hygiene - AutoSleep

http://autosleep.tantsissa.com/hygiene

Fludrocortisone shortage

Today I share with you the January 4, 2019 notice from National Adrenal Disease Foundation. It provided an update for the ongoing fludrocortisone shortage many US adrenally-insufficient patients are facing, including Cushies without adrenal glands, like me.

I recommend that you provide a national drug code (NDC) when you call your pharmacy to ask them to find fludrocortisone acetate for you. The pharmacist tech will simply punch this NDC into their computer to locate this medication.

NDC for IMPAX/ Global is 0115-7033-01.

According to the U. S. Library of Medicine, the NDC for fludrocortisone acetate manufactured by IMPAX/ Global is 0115-7033-01.

NDC for IMPAX/ Global is 0115-7033-01.

In addition, I have my endocrinologist write my prescription for fludrocortisone for 0.1 mg bid or twice daily, even though I only take 0.2 mg fludrocortisone during the summer. This allows me to build a small stock pile of this life-saving medication during the other times of the years. It is critical for us to be prepared for times of natural disasters like fires, hurricanes, snow storms, manufacturing delays, shutdowns, or shortages. 

We have to be smart about how to keep ourselves alive. 

Download NADF's original statement here.

Excerpt:

"This is an important update on accessibility of fludrocortisone acetate. Barr/Teva has informed us that the company is phasing out their production of fludrocortisone acetate. We want to reiterate that there are other manufacturers of our life-preserving aldosterone hormone replacement fludrocortisone acetate including Impax/Global Laboratories, Inc. and for those with government insurance, AvKARE. However, AvKARE is experiencing a manufacturing delay so that product may also be temporarily unavailable. 

To complicate matters, McKesson is one of the largest pharmaceutical distributors in the USA, and has exclusive contracts with many manufacturers on the supply side, as well as many of the large pharmacy chains and mail order pharmacies on the distribution side. In this case, McKesson has an exclusive contract with Teva/Barr for fludrocortisone acetate, and does not supply Impax products.This means that patients might find ordering fludrocortisone acetate from one of the larger pharmacies as impossible, because that pharmacy has McKesson as their pharmaceutical product supplier. 

NADF's updated (12/31/18) recommendations for obtaining fludrocortisone acetate should you run into problems: 

• Ask your pharmacist if they have contacted their wholesaler network and exhausted all options
   
• Try a smaller non-chain/local pharmacy since they are often not bound by exclusive contracts. If you have a good relationship with your pharmacist where the Rx is unavailable, ask them if they mind calling around to a few local pharmacies to check stock for you and then they can transfer the Rx electronically or via fax
• Try a hospital pharmacy
• If you are still not able to obtain your fludrocortisone acetate, please contact NADF at nadfmail@nadf.us

*************************

You can share your experience getting your fludrocortisone prescription filled by taking this NADF survey. It's short--only four questions. 

Getting your Cortisol Dosage Right

Doctors generally scare patients from taking extra cortisol (ie updosing or stress dosing) after pit surgery, adrenal surgery, or removing both adrenal glands (BLA). This is rich. They barely believed that we had Cushing's in the first place, yet now we must respect them as experts in the damage caused by excess steroid replacement. They rail against the damage of long term cortisol replacement TO CUSHING'S PATIENTS, as if we don't know, as if we aren't still living the nightmare of excess cortisol made in our bodies. Many doctors refuse proper cortisol evaluation, imaging, or simple blood tests to evaluate related pituitary and adrenal hormones even when the patient asks. Doctors delay diagnosis in every way possible, leaving us with rampant cortisol excess for 7-10 years on average.

So let me be clear.

Updosing for illness is life-saving in the short term. The long term doesn't matter much if we are dead and not here to enjoy it.

