Treatment
You've come a long way...it has probably been months since you found a reliable, knowledgeable treating endocrinologist who began the diagnostic process for Cushing's. When you received your diagnosis...when the tumor was visible on MRI or CT, you probably jumped for joy! How strange, that you would be happy the doctors found a tumor? Well, it's not so much that you're happy you have a tumor in your body; it's that there is an answer to all these bizarre, unexplained changes that have been happening to you for quite some time. You're not a crazy hypochondriac after all! (And like I told my husband after my MRI confirmed that I had a pituitary tumor, "See?? It was all in my head!!")
Once reality sets in, it is time to figure out the best method of treatment for your type of tumor...and that can be a scary situation. In cases where an MRI came back normal but IPSS reveals abnormal cells or a tumor too small for surgery, some treating doctors will offer you a choice of observing the tumor and controlling Cushing's with medication. Another alternative would be radiation therapy. Generally, the go-to method for treating these tumors is surgical removal.
If you have been diagnosed with Cushing's Syndrome and have an adrenal or ectopic (pancreas, lung, thyroid) tumor, a relevant form of surgery will be presented to you by your surgeon. A lot of times, just the tumor itself can be removed. In advanced or malignant (adenocarcinoma) cases, it is possible that the entire organ or gland on which the tumor resides will be compromised as well. Some people have a small resection of the organ or gland; others have the organ or gland removed in its entirety, along with the tumor (for example: adrenalectomy). In cases of malignancy, an appropriate continuing treatment (such as chemotherapy, radiation, or medication) may be recommended. (Please feel free to research treatment options for Cushing's Syndrome tumors - remember, this site is tailored to Cushing's Disease, which is strictly pituitary related.)
Once reality sets in, it is time to figure out the best method of treatment for your type of tumor...and that can be a scary situation. In cases where an MRI came back normal but IPSS reveals abnormal cells or a tumor too small for surgery, some treating doctors will offer you a choice of observing the tumor and controlling Cushing's with medication. Another alternative would be radiation therapy. Generally, the go-to method for treating these tumors is surgical removal.
If you have been diagnosed with Cushing's Syndrome and have an adrenal or ectopic (pancreas, lung, thyroid) tumor, a relevant form of surgery will be presented to you by your surgeon. A lot of times, just the tumor itself can be removed. In advanced or malignant (adenocarcinoma) cases, it is possible that the entire organ or gland on which the tumor resides will be compromised as well. Some people have a small resection of the organ or gland; others have the organ or gland removed in its entirety, along with the tumor (for example: adrenalectomy). In cases of malignancy, an appropriate continuing treatment (such as chemotherapy, radiation, or medication) may be recommended. (Please feel free to research treatment options for Cushing's Syndrome tumors - remember, this site is tailored to Cushing's Disease, which is strictly pituitary related.)
Transsphenoidal Surgery
artwork by Jenifer E. Williams
While it doesn't scream "brain surgery," transsphenoidal surgery (TSS) is exactly that! If you remember from "The Master Gland" page, the pituitary gland (which is where the Cushing's Disease tumor will be located) sits at the base of the brain, "between the eyes and behind the nasal bones." Transsphenoidal surgery, while a delicate procedure, is the least invasive form of brain surgery today. Most pituitary adenomas associated with Cushing's Disease are located on the anterior lobe of the gland. (My tumor was in fact located on the gland's anterior lobe, at a downward angle, but was also resting against the right optic nerve, as confirmed by a neuro-ophthamologist. I could actually feel that! What a strange sensation! Had the tumor been compressing the optic nerve, I could have had any number of vision problems.)
After your brain MRI confirms a tumor on your pituitary gland, your treating doctor will refer you to a neurosurgeon and an otolaryngologist (ENT doctor). Together, these doctors will work together to perform your TSS. In some cases, you will be referred to a neurosurgeon only, as many of these doctors have their own ENT fellows that work alongside them in the operating room. You will meet with this elite surgical team before your procedure to go over the particulars of the surgery (risks, side effects, insurance coverage, duration of your hospital stay, current and post-op medications, recovery, and any alternative methods that may be available).
You will also have to undergo several pre-operative tests, required by your medical care team, before clearance is given for you to have the surgery. These tests will include a CBC, additional MRI or CT scan, EKG, and any other specialist consults depending on your specific health conditions (ex: stress test as ordered by your cardiologist, diabetic counseling as ordered by your endocrinologist, etc.). You will also be evaluated by the performing anesthesiologist, and you may be offered psychological counseling. (Let's face it - Cushing's is not at all kind to our psyche, and we could all use a little help in this department on some of our worst days. It is also helpful to find Cushing's support groups, either locally or online. While friends and family can be wonderful "therapists," it helps to talk to people who can relate to your feelings because of their personal experience with this disease.)
