Sunday, November 30, 2014

LA trip: MRI, LFT's and Keto, Dr. F, AGHD, friends and a documentary

Yeah, so that is a really long title, but I have a lot of updating to do on many subjects!

If you remember, Dr. F (my endocrinologist in LA) put me on Ketoconazole at my last appointment, gave me lab slips to check my liver enzymes after about 2 months on the medication, and gave me an order for a repeat MRI after about 3 months of treatment with keto.  You see, it appears that when keto messes with the feedback loop of ACTH and cortisol, it can sometimes spur the tumor to grow or produce more, making it more obvious.  I just say "makes it angry", so now you'll know what I mean by that.  But Keto also commonly causes liver damage, making the liver "angry" too.  (And this is the safest and least damaging of the few medications available for Cushing's; to my knowledge of the others, they involve multiple organs and can be really harsh).  I was on keto after my initial diagnosis in 2013 while I waited for surgery, and had no real problems.

Anyway, there are no 3T MRI machines in "Nowhere, Alaska", and no roads out.  The closest is Seattle and that place charges boatloads for their "magical" MRI's, making it cheaper by far for me to pay the added expense to fly to the Los Angeles area and have my MRI done there.  Plus, I like to.see Dr. F while I'm in town as in-person appointments just work so much better for me than phone ones or email ones.

So the second week of November, Britton and I flew down for my MRI and appointment.  We were lucky and were able to meet with a number of "Cushie's" while there (Friends I've made online, most I'd not met in person yet, all who have or had Cushing's Disease) which made the trip just amazing.  I'll talk more about that later and start with the medical stuff.

So, my MRI went well.  The machine in Torrance is SO much more comfortable than the GE one I was in at MDA, and this one didn't try to burn my arms at the end, though their headphone music system wasn't working so I had to spend an hour with my thoughts and some really loud noises with nothing to look at (no, falling asleep isn't an option for me; I wish!). 

When we met with friends at the hotel that afternoon, we put my disc in their computer and sent a drop-box copy to another "cushie" friend who loves to read MRI's as a hobby (not an MD, but she's pretty dang good).  There were new signs of empty sella, which means my pituitary is flattening, and there is (Cerebro-Spinal Fluid) CSF in my sella.  Empty sella syndrome tends to lead to hypopituitarism.  So far I'd been okay and my pituitary was still functioning normally...though that isn't to say it won't lose function over time.  There is no way to know, we just have to watch symptoms and test routinely as time goes on to see if things worsen.

There were two other things noticed...a possible new tumor on my MRI, when compared to my old pre-op MRI (different spot but same side of pituitary), and some crazy stuff going on in my sinus that looks like it is full of bubbles (on CT in August it looked like a solid mass about 2cm by 2.5cm, that the ENT's thought was scar tissue).  None of us had seen that before, and my friend called it a "train wreck."

At my appointment, we discussed my liver function tests (LFT's), which I had drawn the week before and had come back significantly elevated (like 5 times the high end of the normal range), and I was taken off of ketoconazole.  Luckily I'd swung into a low cortisol cycle the week before and had stopped taking it because it was making me swing too low and I hadn't restarted yet. 

Not surprisingly, Dr. F viewed my MRI and made the same statements my friend had (aside from the sinus one).  He suggested I send my MRI on to Dr. McC (neurosurgeon in Houston) to get his opinion on whether that was a target (ie, tumor).  The radiologist later called it "changes from surgery", but he also admitted to missing the original tumor on my very first MRI, so I'm waiting for an expert to weigh in on that.  Dr. F also wanted to retest my thyroid and growth hormone (GH), because those are usually the first two hormones to be affected by pituitary damage (the empty sella).

