SCOPPE Survey

What we did:

• Survey of 270 pheo para patients on our online Scan of Pheo Para Patient Experiences (SCOPPE), conducted in mid 2022.
• This patient survey received clearance from an Research Ethics Board
• Responses were collected for six weeks.
• Respondents were recruited through social media, website and email channels

What we found:

• Pheo Para patients frequently reported delays in diagnosis (median or “middle” time was 29 months) and almost half (49%) saw four or more health care professionals before they received a diagnosis of pheo or para.
• Misdiagnoses were similarly common, with 49% experiencing one or more initially incorrect diagnosis, such as menopause, thyroid disorders, and even hypochondria.

In the words of some of our survey participants:

When I found out I had a glomus jugular tumor I didn’t know who to talk to. Nobody had heard about this type of tumor, not even my doctors. I felt so alone.

It’s been very challenging, advocating for myself and pushing doctors and medical team to order the proper tests.

Misdiagnosed too many times, which caused the tumors to grow too large.  Too many surgeries and too much radiation in one area.  I am limited to treatments now due to being misdiagnosed twice.

Where do we go from here?

• Broaden patient access to experienced and expert health care professionals, through expansion of the Pheo Para Alliance Centre of Excellence program
• Deepen knowledge of pheos and paras within the medical community, through presentations at professional conferences and explore increasing pheo para content in medical education

What we found:

• The most commonly experienced issues were lack of access to an experienced pheo para medical team (28.9% of participants), lack of communication among specialists (28.9%), and lack of access to information about their specific medical condition (25.6%).
• All of the issues were rated as important by a large majority of our participants.
• Of particular importance were lack of access to an experienced pheo para team, diagnostic tests and treatments, and regular monitoring; delays in receiving treatment and specialists’ opinions; and lack of communication between specialists. These issues were rated by more than 80% of the participants as “very” or “extremely’ important.
• It also is interesting that participants were relatively consistent in their ratings, as shown by the way that scores clustered together closely.

In the words of some of our survey participants:

Confusion and overwhelmed from different specialists not explaining corresponding treatments and their relation to each other.

Had blood tests and told I didn’t need to be monitored anymore because results weren’t elevated, even though I still have some symptoms.

It’s so rare, no one believes it, so I had to do all my own research and fight to get tests.

Felt like I have fallen between the cracks.  I have to fight for all of this.  There is a lot of trouble with communication and it is so frustrating as it is hard to have any kind of life while going through this, as well as being your own case manager.

Where do we go from here?

• Important issues for pheo para patients were delays, lack of access to experienced and expert medical teams, appropriate treatments and appropriate monitoring protocols, as well as communication difficulties among specialists indicates a priority issue for PPA. Our Pheo Para Alliance Centre of Excellence program, which identifies medical facilities with research and clinical expertise in pheo para worldwide, was designed to address these difficulties.  In the coming year, we will continue to expand this program and increase patient awareness of its benefits.
• A majority of participants had difficulty with access to relevant information and ranked this as an important or very important issue. We will seek to expand patient and caregiver knowledge of existing educational resources already available of the PPA website and other sources, as well as determining additional educational priorities (e.g., increased accessibility for language and special needs) and distribution processes.
• We also are exploring ways to increase knowledge about pheo para among general practitioners, emergency room doctors, and other health care providers. For example, we are increasing our presence at professional conferences to present our survey results and engage attendees.

What we found:

• Respondents to the survey were mostly female, US residents, white, and well-educated, with a median age of 52 years
• We hoped to analyze demographic responses to identify unmet needs of patient’s with different backgrounds, characteristics or experiences.  The respondents were not diverse enough to to this analysis.

Where do we go from here?

• We would like to broaden the representation of the responses with future surveys.
• Identify strategies for structural change to PPA to ensure patients are represented equitably & resources are accessible.
• Our IDEA (Inclusion, Diversity, Equity & Accessibility) committee, recently launched in late 2022 can help guide us in this area.

What we found:

• Pheo para patients are finding helpful support from a wide range of sources. All of the potential sources of support listed above received ratings of “helpful”, “very helpful”, or “extremely helpful”).
• Although help category ratings were close to each other, the highest helpfulness ratings were given to family members and significant others. These two sources of support were rated as “very” or “extremely” helpful by 61% and 64% of participants, respectively.
• An important source of support came from other patients, likely provided by peer-led programs and support groups. The Pheo Para Alliance and Facebook support groups were specifically mentioned as very helpful supports.
• We noted that participants found webinars, as well as research and online journals, to be very helpful, and rated them similarly to several more interpersonal support opportunities.
• Some other sources of helpful support reported by participants included local researchers, pathologists, and physiotherapists.

