There are various methods of treatment for endogenous ("in the body") Cushing's Syndrome/Disease, but the primary method is always surgery to remove the cause: the tumor tissue.
Ectopic disease can originate anywhere in the body, but it is generally found in the lung tissue. The method of treatment and the surgery performed varies by location. This type is frequently cancerous, but is the rarest of the rare, and very few (VERY FEW) have this form of Cushing's.
With adrenal disease, the surgery will consist of an experienced surgeon removing one or both adrenal glands (unilateral- or bilateral- adrenalectomy, respectively). This is generally done laparoscopically through the abdomen, as the adrenals are located atop each kidney. Adrenal Cushing's Syndrome is the second most common form of endogenous Cushing's, but it is a very late second (which will become clear below).
In Cushing's Disease, which is pituitary in origin, surgery to remove the tumor is preformed by endo-nasal or transphenoidal surgery (meaning, they use small instruments and go through your nose and sinuses to access the pituitary at the base of the brain). This is the most common form of Cushing's, with something like 80-90% of endogenous disease being pituitary in origin.
Surgery recovery and remission/cure will be addressed in another post.
When surgery isn't effective in bringing lasting remission (or fails), or when surgery isn't an option for whatever reason, other methods of treatment can be explored.
One such option is medical therapy, wherein the patient takes medications to lessen or, hopefully, stop the effects of Cushing's Disease/Syndrome. There are a few medications on the market, but they all have some pretty nasty side-effects, some can cause permanent damage to organs, are generally costly (anywhere from $200/month up to $15,000/month), and are not a permanent option, though they can buy you some time and help reverse some effects of this illness to make you a better candidate for surgery.
A second non-surgical option is radiation. There are different types used, but the basic gist is that certain parameters must be met to be a candidate, and while this form of treatment can bring a permanent cure, it too comes with risks. Frequently the radiation damages the organ (such as pituitary) along with the tumor cells, which may lessen or stop production of certain essential hormones, requiring replacement through medication. Also, there is generally a long wait for the treatment to take full effect, anywhere from 1-5 years, during which time the patient usually is on medical therapy.
I would also like to address failed surgery or recurrence of Cushing's Disease (pituitary). When Cushing's Disease is back after a first surgery (or second, etc), generally the endocrinologist and neurosurgeon look for a "target" on MRI. If a target (suspected tumor) is found, the most likely treatment is generally repeat pituitary surgery, though in some cases medication is used for a while or radiation is tried. There are increased risks to second (and later) pituitary surgeries, and the statistical cure rate also drops, so sometimes medication or radiation therapy are preferred instead.
If a target is not found, the patient can agree to "exploratory" surgery hoping one is found, partial pituitary removal, or complete pituitary removal. These can be reasonable options, and each person and medical team must weigh the benefits and risks for each individual case and circumstance. But frequently when a surgical target is not found, the patient is either put on medication, sent for radiation therapy, or (if it is a slow-growing tumor, hyperplasia, etc) the patient may undergo Bilateral Adrenalectomy.
Bilateral Adrenalectomy (BLA) as a course of action following failed surgery or recurrent disease is becoming more common. Cushing's is a very drastic disease that has an average mortality of 5 years untreated...the patients will continue to get sicker and sicker, and eventually die from it's effects. Many are already quite ill at the time of their first diagnosis. Sometimes drastic disease requires drastic actions. Removing the adrenal glands will not stop the signal from the tumor tissue calling for the production of more cortisol, but it will stop the reception of that signal (ie, there are no longer adrenal glands to receive the ACTH signal and produce excess cortisol on-demand). This will leave the patient dependent on replacement hormones for life (a state closely related to Addison's Disease) as cortisol is a necessary hormone, but it is in essence trading an untreatable disease for a treatable one. Many BLA-ers as we call them, after adjusting to their new routines and allowing their body time to heal, actually begin to see marked improvement in their quality of life and symptomology. It is still a delicate balance, as their body cannot produce two necessary hormones, and comes with many risks, but with careful planning, education of the patient (family, friends and medical personnel too), and quality medical care, a long, active life is definitely possible.
Personally, I am still awaiting further testing to confirm that I am not in remission, and a pituitary MRI and follow-up with my neurosurgeon in about 3 weeks will tell us whether a second pituitary surgery or BLA will be the next step for me (if I am indeed not in remission). While I would prefer another pituitary surgery that led to remission/cure without pituitary damage, the risks of pituitary damage from repeat surgery (along with CSF leaks, etc) are very likely. So it IS likely that hormone replacement post-op will be necessary, and the pituitary makes a large number of necessary hormones. I would prefer to replace two hormones from adrenalectomy over 6+ hormones from pituitary damage. The balancing required can be quite confusing, medications costly, etc. While the threat of adrenal crisis is real, if a BLA is in my future, we have decided as a couple that even if I were to die in 5 or 10 years from such an event, we would prefer I have 5-10 good years where I could be actively involved and enjoying family life (with BLA) than to spend 5-10 years becoming bed-bound, increasingly ill, miserable, and dying from this disease. It is a tough decision, and even though I haven't hit that stage yet, I am sometimes hit with a very real fear of what such a decision would mean for the long-term. Would it be worth it to be "healthy?" Absolutely.
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