Thursday, February 7, 2013

It's Back...

We spoke with Dr. Friedman this week and he confirmed what we already knew. Alex is producing cortisol, yet again. She had both a pituitary MRI and an adrenal CT recently and both came back clear. As much as that sounds like good news our Cushie friends know this is not. Without a surgical target nothing can be done. 

So, we wait. In April she will have an other abdominal CT; there is a new type of scanner, a 320 slice, and we have located one in the Bay Area, only a couple hours from home. In the meantime we hope the weight gain abates, she tries another round of  Ketoconazole, a drug that can reduce cortisol (but that she have had no luck with in the past) and we pray that a target appears on the next scan.

Monday, July 16, 2012

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. 

Monday, June 25, 2012

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.

Thursday, May 24, 2012

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!

Monday, April 30, 2012

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!   

Sunday, April 29, 2012

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. 

Saturday, April 28, 2012

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.

Friday, April 27, 2012

Where To Go For Help

I want to share a couple important links today.
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.
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.

Thursday, April 26, 2012

But You Don't Look Sick

The "Spoon Theory" by Christine Miserandino is a Must Read

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?

Wednesday, April 25, 2012

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.

Tuesday, April 24, 2012

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". 

Monday, April 23, 2012

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. 

Sunday, April 22, 2012

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.

Saturday, April 21, 2012

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.

Friday, April 20, 2012

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.

Thursday, April 19, 2012


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!

Tuesday, April 17, 2012

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".

Monday, April 16, 2012

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.

Sunday, April 15, 2012

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.

Saturday, April 14, 2012

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
Daytime Fatigue
GI Issues
Diminished Resistance to Infection
Menstrual Irregularities
Easy Bruising
Body Temperature Abnormalities
Visual field disturbance
High Blood Pressure
Fertility Problems
Male Pattern Hair Growth in Women

Friday, April 13, 2012

Cynical, Who Me?

What I have become ...


  [sin-i-kuhl]  Show IPA
like or characteristic of a cynic distrusting or disparagingthe motives of others.
showing contempt for accepted standards of honesty ormorality by one's actions, especially by actions that exploitthe scruples of others.
bitterly or sneeringly distrustful, contemptuous, orpessimistic.

Thursday, April 12, 2012

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.

Wednesday, April 11, 2012

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. 

Tuesday, April 10, 2012

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.

Monday, April 9, 2012


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

Sunday, April 8, 2012

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:


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. 

Saturday, April 7, 2012


Wordle: Untitled

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".

Friday, April 6, 2012

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.

Thursday, April 5, 2012

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

Wednesday, April 4, 2012

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)

Tuesday, April 3, 2012

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.

Monday, April 2, 2012

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 the latest edition of "Life With Alex"? It's back.

(continued tomorrow)

Sunday, April 1, 2012

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.

Wednesday, November 10, 2010

Still Learning

Before I even get started with a new topic, I need to say YAY! For those who haven't already heard the MRI was fine. "No definite tumor".

Anyway, when we started with the new endo she suggested we increase Alex's dose of hydro. Dr. F. had suggested this as well. It's funny how resistant we are and how hard we fight against excess steroids after living with Cushing's. We have been vigilant about trying to reduce Alex's dose to the absolute minimum amount she could function on in hopes of further weight loss.

Sometimes it takes an outside perspective I suppose. Retrospectively, I should have seen it. Headaches daily, terrible fatigue, nausea...and it didn't dawn on me that this was all caused by not enough hydro? Add to that the labs and the fact that it was so incredible hard to wean? Needing extra hydro a couple times a week just to keep from puking? Oh, and two crisis siuations in a 5 month period?

So, over a month ago we increased the hydro from 15mg daily to 20mg daily. Guess what? She feels better! And, surprisingly, she hasn't been gaining weight of showing any other "Cushie symptoms". In fact, she has lost another couple pounds.

Like I said, still learning!

Monday, October 25, 2010

I Don't Want To Think About It

Nelson's Syndrome. Aside from rest tissue it's BLAer's biggest worry.

For the first year post BLA Alex's ACTH levels stayed under 100. They are expected to be high due to the negative feedback, and 100 is a perfectly normal level for a BLAer (though not for a normal person). Over the past several months those numbers jumped into the 350-550 range. Alone those numbers are still acceptable, but her skin has started darkening as well. That makes two out of the three signs, so Friday she had an MRI to look for the third and final symptom. I don't know how soon we will hear back with results, but if the tumor is growing it will mean another pituitary surgery and possibly radiation as well. The daily headaches are back, so we are definitely concerned.

