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.

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 77

90. What causes pain in mast cell disease?

  • Most mast cell patients experience some kind of pain. Because mast cells are involved in pain sensation and inflammation, mast cell patients are at risk of pain by different mechanisms throughout their body.
  • Mast cells are involved in nerve pain. Mast cells often live very close to nerves, sometimes so close they are touching. When nerve cells feel pain, they release mediators to activate mast cells. The mast cells then activate other nearby nerve cells. The result of this is that the brain gets a pain signal from lots of nerves, not just the nerves that initially felt the pain, so the pain you feel is worse.
  • Mast cells participate in inflammation. One of the big things they do is send signals to other immune cells to come to the site of inflammation. These cells release mediators that can cause pain or make it worse. Nerve cells nearby will send a stronger pain signal again in response to these immune cells causing inflammation.
  • Mast cells are involved in hyperalgesia, when your nerves are very sensitive and send a stronger than normal pain response to things that shouldn’t normally be very painful. For this reason, many mast cell patients have a heightened pain response, even to things that aren’t normally very painful.
  • Mast cells are associated with a number of chronic pain conditions.
  • Visceral pain is when you feel pain in your internal organs, like your GI tract or your liver. Visceral pain is often not localized so it can be hard to tell what is actually hurting. Mast cell patients often report visceral pain.
  • Pelvic pain is linked to mast cell activation and can cause serious symptoms, including painful sex. Pelvic floor dysfunction is sometimes seen in mast cell patients. Interstitial cystitis, chronic inflammation of the bladder, is also driven by mast cells, although it’s not exactly clear how.
  • Mast cells are major players in GI pain. Mast cell degranulation activates the nerves inside the GI tract, which can cause abdominal pain. This causes pain in a number of GI diseases aside from mast cell disease.
  • Many mast cell patients have connective tissue disease like Ehlers Danlos Syndrome. This can cause the organs to not be supported properly, causing them to move around, activating a pain response.
  • Mast cells can cause bone pain in multiple ways. In systemic mastocytosis, production of so many mast cells in the bone marrow can cause pressure inside the bone that causes pain. Mast cell mediators can cause dysregulation of the system that degrades old parts of the bone and replaces it with new, stronger bone. This can cause the bones to be too thick or too thin. Mast cell patients may have bone disorders as a result and should be especially watchful for Mast cell mediators like histamine can also irritate the cells on the outside of the bone, causing pain.
  • Mast cell activation can cause headaches and migraines. Mast cell mediators can affect how much blood is getting to the head and brain, which can cause pain. Many mast cell patients have POTS, which can also cause the same problem.
  • Systemic mastocytosis patients can have dense infiltration of their organs by mast cells. This infiltration punches holes in the tissue, leading to inflammation and pain.
  • Cutaneous mastocytosis patients have similar issues with infiltration of the skin.
  • Infiltration is NOT necessary for mast cell activation to cause pain.
  • Mast cell patients have to be cautious in how they treat their pain as many medications for pain management can cause mast cell degranulation.
  • NSAIDs can be used in patients that tolerate them.
  • Acetaminophen and tramadol are considered mast cell friendly.
  • Gabapentin and pregabalin are sometimes used for neurologic pain in mast cell patients.
  • If opiates are needed, fentanyl and hydromorphone are preferred. Morphine is a massive mast cell degranulator and should be avoided.
  • Certain numbing medications can trigger mast cells, like ester caine anesthetics.
  • Cyclobenzamide is a muscle relaxer commonly used in mast cell patients.

 

For more information, please visit the following posts:

Mast cells in nerve pain

The Provider Primer Series: Medications that impact mast cell degranulation and anaphylaxis

Premedication and surgical concerns in mast cell patients

The Sex Series – Part Six: Male pelvic floor and mast cells

The Sex Series – Part Eight: Female pelvic floor dysfunction

The Sex Series – Part Nine: Female pelvic floor dysfunction

 

Colorado heartbeat

I am alive. I haven’t been feeling well. The adrenal crisis really kicked my ass and, if I’m honest, scared the shit out of me. It has taken me longer to recover than I anticipated and have had to focus my meager available energy on work. I didn’t realize how long it had been since I posted. Sorry if I scared anyone.

