Basics: Post-Op BLA

Adapted from a thread on the message boards.

‘m going to try to keep all of my post-op BLA updates in this thread. I am hoping it will eventually show positive progression and be a realistic and inspirational thread for others.

Today I am two weeks post-op BLA. So far, no scares. I am on 30/20/20 of hydrocortisone and weaning by 10 mg every four days. I am sleeping a good bit during the day and resting a lot to get my strength back. If I am upright too long my abdominal area aches and I get fatigued, sometimes it still aches even if I am not upright. My nighttime sleep has been good. I’m waking up only 1-2 times to go to the bathroom (I think the meds are making my bladder more active than normal), but otherwise am sleeping through the night which is a huge change from Cushing’s. I am hoping this is due to being Cushing’s-free rather than just due to the pain meds I am taking right now. We’ll see if this lasts as I drop the pain meds and hopefully the nighttime urination will let up as the hydro levels drop.

Also, and I don’t think its my imagination, but some of my stretch marks are getting lighter. In particular, the ones that formed on my legs after my pit surgery. This is a positive sign! I showed my mom and hubbie and they could both see the change too. Unfortunately, my hump is bigger right now than pre-BLA and my cheeks are still pretty red, but I bet this will change as I wean down.

No weight changes as of yet, but not expecting any because I am still on such a high dose of hydrocortisone. I was 198 the day of the BLA, which was about 15 pounds heavier than the day of my pit surgery seven months ago. For the first week and a half after the BLA I was really, really bloated – and it was all in the stomach area. Most of this bloating has gone down in the past two days.

I’ve watched my calorie intake throughout the battle with Cushing’s but I started a food journal yesterday just to make sure I am keeping myself in check. I’m eating 1500 calories a day. I noticed right away that I haven’t even been eating that much on a normal basis because I actually had to eat more than normal to meet the 1500 calories. So that’s also a good sign that watching my food intake won’t be a big change in order to help the weight to come off.

So that’s really the main things happening right now. Just taking things slow and steady and trying to have realistic expectations!


I had my six week post op appointment in Seattle last week. My weight is actually up (204, I was so bummed that I went over 200). But Dr. L said not to worry, that its normal to gain weight during the weaning process. I am still on a 1500 calorie a day diet and will stay there until I start to see weight loss and then I’ll reassess calories then. I was advised that weight will probably start to fall off when I’m six months out from surgery, so I am trying not to focus too much on it or get discouraged.

My nighttime sleep is weird right now. I’m not waking up all night long like I was before the BLA, but I can’t fall asleep at night either. I lay awake until 1 or 2 am. On the flip side, I am waking up at a normal hour – 7 am.

I just started weaning to 20/5 of hydrocortisone. It is pretty rough. The wean from 20/10/10 to 20/10 was hard, but this is even harder. Feels like the flu, achey all over, headaches, sleeping all day (which probably doesn’t help me fall asleep at a good time at night!). I have realized that I must take the wean really slowly now. The goal is to get to 20 or maybe just a little less and hopefully that dose will work for me.

In other news, I got the path report back on my adrenals – my adrenals combined weighed in at 30 gm (normal combined weight of adrenals should be between 8-12 gm). The left one was twice the weight of the right one, and they had “subtle vague expansion” and “microscopic nodularity” suggestive of adrenocortical hyperplasia.

So I am feeling very validated at this point and I know I made the right decision to have the BLA.


I’m just past the 3 month post-op anniversary. Some things are better and others are still the same. But more positive changes than anything.

We’ll get the negative overwith first – my stretch marks did an about-face and actually got a lot worse about a week after I got down to my physiological dose (20mg). Dr. L said not to worry, they’re just showing up now due to past cortisol exposure. Still, they’re pretty bad. So I was disappointed in that. My period still has not come back since I had the pit surgery. All my hormones are fine except the progesterone, but progesterone supplements are not helping. We’re taking a wait and see approach to give my body some time to get over the shock of two major surgeries.

Other than the stretch marks, the other Cushing’s symptoms are slooooowly getting better. I am sleeping pretty well now, able to fall asleep in the evening and sleep until 5:30 or 6 am until waking up. Its a lot better than waking up at 3 am every night for sure. My hump looks a little smaller (I think). My cheeks are still red, but my face is maybe slightly slimmer (I think). I’ve lost six pounds (with 80 more to lose), but am still heavier than I was the day of my BLA. Although my stomach doesn’t pooch out so much anymore, so I look less pregnant. My hair has stopped falling out.

I have been working out for a few weeks now and my strength is really starting to improve. Walking is very good for me. I’m eating about 1200 calories a day and dropping down this low seemed to jumpstart some weight loss. I am hoping it continues. I’m certainly doing nothing food-wise to keep the weight from coming off.

I was tested for insulin resistance and any thyroid problems – everything came back normal. My ACTH was super low when it was last checked – came back at 3. (yay!!!)

I went back to work 80% time this week. I’m trying to work short days but my work is very demanding so I will probably have to end up working 4 days a week and taking off one day a week to rest. I am very tired at the end of the work day. Exposure to stress is also very hard on my body – the stress I have encountered this week has caused nausea, diarrhea and one time I had to take straight to the bed and lay down all evening. Right now I feel like I am not as sharp and “on the ball” as I used to be.

My sinus infections from the pit surgery keep continuing about every 6-8 weeks. I’ve probably had at least 4-6 sinus infections since March. At the last visit to the ENT doc, she said she thinks I have a deviated septum from the pit surgery and may need surgery to correct it. I have a CT scan on Tuesday so hopefully we’ll know more soon on whether I am having another surgery.

But overall, I just feel better. The Cushing’s symptoms are slowly fading, but at least we’re going in the right direction. I am trying to be patient, and trying to remain motivated. I have to admit I am becoming very impatient for the weight to come off and still harbor fears that it won’t. I am considering throwing my scale in the spare bathroom and forgetting its existence for a while.

I hope my next update will have tons more good things to share.


So I am 6 months post BLA today. Yay! This is the magical date – things are supposed to start changing more quickly after passing this milestone. Here’s the stats so far:

20 mg hydrocortisone per day

0.1 mg florinef per day

Had thyroid checked in January – fine

Had glucose tolerance test in January – fine, no insulin resistance

Dr. L didn’t think I had GH issues at my 3 month post op appointment

Estrogen and all other female hormones fine except progesterone, taking prometrium to try to induce period with no success so far

I started losing weight at the end of January through mid March. I lost 10 pounds. But now, I haven’t lost any weight in over a month and I’ve actually regained two pounds. I am exactly what I weighed the day of my BLA now.

I’ve been working out 90 minutes 4-5 days a week (elliptical machine and weights). I’m eating net 1200 calories a day (which means I am actually eating more than 1200 because of all the exercise I am doing) and very closely tracking calories on livestrong.com.

I have to say I am very frustrated at this point because I’m working so hard and not losing weight. I’m going to bring this up with Dr. L at our six month post op appointment. If some other BLArs could chime in and tell me what to expect for the next six months, I would greatly appreciate it. Just starting to get a little nervous here.

As far as the Cushing’s goes, I have more energy and I am sleeping better. Most nights I sleep through the night and if I wake up, its only once and closer to 6 am than 3 am like it used to be. Hump is still there, hasn’t gone away but is a tad smaller. Hair stopped falling out a while ago and has stayed just fine, no relapse.

The stretch marks (which had gotten worse after the BLA) are getting much better, at least the ones on my legs. Those are noticeably better. I’ve gotten comments that my face is slimmer and I look like I’ve lost weight. I’ve gone down from third trimester maternity pants to second trimester pants. That is some progress because I look less pregnant.

Since my last update, I have had three severe episodes of AI. All occurred late at night following a week of being pushed beyond my medical restrictions at work. 32 hours a week seems to be a good balance though, more than that causes me to be really tired and at risk for AI.

I’ll close out with a great accomplishment story. Hubbie and I went on a cruise to Mexico and Belize. I was able to do a hike through the jungle (which was relatively level, for a jungle). But the best part was when we got to a clearing and saw the Mayan temples. You could climb one that was about 45 meters high with very steep stairs to the top. Of course my hubbie was the first in the group to take off up the temple. The stairs were so steep they had a rope that came from the top all the way to the bottom to pull on to help yourself get up. This was the type of thing that, before Cushing’s, I would have been right there with my husband.

He was about halfway to the top when I said, “Heck, I’m going too.” Probably shouldn’t have, but I took off up the temple stairs after him. I climbed up and up in the Belize heat and made it to the top. The view was rewarding, but the greater reward was that I could DO it. I was getting part of my life back – the adventurous, hiking, exploring, running-being-free part.

That part was the best. :D


I am weight training, 4x a week for 20-25 minutes per session, on machines, not free weights. I want to make sure I’m not getting the wrong form. I am pushing myself, sometimes only able to do five reps at a time because of the heaviness of the weight. I do a total of 3 sets of 10 reps per exercise. I’m doing upper body and lower body on different days, so 2 days a week of upper and 2 days of lower, never back to back.

My diet is good. Short of starting to cut out food groups altogether, there’s not much else I can do. I eat either whole grain cereal and skim milk or two boiled eggs and skim milk for breakfast. My mid-morning snack is fruit – usually a cup of red grapes or an apple. Lunch is a salad with grilled chicken or a Lean Cuisine or Smart Ones that has fish as the main entree – nothing over 300 calories. Mid afternoon before working out I have fat free yogurt or 30 almonds. Dinner is normally something like stuffed green peppers or chicken fajitas – usually about 500-600 calories.

I weigh/measure just about everything . . .


