About 20 years ago, Linda Hidahl’s life took a bewildering turn. The Denver native was in her mid-20s and struggling through a painful divorce when her body threw her another curveball. Hidahl began unexpectedly gaining weight that she couldn’t shed. Eventually she added 90 pounds to a slight frame, ballooning to 210 pounds.
“It didn’t matter how much I dieted and worked out,” she said. “I could sniff lemon juice and lettuce and gain 10 pounds.”
The woman who had run, hiked, biked and kick-boxed became more sedentary. That wasn’t only because of her weight. A host of other problems plagued her, including rolls of fat on her neck, abnormal hair growth, stretch marks and bouts of vertigo. Her health suffered with high blood pressure and Type 2 diabetes that required thrice-daily insulin tablets.
She also developed osteoporosis (porous and brittle bones) that caused frequent fractures, including nearly two dozen rib breaks. In 2010, Hidahl fell down the stairs at her job as an X-ray technician with a Thornton clinic. The spill broke her hip, and she lost her job and her health insurance.
Hidahl felt increasingly isolated and depressed. She didn’t date for years after her divorce. She recalls the frustration of people telling her that she’d gained weight because she was lazy and that she lied about her dieting attempts.
“Being in physical pain for so long really jacks with your brain,” Hidahl said. “I was always tired. I never felt good. It gets to where you want to do nothing more than go to sleep at night and never wake up again.”
Pea-sized problem: the pituitary gland
The culprit in all this misery was Hidahl’s pituitary gland, an organ about the size of a pea that sits at the base of the brain. Its tiny size belies its power. The pituitary plays a key role in producing hormones that in turn trigger the activities of other glands that regulate vital bodily functions, including growth, blood pressure, blood sugar, metabolism, reproduction and more.
When the pituitary gland is functioning properly, it helps the body maintain a healthy balance of hormones. Sometimes, though, the pituitary goes haywire and causes unpredictable health issues. It was one of these pituitary glitches that scrambled Linda Hidahl’s life.
She had Cushing’s disease, the result of a pituitary tumor that upsets the body’s hormone balance. The problem works like this. The pituitary gland naturally secretes ACTH (adrenocorticotropic hormone). This hormone tells the adrenal glands, which sit atop each of the kidneys, to produce cortisol, a steroid that regulates blood sugar, blood pressure, response to stress and other functions.
In Cushing’s disease, however, a pituitary tumor causes the gland to overproduce ACTH, which in turn triggers the adrenal glands to generate too much cortisol. The results are what Linda Hidahl saw in the mirror and felt in her bones. Silently, Cushing’s disease inflicted the Type 2 diabetes and high blood pressure that threatened her cardiovascular health and her life.
Care for Cushing’s disease
Hidahl, however, was fortunate to receive care for Cushing’s disease from the multidisciplinary Pituitary Program team at the University of Colorado (CU) School of Medicine on the Anschutz Medical Campus. Dr. Kevin Lillehei, chair of the Department of Neurosurgery at CU and a member of the team, performed microsurgery to remove a pituitary tumor only a few millimeters in size in September 2014.
Hidahl’s recovery took time, but in the months after the surgery, 90 pounds melted away from Hidahl’s frame. She got her Type 2 diabetes and hypertension under control and three years ago, returned to work in a new job as a hair stylist. She is still severely slowed by osteoporosis, but is back to hiking and savors a new-found sense of confidence the disease and its symptoms took from her for many years.
Lillehei, who has performed more than 2,000 pituitary surgeries, helping to make CU’s Pituitary Program a national leader, said he’s seen many patients who get care for Cushing’s disease go through similar transformations.
“It’s incredibly rewarding,” he said, looking at a series of photos of Hidahl before surgery, immediately after, and months later that showed the dramatic changes in her appearance.
Help for Crushing’s disease only one part
In fact, helping people with Cushing’s disease is just one part of the Pituitary Program, which diagnoses and treats a variety of conditions with many different symptoms in a team-based clinic at UCHealth University of Colorado Hospital. A multidisciplinary team that includes specialists in endocrinology, neurosurgery, neuroradiology, neuropathology and radiation oncology holds monthly conferences to discuss all pituitary cases and design a course of treatment best suited to each patient. A patient coordinator, Angela Grant, helps individuals navigate the details of their treatment plan.
The work always begins with analyzing the hormone production from different glands, said Dr. Margaret Wierman, an endocrinologist and professor with the CU School of Medicine. She heads the Pituitary, Adrenal and Neuroendocrine Tumor Program at CU.
“We’re the hormone detectives, and we find out if people have signs and symptoms of either hormone overproduction or a lack of hormones,” Wierman said.
Cushing’s disease offers a window into the collaborative work the Pituitary Program provides. An MRI alone won’t result in neurosurgery. Lillehei said that about half the time MRIs are normal, even if there is a tumor. In 10% of cases, Wierman added, an MRI shows an abnormality in a healthy person.
Imaging is an important part of a Cushing’s disease diagnosis, but the centerpiece is an extensive workup that includes a 24-hour urine test to confirm the patient’s body has too much cortisol. The labs also include sampling blood and saliva for cortisol and another that measures the response of the adrenal gland to a drug used to suppress cortisol levels.