In the midst of these busy and exhausting last few weeks, I decided to run away from my life for a long weekend in Colorado. Colorado is my happy place. It is so beautiful that words don’t seem worthy to describe it. It is quiet and clean. It represents every amazing thing the earth is capable of. I have visited twelve times and I am always just as awed as I was the first time.

Since 2007, a series of people in my life who don’t know each other have moved to Colorado. It started when my best friend at the time moved to Broomfield from New Hampshire. I visited her several times. Then my uncle and his wife moved to Denver and then Golden. In 2014, one of my best friends, Priscilla, discovered that her masto daughter, who had such frequent severe reactions that she literally could not go outside during the day, didn’t have reactions in Colorado. Pris went back to Texas, packed up her family’s life, and moved to Summit County. It has been three years and her daughter no longer has severe reactions.

Priscilla had not spent much time in Colorado before moving there so there are still a lot of places that I have been in Colorado that she hasn’t. Last time I visited, I took her to Garden of the Gods in Colorado Springs. This time, we went to the Stanley Hotel in Estes Park, high in the mountains above Boulder. The Stanley is beautiful and interesting in its own right but it is most famous because the Overlook Hotel in the Shining is based upon the Stanley Hotel.

I am a huge horror movie fan and like the connection to the Shining. But that’s not the reason I go to Estes Park again and again. I go because Estes Park is the edge of my personal universe. I feel very much that if I don’t watch my step there that I could just fall into oblivion. I don’t know what it is about Estes Park that affects me so much. I don’t know if it’s because it is nestled in tightly among the peaks of formidable mountains or the remoteness or the quiet. Something about standing on the porch of the Stanley Hotel and looking down at the sparse twinkling lights below makes me feel tiny against the awesomeness of the universe. The whistling of emptiness is palpable as it runs down these peaks and into the valleys below. It is both lonely and heartbreakingly beautiful at once, both somewhere I enjoying being and somewhere I fear being abandoned.

The last several weeks have been difficult for me. Fall is always hard and the adrenal crisis really exacerbated things. I have almost no energy and am so tired that it takes a huge amount of effort to make my brain work. I find myself needing more sleep and I have always needed a lot. I am having a lot of pain and am so sore that I haven’t done yoga in a couple of weeks. I am limiting my activity and keeping my stress level down. Fall and early winter are always hard for me and I know I will feel better in January. I’m biding my time and listening to my body to give it what it needs in the meantime.

If I’m honest, I was not in great shape for travelling when I went to Colorado. Traveling has been mentally taxing for a while because getting on a plane with luggage full of IV fluids and meds and syringes is not easy. This is different. I am not recovering well from the physical strain on top of feeling generally poorly when I left. Travel is hard on my body and I can’t deny that as time it goes, it is getting harder. Each trip takes longer to recover from. I spend most of my time in bed when I arrive.

I had to stop traveling for years because of my health. Every trip I take now feels like a gift. It is frightening to imagine that the physical strain of travel may eventually become insurmountable. I won’t lose travel. I can’t lose travel.

I don’t know long I will be able to travel. Hopefully I have years left. But just now it feels like my body might not allow that. It feels like I’m walking along the edge and if I fall, I will never get myself back.

Ruthless

I was discharged from the hospital last night. I was admitted on Monday after going to the ER. I wasn’t having anaphylaxis. I wasn’t sure exactly what was wrong. But I felt disgusting. I had a pounding headache and bad bone pain in both legs and my pelvic bones. I was exhausted. I was so tachycardic that it made me short of breath. My blood pressure was all over the place. I was miserable. Nothing I did was helping. So I went in.

I am adrenally insufficient. I have been for years, since daily high dose prednisone in 2013/2014. My body doesn’t make cortisol so I have to take daily steroids to compensate. When my body is under physical stress, I have to take more steroids to cover the additional need for stress hormones. If I don’t, it can cause a life threatening situation called an Addisonian crisis. I had a crisis in May 2014 that lasted several days. Since then, I am very careful to monitor my body for signs of low cortisol.