My thyroid is still happy, but my IGF-1 (the test they run to check GH) had dropped by half and was now well below the normal range for my age and gender.  I'm not sure if my low IGF-1 was affected by my liver damage, so we are retesting my liver weekly and then will go from there.  If that IGF-1 stays low, I will need a GH stim test to prove my pituitary isn't capable of producing enough (for the insurance company who HATES to pay for this expensive medication --and it IS expensive) and then I will go on daily injections for life to replace the hormone my body can no longer make.  (FYI, a stim test is where they force your body, through medication, to make as much growth hormone as it can and test at exact time intervals to see your body's response.)

Here is where you say, "Growth hormone?!  You're 33!  Surely you've stopped growing by now?  Why would you need daily GH?"  Well, here's why: Adult Growth Hormone Deficiency (AGHD) causes all kinds of problems.  It's like cortisol in that it does a specific job, and that's all we tend to think about, but when it is out of whack, all KINDS of body systems suffer.  Just to name a few, it leads to abnormally high cholesterol and thus cardiac/artery issues.  It leads to thinning bones and muscle loss.  It leads to accumulation of visceral fat.  The list goes on, and it is not pretty.  Combine that with the big list of what Cushing's does, and some of those equate to a double whammy.  I'm 33, I certainly don't want a heart attack or osteoporosis already!  The side effects of taking too much are swelling or pain in the hands...when that hits, you can simply lower your dose.  Not hard to balance those risks/benefits, eh?  And just in case you're wondering, it is taken for life because pituitary's aren't known to recover.

Livers, though, can.  I've since tested on the 20th and 26th, and from the 7th to the 26th, my LFT's dropped by half!  Half of that in those last 6 days!  It looks like the momentum of my liver's recovery with the medication out of my system is improving and I'll be within normal range in no time.  I will test those again this next week and see how I'm doing (Dr. F's suggestion), and then we can progress with testing for GH and be certain the results will be accurate. 

If I need a stim, it cannot be done locally (I've called everyone including the hospital), so I've already contacted Swedish and would have it done by Dr. Yuen there.  He's an endocrinologist on Dr. F's recommended endo's list who used to work out of OHSU in Oregon and recently switched to working at Swedish's pituitary center.  YAY!  His scheduler said all I would need is the order from Dr. F and/or a referral sent to them and I could schedule my GH stim with about a week wait between making the appointment and having the test (Minus the winter holiday weeks).  Bonus: He's done MANY studies on growth hormone. 

And something else that is awesome?  Both a Cushie friend and an AGHD/hypo-pit friend (that I met at the Magic Foundation's adult conference this April) live in the general area and have offered me a place to stay!  Obviously this calls for two trips, right?  (Oh, I wish!!!)

Hmm...what else?  Oh, friends and the documentary.  Well, that part was just awesome, but this post is already long and wordy so I probably should save it for a future post.  CLIFF HANGER!

ETA: I forgot to mention that I am also retesting for Cushing's, to see if my testing is more definitive for a repeat diagnosis (and moving on to repeat surgery).  It'll take me some time,'s a great many tests that must be done while I'm in a high cycle.  Add to that that neither lab in town can do all of the tests (each can do some of my list, meaning all get hit but I have to juggle between them) and they have different drop off restrictions for time of day and days of the week.  Thanksgiving didn't help that, either.  ;)

Friday, September 26, 2014

Persistent Cushing's Disease -Update 9/26

I was able to get into an ENT at Cedars-Sinai and it turns out that the sinus infection is gone, but I'm left with a LOT of scar tissue in my sinuses that is very easily inflamed.  Fun times!  This MD actually scoped me to see what is going on up there.  He didn't swab it either, but that's understandable if the infection is gone.  I'm to try nasal steroids (endo okay'd it), use a humidifier, not use any fans, A/C, etc., Sudafed when I get congested, and contact him again in a month or two if that doesn't work.  It isn't really working, but we shall see.  I'm not feeling well enough to fight that battle right now, though I change my mind every time the sinus pressure becomes severe.  :)

I saw my Endocrinologist as well; he agreed I am still sick, but my testing is too mild to move on to a BLA.  (I knew it, but, UGH!)  I am on ketoconazole now to lower my cortisol, but mostly because it disrupts the feedback loop and can "anger" the tumor.  This can lead to tumor growth (so that a target shows up on MRI) and/or the tumor can start to ramp up it's production, overpowering the keto's effects.  I had this happen last year when I was on keto.  After about a month or so, it stopped working as well to lower my high cycles and I had to up my dose.  Sometimes my endo uses this medication like a further test for proof of disease.  And when that overpowering happens, going off the keto and retesting can yield much more definitive test results. 