All bodies use cortisol to fight off infection. Our bla bodies will fight off infection but it will use up our daily dose of cortisol replacement to do it, leaving us under-replaced and on a path to adrenal crisis. Diarrhea and vomiting both cause dehydration which alters how much fludrocortisone/ aldosterone replacement we have to rebalance our fluids. If a BLAer vomits, dehydration is an issue but the more dire concern is that oral meds won't be absorbed before vomiting again. In this case, we can take 20 mg HC under the tongue, take anti nausea medicine like zofran or phenergan to hold off the vomit. If we can't take extra cortisol by mouth, we must go to the ER 1) for IV fluids due to dehydration and 2) IV hydrocortisone. For diarrhea, dehydration happens but we can easily take oral steroids under the tongue to keep AI and AC at bay. Those of us who have D instead of V in adrenal insufficiency have more time to figure things out. After 4-6 bouts of diarrhea, then we have to go to the ER for IV fluids even if we keep the cortisol dose down. So it is a tricky flowchart to follow but we must do it when we are sick.

Docs tell us to just stress dose with fever. This is not true. Many of us need more cortisol meds for urinary tract infections, simple colds, upper respiratory infections, pneumonia, shingles, pain from kidney stones, etc. BTW I've had all those post op BLA. I needed more cortisol that the 20-25 mg HC i was taking daily. Once, I was feeling very tired and worn down one Monday. All I wanted to do was sleep. Wednesday morning I coughed once. I knew I was sick. I went to urgent care that same morning and I had pneumonia.

Our bodies will tell us it needs more cortisol. We must learn the cues, trust ourselves, take doc advice into consideration but ultimately take meds as we need to, without worry of judgment or repercussion from the docs. They don't live without adrenal glands. We do. All 230+ in the BLA group do. That is why I place 40% of my faith into the answers I get from BLAers, 10% from doctors, and 50% from my experience, knowledge, and instinct. I encourage you to gain enough knowledge and surround yourself with experienced Cushies for you to do the same.

Patient Portals

Back in my day, when I was first testing for Cushing's in 2007, there were no online patient portals. It would take two to three weeks for the results from simple blood tests to come in, so said the doctors and staff. 

Determined not to wait until medical staff called me with results, I bought a fax machine and a second phone line. I wrote my own 800 fax number on the lab requisition in the CC place. I told the labs it was my pcp doctor. They believed me and duly entered that number into their system. 

I would get my results via fax at the same time the ordering physician did. No additional waiting.

How did I know what the results meant? I made it my business to learn it all. Lab Tests Online 

https://labtestsonline.org/  is the best resource I've found that gives simple yet thorough descriptions of the test, the reasons doctors order that test, and most importantly, what high or low results mean. 

I soon realized that doctors and staff tell us results take much longer to come in because they are building in the time it takes for the doctor to review each person's lab work and pass along next steps to their staff: call patient and schedule a follow up, add or change medication, refer to specialist, etc. Then we waited for that staffer to make it through those calls to the patients, adding days of waiting. 

Be thankful that we have patient portals now, and many times, we only have to wait 48 hours after results are released to the doctor before they are automatically released to us. 

Insurance Inspection: It's Open Enrollment Time

Today, I share with you some wise words from a post op Cushie and patient advocate, Susan G. She moderates the Facebook group, The Many Faces of Cushing's. 

Spend some time learning about your health insurance coverage.

"Open Enrollment for insurance will be starting very soon. Now is the time to look over your plan very carefully and make any changes. Look at your provider directory online, and search for some of the major medical facilities (MD Anderson, John Hopkins, Cleveland Clinic, Emory, Mass General, Cedars Sinai, etc). See if LabCorp and Quest are covered medical labs. Look at your out-of-network requirements/costs.

If you are going to aggressively seek treatment (especially surgery) in 2019, find a plan that gives you a wide range of options. For instance, if you know you want Dr. Chiang to perform your bilateral adrenalectomy, move to a plan that lists him as a provider. Your monthly premiums might go up, but that will be less expensive than paying for surgery out-of-network.

