The use of intravenous fluids for management of mast cell disease

I get frequent consult requests from patients specifically around the use of IV fluids to treat mast cell disease. I am often asked to provide references for papers that show its use and benefit. I am not able to provide any such references because there are none. There has been no organized study for the use of intravenous fluids to manage symptoms from mast cell disease.

Despite this fact, use of intravenous fluids in mast cell disease is increasing in popularity, largely because it works, and word of effective treatments travels fast in a rare disease community. While there is no firm answer for why it helps, there is a reasonable explanation: it treats both deconditioning and POTS and many mast cell patients have one or both.  I wrote a seven part series on why exactly intravenous fluids help in these situations. I have also written in great detail about the way that mast cell disease and POTS interact.

A paper published in early 2017 reestablished the finding that use of intravenous fluids helps POTS. Treatment lengths and infusion volumes varied from person to person. Despite these variations, use of IV fluids decreased symptoms and improved quality of life for POTS patients. The link to the abstract is here.

Many mast cell mediators are vasoactive, affecting the permeability of blood vessels. This means that mast cell activation causes third spacing, the loss of fluid from the bloodstream to the tissues, where the body cannot use it. This functional dehydration can cause a lot of symptoms, not the least of which is exhaustion and difficulty standing or exercising. For obvious reasons, this will be further exacerbated in a patient that is deconditioning or who has also has POTS.

Orthostatic symptoms can be very activating to patients and managing them effectively can help significantly. I have seen IV fluids work where more traditional methods like drinking lots of fluids and consuming lots of salt, or medications like fludrocortisone have not helped. Additionally, the first line tools for managing POTS, beta blockers, are contraindicated in patients at increased risk for anaphylaxis and therefore in people with mast cell disease.

I am a fervent supporter of IV fluids (also called volume loading) in the context of mast cell disease. I have seen it stabilize patients and reduce the frequency of anaphylaxis and severe symptoms, especially orthostatic symptoms and GI symptoms.

I personally use IV fluids. If I don’t receive IV fluids at least three times a week, my orthostatic symptoms become so severe that it is difficult to stand or even move. This in turn triggers mast cell reactions. The benefits of IV fluids to my personal health are significant. Many patients report the same.

While I support the use of IV fluids in the context of mast cell disease, patients should be aware that there are infection risks associated with repeated IV access or placement of a central line. The risks are much lower for repeated IV access as central lines have a host of other risks, including blood clots, and infections have the potential to be much more serious. However, IV access can be difficult for mast cell patients. The treatment value of IV fluids should be weighed on a case by case basis and IV access on a case by case basis.

For additional reading, please visit the following posts:

Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 1

Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 2

Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 3

Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 4

Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 5

Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 6

Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 7

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 12

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 31

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 32

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 61

75. What other diseases and disorders are commonly associated with mast cell disease?

I often joke that it would be easier to list what conditions are not commonly associated with mast cell disease because so many conditions occur alongside it. However, there are some conditions that you see a lot in the mast cell population relative to others. In every instance, mast cell disease has the potential to irritate the other condition and vice verse.

Clonal hematologic disorders. Systemic mastocytosis is so frequently accompanied by other blood disorders that it has a diagnosis specifically for this phenomenon: systemic mastocytosis with associated hematologic disorder (SM-AHD). It is estimated that up to 40% of patients with SM eventually develop another clonal hematologic disorder. A clonal hematologic disorder is a condition in which your bone marrow makes too many blood cells. Examples include chronic myelogenous leukemia, acute myeloid leukemia, polycythemia vera, myelofibrosis, and essential thrombocythemia.

Unlike mastocytosis, MCAS can occur secondarily to lots of conditions. In some instances, it’s not clear if the MCAS is secondary to a condition or the condition is secondary to MCAS or neither.

Heritable connective tissue diseases. Ehlers Danlos Syndrome (EDS), is the most common connective tissue disease in the mast cell population. There are multiple types of EDS. While hypermobility type EDS (formerly called Type III) is the most common in MCAS patients, other forms occur also. Other connective tissue diseases seen in mast cell patients include Marfan Syndrome and Loeys-Dietz Syndrome.

Dysautonomia. Dysautonomia is a condition in which your body’s autonomic nervous system doesn’t regulate essential bodily functions correctly. POTS is the most common form of dysautonomia found in mast cell patients but other forms occur, too.

Mast cell patients commonly have MCAS, EDS and POTS together. They cooccur so commonly that some experts think that that this presentation is actually one overarching disease rather than three separate ones affecting mast cell patients.

Eosinophilic GI disease. Mast cells are closely related to eosinophils. They activate eosinophils and eosinophils activate them. Mast cell patients sometimes have eosinophil GI disease where eosinophils activate to lots of triggers and damage the GI tract.