I’m 7 months 9 days post op today. The weight has changed a little, but only a little. At least its going down and not up, but I admit I am frustrated with my progress. I told Dr. L about my intense working out and dieting and he suggested I wean some more. So I weaned to 17.5 mg of hydro first and then down to 15 mg. I’ve been at 15 mg for 3 weeks now. The past week I started to see some progress – I lost 2.5 pounds this past week, so now for a weight loss total of 12 pounds since January. This is in conjunction with a 1200 calorie a day diet. I’ve now gone to a combination Zone diet (30 protein, 30 fat and 40 carbohydrates) and sort of low glycemic index – just as little sugar as possible. So I am eating a lot of bran, salads, chicken and fish. I’ve instituted a “salad for dinner two times a week” rule at home, which my lovely, Southern-food loving husband has generously agreed to go along with.

May was not as good a workout month as March and April. However, we did a one week vacation with LOTS of activity – hiking every other day for 2-3 miles, and we did a 14.5 mile bike ride at the end – it was mostly flat, but still! That was a long way and I was so proud of myself when I finished it. It was a struggle, but I did it.

I also got my period for the first time in over a year in May. I wonder if it is related to weaning to 15 mg? We will see if it comes back in June . . . .

Other things have gotten much better – sleeping well through the night, feeling better in general. My hair was much better until the past two weeks or so when I’ve seen more of it coming out in the shower than normal (what is that all about?!?!?) but not falling out on a regular basis like it was with Cushing’s there at the end.

I am losing some inches for sure and I don’t look as pregnant as I used to, I was able to drop from my maternity black dress pants to a size 18 pants (although the legs are still huge). I am still in my maternity jeans but I did go from trimester three to trimester two! I picked up prescriptions at the pharmacy today and my pharmacist said “You are looking great!” So that was nice to hear :)

So all in all, very very slow but seeing some progress now. I think its going to be a very long process with lots of hard work and healthy eating. It may take some more weans too, depending on whether I hit a wall again.

I know you and a lot of other BLA-ers are struggling right now. Its hard. I feel good right now because the scale went down this week and I’ve seen some physical changes in how my clothes are fitting. I know its depressing when you are not seeing that. But for you and everyone else, just hang in there. Do as much physical activity as you can, and at least control your diet, because that is in your control. I know we’re told the weight is supposed to come off on its own but I can tell a difference when I’m eating right and when I’m not. At least for me, I think it does help with the weight loss. At least psychologically I know I’m doing everything I can to make it come off.


By way of a mini update, I have lost another 2 pounds since I posted three days ago. This is getting exciting! And its not just water, you know the size 18 blank dress pants I just talked about in my last post? They are now TOO BIG!!! A friend of mine hadn’t seen me in two weeks and she was shocked today just to see the changes that have happened in two weeks. It really is noticeable.

Ok, hope I am not jinxing myself. When I update again in a few weeks hopefully I can report a very large weight loss and even more changes!


So, today I am 9 months post-op BLA. Its been almost two months since my last update. There’s been a lot of developments:

– In July, I got the results of my bone density scan: I have osteopenia and a severe vitamin D deficiency. I am now on 1200 mg of calcium a day and 50,000 IUs of Vitamin D a week.

– Hair is doing great! Not falling out, shiny, less frizzy.

– Energy is ok. Work is wearing me out, still working me beyond my medical restrictions, but I am supposed to be moving into a new job at the end of next month that will hopefully take care of some of that. I tend to get sleepy during the day and stressful days make me weak. I’ve also started waking up in the middle of the night again (NO!!! Why is this happening?!?!?) and there for a while I was waking up to pee in the middle of the night again. I wish that would stop because I was enjoying sleeping all the way to the morning.

– Stamina is great. I did a two-hour workout last week (weights and cardio) that was intense and awesome. I was so proud when I was done. I am considering returning to kickboxing in a few months if my Vitamin D levels go up and I have some confidence that my bones have gotten stronger.

– The weight is stalled out. I have lost 16 pounds now, but I haven’t lost a pound since mid-June. I weaned to 10 mg of hydro about three weeks ago and no results even with doing that. I don’t feel comfortable going any lower than that. Still at 1200 calories a day and low glycemic diet, heavy on protein, very little to no bread or cereal products. Husband and I met with reproductive endo here in Atlanta today (who I love!) and he expressed concern. My thyroid and insulin resistance tests are normal but he’s putting me on some Synthroid and Glucophage and some Prometrium. When I got my period in May the weight was just falling off . . . he thinks its PCOS-like issues and this combo of meds might help. So we’re going to try that and see how it goes.

– Stretch marks are much, much better – I noticed a marked difference after I weaned to 10 mg hydro. BLA scars are lightening too, especially with help of some new special cream from my dermotologist.

– Haven’t gotten my period again :( Boo. Hopefully the above cocktail will help with that.

– Had lasik surgery!!!! I love it. I did stress dose 30 mg extra for that. I did just fine.

So, positives yes but still very bummed about the struggles with the weight. I am hoping the new medicines will give me some results. I also feel like I’ve become more emotional lately because I’m tired of eating lettuce, spinach and egg whites (yes, that makes up a large portion of my diet) and working out and getting no relief. I hate being emotional and moody and feeling like I just can’t take it anymore. So I certainly do have those days. But thankfully they are just days – usually just one – and it passess and the next day I’m back in the battle. Because really – what else can you do?


I’m 10 months post-BLA today. Unfortunately, this update is not going to be as positive as some of my past updates.

The weight loss stands at 20 lbs now. I did start on Metformin and Synthroid at the beginning of August. I lost five pounds right away the first week, and then the weight loss stopped and I have gained back one pound. Nothing else has happened since then (despite doubling the dose of Metformin).

I can’t deny that I have become extremely depressed. Its been building for several months now. Its not just having the extra weight, but the weight keeping me from what I want to do – principally, have a baby. I’ve just lost interest in so many things and I am very down, despite the progress I have made in other areas of recovery.

I have discussed this with both Dr. L and my reproductive endo. I am going to Seattle in two weeks and we’re doing a round of labs and a growth hormone stim test. GH deficiency would explain a lot of things – the large amount of weight around the middle, the Cushie-like shape I still have. I still have a bit of a hump too.

My reproductive endo is re-testing all my thyroid hormones, estrogen, progesterone and a few others soon as well.

I am beginning to suspect I have slowly been becoming hypo-pit. Or perhaps hypo-pit in an intermittent way. I have no menstrual cycle anymore. I have ostepenia. I have energy to do stuff but then I get exhausted and sometimes it takes me days to recover. I have hot flashes, memory issues, loss of libido and insulin resistance. And, again, super slow weight loss that seems to go up every time I eat anything other than raw vegetables. I also have on and off DI.

So, I guess I am just at the end of my rope. I hope that someone can fix me. Because something is still clearly wrong.


I’m now 10 1/2 months post-op BLA. I just completed a visit to Dr. L in Seattle. I did the GH stim test and labs for thyroid, ACTH and some other things.

As I suspected, I do have some continuing issues – I am severely GH deficient. I didn’t stim above 0.9 during the entire stim test. I’ll be starting on GH as soon as possible.

My thyroid numbers are all in the normal range but they are low normal. We’re upping the Synthroid to 125 mg per day.

My MRI was clear – no new tumor (yay!) and my ACTH was 40. So that is all good. I feel hopeful that I am doing good in some areas and now we have identified the areas that are causing me problems.

I also had estrogen, FSH and LH tested today. I am hoping to find out if I am deficient there even though I haven’t been in the past – I have a suspicion the estrogen may be low now.

So, we’ll see where we stand in a few months when this medicine has had some time to kick in.


Today is the one year anniversary of my BLA. I am doing well. I’ll update here and post a separate 1 year post-op BLA thread so those who don’t follow here can be encouraged by my, dare I say it, success story?

The past month and a half I have seen some significant improvement. Here’s the breakdown:

Medicine every day:

 

12.5 mg of hydro (all taken in the morning)

0.1 mg florinef

1500 mg Metformin at night

125 mg of levothyroxine

Calcium pill and daily multivitamin

Progestrone pills on days 1-10 of each month

To start 0.2 mg of Genotropin in next few weeks

 

Energy: The thyroid medicine has helped a lot with energy. My thyroid numbers were all normal but just a bit on the low normal, so the docs didn’t think I needed meds. But I did, it has helped a lot. I am still tired but I am a lot better than I was. My GH is supposed to arrive today (yay!) so that should also help me on my path to recovery.

 

Weight loss: I haven’t really lost weight in the past few weeks but inches, oh my! I have lost inches. I have gotten tons of comments from friends, family, coworkers, etc on the change all over – face, body, etc. I am now down to a size 14. That is down from being mostly in maternity clothes and barely squeezing into a few size 18 pants a year ago. No more maternity clothes for me (for now!). Its so nice to be shopping in the regular clothes again. I have gone a bit crazy buying some new things – skinny jeans, sweaters, ballet flats, boots. I am all decked out for fall in the latest styles. It feels so good to be stylish and to have choices again.

 

The pregnancy look is gone. No more comments on when I am due or what sex the baby is. That is an awesome feeling. I’ve lost 20 solid pounds, some days a little more but it seems to always go back to that 20 number. I am trying not to weigh too much until the GH has a chance to start working.

 

Stretch marks: My stetch marks have really done some fading. Somedays they are more noticeable than others, but they are so so so much lighter than they were.

 

Hump: My hump is much smaller – its barely there at all now, I probably see it only because I am paranoid. But I have no issue wearing tank tops or anything that shows the back of my neck.

 

Hair: My hair has grown long and thicker than it used to be. Much less oily! I don’t have to wash it every single day now. I can put it in a ponytail on the weekends and it looks cute and not greasy.

 

Sleep: I am sleeping great. I sleep all through the night and don’t wake up anymore. That has become very consistent, which is a wonderful thing. I still feel tired though because of the GH but hopefully that will improve.

 

Activities: I work 32 hours a week. This works well, it gives me an extra day a week to rest and recover from the work week. I exercise often, I went back to kickboxing this month which is kicking my butt but I need to rebuild muscle. Its also nice to get back to your old hobbies.