Don't assume that the "standard" (more expensive) is better than "basic" (less expensive). Because I am post-surgery, and most of my medical expenses are lab tests, I switched to the Basic plan because they cover labs at 100%. Yes, if I end up having to have surgery the hospital costs will be higher, but probably not much more than I've saved on premiums throughout the year.

Don't assume that the lower deductible plan is better. Look at the various copays and premiums. A high deductible may never be met (if you are fortunate and don't have to see many doctors) but those premiums are a guaranteed expense.

Finally, remember that this is an election year probably one of the most important of our lives. Denying coverage for pre-existing conditions is still a possibility, and Medicaid is on the chopping block. Vote for your Senators and Representatives like your life depends on it -- because this year it might.

Just in case someone is not really familiar with the terminology:

Deductible is the amount that you will be required to pay out of pocket before insurance starts paying anything. 

Annual out-of-pocket is the total amount you will have to pay. In other words, it includes your original deductible and then the amounts that insurance won't cover ($50 emergency room copay, 20% of surgical procedures, etc.). Once you hit the OOP, you pay nothing the rest of the year."

Guilt. Pain. Depression.

I sit on my back porch staring at the flower across the yard.

I hear the birds sing in the distance.

I hear my service dog's nails hit the pavement.

I feel the heat of the intense Texas sun on my body, while tears fall down my neck.

I feel numb in this world.
I feel lonely and unfulfilled.
I barely feel alive.

Why do I feel this way?

I fill the day by playing a few games on my phone throughout the day. World Chef and Best Fiends, if you must know. I scold myself that this is the reason I can't get anything done. I vow to wake up the next day with a plan, a list, with focus. I am so behind. I have to get some things done. So I document all the tasks that need my attention, the bills that must be paid, the items that can no longer go unattended. I go to sleep with some hope that tomorrow will be better.

Then, it's not. It is the same as it was yesterday and the day before that, and the week before that.

That's the way it is with depression. Our bodies are suspended in time -- part of today but not fully --while the world continues to race past us with each second that goes by. We ourselves don't move but we constantly feel the wind of being left behind.

I can't do anything.

I have no motivation -- for even fun things, for important things, for myself, for my family.

Even with a list in hand, I have no 'get up and go.' I reprimand myself to get up out of the bed in the morning, and when I am finally out of bed, I long to go back 'home.' That's all I want to do... sleep. Even when I wake from a three hour nap, the relief I feel is brief. 

My mind caves under the pressure of consciousness. I didn't cook dinner. Again. I didn't go to the grocery store, and I didn't order groceries to be delivered. Again. I didn't send my husband a grocery list for him to stop at the store on the way home, because after 13 years together and with a 10-year-old child, he never knows what to buy for us.

I left my child alone again, with only the company of the TV to stimulate her brain and senses. I feel great guilt over this. TV is bad for kids. Video games are bad for kids. Yet what do I expect her to do when I leave her alone during summer? If I can let it go, I do, but on bad days like today, I realize that my poor child has spent far too many days in front of the TV while Mama slept. I realize that is not the priority for worry today, but each of these thoughts weigh me down, trapped in a world of things I can not do and can not change.

This is my life after Cushing's. I don't know how I got here, and I don't know how to get out. I see no glimpse of my old self, of my old life, and yet I see no path forward for me to reach a new level of happiness just as I am.

I feel the weight of the world on my shoulders. Ok maybe not the world but definitely the weight of my life and that of my family. I don't know how to carry this guilt and anxiety about the guilt with me anymore. I don't want it. I want the problems to go away, and I don't want to feel this way anymore. Yet, I don't know how it will ever go away when so much of the load falls on my shoulders. I have outsourced and paid heftily for everything I can. I pay housekeepers to come weekly to clean the house and wash our clothes. I have already perfected saying no. I say no so much that when I want to do something, there is no one around or nothing to do. 

The remaining jobs are mine, and I can't do them. I don't want to do them. They are too hard. I am a wife and a mother, and I don't feel very good at either.

