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.

Monday, May 26, 2014

So you think you have Cushing's? Here's what to do...

A link with suggestions from people who've been there, done that...and who really do know their biznatch:  Cushiwiki  This will link you to page two of a three page game plan.  Buckle down; it's going to be a bumpy ride but you CAN make it to the other side!