Immunodeficiency. Conditions that specifically impair a person’s immunity, especially those that affect T or B cells, like SCID or CVID, are not unusual in mast cell patients.

Gastrointestinal disease. Mast cells normally live in the GI tract so they are very sensitive to GI inflammation. MCAS can occur secondarily to lots of GI diseases. Crohn’s, ulcerative colitis, inflammatory bowel disease, and irritable bowel syndrome are examples. GI disorders that specifically affect motility are also seen in mast cell disease, like gastroparesis and chronic intestinal pseudoobstruction.

Allergies. Some mast cell patients have true IgE allergies or other allergic disorders like atopic dermatitis.

Autoimmune disease. Autoimmune disease is more common in MCAS patients than in SM patients. The specific disorder could be virtually any autoimmune condition, including rheumatoid arthritis, lupus, Hashimoto’s thyroiditis, autoimmune urticaria, and many others.

Adrenal insufficiency. The body’s mechanisms for produce stress hormones like cortisol can become dysregulated in mast cell patients. This results in a situation in which the body does not make enough steroids of its own to take care of the body during periods of stress. Patients with adrenal insufficiency are dependent upon daily steroids to stay safe.

Chiari malformation. This condition affects the space around a person’s brainstem, causing a wide array of symptoms. Some patients have surgery for this condition.

Asthma. It is difficult to draw an exact line where mast cell disease ends and asthma begins in mast cell patients as the symptoms can be virtually identical.

This list is not exhaustive. Many other conditions sometimes occur in mast cell patients.

For additional reading, please visit the following posts:
The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 31
The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 32

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 57

71. What other diseases “look like” mast cell disease?

Mast cell diseases have many symptoms that are also commonly found in other disorders. This is one of the reasons why it is difficult to diagnose correctly. The following conditions have symptoms that can look like mast cell disease.

Neuroendocrine cells are specialized cells that help to pass signals from the nervous system to nearby cells, causing those cells to release hormones. There are many types of neuroendocrine tumors. Some conditions that look like mast cell disease are caused by these tumors. Symptoms from them are caused by the response of too much hormone.

Carcinoid syndrome is the result of a rare cancerous growth called carcinoid tumor. This tumor releases too much serotonin into the body. This can cause flushing, nausea, vomiting, diarrhea, difficulty breathing, and cardiovascular abnormalities such as abnormal heart rhythm. Mast cells also release serotonin but they release much less than carcinoid tumors.

VIPoma means vasoactive intestinal peptide –oma. When a word has –oma at the end, it means that it is a tumor. A VIPoma is a tumor that starts in the pancreas. It releases a chemical called vasoactive intestinal peptide. VIPoma can cause flushing, low blood pressure, and severe diarrhea leading to dehydration. A VIPoma can also abnormalities in the composition of the blood. Many patients have low potassium, high calcium, and high blood sugar.

Pheochromocytomas start as cells in the adrenal glands. They release excessive norepinephrine and epinephrine. They can cause headaches, heart palpitations, anxiety, and blood pressure abnormalities, among other things.

Zollinger-Ellison syndrome is a condition in which tumors release too much of a hormone called gastrin into the GI tract. This causes the stomach to make too much acid, damaging the stomach and affecting absorption.

Some blood cancers can cause mast cells to become overly activated. They may also cause an increase in tryptase, an important marker in diagnosing systemic mastocytosis.

Some other cancerous tumors like medullary thyroid carcinoma can cause mast cell type symptoms including flushing, diarrhea, and itching.

Most diseases with any allergic component can look like mast cell disease.

Eosinophilic gastrointestinal disease occurs when certain white blood cells called eosinophils become too reactive, causing inflammation to many triggers. Furthermore, people are more frequently being diagnosed with both EGID and mast cell disease.

Celiac disease is an autoimmune disease in which gluten causes an inflammatory reaction inside the body. The damage to the GI tract can be significant. Malabsorption is not unusual. Children with celiac disease may grow poorly. Bloating, diarrhea, ulceration, and abdominal pain are commonly reported.

FPIES (food protein induced enterocolitis syndrome) can cause episodes of vomiting, acidosis, low blood pressure and shock as a result of ingesting a food trigger.

Traditional (IgE) allergies can also look just like mast cell disease. They are usually distinguished by the fact that mast cell patients may react to a trigger whether or not their body specifically recognizes it as an allergen (does not make an IgE molecule to the trigger). Confusingly, it is possible to have both traditional IgE allergies and mast cell disease.

Postural orthostatic tachycardia syndrome (POTS) is commonly found in patients with mast cell disease. However, POTS itself can have similar symptoms to mast cell disease. Palpitations, blood pressure abnormalities, sweating, anxiety, nausea, and headaches are some symptoms both POTS and mast cell disease have. There are also other forms of dysautonomia which mimic the presentation of mast cell disease.