I'm lost in the mundanity of my world. I don't want to be the inventory clerk of my house. I don't want to be responsible for cooking dinner every day and feeding my family and myself three times a day. I don't want to be asked where something is. I don't want to open to mail and pay bills. I don't want this to be the only thing I do with my day, so I avoid it. When I try, I quickly realize the limitations that Cushing's has placed on my body and mind. The chores go undone for another day.

With so much on my "To do but I don't want to do" list, I never get on to anything else, anything that I want to do, anything that piques my interests and makes me smile a bit. You see, I don't have the energy for those things either. On days when the schedule is empty and I could spend all day working on my interests and hobbies, I don't. I don't feel like it. I think about things I want to do when there is no time to do it. My curiosity isn't in sync with my abilities or mood. I feel guilty about that, too.  

As my day continues, so do admonishments I heap onto myself. Why don't I use my time more wisely? I'm such a phony because I am all talk. I have been talking about becoming fluent in French and moving to Europe, for how long? 30 YEARS?! I talk about going to the French movies that play once a month at the local college. I talk about studying the ancestors in my family tree. I talk about all the ways I need to renovate my house, projects to complete in my back yard and my front garden. I talk about how I have several children's books ready to write and how I just need to write them down. I even have a reminder on my phone to remind me daily to "Write for 30 minutes." Yet, I don't. I don't write, and I don't do any of the things on a daily basis that bring me joy or excitement. I feel guilt over that.

Who can make me do what I want besides me? Who stands in the way of me doing what I want to do? Me.  All blaming fingers point to me. I have 24 hours of time every day, just like you do. I am sensitive to all that I am *not* doing every day. I see around me and I hear the messages... "Live today like there is no tomorrow." I know what I am supposed to do, but I just ... can't. I'm burdened by all the living I'm supposed to cram into one day because those days turn into my life. Yet, I do none of those things that I say I want to do. I just don't want to do anything.

The days pass slowly into the next, with the only difference between them is how late I woke up, whether I managed to wear real clothes or even shower, or am I lying down on the couch or in the bed when my husband comes home. There are no other highlights in my day.

Sure, I have my daughter. As much as I love the dickens out of her, she is learning to be independent. As she does that, she realizes that she doesn't have to agree with Mama all the time. She is her own person, with her own ideas and desires. As outspoken as I am, I still sit here dumbfounded that my daughter who inherited this gene wields it against me. I have such a low tolerance for the arguing. I can't withstand the constant questioning of why I said no. With multiple explanations with more depth, she still demands more of me. I snap to break the intensity. No TV for you. No you can't go to your friends. She stomps off. Doors slam. I sit and wonder how on earth am I supposed to adapt or how will she?  I can't pass down life skills like coping when I feel like I can't cope.

My attempt to squelch the increasingly stressful episodes leave me feeling like a failure as a mother. Why can't I be more patient? She is just a kid. Why does she act that way? She must have learned it from me. She sees my inability to cope with stress, and she is talking back and acting out like I do. Either way, it is my fault. I'm the parent. I'm the mom. My child is a reflection of me.

No one cares that I am sick.

I feel like I can't win.

I don't know when it will end.

I think of all the expectations that I don't meet and I don't care about meeting. Yet the expectations I have for myself, for this life, I just can't let them go.

How do I forget everything I always wanted for my life? How do I throw all that away and dream up new dreams that are so small, they are barely an accomplishment? I have no expectations, and I have no goals. Why? Because I have held tight to small, achievable goals, and when the deadline comes up, I'm a no show. I flake. I can't hack it. I've learned not to make such deadlines for myself. 

I've seen the self-help shows and read the books and articles. I need to be more grateful for what I have. I run through all the things for which I have gratitude. I write it down only to completely forget about it a few days later. My mind is like a sieve. With all the things I forget, why won't I let myself forget all the things I never accomplished and the things I've done wrong?

How do I focus on today, when today is filled with no motivation and wanting to check out of this world with a nap? How do I focus on what I want when I have no ability to achieve my goals?

