Five years ago, Barbara Hope-Gaiti found herself in a battle with a disease that hides in plain sight.
Hope-Gaiti, now 71, didn’t worry too much about the red, rough patches of skin when they first appeared. She didn’t like their looks and they irritated her skin, but they weren’t painful. But as time went on, the mysterious condition claimed more bodily real estate, eventually covering swaths of her torso, breasts, stomach, thighs and back with small bumps.
“You could not see the skin,” Hope-Gaiti said.
She visited dermatologists in Boulder and in her native New York City, going through upwards of 40 skin biopsies and blood tests without getting an answer for the ailment.
Nonetheless, the condition didn’t stop Hope from living a full life. It didn’t sap her energy. She and husband Donald continued their work with White Knight Productions, a company they formed 13 years ago to serve not-for-profit organizations. Hope-Gaiti also had others to worry about. Her younger brother was battling stage 4 glioblastoma, an aggressive brain cancer that ultimately took his life. During the same period, her older brother would also fall ill and quickly pass away.
The emotional ordeal led Hope-Gaiti to suspect that her skin problems were stress-related. But they persisted after her brothers died. She finally determined she had to find an answer for it.
“I got to the point that I decided I have to take care of me now,” Hope-Gaiti said.
Getting below the surface
Hope-Gaiti’s search led her early in 2017 to the University of Colorado Cancer Center, where she finally found her answer: a rare cancer called cutaneous T cell lymphoma (CTCL). The diagnosis resulted from collaboration between oncologist Dr. Bradley Haverkos, and dermatologist Dr. Theresa Pacheco, and their care team.
Just a month before Hope-Gaiti’s arrival, Haverkos and Pacheco had launched the Multidisciplinary Cutaneous Lymphoma Clinic, the only one of its kind in the Rocky Mountain region, joining forces with other providers to assess, diagnose and develop coordinated plans of care for patients with a disease that often baffles even highly skilled skin disease and cancer specialists.
Cutaneous lymphoma is a type of non-Hodgkin’s lymphoma: It attacks the white blood cells – T, B and NK, or natural killer, cells – that are the foot soldiers of the body’s immune system. Its distinguishing feature is that the assault arises in and on the skin. Roughly three-quarters of the cases are CTCLs, which produce red skin, scaling, tissue thickness and often intense itching. It is therefore easily mistaken for eczema or dermatitis by dermatologists who are unfamiliar with its appearance, Pacheco said.
That Hope-Gaiti went four years without a diagnosis isn’t unusual, Haverkos said.
“The median time from presenting with a rash to diagnosis is six years,” he said. “The slow-growing nature of this cancer means that it often has a bearing on quality of life. The biggest thing that we see is lots of anxiety that builds in patients because they feel the condition is undertreated and that their providers aren’t listening to them.”
In addition to getting a diagnosis and treatment – more on that later – Hope-Gaiti can count herself fortunate in her bout with CTCL: She didn’t have any itchiness.
“Many patients have such severe itchiness that it can literally drive them crazy,” said Haverkos, noting there are cases of individuals so tormented that they take their own lives.
That extreme discomfort and the risk of cutaneous lymphomas progressing from the skin to other parts of the body highlights the importance of early detection, Haverkos said. As in Hope-Gaiti’s case with CTCL, rough patches of skin can progress to plaques, defined by the small bumps that are actually tumors. If the disease invades other organs, it is harder to treat and outcomes deteriorate. The disease lays siege to the immune system, leaving patients vulnerable to deadly infections, he noted.
“Without good treatment, people do die from the disease, especially when there is blood and organ involvement,” Haverkos said.
The only “cure” for cutaneous lymphoma is a bone marrow transplant, Haverkos said, but a variety of treatments can do a good job of controlling it. Some are “skin-directed” options like UV light therapy, topical medications, such as steroids, and radiation. In other cases, providers turn to “systemic” therapies. Hope-Gaiti, for example, initially underwent a procedure called photopheresis, which involved drawing her blood, separating the white blood cells, mixing them with a substance that sensitizes them to UVA light, and reinfusing them into her body. The sensitized cells stimulate the immune system to fight the rogue T cells.