You will be given a list of instructions to prepare you for surgery. These instructions will tell you how to prepare for the procedure, including when to stop taking certain medications and when to stop eating and drinking prior to the surgery.
For the surgery, a heparin lock will be inserted into a vein in one of your arms. Through it, you will be given general anesthesia, meaning you will be unconscious throughout the procedure, and you will have no memory of the surgery.
(The operating room sure can be intimidating, full of equipment and lights and lots of people, but one of the coolest things I remember before "drifting off to sleep" was looking at all of the monitors and seeing my name on the screens. These people - doctors, nurses, and techs - were all there just for me, to save me from Cushing's Disease. That was my operating room, my medical care team, my time, my release from this debilitating disease.)
All surgeons have different protocols, but I'm going to give you my surgeons' scenario, and I'll explain it in the easiest way possible:
Once I was unconscious and an endotracheal tube was inserted for respiratory purposes, the procedure was first handled by my ENT doctor. Using an endoscope and other intricate devices, he made his way through my nasal passage and approached the sphenoid bone at the back of my nose. He broke the bone and then perforated through the sphenoid sinus. The bone at the posterior of the sinus is the bottom of the sella turcica, which houses the pituitary gland. My ENT doctor carefully interrogated this bone as well, leaving it open for my neurosurgeon to do his magic - the pituitary resection. (Need a recap on where everything's at? Go back to "The Master Gland" page.)
Removing pituitary tumors is not a "get-in-and-get-out" kind of performance when you take into account that your surgeon is working in an area not much bigger than a centimeter or two. Add in the lingering fact that your optic nerves and internal carotid arteries run through this same space, and you can image how steady-handed these medical professionals have to be! My neurosurgeon had the tedious responsibility of removing my tumor - piece by piece, glob by glob - until it was gone. A small amount of healthy tissue was taken as well to confirm that there were no microscopic abnormal cells hanging around in my pituitary gland.
Once my neurosurgeon was done, it was up to my ENT doctor to "patch me up." The sella turcica and sphenoid bone were fitted back into place and secured with hemostatic agents and "glue" (to control bleeding and hold everything in place, respectively). Surgical packing was inserted up through my nasal passage and anchored with a string that was taped to the bridge of my nose.
If a pituitary resection is successful, the lab work over the next several hours will be all the verification you need. It will make your surgeon very happy if your cortisol levels plummet to <1, and you (as the patient) feel not so great. Not that your surgeon wants to see you sick! See,when cortisol levels plummet to <1, this means that the pituitary adenoma was removed completely, but now your gland is essentially too weak to carry on its normal duty of stimulating the adrenal glands to produce that lovely steroid hormone (cortisol). Signs of too little cortisol in the body mimic flu-type symptoms, hence the not feeling so great. Again, these are just normal signs that the tumor was completely removed with no indication of abnormal cells lingering about. Surgical success!
After the surgery, you will be taken to a neuro-ICU or step-down unit where you can recover under the watchful eyes of neuro-ICU nurses. You most likely will not rest or sleep much, as the nurses need to monitor your post-op progress very carefully. Every hour or so, you will be asked to repeat the same tasks over and over again, which involve answering simple questions (what's your name? who is the president? what procedure did you have done?) and performing simple motor skills (touch your nose; raise your legs; follow the nurse's finger with your eyes only). At some point within the next 24 hours post-op, you will have to undergo a brain MRI. This can be quite uncomfortable, especially given the fact that you may be nauseous, weak, and possibly in pain. The MRI techs will do their best to make you comfortable and get the scan over with as quickly as possible.
You will be able to resume normal eating and have bathroom privileges as soon as you are coherent and have been evaluated by your treating surgeons and doctors. After about 3-4 days in the hospital, if you are doing well with no adverse side effects or problems, you will be discharged to go home. In addition to any pain medication and antibiotics your surgeon or treating doctor may have prescribed, you will be given a medication regimen for cortisol replacement (hydrocortisone/Cortef, prednisone, etc.) which you would have started within the first 24 hours after your surgery. The reason you will need cortisol replacement is because your pituitary gland is weak, or "asleep" as most doctors term it. You will start on a high dose of replacement meds and take them in the morning and in the early afternoon. You will gradually taper off these drugs until your pituitary gland has "woken up" and can secrete appropriate amounts of ACTH to stimulate the adrenal glands to produce normal levels of cortisol.