Thursday, September 30, 2010

The New Endocrinologist

After the fiasco reported in my previous post I've searched out a new endocrinologist. I've asked around and slogged through every local endocrinologist's web site trying to find someone I thought would be receptive. Today was the big day...we met with Dr. Lee at UC Davis.

We first met with her "helper" (intern?) and he actually spent about 45 minutes in the room with us going over heaps of old paperwork, attempting to assemble the puzzle that is Alex. Once he was done he consulted with Dr. Lee and then the two of them came in together and spent an additional 20 minutes.

Both Alex and I liked the doctor and felt she was competent. She asked the right questions and didn't say anything that was cause for concern. I guess we won't really know how good she is until we hit a snag, and see how she reacts. She suggested Alex increase her hydro dosing. We have been reluctant to go this route, but have to admit Dr. F said the same thing. With ACTH in the 300-400 range, and Alex feeling tired all the time, coupled with a need for extra hydro several times a week I think it's a valid option to try. We are going to give it a shot and hope the weight doesn't start piling on. She is okay with Alex's fludro dosing, and agrees that when fall weather actually gets here( it's 100 degrees today) we will likely need to reduce. She's running thyroid labs, but thinks Alex is likely at the top dose necessary. She's running all the sex hormones as well, so that may get us somewhere.

All in all it went well, but only time will tell.

Saturday, August 7, 2010

Thrown Under the Bus~Okay, Not Literally

I'm still so enraged I don't know if I can write down what I'm feeling, but I'm going to give it a shot.

This past week has been a series of "AI (adrenal insufficiency) events". Starting with Tuesday: Alex woke up vomiting. We gave an injection of 100mg solu-cortef, but chose not to go to the ER (though Dr. F. tells us this is the proper course of action). Going to the ER in the past hasn't worked out well and it's something we'd like to avoid if at all possible. Over the next few days Alex was unable to eat more than a few bites but she was keeping down some fluids. We continued "stress dosing" when she felt she needed additional hydrocortisone. I just want to add here for my "non-Cushie" readers: without adrenals your body has no means of balancing fluids and minerals on it's own. Vomiting and diarrhea can be life threatening.

Moving on to Thursday night: Alex took an extra dose of hydro before bed but woke up at 2:00AM feeling awful; whole body shakes, diarrhea, extreme nausea. We opted to do another injection of solu-cortef. An hour later she wasn't feeling much improved so we decided it was time for a trip to the ER. They actually did a great job in the ER. They followed Dr F's emergency letter to a T: they gave fluids, solu-cortef, Zofran for nausea and Ativan for anxiety. It was about 5:00am by the time they gave her the meds and she fell asleep for a while after. They also called Alex's pediatrician. Another Dr. was on call but forwarded the message and the wonderful Dr. Leong was in the ER to see Alex at 8:00am.

Alex's heart rate had dropped from her normal 80-90 down to the low 50's. In the past this has been a marker for low potassium for Alex so Dr. Leong ordered a few rounds of potassium and solu-cortef if needed. Alex felt she still needed more solu-cortef so they ran another round of 100mg. Because they weren't seeing much improvement, and because of the the low heart rate they decided to admit. They put us in a nice private room upstairs and Alex was feeling pretty normal. Dr. Leong came by on her lunch break to order more labs, see how the EKG looked and check in with us. At that point she said the EKG was showing A-fib, and thought we would be better served in Sacramento where they could put her in a peds ICU for closer monitoring. She also said that we would have a staff doctor there at all times and that Alex's local peds endo would be able to care for her in conjunction with the doc on the floor.Before leaving Roseville Alex was given another 50mg of solu-cortef with Dr. Leong ordering 50mg every 4 hours or 100mg every 6 hours if needed.

So, here's where the wheels came off the bus. We arrived in Sac with the belief that Alex would be on a continued regime of weaning, or more if necessitated. Wrong! The floor doctor said we needed to wait until Alex's endo arrived. By this time Alex is completely flipped out. She is crying and truly believes she is going to die. Finally her endo arrives and tells us "she is not in crisis; she does not need any more steroids". I just thought she was freaked out before. A few more of his comments if you will? "a patient can not tell if they need steroids"~ "steroids are bad for you, you need to quit taking these"~ "have you seen a therapist? this level of anxiety is not normal"~ "you are fine; nothing is wrong with you; the labs say you are fine; quit crying". We were both nearly speechless. Where did this all come from? This endo was one of the many who failed to diagnose Alex. On the other hand we were giving him a second chance because he was willing to help us when we were testing with Dr.F. We are angry (livid?), sad, and betrayed. Did he have us come to "his hospital" so he could belittle us?