This is depression. 

Depression is the cycle of impossibility that never lets you see the glimpse of the light saying you can. That it will, somehow, happen for you--that you can do it.

Depression is sitting here wondering why you are just sitting here.

Depression is knowing you are not doing enough with your time and your life and not being able to do anything to self-correct.

Depression is spending weeks like this and only realizing later that you are indeed depressed. No other outward signals tell you that this is happening to you. Again, the weight of this discovery is on your shoulders to resolve, next to the heavy load you carry.

Once I realize I am depressed, I dutifully make the appointments with my psychologist and psychiatrist only to realize it is Friday, and I will have to muddle my way through the weekend and next week until 'the next available appointment' on Wednesday.

When the thoughts are swirling around my head so fast I don't know where else to put them, I come to a blog I have maintained for a decade.  I write it all down. On the one hand, I hope that writing it down will remove it from my head space. On the other, I hope someone who reads this blog will see herself in these words so she knows she is not alone.

I'm just hoping for my mind to quiet and to let me see some sparkle of hope that tomorrow can be different, that tomorrow can be better.

I close my eyes and check out of this world, hoping that my respite from the worry over my pain, emotions, and thoughts will give my body a few hours to reset and start again. Technology teaches us that when things go haywire, log out and try again later.

I have to put my faith in naps, because it is the only tool in my tool box that I can grab now.

I'll deal with all the guilt of inactivity and non-accomplishments later.

For now, my brain and my heart need a break.

I reach for the only thing that quiets my mind from the weight of the indignities.

I crawl into bed for a sweet nap and hope that I can tomorrow.

Cleaning while Cushie

Cushing's causes depression. Not over the way we look but by the chemical hormones our pituitary puts or doesn't put out. Excess cortisol causes depression, too.

Add that to the muscle atrophy and pain many feel when standing still, and simple things like washing dishes becomes unbearable and is then avoided at all cost.

That's why the article, "How to clean your kitchen when depressed" spoke to me. 

Clean in short spurts a few times a day. I'm already in the kitchen. I'll likely clean longer than the time it takes to pop popcorn.

I hope it works for me, and o Hope it works for you, too!

Be sure to like my facebook page: Fight Cushing's with Moxie. Bye for now!

Cushing’s from Steroids

Think back to all your doctors appointments: How many times were you offered steroids for treatment? Steroids are passed out like candy in medical offices, urgent cares, and ERs these days. Every time, the doctors will assure us that we can't get Cushing's from these low dose steroids, even if used long term. We have always heard quite a different story from patients in our online community. Now here's an article from a medical journal saying the same thing. Finally.

Too little or too much corticosteroid? Coexisting adrenal insufficiency and Cushing's syndrome from chronic, intermittent use of intranasal betamethasone

https://www.edmcasereports.com/articles/endocrinology-diabetes-and-metabolism-case-reports/10.1530/EDM-13-0036

NOTE: Thanks to my Cushie friend Susan F for the find.

Be sure to like my facebook page: Fight Cushing's with Moxie. Bye for now!

Initial Cushing’s Work Up

Many tests must be run to rule out the diagnosis of Cushing's, yet we hear from many who suspect Cushing's that their primary care doctors or endocrinologists REFUSE to order these tests because they say Cushing's is so rare that it is impossible to have it. Most just say we have PCOS and give us anti-depressants and metformin.

RIDICULOUS.

Yet, what doctors don't tell us is that Cushing's must be ruled out to make the diagnosis of PCOS. In order to rule Cushing's out, doctors must run tests to gauge the status of all the hormones of the hypothalamus-pituitary-adrenal axis, succinctly called the HPA axis. 

Doctors will try to run thyroid tests and tell you that you are fine. No no no. They just order ALL the hormones produced by or related to the pituitary and adrenal glands.

So, what can you do to ensure the doctor orders the tests and you don't waste your time and money on a doc that won't do anything to help you?

