Three Years Ago Today

Three years ago today Alex had her adrenal glands removed. Four weeks ago today she had an ablation done on the suspected area of rest tissue. It feels like today is a day I should do an update...


As of the past two weeks I'm having some serious doubts that the procedure worked. At the two week point I was feeling oh-so-confident. Not so much any more. To begin with her weight dropped a few pounds and she was feeling much better. She had no new acne and was sleeping at night. Sadly, over the past 2 weeks her weight has gone up about 10 pounds. She has actually been feeling pretty well, so that's obviously a positive sign but there have been a few nights when she seemed pretty ampy and a few days where she was having some pretty big mood swings. 


She's been on 15 mg of hydro since a couple days after the procedure. That's the same as the lowest dose she has ever been on since the BLA. After the recent weigh-in she dropped 2.5mg off of her afternoon dose to bring her overall dose down to 12.5. I'm definitely worried. Again. 

So I Don't Forget

Before I forget everything that's happened I need to write it down. I know there will be others that follow us down this path and I want to be able to share accurately. 


Two months ago Dr. Friedman in LA referred Alex to Dr. Schaffer at Scripps Green Hospital in La Jolla (at my request). Dr. Schaffer was kind enough to look at Alex's abdominal CT (I asked his permission to mail it to him) before meeting with us. He also asked his interventional radiology team to take a look at the disk. The team agreed that there was an area they found suspicious and we met with Dr. Schaffer last month to discuss options. Dr. Schaffer though an ethanol ablation would be the best choice and we agreed. I want to note here that the UCSF team did not see a worthy target on this same CT.


We returned home and scheduled the procedure for June 18th. During the month at home UC Davis finally got the (2) PET/CTs with cosyntropin stimulation scheduled and completed. Two radiologist and the local endo viewed the scans and agreed that nothing "lit up" to indicate stimmed adrenal tissue. This was disappointing news but we decided to move forward with the ablation despite it. Incidentally noted on the CT was a pulmonary nodule that we will need to have further evaluated. 


The procedure was scheduled for Monday so we flew in on Sunday. The people at Scripps seemed organized and efficient. The only thing that put us behind schedule a bit was a last minute pregnancy test (if you are female and over a certain age...). They gave Alex a nice cocktail of 100mg solu-cortef, Versed, and Fentanyl and whisked her away. The procedure was CT guided; they went in through her back, next to her spine. The nurse said Alex took a nice little nap and Alex remembers nothing. The procedure took about 30 minutes and two hours later they were ready to release her. She was in some pain when the meds wore off and they allowed her to take a dose of Ultracet. 


The recovery hasn't been bad. She has only taken the Ultracet once since that first day and has taken 600mg of Motrin twice. On Tuesday she took a total of 30mg of hydrocortisone and by Wednesday and Thursday she was down to 20. She managed the next 2 days on 15 mg but yesterday was feeling she needed (and took) an additional 2.5mg. 


I'm feeling cautiously optimistic that it worked but I want to give it a few days before I say more.

San Diego Round One

We flew in to San Diego on May 10th and saw the surgeon, Dr. Schaffer on the 11th. He spent a long time in the exam room with us discussing different type of ablation and why he thinks ethanol is the best choice. He reviewed Alex's abdominal CT with us and pointed out the area he thinks may be adrenal tissue. He described how they would do the procedure and what the risks involved are. The appointment went really well and he referred Alex to the Interventional Radiologist for the procedure.

And then we waited. And waited. And waited. I called the doctor's office ever day at least once a day. I called the scheduling coordinator, I called the authorization coordinator, I called the new insurance plan, I called the old insurance plan. Ground Hogs Day. Day after day the request got turned in to our old insurance and denied (duh). We knew they wouldn't accept the old insurance. I still don't know if it was human error or computer error but after a week I finally got the admission department to completely delete the old insurance info from their system. We flew home that Friday and the authorization request was finally delivered to the correct insurance that day.  After waiting an entire week in San Diego the approval went through the same day it was submitted.

