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mast cell disease

Symptoms of mast cell disease

The following is a generalized list of common symptoms associated with mast cell disease. It is not comprehensive and does not include laboratory or associated diagnoses.

General: fatigue, malaise (“being out of it”), weakness, severe unprovoked sweating, weight gain or loss

Skin: Rashes and lesions of any kind, itching, flushing, angioedema, stretch marks, dermatographism, poor wound healing, alopecia, abnormalities of finger or toenails

Eyes: Irritated eyes, red eyes, excessive tearing, dry eyes, difficulty focusing vision, lid tremor, sensitivity to lightness, including sunlight, general inflammation

Ears: Inflammation of the ear, “ear infections,” hearing loss, sensitivity to sound, ringing in the ears

Mouth/throat/sinuses: Generalized pain of several qualities, but often burning, ulceration, “canker” sores, swelling (angioedema), dental decay, abnormalities in taste, taste of metal, throat discomfort or irritation, need to clear throat frequently, post nasal drip, nose bleeds, irritation of sinuses, sinus congestion

Respiratory: laryngitis, bronchitis, pneumonitis (frequently confused with pneumonia), recurrent cough (usually dry), shortness of breath, wheezing

Cardiovascular: lightheadedness, weakness, dizziness, vertigo, fainting, high or low blood pressure, palpitations, rapid heartbeat, abnormalities in heart rhythm, chest pain, hemorrhoids, edema

Gastrointestinal: abdominal pain, diarrhea, constipation, difficulty swallowing, swelling of any part of the GI tract, nausea

Neurologic: Headache, migraine, “about to faint,” fainting, numbness, pins and needles, neuropathy, tics, tremors, seizures

Psychiatric: Anger, depression, PTSD, anxiety, memory difficulties, anxiety, panic disorders, insomnia, sleep disorders

The hallmark of mast cell disease is allergic-type reaction to a variety of stimuli. These can also occur to substances benign to most people, including scents, “hypoallergenic” materials, heat, sunlight and water. Some people experience anaphylactic reactions that require epinephrine.

References:

Afrin, Lawrence B. Presentation, diagnosis and management of mast cell activation syndrome. 2013. Mast cells.

The other kind of hope

I am an optimistic person. My optimism borders on religious; when I despair, it is all that I have. I am good at finding silver linings, at genuinely feeling fortunate or lucky or grateful for the little upturns of bad situations. I enjoy talking to my father when he has to drive me to work. I like snuggling with Astoria when I have to spend the day in bed. I am grateful for my awesome friends, family and coworkers who help me out. All of these things happen because I am sick, manifestations of the impact my illness has on my life. They give me hope that I can keep doing this.

But there is this other kind of hope, more insidious and malignant. I woke up this morning on my own after sleeping for nine hours. This is the second night in a row I have done this. I felt okay when I woke up. Some bone pain, but overall, better than normal. And then it happened, that dangerous optimism – maybe I’m getting better. Maybe this is when I start to get better.

It never is. I know logically that two days of good sleep doesn’t mean I’m headed for a remission. I wish I didn’t feel these things so intensely. But I do.

Even after all this time, I still can’t believe that I will never get better. I can know it in my mind, but my heart just won’t accept that this is anything but temporary. This hope for impermanence can be so painful.

Sometimes I wish I weren’t so hopeful. It is just so hard to live with the perpetual disappointment.

Lesser known mast cell mediators (Part 3)

Substance P is a neurotransmitter and modulates neurologic responses. It is found in many sensory nerves as well as the brain and spinal cord. It participates in inflammatory responses and is important in pain perception. It is involved in mood disorders, anxiety, stress, nerve growth, respiration, neurotoxicity, nausea, vomiting and pain perception. Its release from nerve fibers into the skin, muscle and joints is thought to cause neurogenic inflammation.