After your brain MRI confirms a tumor on your pituitary gland, your treating doctor will refer you to a neurosurgeon and an otolaryngologist (ENT doctor). Together, these doctors will work together to perform your TSS. In some cases, you will be referred to a neurosurgeon only, as many of these doctors have their own ENT fellows that work alongside them in the operating room. You will meet with this elite surgical team before your procedure to go over the particulars of the surgery (risks, side effects, insurance coverage, duration of your hospital stay, current and post-op medications, recovery, and any alternative methods that may be available).
You will also have to undergo several pre-operative tests, required by your medical care team, before clearance is given for you to have the surgery. These tests will include a CBC, additional MRI or CT scan, EKG, and any other specialist consults depending on your specific health conditions (ex: stress test as ordered by your cardiologist, diabetic counseling as ordered by your endocrinologist, etc.). You will also be evaluated by the performing anesthesiologist, and you may be offered psychological counseling. (Let's face it - Cushing's is not at all kind to our psyche, and we could all use a little help in this department on some of our worst days. It is also helpful to find Cushing's support groups, either locally or online. While friends and family can be wonderful "therapists," it helps to talk to people who can relate to your feelings because of their personal experience with this disease.)
You will be given a list of instructions to prepare you for surgery. These instructions will tell you how to prepare for the procedure, including when to stop taking certain medications and when to stop eating and drinking prior to the surgery.
For the surgery, a heparin lock will be inserted into a vein in one of your arms. Through it, you will be given general anesthesia, meaning you will be unconscious throughout the procedure, and you will have no memory of the surgery.
(The operating room sure can be intimidating, full of equipment and lights and lots of people, but one of the coolest things I remember before "drifting off to sleep" was looking at all of the monitors and seeing my name on the screens. These people - doctors, nurses, and techs - were all there just for me, to save me from Cushing's Disease. That was my operating room, my medical care team, my time, my release from this debilitating disease.)
All surgeons have different protocols, but I'm going to give you my surgeons' scenario, and I'll explain it in the easiest way possible:
Once I was unconscious and an endotracheal tube was inserted for respiratory purposes, the procedure was first handled by my ENT doctor. Using an endoscope and other intricate devices, he made his way through my nasal passage and approached the sphenoid bone at the back of my nose. He broke the bone and then perforated through the sphenoid sinus. The bone at the posterior of the sinus is the bottom of the sella turcica, which houses the pituitary gland. My ENT doctor carefully interrogated this bone as well, leaving it open for my neurosurgeon to do his magic - the pituitary resection. (Need a recap on where everything's at? Go back to "The Master Gland" page.)
Removing pituitary tumors is not a "get-in-and-get-out" kind of performance when you take into account that your surgeon is working in an area not much bigger than a centimeter or two. Add in the lingering fact that your optic nerves and internal carotid arteries run through this same space, and you can image how steady-handed these medical professionals have to be! My neurosurgeon had the tedious responsibility of removing my tumor - piece by piece, glob by glob - until it was gone. A small amount of healthy tissue was taken as well to confirm that there were no microscopic abnormal cells hanging around in my pituitary gland.
Once my neurosurgeon was done, it was up to my ENT doctor to "patch me up." The sella turcica and sphenoid bone were fitted back into place and secured with hemostatic agents and "glue" (to control bleeding and hold everything in place, respectively). Surgical packing was inserted up through my nasal passage and anchored with a string that was taped to the bridge of my nose.
If a pituitary resection is successful, the lab work over the next several hours will be all the verification you need. It will make your surgeon very happy if your cortisol levels plummet to <1, and you (as the patient) feel not so great. Not that your surgeon wants to see you sick! See,when cortisol levels plummet to <1, this means that the pituitary adenoma was removed completely, but now your gland is essentially too weak to carry on its normal duty of stimulating the adrenal glands to produce that lovely steroid hormone (cortisol). Signs of too little cortisol in the body mimic flu-type symptoms, hence the not feeling so great. Again, these are just normal signs that the tumor was completely removed with no indication of abnormal cells lingering about. Surgical success!
