Thursday, May 24, 2018

Elusive Remission: More Pituitary Treatments or BLA


Today, someone on the message boards asked about removing both adrenal glands, a surgery called bilateral adrenalectomy (BLA), as a treatment for persistent pituitary Cushing's.  Here is my response:

I've noticed that the people who replied have never had BLAs. I'm the moderator of a group with 180 BLAers. 

Based on our group, people have BLAs for several reasons.

1) PITUITARY HYPERPLASIA
Patients pursue BLA when pathology from pituitary surgery shows pituitary hyperplasia, meaning there is no one tumor that can be removed as the pituitary is covered in abnormal
pituitary cells. Further pituitary treatment would be put the patient into remission.

2) INOPERABLE TUMORS and RADIATION.
The pituitary tumor is inoperable because it is located near the carotid artery or optic chasm. Additional pituitary surgery can lead to stroke if the tumor is located near the carotid arteries or blindness if the tumor is located near the optic chasm. Many docs want to pursue radiation even in these delicate places, but the radiation beams are 2-3 millimeters wide, and the tissue left behind after our 2-10+ millimeter tumors are removed is extremely small. So... that laser is still too wide for my comfort. Would an artist paint a woman's face on a standard canvas with a brush used for painting the side of a house? No, the brush width is too wide to capture the delicate details needed for a portrait. Paint would go where you don't want it, and that is the problem with radiation. On bigger tumors in other parts of the body, 2-3 mm beam sounds small, so radiation risks are lower. For us, there is a risk that radiation misses or overradiates the intended area. A close friend of mine had radiation damage to the hypothalamus when radiation meant for her pituitary missed. She was very ill and couldn't control her body temperature for last five years after radiation. She was extremely hot when cold out and extremely cold when hot out. Think about that: radiation that missed prevented a mammal/ human from doing a totally mammalian thing:  self-regulating its temperature of 97-98.6 degrees! So FYI radiation is not the cure that doctors say it is. To this day, I fear radiation far more than I ever feared BLA. After seeing what my dear friend went through, in my opinion, it is more dangerous than a BLA. Would the doctors agree? No. They want you to have radiation so they keep their facility success rates higher, oh, and so you have the chance at successful treatment. Plus, they will never personally see and treat 180 BLA patients in their practice. Unfortunately, their recommendations are so often based in fear of the unknown vs what is possible. We BLAers face fear daily and live the best life we can despite it. 

Also, a big one—radiation takes 3-5 years to actually work. In the meantime, the patient is still experiencing high cortisol symptoms and  horrific, unreversible damage to the body continues. The doctors may put patients on drugs like Korlym, Signifor, or ketoconazole, but all are meant for short-term, band aid use no matter what drug reps or docs being paid to prescribe the drugs say. 

So, the questions becomes, how much more damage can your body take? How much more of life are you willing to miss while waiting for the radiation your doctor wants for you to work?

3) PITUITARY HORMONE REPLACEMENT AFTER ANY PITUITARY TREATMENT.
The pituitary is the master gland and it makes many neurotransmitters and hormones that docs can't test for. Each successive pituitary surgery—even performed by skilled neurosurgeons—will damage the pituitary. The more pituitary surgeries means more damage to the normal homeostasis the gland has, even if the surgeons just removes the tumors. I replace most of the pituitary hormones after two unsuccessful pituitary surgeries: two thyroid medicines, growth hormone injections, progesterone, estrogen patch, testosterone, melatonin for sleep. I do not replace vasopressin for diabetes insipidis. I'm trying to get oxytocin testing soon. 

   PS Patients with adrenal Cushing's who don't have pituitary surgeries prior to BLA tend to do better than the rest of us. That right there shows me the damage that any pituitary surgery does. 

4) FERTILITY. Cushing's strikes woman five times more than me, as 4 of 5 Cushies are female. Therefore, fertility is a big topic largely ignored in the consultations female patients have with even the top endocrinologists and surgeons. If you have not had children or were hoping for more children, successive pituitary surgeries reduce your chance to have a child naturally. Doctors will tell you—as they swore to me—that reproductive endocrinologists can get you pregnant with fertility drugs. While true, many of us have no money left after Cushing's diagnosis and treatment to pay for the advanced fertility treatments like egg retrieval and in vitro fertilization when first line meds like clomid to increase egg production fail to work on a damaged pituitary. For me, I tried to get pregnant between my first and second pituitary surgeries. I didn't ovulate because LH and FSH wouldn't budge. They would boost me to create follicle/ eggs with clomid and then boost me with meds to release the eggs but I never made enough viable eggs. Month after month, this didn't work. After six months, my husband and I decided that our family with one child was complete and I moved on to second pituitary surgery. Therefore, fertility expectations must be a consideration for the patient even if the doctors (who have their own families and never had to make this decision) don't consider it or even mention it.