Finally on Monday they were able to get the authorization to the IR scheduling department. They offered us an appointment on the 29th, but airfares so close in would have cost us $1000.00 and I just couldn't  do it. The following week Alex has 3 days of testing she can't miss. The week after that the doctor is on vacation. So, she is now scheduled for June 18th. Alex doesn't mind the wait but the mood swings she is getting with the high cortisol are about too much for me!

The Final Day ~ Cushing's Awareness Challenge

Thirty posts in thirty days; I did it! 


When I initially started blogging, years ago, it was really for my own mental health. It was a way to get things off my chest, and a journal, so that I could look back. I never intended to share it beyond   some limited boundaries. Over time, I've been contacted by several strangers (who have somehow found me) searching for answers to their own health problems and I've come to realize it is doing some good; I am doing some good. It's been frightening to share with friends and family this month but if a single person benefits, well, that's enough. 


Since this has all been about awareness I wanted to note: in the past 30 days I have had 1353 page views (my own aren't counted). Thanks for dropping by!   

The Financial Aspects

I know, personally, the monetary impact a chronic illness can have on a family, and I'm reminded time and time again, as I see friends who have no other choice but to declare bankruptcy over medical bills. As if illness isn't enough of a burden on these families the financial strain can be devastating. I don't think most people realize the financial damage illness can cause a family or individual until it happens to them.


Even with insurance, the costs can be monumental. It just doesn't seem right to me that as a family of four we are paying $1447.00 a month for insurance. On top of that, add high deductibles that must be met, office visit co-pays, a 30% "share" until we hit the max out-of-pocket each year, doctors who are not covered by insurance, and lets not forget all the prescriptions with their separate deductibles and co-pays.  


In an article  from CNN Health : "This year, an estimated 1.5 million Americans will declare bankruptcy. Many people may chalk up that misfortune to overspending or a lavish lifestyle, but a new study suggests that more than 60 percent of people who go bankrupt are actually capsized by medical bills.

Bankruptcies due to medical bills increased by nearly 50 percent in a six-year period, from 46 percent in 2001 to 62 percent in 2007, and most of those who filed for bankruptcy were middle-class, well-educated homeowners, according to a report that will be published in the August issue of The American Journal of Medicine."

We have been lucky, though we do have a continuous stream of medical bills coming in the mail it hasn't driven us to that point. Yet. Here I should note: I was wary of sending Alex's CT to San Diego; the doctor is not  a provider on her insurance plan and her plan offers zero "non-preferred provider coverage". It appears that the hospital there is, and our hope is that the procedure will be done at the hospital, instead of at the clinic location, so the majority of the cost will be covered as a hospital procedure. Alex will be a "cash pay" patient with the doctor as she is with her endocrinologist in LA. If insurance denies the procedure I will be plotting a way to pay cash for that as well. 

Why Me? Why Indeed

Initially I thought "Why me; why Alex; what did we do to deserve this?"

I eventually moved on, to thinking God was trying to teach me a lesson~ about patience (it's a lesson I'm still working on).

Finally, I concluded, God knew I could do this. He trusted me to take care of Alex. He trusted me to fight and find answers. He knew I had the strength even when I didn't know I had it.

I've come to believe that this is what I am meant to do. Not only am I here as Alex's guardian,  I'm also here as an advocate/supporter to other Cushing's patients as well. I think it's my calling.

Where To Go For Help

I want to share a couple important links today.
First: http://cushings-help.com/
Without cushings-help, and all the great people I met on the message boards there, I doubt that Alex would still be alive. If you think you (or someone you know) might have Cushing's, I urge you to join the message boards (it's free) and learn more.
Second:http://www.goodhormonehealth.com/
This link takes you to Dr. Friedman's web site. Dr. F is the guru for nearly all the Cyclic Cushing's patients I know. You can research info on his web site, learn more about his practice,or even book an appointment.

But You Don't Look Sick

The "Spoon Theory" by Christine Miserandino is a Must Read
http://www.butyoudontlooksick.com/articles/written-by-christine/the-spoon-theory/


While Christine, who wrote the Spoon Theory, suffers with Lupus, the theory is applicable to all "invisible" diseases. The first time I read this I just sat and bawled; it fit so perfectly. I printed it out an took it to Alex who was laying in bed, low on "spoons" and we cried some more. I wanted to print it out and send it to everyone I ever met. I hope you will take the time to read it. 