Urocortin is related to corticotropin releasing factor (CRF.) It strongly suppresses blood pressure and increases coronary blood flow. It is thought to have a role in increasing appetite during times of stress.

VEGF-A (vascular endothelial growth factor A) is a member of the platelet derived growth factor (PDGF)/VEGF family. It is important in nerve biology and is the substance mainly involved in inducing growth of blood vessels. It is heavily involved in diseases that involve blood vessels, like diabetic retinopathy and macular degeneration. It is a vasodilator and increases permeability of the smaller vessels.

VIP (vasoactive intestinal peptide) is a small protein like molecule used by nerve cells for communication. It stimulates heart contraction, vasodilation, lowers blood pressure, and relaxes the smooth muscles of the trachea, stomach and gall bladder. It also inhibits gastric acid secretion and absorption in the intestine.

Mast cell kininogenase removes a portion of a compound to release active bradykinin. This is important in the kinin system.

Phospholipase A2 promotes inflammation by initiating formation of arachidonic acid, the precursor needed to form many inflammatory molecules, including prostaglandins. Excessive levels of phospholipase A2 can lead to increased vascular inflammation, such as a seen in coronary artery disease and acute coronary syndrome. Elevated PLA2 is found in the cerebrospinal fluid of people with Alzheimer’s disease and multiple sclerosis.

Corticotropin releasing hormone (CRH) is a hormone and neurotransmitter. High CRH levels have been associated with Alzheimer’s disease and severe depression. CRH is produced in the hypothalamus and is carried to the pituitary gland, where it stimulates secretion of adrenocorticotropic hormone (ACTH.) ACTH drives synthesis of cortisol and other steroids. Imbalance of these hormones can have dire consequences.

Endothelin is the most potent vasoconstrictor currently described. It raises blood pressure and if uncontrolled, hypertension may result. It is involved in many disease processes, including cardiac hypertrophy, type II diabetes and Hirschsprung disease.

Chondroitin is found largely in connective tissues and is a principal component of cartilage. It is typically bound to other components when released from mast cells and interacts with a variety of molecules.

Hyaluronic acid is widely found in epithelial, neural and connective tissues. It participates in a variety of reactions and sees significant turnover daily. When hyaluronic acid is degraded as part of the turnover, its degradation products can cause inflammatory responses.

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

The kinin-kallikrein system is a hormonal system with effects on inflammation, blood pressure, coagulation and pain perception. This system is known to have a significant role on the cardiovascular system, including cardiac failure, ischemia and left ventricular hypertrophy. Despite significant research, it is not entirely understood.

Kininogens are proteins that have extra pieces on them. Kininogenases cut off those extra pieces. Active kinins that can act on the body are the result of this action. So kininogenases change kininogens to form kinins.

There are two types of kininogens: low molecular weight (smaller) and high molecular weight (larger.) We are going to focus on HMW, which circulates in the blood.

Also circulating in the blood are two other components called prekallikrein (sometimes called Fletcher factor) and Hageman factor (Factor XII.) When Hageman factor lands on a negatively charged surface, it changes shape and becomes Factor XIIa. Factor XIIa changes the prekallikrein to kallikrein. Kallikrein is a kininogenase.

When kallikrein finds a kininogen, it cuts off the extra piece to release bradykinin. Bradykinin is a kinin and is ready to act on the body.

Bradykinin has several functions in the body. It contributes to contractility of duodenum, ileum and cecum. In the lungs, it can cause chloride secretion and bronchoconstriction. It can cause smooth muscle contraction in the uterus, bladder and vas deferens. It contributes to rheumatoid arthritis, inflammation, pain sensation and hyperalgesia. It also induces cell proliferation, collagen synthesis, and release of nitric oxide, prostacyclin, TNF-a and interleukins. It can also cause release of glutamate by nerve cells. Glutamate has a variety of actions in the body and excessive release can cause epileptic seizures, ALS, lathyrism, autism and stroke.