   PS Many women get pregnant after BLA. I've counted 25+ babies in our group, all conceived easily and surprisingly after BLA. In most instances, mothers were shocked but elated with these pregnancies, since they had no idea they were able to have children after years of infertility that Cushing's causes.

5) NELSON'S. Nelson's occurs in less than 10% of the 180 BLAers on this group. Is 10% still too high for comfort? Of course. However, that means 90% didn't develop Nelson's, and that is a good thing. We have BLAers in the group who have had their BLAs 40-50 years ago to 15-20 years ago to 3-5 years ago to the newly adrenalless. So the group is good for longitudinal studies. 

   PS I have pituitary tumors 3 and 4 percolating up in my pituitary right now. They aren't producing excess ACTH or pressing against anything, so we will monitor it. Even with those pesky things up north, I still have no regrets about choosing to have a BLA. 

6) MEDICATION EVERY DAY. If you had one or two pituitary surgeries, chances are high that you must take medication daily to replace the pituitary hormones lost from those surgeries or just the presence of the tumor(s). For us BLAers, we know that taking cortisol every day is easier to manage than controlling Cushing's excessive cortisol. We know that we control the cortisol; the cortisol doesn't control us. We Cushies know how devastating the pervasive attack of cortisol is on our bodies and minds. For us, BLA provides long-sought-out relief. We get into a daily routine with timers, cortisol pills, aldosterone pills, salt intake (pickle juice, pretzels, broth), and electrolyte drinks. Since the adrenal glands control electrolyte balance, we must stay hydrated daily. We drink lots of water! It becomes a new way of life. We take things easy because we have to. We learn to say no more because we pay the price and the stress will send us into adrenal crisis. And we know that our bodies give us lots of warning. If unheeded and untreated, we go from adrenal insufficiency to adrenal crisis. 

Think of it this way: you wouldn't stop driving just because you will have to stop your car at intersections, where you can die if you fail to do so and you can get crushed in the middle of intersections, right? What do you do to keep yourself safe at intersections? You stay alert. Keep your eyes open for the light to change from green to red. You travel at posted speeds. You apply the brake and slowly bring your car to a safe stop in front of the white line. It is the same for adrenal crisis. We take the necessary steps to prevent us from going into the dangerous intersection of adrenal crisis. As long as we commit to doing that every day, just as we do when driving, then we can stay safe.

   PS I got a service dog to keep me from adrenal crisis. In the year I have had him, I've been able to stay ahead of adrenal crisis and even adrenal insufficiency, as he alerts me to chemical changes in my body before I am symptomatic. That's so helpful!

7) IRREVERSIBLE. While BLA seems intimidating and overwhelming for others, we BLAers know it saved our lives and gave us back a new life without cushing's. For all its risks, that hope and relief cannot be understated. When asked if they regret their BLA, 3-5 out of 180 say they regret it. Those patients have poor follow up care, don't get tests regularly, and don't push doctors for what they need or don't change doctors to get what they need. Overwhelmingly, people are happy they made the decision to have a BLA. It is definitely a no-going-back decision but most of life's big decisions are. We must make the best decision we can at the time we have to make the decision, weighing benefits vs risks. We step forward and hope for the best. The group is a lifeline to all of us who have to move forward in our daily lives and experience things doctors only read about in medical journals or know second-hand from hearing their patients tell them. Patients know more than doctors about BLA, but we strive to work as partners with our doctors to get the best care. We patients save each other's lives, and I'm grateful to have built this group from one Cushie to 180 BLAers in the past 4.5 years. 

I have lots to say about BLA, but I need to go now.

   PS Please excuse typing errors. I'm on my iPhone in bed!Be sure to like my facebook page: Fight Cushing's with Moxie. Bye for now!

Friday, May 11, 2018

Comprehensive List of Cushing's Symptoms collected by a Cushie

I've spent the last 11 years of my life surviving Cushing's and its aftermath by immersing myself in the patient community online. I noticed fairly early on that the symptoms listed on the websites of the major medical centers didn't quite reflect all the symptoms that I and my friends on the Cushing's message boards faced daily. The medical sites always listed their top 10. This has always perplexed me, because it is widely known that Cushing's, or excess cortisol, affects every cell and system in the body. Therefore, those medical sites doesn't really cover the extent of the damage we patients know that uncontrolled, excess cortisol causes.