If the tests confirm high cortisol levels, the challenge is to find the source of the overproduction. Some people have the symptoms that Cushing’s disease patients have – collectively called Cushing’s syndrome – but produce too much cortisol because of a problem elsewhere in the body, such as the adrenal glands or possibly even the lungs or pancreas. In those cases, Wierman and her team pursue a different solution.
To confirm that the pituitary is overproducing ACTH and increasing cortisol levels, the team performs an outpatient test called inferior petrosal sinus sampling. With the help of an interventional radiologist, providers thread a catheter through the patient’s femoral vein in the groin, up to the base of the skull and the inferior petrosal veins, which drain the pituitary gland. They draw ACTH levels from both the left and right veins and from a source outside the area. Measuring ACTH levels on both sides helps to predict where the tumor may be, Lillehei said.
Providers draw two different samples, looking for ACTH levels in the inferior petrosal veins that exceed the levels in the outside area by specific ratios. If the levels point to a tumor, the next step is surgery to find it and take it out.
That’s Lillehei’s job, but he emphasizes that neurosurgery, when it is appropriate, is the end result of the hard work that informs it.
“It’s critically important that people are [carefully] selected and worked up,” he said. “If you bring someone to surgery who doesn’t need surgery, you’re not going to have a good result no matter what you do,” Lillehei said.
Probing the pituitary
He removes pituitary tumors like Hidahl’s by entering the nasal cavity with equipment that includes either a microscope or endoscope for imaging and tiny surgical instruments. He uses microtools to remove enough of the sphenoid bone, which lies behind the nose and between the eyes, to open the skull base and get to the pituitary gland. Since most of the tumors are very small, some only about 3 millimeters, the incision must be wide enough that he can see the entire pituitary gland and find the tumor.
If he doesn’t know the exact location of the tumor, Lillehei works first around the outside surface of the gland, then gradually makes deeper incisions. If he’s unsure that tissue he removed contains the tumor, he sends frozen sections of tissue to Dr. BK Kleinschmidt-DeMasters, a neuropathologist with the Pituitary Program team, who analyzes the tissue for signs of tumor cells.
“In 20 minutes [Dr. DeMasters] can tell you if you missed it and you need to look somewhere else,” Lillehei said, adding that she is among the nation’s leading neuropathologists.
Once he finds the tumor, Lillehei removes it, along with a small margin of extra tissue to eradicate stray tumor cells. If a few stay behind, the disease can recur, which Wierman said happens in about one case in five. After surgery, she said, it’s important that patients see their endocrinologist yearly and then intermittently for the rest of their lives.
After the fall
Linda Hidahl’s path to recovery was tough at first. After breaking her hip, she had another fall that tore a four-inch-square gash in her leg. A wound specialist debrided the wound repeatedly but without much success. When he expressed frustration with the wound’s resistance to healing, Hidahl mentioned that she had years before received a diagnosis of Cushing’s disease that went untreated because of a combination of problems, including losing her insurance. With that, the physician determinedly set out to find her an endocrinologist. He succeeded, and Hidahl eventually saw Lillehei, who bluntly told her that without surgery she likely wouldn’t survive more than six months longer.
He didn’t need to do much to convince her. She was ready for a resolution to the problem, whatever that might be.
“At the point that [the surgery] happened, I’d been sick so long and in so much pain that I honestly didn’t care,” she said. “I didn’t care if I woke up from the surgery.”
She did wake up, of course, and began a recovery that had its own challenges. A bout of pneumonia prolonged her hospital stay, and her rapid weight loss, while obviously welcome, came at the price of feeling sick and unable to keep food down. She initially found it hard to find a new, more hopeful path after so many years of battling weight and other Cushing’s-caused problems.
“I didn’t see a different person when I looked in the mirror,” Hidahl said, adding she struggled with fatigue for six months after surgery.
Steroid balancing act
That’s not unusual, said Wierman, noting that the pituitary gland has to “wake up” after the surgery. During this time, it no longer produces ACTH, ironically requiring patients to take steroids so the body that had suffered with too much cortisol now gets enough of it.
Patients gradually taper off the steroids – prednisone or hydrocortisone — as the pituitary awakens, a process that can be challenging.
“The body has been addicted to high cortisol levels,” Wierman said. It took Hidahl about two years to wean off of the steroids completely.
A new look after getting care for Cushing’s disease
Linda Hidahl is grateful for the care she received from all her providers at UCHealth, even as she knows that she lost much to Cushing’s disease. While she relishes having energy and feeling good enough to hike, the osteoporosis that Cushing’s disease caused will never allow her to get back to the level of activity she once enjoyed, nor can she work any longer as an X-ray technician because of it. But she has a new life, having remarried in July 2017 after breaking from her shell of isolation and introducing herself to her husband-to-be. She doesn’t reflect on what Cushing’s disease took from her so much as what it taught her.
When Hidahl stands behind her chair, cutting and styling hair, she sometimes finds herself giving therapy to people suffering from frustrating and poorly understood conditions, like fibromyalgia. She recalls her own dark times in the grip of a mysterious disease.
“I feel like it made me a much more empathetic person to all kinds of disabilities – things you can see and things that you don’t see,” Hidahl said. “A lot of people don’t understand that just because you can’t see it that there is not pain.”