Find a light

I have been writing stories for a long time. I have been keeping journals since I was six and they are full of them. I always wrote about my actual day to day life in journals but never enjoyed autobiographical writing and didn’t think I was good at it. That’s entertaining now to think about, considering that I have spent the last four years digging out the hardest parts of me to share with the internet. But before I was Lisa from MastAttack, I was Lisa Klimas who wrote fantasy stories full of witches and magic that was intended to be shared with no one.

I don’t write fantasy stories anymore. (Except maybe the ones in my head where I fantasize that I’m not living in this body anymore.) I stopped when I started writing MastAttack; I’m not sure why, exactly. But even though I don’t write in this genre anymore, I am still very prone to seeing my life through the lens of fantasy; particularly, I relate aspects of my life to symbolic elements I used to use in stories. This is pretty obvious for anyone who has been reading for a while; I talk about the sky and stars and the vast endless space beyond this world in a lot of posts. Somehow, despite writing very frankly about my life, in my mind this is still a story about someone else, a fantasy story where anything could be real, and where the protagonist could do anything with the right magic.

The past six weeks have been difficult for me in ways that I could never have imagined. I was hospitalized for CDiff in Florida, and then again for CDiff in Boston shortly after I got home. On the last day of my second admission for CDiff, I found out that someone I love very much is very, very sick. The kind of sick that makes me numb to think about. It seemed like things started to level out with my health. I was pretty beat up but the infection seemed to finally be under control, and I was more available to help out with family things. Things seemed more manageable. Until they weren’t.

Last week, I noticed a very minor skin irritation around my port access site. Very minor. The kind I have had many times before just by virtue of having mast cell disease and having fair skin that marks easily. But I’m very meticulous about my port so I called my home IV nurse to come look at it. She came and agreed that it was probably a mast cell reaction or a very minor skin infection. It wasn’t hot, swollen, or painful. The redness was faint and covered a very small area around the needle site. I had no fever, chills, or other signs of infection. She was so confident that it was just a skin irritation that we accessed the port. I called my PCP for some prophylactic cephalexin in case it was a skin infection and breathed a sigh of relief.

I woke up in the middle of the following night with a fever and a much redder and angrier looking port. I took mast cell premeds and went to the hospital immediately. And even the ER nurses and doctors didn’t think it was a serious infection. It still wasn’t swollen, painful, or hot. (For people wondering why I’m harping on this, the four cardinal signs of infection are tumor (swelling), rubor (redness), calor (heat), and dolor (pain).) But they weren’t taking any chances and we spent an hour finding a peripheral vein to use while we sorted out my port. My white count was elevated but it is always is. They told me that they expected I would be admitted, get a few doses of IV antibiotics through the port, and go home in a day or two with the port.

Things didn’t exactly happen like that. Over the course of the day, I got sicker. I started feeling sicker, and I started having more mast cell activation. My white count went from normal for me level to through the roof in a few hours. I was rushed into an interventional radiology suite where everyone still thought that the port was not really infected. They told me after the first incision that things looked good. Then they took out the port and all hell broke loose.

There was an infection underneath my port, beneath the port and chest wall. One that had been brewing for a while. Suddenly everyone was talking in loud voices and debriding the poorly numbed cavity. I should have asked for anesthesia before the procedure but I had never had an IR procedure that required more than fentanyl and midazolam so it didn’t occur to me until they were literally carving pieces of tissue out of my chest. That is not a mistake I will make again.

I was hospitalized for several more days, including a total of three days without a central line. I blew in the neighborhood of ten peripheral lines and it would have been more if I hadn’t had good nurses who babied them to try and make them last. I get IV benadryl six times a day most of the time and that’s part of why I have a central line. IV Benadryl is really damaging to blood vessels and soft tissue, and my blood vessels are already in pretty rough shape. Getting IV Benadryl through a peripheral vein is miserable. It feels like knives shooting up your arm. I used all my tricks and wrapped my arm up in a hot, wet towel for several minutes before every dose, and we diluted it and pushed it slowly and flushed it slowly, and it was still excruciating every single time.

They put in a PICC line last Monday so I could go home and keep up with my home IV meds and fluids. I came home on IV antibiotics every eight hours for the line infection and oral antibiotics every six hours to hopefully prevent me from getting CDiff again from the other antibiotics.

The catheter tip grew out Staph aureus, meaning I had a staph infection a couple of inches from my heart. I got very lucky that I was treated fast enough that it never become a fulminant bloodstream infection but it came pretty close. Another few hours and this story might have had a very different ending. As it is, I suffered a lot for several days, and left the hospital with a hole in my chest so deep that it is nearly to the chest wall, deep enough that I cannot pack it myself because I cannot see the bottom.