To see for yourself, take a look at the websites for these major medical centers: 
We Cushing's patients have such an acute awareness of Cushing's oftentimes because we are driven to figure out what is wrong with us. We ask each other online if the strange symptoms we have can actually be caused by excess cortisol or other disruption to the hypothalamus-pituitary-adrenal axis, also known as the HPA axis. So many times, doctors have scoffed at us or outright laughed at the mere suggestion that a symptom we question could be related to excess cortisol. Scorned, we turn to the Cushing's community for validation, and we usually get it. The pituitary is the master gland and controls the hormones made in the body. You can sure bet that there are many more symptoms that Cushies commonly face than the medical community is presenting to us. Eleven years is a lot of time I've been reading Cushing's stories online, and that is one thing I know for sure. 

To rectify that and to educate as many patients as I can about this deficiency, I have prepared the following list over the past several years. I haven't published it before now because it took me a while to remember all the symptoms. Now, I feel it is 98% complete, and that has to be good enough for me to post.


Please note that I don't expect for you to have every symptoms on this list. I don't have every symptoms and it is likely no other Cushie will have every symptoms. However, the real question is: 


From this list of 80 symptoms, how many don't apply to you?

If it were only this simple.
Please take note of which symptoms do not apply to you.

have     not       % Cushing's
                    symptoms I have
 75         5          94%
 70       10          88%
 65       15          81%
 60       20          75%
 55       25          69%
 50       30          63%

So as the table shows, you could go through this list, not have THIRTY symptoms, but still have nearly TWO-THIRDS of the symptoms of Cushing's. That is really quite remarkable. Please use this list to pursue a proper evaluation for Cushing's and any disruption to the HPA axis.

**For tips on how to do that, check out one of my first posts to my blog, "Tips to Steady your Feet and Get Diagnosed Quickly**


Cushing's symptoms you can see

Before I reveal the list, I thought I'd show you the plethora of diagrams showing these freakish changes high cortisol causes. Even these diagrams vary! I am convinced there is no way to make an infographic that lists all the related Cushing's symptoms. There are just too many!!

Do   you   see   yourself  in   these   images?
















*Symptoms caused by Prolonged Exposure to Excess Cortisol*
compiled by Moxie MelissaTX over the years (www.cushingsmoxie.com)

The symptoms of Cushing's result from an excess of cortisol. Most patients develop at least a few of these symptoms, and the symptoms typically worsen over time. However, each person's symptoms depend upon several factors, including:

CUSHING'S SYNDROME SYMPTOMS
The symptoms of Cushing's syndrome result from an excess of cortisol. Most patients develop at least a few of these symptoms, and the symptoms typically worsen over time. However, each person's symptoms depend upon several factors, including:
The degree and duration of cortisol excess
The levels of other adrenal hormones
The underlying cause of Cushing's syndrome"
Source: "Cushing's: Beyond the Basics" (Aug 2018)
GENERAL HEALTH
Can you keep up with the grandmas in your life? Cushing's gives us "old people ailments" not normally seen in younger patients, such:
  • high blood pressure
  • type 2 diabetes, high blood sugars or hypoglycemia, glucose intolerance
  • osteoporosis or frequent bone breaks from little impact
  • narrowing social life
  • suppressed or weakened immune system, "always sick," reoccurring infections
  • kidney stones
  • acid reflux
  • blood clots that cause stroke, pulmonary embolism, or deep vein thrombosis
  • urinating in the middle of the night
  • loss of balance, frequent falls
  • extreme fatigue
  • poor memory
  • slow word recall
  • cataracts or glaucoma
  • shingles
MUSCULOSKELETAL CHANGES: Excess cortisol causes the muscles of the upper arms and legs/hips to become weaker. 
• muscle wasting; heavy arms and pain in legs
      difficulty holding up blow dryer
      getting out of chair
     • climbing upstairs
      standing
     • failed squat test (squat, can't get back up without assistance)
• loss of balance, frequent falls
• pain in the feet upon waking, difficulty walking; docs call this plantar fasciitis
• dropping things from the hands when you think you are grabbing on tightly
• bone loss that leads to 
     • "bad teeth" that crack easily
     • broken bones
     • osteopenia or osteoporosis (especially at a young age) 
• growth retardation/ stunted growth in children