In the Cushie Community we reference "spoons" on a regular basis: "I'm low on spoons", "do you have any extra spoons?", "I don't think I have enough spoons to make it through today". If you've ever heard of anyone talking about spoons as if it's some code language, now you will understand.


Got Spoons?

Rest/Remnant Tissue

It's a medical fact that cortisol is normally made only by the adrenal glands. Alex had her adrenal glands removed  to prevent her body form making cortisol. So the question is...where is the cortisol coming from?There are basically 3 possibilities:
1.) There is a tumor somewhere in her body making cortisol. This is called an ectopic source. It is highly unlikely that Alex has this type of tumor. All of the testing done prior each of her surgeries as well as her pituitary pathology contradict this idea. The fact that she had a 2 year remission after the removal of her adrenal glands is another reason to believe this isn't the case.
2.) Adrenal remnant tissue can be left behind at the time of surgery. In some cases an inexperienced surgeon can leave behind some of the adrenal tissue. Alex's surgery was over 6 hours long, her surgeon is highly respected for this type of surgery and he explained how he removed extra tissue around the adrenal to prevent this from happening. I don't feel like this was a "botched" surgery, but I think it possible that a remnant could have been missed. Her pathology indicated a ruptured cyst, possibly when it ruptured a bit of tissue migrated away from the adrenal? That's speculation. The surgeon also told us immediately after surgery that Alex had very large "sticky" adrenals, they were adhered to her other organs. It seems like a bit could have been missed there as well.
3.) Rest tissue is our top contender. During embryonic formation the adrenal gland "migrate" before they get to their final destination atop the kidneys. 
From this article
"Adrenal rests (AR’s) or adrenal rest tumor is a tumor consisting of ectopic adrenal cortex tissue. The lesion is relatively common, detectable in as many as 20% of post-mortem examinations." 
Our assumption at this point is that one of these pieces of adrenal tissue has been stimulated by ACTH to the point that it is making significant amounts of cortisol. This is what we hope is showing on the CT and what the interventional radiologist hopes to ablate.

Flying On Faith

Yesterday morning I received an email from Dr. Schaffer in San Diego (actually I received three within an hour). In his emails he agreed that an ablation would be the best option for Alex at this point. He has discussed Alex's CT with his Intervention Radiologist and they are prepared to give it a shot (pun intended). He asked that I call his office and set up an appointment. Yesterday I was able to get Alex's information entered into their system but I had to wait until today to get anything scheduled.


This morning when I called they were able to schedule an appointment for Friday, May 11th. But, they checked with our insurance and found that they can't even  initiate authorization for the procedure until Alex actually meets, in person, with the doctor. They can't make any promises but their plan is to try to get authorization as soon as the appointment is complete. Our plan is to stay and hope for the best. Insurance could deny the procedure, or they could drag there heels on authorization. Our only other option would be to fly home and go back again later which doesn't seem like a cost effective choice (but then again it may be). 


I'm an organization fanatic, so this is really a hard one for me. Of course, I have to keep in mind that there are no guarantees the procedure will work either. I'm really trying to "let go and let God". 

Cortisol Production Post BLA

We are told that bilateral adrenalectomy is going to be "the cure" once and for all, and in most cases it truly is. But, I have been researching more on this subject sine Alex's recurrence. One of the articles I found click here states "Residual adrenocortical function have been demonstrated in 9/37(25%) after TBA for CD". At UCSF the endocrine department and tumor board told us "we don't know what normal cortisol levels are post BLA, no one ever checks". Their point being, people get better and there is no need to check for a hormone that should no longer be present. 

Once again, I have a theory. Assuming that the vast majority (90% ?) of  BLA patients had "florid" Cushing's (as opposed to cyclic) prior to BLA, then we could assume that any cortisol produced post BLA would be in a normal pattern as well. Based on this assumption, all it would take would be a decreased dose of steroid to solve any problem. 

In the cyclic patient population we are seeing a higher number of patients experiencing problems with cortisol production post BLA. Here, my assumption would be that the naturally produced cortisol levels are again fluctuating like they were prior to BLA. With this cyclic production, cortisol supplements can not be accurately determined and once again patients find themselves in limbo. 