1) Do your homework.
Prepare for your doctor's appointment. Read the tips I wrote 10 years ago about how to prepare for your doctor visit.
Tips to steady your feet

2. Make sure your symptom list is comprehensive. Do not start off with weight gain as your main symptom. Docs will take that and talk about diet and exercise. You will never get to talk again. That doc will go on and on and you will feel defeated. Peak their curiosity by relaying this myriad of strange and seemingly unrelated symptoms. I listed the symptoms in a specific order to maximize impact. 

List of Cushing's symptoms collected by a Cushie

3. Be prepared to be blown off.
That doctor, not matter how good he/she is supposed to be, will act like it is impossible for you to have Cushing's. It happens all the time, to nearly all of us, so don't be alarmed. Knowing this in advanced should make you get serious about the task at hand. That doctor is the gatekeeper who will decide what happens to you next. Please understand that it is your job to make sure you get what you need out of that appointment, namely lab orders or imaging orders. 

4. Are you sick or not?
Sit up straight. Be confident. You know your body better than anyone, and you know something is wrong. Don't let doctor interrupt you and dismiss you. Your life depends on you standing up for yourself. Now is not the time to diminish your symptoms and downplay its effects. If you find your doctor doing that for you,  you must stand up for yourself and get the help you came for.

5. Psyche yourself up and refuse to leave without being properly evaluated for Cushing's.

When the doctor begins to tell you how Cushing's is so rare, remind him/her that doctors are taught in medical school to look for the one diagnosis to explain all the symptoms, not have several scattered diagnoses. Also, tell them about the probability of having Cushing's, and how if all the test results, symptoms, and imaging results suggest Cushing's, it is likely Cushing's. If it walks like a Cushie and cries like a Cushie, it's a Cushie. 

Gambling on a Cushing's diagnosis: What are the Odds?

6. Be knowledgeable and medically aware.
I will list all the labs that will give you the big picture about your hormone health. I know what all of these tests are, and you need to know them, too.  Spend some time on this super helpful site, Lab Tests Online. Search for the test in the search box. Then scroll down to common questions. My favorite section is "What do my test results mean?" This section is awesome because it tells us what both high results and low results mean. I have been using this site for over a decade, and it has been the cornerstone to my medical knowledge.

Print the following list and say to the doctor: 

"I've done extensive reading in the medical journals. The results of these tests I'm suggesting will give us the most comprehensive look at how my HPA axis is functioning." If the doctor says, S/he can order it but won't know how to interpret it, thank them and say, You will understand what the tests means. After all, you have been researching that, too! (right?)

*Tests to check overall health and HPA axis

• Comprehensive metabolic panel (CMP)

• Complete Blood Count (CBC)

• Hemoglobin a1c

• Ferritin

• Vitamin D

• Vitamin B12

*Pituitary and adrenal hormones

• cortisol serum, 8 am

• ACTH plasma, 8 am (drawn into chilled tube, spun down immediately) 

• aldosterone

• renin (drawn into chilled tube, spun down immediately)

• DHEA-S

• IGF-1 (for GH)

• FSH

• LH

• prolactin

• estradiol

• progesterone

• testosterone, total + bio available

• TSH

• free T-3

• free T-4

• reverse T-3

*Cortisol testing

• low dose dexamethasone suppression test

• consecutive days doing urine collection and saliva test on the same day*

      • 24-hr UFC x 4
      • midnight saliva cortisol x 4  (Docs suggest 11 pm but we know 12:15 am works better)
      • midnight cortisol serums x 10, completed at the lab at the local hospital. 

         — this order for blood tests must be on the doctor's prescription pad,
              with patient's name, date of birth, test name and frequency, and diagnosis code.

