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.