An Analogy To Explain

If the tumor is in her head why did they remove her adrenal glands? I'm going to use an analogy to explain but let me give you a little background first.


By far, most cases of Cushing's Syndrome are exogenous~ meaning they originate "outside" the body. If a person takes corticosteroids to treat a disease, be it anything from cancer to asthma, an excess of those steroids can cause Cushing's. In these cases the patient can normally be weaned off steroids and carry on with life.


On the other hand, with endogenous production~ the steroids are produced by the patient's own body, in excess. We need these steroids, cortisol specifically, to survive, but too much is a very bad thing. In most of these patients a pituitary tumor is the culprit. The pituitary, a tiny pea size gland at the base of the brain, secretes ACTH which signals the adrenal glands (bean sized gland that sit atop the kidneys) to make cortisol. When an ACTH secreting tumor is present in the pituitary it produces an abundance of ACTH and the adrenals overproduce.


In Alex's case the neurosurgeon wasn't able to remove all of the pituitary tumor. She has pituitary hyperplasia; the gland is riddled with fragments of tumor. I've heard it described as "appearing like the marbling of fat in a steak". Because they were unable to remove the source of the ACTH they instead removed the end target, the adrenal glands.


So, here's my analogy: The pituitary is a radio signal tower, it sends out a signal to the radio (in this scenario your adrenal glands). In Alex's case the "signal" was sporadic ( the radio would switch randomly from whisper quiet to pounding decibels). First they did pituitary surgery to try and repair the "tower/signal". It  didn't work. They said they could try again but the chance of success was less than 50%. As an added problem, whether or not they could "repair the signal/tower", it was likely that they would to do further permanent damage to the pituitary or surrounding structures (the TV wouldn't work either). The only other available option was removing the adrenal glands, or "throwing away the radio". We chose that option. Life without radio isn't as enjoyable as life with radio but it's better than a radio that gets stuck at high volume and can't be shut off.

Maybe He Sees Something!

Last night I received an email from the surgeon in San Diego.  I've been waiting on pins and needles for the past month.  Here is part of what he had to say:


I believe that there may possibly be residual adrenal tissue on the left side in the area of the prior adrenalectomy...


The tissue demonstrating increased enhancement on the scan ends just next to the metal clips placed on the adrenal vein at the time of the surgery..
.
...this is a subtle finding...

I cannot guarantee that this is, in fact adrenal tissue and not nerve or lymph node tissue.


He has asked permission to discuss the scan with his  Interventional Radiology docs, who would actually be doing the procedure if (it's a big if) they agree with his findings. 


As far as anyone knows this ablation procedure to "destroy" adrenal rest tissue has only been done once. It was done by these docs in San Diego and it was a success. We are more than willing to give it a shot and hope for the best.  


For now, more waiting...but I'm hopeful again. If the IR docs give us an okay and insurance will authorize it we are ready to get on the next plane out.
 

Be An Informed Patient

One of the most important lessons I've learned is that patients need to be informed about their own medical conditions. Even with great doctors too may things can slip through the cracks.

Alex is scheduled for an EGD/colonoscopy later this morning (did I mention she has Crohn's disease too?). Yesterday I spent a good part of the day making and receiving phone calls and still, this morning, I am uncertain that things will go smoothly. When we saw the GI specialist 6 weeks ago I had to point out to her that Alex would need stress dose steroids for the procedure. She agreed and asked that we check with Alex's endocrine team for instruction. Within a week I mailed the GI doctor a copy of an email from the UC Davis endocrinologist as well as a "Addisons Disease Clinical Advisory" sheet. I followed this up two weeks later with an email message, checking to see that it was received, but received no response. This week I spoke to people at both the doctors office and at the surgery center several times each,  in an attempt to make sure at least some of the procedures will be followed.

As of 4:30 yesterday a nurse finally decided they would "try" and get the Solu- Cortef ordered in time for today's procedure. Will they monitor heart rate and B/P? Will they run electrolytes? Do they have any concept of how dehydration (from the "clean-out" and withholding of fluids) can affect someone without adrenal glands?  I just keep thinking how dangerous this would be for an uniformed patient.

