Before I begin to share my epic battle with pheochromocytoma.. you have to know what this crazy disease is! Keep reading to understand what it is to be a ‘zebra’
WARNING – MEDICAL JARGON OVERLOAD 🤓
Pheochromocytoma is an extremely rare tumor that develops in cells in the Center of the adrenal gland (adrenal medulla). We all have two adrenal glands, one above each kidney. These two adrenal glands produce hormones that give instructions to virtually EVERY organ and tissue in the body. The adrenal medulla controls hormones that initiate the flight or fight response.
These two small glands impact every organ and tissue in your body. In other words, no adrenal glands, you die.
(Kind of a big deal)
But here’s the thing – the pheochromocytoma tumor actually releases these same hormones in lethal amounts, causing persistent or episodic high blood pressure and rapid heart rate. If left untreated, or (often undiagnosed!) it can lead to life threatening damage to other parts of the body (ex) stroke, heart disease, nerve damage in the eye, kidney failure, and even death.
Translation: The pheochromocytoma tumor makes you overdose without warning on adrenaline hormones!
Sometimes can be triggered by things such as movement, exercise, certain foods and drinks containing tyramine – breathing… and sometimes it’s just sporadic and can happen entirely unprovoked.
When the tumor releases its high amount of adrenaline into the body, it causes a severe and sudden crisis and this results in a spike of blood pressure and heart rate. The symptoms this causes may include:
- Severe onset headache
- heart palpitations
- severe pain in the body/eyes/head/joints
- chest pain resembling heart attack
- symptoms can vary depending on the individual – less or more
Some people may also find they can go days without an attack, months even, some may find their tumors are highly episodic and have attacks several times a day. Also, not everyone will have every symptom listed above, some people may just have a high heart rate and sweating for example, or high blood pressure and headache, it varies depending on the person. Some may not have any symptoms at all and get diagnosed by accident (this is rare).
Research still has not shown what exactly causes the development of this tumor. One thing they can confirm is the tumor is made up of cells called chromaffin cells, these produce and release certain hormones, primarily adrenaline (epinephrine) and noradrenaline (norepinephrine)
Adrenaline and noradrenaline are hormones that we actually need to function as the trigger for your body’s ‘fight or flight’ response to a perceived threat. (cool, right?) These hormones prompt higher blood pressure, a faster heart rate and a boost in other body systems that allow you to react quickly with a burst of energy. *Think, lion attack*!
The pheochromocytoma ultimately makes you overdose on these hormones, without warning, which we in the community ‘attacks’ or episodes. Essentially your tumor is attacking you with your own damn adrenaline, assholes. (the pheo, not you!)
Unfortunately these tumors can also invade the heart, head, neck, bladder, abdomen, and the spine. These little suckers can also produce dangerous hormones. However these tumors are then called paragangliomas, (a pheo that is not located on the adrenal gland)
There are several rare genetic mutations pheo patients are tested for following their illness, most people will find they have a familial genetic disorder which causes the pheos and also are more at risk ordinarily for it to reoccure.
(I however was not one of them, I tested negative for any genetic mutation. Making my pheo sporadic, which sometimes can be seen as a good thing, however some see it as another hindrance being unable to detect or explain why this happened to me)
Even more rarely.. a pheochromocytoma is cancerous (malignant), and the cancerous cells spread to other parts of the body (metastasize)
You will see under ‘My Very First Pheo‘ a little more in detail of how you get to this diagnosis of such a rare disease, but basically…
If you are having episodes of medication resistant high blood pressure, rapid heart rate, severe onset headache, shaking, sweating, flushing, going pale, vomiting, etc… and then you’re back to fabulous, your doctor SHOULD eventually take a hint that it may be time to do some testing for this disease.
Instead, most people are often misdiagnosed with depression, anxiety, insomnia, you catch my drift?
There are a few biochemical tests they need to run to test you for this tumour, one being a 24hr urine analysis (urine catecholamines) along with a special blood sample (plasma metanephrines). Chromogranin A should always be checked as well as dopamine (3MT)
there is such a thing as false negatives.. Chances are if the tumor is very active, it’ll show results through the roof – but ….. It can also be tricky and completely show nothing even though you are are highly symptomatic.
(yes, this happened to me, it actually happens to a lot of us) in which case if you’re sure of your symptoms (listed above) you need to be persistent and don’t give up with your doctor! You KNOW your body! It may be time to get imaging done and look for the tumor rather than just rely on the urine/blood tests.
Surgery is the preferred method of treatment for this tumor, even though it is an extremely risky surgery, this is all they can do to hopefully remove the tumor and have you back to some what normal.
Other treatments such as chemo and radiation are not used as a first line treatment simply because the tumor cannot be fully eliminated in it’s entireity. These treatment options are only used when the tumor is inoperable, to hopefully shrink the tumor and extend life. (These options are used when the disease is cancerous, metastatic, and surgery is not an option anymore)
Pheochromocytomas and paragangliomas are no longer classified as ‘benign’, as of 2017 WHO categorized them as non metastatic or metastatic. Which means they are a form of neuroendocrine cancer
All pheo paras require lifelong follow up, no exceptions! Even if sporadic and no genetic mutation is found, it can still recur.
Stay tuned for more info under my pages…