 

Attention and Memory: This isn’t always as great, hoping the GH will help. I don’t focus as well as I used to or catch spelling details at work like I used to. Sometimes my memory is sharp and other times I forget something someone just told me or how to spell a word or the names of objects (or even people at times). Again, hoping GH will help here.

 

Female stuff: No period still, progesterone is not really helping. I think if the GH doesn’t help here, we’re going to move on to estrogen therapy in a few months. We’re hoping to start trying for a baby at the end of next year.

 

Other health issues: High blood pressure went away immediately after the BLA. I never did have a blood sugar problem so no issues there. I do have osteopenia which we are working on with more calcium and Vitamin D and weightlifting exercies. Again, hoping GH will help here.

 

Emotional: Really doing much better. I was getting really depressed for a while there, about 7-10 months post op. Even though I thought my expectations of recovery were reasonable, I was frustrated with my progress. I had hoped to lose more like 30 or 40 pounds in my first year. But, I found out I had other issues (thyroid and Gh deficiency) that were messing with that goal. So I only got halfway there but it wasn’t my fault. I also thought the weight would “fall off” more than it has, but it hasn’t. I’ve had to diet and exercise hard for every pound lost. Don’t know if that’s normal or if its just me or because of the thyroid/Gh issues we are still working on. But it helped me to know that at least there was a medical reason for my frustrations!

 

But overall I am really doing well. No one who meets me for the first time has any idea that I’ve been sick. I recently started a new position with my same employer (which has been going well) and I met my new team and everyone commented on my “glowing skin” and “happy nature.” I have no regrets about the BLA.

 

My advice to anyone considering it or just having had the BLA is: patience, patience, patience. Realistic expecatations. Then, hard work on controlling your diet and being physically fit. Do everything you can towards getting better, and then if time and hard work don’t pay off, don’t hesitate to detail your hard work and patience to your doctor and tell them to find out what else is holding you back.

 

I hope this is an inspiration to anyone out there who is struggling right now.


Wow, I am way overdue for an update! I’ve been out working, having fun and living my life!

Its been a little over three months since my last post here. I am now 1 year, three months and ten days post op BLA. Here’s the breakdown of where I am now:

Medicine every day:

 

12.5 mg of hydro (all taken in the morning)

0.1 mg florinef

1500 mg Metformin at night

150 mg of levothyroxine

Calcium pill and daily multivitamin

Progestrone pills on days 1-10 of each month

0.2 mg of Genotropin 7 days a week (started in November)

 

Energy: I am doing great here. I am tired sometimes and traveling or working long hours wears me out, but I have limited that in my life with my new job. At my new job I am working full time now, 40 hours a week. I also work out 5-6 days a week now at very energetic things like kickboxing (with punching bags) or the elliptical machine.

 

Weight loss: I didn’t lose anything between my last post and the end of December. When my thyroid medicine was raised to 150 mg at Christmas, combined with continuing my workout and diet, I really started to see results. I have lost 10 lbs since then, for a total of 31 lbs now. Still, I am not losing at the rate I should be for the math of the intake/output of my diet and working out. We are working on that, possibly some more meds to come soon. But it is much, much improved! I have about 35 pounds to go until I am at a good weight for me. Ideally I’d like to lose 45 more but 35 more would be a healthy weight for me.

 

The best thing I did was have my husband hide the scale. I only weigh every six weeks now. Now I can focus on the process and not focus on how hard it is to get the scale to go down or get depressed when it doesn’t budge. I am now in size 12 clothes. I was a 6-8 before Cushing’s, sometimes I could wear a 4. I have a few more sizes till I can wear most of the clothes in my closet.

 

Stretch marks: This is about the same since my last post. My stetch marks are almost all white. Somedays they are more noticeable than others or pinker than usual, but they are so so so much lighter than they were.

 

Hump: Same as last post – much smaller and hardly there at all.

 

Hair: Same as last post – doing great.

 

Sleep: Same as last post – doing great.

 

Activities: Like I said above, working 40 hours a week, kickboxing probably 3-4 times a week, other days I work out on the elliptical machine and lifting weights. I go walking or hiking with my husband on the weekends if the weather is nice, but this low impact working out didn’t do much for the weight loss. The kickboxing has really helped.

 

Attention and Memory: This is about the same as last time. I can’t tell that it has improved all that much. I forget things (like reminding my husband to do something when he has asked me to remind him) all the time.

 

Female stuff: No period still, had blood drawn for estrogen today. Will see whether I am going on that or not.

 

Other health issues: High blood pressure went away immediately after the BLA. No return of that, blood pressure is very good. I never did have a blood sugar problem so no issues there. I do have osteopenia which we are working on with more calcium and Vitamin D and weightlifting exercies. Again, hoping GH will help here. My sinus issues have really escalated and just never got better after surgery. I’ve had a persistent sinus infection for two years. I have mold and some other bacteria in there that countless treatments have not killed. I am having the sinuses washed in a surgery at the end of the month and am now working with an infectious disease doctor to try to kill it. Its too gross to talk about!

 

Emotional: I am really doing well. The recent weight loss has really pleased me. I don’t think I am at the maximum improvement for my weight loss rate yet, but hopefully we are getting there. I am pulling out old clothes I haven’t worn in years out of my closet. I now officially weigh less than my husband for the first time in over two years, which is also wonderful.

 

So, that is about it for now. I will update again when there are more developments!


P.S. – Notable fitness accomplishment! Six weeks ago throughout a kickboxing class I could do about 5 girlie push-ups (on knees). Last night at kickboxing class I did a total of 5 interspaced intervals of 10 for a total of . . . 50 push-ups!

 

The power of regular exercise and GH unites!


Today is the two year anniversary of my BLA. It is hard to believe that much time has passed. I can say with 100% confidence that I am doing so much better and that the BLA was the right thing for me.

I’ll update this along the same lines as my one year update, just in the name of consistency:

 

Here’s the breakdown on my meds:

 

Medicine every day:

 

7.5 mg of hydro (all taken in the morning)

0.1 mg florinef

125 mg of levothyroxine

Calcium pill and daily multivitamin

Prenatal vitamin

0.6 mg of Neutropin (next month will be going up to 1 mg Neutropin)

Birth control pills (formerly was taking 0.2 mg estrogen supplement and progesterone on days 1-10 of month)

2 tsp. of Royal Jelly and Bee Pollen in honey daily

Flonase

 

Energy: The thyoid and GH have helped a lot in this area. I could still use a little help because my GH is still very low, but I really am doing great anyways. Getting the thyroid dose right has been a battle, but I think we finally found the right dose.

 

Weight loss: I have now lost a total of 34 lbs, down from high of 206 to 172. At 5’5 I am a normal size 12 and its great. I look and feel like a normal person again (my mom even says I am “skinny” but I don’t know about that!) I am losing more inches now than I am weight. This is partly due to the need for higher GH, and partly because I am not doing the hard working out and strict dieting because my hubbie and I are working on Baby #1!!! I have fought hard with diet and exercise for every pound lost – nothing has come off easily for me.

 

So, the pregnancy look may be back in a few months, but this time it will be because I am actually pregnant

 

Stretch marks: i barely notice them at all now. My BLA surgical incisions have done a great job fading as well. I don’t know if a bikini is ever in my future, but if I am in that great shape again I might wear one around family and friends despite the scars.

 

Hump: Gone

 

Hair: Doesn’t fall out anymore, its grown long and thicker, less oily. I think the prenatal vitamins have helped in that area too.

 

Sleep: I sleep like a baby every night. I have been for a while. No more waking up, no more problems falling asleep. I do need more sleep than most people, and I am wondering if this is still due to the GH deficiency.

 

Activities: I work 40 hours a week and have been since probably the beginning of the year. I’ve been in my new job now for a year and it has been such a blessing. The reduced stress makes it possible for me to work full time.

 

Attention and Memory: This is the same as last year. It isn’t always as great, hoping the GH will help. I don’t focus as well as I used to or catch spelling details at work like I used to. Sometimes my memory is sharp and other times I forget something someone just told me or how to spell a word or the names of objects (or even people at times). Again, hoping GH will help here.

 

Female stuff: I need a combination of estrogen and progesterone in order to have a period. This still does not cause ovulation. So, we are using fertility mediation to induce ovlutation in order to get pregnant.

 

Other health issues: Same – High blood pressure went away immediately after the BLA. I never did have a blood sugar problem so no issues there. I do have osteopenia which we are working on with more calcium and Vitamin D and weightlifting exercies. Again, hoping GH will help here. I had some problems with my gums recessing and GH and better female hormones have helped there too.

 

My sinus recovery from the pit surgery has really been hard, perhaps my worst problem of all. I had surgery in April to correct the deviated septum caused by the pit surgery. I have been on and off antibiotics like crazy. I was a habitual Neti-Pot user with no improvement. Finally, I started using those spray irrigation cans twice a day, combined with Flonase to lessen the mucus, and that has helped for the past 8 weeks. I’ve seen my best improvement since by pit surgery 2.5 years ago. So let’s hope that continues.

 

Emotional: i am really very happy in my life. I am not depressed anymore and so many good things are happening to me. I thought I would have lost more weight by now but solving the GH deficiency has really taken a long time (and its still not resolved yet). Also, its important when using fertility medications to take it easy and not eat a restrictive diet, so I’ve been focusing more on the things to help us have a baby more than weight loss. I pray we are successful in having kids, and I will get back on the weight loss track after that. But its so positive to shop in normal clothes and not even be considered plus size anymore!

 

My relationship with my husband is great, unlike so many relationships we pulled together through Cushing’s and it made us stronger.

 

I am still working to have patience in the recovery and just to recognize that it goes on for a long while. I am two years out and things improve all the time. Its just good to be in a place where things are getting better rather than worse, and I can eat a piece of pizza and not gain 5 lbs, and actually be out enjoying life. Hopefully this next year I can tackle motherhood too :)


So far the BLA hasn’t been the doctor’s concern at all for getting pregnant. The problem has been the lost pituitary hormones from the pituitary surgery. If I get pregnant, there will be focus on keeping the cortisol levels appropriate, as they rise naturally during pregnancy and my meds will have to do that. But I would guess someone who did not have a BLA and had pit surgery and is still reliant on cortisol replacement would have the same issue.