EXAMPLE:      

Moxie Melissa dob: 11/1/1980

                    

12 am + 12:30 am cortisol serum x 10

E24.9 Cushing's syndrome, unspecified

The way the MRI machine "slices" through the
pituitary at 2-3 millimeter slices.
Source: Oregon Health Science University,
School of Medicine, Diagnostic Radiology

                 

*Imaging (when the time comes, not part of initial workup)

• CT scan of adrenals

• MRI of the pituitary, dynamic protocol
   — Images are taken. Then IV contrast is given while images are taken. This is the dynamic part of the protocol. These images are critical for radiologist and neurosurgeon to see how the contrast dye is absorbed. Normal tissue will absorb the contrast uniformly, but tumors/adenomas will not absorb the contrast.   
Learn more about dynamic protocol of the pituitary.

7. Persistance is key. So is a smile.
Have a "Get It Done," "Won't Take No for an Answer", but "Catch more Flies with Honey than Vinegar" attitude. Persistance is key. So is a smile. 

8. It's not you. It's them.
Remember that some doctors are jerks and decide if they will help you. In the end, it could be them and not you. If you realize you will not get anywhere after concerted efforts, tell the doctor that you are disappointed in this service, and you won't be coming back. Or, like many Cushies have been reduced to, hold back your emotions for as long as you can and burst into tears in your car. It happens, and it isn't your fault.  Read this link about some doctors being bad and you will realize, this is an uphill battle even if we didn't have Cushing's. It is just a dire situation once we finally find the name to what ails us after so many years. 

Condescending doctors and how to deal with them

9. Graphics for diagnosis
I've included this graphic only as a sample, not as the gospel. Why? Because this and the many graphics found in Google images only apply to patients who are FLORID, meaning cortisol results will all be high. These processes do no apply to CYCLICAL patients like me, who despite having normal UFCs and suppressed on the low dose dexamethasone suppression test, STILL DO HAVE CUSHING'S.

Source: American Association of Endocrine Surgeons

My very own CSI: Cyclical Cushing's

*~*~*~*~*~*~*~*~*~*~*

I hope this helps prepare you for your visit to the endocrinologist. Despite Cushing's being an endocrine disorder, these doctors are convinced before you even talk that you don't have it. They can tell just by looking at you! Understand the wall that stands before you, and figure out a way to get to your goal: a proper Cushing's work up.

Be sure to like my facebook page: Fight Cushing's with Moxie. Bye for now!
x

        

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!

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: 

• American Association of Endocrine Surgeons
• Cedars Sinai (Los Angeles)
• Emory University (Atlanta)
• John Hopkins 
• Massachusetts General Hospital/ Harvard
• Mayo clinic
• NIH: National Institute of Diabetes, Digestive, and Kidney Disease
• Oregon Health and Science Center
• Pituitary Network Association's "list of the top pituitary and hormonal health hospitals in the world"
• UCLA
• University of Texas Southwestern (Dallas)
• University of Virginia
• Vanderbilt

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:

●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
  
• bursitis (my most recent addition)

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, groin, buttocks or under breasts, stomach flab or 'pannum'

• skin tags

• psoriasis

SLEEP TROUBLE/ FATIGUE
• exhaustion/ extreme fatigue plus insomnia; want to sleep but can’t

• extreme daytime fatigue

• extreme fatigue in afternoon, then second wind that keeps you up all night, crash at 4-6 am, wake 10a-12p
• 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

• night sweats, sometimes drenching clothes

MENTAL HEALTH
• personality changes
• anxiety 

• irritability

• hostile/ short fuse

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

• 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

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

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

• high blood pressure

• cardiovascular disease 

• heart arrhythmia

• tachycardia

• 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
• keratoconus (steepening of the cornea)

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 (amennorhea)

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.

I've included some articles I wrote to help get you started here: Initial Cushing's work up

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 I post here on my blog. Also, feel free to search for me on Facebook at Moxie MelissaTX or moxiemelissa at symbol gmail dot com.  I am happy to answer your questions, help you find the right type of group for online patient support. We even have groups for patients in every US state!  There are Cushies everywhere. Be sure to create a safety net around yourself and get support. No one understands how terribly devastating and life-altering Cushing's is like other Cushing's patients.