So anyway, I'm trying to get on the dosage of keto he wants me on.  With my cyclic disease and the bulk of my tumor gone, my high cycles are lower (though still too high) and my low cycles are lower as well.  It's been a challenge, but I'm keeping at it.  After 2 months on the medication I will do some lab testing to check my liver function to be sure I'm tolerating it well in that regard, then in November I will have a repeat MRI and follow-up appointment to reassess the plan.

If a pituitary target does show up, I will move on to repeat pituitary surgery (he might want me to go off the keto and test another cycle first, who knows?).  This isn't the best option, but it isn't the worst (medication).  2nd surgeries have only a 50% remission rate, a significantly higher risk of pituitary damage causing hypopituitarism (and requiring lifelong hormone replacement), and a much higher risk of CSF leaks (requiring repair, not being upright, etc). 

If a target does not show up by November, he may have me stay on medication (I pray to God that is not the case!), he may have me go off medicine and retest to see if my results are far more definitive allowing for BLA clearance, or he might consider that as ample proof along with my lab work and allow me to move on to my bilateral adrenalectomy surgery. 

There you have it! The keto definitely helps in some ways (I can sleep at night), but it brings me too low during the day when my cortisol levels are already lower than they should be (due to the flipped diurnal rhythm of Cushing's Disease).  I'm playing the waiting game and hoping it ends soon.

Friday, August 8, 2014

Update August 2014

Well, I guess I should update everyone who might be reading.  My local friend, Amber, is traveling to see Dr. F for a new patient appointment, and Britton decided I should go with her!  I'll be getting my own follow-up appointment as well, in about 3 weeks.  We're both "invalids" but in different ways, have similar schedules and a very similar love of good food.  I can't wait! 

I now have 8 diagnostic high tests.  I have 6 diagnostic 17 hydroxycorticosteroids averaging double the range (but ranging from just decimal points over to three times the high end of normal).  At least these are consistently coming back high, showing there is indeed an issue, but the testing might be a little one-sided for BLA surgery clearance.  I have one diagnostic midnight serum of 8 (in a range to 7.5) and one high 10 hr UFC.  I have a feeling with these he will want me to go on medication for a while, especially since I'm going downhill so quickly with my mobility, etc., but I am STILL really hoping for surgery this fall.

I've had to stop testing to treat my sinus infection.  It is a little overly-attached if you ask me.  I'm on antibiotic #4 now, probably day 50 of antibiotic treatment so far.  It looks to be sphenoid-based, and after this last 30-day stint on med 4 is completed, I'll be getting a CT scan and ENT appointment to see whether it is gone or if I need a procedure to help drain the sphenoid sinus. 

We fly out the week after to see Dr. F.  I am REALLY looking forward to seeing him in person and at the very least, getting a good game plan together, even if it isn't what I am hoping for.  And, I am looking forward to a bit of a break from my day-to-day to spend some time with a friend relaxing, eating good food, and trying not to get too hot in Beverly Hills in August!

Wednesday, August 6, 2014

Pituitary conditions, PNA letter to families and friends

Dear Pituitary Families and Friends,

For your sake and the sake of your loved one, we ask that you take a few minutes to read this article. You may think pituitary patients are difficult and tend to complain a lot, but please let us explain. One of the hardest things for pituitary patients is the fact that there are a lot of unknowns. There are multiple symptoms that may (or may not) occur. The s...ymptoms may subside for a while, only to come back unexpectedly, much worse than before.