Bradykinin acts on the endothelium, the cells that line the inner surface of blood and lymphatic vessels, to cause the blood vessels to dilate. This decreases blood pressure. It also regulates sodium excretion from the kidneys, which can further decrease blood pressure. Kininogen levels are reduced in hypertensive patients. Kinins, including bradykinin, oppose the action of angiotensin II, a hypertensive agent.

So how are mast cells related to this system? A couple of ways. The first way is that they release kininogenases and bradykinin. Tryptase can actually behave as a kininogenase. The second way is by being the exclusive producers of heparin.

As I mentioned above, Factor XII needs to change to Factor XIIa to initiate the formation of bradykinin. It does this when it contacts a negatively charged surface. In the lab, you can use a surface like glass for this. But in the body, it often happens on the surfaces of large, negatively charged proteins like heparin. (Side note: Factor XII is part of the clotting cascade. It can be activated by medical devices like PICC lines and that is why they carry a risk of clot formation.) So by releasing heparin, mast cells cause the formation of bradykinin. When the mast cells release heparin in inappropriate amounts, too much bradykinin is formed.

Overproduction of bradykinin is one of the principal causes of angioedema. In hereditary angioedema, the body is deficient in a component that regulates bradykinin. One of the reasons that physical trauma can cause mast cell degranulation is because it causes formation of bradykinin. Bradykinin in turn causes mast cell degranulation with release of histamine and serotonin, among other contents.

Bradykinin antagonists are being researched as possible therapies for hereditary angioedema. Icatibant is one such medication. Bromelain, found in the stems and leaves of pineapples, are known to suppress swelling caused by bradykinin. Aloe and polyphenols, like those in green tea, are also known to suppress bradykinin activity.

References:

Kaplan AP, Ghebrehiwet B. The plasma bradykinin-forming pathways and its interrelationships with complement. Mol Immunol. 2010 Aug; 47(13):2161-9

Oschatz C, et al. Mast cells increase vascular permeability by heparin-initiated bradykinin formation in vivo. Immunity. 2011 Feb 25; 34(2):258-68.

 

Brunnée T, et al. Mast cell derived heparin activates the contact system: a link to kinin generation in allergic reactions. Clin Exp Allergy. 1997 Jun;27(6):653-63.

 

 

Lesser known mast cell mediators (Part 2)

Arylsulfatase A, also called cerebroside sulfatase, breaks down compounds to yield cerebrosides and sulfates. Cerebrosides can be either galactocerebrosides, which are found in all tissues of the nervous system; or glucocerebrosides, which are found in the skin, spleen, red blood cells and, to a lesser extent, tissues of the nervous system.

Arylsulfatase B, which has several other names, breaks down large sugar compounds, especially dermatan sulfate and chondroitin sulfate. Arylsulfatase B is mostly found in the liver, pancreas and kidneys.

Mutations in the gene for either arylsulfatase can lead to a variety of heritable disorders, including mucopolysaccharidosis VI and metachromatic leukodystrophy.

Chymases include mast cell protease 1, mast cell serine proteinase, skeletal muscle protease and so on. They are found almost exclusively in mast cells, but are present in small amounts in the granules of basophils. They have several functions, including generating an inflammatory response to parasites. They convert angiotension I to angiotensin II and therefore impact hypertension and atherosclerosis.

Bradykinin causes dilation of blood vessels, which induces a corresponding drop in blood pressure. It achieves its action by triggering release of prostacyclin, nitric oxide and endothelium derived hyperpolarizing factor. It also causes contraction of non-vascular smooth muscles in the respiratory and GI tracts, and is involved in the way the body senses pain. Bradykinin is important in angioedema.

Angiogenin, also called ribonuclease 5, stimulates the formation of new blood vessels. It drives the degradation of the basement membrane and local matrix so that endothelial cells can move toward the vascular spaces.

