis a lot and in several ways. I have written extensively about this before.

The activity of the HPA axis can either activate mast cells or stabilize them. It can tell the body to make epinephrine, which decreases mast cell activation. But it can also tell mast cells to make inflammation.

It also works in the other direction. Mast cell activation can activate the HPA axis or not, but it usually activates it. If mast cells generate enough inflammation, that can turn on the HPA axis, which in turn activates mast cells even more. This basically means that if you have frequent mast cell activation, your body can end up in a constant fight or flight response. The inflammation generated can be enormous.

When the body has been in a stress response for too long, the adrenal glands can stop making cortisol, causing adrenal insufficiency. This can cause mast cell activation.

Steroids like prednisone mimic the action of cortisol, the stress hormone. Steroids are sometimes used to treat mast cell disease. The purpose of the steroids is to make cells like mast cells stop causing inflammation. If you take systemic steroids like prednisone routinely, your body can become confused and stop making cortisol on its own. This means that when you stop taking the prescription, your body will not have enough cortisol, causing adrenal insufficiency. This activates mast cells in a huge way. Patients often have a hard time getting back to a good baseline without steroids if they have been on steroids for a while.

There is an autoimmune disease called Addison’s Disease that causes adrenal insufficiency. MCAS sometimes occurs secondary to Addison’s.

 

For further reading, please visit the following posts:

The effects of cortisol on mast cells: Cortisol and HPA axis (Part 1 of 3)
The effects of cortisol on mast cells: Cortisol and HPA axis (Part 2 of 3)
The effects of cortisol on mast cells: Cortisol and HPA axis (Part 3 of 3)
Corticotropin releasing hormone, cortisol and mast cells
Mood disorders and inflammation: High cortisol and low serotonin

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.

 

The push down

I am a very busy person. I sometimes don’t realize how busy I am. I work full time. I study. I write and research for MastAttack. I contribute to care plans and work with patients. I am an involved auntie. I spend a lot of time with my family. And of course, I am sick, and being sick accounts for the majority of my life.

I like making plans. I have always liked making lists and checking things off. These days, I have so much to do that I have to be really organized to get anything done. Everything goes into my planner. I read it every day as soon as I wake up to ground myself and focus. Every task has an assigned completion date and time allotment. If I have ever agreed to answer a question for you at a later time, or to research something, or to do something for you, you are in that planner.

My planner keeps track of everything I do and, often, everything I plan to do, but which I do not complete by the anticipated time. As time goes on, I accumulate more and more outstanding tasks. For the most part, they get completed eventually, but it is sometimes several days or weeks later than intended. Like everything else in my life, my ability to stay on task is fully dependent upon my health. Because my health is unpredictable, everything else in my life becomes unpredictable, too.

For the last several months, I have been trying to buy a home. I have been trying to find a two family home so that my parents could live in one while I live in the other. Initially, it looked like the easiest option would be to level my parents’ house and build a two family on that property. Then we looked at existing two family homes. Then we looked into modifying a single family home to make it two separate apartments. The last eight months has been consumed by this.

Yesterday, we looked at a house that largely met all of our needs. We discussed making an offer and ultimately decided against it. For the most part, the experience of looking for a place to buy has not been particularly upsetting me, but yesterday, it upset me. I’m not sure why. I suddenly felt overcome by the weight of all the emotional things I have tied up in this house hunt.

For a disabled woman, I am in a pretty plum position right now. I work full time for an employer that accommodates and respects me. I can afford to pay my living and medical expenses and have some left over to travel and do fun things. I have excellent insurance. I have tried hard to set myself up as much as I possibly can for the eventuality of becoming unable to work and dependent upon disability benefits. With the exception of traveling, I live pretty frugally. I live in a very small rented apartment. I don’t eat much. I don’t really shop unless I need something specific. Aside from having two dogs who both need healthcare and medication, my health is my only major expense. This has afforded me an enviable financial position for someone as sick as I am.

I am recently coming to grips with the fact that while I am in a good position now, it could all be gone in an instinct. Any instant. Maybe this one right now. Maybe one tomorrow. I could become unable to work immediately. And while I have been able to save some money, it is impossible for me to put aside enough money fast enough for me to not have to worry about it. There’s just not enough time. No matter what I do or how well I plan, my financial situation will inevitably become unstable. This disease has robbed me of the ability to plan for my future.

My fear of instability is largely what has driven my search for a new home this year. I have lived in my current apartment for years. I don’t have any reason to think I’ll need to leave soon. There are things I don’t like about the apartment but it is cheap and meets my needs. But I don’t own this place and that means that the stability of my housing situation is fully dependent upon other people. I don’t have family in a position to take me in. I can’t live in my parents’ house. That means that if I suddenly had to move and couldn’t work, I have no idea where I would even go.

I have this life now with a safe, clean place to live near my support system, good healthcare, and a stable job that allows me to support myself well. But this could all be gone in an instant. I could do everything right, make every decision right, and still end up with nothing. That is almost certainly what will happen.

There are moments when my fear consumes everything. It is my omnipresent companion, the harbinger of a coming plague. It threatens to devour every good thing in my life, to just eat and eat and eat until there’s nothing left.

So what do you do when this life you love will be gone soon? You enjoy it. You use all your strength and you push down this fear and you enjoy it.

I will never be able to save enough money to keep me financially stable and independent. If I spent nothing on anything except my absolute life essentials, it wouldn’t matter. I try to remember this on the days when I book airfare or buy myself a new dress. I do not have to feel guilty that I am not saving every single cent I can when it wouldn’t matter anyway. It is okay to relish being alive. It is okay to have as much fun as I can. It is okay to spend money to be comfortable and happy.

Last night, as I was making notes about projects I plan to complete in 2018, I was inexplicably overcome with this sense of calm. As I looked over my upcoming trips noted in my planner calendar, for the first time in a long time, I felt perfectly at peace. It is okay to spend money to go to Utah and California and Australia. It is okay to use this body for everything it is good for until the time when it is no longer good for anything.

I’m heading to Florida for a week after Christmas to visit my Masto Little Sister, Nicole, and my dear Kristina Brightbill (who is making AMAZING gains). I’m going to enjoy every minute of it.