Part of the shock of this situation is that I have had a central line for four years and have never had an infection. I am a maniac about my line and sterilizing it and being meticulous not to contaminate anything. I avoid hospitalization like the plague so that I’m the only one touching my line. When my last port was removed, after three years and three weeks, I was told that it was the longest continually accessed port in a BWH patient never to be infected. In total, I had three central lines (a PICC line and two ports) for three years and eleven months before we found any evidence of infection.

I couldn’t figure out how it got infected, and especially how the infection got behind the port. In retrospect, I think might have been contaminated when it was implanted. I don’t know how else I would have seeded a bacterial infection between the port and chest wall, but not over the port at all. I’ve felt sicker and more activated the last few months and it’s possible I had an infection growing all that time. It’s also possible that this isn’t the case but my instincts feel like this is the explanation. Either way, I’m never going to know for sure.

I came home earlier this week and was very relieved to see my family, my dogs, and my bed. I wasn’t feeling super but I just had a line infection so I wasn’t really expecting to.

Yesterday, I started having a GI bleed bad enough that I called an ambulance. While on the phone with 911, I started having anaphylaxis because that’s just how we do now. I laid down and used an epipen while feeling pretty dejected that I was now going to be admitted to the hospital for the fourth time in six weeks. I have been admitted for a total of 17 days this year. We are only 49 days into 2018.

Last night, I sat in my isolation room and watched it snow from the tenth floor. It was the middle of the night and the world was silent as snow covered every darkness and imperfection below. Not the terrible storm it was rumored to be. Just snow. Snow that is already melting under a clear blue sky and a brilliant and victorious sun. Light always wins.

I know that I am not really the protagonist of a fantasy story and that I’m not really going to slay the dragon that is my poor health. My health is bad. I have largely made peace with that. I want it to be better but I don’t expect it to be. But I also know that for every evil there is a pure good, and that bad things happen to me but the worst things don’t, and that I won’t always feel this way.

I don’t know how I will get through this or how long it will take or what I will lose along the way. I just know that the snow has fled under a brilliant sun and that somewhere, somehow, I will find a light.

Cut out the broken pieces

I was hospitalized for almost a third of January. In the first hours of 2018, I was put into an ambulance and brought to a hospital where I know no one a thousand miles from home. I arrived with a high fever, continuous, forceful vomiting, and colitis symptoms from hell. A couple of days later, I tested positive for CDiff, a severe colon infection that is dangerous and difficult to treat.

Metronidazole calmed the CDiff enough for me to be discharged. I stayed in Florida several more days, unable to get home because of the storm. I was home only a few days before I was once again brought to a hospital by ambulance with severe colitis. The pain was literally blinding. My vision flashed white at the worst points, lightning coming in waves.

I live with serious chronic pain and almost certainly will for the rest of my life. I am not a stranger to pain. This is the worst pain I have ever experienced. I am not a screamer with pain. I was screaming.

The CDiff was no longer being controlled by metronidazole. I was admitted and we started oral vancomycin four times a day.

I have been sick a long time. I think this was the sickest I have ever been.

I also had some strong emotions to contend with, namely that I was angry. This isn’t the first time I’ve had a major problem with my colon. It has been a consistent problem for years and is usually the reason I end up in the hospital. I have had multiple colon surgeries and so many scopes and biopsies that I sincerely can’t remember how many I have had. Lower GI bleeding is part of my baseline. I have to undertake extraordinary interventions to get stool out of my colon. My colon does not work. It performs none of its intended functions.

When I had my last colon surgery, I begged my doctors to take out the whole thing. I begged them because I knew it would never stop being a problem and would never stop sending me to the hospital periodically. They wouldn’t do it. They took some of my colon but they didn’t take all of it and look where we are now. We’re here, in a place that would be impossible to get to but for the fact that my damaged, bleeding, useless colon is still inside my body. A place we could not have gotten to if they had just cut out this broken piece of me. But they didn’t.

The oral vancomycin worked well for me. I medicated heavily to suppress mast cell reactions to the med, which is a known degranulator. After a few days, my symptoms improved significantly, but my pain was still bad. It took another few days to be able to eat anything and to manage my pain without IV meds.

By the end of my hospital stay, I was very ready to go home. I was cautiously optimistic that the oral vancomycin would work well for me and that the worst was behind me. I packed up all my stuff and filled all my prescriptions and waited in my isolation room for my uncle to arrive to pick me up.

