And It's About Time There Was Some Support For Cushing's!
I suspect that a little “Cushie Alien” began invading my body not too long after I married Charlie in July of 1992. At that time I weighed about 140 pounds and had just “graduated” from a size 10 to a size 12. Because I had just turned 40, I attributed my weight gain to the stresses of getting married, moving, my husband going back to school, and both of my parents getting cancer and eventually dying, not to mention my changing hormones and the normal aging process.
After two miscarriages, we were told that I had “old eggs,” so I half-heartedly gave up on my lifelong dream of having children of our own. Looking back, I now realize that Cushings may well have attacked my hormones enough to have interfered with my pregnancies. A few years later, still looking pregnant and praying that I could be, I was forced to have a hysterectomy due to fibroid tumors and we really had to give up the dream of having children of our own. At the time of my hysterectomy, I was also diagnosed with osteopenia. I later learned that osteoporosis and fibroids are often associated with Cushing’s.
Married life and multiple foot surgeries had changed my eating and exercise habits, and when the hot flashes came even harder I tied all these into a bundle as my reasons for continuing to gain weight, to the point of being over 200 pounds at a height of 5’6” on a relatively small frame. But when I really tried my best to lose the weight - hardly ate anything, exercised faithfully (hating every minute of being sweaty, red-faced and exhausted), my husband suggested that something must be wrong with my thyroid, so he suggested that I see an endocrinologist.
The process of my diagnosis of Cushing’s began when on 1/12/06 the endocrinologist noted my “buffalo hump” along with the centrally predominant weight gain and “padded” shoulders that are characteristic of Cushings. Screening studies showed an elevated AM serum cortisol level of 32.4 as well as an elevated ACTH of 39. A 24-hour urine for free cortisol (UFC) was also elevated at 194. The serum cortisol came down to 19.4 after I took 0.5 mg dexamethasone, not realizing that I was to take both tablets for the standard dexamethasone suppression test. Follow-up studies on 3/1/06 showed an AM serum cortisol minimally elevated at 23.9, but with ACTH elevated even more than the previous test, this time at 46. However, the afternoon serum cortisol level was 3.4, which puzzled my endocrinologist (he suspected a possible error at the lab, but since then I have learned that my Cushing’s is cyclic – with cortisol levels fluctuating from normal to four times the normal limit). On suppression with 2 mg of dexamethasone, an elevated UFC dropped from 224 to below detection, and a minimally elevated serum cortisol of 22.5 dropped to 1.6. During that time, ACTH dropped from 69 to 12.
These findings left questions regarding a diagnosis of mild Cushing's or functional hypercortisolemia. Therefore, on 3/28/06 an MRI of the pituitary was performed, which indicated a possible left pituitary micro adenoma, reportedly measuring 3mm. x 4mm. x 6mm. Because this could have been incidental, a nighttime salivary cortisol level was recommended and done at 11 PM on 5/22/06. The salivary cortisol level was elevated at 0.281 (The reference laboratory reports that patients with Cushing's Syndrome have concentrations greater than 0.112.). The recommendation of the endocrinologist was inferior petrosal sinus sampling (IPSS).
Not having anyone locally available to perform this delicate procedure, a second opinion was sought from another endocrinologist. Additional serum and urine cortisol levels were taken, all of which were elevated. Again, her recommendation was petrosal sinus sampling. I then consulted with a neurosurgeon, who agreed that the MRI was inconclusive, and when I asked about the IPSS, he suggested that an experienced doctor should perform this procedure, and he could not recommend anyone local.
The more I read about Cushings, the more I believe that my life – or at least the quality of my life - depends to a significant extent upon the doctor’s degree of experience with the disease and the intricacies of its diagnosis. I learned that my insurance (United Health Care) considers looking for a tumor a cancer intervention, and that if I travel to a center for excellence in cancer, my medical expenses will be covered without even a co-pay and even my travel, lodging and meals will be covered for myself and a travel companion if I have to travel more than 100 miles to the center. That was when we decided to go to the Cushings experts at Mass General.
I absolutely love my doctor at Mass General, Dr. Steven Russell. He is not only knowledgeable and professional but also thorough, conscientious and caring. In an eloquent letter to my local endocrinologist, he summarized the biochemical and imaging findings related to my diagnosis of ACTH dependent Cushings.
Early in 2006 I’d had three UFC’s that were elevated above the normal range. The first two of these, in February and March, were approximately fourfold elevated above the upper limit of normal. Associated with both of these were ACTH levels that were elevated. Also in March a 2-day dexamethasone suppression test observed the UFC falling from a baseline of 223 mcg/dL to an undetectable level, and an MRI of the pituitary revealed a hypo-enhancing lesion approximately 5 x 4 x 3 mm. A salivary cortisol in May was approximately 2.5 times the upper limit of normal at 0.281.