We spoke again with the intensivist on the floor. She said she was deferring all endocrine treatment to him. Now what? Knowing there would be no more steroids what are we supposed to do? We pondered it for a while and did a lot of agonizing (on my part) and crying (on Alex's part). The final decision? Ummm....cheat. Against medical advice I slipped her 40mg of hydro from the stash in my purse. I figured it would be at least enough to hold her till they could get some more fluids in. Brian had a friend follow him to the hospital so that they could leave a car there for our escape. While Brian was there I asked the nurse "So, how bad is everyone going to freak out if we say we are leaving?" She was very sweet and said she would let the doc know and that it wouldn't be a problem. The doc came in and talked to us and said she didn't have a problem with us leaving, just watch the fluids and try to get Alex eating some. She even volunteered a bolus of fluid before we left. Brian left for home at that point and we accepted the offer of fluids pushed over an hour. Of course by the time the fluids were done Alex was gonzo asleep. Around 11:00 we were on the road for home.

The 40mg I gave her at 6:00 held her for the night and this morning I gave her 20mg instead of her regular 11.25. We can only hope that whatever caused the onset of all this has abated. If not I don't know what our next step will be. By the way? We won't be seeing that endo again. Ever.

Friday, July 23, 2010

Monday, July 19, 2010

Pictures As Promised

These are "the official one year post op photos".



Friday, July 16, 2010

It's Been One Year Today!

Today is the one year anniversary of Alex's BLA. We have no regrets, but it has been a very slow recovery. All things considered she is so much healthier now than she has been in years.

Current meds are:
Hydro~ 11.25mg @ 7:00am and 3.75mg @1:00pm
Fludro~ 1 pill @ 7:00am 1/2 at 1:00pm and 1/2 @ 5:00pm
Thermotabs~ 2 pills daily (to replace sodium in the heat)
Vyvance~ 30mg daily (for ADHD)
Levoxyl~ 75mcg daily
Nexium~ 40mg daily
Ironsorbs~ daily
Vitamin D~ once weekly

Adding the Vyvance and the Levoxyl have made an incredible difference. Alex's energy level is so much better now. She is up at 8:00am nearly every day. It may not sound like much but she now does her hair and makeup daily, wears clothes (not jammies) daily and leaves the house to do stuff every day. She is able to do several things in a single day, and she doesn't need a full day to recover if she leaves the house.

She has come so far. It used to be that she spent most days in bed. I had to force her to shower. She was in pain and crying several times a week. She told me several times that she just wanted to die. I would check on her when she was sleeping to see if she was still alive. We had to plan everything around her health.

The stretch marks are gone, the hump is nearly gone, the acne is gone. The headaches are infrequent, the GERD is still a problem but Nexium works, the other GI stuff is under control. She is no longer insulin resistant and her cholesterol is in normal range without meds. The Levoxyl has nearly normalized her periods; we are no longer considering uterine ablation. Weight loss has been slow, 35 pounds to date, but it does seem to be coming off again.

The most remarkable thing is that we are now leading a pretty normal life. It isn't just focused on Cushing's. I still foresee continued improvement. It doesn't seem to have stalled out. If this were "it" it would have still have been worth it without regrets. But, I think the future holds more positve changes to come.

Thursday, June 17, 2010


The school year ended 3 weeks ago and Alex and I met with her counselor and signed up for next year's classes. This August Alex should be starting her senior year. The thing is she only has credits equal to 2 years completed. We had really hoped she would be able to catch up this past year but the reality has been different.

Options include taking the state proficiency exam (they don't offer a GED 'till you are at least 18), trying to cram two years of work into one, or planning on taking an extra year of H.S. Alex plans on going to college so she has decided she would rather plan on an taking two senior years. This will give her more of an education under her belt so that college won't be so intimidating. Her counselor is fine with this option as long as Alex is making "good progress" this coming year.

I think it's the best option, but it's going to be really hard when her friends are graduating and leaving home and she is going back for another year. I guess this means we will be skipping senior pictures, and all the other "senior stuff" for another year. It may be the best option, but it still makes me so sad for her.

Tuesday, June 15, 2010

It's Been A While

Time flies! Improvements are so slow it seems like nothing changes. On the other hand when I look back things are still continually changing. So, lets see...