A couple of hours before my uncle arrived, I got a phone call. I was walking around the floor and was in a hallway quiet enough that I could hear a conversation on the phone. I had this weird moment of foreboding when I answered the phone. I know it sounds cliché and theatrical but I really did. I sat down in a chair near the elevator bay and answered the phone and my entire life exploded.

As I sat there talking, something inside of me broke. The pain was immediate and crushing. I couldn’t breathe. I couldn’t stand up. I couldn’t think. My entire life changed and it will never change back. The life I had was gone in a moment, ripped away by a dark, whipping current. I blame this broken piece because it started there. Another broken piece that can never be fixed, that will poison me every day of my life. Another broken piece that can never be removed.

There are some realities too horrible to imagine. When you try, your mind just shows the future the way your life is now. Your mind cannot envision a life without certain people because it is so horrible that surely such a life cannot exist.

I have so many things to say but my soul is too tired and worn to speak them. The effort required is too great. Instead I swallow them down. They collect behind my stomach, cleaving to my spine, growing larger and larger with each truth I cannot get out. A broken piece of my own making that can never be cut out.

Locking down the blog

Hey, everyone,

Tomorrow, I am setting a bunch of technical posts to private for a few days to reorganize some things and take a break for a few days. I suggest that you print or screencap any posts that you need in the next few days tonight. All of the information that has been here will be here again within the week. Some of it may be consolidated with other posts.

I have made the decision to remove last night’s post and to replace it completely with a different post. I anticipate this new post will be up next week. All language on that topic has to be carefully reviewed because of my job and this takes extra time.

I am having a deeply trying time right now. My health has not been good and I am struggling with some very difficult and upsetting news in my personal life. I would appreciate any good energy, prayers or love you can send my way. Thanks for your support.

Xoxo,

Lisa

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 83

96. Why are cancer drugs used to treat mast cell disease?

Disclaimer: The following post was written by me in my capacity as a subject matter expert in mast cell disease and author of MastAttack. This is not work product of my position as a Senior Scientist for a large research organization. All below statements are attributable directly to me in my role as author of MastAttack and are in no way attributable to my employer. Information presented here is publicly available and includes no confidential information learned in my capacity as a Senior Scientist for my employer.

