I know that some of my posts are too technical for some of my readers, so I’ll make amends right now. An important paper just appeared in the journal Neurology and Therapy called “The Lived Experiences of People with Progressive Supranuclear Palsy and Their Caregivers.” 

The nine authors were led by Dr. Gesine Respondek, a well-published PSP expert formerly at Hannover Medical School in Germany and now at Roche Pharmaceuticals.  The others are a diverse group from five different European medical institutions, two patient advocacy organizations and the study’s sponsor, the Belgian drug company UCB Biopharma.  They performed one-hour interviews of 21 patient/caregiver pairs, 7 patient organization representatives, 21 nurses and 42 neurologists in France, Germany, Italy, Japan, Spain, the UK and the US.  The patients and caregivers also completed smartphone-based, 7-day diaries with photos and formal daily questionnaires.  The analysis used a qualitative approach rather than attempting to fit the subjective information into a standard statistical model used in most medical research.

The study identified barriers to optimal care, the emotional responses to being a patient or a caregiver, and major “pain points.” The areas identified as important were:

• delays in seeking medical advice for the initial symptoms because of apathy or misattribution of the symptoms by the patient or family
• lack of awareness of PSP by non-neurologists
• delays in even the neurologist suspecting PSP because of delayed appearance of downgaze difficulty or other hallmarks
• a feeling of being overwhelmed by the diagnosis and its implications
• delays in being referred by the general neurologist to a movement disorders specialist
• diagnostic uncertainty even by the movement disorders specialist because of the overlaps between PSP and other candidate diagnoses
• absence of objective diagnostic tests
• a lack of empathy by the neurologist
• frustration in having to settle for symptomatic treatment rather than disease-modifying treatment
• the problem of being “no longer you”
• the loss of independence in daily activities
• lack of consistency in the rating and monitoring of symptoms
• lack of guidelines and quality care standards for PSP management
• stresses in confronting the end of life
• caregivers feeling frustrated, sad, lonely, guilty and unsupported

The most important stresses among these related to the delays in receiving a correct diagnosis.  The countries differed in some areas with Japan offering the best support, information and home care. 

The authors concluded with these recommendations:

• More countries should create patient organizations dedicated to PSP.
• Time allotted for consultations should be longer to allow the clinician to better educate the patient/caregiver.  If this is not possible, then providing formal follow-up time by phone or video would be a good substitute.
• To assist in the above, one of the shorter versions of the PSP Rating Scale should be widely adopted by neurologists in order to provide patients with an quantitative measure of their status within the time allotted at the visit.
• At-home follow-up by a nurse specialized in parkinsonism, when financially feasible, would help.
• Closer collaboration between patient organizations and clinicians should be facilitated.
• More information should be available on “financial support, life expectancy, nutrition and tube feeding, and preparation for end-of-life.”
• There should be better access to support for patient and caregiver in the form of adult day programs, support groups, respite care, home health care, social work, caregiver training and psychological support.
• Tele-health forms of occupational therapy should be available.
• Clinicians should be honest and open with the patient and caregiver about the unpleasant truths and the uncertainties.
• The needs of the caregiver should be as important as those of the patients to clinicians, support organizations, insurors and policymakers.

As my own editorial response, I’ll say that:

• Many of these recommendations are for services already available in the US via CurePSP and in the UK via the PSP Association.  But funding limitations at these charities limit their reach. 
• CurePSP and the PSPA already offer many forms of layperson and professional education where funding is not an issue. They just have to get it to the right people.
• Creating new or more educational materials for clinicians who can read English is not a priority.  We just need to grab their attention and convince them to devote some time and energy from their busy schedules to learning the material. Providing the materials in other languages would also help.
• CurePSP’s Centers of Care network, which is only just getting started in earnest, is attempting to address many of the deficiencies on the part of professional education and access to care.  The best example is its “Best Practices” paper advising on treatment options and published last year.
• I hope that there can be a radical change in how most physicians and insurers see PSP.  The current, “Oh, PSP is just a disease that old people get, and you’ve got to go sometime, and there’s nothing to be done.” has to change to, “PSP is a disease that reduces the quality and quantity of one’s retirement years and its sufferers and their families can benefit in many ways — both psychological and physical — from better access to care, faster diagnosis, and delivery of well-informed and empathetic symptomatic management.”