Leptin is the hormone that regulates hunger. It is mostly produced by fat cells, but is released by mast cells as well. When a specific amount of fat is stored in the body, leptin is secreted and tells the brain that it is full. It opposes the action of ghrelin, the hormone that tells your body it is hungry.

Renin, also called angiotensinogenase, is a critical component of the renin-angiotension system (RAS) that controls the volume of fluids not in cells, including blood plasma, lymph and interstitial fluid. It regulates the body’s mean arterial blood pressure. It converts angiotensinogen to angiotensin I.

Somatostatin, also growth hormone inhibiting hormone (GHIH), regulates the endocrine system, transmission of neurologic signals and cell growth by acting on somatostatin receptors and inhibiting the release of various secondary hormones. It inhibits secretion of glucagon and insulin. It is secreted throughout the GI system and decreases stomach acid production by downregulating the release of gastrin, secretin and histamine.

Lesser known mast cell mediators (Part 1)

I have posted at length about the roles of histamine and serotonin. Here are some less well known mast cell mediators. I will be doing in depth posts on the more relevant substances in the near future.

Monocyte chemotactic protein 1 (MCP-1), also known as chemokine ligand 2 (CCL2), draws other white blood cells, including memory T cells, monocytes and dendritic cells, to the site of injury or infection. It has important functions in neuroinflammation as seen in experimental autoimmune encephalitis, traumatic brain injuries, epilepsy and Alzheimer’s disease; and in diseases with pathologic infiltration of monocytes, like rheumatoid arthritis.

Chemokine ligand 3 (CCP7) recruits monocytes and regulates macrophage activity. It is known to interact with MMP2.

MMP2 (matrix metalloproteinase 2) is involved in tissue remodeling, reproduction and fetal development. It degrades type IV collagen. It has regulatory effects on the menstrual cycle and has been tied to growth of new blood vessels.

Interleukin 8 (IL-8), also known as neutrophil chemotactic factor (NCF), draws other white cells, mostly neutrophils, to a site of infection. It can activate multiple cells types, including mast cells, and promotes degranulation. It has been linked to bronchiolitis, psoriasis and inflammation.

MCP-4 (CCL13) attracts T lymphocytes, eosinophils, monocytes and basophils to an area of inflammation. Improper regulation can exacerbate asthma symptoms. Mast cells can release MCP-1 when stimulated by TNF-a and IL-1.

CCL5 (RANTES) attracts T cells, eosinophils and basophils. When IL-2 and interferon-γ are present, CCL5 activates natural killer cells and causes proliferation of the same. It is also important in bone metabolism.

CCL11 (eotaxin-1) specifically recruits eosinophils and is heavily involved in allergic inflammatory responses.

CPA3 (carboxypeptidase A3) digests proteins. It is released complexed with heparin proteoglycan along with chymase and tryptase.

Both interferon α (IFN- α) and interferon β (IFN-β) are made in response to viral infections. Their activities are regulated by IFN- γ. IFN- γ also draws white cells to the site of inflammation. Failure to properly regulate interferon levels can cause autoimmune disease. Interferons are so called because of their ability to “interfere” with viral infection. They are responsible for “flu type symptoms,” such as fever, muscle aches and lethargy.

All mediators listed here are produced by mast cells and stored in granules until degranulation.

 

Treatment of anaphylaxis

Treatment of Anaphylaxis: ABC

Remember the mnemonic ABC.

A: Adrenalin (epinephrine)

Epinephrine is the recommended drug for treating anaphylaxis. It works by stimulating alpha- and beta-adrenergic receptors to inhibit mediator release by both mast cells and basophils. Use of epinephrine at onset of symptoms inhibits the release of PAF, which is largely response for the life-threatening manifestations of anaphylaxis.

B: Benadryl (diphenhydramine)

Antihistamines will NOT stop anaphylaxis. They help to manage the symptoms experienced subsequent to the reaction.