  • There are a number of medications used to treat cancers that are also used to treat mast cell disease. Some of those medications are old school chemotherapies, some are newer, targeted chemotherapies, and some help to control the immune system.
  • In mastocytosis, the body makes too many mast cells. If the bone marrow makes way, way too many mast cells, and those mast cells don’t function correctly, the mast cells can act like cancer cells. This can cause the mastocytosis to behave like cancer.
  • Systemic mastocytosis has several subtypes. The least serious forms do not act like cancer.
  • Indolent systemic mastocytosis (ISM) is the least severe form of systemic mastocytosis. ISM has a normal lifespan. While patients with ISM are at risk of dying for anaphylaxis, an important distinction is that patients with ISM do not die because the mast cell disease acts like a cancer. ISM does not act like cancer.
  • Smoldering systemic mastocytosis (SSM) is a moderately serious form of systemic mastocytosis. SSM can shorten lifespan. In SSM, the body is starting to make lots more mast cells than it should. Those mast cells can affect how organs function. SSM acts like an early cancer.
  • SSM requires treatment to stop it from becoming a more serious form of mastocytosis called aggressive systemic mastocytosis (ASM) that acts like a serious cancer. The treatments used to manage SSM are also used in some cancer patients to help fight cancer. These include meds that affect your immune system, like interferon; newer targeted therapies and chemos, like tyrosine kinase inhibitors; and older chemo drugs, like cladribine.
  • Aggressive systemic mastocytosis (ASM) is a serious form of systemic mastocytosis. ASM shortens lifespan significantly. In ASM, the body makes way too many mast cells. The bone marrow churns out so many mast cells into the bloodstream and then the abnormal mast cells get stuffed into various organs. The mast cells cause organ damage and can cause organ failure. ASM is often referred to as being malignant because it behaves just like a cancer. It is also treated like a cancer.
  • As mentioned above, interferon is a therapy that can affect how the immune system works. Interferon is sometimes used for ASM but it is less commonly used in ASM than in SSM. ASM patients need more aggressive treatment. Newer targeted therapies like tyrosine kinase inhibitors and multitarget kinase inhibitors are frequently used in ASM. Some of these newer therapies are FDA approved for treating some ASM patients. Cladribine and hydroxyurea are still common treatments for ASM.
  • Mast cell leukemia (MCL) is the most serious form of systemic mastocytosis. MCL greatly reduces lifespan. MCL causes production of an unbelievable number of mast cells. There are so many mast cells that they cannot all get stuffed into organs like ASM. This means that while there are lots of mast cells in the organs in MCL patients, there are so many mast cells like that there are still tons of them in the bloodstream. This leads to rapid organ failure, leading to death. Mast cell leukemia is cancer. It is treated like cancer with newer therapies like tyrosine kinase inhibitors and multitarget kinase inhibitors, as well as hydroxyurea or cladribine in some cases. As in ASM, some of the newer therapies are FDA approved to treat mast cell leukemia.
  • Sometimes patients with systemic mastocytosis develop a second blood disorder. This is called systemic mastocytosis with associated hematologic disease. Sometimes this second blood disorder is a form of cancer, like chronic myeloid leukemia. In these instances, the other blood disorder would be treated using cancer medications.
  • Mast cell sarcoma (MCS) is a cancerous form of systemic mastocytosis. Patients with MCS rapidly develop MCL and are treated as described above.
  • None of the therapies I mentioned here are indicated for cutaneous mastocytosis. Cutaneous mastocytosis does not behave like a cancer and is not treated like one.
  • In recent years, two other forms of mast cell disease have been described: mast cell activation syndrome and monoclonal mast cell activation syndrome.
  • Monoclonal mast cell activation syndrome (MMAS) is often considered to be a “pre-SM”. It is treated like indolent systemic mastocytosis and does not behave like a cancer.
  • Mast cell activation syndrome (MCAS) is not know to be an early form of SM. Many people live with MCAS for decades without ever developing SM.
  • Despite the fact that mast cell activation syndrome, monoclonal mast cell activation syndrome, and indolent systemic mastocytosis do not behave like cancer, cancer therapies are sometimes used in these patients. They are used when other therapies have failed and their symptoms are still poorly controlled. Generally, they are used when persistent mast cell activation becomes life threatening. In some instances, they may be used when a patient’s symptoms are not life threatening but are very disabling and cause a poor quality of life. In these cases, the patient and their provider make the assessment that they are able to assume the risk of using these medications.
  • There is very little data on the use of chemo and targeted therapies in patients with MCAS, MMAS and ISM, and no cancer therapies are FDA approved for these conditions. However, use of cancer meds for nonmalignant conditions is not that unusual. It is pretty common in autoimmune disease where lower doses of chemotherapy drugs can be effective in controlling the disease. Basically, the idea is that if we know that these therapies help forms of mast cell disease that behave like cancers then it might help those forms that don’t act like cancer.
  • On a number of occasions, I have seen patients discussing the dangers around certain cancer meds that are sometimes used to treat mast cell disease. In particular, I have seen comments that newer targeted therapies “do not kill cells”, “cannot cause organ damage”, and are “harmless.” This is completely untrue. There are thousands of articles on the side effects and complications of all of the meds I have described here. None of them are harmless. Patients need to understand the risks associated with these therapies.
  • I would like to add a note about something sort of related. Xolair is an anti-IgE medication that is used by many mast cell patients. It is a subcutaneous injection and is administered in a healthcare setting. Patients are required to stay in the office for a little while after the shots are given to be sure that they don’t have a bad reaction. Because the patient is monitored in the office after the shot, the provider’s office will bill insurance for the observation period. The old billing code for this often comes up as “chemotherapy observation” because the same code was used for patients who needed monitoring after chemo. This means that patients may see “chemo” on the explanation of benefits from their insurance company. This does not mean that they received chemo. Xolair is NOT chemotherapy. It’s just a quirk of the medical billing. There is now a new code for post injection observation for meds that are not chemo but not everyone has caught up to it. Just figured I would mention this as people ask about it from time to time.

Announcement: Super T’s Mast Cell Foundation

Hey friends, family, colleagues, and MastAttackers,

It is my honor to announce that I will be a member of the Patient Advisory Council for Super T’s Mast Cell Foundation, an organization that provides support to mast cell patients in need. STMCF is the dream of Taylor Nearon made real and is being carried on by her friends and family following her death from MCAS at the age of 20. I cannot wait to help further her vision. Please visit the Facebook page for STMCF and like/follow to share in our story.

My participation in STMCF will occur alongside my continued work as author and founder at MastAttack.

Thanks so much for your support!

Xoxo,
Lisa

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)