In case it’s not obvious where I’m going with this, I was having an Addisonian crisis. My cortisol level was almost undetectable. It didn’t feel like it has in the past and I have no idea what triggered it. I took some extra steroids and stayed a while to see how much it helped. I sat in my hospital bed all night, headphones in, doing work. Work is rapidly becoming the only thing in my life I can control.

I take a lot of medications. I take handfuls of pills every day. I use IV meds and IV fluids every day. I get weekly and monthly injections. The schedule I have to keep in order to accommodate taking all those meds is insane. I basically take medication every thirty minutes while I’m awake. I have to carefully time my meals and anything I drink. I can only exercise at specific times. I can only shower 1-2 hours after night time meds. I have to be very careful with things like public transportation and going to a crowded place in case I get stuck somewhere.

I professionally develop diagnostics to determine which patients will benefit best from clinical trial therapies. It is easier to develop diagnostics than to manage my health from day to day.

People often ask how I am able to travel and work full time. IV Benadryl is not the entire reason I can do those things but it is a huge part of it. I have been using IV Benadryl at home for almost four years. It has kept me out of the hospital on many occasions. It has prevented many reactions from turning into anaphylaxis. It gives me much more control in emergency situations and has kept me safe in many situations that could otherwise have been catastrophic.

Despite how much this drug has helped me, I literally get anxiety thinking about how much IV Benadryl has helped me. The reason for this is that it is a nightmare getting ahold of it. It is not expensive and it is not a controlled substance. Still, getting this is a weekly stressor. I get all my IV meds, infusions, and line care/nursing through a home infusion company. Despite the fact that they provide me with a supply of everything else I need several days before I need them, they will not do this with Benadryl. My doctor has asked. I have asked. My nursing team has asked. I have asked all the people who can be asked. The pharmacy will not do it. Instead, they insist that they deliver me meds on the day that I will be out of them, meaning that if that order does not arrive as expected, I could be out of medication.

I have been a patient there for four years. My nursing care and line care has been amazing. That’s why I have stayed there. I have had a continuously used central line for all that time. I have never had an infection. The reason I have never had an infection is because I adhere to really extraordinary guidelines pertaining to contamination. If I think there is a tiny chance that something is contaminated, I just throw it away and start over. If a needle slips, or I drop something, or accidentally touch a surface with the syringe, I throw it away. This means that I throw away a vial of Benadryl every few days. I have asked the pharmacy to provide me with a few extra days of meds every few months to cover for this situation. They won’t.

The worst part of this whole situation is that my meticulousness, which is regularly commended by my providers, means that I am already working with less medication than I should be. Compounded with this pharmacy’s actions, I have been left without medication on several occasions.

Someone new at this pharmacy recently decided that they would send me my meds a day early. This seemed like a great plan. I was totally onboard. But I was still nervous that it would get messed up. It also didn’t allow for short term changes or short notice deliveries. Earlier this week, when I was in the ER before being admitted, I had to use my own meds for more frequent dosing because they weren’t getting my meds on time. (This was acknowledged and approved by the ER team – I would never do this unless they were aware and onboard.)

The bottom line is that my pharmacy delivery is going to be a day late again after being assured again today that it would arrive today. Because I had to discard two vials over this past weekend for possible contamination and I needed to use extra on Monday (as directed), I am now out of this med. Again.

I had a legit breakdown tonight when I realized that I was going to be out of this med again. Screaming, hysterically crying, the whole thing. This has been such a struggle for so long over something that is really, really stupid to argue about. No one argues that I don’t need the med. I don’t take more than I am directed, ever. No one argues that I should discard anything that might be contaminated and start over. But no one in any position to authorize something as silly as giving me three or four extra vials a week will do so. So I will be up all night hoping I don’t end up having anaphylaxis and going to the hospital if I have serious symptoms that could potentially become anaphylaxis even if they didn’t start that way.