A CT of the adrenal glands in July 2006 was read as normal, but a later CT of the abdomen with contrast demonstrated a possible 1.3 cm low-density lesion in the left adrenal gland. A third UFC in July 2006 was only modestly elevated at 76 mcg/dL. In November 2006, my blood pressure was elevated at 165/90 and there was evidence of pitting edema of the extremities.
Dr. Russell believed that the suppression of my UFC level with dexamethasone is consistent with a pituitary source; but he agreed that localization should be confirmed with an IPSS, as the pituitary mass is small enough that it could be an incidental finding, and although unlikely, there is a possibility that the adrenal lesion could be secreting ACTH. In addition, given the suggestion of an adrenal nodule on the abdominal CT, Dr. Russell ordered a 24-hour urine for free metanephrines in order to rule out pheochromocytoma. Finally, and hopefully this is not the case, there is also the possibility of an ectopic tumor that suppresses with dexamethasone.
Since a UFC following my November 2006 visit with Dr. Russell was relatively low, he suggested that I may have cyclic Cushings, and ordered a two-week period of midnight salivary cortisol collections in order to establish my cycle so that we could schedule the IPSS at a time that my cortisol levels would most likely be high. If Cushing’s disease were to be diagnosed via the IPSS, Dr. Russell would schedule transsphenoidal surgery by Dr. Brooke Swearingen at Mass General. Following the transsphenoidal surgery and cure of the Cushing’s, if my hypertension persists, Dr. Russell recommends screening for hyperaldosteronism with a plasma aldosterone level to renin activity ratio.
February 6, 2007 - Dr. Russell received the results of my salivary cortisol tests through January 22nd so far: January 17th – 6.1; January 18th – 8.5; January 19th – 4.4; January 20th – 1.7; January 21st – 3.1; and January 22 – 5.2. He saw the same pattern in January that he saw in December in my salivary cortisol levels (peaks every 4-5 days), and based on these levels he anticipated that my cortisol would peak again on February 11th, the 16th and the 21st. Since the Inferior Petrosal Sinus Sampling (IPSS) must be done when cortisol levels are high, he checked with the surgeons that do the procedure at Mass General to see which date we could tentatively schedule it, assuming that the pattern continued to hold out.
Whatever day the procedure was scheduled for, we would fly to Boston two days before: the first day to pick up the jug, the second day to do the urine for free cortisol (UFC) test and the third day for the procedure, so that hopefully the fourth day we could fly home, if I was up to the travel. If the cortisol level wasn't high on the day scheduled for the procedure, we'd have to delay the procedure until the level is high - otherwise it's no good. So in addition to continuing to monitor my salivary cortisol levels to anticipate the peaks, the UFC would be the final confirmation of the cortisol level at the time of the procedure.
Because Dr. Russell projected that I was beginning a peak cortisol level, I brought my handy-dandy jug with me again to work for a second 24-hour urine for free metanephrines (UFM) collection. The first UFM done a few weeks prior was low, correlating with a low salivary cortisol level. So if the second one was low while my salivary cortisol level was high, he would be satisfied that the "guilty" tumor was not as likely to be in my adrenals as in my pituitary. This Cushings is SO COMPLICATED - but it is fascinating!
February 18, 2007 - We finally got the call from Dr. Russell at Mass General that we've been waiting for to "come on down" for the final diagnostic procedure that will hopefully determine the location of the tumor in my pituitary. My dear friend and prayer partner, Eileen Appleton, will be flying to Boston with me at the crack of dawn tomorrow, February 19th. Depending on my cortisol levels, which must be high in order for the procedure to be effective, I hope to have the inferior petrosal sinus sampling (IPSS) done on the 21st. If all goes well and I am up to traveling, we can fly home on the 22nd. If the cortisol level is not high on the 21st, we may have to wait a day or two until the level is high to do the IPSS. Following the procedure, assuming that the "guilty" tumor is identified as being in the pituitary, we simply go home and wait for Dr. Swearingen to schedule the transsphenoidal surgery to remove it.
February 23rd Praise Report - I just returned from Boston, having had the most wonderfully orchestrated care by the fabulous staff at Massachusetts General Hospital! My most sincere gratitude is extended to my attending physician, Dr. Russell, as the “Maestro,” (with the help of Dr. Leonard Tochka and the kind folks in the MGH Core lab) in obtaining lab results for salivary cortisol levels sent by air courier to Mayo Clinic for overnight analysis, in conjunction with the serum and UFC analyses done at MGH in preparation for and following my IPSS. I also thank Dr. Scott Broadwell and the staff in the MGH radiology department for the excellent care I received during the IPSS. I cannot praise them all enough for the way everyone worked together to obtain the most reliable results possible within a very tricky time period due to the cyclic nature of my Cushing’s.
Dr. Russell’s report stated, “IPSS shows clear centralization and lateralization to the left in the setting of an elevated UFC. MRI showed hypoenhancing lesion on the left of ~ 6 mm.” I could not ask for a more encouraging diagnosis!