Alex's weight loss seems to be stalled at the 25-30 pound mark. She is still working on it but as soon as she loses a few pounds she gains them back a week later. She really no longer has any of the "Cushie" symptoms. At this point it's more about getting her body back to a normal state and adjusting meds, still.

Hydro dose is still the same, though we have had to switch up the timing a bit to avoid headaches every afternoon. Along with 2 fludro a day she is taking 2 salt pills. They seem to help as much as the fludro with keeping her fluid and electrolytes balanced. One week ago we increased the Levoxyl to 75mcg. The Levoxyl has been a really good thing. It has helped with energy levels and helped with the menses. We are hoping the increase of dosage will cause further improvements. The Vyvance isn't working as well as it was so she will start this week on an increased does (30mg) of that as well.

We were talking this weekend about how the changes really do sneak up on you. We were out running errands for several hours and didn't have to slow down or give up and come home. A year ago if we made it through Costco and back home she would have been done for the day.

I'll make sure and post next month when she reaches the one year mark. Hopefully with a picture of the changes!

Tuesday, April 6, 2010

Lots of Things

I don't know why I haven't been blogging I actually have quite a bit to say.

The latest news: Alex no longer has high cholesterol or pre-diabetes (insulin resistance). The latest labs are well within range with no medications to help out. Current weight loss is at 25 lbs.

GYN news: Dr. Warner is unable to regulate Alex's cycles with progesterone and thinks a uterine ablation is the answer. Alex and I were both totally on board with the idea till I asked some of my Cushie friends. They have made me re-think and we are going to try more alternatives first. This week Alex will be seeing my GYN for a second opinion. Alex is certain that she does not, and will never want, to bear children of her own. With pituitary damage it will be hard to get pregnant, with no adrenals it would be tricky to sustain a pregnancy, but for her, first and foremost, is that she doesn't want the posssibility of passing on the disease to a child. We both feel adoption will be a better alternative when the time comes. My Cushie friends have advised that it's awfully early to close the door on fertility.

Endo news: Today Alex starts on Levoxyl 50mcg. We persuaded the local endo to do a trial run on thyroid meds. Her numbers are within range but low. We are hoping this is the key to further weight loss, increased energy and regulation of menses.

ADHD news: Alex has been on Vyvance for about a month and a half. It gives her a bit more energy and does help her to focus some. She thinks it makes her feel a bit "flat". I tend to like her a bit "flatter" than usual. I see a huge difference in her ability to have a coversation without being all over the place.

Other news: We got Alex an '02 Honda Accord last week. I got a really good deal through an ad on Craig's List. Our deal has been that the kids have to pay for half of their first car. She will be making monthly payments to us for her half. I think I'm as excited as she is. It is really nice for her to be able to do things on her own.

Wednesday, March 3, 2010

The ER~ Round Two

Over the weekend things improved very little. Alex needed 100-150mg of hydro a day to keep her out of A.I. Through all of this we have been keeping an eye on her pulse and B/P. Since the BLA we have found the balance of meds is easier to determine based on her numbers. Early Saturday afternoon we noticed her pulse was quite low, without a change to B/P. By Saturday evening I paged Dr. F. because pulse was in the 40-50 range (it's usually 80-100 for her). We kept an eye on her for several hours and never got a call back from Dr. F. I spoke with a couple other BLA ers who advised we go to the ER, but admitted they probably wouldn't if it were them. The low pulse stayed all of Sunday as well and was at 42 when I woke her on Monday for meds. I called the pediatrician and spoke with her when she called back. We decided the best bet would be the ER to get things done quickly. Dr. Leong call ahead and spoke with the ER doc.

They did get us past triage quite quickly and got labs and an EKG going. The EKG showed the bradycardia but nothing else significant. The labs showed potassium of 3.2 (range 3.5-5.1). The ER doc didn't think that the slightly low K could cause Alex pulse to plummet. He consulted with Dr Leong, Alex's local endo, and a cardiologist. Dr. Leong came through again by pushing him to give Alex potassium, since she and I had discussed it earlier. They checked in with us an hour after Alex took the K and her pulse was still low. They basically said "we were right that didn't help". Another 2 hours went by before any one even came in the room again. At that point the doc came in to send us home, assuming her pulse was still in the 40's but with no other symptoms they didn't have a reason to keep us. He was quite perplexed when I pointed to the monitor. Her pulse was bouncing around A LOT, but it was between 70-112. I'm still not so sure he believed it was the K that changed things. I have a hard time with the fact they were planning on sending us home with her pulse at 44; pulse under 40 is hitting the cardiac arrest stage.