Korlym

We are nearing the end! The makers of Korlym are donating $5.00 for ever person who clicks on the link. There is nothing to do but click; they make the donation that will support Cushing's awareness. Thank you!  http://cushingsawareness.korlym.com/

A Thousand Words

In lieu of an in depth post today I give you photos. These were taken only 6 months apart, way back when I first asked a doctor to test for Cushing's.  Alex's original tests came back negative because she was cyclic. The doctor said "it's just a bad picture".

Stretch Marks

This is what the stretch marks (striae) associated with Cushing's can look like. Prior to Alex's BLA these were popping up in the most unusual places, like her collar bones and behind her knees.

Unexplained Weight Gain

In one of my earlier posts I talked about Googling "unexplained weight gain" and learning about Cushing's Syndrome. Today I want to talk a little more about that.

It's scientific fact that calories consumed compared to calories expended are, mathematically, going to tip the scale one way or another over time; 3500 calories is equal to one pound. If a person eats 3500 calories more than their body needs they will gain one pound. It works the other way too, of course; a deficit of 3500 calories will equal a one pound loss. There's nothing tricky about it, right?

With Cushing's, calories consumed have no bearing on weight gain/loss. The metabolism takes on a mind of it's own when all that excess cortisol is present. Can you imagine how it feels to be told, by a disbelieving  doctor, that "diet and exercise" are the only key to your problems? Personally we heard, "maybe your portion control is off",  "she is sneaking food", "do you shop at Costco?", "that's just not possible", "cut back on soda". We found this truly offensive. The insinuation that we are stupid, or worse, liars. Alex learned to count calories by the time she was ten. Soda? Are you kidding? She wasn't even allowed juice. Costco? Yes, to buy fruits and veggies in large quantities. The worst thing is that, for a time, we wanted to believe that the doctors were right. We went to nutrition classes, we recorded every bite, to prove what was going on, only to be called liars.

Do I still have some anger? You bet. I do have a point here though. You know your body, mom's know their kids bodies. Don't be dissuaded by a name followed by "M.D." If you know something is wrong, keep searching until you find help.

Do You Know Someone?

Maybe you know someone? Do you have a friend or relative that complains that no matter what they do they can't lose weight? Sure, there are folks who complain, but I mean the ones that really are doing "everything right" and yet are overweight and having health issues. In the Cushing's Community most of us believe that Cushing's isn't as rare as they say, only rarely diagnosed. Case in point: Alex was told by no less than 6 endocrinologists that she "couldn't have Cushing's because it was too rare", aside from the loss of logic, this means each of those doctors will still claim that they have no Cushing's patients. I have to wonder how many others were turned away and went home to suffer or die. Cushing's can't be diagnosed with a single lab test, it generally takes many, many tests. If your gut tells you something is wrong, and your doctor won't listen it's time to find a new doctor.

Here are some of the symptoms~ you don't have to have them all~ I hope they don't sound familiar!

Unexplained Weight Gain
Central Obesity
Moon Face
Buffalo Hump
Stretch Marks
Headaches
Acne
Anxiety
Insomnia
Daytime Fatigue
GI Issues
Diminished Resistance to Infection
Menstrual Irregularities
Easy Bruising
Body Temperature Abnormalities
Visual field disturbance
High Blood Pressure
Depression
Fertility Problems
Male Pattern Hair Growth in Women

Cynical, Who Me?

What I have become ...

cyn·i·cal

   [sin-i-kuhl]  Show IPA

adjective

1.

like or characteristic of a cynic;  distrusting or disparagingthe motives of others.

2.

showing contempt for accepted standards of honesty ormorality by one's actions, especially by actions that exploitthe scruples of others.

3.

bitterly or sneeringly distrustful, contemptuous, orpessimistic.

Health Insurance

I am a registered voter, and I do vote, but I consider myself pretty apolitical. With all the health care legislation in the media right now I find I have a lot of opinions yet I agree with no one. I am reminded why I don't follow politics; it's because I feel helpless and angry when I do. As self-employed small business owners (and I mean small, only a couple employees) we are obligated to buy "individual insurance". In this position I have a good understanding of what health insurance actually costs and how politics can change the climate and increase our costs exponentially. On the other hand, as a parent of a chronically ill child I want some help from the government in affordable healthcare for my child. I certainly don't have the answers but I see an abundance of discrepancies.