I am very, very tired of this life. I have lots of good days. But as time as gone on, the bad days have gotten much worse. There is no aspect of living with chronic, life threatening health problems that is not stress. I really want to just rip this port out and stop taking IV meds and stop working and stop fighting every single day. I just want to rest. I want it to be quiet. I don’t want to have to explain myself over and over and beg people who just don’t care to help me.

 

There is a ruthless truth to chronic illness, one that has taken me years to come to terms with. It is this: that fighting against my illness and the life it gave me is not a successful way to improve it. I cannot overcome this disease. I can only cooperate with it. I have to learn to live with it, have a relationship with it, greet it every morning and say goodnight when I close my eyes at night. It is a part of me that cannot be cut out or ignored. And to have a life with it, a good one, I have to want that life. I can’t fight for a life I don’t want. My insistence upon having a good life with this disease is not a choice. It is a survival mechanism. It an instinct.

I know that this will pass, but there are some days when I don’t want to do this.

I don’t want to fight anymore. I don’t want to be afraid.

And tonight, I don’t want this life.

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 76

I get asked a lot about how mast cell disease can affect common blood test results. I have broken this question up into several more manageable pieces so I can thoroughly discuss the reasons for this. The next few 107 series posts will cover how mast cell disease can affect red blood cell count; white blood cell count, including the counts of specific types of white blood cells; platelet counts; liver function tests; kidney function tests; electrolytes; clotting tests; and a few miscellaneous tests.

89. How does mast cell disease affect platelet counts?

Before I continue, I want to explain one basic fact. Even though they are often included in the term “blood cells”, platelets are not actually cells. They are actually pieces of an original large cell called a megakaryocyte that lives in the bone marrow. Even though platelets are not really cells, they more or less act like they are.

An unusual thing about platelets is that sometimes a specific trigger can cause platelets to become lower or higher.

There are several ways in which mast cell disease can make platelet counts lower.

  • Swelling of the spleen. This can happen in some forms of systemic mastocytosis, and may also happen in some patients with mast cell activation syndrome, although the reason why it happens in MCAS is not as clear. Swelling of the spleen can damage blood cells and platelets, causing lower platelet counts. If the spleen is very stressed and working much too hard, a condition called hypersplenism, the damage to blood cells and platelets is much more pronounced. This may further lower platelet counts. Hypersplenism occurs in aggressive systemic mastocytosis or mast cell leukemia. It is not a feature of other forms of systemic mastocytosis and I am not aware of any cases as a result of mast cell activation syndrome.
  • Medications. Some medications that are used to manage mast cell disease can cause low red blood cell count. Chemotherapies, including targeted chemotherapies like tyrosine kinase inhibitors, can cause low platelet counts. Non steroidal anti-inflammatory drugs (NSAIDs) are used by some mast cell patients to decrease production of prostaglandins. They can interfere with platelet production in the bone marrow. Proton pump inhibitors, often used by mast cell patients to help with GI symptoms like heart burn, can decrease platelet coun Some H2 antihistamines can also lower platelet production. However, none of these H2 antihistamines are currently used in medicine.
  • Heparin induced thrombocytopenia. Mast cells make and release large amounts of heparin, a powerful blood thinner. When there is an excessive amount of heparin circulating, it can cause your body to incorrectly produce antibodies that cause an immune response to heparin. A side effect of this situation is that platelets are activated incorrectly, which can lead to the formation of blood clots and low platelet counts. Heparin induced thrombocytopenia has only been definitively described in patients who receive medicinal heparin as a blood thinner. However, it is reasonable to assume that this situation can also affect mast cell patients who have higher than normal levels of platelets circulating in the blood.
  • Liver damage. Liver damage is associated with malignant forms of systemic mastocytosis such as aggressive systemic mastocytosis and mast cell leukemia. Liver damage can also occur as the result of IV nutrition, which is sometimes needed by patients with mastocytosis or mast cell activation syndrome. When the liver is damaged enough, it may not make enough of the molecules that tell the bone marrow to make platelets.
  • Excessive production of blood cells. In very aggressive forms of systemic mastocytosis, aggressive systemic mastocytosis or mast cell leukemia, the bone marrow is making huge amounts of mast cells. As a result, the bone marrow makes fewer platelets and cells of other types.
  • Vitamin and mineral deficiencies. Chronic inflammation can affect the way your body absorbs vitamins and minerals through the GI tract, and the way it uses vitamins and minerals that it does absorb. Deficiency of vitamin B12 or folate can decrease platelet production.
  • Excess fluid in the bloodstream (hypervolemia). In this situation, the body doesn’t actually have too few platelets, it just looks like it. If your body loses a lot of fluid to swelling (third spacing) and that fluid is mostly reabsorbed at once, the extra fluid in the bloodstream can make it look like there are too few platelets if they do a blood test. This can also happen if a patient receives a lot of IV fluids.