Later that night Dr. Leong called to get the scoop. She sent over an RX for K light.The pharmacy had a hard time getting the K and we weren't able to pick it up until last night. Alex drank Gatorade by the gallon yesterday and we managed to keep her pulse over 50.

I'm still trying to process this but here's my theory: Without adrenals a body loses NA and hold K. Florinef reverses this. In theory then, too much Florinef should make a body hold NA and lose K. If a person takes high dose hydro some of it will convert to mineralcorticosteroids (Florinef). So, in Alex's situation I think the high doses of hydro over several day (which she needed for the fever/virus)while on her normal dose of Florinef resulted in the equivalent of too much Florinef causing her K to drop. I don't know if this is even possible but for the moment anyway I'm sticking with it.

Saturday, February 27, 2010

It's Been a Tough Week!

Alex and I flew to L.A. on Tuesday. I booked everything believing we had a Wednesday night appointment with Dr. F. Apparently they have changed from Wednesday night to Tuesday nights without updating their online schedule. I knew about the change before we left home, but just left all the reservations in place, planning on a return trip Thursday.

The visit with Dr. F was just a yearly "in person" appointment we try to do. Since I do all the talking when we have phone appointments I think he and Alex should physically see each other once a year.

Monday night Alex felt like she was getting a cold; no big deal. By the time we got to L.A. on Tuesday morning she wasn't feeling well. Around 7:00 Tuesday night I could tell she had a pretty good fever going on. We didn't have a thermometer with us but I gave her 600 mg of Motrin. Two hours later we got Dr. Z to scrounge up a thermometer, at that time the fever was 101.4. I have to wonder what it would have been without the Motrin. The appointment went fine. No big news, but we weren't expecting any. Alex took an extra 5mg of hydro that night.

Tuesday morning I woke Alex at 7:00 for her first dose of meds. She said she was feeling awful, but fell back asleep for about 45 minutes. When she woke she said she knew she needed more hydro, and Motrin, but was feeling really nauseous. She tried taking the hydro but instead started vomiting. For the next two hours we debated what to do. Page Dr. F? Go to an unfamiliar ER? She really tried to take more hydro but was getting panicky knowing how bad she needed it. I got out the emergency injectable; by then we were both scared. I'd read the directions on how to use it a million times, but realized then that I still had questions. Thank God for I got on line and posted a message. Within 2 minutes I had people (love you guys) calling to talk me through giving the injection.

The 100 mg was enough to bring Alex out of the immediate crisis. I spent the next two hours on line and on the phone changing plans and reservations. We flew out of L.A. at 5:00PM. We both thought the 100mg would be enough to keep her going long enough to get home. It was, with a some more hydro taken orally.

Thursday she still needed loads of hydro, over 100 mg for the day. We called the pediatrician on Thursday and made an appointment for Friday morning to see if she could figure out what was causing the need for all the hydro. Alex has been exposed to strep, but her throat wasn't super soar.

Friday morning Alex took 20mg at 7:00, but by 8:00 knew she needed more but was again too nauseous to take it. I called the local endo who told us to go straight to the nearest ER. We went, and while we were there waiting called the pediatrician and left a message that we wouldn't make the appointment because we were at the ER.

Two hours after arriving at the ER they got around to giving Alex 100 mg of hydro IV and a bag of fluids. Now I really understand why Dr. F gives us emergency injectables and says to use them before heading to the ER! I have to say how much Alex and I love her pediatrician. Dr. Leong called me twice while we were in the ER and also called both the local endo and the ER doc to consult. Bless her heart.

Results of a chest x-ray and all labs came back normal, so they sent us home! Huh? No one is interested in figuring out why this is happening? I tried to explain that while they got us out of immediate crisis (good) that as soon as the dose wore off we would be back in the same spot (bad). They said "follow up with your regular doctor". We drove home and called Dr. Leong who said to come immediately. She did a thorough once over on Alex and found nothing wrong, but agreed that the high dose steroids could be masking whatever is causing the problems. She did a swab (3 day) for strep and gave Alex wide spectrum antibiotics to wipe out whatever might be lurking.

We will see how this goes. Alex started the antibiotics last night. When I woke her for her 7:00 dose this morning she took a 40mg of hydro just in case.

I feel like the conductor of a run away train.

Friday, February 12, 2010

Way Back When...

I've had a couple people lately ask about the early changes in Alex. These are her school pictures taken one year apart; back in 4th and 5th grades. Can you believe this wasn't enough to motivate a doctor to help?