Achlorhydria is a condition in which the stomach does not produce enough acid to break down food properly. This can cause a lot of GI pain, malabsorption, anemia, and weight loss.

Hereditary angioedema and acquired angioedema are conditions that cause a person to swell, often severely. Swelling may affect the airway and can be fatal if the airway is not protected. Swelling within the abdomen can cause significant pain and GI symptoms like nausea and vomiting.

Gastroparesis is paralysis of the stomach. People with GP often experience serious GI pain, vomiting, nausea, diarrhea or constipation, bloating and swelling.

Inflammatory bowel diseases and irritable bowel syndrome can all cause GI symptoms identical to what mast cell patients experience.

This list is not exhaustive. There are many other diseases that can look similar to mast cell disease. These are the ones I have come across most commonly.

For more detailed reading, please visit the following posts:

Gastroparesis: Part 1
Gastroparesis: Treatment (part 2)
Gastroparesis: Diabetes and gastroparesis (Part 3)
Gastroparesis: Post-surgical gastroparesis (Part 4)
Gastroparesis: Less common causes (Part 5)
Gastroparesis: Autonomic nervous system and vagus nerve (Part 6)
Gastroparesis: Idiopathic gastroparesis (Part 7)

Food allergy series: Food related allergic disorders
Food allergy series: FPIES (part 1)
Food allergy series: FPIES (part 2)
Food allergy series: Eosinophilic colitis
Food allergy series: Eosinophilic gastrointestinal disease (part 1)
Food allergy series: Eosinophilic gastrointestinal disease (part 2)
Food allergy series: Eosinophilic gastrointestinal disease (part 3)
Food allergy series: Eosinophilic esophagitis (Part 1)
Food allergy series: Eosinophilic esophagitis (Part 2)
Food allergy series: Eosinophilic esophagitis (Part 3)

Angioedema: Part 1
Angioedema: Part 2
Angioedema: Part 3
Angioedema: Part 4

Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 1
Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 2
Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 3
Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 4
Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 5
Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 6
Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 7

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 41

50. How does mast cell disease affect hearing?

For readers who don’t know, I lost the majority of my hearing in 2009. I am profoundly deaf in my left ear and have moderate to severe hearing loss in my right. This happened years before I was diagnosed with systemic mastocytosis or Ehlers Danlos Syndrome.

Mast cell disease affects hearing in multiple ways. Some related diagnoses also affect hearing.

Mast cells are involved in sensorineural hearing loss. The exact role of mast cells is still being researched but hearing loss is not an unusual complaint for mast cell patients. Mast cell disease can also cause auditory processing disorder. This condition makes it difficult to understand speech. Ringing in the ears (tinnitus) is also a symptom of mast cell disease.

Many mast cell patients also have Ehlers Danlos Syndrome (EDS), a disease in which the body makes defective connective tissue. EDS patients are vulnerable to both sensorineural hearing loss, in which the nerves don’t correctly transmit sound from the ear to the brain, and conductive hearing loss, in which the ear is not able to carry the sound waves correctly to the inner ear. Having both types of hearing loss, sensorineural and conductive, is called mixed hearing loss.

Many mast cell patients are deconditioned. This means that their body has undergone lots of changes as the result of not being active. Sensory processing is affected in deconditioned patients. In particular, sounds must be louder to be heard correctly. POTS patients sometimes experience something similar.

Having certain autoimmune disorders can increase the risk of autoimmune inner ear disease, resulting in hearing loss. Many mast cell patients also have autoimmune disease.

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 32

39. How are mast cell disease, Ehlers Danlos Syndrome and POTS connected? (Continued)

I’m answering this question in two parts because there is a lot of information to relay and it’s important that it is done clearly. This is the second part.

Mast cells are found throughout the body. There is no record of a person living without mast cells. They perform many essential functions. This is the reason why killing off all of a person’s mast cells is not a viable treatment for mast cell disease. While mast cells cause so many symptoms and problems for patients with mast cell disease, life is unsustainable without mast cells.

Let’s specifically consider just a few of the mast cell’s essential functions here and how they relate to POTS and EDS.

Mast cells help the body to regulate blood pressure and heart rate. Many of the mast cell’s chemicals do this so it happens in many different ways all stemming from mast cells. This means that when mast cells are not behaving appropriately, there are many ways in which this dysfunction can lead to not regulating blood pressure and heart rate correctly.