Exhaustion, fatigue, weakness in limbs, loss of libido, infertility, headaches, excessive sweating, apathy, anger, rage, depression, mood swings, uncontrollable weight gain, diabetes, and visual disturbances. These are just some of the symptoms of a pituitary disorder. Taken individually, these symptoms can be debilitating, but together they can be devastating. Can you imagine the burden on the patient if they also lose support from the people they depend on the most?

Symptoms such as the ones listed above can adversely affect the family unit. The apathy for example, can make family members feel like the person is disconnected or doesn't care what is happening with everyone else. Because of the disease, the patient may not even recognize that he or she is behaving this way. In addition, anger, depression and uncontrollable rage can also make the family feel like they are under siege: abused, unloved, unappreciated and unneeded. The fact is, this is when pituitary patients need their family the most. It's understandable to want to pull away and retreat emotionally, but that just ends up isolating the patient even more. These are the times that your loved one needs you to stand by him or her and try to understand that the disease is controlling the bad behavior. If your loved one had cancer would you accuse that person of being lazy or making up the symptoms? Of course not, but this happens regularly to pituitary patients. We all know that cancer is a frightening disease that does horrible things to patients. Many people do not know about pituitary/ hormonal disorders and the negative effects it has on a person's everyday life.

In times of crisis, the family dynamics become magnified. Patients dealing with a diagnosis of an unknown intruder in their body need the support and understanding of their family more than ever.

Now imagine that the intruder is unseen (have you ever tried to make sense of those MRIs?) and not clearly definable. When people hear that their loved one has a pituitary tumor, but that it is not cancerous or terminal, they are relieved and think that everything is going to be okay. Unfortunately, what they don't know is that the pituitary issue will affect every aspect of the patient's life, possibly indefinitely.

When people hear about a tumor, they don't think about how it diminishes the patients' mental health, their quality of their life, or their ability to accomplish everyday tasks. Pituitary tumors are usually benign, but they do cause some severe symptoms and they can require a lifetime of treatment and monitoring. Cancer is something that you can fight and BEAT, but for most pituitary patients, the battle goes on and on and on.

Understanding and acceptance truly comes from knowledge. The more you know about the disorder, the better prepared you are to support your loved one through his or her daily struggles. It can be very frustrating to have to care for someone who is ill, to have to pick up the slack with the daily chores they used to be able to do. When a patient is diagnosed with a pituitary or hormonal disorder, it is important that the family comes together to develop a plan of attack. Someone faced with a chronic illness will be able to handle it much better if they have the necessary support. The first and most important thing that any loved one can do is get educated about their loved one's disorder. Read the information contained on our website, purchase our Pituitary Patient Resource Guide, and learn as much as you can from trusted sources. Our information is compiled from the world's renowned experts in pituitary medicine.

The more you are aware of up front, the better equipped you will be to make decisions. Your family will have to make adjustments. This is not going to be easy, but it can be made less stressful. Within the immediate family/household, all roles need to be assessed and redefined if necessary. For example, the person with pituitary disease may not be strong enough to carry out their regular household chores or do the same amount of driving. They may be too scattered to effectively manage the family's finances or schedules. Their emotions may get out of control at home or at work.

If you know going in what changes to expect, they are much easier to accept and adapt to. One thing that is very important to note; if family and friends are not supportive, if they do not believe the patient when he or she reports struggles caused by this disorder, it can exacerbate your loved one's symptoms. The fatigue and strain that they go through will be amplified if they have to justify their symptoms to people. Sometimes people are accusatory and assume that the patient is just lazy. Patients are told that they'd probably feel better if they just ate a little less and exercised a little more. This kind of attitude undermines the patient's treatment. There is a medical explanation for their symptoms and for their inability to live a normal life. It is not just another excuse. They did not choose this illness. They do not want to miss out on family functions; they don't like that they can't do their part. They cannot control their symptoms, so they need your understanding. Thank you for everything that you do to be supportive. The PNA is here to support you in your quest for information. Please contact us with your questions and we will be happy to point you in the right direction.