There is also some focus on cortisol dosage if I have morning sickness in order to avoid AI, but the docs don’t seem too concerned and feel confident we can handle it.

PS- this was why I chose the BLA over the second pit surgery, although I lost ovulation with the first pit surgery, so fertility meds were unavoidable.


Wow, I can’t believe it, but yesterday was the three month (year!) anniversary of my BLA. I am doing awesome. Honestly, I hardly come on the boards anymore but I am trying to update this thread at least yearly in the hopes that it will help someone. Here is an update on the areas I have traditionally noted:

Here is the breakdown on my meds:

 

Hydrocortisone: There is controversy here. Technically, I am supposed to be taking 7.5 mg a day as the minimun. But its too much for me. I can live without it. I have gone months living without it. Every now and then if I feel bad I will take 5 mg. The rest tissue testing I have done at Vanderbilt has been negative for rest tissue, but clearly something is going on. I’ve also lost weight being off of the hydro.

 

Fludrocortine: Again, I am supposed to be on 0.1 mg a day, but I can live without it. I may need to take a pill once every three or four weeks, but otherwise I am fine right now.

 

125 mg of levothyroxine

 

0.6 mg of Nutropin

 

Calcium, multivitamins

 

Vaginal progestrone suppositories – these, combined with no hydro, have really helped the weight peel off

 

Estrogen patch – same, have helped the weight come off, because oral meds interfere with GH

 

Energy: I am doing great, working 40+ hours a week. Sometimes pain in my knees interferes with my workouts, but otherwise I am doing fine as long as I get 8-9 hours of sleep a night.

 

Sleep: doing great, fall asleep and usually no waking up.

 

Weight: Awesome, i made huge strides this year with the change in the manner in which female hormones are put into my body and going off the hydro. I lost 30 lbs this year, and I have now lost 64 of the 66 I gained with Cushing’s. I am wearing a size 6 or 8 depending on the store.brand. Before Cushing’s it was a 6 or a 4. But after all this, I consider this a huge success story :)

 

Hump: still gone, and man, do I have collar bones now!

 

Hair: still doing great

 

Stretchmarks: Not very noticeable, and the BLA scars are very faint. A friend of mine (who saw them after surgery) saw them yesterday for the first time in three years and was amazed.

 

Other health issues: High blood pressure gone, high cholesterol gone, sinus issues are still present but I have now had two sinus surgeries. I may be going into IV antibiotic therapy next.

 

As far as Baby #1, I had a miscarriage in March but we determined the reason was not Cushing’s related and another fixable problem I had. So, hopefully in the future I will get my bundle of joy. I am much happier that I am now at a healthier weight for it (142 lbs at 5’5).

 

Again, so happy I made this decision. I consider myself fully cured, and I am still losing weight now without much effort. Before this year, I was fighting against unbalanced hormones and while I did lose 34 lbs during that time, it took me two years! This year, only one year and 30 lbs. Balanced hormones are totally necessary, but you also need the proper manner of distribution to your body, and healthy eating and exercise.

 

I hope this helps someone along their Cushing’s journey! There is hope and light at the end of the tunnel.


Time for another update I guess.  I am continuing to do really well.  I am down to 118lbs at 5’5.  I am a size 4, sometimes a size 2.  I never thought I would see any of those numbers again, but here I am!  I am feeling good in pretty much all respects.  The only bad thing is that I seem prone to sports injuries. I don’t know if its because I’m post-Cushings or if its just me.  I’ve been in physical therapy twice in the past year now.  But I am continuing to be active and have a healthy lifestyle.

I hope everyone is doing well.  As always, let me know if you have questions about anything in my journey.


Wow I didn’t realize how long it had been since my last update! So much has happened in the last 8 years. I’ve gotten divorced and since remarried. The biggest update is that I am pregnant from IVF and expecting my first child. There was always a question after my pituitary surgery on whether this would be possible. But I froze my eggs in 2013 and 2014 and finally can say that investment paid off :)

The pregnancy has put a lot of stress on my body so I’ve had to go back on hydrocortisone and fludro. I’ve been off of both for about ten years now and surviving just on my rest tissue.  I’ve done incredibly well! So far I’ve only gained a little more than what you are supposed to while pregnant so losing the weight will be my next project once this baby is born. I’m in my third trimester now.

Its been an incredible journey. I remember reading these boards and struggling to find anyone who had had a BLA and then gotten pregnant. I hope my journey will continue to help and inform others.

Basics: Adrenal Surgery: One Patient’s Experiences

Extracted and adapted from this series: https://cushings.invisionzone.com/topic/51040-on-my-way-to-getting-well/

Post 1) I was officially diagnosed with Cushing’s yesterday. I have a CT scan to check on my adrenal tumor and a meeting with my surgeon tomorrow. Hopefully they will schedule surgery for Monday or Tuesday. I have suffered over a year with this, been in congestive heart failure, and believe this cortisol caused my son to be stillborn in March. It’s been the year from hell. Please pray that all goes well tomorrow and that I will be cured of this once and for all!!

Post 2) Surgery set for the 23rd!!!!! He is planning a right adrenaltectomy. I am so darn excited…

Post 3) I’m almost two weeks out of adrenal surgery. He removed the tumor & my gland. This has been the hardest and most painful two weeks of my life. I am already noticing little changes in my body. My skin is getting texture, my hair is not as brittle, my swelling goes down each day, and my nails are white instead of yellow and are stronger. I am getting hair back on my arms, legs, & feet too. I can’t wait to continue to get well. I am ready to be able to get out and about. I am pretty much housebound now because of the pain of the withdrawal from the cortisol. I stay on my painkillers and rest in my recliner. Hubby bought it for me because I can’t sleep in the bed comfortably. He’s the best. He’s been sleeping on our air mattress in the living room with me for almost 2 weeks now. He is always there to help me get out of the recliner when I need to. He is amazing. Just wanted to update you all. Getting better everyday.

Post 4) I am on 40mg Hydrocortisone daily right now. I will have my first wean close to Christmas. I have an appt. on the 21st with my endo. She is fantastic and saved my life from this stuff. I am so blessed. Today is a rough day. I did have 2 good days in a row which was a huge blessing. Thanks for thinking of me!

Post 5) Well, I just survived month 1 of recovery. It was HORRIBLE. I have never had so much pain in my life. I am still on 40 mg and my endo. wants me to wean 10 mg starting on the 27th. We’ll see how it goes. I have so much pain, shaking, chills, no sleep NOW. I can’t imagine how its going to be on a lower dose. My cortisol level was SO HIGH (2107) before surgery. I knew this withdrawal was going to be terrible. SHe had never seen a level as high as mine before. The lab actually tested my urine twice because they didn’t believe it the first time. I am doing a lot of resting right now. I am very nervous about my mother leaving on New Year’s Day. I don’t know how I am going to handle my 3 year old on my own. I hurt so badly and my vision isn’t the greatest yet. Thanks for thinking of me and writing me back.

Post 6) We have another call into my endo about my suffering. I have done nothing but shake uncontrollably all day so far. I hurt so badly. I am up every hour at night writhing in pain. I refuse to suffer like this anymore. I want some relief. Thank you so much for all of the advice. It means the world to me. Great news is that I am off my BP meds as of today!! Cardiologist’s office said I could quit them. I am thrilled. Now to get this pain under control.

Post 7) Endo said we can do whatever I can tolerate. I am now doing 20/20/10 instead of 20/10/10. I am still in pain, but it’s a little more tolerable. She said if I am just miserable and can’t take the pain, then I can do a bedtime dose. I am going to try melatonin to help me sleep per her suggestion. She wants to see how I do on this new dose and start a slow wean in a few weeks.

Post 8) Things have been getting better by the week. New years day was my best physical and mental day so far. I can actually feel my old self returning! !! Today I have lots of bone/muscle pain. Its better than a few weeks ago by far. Yesterday I was able to enjoy my son and play with him for the first time in a long time. I could even dance a little with him. He was so happy. I am down to 20/17.5/10& am handling it well. The pain is tolerable. My hump is almost gone, my stomach is mushy and shrinking, skin is peeling and improving, hair is growing in normally. I will be six weeks out this Wed.

 

Light Up for Rare

The National Organization for Rare Disorders (NORD) asks Americans to plan ahead to participate in the Light Up for Rare campaign to raise awareness of rare diseases.

NORD is the U.S. sponsor for  Rare Disease Day  on Feb. 28. The annual awareness day spotlights approximately 7,000 rare diseases that affect more than 300 million people worldwide. More than 25 million Americans and their families are believed to be affected by rare diseases.

Participants are encouraged to light or decorate their homes in blue, green, pink, and purple at 7 p.m. local time on Feb. 28. (Blue should be used if only one color is possible.) NORD suggests using NovaBright to light up a building, monument, home, or neighborhood in these rare disease colors.

To join the Light Up for Rare campaign, sign up here. Participants should complete the applications required by the landmarks they pledge to light up, which could include historic buildings and homes, schools and universities, businesses, stadiums, bridges, and monuments. A downloadable template request is available to ask cities and buildings to participate in the initiative.

Once requests are approved, participants should inform NORD so the organization can track the buildings that will be illuminated for Rare Disease Day.

Light Up for Rare is part of the Global Chain of Lights campaign, which aims to unite the rare disease community across the globe and symbolically break the isolation caused by the COVID-19 pandemic.

The European Organization for Rare Diseases (EURORDIS), NORD’s counterpart in Europe, is coordinating the Feb. 28 awareness day there along with several patient advocacy groups. On leap years, Rare Disease Day falls on Feb. 29, the rarest day of the year.