  • Histamine can affect blood pressure and heart rate differently depending upon how it acts on the body. If it uses the H1 receptors, it can cause low blood pressure. If it uses the H2 receptors, it elevates blood pressure. If it uses the H3 receptor, it can cause low blood pressure. When it does this at the H3 receptor, it’s because it tells the body not to release norepinephrine. Not releasing as much norepinephrine lowers heart rate and making the heart beat more weakly.
  • Prostaglandin D2 lowers blood pressure and causes fast heart beat. However, the molecule made by breaking down PGD2, called 9a,11b-PGF2 increases blood pressure.
  • Vasoactive intestinal peptide lowers blood pressure.
  • Heparin, chymase and tryptase can decrease blood pressure. They do this by helping to make a molecule called bradykinin. When this happens, a lot of fluid falls out of the blood stream and gets stuck in the tissues, causing swelling.
  • Thromboxane A2 increases blood pressure.
  • Many mast cell molecules affect the amount of angiotensin II. This molecule strongly drives the body toward high blood pressure. Some mast cell molecules that affect blood pressure this way include chymase and renin.

Another very essential function of mast cells is to make connective tissue. Mast cells help the body to shape itself correctly and to make tissue to heal wounds. When mast cells are not behaving appropriately, their dysfunction can interfere with making connective tissue and wound healing. It can cause wounds to heal very slowly or for there to be too much scar tissue. It can also cause the connective tissue to be too weak or too strong.

The interaction between POTS and mast cell disease

In POTS, the body is already predisposed toward not regulating blood pressure and heart rate correctly. When a person with POTS stands up, their body quickly causes the heart to beat very fast. When your body does this, it takes steps that cause mast cells to become activated. In turn, the mast cells release chemicals to try and regulate the heart rate. However, if you have mast cell disease, the mast cell may release the wrong chemicals, or too many chemicals, failing to regulate the heart rate. This in turn results in a situation where the body becomes very stressed. Stress activates mast cells, which results in more release of chemicals. Patients can very easily become trapped in a cycle where POTS and mast cell disease irritate each other.

POTS can be exacerbated by the use of medications that affect blood vessels. Medications that are vasodilators (that make the blood vessels bigger) are taken by many people, including mast cell patients. In some people, using medications that blocks the action of histamine or prostaglandins can help to improve symptoms of both POTS and mast cell disease. Conversely, some of the medications used to manage POTS, like beta blockers, can trigger mast cell reactions and raise the risk of anaphylaxis. However, some POTS treatments can also help alleviate mast cell symptoms, specifically the use of IV fluids.

A paper published in 2005 found that hyperadrenergic POTS was sometimes found in patients with mast cell activation disorders.

The interaction between EDS and POTS

POTS is a form of dysautonomia. Dysautonomia means dysfunction of the autonomic nervous system. This is the part of your nervous system that helps to control automatic functions like heart rate, blood pressure and digestion.

In EDS patients, the body does not make collagen correctly. Collagen is the most common connective tissue protein in the body. This can cause vascular laxity. Blood vessels change size depending upon how much blood they need to move through them. If they get larger, it is called vasodilation. When they get smaller, it is called vasoconstriction. When a person has vascular laxity, their vessels can get larger than they should and they can stay that way longer.

POTS is the most common form of orthostatic intolerance in HEDS. Orthostatic intolerance is when a patient has symptoms specifically as the result of standing up. All EDS patients have more autonomic symptoms than healthy people. Among patients with EDS, autonomic symptoms are more common and more severe in HEDS. 94% of HEDS patients have orthostatic symptoms, including lightheadedness, dizziness, palpitations, nausea, blurred vision, and anxiety. Dysautonomia is much worse in HEDS compared to CEDS and VEDS patients.

Patients with HEDS were found overall to have overactive sympathetic nervous systems. However, when their body needed to activate in response to regulate heart rate and blood pressure in response to changing position, their responses were not strong enough.

In EDS patients, the connective tissue does not support blood vessels enough. This makes the harder for the blood vessels to get the blood back to the right places when you stand up, exacerbating POTS.

The interaction between EDS and mast cell disease

Mast cells are involved in making and repairing connective tissue, which involves collagen. For this reason, there are many mast cells living in connective tissues. Mast cells are stimulated when the body is making or trying to make collagen. Because EDS causes the body to make collagen incorrectly, mast cells can become activated to try and make collagen and other connective tissue correctly. When mast cells in one place are activated a lot over a long time, they can activate other mast cells elsewhere, resulting in systemic symptoms.

The interactions among mast cell disease, POTS and EDS

It is undeniable that there is an association among mast cell disease, EDS and POTS. However, there is not much data published on this topic. There was a poster presented in 2015 that found some combination of EDS, POTS and MCAS in a group of 15 patients. This is a very small population and we need larger studies to understand incidence. There is ongoing work to tie this group of conditions to specific genetic markers. However, this also requires further investigation and more patients. In the absence of hard data, we are forced to use some early data and understanding of similar conditions to try and figure out exactly what happens. As more data comes out, this understanding may change.