KiDNEY ISSUES/ ELECTROLYTE IMBALANCE
• nocturia (urinating during the night)
• polyuria (volume of urine is high)
• excessive sweating for no reason
• temperature control issues (hot when cool out; cold when hot out)
• pitting edema (swelling) in legs, feet, ankles

SKIN CHANGES
• progressive weight gain of ___ in ___ months despite careful exercise and nutrition control with food diary
• weight gain in the neck, trunk, and abdomen called central obesity, look pregnant
     • skin that hangs down below your belly button called pannum
     • moon facies (roundness of the face); can’t see the ears of a Cushing’s patient
     • supraclavicular fat pads (collar bones aren’t visible; compare to Hollywood actresses on red carpet)
     • buffalo hump at base of neck; hump can be hard or soft; painful when hard; sometimes difficult to turn our necks; place four fingers sideways on the back of your neck with pinkie touching hair line...your forefinger will be touching the top of buffalo hump   
• thin and fragile skin
     • facial plethora (red cheeks look like rosacea)
     • skin tears easily (bandage removal)
     • skin or injuries slow to heal   

      bruise easily
     • infections 
     • red/ purple striae/ stretch marks (small to very wide makes on abdomen and breasts but can also affect thighs and armpits) (only 50% of patients will have this, NIH 2017); striae rubra (red) can fade to striae alba (lighter than skin) in cyclical Cushing's patients 
• acanthosis nigricans (darkening of skin from insulin resistance) typically seen on elbows, knees, knuckles, arm pits, back of neck
• brittle nails
• acne 
• pimples, boils (furuncles) or hidradenitis suppurativa in skin folds in arm pits, under breasts, groin, buttocks
• skin tags
• psoriasis

SLEEP TROUBLE/ FATIGUE

• exhaustion/ extreme fatigue plus insomnia; want to sleep but can’t
• if asleep at night, then waking up at 4 am for “no reason”
• insomnia, wired at night
• inability to fall asleep or inability to stay asleep
• extreme daytime fatigue
• extreme fatigue in afternoon, then second wind that keeps you up all night, crash at 4-6 am, wake 10a-12p
• night sweats, sometimes drenching clothes
• anxiety 
• irritability
• hostile/ short fuse
• don't want to commit suicide but wouldn’t mind not waking up
• loss of hope, can’t see light at the end of the tunnel
• loss of interest in hobbies
• difficulty focusing
• loss of motivation; incomplete projects

MENTAL HEALTH

• personality changes
• emotional volatility
• crying
• rage
• overwhelmed
• medication-resistant depression = depression that fails to respond to medications
• social anxiety/ limited socializing
• suicidal thoughts

COGNITIVE CHANGES

• brain fog
• confusion
• sluggish brain
• delayed word recall
• poor memory
• running into door jams/ scraping arms as you pass through
• blurry vision/ double vision
• keratoconus (steepening of the cornea)

HEART ISSUES:  Excess cortisol raises blood pressure and puts stress on the heart and vascular system.

 high blood pressure
 cardiovascular disease 
• blood clots 
     • need for blood thinners 
     • could lead to deep vein thrombosis, pulmonary embolism, stroke

VISION CHANGES
• headaches
• loss of peripheral vision
• tripping and clumsiness
• eye pain
• ptosis (eye drooping)
• cataracts
• glaucoma

HAIR CHANGES
• hirsutism or unwanted, male-pattern hair growth in women
     • dark, coarse hair on body areas such as face (lip, chin), chest and back
     • balding that causes thinning hair at temples or hair loss in clumps
• texture of hair changes: curly hair goes straight, straight hair turns curly 

REPRODUCTIVE HEALTH
• infertility
• loss of libido
• inability to orgasm
• sexual dysfunction
     • men: erectile dysfunction
     • women: irregular menstruation or lack of menses

ISSUES WITH OTHER ORGANS
• peptic ulcers
• pancreatitis
• fatty liver
• high calcium (get tested for MEN-1)

That is quite a list, right? High cortisol really put our bodies through the ringer. 

What do you think? Did I miss any symptoms? Feel free to comment below and tell me. I'll research your suggestions in pubmed and Google Scholar, and maybe I'll add it to the list! In the future, I hope to hyperlink each symptom in the list to other sites that mention the symptom's relationship to the HPA axis.

To print this list (will return to provide this), go to this version which omits the Cushing's body graphics.

Be sure to read my article, "Gambling on a Cushing's diagnosis" which can help you convince your doctor to send you to an endocrinologist or test you based on the probability that you have all these symptoms at the same time.

Be sure to like my facebook page: Fight Cushing's with Moxie to be the first to get new articles.