On a personal level, by fall we will be paying more monthly for health insurance for the four of us than the monthly payment on a 30 year, $250,000.00 mortgage. It just doesn't seem right, does it? This would be if Alex is accepted into the High Risk Pool where costs are "managed". We made a wise decision when the kids were small and bought them individual "child" policies with better coverage than Brian and I have. It has been a God send to have decent coverage for Alex. However, when she turns 19 in September she will "age out". The policy Brian and I carry does not have a government mandate that they add her because of it's grandfathered status. Not that it matters, because, guess what? While the government mandated that insurers must accept children with pre-existing conditions it did not mandate cost control. This means they can charge any price they want! What good is insurance if you can't afford the premiums? I guess its time to jump into that High Risk Pool.

One more thing? I checked into Medi-Cal. As long as Alex lives at home (no matter what her age) she must qualify (or more specifically, not qualify) based on our income. Maybe it would be cheaper to kick her out and let Medi-Cal pay for her health care. Really? I just don't get it.

The Losses

It makes me sad to tally it up and yet I know this is only a portion of the things Cushing's has stolen.

Sports were the first thing to go. Alex loved soccer and played for many years before she got too sick. That same year illness forced her to give up dance; she was involved in tap, ballet and jazz at an advanced level for her age. Despite her tenacity she missed a day of the try-outs for junior high volleyball and cried knowing it meant she wouldn't make the team.

A social life. The isolation that came with being too unwell to attend regular school started at 6th grade. The school sent a "home/hospital" teacher to tutor Alex from home for the majority of middle school. How does a child make and sustain friendship when they can't attend school or play sports? Alex was determined to try and live a normal life despite the debilitating daily headache and signed up for traditional high school. She made it through half of her freshman year and attended a single dance before the school could no longer abide her lack of daily attendance. Since then she has attended (and I use the term loosely) Independence High, where students work from home at their own pace and meet with teachers weekly one on one. No dances, no clubs, no sports, no social interaction. I know for some teens this situation would be a dream come true; she isn't one of them.

An education. Sick again, it doesn't look like she will be graduating. She's been accepted and plans to go to college in the fall, but if she doesn't graduate college will be put on hold.

A normal body. She has been "the fat girl' since 4th grade when the weight gain started. Dieting and exercise don't work if you have Cushing's. Can you imagine gaining 10 pounds a week while eating 1500 calories a day and exercising (and having chronic diarrhea)? Even knowing, she continually tried to lose. No swimsuits, no laying on a beach, no pool parties, for the girl with Cushing's.

The possibility of having a child. Is it possible? Maybe. As far as getting pregnant, she doesn't make LH or FSH any more. She takes estrogen and progesterone to supplement what little she makes; with the help of a reproductive endocrinologist, maybe. What about carrying a child? It would be risk to both Alex and the child. Medication dosages would need to be carefully controlled. Then there is the biggy...what about the possibility of passing on the disease? She says she wouldn't take that risk.

My heart breaks for her to have never experienced a normal childhood or teen years. Life isn't fair; but you shouldn't have to learn that lesson so early. 

The Community

I am thankful to have been accepted into the "Cushie Community". As a mom of a child with Cushing's I don't quite fit in. I don't know what it feels like to live in a body with Cushing's. I know it's not the same, to be on the outside looking in.  I hope my empathy is felt. I hope my knowledge is helpful. I worry that I will be misunderstood or dismissed as someone who truly doesn't know what it's like to suffer this terrible disease.

Maybe

Maybe we would have been the lucky mother daughter pair who ended up this close anyway

Maybe it was because of Cushing's

Maybe it was having to endure this as a team

Maybe it was her trusting me to fight for her life

Maybe it was me knowing that I could lose her to this disease

Maybe we just got lucky

Life Changing Email

There are days when my in-box leaves me speechless. Mixed in among the email offers for male enhancement  and  Groupons  I find life changing messages.


After sending an email to the National Institute of Health in Maryland in 2007 I received this response:


"...Dr Stratakis agreed that we would accept her for evaluation..."