In the words of some of our participants:

Mostly online pheo para patients. My other friends do not understand my disease, journey.

Love this support group!!! Thank you.

Regional get togethers of my own friends with ppgl (pheo para)

Very little help available across medical or home help

Where do we go from here?

• It was encouraging that most of our participants had one or more sources of significant support. However, there may also be a sizeable number of participants who did not find any of the suggested sources to be helpful. The PPA will continue to attempt to identify participants seeking support and develop resources to address their support needs.
• It is important to ensure that both interpersonal and online support for patients with pheo para are well resourced, given the effectiveness of these sources of help.
• Given the role of family in supporting those with pheo para, educational materials designed to be accessible to family members should be expanded.
• The Pheo Para Alliance offers monthly peer support zoom calls and individual peer support contact. Where possible, these activities might be increased to include regional in-person opportunities for patients to connect.
• If you are interested in participating in a peer support activity, please contact us at info@pheopara.org.

What we found:

• With respect to assistance in managing their pheo para, participants identified all six areas of potential assistance, on average, as between “very” and “extremely helpful”. These sources included more available treatments, better access to specialized treatment centers and experts, more information about – and access to – pheo para clinical trials, more knowledgeable healthcare professionals, better coordination among their pheo para medical team, and more educational information about their illness from their medical team.
• Better access to experts and more knowledgeable health care experts were identified as particularly important for managing pheo paras, with 91.8% and 88.8% of patients rating them as “very” or “extremely helpful” respectively.

Where do we go from here?

• Patients with pheo para clearly expressed the need to have better informed, expert health care workers. Our PPA Centers of Excellence were designed to put patients in contact with experienced pheo para clinicians, who could provide coordinated multidisciplinary care.  Although the number of Centers is increasing, many patients are located a considerable distance from a Centre.  We will be widening our network of Centers and also look for ways to establish effective long distance medical care for pheo paras.

What we found and some implications:

• A large majority of patient participants (73.5%) prefer to make decisions about the management of their pheo para in partnership with their medical team. This partnership approach was in comparison to having their team guide them to make their own decisions or making their own decisions with some input from their medical team. Such partnerships require informed patients and caregivers, as well as health care professionals who are knowledgeable about pheo para and willing to be responsive to patient need for information, priorities, and concerns.
• The majority of participants rated the overall quality of healthcare provided to pheo para patients as very good (29.0%) or excellent (28.0%). However, 10.6% rated the overall care as poor and another 15.5% rated it as only fair.  It is important to learn more about the reasons for these relatively low ratings and explore potential remedies.
• We also asked participants to indicate how much their pheo para presented a financial difficulty for them, on a 4-point scale ranging from “no current out-of-pocket expenses” to “severe difficulty”. On average, participants rated their health expenses as presenting between “very little” and “moderate difficulty”. For approximately a quarter of our participants (26.1%), government health care or insurance covered all of their pheo para expenses.  A similar percentage (25.5%) reported moderate financial difficulty.  For 13.5% of participants, however, pheo paras led to a severe financial difficulty.  The most expensive aspects of the participants’ pheo para condition were health insurance, diagnosis, and treatment costs. Given that 39% of our participants reported at least moderate financial difficulty related to pheo para, it’s important that patients be aware of any available funding, as well as receiving assistance in completing insurance and other forms that may result in financial aid.
• We asked our participants if they were aware of a variety of potential treatments for pheo paras. Treatments included PRRT Therapy (e.g., Lutathera), surgery, MIBG therapy (e.g., Azedra), somatostatin analogues (e.g., octreotide), chemotherapy, radiofrequency, external beam ablation, and clinical trials with another type of therapy.  Our patient participants were fairly well informed about potential treatments for pheo paras, even if they were not available where they lived. This finding is encouraging because an informed patient is more likely to receive appropriate care and makes patient advocacy more effective.

• • Pheo Para Alliance Patient Centered Research on Challenges for those with Pheochromocytoma and Paraganglioma, NANETS Multidisciplinary NET Medical Symposium, 2022
  • The Pheo Para Superheroes Have Spoken – Listen Up!  The SCOPPE Survey.

• Patient centered research & management with Pheo Para Alliance to improve patient care (oral presentation). International Symposium on Pheochromocytoma, 2022