After the surgery, you will be taken to a neuro-ICU or step-down unit where you can recover under the watchful eyes of neuro-ICU nurses. You most likely will not rest or sleep much, as the nurses need to monitor your post-op progress very carefully. Every hour or so, you will be asked to repeat the same tasks over and over again, which involve answering simple questions (what's your name? who is the president? what procedure did you have done?) and performing simple motor skills (touch your nose; raise your legs; follow the nurse's finger with your eyes only). At some point within the next 24 hours post-op, you will have to undergo a brain MRI. This can be quite uncomfortable, especially given the fact that you may be nauseous, weak, and possibly in pain. The MRI techs will do their best to make you comfortable and get the scan over with as quickly as possible.
You will be able to resume normal eating and have bathroom privileges as soon as you are coherent and have been evaluated by your treating surgeons and doctors. After about 3-4 days in the hospital, if you are doing well with no adverse side effects or problems, you will be discharged to go home. In addition to any pain medication and antibiotics your surgeon or treating doctor may have prescribed, you will be given a medication regimen for cortisol replacement (hydrocortisone/Cortef, prednisone, etc.) which you would have started within the first 24 hours after your surgery. The reason you will need cortisol replacement is because your pituitary gland is weak, or "asleep" as most doctors term it. You will start on a high dose of replacement meds and take them in the morning and in the early afternoon. You will gradually taper off these drugs until your pituitary gland has "woken up" and can secrete appropriate amounts of ACTH to stimulate the adrenal glands to produce normal levels of cortisol.
Recovery
Now that your tumor is gone, you are considered "in remission" with Cushing's. The tumor removal is not a 100% cure. Your symptoms and health issues will gradually go away or lessen over time. Some people may experience a complete cure from certain ailments, while others still have to deal with their detriments. Once you have had Cushing's Syndrome or Cushing's Disease, of course, you are at risk for recurrence. After 15-20 years with no recurrence of a tumor, you can consider yourself cured. However, the prognosis for Cushing's Syndrome or Cushing's Disease remission without a recurrence is good, especially with the life-long follow up treatment from your medical care team.
For up to 6 weeks after your TSS (transsphenoidal surgery), you will not be able to blow your nose, cough, strain on the commode, drink through a straw, bend over, pick up anything over ten pounds, or operate a vehicle. If you have to sneeze, you will need to "mouth-sneeze" - open your mouth and let it all out that way, as gently as possible! Anything that creates negative pressure in your head can have serious complications on the surgical site and land you back in the operating room. Fluid intake must be kept to a minimum during the first week to two weeks post-op. People that have Cushing's Disease and undergo transsphenoidal surgery are at risk for developing diabetes insipidus, which is a sudden onset (yet less severe form) of diabetes. It is characterized by extreme thirst and the excretion of large amounts of urine. If this happens to you after surgery and you have not been drinking fluids to excess, then you need to notify your surgeons and treating doctor immediately. Diabetes insipidus is the result of your pituitary gland not adequately producing the hormone, ADH, which helps the kidneys balance fluid within the body. Drinking limited fluids can help you and your medical care team quickly identify this problem, should it arise.
As I mentioned, you will have a regimen for cortisol replacement after your surgery because of the lack of ACTH secretion from your pituitary gland, which stimulates cortisol production from the adrenal glands. This is what is known as secondary adrenal insufficiency. (Not to be confused with primary adrenal insufficiency, or Addison's Disease.) Adrenal insufficiency can be a life-threatening situation if your body does not have enough cortisol to sustain a cold, infection, or extreme stress.
Without the vast amounts of cortisol racing through your body like before your surgery, you will feel different. It probably won't be the good kind of different you have been looking forward to. Depending on how long you have had Cushing's Disease, the tapering process and the recovery period may not be as smooth and quick as you anticipated.
As you taper off your cortisol replacement meds, you will go through periods of "withdrawal." (If you think about it, your body has been addicted to cortisol for quite some time. Going with lesser quantities in your system is, essentially, withdrawal.) You will experience flu-type symptoms, aggravation, mood swings, joint pain, fatigue, muscle tenderness/weakness, and just an overall sense of not feeling well. Some patients have stated that the recovery from Cushing's is worse than the disease itself. Just like the Cushing's symptoms and their level of severity, Cushing's recovery is different for every person - some slow, some average, some much better than others. It is never a good idea to rush through the tapers - all you are doing is setting yourself up for pain and misery and a very hard time trying to cope.
Set realistic goals. If you have been suffering from Cushing's Disease for a long time, plan on your full recovery taking at least a year or two. That seems like an awfully long time, but look at it as a year or two that you may not have had if your Cushing's Disease was mis- or undiagnosed.