Another weapon is drugs, including a group that targets and blocks cellular proteins implicated in lymphomas. Haverkos enrolled Hope-Gaiti in a trial of an oral medication, Tenalisib, that for now has cleared her skin of the plaques that had covered them. The drug, developed by Rhizen Pharmaceuticals, treats a specific type of CTCL and is aimed at patients for whom previous treatments have failed or those who have relapsed. Tenalisib received “Fast Track” designation from the FDA in April, meaning that the clinical review period is six months rather than the usual 10.
Haverkos, who has presented findings from the clinical trial of the Rhizen therapy, offered a note of caution about Tenalisib and other systemic treatments. “None of the drugs as yet are homeruns,” Haverkos said. “It’s trial and error and they may take a couple of months to work.”
The complexity of the disease demands the coordinated response of a multidisciplinary clinic, but laying the groundwork for it at UCHealth required months of preparation. Both Haverkos and Pacheco have long recognized the need. He completed a three-year fellowship in hematology and oncology with the Ohio State University Hospital Program, during which he co-authored a paper detailing the importance of multidisciplinary care in treating patients with cutaneous lymphomas.
Pacheco began her career at the Cancer Center in 2000 working with oncologists and cutaneous lymphoma specialists John Aeling and Paul Bunn, both of whom she says were important mentors and supporters as she built her expertise in diagnosing and treating the disease. Aeling eventually retired, and over the years Pacheco steadily built her patient caseload. But without a dedicated multidisciplinary clinic, she frequently had to consult with Bunn – whom she described as a strong and unselfish mentor – on the fly.
After Haverkos arrived from Ohio State in 2015, Pacheco says she began meeting with him monthly, and their conversations eventually led to ironing out the details for a new clinic. The two also made their case to Clay Smith, MD, associate chief of the Division of Hematology at the University of Colorado School of Medicine and director of the UCHealth Blood Disorders and Cellular Therapies Center, and David Norris, MD, chair of the University of Colorado School of Medicine’s Department of Dermatology, and got approval from both to move forward, Pacheco added.
In addition to Haverkos and Pacheco, the team includes a nurse navigator to handle all the preparation work for patients – obtaining medical records, scheduling biopsies and lab work, resolving prior authorization and insurance questions, and so on – before they meet with Haverkos and Pacheco in the clinic. Skilled nurses in the Dermatology and Cutaneous Oncology clinics work closely with patients to answer questions, provide education, and generally direct them through what can be a maze of care. A dedicated pathologist, clinical pharmacist, radiation oncologist and wound care specialists provide key clinical support.
Building that team required a great deal of heavy lifting, much of it taken on by a pair of nurses, Karee Hutches, with the Bone Marrow Transplant Clinic on the oncology side and Cristine Gentert, in the Dermatology Clinic. Hutches worked on the project as part of earning her Level 3 credential for UEXCEL, UCHealth University of Colorado Hospital’s professional development ladder for nurses. Gentert, who is now a nurse navigator in the Transplant and Hepatology clinics at UCH, said she was frequently assigned to work with Pacheco and was “honored” to be part of the plan to streamline care to patients with cutaneous lymphoma.
Hutches said planning for the new clinic began in October 2016, three months before its official opening in January 2017. An early decision was for providers to see patients in the Dermatology Clinic, because of its procedural room space for biopsies. Just as Haverkos and Pacheco looked to Ohio State, Stanford University and Memorial Sloan Kettering as models for a cutaneous lymphoma clinic, Hutches and Gentert drew on the experience of other multidisciplinary clinics at UCH – Gastrointestinal and Breast Cancer, for example – for guidance on key operational details like scheduling. One important decision: Haverkos and Pacheco would see patients together whenever possible.
Ironing out the many operational details was not an end in itself but a means to improving patient care, Hutches emphasized. “We wanted to increase access to care for patients with a rare disease and make their time with us a good experience,” she said.
An efficient operation means that a patient who needs radiation treatment, for example, can often get it on the same day from radiation oncologist Rachel Rabinovitch, MD.