C: Corticosteroids (hydrocortisone, prednisone, etc)

Corticosteroids will NOT stop anaphylaxis. It can decrease risk of biphasic or protracted anaphylaxis.

 

Standard treatment for anaphylaxis

  • Epinephrine
  • Airway maintenance.
  • Oxygen, 6-8L/min.
  • IV hydration. 25-50 ml/kg of lactated Ringer’s solution or normal saline.

 

Treatment of anaphylaxis in mast cell patients

  • 0.3ml of 1:1000 diluted epinephrine, repeated 3x at five minute intervals if BP is less than 90 systolic (0.1ml for children under 12)
  • Diphenhydramine 25-50mg (12.5-25mg for children under 12) orally, intramuscularly or intravenously (slow push) every 2-4 hours; or hydroxyzine 25mg (12.5mg for children ages 2-12) orally every 2-4 hours
  • Methylprednisolone 120mg (40mg for children under twelve), intramuscularly or intravenously
  • 100% oxygen by mask or nasal cannula
  • Nebulized albuterol

Reference:

Emergency Room Response Plan. The Mastocytosis Society. Recommended by Dr. Mariana Castells.

 

Other treatment options

  • Diphenhydramine 50mg or more in divided doses, oral or IV. Maximum dose is reported as 300mg (5mg/kg) for kids and 400mg for adults (under supervision.)
  • Ranitidine 50mg in adults, 12.5-50 mg (1mg/kg for kids), administered by IV as 5% solution, total of 20ml, over five minutes.
  • Albuterol 2.5-5 mg nebulized in 3ml normal saline, or levalbuterol 0.63-1.25 mg nebulized in 3ml normal saline as needed.
  • Aminophylline, IV loading dose 5-6 mg/kg over 20 minutes, followed by IV infusion, 0.5-0.9 mg/kg/hr. Useful for persistent bronchospasm.
  • For persistently low blood pressure, consider dopamine 400mg in 500ml, intravenously at dose of 2-20 mcg/kg/min.
  • Glucagon 1-5mg (20-30mcg/kg, max of 1mg for kids) intravenously over five minutes, followed by IV infusion of 5-15 mcg/min.
  • Methylprednisolone 1-2 mg/kg/24 hours.
  • Sodium bicarbonate, 0.5-1 mEq/kg every five minutes as determined by arterial blood gases. Useful for persistent low blood pressure or acidemia.
  • Methoxamine 10mg has been reported as working following failure of epinephrine. Has been suggested as a next-line medication following failure of second dose of epinephrine; has not seen much use.

References:

Higgins DJ and P Gayatri. Methoxamine in the management of severe anaphylaxis. Anesthesia 1999: 54(11), 1126.

Neugut et al. Arch Int Med 2001

Yocum et al. J Allergy Clin Immunol 1999

Sampson H. N Engl J Med 2002

Sampson et al. J Allergy Clin Immunol 2006

Sheikh et al. BMJ 2006

Kemp SF and Lockey JACI 2002; 110:341-8

MCAS: Respiratory symptoms

Pharyngeal (throat) symptoms are quite common in MCAS and as usual, highly variable. Burning, painful, and irritated throats are frequently reported, and often automatically treated as viral infection or Strep throat, with no culture or negative culture. This pain can be chronic or intermittent. A chronic tickle in the throat or need to clear the throat is often present. Sinus congestion can lead to postnasal drip.

Sometimes MCAS reactions are localized to the throat, inhibiting ability to swallow or sometimes even breathe. This symptom is due to angioedema, and if breathing difficulty is observed, emergency treatment with epinephrine is required.

The most frequently noted lower respiratory symptom is a low level difficulty of breathing. This often presents as occasional wheezing, or feeling like you can’t get a deep breath. Patients rarely have severe wheezing, though it does sometimes happen. Chest x-ray and pulmonary function testing are usually normal.