After sending a disk of Alex's pituitary MRI (that had been read as "clear" by a radiologist) I received this response From Dr. Ian McCutcheon, at MD Anderson Cancer Center in Texas, on New Year's Eve 2007:

"...there is indeed a lesion occupying much of the right half of the pituitary gland..."


And from Dr Friedman, on Easter Sunday 2008 I received this:

"I THINK THAT IS ENOUGH FOR THE DIAGNOSIS OF CUSHING'S. PLEASE FAX ME BACK THE CONSENT. HAVE YOU DECIDED ON A SURGEON?"


Where would we be without computers and email? I don't think it's an exaggeration to say I don't think Alex would be alive today. 

Wordle

How Is She?

"How is Alex?", I get asked all the time and never really have an answer. "Fine."? Do you really want to know? "Hanging in there" is the answer I give most often. For a while she had improved so much that I could honestly answer "she's doing well" but we haven't seen one of those days in months. At best there were days when Alex could keep up with me. At worst it was like having a sickly 80 year old in the house. There's never been a time when she could function as a normal teenager. Lately, she spends most of her time in bed.

When she does go out she's brilliant at wearing the mask of normalcy and telling people she's "fine", but it's an illusion. She still believes she may be able to have a "normal" life one day: with a completed education, a full time job, a husband, and the ability to travel. I hope shes right. I hope some day I'll be able to answer the question with "Great, she's doing great".

How Rare Is It?

We have reached a point where it is getting nearly impossible to find doctors who can help; the rarity of the disease was bad enough but we have moved beyond that now, to a land where doctors haven't been. We are now trail blazers. To help you understand, here are some statistics (believable or not), as they relate to Alex...

One in a million people are diagnosed with Cushing's Disease in the U.S. every year
Only 10% of those diagnosed are children (Alex was 14)
Possibly 10% of those are cyclic (Alex is)
Most (60-90%) are cured with one pituitary surgery (her tumor couldn't be completely removed)
Nearly all (95%) are cured with bilateral adrenalectomy (hers was not a cure, only a remission)

It's like being attacked by a bear and struck by lighting on the same day. Alex isn't one on a million, she's one in a billion, or maybe that's trillion? Math was never my thing.

Meds, Meds, Meds...

The medical techs always ask, as we sit down in the exam room, "Are you on any medications?". Alex and I give each other "the look". Yes. Lots.

We'll talk cost another day. 

Hydrocortisone ~  twice daily for cortisol replacement

Fludrocortisone ~ twice daily for aldosterone replacement

DHEA 25mg ~ once daily for, well, DHEA replacement (adrenals normally make it)

Estrogen/Progesterone combo ~ to regulate periods and prevent migraines

Nexium  40 mg ~ once daily for GERD

Vyvance 50 mg ~ once daily for ADHD

Levoxyl 150 mcg ~ once daily for  central hypothyroidism

Clarinex 5 mg ~ once daily for year 'round allergies

Ironsorbs ~ three times daily for severe anemia

Vitamin D-3 50,000 IU ~once weekly for very low D levels

Albuterol Inhaler ~ as needed for asthma

Nasonex ~ as needed for allergies

Tramdol ~ as needed for pain (headaches)

B-12 ~ for improved energy?

Oh, and salt, in the form of Thermotabs, to replace lost sodium

How Many Doctors does It Take

How many doctors does it take to get a diagnosis and cure? Still counting...
I've tried to make a list, off the top of my head, of every practitioner we have seen, but chances are I've forgotten some. 


Endocrinologist (12) ~ for all the endocrine issues (currently 3)
Neurologist (3) ~ for the 24/7 migraine
Neurosurgeon (3) ~ for consultations and surgery
Ophthalmologist (3) ~ for vision issues caused by the tumor
Orthopedist (2) ~ for broken bones caused by excess cortisol
Gynecologist (3) ~ for irregular female hormones
Laprascopic Surgeon ~ (3) for consultations and surgery
Gastroenterologist ~ for the GI issues (GERD and Crohn's)
Pediatrician ~ for everything
Oncologist ~ for unusual blood tests
Hematologist ~ for severe anemia
Otolaryngologist ~ for  frequent sinus infection created by surgery
Cardiologist ~ for heart problems including atrial fibrillation
Immunologist ~ for the compromised immune system
Podiatrist ~ more broken bones (Cushing's makes them break easily)
Psychologist ~ for the docs who said it was all in her head
Physical Therapist ~ after bones and muscles recovered from breaks
Dietitian ~ for the docs who wouldn't believe she was eating 1000 calories a day and gaining weight
Nutritionist ~ same as above
Chiropractor ~ for migraines and misalignment due to the distended belly
Acupuncturist ~ for migraine pain
Biofeedback specialist ~ for migraine pain
Dentist ~ high cortisol destroys teeth
Orthodontist ~ palate damage from thumb sucking (from pain and stress)