“We have her on speed dial,” Hutches said. Likewise, a certified wound care nurse is generally readily available for treatment without having to schedule a separate appointment.
In addition, Hutches and Gentert – and now Dermatology nurse Jamie Vang – worked closely together to serve as a “liaison between the patient and physician,” as Hutches put it. They provide education for patients, go over their after-visit summaries, answer questions about their prognoses, and so on. Early on, Hutches and Gentert got phones dedicated to communications about cutaneous lymphoma patients.
“We were often the initial point of contact for patients,” Gentert said. For patients who often had gone five or more years without a diagnosis, it was important that she and Hutches “explain the process and build rapport with them.” That work, including pointing patients to useful websites like that of the Cutaneous Lymphoma Foundation, encouraged them to ask Haverkos and Pacheco and other providers “questions relevant to their condition,” Gentert said.
“We’re still working on patient education from a nursing perspective,” Hutches added. “I’ve made a specific effort to increase my own education and learn from my dermatology colleagues, such as how to treat a lesion when it pops up and how medications can irritate the skin. We share knowledge.”
The work of Gentert and Hutches “completely changed the care we provide in a better way,” Pacheco said. “It’s faster, more coordinated and more efficient. We’ve relied on a string of very smart nurses.”
The effort has paid off. Haverkos said the clinic, which is open one half-day a week, routinely sees two or three new patients each session and about a dozen total cases throughout a typical afternoon. That’s well above the numbers seen before the clinic opened.
The collaborative spirit at the heart of the clinic paid off for Barbara Hope-Gaiti. After receiving a referral to the UCHealth Dermatology Clinic from her Boulder dermatology specialists late in 2016, she initially saw Norris, who ordered another biopsy and blood tests that were inconclusive. Norris recommended an appointment in the then-new Multidisciplinary Lymphoma Clinic. Haverkos and Pacheco concluded that Hope-Gaiti needed additional blood tests. She was startled when she learned that meant drawing 13 vials of blood to be sent for exhaustive testing.
Daunting as that sounded, Hope-Gaiti was ready to get on with it.
“I felt like I was in the right place at the right time,” she said. “I found both Dr. Haverkos and Dr. Pacheco incredibly communicative, and I needed their focus and direction. It was time for me to know what was going on.”
When the tests confirmed the CTCL diagnosis, Hope-Gaiti began the photopheresis, which required treatment on consecutive days every other week. Admittedly leery of needles, she’d been relieved with how smoothly drawing 13 vials of blood had gone on her initial visit. The photopheresis treatments meant regular vein punctures that again produced little discomfort – important for a patient dealing with a mysterious disease with an uncertain prognosis.
The photopheresis produced some relief, reducing the rash by about a third without any side effects, Hope-Gaiti said. In November 2017, Haverkos and Pacheco asked her to consider enrolling in the Tenalisib trial, not an easy decision as it required not only a PET scan but also a bone marrow sample to ensure the cancer hadn’t spread beyond the skin. Hope-Gaiti agreed, and November 27 became a red-letter day: It was her 71st birthday and she began the study.
She started on a regimen of four pills a day with regular blood tests and anti-viral medications to protect her suppressed immune system from infection. Today Hope-Gaiti takes two pills with the anti-virals, gets blood tests every two weeks and sees Haverkos and Pacheco regularly in the clinic. She does so gladly. Her skin remains clear. She now awaits the FDA’s decision on approving Tenalisib.
“It’s given me a lot of hope that other [treatments] will be found as well,” Hope-Gaiti said.
In the meantime, she continues to work out and savor each day of her life. “That’s one of the largest lessons I’ve learned,” she said. “I’m incredibly grateful to Dr. Haverkos and Dr. Pacheco and the entire unit.”
Hope-Gaiti wants her experience to have broader impact. Earlier this year, she attended a conference on leukemia and lymphoma and another on CTCL, where she met with other patients dealing with the disease. She also wrote to dermatologists who had treated her to inform them of her diagnosis.
“I want more dermatologists to know about it,” she said, adding that she strongly believes many people have undiagnosed CTCL. “I want to be more of a voice because I don’t feel CTCL has a voice.”