Download the Light Up for Rare toolkit here. Information on how to illuminate a building can be found here

The general public, as well as caregivers, healthcare professionals, researchers, clinicians, policymakers, and industry representatives are encouraged to participate in Rare Disease Day advocacy and events. Other toolkits and resources for Rare Disease Day are available here.

After buildings and landmarks are lit up in Rare Disease Day colors, participants are encouraged to share photos and videos on social media. Please use the #RareDiseaseDay and #ShowYourStripes hashtags so the efforts can be spotlighted.

More information at https://rarediseases.org/rare-disease-day/rare-disease-day-light-up-for-rare/

Basics: The Pituitary Gland: Small But Mighty

The pituitary gland works hard to keep you healthy, doing everything from ensuring proper bone and muscle growth to helping nursing mothers produce milk for their babies. Its functionality is even more remarkable when you consider the gland is the size of a pea.

“The pituitary is commonly referred to as the ‘master’ gland because it does so many important jobs in the body,” says Karen Frankwich, MD, a board-certified endocrinologist at Mission Hospital. “Not only does the pituitary make its own hormones, but it also triggers hormone production in other glands. The pituitary is aided in its job by the hypothalamus. This part of the brain is situated above the pituitary, and sends messages to the gland on when to release or stimulate production of necessary hormones.”

These hormones include:

  • Growth hormone, for healthy bone and muscle mass
  • Thyroid-stimulating hormone, which signals the thyroid to produce its hormones that govern metabolism and the body’s nervous system, among others
  • Follicle-stimulating and luteinizing hormones for healthy reproductive systems (including ovarian egg development in women and sperm formation in men, as well as estrogen and testosterone production)
  • Prolactin, for breast milk production in nursing mothers
  • Adrenocorticotropin (ACTH), which prompts the adrenal glands to produce the stress hormone cortisol. The proper amount of cortisol helps the body adapt to stressful situations by affecting the immune and nervous systems, blood sugar levels, blood pressure and metabolism.
  • Antidiuretic (ADH), which helps the kidneys control urine levels
  • Oxytocin, which can stimulate labor in pregnant women

The work of the pituitary gland can be affected by non-cancerous tumors called adenomas. “These tumors can affect hormone production, so you have too little or too much of a certain hormone,” Dr. Frankwich says. “Larger tumors that are more than 1 centimeter, called macroadenomas, can also put pressure on the area surrounding the gland, which can lead to vision problems and headaches. Because symptoms can vary depending on the hormone that is affected by a tumor, or sometimes there are no symptoms, adenomas can be difficult to pinpoint. General symptoms can include nausea, weight loss or gain, sluggishness or weakness, and changes in menstruation for women and sex drive for men.”

If there’s a suspected tumor, a doctor will usually run tests on a patient’s blood and urine, and possibly order a brain-imaging scan. An endocrinologist can help guide a patient on the best course of treatment, which could consist of surgery, medication, radiation therapy or careful monitoring of the tumor if it hasn’t caused major disruption.

“The pituitary gland is integral to a healthy, well-functioning body in so many ways,” Dr. Frankwich says. “It may not be a major organ you think about much, but it’s important to know how it works, and how it touches on so many aspects of your health.”

Adapted from http://www.stjhs.org/HealthCalling/2016/December/The-Pituitary-Gland-Small-but-Mighty.aspx

Thoughts? Share on the message boards.

Basics: Testing: IGF-1 (Insulin-like growth factor 1)

Aim—To contribute to the debate about whether growth hormone (GH) and insulin-like growth factor 1 (IGF-1) act independently on the growth process.

Methods—To describe growth in human and animal models of isolated IGF-1 deficiency (IGHD), such as in Laron syndrome (LS; primary IGF-1 deficiency and GH resistance) and IGF-1 gene or GH receptor gene knockout (KO) mice.

Results—Since the description of LS in 1966, 51 patients were followed, many since infancy. Newborns with LS are shorter (42–47 cm) than healthy babies (49–52 cm), suggesting that IGF-1 has some influence on intrauterine growth. Newborn mice with IGF-1 gene KO are 30% smaller. The postnatal growth rate of patients with LS is very slow, the distance from the lowest normal centile increasing progressively. If untreated, the final height is 100–136 cm for female and 109–138 cm for male patients. They have acromicia, organomicria including the brain, heart, gonads, genitalia, and retardation of skeletal maturation. The availability of biosynthetic IGF-1 since 1988 has enabled it to be administered to children with LS. It accelerated linear growth rates to 8–9 cm in the first year of treatment, compared with 10–12 cm/year during GH treatment of IGHD. The growth rate in following years was 5–6.5 cm/year.

Conclusion—IGF-1 is an important growth hormone, mediating the protein anabolic and linear growth promoting effect of pituitary GH. It has a GH independent growth stimulating effect, which with respect to cartilage cells is possibly optimised by the synergistic action with GH.

Keywords: insulin-like growth factor I, growth hormones, Laron syndrome, growth

In recent years, new technologies have enabled many advances in the so called growth hormone (GH) axis (fig 1). Thus, it has been found that GH secretion from the anterior pituitary is regulated not only by GH releasing hormone (GHRH) and somatostatin (GH secretion inhibiting hormone), but also by other hypothalamic peptides called GH secretagogues, which seem to act in synergism with GHRH by inhibiting somatostatin. One of these has been cloned and named Ghrelin. The interplay between GHRH and somatostatin induces a pulsatile GH secretion, which is highest during puberty. GH induces the generation of insulin-like growth factor 1 (IGF-1, also called somatomedin 1) in the liver and regulates the paracrine production of IGF-1 in many other tissues.

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The cascade of the growth hormone axis. CNS, central nervous system; GH, growth hormone; GHBP, GH binding protein; GH-S, GH secretagogues; IGF-1, insulin-like growth factor 1; IGFBPs, IGF binding proteins; +, stimulation; –, inhibition.

IGF-1

IGF-1 and IGF-2 were identified in 1957 by Salmon and Daughaday and designated “sulphation factor” by their ability to stimulate 35-sulphate incorporation into rat cartilage. Froesch et al described the non-suppressible insulin-like activity (NSILA) of two soluble serum components (NSILA I and II). In 1972, the labels sulphation factor and NSILA were replaced by the term “somatomedin”, denoting a substance under control and mediating the effects of GH. In 1976, Rinderknecht and Humbel isolated two active substances from human serum, which owing to their structural resemblance to proinsulin were renamed “insulin-like growth factor 1 and 2” (IGF-1 and 2). IGF-1 is the mediator of the anabolic and mitogenic activity of GH.

CHEMICAL STRUCTURE

The IGFs are members of a family of insulin related peptides that include relaxin and several peptides isolated from lower invertebrates. IGF-1 is a small peptide consisting of 70 amino acids with a molecular weight of 7649 Da. Similar to insulin, IGF-1 has an A and B chain connected by disulphide bonds. The C peptide region has 12 amino acids. The structural similarity to insulin explains the ability of IGF-1 to bind (with low affinity) to the insulin receptor.

THE IGF-1 GENE

The IGF-1 gene is on the long arm of chromosome 12q23–23. The human IGF-1 gene consists of six exons, including two leader exons, and has two promoters.

IGF binding proteins (IGFBPs)

In the plasma, 99% of IGFs are complexed to a family of binding proteins, which modulate the availability of free IGF-1 to the tissues. There are six binding proteins. In humans, almost 80% of circulating IGF-1 is carried by IGFBP-3, a ternary complex consisting of one molecule of IGF-1, one molecule of IGFBP-3, and one molecule of an 88 kDa protein named acid labile subunit. IGFBP-1 is regulated by insulin and IGF-1; IGFBP-3 is regulated mainly by GH but also to some degree by IGF-1.

The IGF-1 receptor

The human IGF-1 receptor (type 1 receptor) is the product of a single copy gene spanning over 100 kb of genomic DNA at the end of the long arm of chromosome 15q25–26. The gene contains 21 exons (fig 2) and its organisation resembles that of the structurally related insulin receptor (fig 3). The type 1 IGF receptor gene is expressed by almost all tissues and cell types during embryogenesis. In the liver, the organ with the highest IGF-1 ligand expression, IGF-1 receptor mRNA is almost undetectable, possibly because of the “downregulation” of the receptor by the local production of IGF-1. The type 1 IGF receptor is a heterotetramer composed of two extracellular spanning α subunits and transmembrane β subunits. The α subunits have binding sites for IGF-1 and are linked by disulphide bonds (fig 3). The β subunit has a short extracellular domain, a transmembrane domain, and an intracellular domain. The intracellular part contains a tyrosine kinase domain, which constitutes the signal transduction mechanism. Similar to the insulin receptor, the IGF-1 receptor undergoes ligand induced autophosphorylation. The activated IGF-1 receptor is capable of phosphorylating other tyrosine containing substrates, such as insulin receptor substrate 1 (IRS-1), and continues a cascade of enzyme activations via phosphatidylinositol-3 kinase (PI3-kinase), Grb2 (growth factor receptor bound protein 2), Syp (a phophotyrosine phosphatase), Nck (an oncogenic protein), and Shc (src homology domain protein), which associated to Grb2, activates Raf, leading to a cascade of protein kinases including Raf, mitogen activated protein (MAP) kinase, 5 G kinase, and others.

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Type 1 insulin-like growth factor receptor gene and mRNA. Reproduced with permission from Werner.

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Resemblance between the insulin and insulin-like growth factor 1 (IGF-1) receptors.

Physiology

IGF-1 is secreted by many tissues and the secretory site seems to determine its actions. Most IGF-1 is secreted by the liver and is transported to other tissues, acting as an endocrine hormone. IGF-1 is also secreted by other tissues, including cartilagenous cells, and acts locally as a paracrine hormone (fig 4). It is also assumed that IGF-1 can act in an autocrine manner as an oncogene. The role of IGF-1 in the metabolism of many tissues including growth has been reviewed recently.

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Paracrine insulin-like growth factor 1 (IGF-1) secretion and endocrine IGF-1 targets in the various zones of the epiphyseal cartilage growth zone.