For those of you fighting to get your kids diagnosed, take pictures frequently. When we see our kids every day we don't notice the changes.

Thursday, January 28, 2010

A Recovery Update

I thought maybe I should post a 6 month post-op blog update so that when I look back I can see where we were at this point. Alex's current dose of hydro is 13.75mg at 7:00 am and 2.5 mg at 2:00 pm. She's taking 2.5 tabs of Florinef daily and 25 mg. of DHEA. She is still on several other meds, but those are the adrenal replacements.

The good: Total weight loss to date, 18 pounds. It comes off in fits and starts, it makes no sense, but anything is better than nothing! The acne is almost completely gone. The stretch marks are gone except a few high on her abdomen. The hump is still there, but smaller. Her face shape has changed, she is smaller around the rib cage and her shoulders are shrinking. She goes to bed around midnight, instead of 4:00am. As far as anxiety, the Rx for Xanax says it was filled in August, and it's still 3/4 full.

The less than good: The weight isn't coming off fast enough :-), she is tired, really, really, tired, all of the time. She has a headache much of the time (not the killer bad ones from before). Her GI stuff is still not great; it comes and goes (pun intended). She still needs Nexium to control the reflux. She still doesn't feel "well enough" to do a full load of school work. Her memory is poor, and so is her ability to focus/concentrate.

I think that about covers it.

Tuesday, January 26, 2010

San Francisco

Alex and I are going to S.F. on Thursday night so that we can meet two future BLAer's. Michelle will be flying in from LA for her pre op (surgery in early Feb.) and Lisa will be flying in from Washington for her first meeting with Dr. Duh. I'm excited that we have helped (re)introduce a great surgeon for those on the West Coast in need of adrenal surgery. We are all getting together for breakfast on Friday morning before their appointments; it should be fun!

Monday, January 25, 2010


Alex did the first months cycle of progesterone. Nothing changed. Using the progesterone for 14 days did seem to mess with the balance of her hydro/fludro, which was not the least bit helpful. Her period was supposed to start within a few days of stopping the progesterone; it didn't start till 10 days later. Her pain and flow were supposed to be lessened: they were the same. The doctor says try another one month cycle and see how it works before we change things up again. So, we are back to trying to be patient.

The pediatrician changed the ADHD med from Ritalin to Vyvance. I'm picking up the script today and Alex will be giving it a try. I'm crossing my fingers.

Saturday, January 9, 2010

MRI Results

The results are in from the MRI last month, and the comments are less than interesting.

Dr. K's office called and said "He says there is no obvious tumor or disease". That was it. I had requested the MRI on disk and written copies of the radiology report and surgeon's report. I explained to the person on the phone that I hadn't received these things, and when pressured she agreed to fax me the two written items. The fax came through and sure enough that was the entirety of Dr. K's comments. The radiology report basically said the same thing, with a nice note saying that they had forgotten to use the dynamic protocol and would suggest they do use it next time. For the record? They "forgot" last year as well and made the same recommendation then. Jeez.

Oh, well, I think we are just not going to worry about it. Obviously there is no target worth going after at this point as far as Dr. K is concerned. Once I get the disk I'm sure Dr. McC would read it for me, but I don't think there's much point. Until next year this is going on the back burner.

Thursday, December 31, 2009


We heard back from the phsychologist last week; they said they will do a complete evaluation but that they have up to 60 days to complete it. Considering this I called the pediatricain who then prescribed Ritalin. She wanted Alex to start on a lose dose (5mg. twice daily) and see how it goes. On Sunday we gave it a try; I gave Alex the Ritalin with her A.M. dose of hydro. She woke up a couple hours later and said she felt less groggy than usual first thing in the morning. She also said she felt "stupid", because she could only focus on one thing at a time. She thought it might be helpful for school work but wasn't so sure she liked the feeling. By noon she was getting a headache and took the second dose. The headache lingered through the afternoon. By early evening when the Ritalin should have been wearing off the headache got really bad. By 8:00 the headache was so bad she thought we might need to go to the hospital. Along with the headache she started feeling A.I. She took 2.5 mg of hydro, waited a half hour, took another 2.5mg waited another half hour and took 5mg. With the extra hydro things started improving including the headache.

Now the questions... was Ritalin the cause of the headache? Was it the cause of the A.I.? Could the headache pain have caused her body to need more hydro? It's frustrating! The plan is to wait a few days and try the Ritalin one more time, one dose in the morning, and try and do school work while on it. If it doesn't work out we'll get a prescription for something else.