This is very much a chicken and egg situation where it’s not clear exactly what begets what. EDS is a genetic disorder and considered primary. However, that does not necessarily mean POTS or mast cell disease is secondary in this scenario.

Regardless of which is the initiating condition, the relationship seems to be something like the following:

1. A patient has EDS. They make defective connective tissue. These defective tissues do not support the bodily organs and vessels properly.

2. A patient stands up. Blood quickly moves from the torso into the legs.

3. The blood vessels in the legs try become more narrow and more able to keep fluid in the bloodstream. However, in an EDS patient, the blood vessels are stretched out and not held in the right place because the connective tissue is too weak.

4. The blood vessels in the legs are not able to pump blood back to the heart quickly enough. The body interprets this as having low blood pressure.

5. The nervous system sends signals to increase heart rate to compensate for the “low” blood pressure.

6. The signals sent to increase heart rate activate mast cells.

7. Mast cells activate release mediators to try and regulate blood pressure and heart rate.

8. Mast cell mediators activate other mast cells, eventually affecting other parts of the body.

9. The molecules released by mast cells make blood vessels bigger and more leaky.

10. As fluid leaves the bloodstream and gets stuck in places where it can’t work (third spacing), blood pressure decreases and heart rate increases. This exacerbates POTS symptoms. The cycle repeats.

For more detailed reading, please visit these posts:

Cardiovascular manifestations of mast cell disease: Part 1 of 5

Cardiovascular manifestations of mast cell disease: Part 2 of 5

Cardiovascular manifestations of mast cell disease: Part 3 of 5

Cardiovascular manifestations of mast cell disease: Part 4 of 5

Cardiovascular manifestations of mast cell disease: Part 5 of 5

Hypermobility Type Ehlers Danlos Syndrome and Autonomic Dysfunction (Part 1)

Hypermobility Type Ehlers Danlos Syndrome and Autonomic Dysfunction (Part 2)

Hypermobility Type Ehlers Danlos Syndrome and Autonomic Dysfunction (Part 3)

Hypermobility Type Ehlers Danlos Syndrome and Autonomic Dysfunction (Part 4)

Hypermobility Type Ehlers Danlos Syndrome and Autonomic Dysfunction (Part 5)

Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 1

Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 2

Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 3

Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 4

Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 5

Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 6

Deconditioning, orthostatic intolerance, exercise and chronic illness: Part 7

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Diseases, Part 31

39. How are mast cell disease, Ehlers Danlos Syndrome and POTS connected?

I’m answering this question in two parts because there is a lot of information to relay and it’s important that it is done clearly.

Let’s talk about what EDS and POTS are first.

Ehlers Danlos Syndrome (EDS) is a connective tissue disease. It can be, and often is, inherited. About 1 in 5000 people have some form of EDS.

There are several subtypes of EDS. The ones you hear about most are called classical, vascular, and hypermobility. The different forms of EDS used to be distinguished by numbers (like Type I, Type II, etc) but now they use descriptive terms instead. Types I and II EDS are now called classical EDS (cEDS); type IV EDS is now called vascular EDS (vEDS); and type III EDS is now called hypermobility type (hEDS or htEDS). There are also other rare variants of EDS.

Each of these subtypes has distinguishing features that make them unique from the other forms of EDS. All forms of EDS cause major systemic dysfunction of connective tissue, the pieces of you that hold your body together and keep everything in the right place. Generally, in EDS patients, their connective tissues tear easily and heal slowly. They usually (but do not always) show hypermobility in their joints (being double jointed or overly flexibility). Skin that is very stretchy or that heals very poorly is common.

Because you have connective tissues holding your whole body together, EDS can affect your entire body. All patients are at risk for symptoms that specifically impact their joints, muscles and bones. VEDS can significantly affect life span because it increases the risk of an aneurysm or a blood vessel bursting. HEDS patients often have cardiovascular, GI, and neurologic symptoms. CEDS patients often display the trademark skin stretchiness and many have extraordinary difficulties in healing incisions and wounds. Of course, many EDS patients have other symptoms, and there is a lot of symptom overlap among these forms. I am just generalizing here.

There is no cure and treatment is largely about managing symptoms and complications. EDS is usually diagnosed by a geneticist. There are genetic markers for most forms of EDS that can be found with genetic testing. However, the most common form of EDS, hypermobility type EDS (hEDS), does not have a known genetic marker. For this reason, geneticists often assess how hypermobile a patient is and then uses that to support the diagnosis of hEDS.