The following is an analysis of whether IGF-1, the anabolic effector hormone of pituitary GH, is the “real growth hormone”.

Is IGF-1 “a” or “the” growth hormone?

The discussion on the role of IGF-1 in body growth will be based on growth in states of IGF-1 deficiency and the effects of exogenous IGF-1 administration. Experiments in nature (gene deletion or gene mutations) or experimental models in animals, such as gene knockouts, help us in this endeavour. In 1966 and 1968, we described a new type of dwarfism indistinguishable from genetic isolated GH deficiency (IGHD), but characterised by high serum GH values. Subsequent studies revealed that these patients cannot generate IGF-1.

This syndrome of GH resistance (insensitivity) was named by Elders et al as Laron dwarfism, a name subsequently changed to Laron syndrome (LS). Molecular studies revealed that the causes of GH resistance are deletions or mutations in the GH receptor gene, resulting in the failure to generate IGF-1 and a reduction in the synthesis of several other substances, including IGFBP-3. This unique model in humans has enabled the study of the differential effects of GH and IGF-1.

Growth and development in congenital (primary) IGF-1 deficiency (LS)

Our group has studied and followed 52 patients (many since birth) throughout childhood, puberty, and into adulthood. We found that newborns with LS are slightly shorter at birth (42–47 cm) than healthy babies (49–52 cm), suggesting that IGF-1 has some influence on intrauterine linear growth. This fact is enforced by the findings that already at birth, and throughout childhood, skeletal maturation is retarded, as is organ growth. These growth abnormalities include a small brain (as expressed by head circumference), a small heart (cardiomicria), and acromicria (small chin, resulting from underdevelopment of the facial bones, small hands, and small feet). IGF-1 deficiency also causes underdevelopment and weakness of the muscular system, and impairs and weakens hair and nail growth. These findings are identical to those described in IGHD. IGF-1 deficiency throughout childhood causes dwarfism (final height if untreated, 100–135 cm in female and 110–142 cm in male patients), with an abnormally high upper to lower body ratio. One patient reported from the UK was found to have a deletion of exons 4 and 5 of the IGF-1 gene and he too was found to have severe growth retardation.

Impaired growth and skeletal development in the absence of IGF-1 were confirmed in mice using knockout (KO) of the IGF-1 gene or GH receptor gene.

Knockout of the IGF-1 gene or the IGF-1 receptor gene reduces the size of mice by 40–45%. Lack of the IGF-1 receptor is lethal at birth in mice owing to respiratory failure caused by impaired development of the diaphragm and intercostal muscles. In another model, the mice remained alive and their postnatal growth was reduced.

In conclusion, findings in humans and in animals show that IGF-1 deficiencies causes pronounced growth retardation in the presence of increased GH values.

The following is a summary of the results of the growth stimulating effects of the administration of exogenous IGF-1 to children and experimental data.

Growth promoting effects of IGF-1

The first demonstration that exogenous IGF-1 stimulates growth was the administration of purified hormone to hypophysectomised rats. After the biosynthesis of IGF-1 identical to the native hormone, trials of its use in humans were begun; first in adults and then in children. Our group was the first to introduce long term administration of biosynthetic IGF-1 to children with primary IGF-1 deficiency—primary GH insensitivity or LS. The finding that daily IGF-1 administration raises serum alkaline phosphatose, which is an indicator of osteoblastic activity, and serum procollagen, in addition to IGFBP-3, led to long term treatment. Treatment of patients with LS was also initiated in other parts of the world. The difference between us and the other groups was that we used a once daily dose, whereas the others administered IGF-1 twice daily. Table 1 compares the linear growth response of children with LS treated by four different groups. It can be seen that before treatment the mean growth velocity was 3–4.7 cm/year and that this increased after IGF-1 treatment to 8.2–9.1 cm/year, followed by a lower velocity of 5.5–6.4 cm/year in the next two years. (In GH treatment the highest growth velocity registered is also in the first year of treatment.) Figure 5 illustrates the growth response to IGF-1 in eight children during the first years of treatment. Ranke and colleagues reported that two of their patients had reached the third centile (Tanner), as did the patient of Krzisnik and Battelino; however, most patients did not reach a normal final height. The reasons may be late initiation of treatment, irregular IGF-1 administration, underdosage, etc. Ranke et al conclude that long term treatment of patients with LS promoted growth and, if treatment is started at an early age, there is a considerable potential for achieving height normalisation. Because no patient in our group was treated since early infancy to final height we cannot confirm this opinion.

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Growth velocity before and during insulin-like growth factor 1 (IGF-1) treatment. Note that in infancy, when the non-growth hormone/IGF-1 dependent growth velocity is relatively high (but low for age), the change induced by IGF-1 administration is less than in older children.

Table 1

Linear growth response of children with Laron syndrome treated by means of insulin-like growth factor 1 (IGF-1)

At start Growth velocity (cm/year) Year of treatment
Authors Year Ref. N Age range (years) BA (years) Ht SDS (m) IGF-1 dose (μg/kg/day) 0 1st 2nd 3rd
(n = 26) (n = 18)
Ranke et al 1995 31 3.7–19 1.8–13.3 −6.5 40–120 b.i.d. 3.9 (1.8) 8.5 (2.1) 6.4 (2.2)
(n = 5) (n = 5) (n = 1)
Backeljauw et al 1996 5 2–11 0.3–6.8 −5.6 80–120 b.i.d. 4.0 9.3 6.2 6.2
(n = 9) (n = 6) (n = 5)
Klinger and Laron 1995 9 0.5–14 0.2–11 −5.6 150–200 i.d 4.7 (1.3) 8.2 (0.8) 6 (1.3) 4.8 (1.3)*
(n = 15) (n = 15) (n = 6)
Guevarra-Aguirre et al 1997 15 3.1–17 4.5–9.3 120 b.i.d. 3.4 (1.4) 8.8 (11) 6.4 (1.1) 5.7 (1.4)
(n = 8) (n = 8)
Guevarra-Aguire et al 8 80 b.i.d. 3.0 (1.8) 9.1 (2.2) 5.6 (2.1)

Growth velocity values are mean (SD).

*The younger children had a growth velocity of 5.5 and 6.5 cm/year.

BA, bone age; b.i.d., twice daily; CA, chronological age; i.d., once daily; Ht SDS, height standard deviation score.

When the growth response to GH treatment in infants with IGHD was compared with that of IGF-1 in infants with LS we found that the infants with IGHD responded faster and better than those with LS. However, the small number of patients and the differences in growth retardation between the two groups makes it difficult to reach a conclusion.

Both hormones stimulated linear growth, but GH seemed more effective than IGF-1. One cause may be the greater growth deficit of the infants with LS than those with IGHD, an insufficient dose of IGF-1, or that there is a need for some GH to provide an adequate stem cell population of prechondrocytes to enable full expression of the growth promoting action of IGF-1, as postulated by Green and colleagues and Ohlson et al. All the above findings based on a few clinical studies with small groups of patients and a few experimental studies remain at present controversial. The crucial question is whether there are any, and if so, whether there are sufficient IGF-1 receptors in the “progenitor cartilage zone” of the epiphyseal cartilage (fig 4) to respond to endocrine and exogenous IGF-1. Using the mandibular condyle of 2 day old ICR mice, Maor et al showed that these condyles, which resemble the epiphyseal plates of the long bones, contain IGF-1 and high affinity IGF-1 receptors also in the chondroprogenitor cell layers, which enables them to respond to IGF-1 in vitro.

Sims et al, using mice with GH receptor KO showed that IGF-1 administration stimulates the growth (width) of the tibial growth plate and that IGF-1 has a GH independent effect on the growth plate. These findings are similar to those found when treating hypophysectomised rats with IGF-1.

In conclusion, IGF-1 is an important growth hormone, mediating the anabolic and linear growth promoting effect of pituitary GH protein. It has a GH independent growth stimulating effect, which with respect to cartilage cells is possibly optimised by the synergistic action with GH.