I am also thankful for the opportunity to stay at MGH at the Inn, a special floor of the La Quinta Inn in Somerville, dedicated to meeting the special lodging needs of MGH patients and their families. The caring folks at this facility make every effort to serve their guests in ways that make them feel at home away from home, and I look forward to seeing them again when I return next month for my surgery.
I praise the Lord for the marvelous way that the entire process went so smoothly with the best possible results toward the cure of my Cushing’s. Not only were these results provided before I even left Boston, but I was also able to schedule my transsphenoidal surgery (removal of the tumor through the nose) to be done by Dr. Brooke Swearingen on March 21st. Again, I thank Dr. Russell, who worked diligently from home on my behalf in spite of being ill with the shaking chills. I just can’t thank him enough! I feel truly blessed to be under the expert care of Dr. Russell and the dear folks at MGH!
I look forward to the surgery on the 21st to once and for all “rip that little alien out of my nose” so that I can go back to being me again instead of this “Blimp with a Buffalo Hump” controlled by an alien invader in my pituitary! I have learned so much from the dear folks on this site. My prayers are with you, and I look forward to our continued sharing of support!
Praise the Lord! His mercies are new every morning, and He is SUCH an AWESOME GOD!
On Wednesday, March 21, 2007 Dr. Brooke Swearingen at Massachusetts General Hospital successfully removed the tumor from my pituitary that he feels was responsible for my Cushing's Disease, and I am now on the way to recovery! The second floor of the hotel we stayed in near Boston is devoted to Massachusetts General Hospital patients and their families, so our "mission field" was full of dear folks who needed our prayers, and thankfully my dear prayer partner, Eileen Appleton, and I were able to pray with and for many of them while we were there.
Unfortunately, before leaving for Boston, I slipped and dislocated my right shoulder, so I have been in a great deal of pain ever since, and now we will be involved in a law suit to hopefully see that no one else is similarly injured at that establishment. Thankfully, since the students at the New York State School for the Blind where I work will be on Spring Break until April 16th and all I have to do is office work until then, I was actually able to return to work on April 2nd, less than two weeks after the surgery! God is SO GOOD - ALL THE TIME!
Dr. Russell has been monitoring my sodium and cortisol levels, and since the cortisol levels have been either undetectable or very low, he is satisfied that the removal of the tumor is the cure for my Cushing’s. The first week after the surgery I was on dexamethasone, then prednisone, and then after my six-week follow-up Dr. Russell switched me to hydrocortisone (Cortef) as a temporary cortisol replacement. Since I am doing so well, we have gradually decreased my dosage, and I will be going back to see him on August 6th for my next (and hopefully last) follow-up visit, unless the adrenal tumor is found to be the cause of my hypertension.
By the end of May, I weaned myself completely off of the Cortef, and by the end of August I had lost 45 pounds from my pre-surgery weight – half way to my goal of getting back down to my pre-Cushing’s weight! A cortisol stimulation test, additional serum and UFCs confirmed that the Cushing’s, indeed, is cured. However, during my August 6th visit, since I had lost so much weight, Dr. Russell was able to detect a nodule in my thyroid that had been previously hidden by all the Cushie padding. Thankfully, a biopsy revealed that it was benign. The final step is to be certain that the adrenal tumor is not intermittently secreting anything, so one last round of late night salivary cortisol tests is in the works.
Unfortunately, ACL Labs, the laboratory that had done most of my previous 35 late night salivary cortisol tests, is not a network provider with my insurance company (I learned AFTER the labs had been done), and as a result I am now paying over $2,300 in “spit bills,” in spite of several appeals to my insurance carrier and even to the New York State Insurance Board. In order to find a network provider for this coming round of tests, I spent hours and hours over several months, including online searches as well as telephone discussions with my insurance carrier, various laboratories, and Dr. Russell and his staff. So few laboratories do this test that I literally had to ask (beg) my local network lab to add that test to their offered services. I consider this one small step toward greater Cushing’s Awareness!
Meanwhile, while I was in the process of researching laboratories that were not only in my insurance company’s network but also able to do late night salivary cortisol testing, Dr. Russell asked if I would give him permission to use my case in a presentation for Grand Rounds at Mass General. I told him that I would be happy to do anything I can to increase awareness of Cushing’s among endocrinologists and the general public. Let’s hope that with increased knowledge of the intricacies of this Syndrome, more and more lives can be saved, as mine has been!
Thanks again to each and every one of you who has prayed with and for us, and for all of your cards, letters, words of encouragement and acts of service on behalf of Pastor Charlie and me! God bless you all!
- Judi Piscitello
Jesus is Life! My life is in His hands!
"The best and most beautiful things cannot be seen or touched - they must be felt with the heart." - Helen Keller
- Happy moments, praise God!
- Difficult moments, seek God!
- Quiet moments, worship God!
- Painful moments, trust God!
- Every moment, thank God!