There are also reasons why mast cell disease can cause the body to make too many platelets.

  • Anemia of chronic inflammation. This is when chronic inflammation in the body affects the way the body absorbs and uses iron. It can result in iron deficiency. Iron deficiency can increase platelet counts.
  • Hemolytic anemia. In hemolytic anemia, the body destroys red blood cells. This can happen for several reasons that may be present in mast cell patients. Hemolytic anemia can increase platelet counts.
  • Iron deficiency. Iron deficiency for any reason can elevate platelet counts.
  • Excessive bleeding. Mast cell disease can cause excessive bleeding in several ways. Mast cells release lots of heparin, a very potent blood thinner that decreases clotting. This makes it easier for the body to bleed. It is not unusual for mast cell patients to have unusual bruising. Bleeding in the GI tract can also occur. Mast cell disease can cause ulceration, fissures, and hemorrhoids, among other things. Mast cell disease can contribute to dysregulation of the menstrual cycle, causing excessive bleeding in this way. It is not unusual for mast cell patients to have GI bleeding, as well as ulceration, fissures, and hemorrhoids.
  • Sustained GI inflammation. Sustained GI inflammatory disease can cause elevated levels of platelets. Given what we know about mast cell driven GI inflammation, it is reasonable to infer that mast cell GI effects and damage may also elevate platelet levels.
  • Clot formation. If a large clot forms, it can affect the amount of platelets circulating in the blood. Some mast cell patients require central lines for regular use of IV therapies or to preserve IV access in the event of an emergency. Blood clots can form on the outside surface of the line, inside the line, or between the line and the wall of the blood vessel it is in.
  • General inflammation. Platelets are activated by a variety of molecules released when the body is inflamed for any reason. This can translate to increased levels of platelet production.
  • Allergic reactions. Platelets can be directly activated by mast cell degranulation through molecules like platelet activating factor (PAF).
  • Heparin. Heparin can cause platelet levels to increase. As I mentioned above, it can also cause platelet levels to decrease.
  • Removal of the spleen. The spleen can become very stressed and work too hard, a condition called This situation is remedied by removing the spleen. Hypersplenism occurs in aggressive systemic mastocytosis or mast cell leukemia. It is not a feature of other forms of systemic mastocytosis and I am not aware of any cases as a result of mast cell activation syndrome.
  • Glucocorticoids. In particular, prednisone is known to increase platelet counts. Prednisone and other glucocorticoids can be used for several reasons in mast cell patients.
  • Third spacing. If a lot of fluid from the bloodstream becomes trapped in tissues (third spacing), there is less fluid in the bloodstream so it makes it look like there are too many cells. As I mentioned above, this is not really a scenario where you are making too many red blood cells, it just looks like that on a blood test.

For additional reading, please visit the following posts:

Anemia of chronic inflammation

Effect of anemia on mast cells

Mast cell disease and the spleen

MCAS: Anemia and deficiencies

Mast cells, heparin and bradykinin: The effects of mast cells on the kinin-kallikrein system

MCAS: Blood, bone marrow and clotting

Third spacing

Gastrointestinal manifestations of SM: Part 1

Gastrointestinal manifestations of SM: Part 2

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 72

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 73