Postural orthostatic tachycardia syndrome (POTS) is a form of orthostatic intolerance, which means symptoms and problems caused specifically by standing up. POTS patients have a big jump in heart rate when they stand up (increase of 30 beats per minute or heart rate over 120 beats/minute in adults) that is not due to a drop in blood pressure. POTS is a form of dysautonomia, an umbrella term that covers several conditions in which the body is not able to control some of the body’s automatic functions like heart rate and blood pressure. (For those wondering, automatic is not a typo, and I did not mean to write autonomic, which is related here.)

There are multiple types of POTS. I’m just going to cover neuropathic POTS and hyperadrenergic POTS as they are the most applicable here. POTS can be a primary or secondary condition. It can cause very severely disabling symptoms and effects. It can cause a huge array of symptoms, including dizziness; fainting; exhaustion; inability to exercise; nausea; vomiting; major GI disturbances (both diarrhea and constipation); inappropriate sweating; chest pain; coldness, numbness, pain and weakness of extremities; and anxiety. Some patients are unable to stand up at all.

Neuropathic POTS, the most frequently described, is thought to be the result of the veins in the legs not being able to pump blood effectively. When you stand up from a sitting position or laying down, a lot of blood that was in your torso quickly moves into your legs. This happens to everyone. In most people, the veins in your legs are able to tighten and squeeze effectively to pump that blood out of the legs and get it back to your heart. In neuropathic POTS, your veins don’t seem to be able to do this as well so the blood gets stuck in your legs. Your body interprets this as having low blood pressure even though you have enough blood and it’s just not where your body expects it. In response to the “low blood pressure”, your heart starts beating very fast to try and get enough oxygenated blood to every place in your body that needs it.

Hyperadrenergic POTS is less common but relatively more common in mast cell patients. In this form, the body makes too much adrenaline (and often other similar molecules like noradrenaline). These molecules work together to cause the nervous system to tell the heart to beat way too fast in response to standing up and that blood moving into your legs. In patients with hyperadrenergic POTS, blood pressure is often increased while the heart rate is also increased instead of being normal or low as in neuropathic POTS.

The second part of this question (question 39) will be up in a day or two. Sorry for the length but I don’t think there’s a way to answer this question both clearly and with brevity.

The MastAttack 107: The Layperson’s Guide to Understanding Mast Cell Disease, Part 22

I answered the 107 questions I have been asked most in the last four years. No jargon. No terminology. Just answers.

  1. Does mast cell disease cause cognitive issues?
  • Yes.
  • The most common cognitive issue reported by mastocytosis patients is “brain fog”, a sort of difficulty in thinking and reacting normally.
  • Inability to focus, pay attention, find words, and keep things in short term memory are frequently reported by mast cell patients. Attentive deficit disorders are sometimes seen.
  • Aside from the effects of mast cell disease on your body, they also affect the lives of patients dramatically. 42% of mastocytosis patients in one study reported a high stress level. I would be willing to bet that across the entire population of mast cell patients, the number of people that feel a lot of stress is a lot higher than 42%. Many patients feel hopeless, guilty, or like a burden. While this is distinct from depression, a neurologic disorder, these feelings can make it hard for patients to focus or pay attention.
  • Mast cell disease can lower serotonin. Even where this is not the case, mast cells can greatly impact the way serotonin works in the body. Serotonin in a chemical that nerves and other cells use to talk to each other. It is also important in cognition. While this isn’t totally understood yet, it appears that increasing serotonin levels can improve memory and decrease impairment. It can also improve ability to learn things. Not enough serotonin was associated with memory and learning difficulties.
  • When mast cells are activated, your body thinks there is an emergency or an infection. It can activate a stress response. One of the things your body does during this response is release cortisol. Cortisol can further activate mast cells. It is also released by mast cells. Over time, more cortisol than normal can really fatigue the body. Long term stress response is associated with a lot of cognitive issues, including brain fog.
  • Mast cell disease is very disruptive to your sleep cycle. Personally, this is one of the hardest parts of the disease for me. Your body naturally starts releasing more histamine around 10pm, every night, for everyone. Mast cell patients often have worsened symptoms starting around then and continuing overnight.
  • Another mast cell mediator, prostaglandin D2 (PGD2), is the strongest known inducer of sleep in the body. Mast cell patients may have this in excess, making them even more tired.
  • Despite the common idea that histamine makes you drowsy, it actually keeps you awake. Many mast cell patients have insomnia because of the histamine release overnight. This translates to being exhausted during the day when histamine levels drop. Lack of sleep is a well documented cause of cognitive dysfunction.
  • Many mast cell patients have POTS or another form of dysautonomia. These conditions can prevent getting enough blood and oxygen to the brain.

For more information, please visit these posts:

Neuropsychiatric features of mast cell disease: Part 1 of 2

Neuropsychiatric features of mast cell disease: Part 2 of 2

MCAS: Neurologic and psychiatric symptoms

Privilege

My body is changing. I am tired but do not sleep for twenty hours at a time. Bones and angles emerge as my swelling wanes. I exercise. I eat real food. I sleep at night.