References

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2. Laron Z. Growth hormone secretagogues: clinical experience and therapeutic potential. Drugs 1995;50:595–601. [PubMed[]
3. Ghigo E, Boghen M, Casanueva FF, et al., eds. Growth hormone secretagogues. Basic findings and clinical implications. Amsterdam: Elsevier, 1994.
4. Jaffe CA, Ho PJ, Demott-Friberg R, et al. Effects of a prolonged growth hormone (GH)-releasing peptide infusion on pulsatile GH secretion in normal men. J Clin Endocrinol Metab 1993;77:1641–7. [PubMed[]
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7. Laron Z. The somatostatin-GHRH-GH-IGF-I axis. In: Merimee T, Laron Z, eds. Growth hormone, IGF-I and growth: new views of old concepts. Modern endocrinology and diabetes, Vol. 4. London-Tel Aviv: Freund Publishing House Ltd, 1996:5–10.
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11. Rinderknecht E, Humbel RE. Polypeptides with non-suppressible insulin-like and cell-growth promoting activities in human serum: isolation, chemical characterization, and some biological properties of forms I and II. Proc Natl Acad Sci U S A 1976;73:2365–9. [PMC free article] [PubMed[]
12. Laron Z. Somatomedin-1 (recombinant insulin-like growth factor-I). Clinical pharmacology and potential treatment of endocrine and metabolic disorders. Biodrugs 1999;11:55–70. [PubMed[]
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16. Mullis PE, Patel MS, Brickell PM, et al. Growth characteristics and response to growth hormone therapy in patients with hypochondroplasia: genetic linkage of the insulin-like growth factor I gene at chromosome 12q23 to the disease in a subgroup of these patients. Clin Endocrinol 1991;34:265–74. [PubMed[]
17. Rotwein P. Structure, evolution, expression and regulation of insulin-like growth factors I and II. Growth Factors 1991;5:3–18. [PubMed[]
18. Hwa V, Oh Y, Rosenfeld RG. The insulin-like growth factor binding protein (IGFBP) superfamily. Endocr Rev 1999;20:761–87 [PubMed[]
19. Lewitt MS, Saunders H, Phuyal JL, et al. Complex formation by human insulin-like growth factor-binding protein-3 and human acid-labile subunit in growth hormone-deficient rats. Endocrinology 1994;134:2402–9. [PubMed[]
20. Laron Z, Suikkairi AM, Klinger B, et al. Growth hormone and insulin-like growth factor regulate insulin-like growth factor-binding protein-1 in Laron type dwarfism, growth hormone deficiency and constitutional short stature. Acta Endocrinol 1992;127:351–8. [PubMed[]
21. Kanety H, Karasik A, Klinger B, et al. Long-term treatment of Laron type dwarfs with insulin-like growth factor I increases serum insulin-like growth factor-binding protein 3 in the absence of growth hormone activity. Acta Endocrinol 1993;128:144–9. [PubMed[]
22. Werner H. Molecular biology of the type I IGF receptor. In: Rosenfeld RG, Roberts CT, Jr, eds. The IGF system—molecular biology, physiology and clinical applications. Totowa, NJ: Humana Press, 1999:63–88.
23. Seino S, Seino M, Nishi S, et al. Structure of the human insulin receptor gene and characterization of its promoter. Proc Natl Acad Sci U S A 1989;86:114–18. [PMC free article] [PubMed[]
24. Bondy CA, Werner H, Roberts CT, Jr, et al. Cellular pattern of insulin-like growth factor I (IGF-I) and type I IGF receptor gene expression in early organogenesis: comparison with IGF-II gene expression. Mol Endocrinol 1990;4:1386–98. [PubMed[]
25. Kato H, Faria TN, Stannard B, et al. Essential role of tyrosine residues 1131, 1135, and 1136 of the insulin-like growth factor-I (IGF-I) receptor in IGF-I action. Mol Endocrinol 1994;8:40–50. [PubMed[]
26. LeRoith D, Werner H, Beitner-Johnson D, et al. Molecular and cellular aspects of the insulin-like growth factor I receptor. Endocr Rev 1995;16:143–63. [PubMed[]
27. Merimee T, Laron Z, eds. Growth hormone, IGF-I and growth: new views of old concepts. Modern endocrinology and diabetes, Vol. 4. London-Tel Aviv: Freund Publishing House Ltd, 1996.
28. D’Ercole AJ, Applewhite GT, Underwood LE. Evidence that somatomedin is synthesized by multiple tissues in the fetus. Dev Biol 1980;75:315–28 [PubMed[]
29. Nilsson A, Isgaard J, Lindhahl A, et al. Regulation by growth hormone of number of chondrocytes containing IGF-I in rat growth plate. Science 1986;233:571–4. [PubMed[]
30. Baserga R. The IGF-I receptor in cancer research. Exp Cell Res 1999;253:1–6. [PubMed[]
31. Rosenfeld RG, Roberts CT, Jr, eds. The IGF system—molecular biology, physiology and clinical applications. Totowa, NY: Humana Press, 1999.
32. Zapf J, Froesch ER. Insulin-like growth factor I actions on somatic growth. In: Kostyo J, ed. Handbook of physiology, Vol. V, Section 7. Philadelphia: American Physiological Society, 1999:663–99.
33. Laron Z, Pertzelan A, Mannheimer S. Genetic pituitary dwarfism with high serum concentration of growth hormone. A new inborn error of metabolism? Isr J Med Sci 1966;2:153–5. [PubMed[]
34. Laron Z, Pertzelan A, Karp M. Pituitary dwarfism with high serum levels of growth hormone. Isr J Med Sci 1968;4:883–94. [PubMed[]
35. Laron Z, Pertzelan A, Karp M, et al. Administration of growth hormone to patients with familial dwarfism with high plasma immunoreactive growth hormone. Measurement of sulfation factor, metabolic and linear growth responses. J Clin Endocrinol Metab 1971;33:332–42. [PubMed[]
36. Elders MJ, Garland JT, Daughaday WH, et al. Laron’s dwarfism: studies on the nature of the defect. J Pediatr 1973;83:253–63. [PubMed[]
37. Laron Z, Parks JS, eds. Lessons from Laron syndrome (LS) 1966–1992. A model of GH and IGF-I action and interaction. Pediatric and Adolescent Endocrinology 1993;24:1–367. []
38. Godowski PJ, Leung DW, Meacham LR, et al. Characterization of the human growth hormone receptor gene and demonstration of a partial gene deletion in 2 patients with Laron type dwarfism. Proc Natl Acad Sci U S A 1989;86:8083–7. [PMC free article] [PubMed[]
39. Amselem S, Duquesnoy P, Attree O, et al. Laron dwarfism and mutations of the growth hormone-receptor gene. N Engl J Med 1989;321:989–95. [PubMed[]
40. Laron Z. Laron syndrome—primary growth hormone resistance. In: Jameson JL, ed. Hormone resistance syndromes. Contemporary endocrinology, Vol. 2. Totowa, NJ: Humana Press, 1999:17–37.
41. Laron Z. Laron type dwarfism (hereditary somatomedin deficiency): a review. In: Frick P, Von Harnack GA, Kochsiek GA, et al, eds. Advances in internal medicine and pediatrics. Berlin-Heidelberg: Springer-Verlag, 1984:117–50. [PubMed]
42. Feinberg MS, Scheinowitz M, Laron Z. Echocardiographic dimensions and function in adults with primary growth hormone resistance (Laron syndrome). Am J Cardiol 2000;85:209–13. [PubMed[]
43. Brat O, Ziv I, Klinger B, et al. Muscle force and endurance in untreated and human growth hormone or insulin-like growth factor-I-treated patients with growth hormone deficiency or Laron syndrome. Horm Res 1997;47:45–8. [PubMed[]
44. Lurie R, Ben-Amitai D, Laron Z. Impaired hair growth and structural defects in patients with Laron syndrome (primary IGF-I deficiency) [abstract]. Horm Res 2001 [In press.]
45. Gluckman PD, Gunn AJ, Wray A, et al. Congenital idiopathic growth hormone deficiency associated with prenatal and early postnatal growth failure. J Pediatr 1992;121:920–3. [PubMed[]
46. Woods KA, Camacho-Hubner C, Savage MO, et al. Intrauterine growth retardation and postnatal growth failure associated with deletion of the insulin-like growth factor I gene. N Engl J Med 1996;335:1363–7. [PubMed[]
47. Zhou Y, Xu BC, Maheshwari HG, et al. A mammalian model for Laron syndrome produced by targeted disruption of the mouse growth hormone receptor/binding protein gene (the Laron mouse). Proc Natl Acad Sci U S A 1997;94:13215–20. [PMC free article] [PubMed[]
48. Sjogren K, Bohlooly YM, Olsson B, et al. Disproportional skeletal growth and markedly decreased bone mineral content in growth hormone receptor –/– mice. Biochem Biophys Res Commun 2000;267:603–8. [PubMed[]
49. Accili D, Nakae J, Kim JJ, et al. Targeted gene mutations define the roles of insulin and IGF-I receptors in mouse embryonic development. J Pediatr Endocrinol Metab 1999;12:475–85. [PubMed[]
50. Holzenberger M, Leneuve P, Hamard G, et al. A targeted partial invalidation of the insulin-like growth factor-I receptor gene in mice causes a postnatal growth deficit. Endocrinology 2000;141:2557–66. [PubMed[]
51. Schoenle E, Zapf J, Humbel RE, et al. Insulin-like growth factor I stimulates growth in hypophysectomized rats. Nature 1982;296:252–3. [PubMed[]
52. Guler H-P, Zapf J, Scheiwiller E, et al. Recombinant human insulin-like growth factor I stimulates growth and has distinct effects on organ size in hypophysectomized rats. Proc Natl Acad Sci U S A 1988;85:4889–93. [PMC free article] [PubMed[]
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55. Laron Z, Klinger B, Silbergeld A, et al. Intravenous administration of recombinant IGF-I lowers serum GHRH and TSH. Acta Endocrinol 1990;123:378–82. [PubMed[]
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Basics: Testing: Dex Tests

Dexamethasone suppression test measures whether adrenocorticotrophic hormone (ACTH) secretion by the pituitary can be suppressed.

How the Test is Performed

During this test, you will receive dexamethasone. This is a strong man-made (synthetic) glucocorticoid medicine. Afterward, your blood is drawn so that the cortisol level in your blood can be measured.

There are two different types of dexamethasone suppression tests: low dose and high dose. Each type can either be done in an overnight (common) or standard (3-day) method (rare). There are different processes that may be used for either test. Examples of these are described below.

Common:

  • Low-dose overnight — You will get 1 milligram (mg) of dexamethasone at 11 p.m., and a health care provider will draw your blood the next morning at 8 a.m. for a cortisol measurement.
  • High-dose overnight — The provider will measure your cortisol on the morning of the test. Then you will receive 8 mg of dexamethasone at 11 p.m. Your blood is drawn the next morning at 8 a.m. for a cortisol measurement.

Rare:

  • Standard low-dose — Urine is collected over 3 days (stored in 24-hour collection containers) to measure cortisol. On day 2, you will get a low dose (0.5 mg) of dexamethasone by mouth every 6 hours for 48 hours.
  • Standard high-dose — Urine is collected over 3 days (stored in 24-hour collection containers) for measurement of cortisol. On day 2, you will receive a high dose (2 mg) of dexamethasone by mouth every 6 hours for 48 hours.

Read and follow the instructions carefully. The most common cause of an abnormal test result is when instructions are not followed.

How to Prepare for the Test

The provider may tell you to stop taking certain medicines that can affect the test, including:

  • Antibiotics
  • Anti-seizure drugs
  • Medicines that contain corticosteroids, such as hydrocortisone, prednisone
  • Estrogen
  • Oral birth control (contraceptives)
  • Water pills (diuretics)

How the Test will Feel

When the needle is inserted to draw blood, some people feel moderate pain. Others feel only a prick or stinging. Afterward, there may be some throbbing or slight bruising. This soon goes away.