Message to Pituitary Families
By Tammy Mazzella, Pituitary Network Association

Friday, July 11, 2014

What it's like to see a doctor about Cushing's:

I posted this a year or two ago, but that link disappeared, so I'm reposting now that a friend has re-added it! 

So, not all doctors are like this, but MANY are...and most endocrinologists (that should be more educated about this disease) are as well, sad as that is.  There ARE some good ones out there, and we flock to them for help.  They save our lives and we love them eternally for it.  My endocrinologist (one of the ones we all flock to) says that the majority of his patients end up knowing more about this disease than their local endocrinologists do. 

Still, this is too true of our collective, ongoing experiences to not share:

For some reason, it isn't letting me insert the video so you'll have to follow the link.

Even now, after a diagnosis, surgery, etc I am leery of seeing doctors and discussing it, despite LOVING to share the information and help others.  I have ample proof I was and still am ill.  But the vastness of how this disease affects one's system is astounding, so no matter how small the reason for seeing a doctor, everything is impacted by this disease and they end up asking a LOT of questions because they don't know much about it.  When you're sick (on top of your debilitating disease), it's tiring to have to educate your doctors.

Friday, June 13, 2014

Youtube Video

Brief patient interviews were recorded at the Magic Foundation conference in April to be posted on Youtube, as a way to help raise awareness for our diseases.  Here's a link to mine:

And a link to my friend Alicia's:

She's been cleared for her BLA and will be having surgery and hopefully on her way to wellness soon!  I hope to follow shortly behind in her footsteps!

Wednesday, June 11, 2014

Update: Post-op pit, not in remission

It's been a while since I've updated with my own status/progress, so here goes:

I had pituitary surgery on 2-5-2014 and am now 4 months post-op.  We knew early on that it didn't look like remission, I was off my replacement hormones within a month, had to wait for my cycles to normalize a bit (I guess all that pituitary fileting was noticed! lol) and about a month ago now I started testing in earnest for my re-diagnosis.  In April I had a post-op MRI and follow-up with my neurosurgeon, who said I did not have a visible target on MRI, so I wasn't a candidate for repeat surgery or radiation therapy.  So, we now know that a bilateral adrenalectomy (BLA, the surgical removal of both adrenal glands) is in my near future...but I need a multitude of lab tests to prove I need it, and give a surgeon enough reasoning to permanently remove two very vital little organs and put me on life-sustaining medication instead. 

After intensive testing one week, and hit/miss testing the next (I was cycling lower and thus stopped testing), I now have 5 diagnostic-level high lab results.  Because of the severity and permanency of this next surgery, my endocrinologist has asked me to keep testing.  If my pattern holds true (it's never perfect), then in about a week I will start testing again in the hopes of doubling the number of diagnostic-level highs that I have and move on to the surgeon referral process.  It'll take a couple of weeks (2-4) to get my lab results back, and another couple of weeks to get my endocrinologist appointment after that if I do get sufficient highs.  I'm *really* hoping he won't make me go on medication prior to surgery as they all have their (rather large and often permanent) side effects, and I'd like to move forward towards a permanent cure and health!  Not to mention, my deductible is met for the year, so this year would REALLY be nice. 

The surgeon I'm leaning towards is in Wisconsin and has a boatload of experience with this surgery.  The runner up is in Seattle's Virginia Mason (closer to home), but he has a lot less experience with BLA's and rest tissue testing/surgery.  We'll just have to see how things play out and when.  We're hopeful, and I'm starting to allow myself to plan for a near-future in which I am somewhat functional and active again.  I can't wait!  Once again, it sounds silly to be excited and wishful about having surgery to give me Addison's disease, just as it was to be thrilled to be told I had a tumor, dreaded disease, and needed brain surgery.  But, I've been sick for so long and becoming more and more debilitated and sick the longer this has gone on, I am excited at the prospect of any semblance of improvement, health and normalcy!  (Okay, within reason...I am well educated and using logic, etc on this, but...YAY!)  I can feel it is within my reach again.  I'm on the path and moving forward.