At the same time, I am carefully engineering to encourage these continued changes. I still take a ton of medication. I still need IV fluids every day. I still need IV meds. I still need to manage my pain. I still need to be careful. This nethervoid I currently inhabit might never be mistaken for healthy, but it is healthy for me. It is stable at least, predictable. It is good for me.

Last week was composed of the oppressive, sticky summer days that Boston is known for. Heat, humidity and sunlight form my own personal triad of doom. I got halfway through my short walk between stations and started reacting badly. I went into a Starbucks and promptly threw up while hives appeared on my neck. All of my exposed skin was bright red. I took some Benadryl and drank some cold water and waited for things to calm down. They did. I continued on my way to work.

It is hard for me to gauge how bad I look on any given day, as I was for many years in a persistent reactive state. My only indication is that initial surprise when people look at me, that flash of concern as their eyes widen, a brief moment before they recover. I knew as soon as I got to work that I must look terrible.

We have a cold room at work that is essentially an enormous refrigerator. “Girl, you need to go stand in there,” one of my coworkers said with a supportive nod. So I did. It helped. When I emerged, multiple people told me they were worried I would anaphylax and to please take a cab home. I am so fortunate to work with this group of caring, wonderful people that understand my disease and want me to be safe.

I did end up taking a cab home. I didn’t want to, but I did. It’s hard for me to articulate why I didn’t want to, when I knew it was safer and easier, in a way that doesn’t make me sound crazy. Getting in that cab made me sad in this nebulous but palpable way.

Taking the train to work is a privilege. Going to work, cleaning your house, paying your bills, food shopping, making dinner, eating solids, crunching lettuce as you watch television, being part of the world. These are privileges. These are the things you miss when you are hospitalized or so tired that your whole body feels heavy or riding that knife’s edge of anaphylaxis because your body is fighting you on something you need to do.

All of the days you spend fighting – this is what it is for. You fight for these privileges. You fight to be in the world. These are the things you will miss. All you can ever hope for is to wake each day to a world full of mundane privileges.

Some days I want to take the train even if there is a chance I will get sick. Because there is a chance that I won’t. Once that was impossible. Maybe it will be again. Maybe tomorrow it will be impossible, but not today.

I am still sick. I am still in pain. I still have a poorly functioning GI tract. I still carry two Epipens and a backpack full of meds everywhere I go. I am still nauseous. After all of the effort put forth in the last three months, I did not get cured. I got to walk to work sometimes. I got to eat salad. I got to feel the sunlight on my skin. That’s what I got. And it’s enough, and even more than it’s enough, it’s amazing. All of this is amazing. I am alive this summer and I am alive in the heat and I am alive when I’m too hot and I’m alive in the sun.

You cannot always decide what you do, but you can always decide who you are. I cannot always walk in the summer sun, but I am always a person who wants to.

I choose to live in the world and to enjoy it and be alive. I choose this even when it might hurt me. I choose this even when it might kill me. It is where I want to be.

It is a privilege to participate in this world. It is a privilege to be alive.

My exercise program for POTS and deconditioning

I designed the following schedule for myself after being medically cleared to return to exercise following surgery. This routine is not appropriate for everyone. Please speak with your medical provider regarding safe ways to exercise.

I put together this routine for myself by integrating POTS/dysautonomia exercise programs and my own personal exercise history. Even on my most miserable days, I walk for 20-30 minutes, so walking is something that I can trust to not raise my heart rate. I also have been practicing vinyasa style yoga for over fifteen years and started with very easy seated poses and progressed to more fluid sequences (Sun Salutation A 3-5x, Sun Salutation B 3x, followed by whatever sequences I felt were reasonable for that day.)

For the first few weeks, I timed my exercise for about an hour after taking antihistamines. For weeks 1-3, I performed all of my allotted exercise for the day consecutively over about an hour. For weeks 4-8, walking was often broken up over the course of the day as this included walking I did as part of my commute. My first walk of the day occurs within an hour of taking my morning medications and I take meds about an hour before leaving work for the day to cover my commute home.

Slow walking: about 2.5-3 miles/hour
Moderate walking: about 3-3.5 miles/hour

For seated cardio, I just looked around online for some seated cardio that I could do at home. I found a few routines.

For standing cardio, I did various things like jumping jacks and high knees. I usually incorporated bodyweight exercises that I could modify, like squats and planks.

Walking was all done outside. Some was done at night and some during the day. I tried to limit walking during the middle of the day to the extent that it was possible because heat and sunlight trigger me. All other exercises were done in my air conditioned apartment.