Chronic non-productive cough affects a small portion of MCAS patients. They are often diagnosed with reactive airway disease for lack of a better explanation. Prostaglandin D2 is a potent bronchoconstrictor, approximately 10 times more potent than histamine, and is responsible in part for respiratory symptoms.

Mast cells have been implicated a variety of pulmonary pathologies, including COPD and pulmonary hypertension. Allergic asthma is not uncommon in MCAS patients and this population often reports successful treatment with Xolair.

References:

Afrin, Lawrence B. Presentation, diagnosis and management of mast cell activation syndrome. 2013. Mast cells.

Anand P, et al. Mast cells: an expanding pathophysiological role from allergy to other disorders. Naunyn-Schiedeberg’s Arch. Pharmacol. 2012 May.

 

Dignity

I have always been socially awkward. For most of my life, there has seemed to be this set of universally applicable rules that I don’t understand. In particular, I find that I am generally neither embarrassed by not impressed with a variety of circumstances that seem extraordinary to the general population. I am not the kind of person who is upset by immodesty or esoteric ideas or the rending of social rules. I never have been. I don’t know why.

The day before my colostomy surgery, I was lying on my couch, trying to take a nap. My phone rang and it was a woman from an ostomy group. I remembered vaguely telling my ostomy nurse that it was okay to be contacted by this support group. I always say yes to stuff like that because I figure it can’t hurt.

The woman on the other end was very sweet. She asked if I was upset. I told her I wasn’t. She was gentle, but she persisted. She didn’t believe me.

“No, I really just want to be able to shit,” I told her, laying my arm over my eyes to block out the sun. “Honestly, I’m a lot more afraid of the damage from my mast cell disease if I don’t get this out than I am of shitting into a bag.” She was quiet for a while. “What?” I asked.

“Nothing, it’s just….You really aren’t upset,” she said finally.

“No, I’m really not,” I answered. And I wasn’t.

She offered me her many good tips on how to hide your ostomy bag. She clearly cared a lot (and felt badly – I was the youngest person her group had worked with) so I listened. She talked about girdles and pregnancy bands and tank tops and belt things with pouches and bathing suits. It was interesting, in a sort of voyeuristic way. I thought about her when I went swimming a couple of months later. My preparation consisted of putting on my normal two piece bathing suit and going in the pool. I didn’t see any reason to hide it. I wasn’t ashamed of it.

When I found out I was getting a PICC line, several people asked me how I would cover it. “With an occlusive dressing,” I answered automatically every time.

“No, like so people won’t be uncomfortable,” they sometimes followed up.

“It’s not my problem if they’re uncomfortable,” I said with increasing irritation every time I responded. “It’s not my fault I need the line.”

Someone asked me how I covered my port a few days after I got it. “I don’t.,” I said, thoroughly tired of this line of questioning. “It’s just not who I am.”

Modifying my body doesn’t upset me, even in the ways I just described. My hair has been in turns purple, blue, red, pink and red again. I had numerous piercings, all over my body, until once, after a surgery, when I just didn’t want to put the jewelry back in. It didn’t feel right anymore.

I am sure to many people, it must seem like my disease doesn’t upset me. That’s not the case. My relative resignation about the state of my illness is a survival mechanism. It looks a lot like not caring sometimes, but that’s not what it is. The pain shows, but it’s harder to see if you’re not watching carefully. It shows in the moments when I am unable to find even a shred of dignity.

Monday morning, I started anaphylaxing at work. I had a little bit of a cold and was overtired and sore and generally sort of walking the line anyway. I walked up to the nurse’s office and gave myself IV meds while she took my blood pressure. “It’s 88/60,” she told me with wide eyes. I didn’t want to epi at work because then they would have to call an ambulance. I knew the IV Benadryl and steroids would keep me stable for a bit so I called my mother. At the age of 30, I called my mother to come get me at work because I was too sick to get home safely. The nurse emailed my boss to tell him I went home sick, which was for some reason a lot more mortifying than it should have been. My father and cousin had to go pick up my car later. It was embarrassing.  It felt undignified.