Remission vs. Cure

We were told that bilateral adrenalectomy (BLA) had nearly a 100% cure rate. Alex's surgeon said at least a 95% cure rate.  We hoped and prayed that, for a change, she would fit into the "normal" percentile. Nope. Too easy. It's back.

Alex's had her adrenals removed the summer of 2009. Slowly but surely things got better, weight came off at about a pound a week for 2 years. Insulin levels, triglycerides, and cholesterol levels all dropped to normal values. Her face shape changed, her "hump" went away, the stretch marks faded to white, the anxiety and insomnia were gone, she started a part time job and made a dent in catching up with school credits.

Forward to summer of 2011. The signs of Cushing's started creeping back in. First, we assumed it was just too much replacement  steroid. So we reduced, and reduced, and reduced some more. Without adrenals it's typical to need about 20 mg of hydrocortisone daily. Alex went from 17.5 mg daily down to 6.5 mg daily. It was time to talk about rest tissue with the genius doc in LA.

Here we go again; over the past 6 months Alex has been doing frequent lab testing. The results appear conclusive; she is producing cortisol on her own, despite the fact she has no adrenal glands. Like before, she is cyclic and she feels awful. The search is now on to try to locate the rogue tissue so that it can be removed. So far she's had a CT; the surgeon couldn't identify any suspect tissue. We are working on getting a PET scan with Cosyntropin set up. It's been used successfully in the past. Once anyway.  Until they can find and remove the tissue the only other options are a suppressant drug which she tried before (it made her feel even worse and didn't help) or a chemotherapy drug they use to destroy cancerous adrenal glands (that comes with a long list of very serious side effects).

For now I'm praying. A lot. For a CURE.

The Condensed Version

It's a long story...a decade in the making but I'm going to try for a synopsis here for any new readers.

My daughter, Alex, complained of a daily headache starting at the age of 7. By the time she was 10 she was having GI issue daily as well. No doctor could explain why, despite chronic diarrhea, Alex was gaining up to 10 pounds a month. By the age of 12 she was no longer healthy enough to attend "regular" school. At that point she had gained nearly 100 pounds, was tormented by a 24/7 migraine, suffered from frequent abdominal pain, had stretchmarks covering her body, and also suffered from anxiety, acne and horrible insomnia.

During this time I drug her from one specialist to the next, all to no avail. At one point I "Googled" "unexplained weight gain" and burst into tears when I read about Cushing's Disease. I knew, beyond a shadow of a doubt, this was what she had. If only I could convince the doctors.We traversed the country trying to find a doctor willing to "think outside the box" and eventually found a Cyclic Cushing's specialist  in LA, only 500 miles from home.

Finally, Alex was diagnosed with Cyclic Cushing's Disease and scheduled for brain surgery. The surgery was less than successful; they were unable to completely remove her tumor. Another year of waiting and testing ensued before she again went to surgery; this time to remove her adrenal glands; a last ditch effort at a cure. Both glands were removed and life did get better. Over the first 2 years post surgery Alex lost 70 pounds and the majority of her other symptoms vanished. Without adrenal glands life is complicated by life sustaining medications with tricky dosing schedules but it's better than life with Cushing's.

The saga continues....in the latest edition of "Life With Alex"? It's back.

(continued tomorrow)

The Challenge

Welcome to the Cushing's Awareness Challenge! I'll be blogging every day in April in an attempt to get the word out about Cushing's Disease. Follow me and learn more about this awful disease that my daughter Alex has been fighting for a decade.