If I felt like I needed a break while exercising, I took a break. So ten minutes of cardio does not always represent ten consecutive minutes, but rather a total of ten minutes performing cardio exercise.

As I added in more exercise, I increased to exercising four days a week, which means that sometimes I exercise twice in one day. Walking is also split up over the course of the day, as I previously mentioned.

Week One:

Three days:
Twenty minutes of slow walking
Ten minutes seated cardio
Twenty minutes stretching/seated yoga
Ten minutes slow walking

Week Two:

Three days:
Thirty minutes of slow/moderate walking
Ten minutes seated cardio
Ten minutes yoga
Ten minutes stretching

Week Three:

Three days:
Forty minutes of moderate walking
Twenty minutes yoga
Ten minutes stretching

One day:
Sixty minutes of walking

Week Four:

Two days:
Fifty minutes of moderate walking
Twenty minutes of yoga

One day:
Fifty minutes of moderate walking

Week Five:

Two days:
Fifty minutes of moderate walking
Twenty minutes of yoga

One day:
Fifty minutes of moderate walking
Ten minutes of standing cardio

One day:
Sixty minutes of moderate walking

Week Six:

Two days:
Sixty minutes of moderate walking
Twenty minutes of yoga

Two days:
Fifty minutes of moderate walking
Ten minutes of standing cardio

Week Seven:

Two days:
Sixty minutes of moderate walking
Twenty minutes of yoga

Two days:
Fifty minutes of moderate walking
Fifteen minutes of standing cardio

Week Eight:

Two days:
Fifteen-twenty minutes of standing cardio
Twenty minutes of yoga

Three days:
Sixty minutes of moderate walking

 

Edited on 29 Jan 2017 to include weeks 9-12 of this program:

Week Nine:

Two days:
Twenty minutes of standing cardio
Thirty minutes of yoga (intermediate)

Three days:
Sixty minutes of moderate walking

Week Ten:

Three days:
Twenty minutes of standing cardio
Forty minutes of yoga (intermediate)

Three days:
Sixty minutes of moderate walking

Week Eleven:

Three days:
Fifty minutes of yoga (intermediate/advanced, pace moderate/fast)

Three days:
Sixty minutes of moderate walking

Week Twelve:

Three days:
Sixty minutes of yoga (intermediate/advanced, pace moderate/fast)

Three days:
Sixty minutes of moderate walking

Independence Day

I live my life as a series of wagers. A lot of these wagers involve my health. I bet that I can fly if I take enough steroids. I bet that I will get better if I get an ostomy. I bet that I will be more stable if I use IV hydration. I bet that taking this med or that will make me less tired. Sometimes I win. Sometimes I don’t.

The last 18 months of my life have all been one large scale bet. It has been many months of moving the pieces around and trying to shove them into place. It has been emotional and stressful and scary.

I slept through the four weeks following my surgery. I did some other things too, but mostly I slept. One day while I was resting in bed, it occurred to me that all of the strength and stamina I had lost was perhaps for the best. There are few opportunities to reset your body and this was one of them. I wasn’t reacting because I was heavily medicating and resting most of the time. I realized that this might be an opportunity to rebuild my body in a calculated way.

Once I was cleared by my surgeon to exercise, I started an exercise program designed for POTS patients. It was pretty detailed (I’ll do a separate post about this) but involved cardio exercise 3-4 days a week. I haven’t been able to do cardio in years. But I figured it was worth a shot.

The first two weeks were brutally hard. Then it got easier. I am now on the sixth week of a twelve week program. For the first time in many years, I can do cardio (with premedication in a controlled environment) without having a reaction.

I went back to work last week. I took the train to and from work on Monday, Wednesday and Thursday, which also involves about a mile and a half of walking each day. It was pouring torrentially on Wednesday and hot as hell on Thursday. I was exhausted when I got home but I managed to get through each day without napping. I slept every night last week. Getting myself to and from work is a level of independence I have not achieved in a year.

I very rarely drive anymore because I can’t use some of my medications if I need to drive and I have been so reactive that that might have been dangerous. But I made a huge wager on Saturday: I drove myself an hour away to New Hampshire to celebrate the Fourth of July with my friends and nieces. I stayed overnight and went swimming today, deaccessing and reaccessing my port. I drove myself home after being in cold water and direct sunlight for over an hour, stopping at Whole Foods and doing my grocery shopping on the way. I cleaned my apartment, did laundry, made lunch for tomorrow, ironed my work clothes, and watched Shark Week. I did all these things without any help.

The Fourth of July is Independence Day in the US. As I watched the fireworks, it felt like I was celebrating my own personal Day of Independence. I don’t know how long this will last.  But I got this one great week and this one Fourth of July.  And maybe I’ll get more.