Then, later on that night, my dog threw up. I completely lost it. I throw up most days, often multiple times. If it doesn’t all get into its intended receptacle, I just clean it up. No big deal. But these last few weeks have been grosser than usual as pertains to my GI tract and I am so sick of cleaning up shit. I couldn’t deal with it. I just hit a limit of grossness and indignity in my life sometimes and it’s like I can feel my self worth just leeching away from my soul into the air around me.  It’s a perception thing and it’s so individual.  I know how strange it is to some people that I don’t mind being transparent about my life, the external signs of my internal illness visible to the world, but sometimes am upset about needing help.  I know people think it’s strange.  But it’s not my problem, and it’s not my responsibility to behave in a way that makes people comfortable with my illness.

Someone posted this week about a “friend” who was upset that they joked about their illness. My response was something like, “That guy’s an asshat, it’s not your job to act how he thinks sick people should act.” And it’s not. I don’t keep my central lines uncovered because it makes some statement about illness. I keep them uncovered because I genuinely can’t be bothered. It’s just not who I am to care.

But I care about my disease, and I care about the life I can’t have because of it, and sometimes, it’s too much. Some days I am tired of cleaning up vomit and emptying bags of my own waste and cleaning blood stains out of my clothes. Do not mistake my resoluteness for apathy.

This ability to move forward, to not be upset about mundane things – it is a learned skill, not a natural acceptance of the terrible reality of chronic disease. We are just trying to find grace in life. We are just hoping to navigate through the rough pockets with some autonomy and a little bit of dignity.

 

Artifact

I have spent a lot of my professional life using microscopes. There is this rhythm you get into, when you do it a lot; lifting the edge of the slide out of the book with a gloved fingernail, pulling back the guard to slide the glass into place, spinning the fine adjustment back and forth with your fingertips. Sitting taller to look through the eyepiece into the tiny world below. Looking closely to see the ways you may have changed it.

The process of putting a sample on a slide and staining it can change it, sometimes even if you’re careful. Things look desiccated if you dry them too fast; elongated and distorted if you compress the sample. The dye sits heavy in some places and doesn’t wash off completely. When I look at a slide, I’m not looking at a sample. I’m looking at a sample that I changed in some way. These changes can be misread if not careful, because once I make them, they blend right into that tiny world. They are artifacts of my actions, some damage left behind by the process of being examined.

I spent most of last week in bed. My GI tract felt like I had swallowed lava. I had abdominal neuropathy that felt like electrical shocks spiderwebbing out from just below my xiphoid process. I was tired, weak and foggy from the anesthesia. I slept a lot and watched Netflix and did work from the relative comfort of my bed. These symptoms are not really so much from masto so much as they are from the procedure. Anytime I have a procedure, anytime I take medication, anytime I change my life to accommodate my disease. It leaves a lasting change not from the disease itself, but from the treatment of it.  It leaves an artifact.

I have had several surgeries. I have had hundreds of imaging tests. I have had so many scopes I literally don’t know how many scopes I have had. Every day I take handfuls of pills, infusions, injections, push meds.   This week I am acutely aware of the damage I have sustained by virtue of being treated for my disease. My mast cell medications bring my already slow GI tract to a dead halt. But of course the alternative is that I don’t treat my disease – and of course that’s worse. Right? It’s worse, right?

I know it is worse. I know that I will never live safely without meds. I in no way mean to imply that I am stopping treatment. It’s just getting really hard to know what is from my disease and what is from the treatment. It’s getting hard to know all the ways I have been altered by the experience of living with mast cell disease.

I feel more and more the toll this constant need for medical care is taking on me. With my accessed port, my surgical scars, my ostomy,  I myself am becoming increasingly damaged by this process. I am becoming an artifact.