Why the Test is Performed

This test is done when the provider suspects that your body is producing too much cortisol. It is done to help diagnose Cushing syndrome and identify the cause.

The low-dose test can help tell whether your body is producing too much ACTH. The high-dose test can help determine whether the problem is in the pituitary gland (Cushing disease) or from a different site in the body (ectopic).

Dexamethasone is a man-made (synthetic) steroid that binds to the same receptor as cortisol. Dexamethasone reduces ACTH release in normal people. Therefore, taking dexamethasone should reduce ACTH level and lead to a decreased cortisol level.

If your pituitary gland produces too much ACTH, you will have an abnormal response to the low-dose test. But you can have a normal response to the high-dose test.

Normal Results

Cortisol level should decrease after you receive dexamethasone.

Low dose:

  • Overnight — 8 a.m. plasma cortisol lower than 1.8 micrograms per deciliter (mcg/dL) or 50 nanomoles per liter (nmol/L)
  • Standard — Urinary free cortisol on day 3 lower than 10 micrograms per day (mcg/day) or 280 nmol/L

High dose:

  • Overnight — greater than 50% reduction in plasma cortisol
  • Standard — greater than 90% reduction in urinary free cortisol

Normal value ranges may vary slightly among different laboratories. Some labs use different measurements or may test different specimens. Talk to your doctor about the meaning of your specific test results.

What Abnormal Results Mean

An abnormal response to the low-dose test may mean that you have abnormal release of cortisol (Cushing syndrome). This could be due to:

The high-dose test can help tell a pituitary cause (Cushing disease) from other causes. An ACTH blood test may also help identify the cause of high cortisol.

Abnormal results vary based on the condition causing the problem.

Cushing syndrome caused by an adrenal tumor:

  • Low-dose test — no decrease in blood cortisol
  • ACTH level — low
  • In most cases, the high-dose test is not needed

Ectopic Cushing syndrome:

  • Low-dose test — no decrease in blood cortisol
  • ACTH level — high
  • High-dose test — no decrease in blood cortisol

Cushing syndrome caused by a pituitary tumor (Cushing disease)

  • Low-dose test — no decrease in blood cortisol
  • High-dose test — expected decrease in blood cortisol

False test results can occur due to many reasons, including different medicines, obesity, depression, and stress. False results are more common in women than men.

Most often, the dexamethasone level in the blood is measured in the morning along with the cortisol level. For the test result to be considered accurate, the dexamethasone level should be higher than 200 nanograms per deciliter (ng/dL) or 4.5 nanomoles per liter (nmol/L). Dexamethasone levels that are lower can cause a false-positive test result.

Risks

There is little risk involved with having your blood taken. Veins and arteries vary in size from one patient to another, and from one side of the body to the other. Taking blood from some people may be more difficult than from others.

Other risks associated with having blood drawn are slight, but may include:

  • Excessive bleeding
  • Fainting or feeling lightheaded
  • Multiple punctures to locate veins
  • Hematoma (blood accumulating under the skin)
  • Infection (a slight risk any time the skin is broken)

Alternative Names

DST; ACTH suppression test; Cortisol suppression test

References

Chernecky CC, Berger BJ. Dexamethasone suppression test – diagnostic. In: Chernecky CC, Berger BJ, eds. Laboratory Tests and Diagnostic Procedures. 6th ed. St Louis, MO: Elsevier Saunders; 2013:437-438.

Guber HA, Oprea M, Russell YX. Evaluation of endocrine function. In: McPherson RA, Pincus MR, eds. Henry’s Clinical Diagnosis and Management by Laboratory Methods. 24th ed. St Louis, MO: Elsevier; 2022:chap 25.

Newell-Price JDC, Auchus RJ. The adrenal cortex. In: Melmed S, Auchus RJ, Goldfine AB, Koenig RJ, Rosen CJ, eds. Williams Textbook of Endocrinology. 14th ed. Philadelphia, PA: Elsevier; 2020:chap 15.

Review Date 5/13/2021

Updated by: Brent Wisse, MD, Board Certified in Metabolism/Endocrinology, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

From https://medlineplus.gov/ency/article/003694.htm

❓Can You Help?

I’m on my second round of GH (I had to stop the first time due to cancer). I’ve been on Omnitrope since 2006 and the threads inside my pen must have worn down – I’m having a lot of trouble injecting it now.

I insert the needle and have to balance on the edge of the countertop in the bathroom and sort of lean in. Has anyone else had to replace the pen? I told my insurance company who sends the actual drug and they said “not us”. I don’t know if my endo has to prescribe another “starter kit” or what.

I did find a website that will sell me a new one for $200 but I’d rather not buy one that way. I found nothing on the Novartis website about this, either :(

Anyone?

Please respond here or on the message boards.

Thanks!

👍 Research Opportunity! All of Us

I just signed up for this because it may be helpful for researchers at the NIH and elsewhere to learn more about Cushing’s, cancer, whatever else they can learn from my history.
Over 35 years ago, I agreed to be a part of a study at NIH so they could learn more about Cushing’s.  I consider this to be a larger, easier part of what I did back then.

From my bio: https://cushingsbios.com/2018/10/28/maryo-pituitary-bio/

As luck would have it, NIH (National Institutes of Health, Bethesda, Maryland) was doing a clinical trial of Cushing’s. I live in the same area as NIH so it was not too inconvenient but very scary at first to think of being tested there. At that time I only had a choice of NIH, Mayo Clinic and a place in Quebec to do this then-rare pituitary surgery called a Transsphenoidal Resection. I chose NIH – closest and free. After I was interviewed by the Doctors there, I got a letter that I had been accepted into the clinical trial. The first time I was there was for 6 weeks as an inpatient. More of the same tests.

There were about 12 of us there and it was nice not to be alone with this mystery disease. Many of these Cushies (mostly women) were getting bald, couldn’t walk, having strokes, had diabetes. One was blind, one had a heart attack while I was there. Towards the end of my testing period, I was looking forward to the surgery just to get this whole mess over with. While I was at NIH, I was gaining about a pound a day!

The MRI still showed nothing, so they did a Petrosal Sinus Sampling Test. That scared me more than the prospect of surgery. (This test carries the risk of stroke and uncontrollable bleeding from the incision points.) Catheters were fed from my groin area to my pituitary gland and dye was injected. I could watch the whole procedure on monitors. I could not move during this test or for several hours afterwards to prevent uncontrollable bleeding from a major artery. The test did show where the tumor probably was located. Also done were more sophisticated dexamethasone suppression tests where drugs were administered by IV and blood was drawn every hour (they put a heplock in my arm so they don’t have to keep sticking me). I got to go home for a weekend and then went back for the surgery – the Transsphenoidal Resection. I fully expected to die during surgery (and didn’t care if I did) so I signed my will and wrote last letters to those I wanted to say goodbye to. During the time I was home just before surgery, a college classmate of mine (I didn’t know her) did die at NIH of a Cushing’s-related problem. I’m so glad I didn’t find out until a couple months later!

November 3, 1987, the surgeon, Dr. Ed Oldfield, cut the gum above my front teeth under my upper lip so there is no scar. He used tiny tools and microscopes. My tumor was removed successfully. In some cases (not mine) the surgeon uses a plug of fat from the abdomen to help seal the cut. Afterwards, I was in intensive care overnight and went to a neurology ward for a few days until I could walk without being dizzy. I had some major headaches for a day or two but they gave me drugs (morphine) for those. Also, I had cotton plugs in my nostrils. It was a big day when they came out. I had diabetes insipidus (DI) for a little while, but that went away by itself – thank goodness!

I had to use a foam product called “Toothies” to brush my teeth without hitting the incision. Before they let me go home, I had to learn to give myself an injection in my thigh. They sent me home with a supply of injectable cortisone in case my level ever fell too low (it didn’t). I was weaned gradually off cortisone pills (scary). I now take no medications. I had to get a Medic Alert bracelet. I will always need to tell medical staff when I have any kind of procedure – the effects of my excess cortisone will remain forever.

I went back to the NIH for several follow-up visits of a week each where they did all the blood and urine testing again. After a few years NIH set me free. Now I go to my “outside” endocrinologist every year for the dexamethasone suppression test, 24-hour urine and regular blood testing.

Health discoveries come from research. Research starts with you.

Join the largest and most inclusive health research initiative of its kind. You could help researchers find answers to the most pressing health questions.

The All of Us WEAR Study has begun!
As a part of this optional study, you could receive a new Fitbit® to wear. All of Us will be able to get the data the Fitbit collects to help researchers understand how behavior impacts health.

Want to help others, too?  Sign up at https://go.joinallofus.org/

ℹ️ Basics: Cushing’s Syndrome Overview

Cushing’s syndrome is a rare disorder that occurs when the body is exposed to too much cortisol. Cortisol is produced by the body and is also used in corticosteroid drugs. Cushing’s syndrome can occur either because cortisol is being overproduced by the body or from the use of drugs that contain cortisol (like  prednisone ).

Cortisol is the body’s main stress hormone. Cortisol is secreted by the adrenal glands in response to the secretion of adrenocorticotropic hormone (ACTH) by the pituitary. One form of Cushing’s syndrome may be caused by an oversecretion of ACTH by the pituitary leading to an excess of cortisol.

Cortisol has several functions, including the regulation of inflammation and controlling how the body uses carbohydrates, fats, and proteins. Corticosteroids such as prednisone, which are often used to treat inflammatory conditions, mimic the effects of cortisol.

Stay tuned for more basic info…

❣️The Message Boards Turn 21 Today! ❣️

Today  is the birthday, or anniversary, of the Cushing’s Help message boards.  They were started September 30, 2000 (The rest of the site started earlier that year on July 21, 2000)

As of today, we have 73,130 members who have made countless posts.

Find the message boards here: http://cushings.invisionzone.com/