Cushing's Disease is a fatal endocrine disease if not properly treated!!!. Not a lot of people (or doctors) realize that. Many people are told by their physicians that there is nothing wrong with them - that they are just fat and unhealthy; that they need to diet and exercise more. (Usually, nothing is farther from the truth!! I have always eaten well and exercised often. Instead of losing weight, I continued to pack it on without a valid explanation.) In fact, many people become candidates for bariatric surgery and go through with the recommendation. I was one of those "candidates." Deep down, I knew something was really wrong; something that bariatric surgery couldn't fix. Thankfully I chose to keep searching for the root of my problem...I never imagined that root would spring up Cushing's Disease.
There is so much more to my story, and I take it day by day. I want to help others through my experience, and I sure hope that I have done so for just 1 person through this website. For additional help and information, click on the contact button below, and please be sure to email me with feedback about this website.
Take care, and God bless!
For up to 6 weeks after your TSS (transsphenoidal surgery), you will not be able to blow your nose, cough, strain on the commode, drink through a straw, bend over, pick up anything over ten pounds, or operate a vehicle. If you have to sneeze, you will need to "mouth-sneeze" - open your mouth and let it all out that way, as gently as possible! Anything that creates negative pressure in your head can have serious complications on the surgical site and land you back in the operating room. Fluid intake must be kept to a minimum during the first week to two weeks post-op. People that have Cushing's Disease and undergo transsphenoidal surgery are at risk for developing diabetes insipidus, which is a sudden onset (yet less severe form) of diabetes. It is characterized by extreme thirst and the excretion of large amounts of urine. If this happens to you after surgery and you have not been drinking fluids to excess, then you need to notify your surgeons and treating doctor immediately. Diabetes insipidus is the result of your pituitary gland not adequately producing the hormone, ADH, which helps the kidneys balance fluid within the body. Drinking limited fluids can help you and your medical care team quickly identify this problem, should it arise.
As I mentioned, you will have a regimen for cortisol replacement after your surgery because of the lack of ACTH secretion from your pituitary gland, which stimulates cortisol production from the adrenal glands. This is what is known as secondary adrenal insufficiency. (Not to be confused with primary adrenal insufficiency, or Addison's Disease.) Adrenal insufficiency can be a life-threatening situation if your body does not have enough cortisol to sustain a cold, infection, or extreme stress.
Without the vast amounts of cortisol racing through your body like before your surgery, you will feel different. It probably won't be the good kind of different you have been looking forward to. Depending on how long you have had Cushing's Disease, the tapering process and the recovery period may not be as smooth and quick as you anticipated.
As you taper off your cortisol replacement meds, you will go through periods of "withdrawal." (If you think about it, your body has been addicted to cortisol for quite some time. Going with lesser quantities in your system is, essentially, withdrawal.) You will experience flu-type symptoms, aggravation, mood swings, joint pain, fatigue, muscle tenderness/weakness, and just an overall sense of not feeling well. Some patients have stated that the recovery from Cushing's is worse than the disease itself. Just like the Cushing's symptoms and their level of severity, Cushing's recovery is different for every person - some slow, some average, some much better than others. It is never a good idea to rush through the tapers - all you are doing is setting yourself up for pain and misery and a very hard time trying to cope.
Set realistic goals. If you have been suffering from Cushing's Disease for a long time, plan on your full recovery taking at least a year or two. That seems like an awfully long time, but look at it as a year or two that you may not have had if your Cushing's Disease was mis- or undiagnosed.
Cushing's Disease is a fatal endocrine disease if not properly treated!!!. Not a lot of people (or doctors) realize that. Many people are told by their physicians that there is nothing wrong with them - that they are just fat and unhealthy; that they need to diet and exercise more. (Usually, nothing is farther from the truth!! I have always eaten well and exercised often. Instead of losing weight, I continued to pack it on without a valid explanation.) In fact, many people become candidates for bariatric surgery and go through with the recommendation. I was one of those "candidates." Deep down, I knew something was really wrong; something that bariatric surgery couldn't fix. Thankfully I chose to keep searching for the root of my problem...I never imagined that root would spring up Cushing's Disease.
There is so much more to my story, and I take it day by day. I want to help others through my experience, and I sure hope that I have done so for just 1 person through this website. For additional help and information, click on the contact button below, and please be sure to email me with feedback about this website.
Take care, and God bless!
