Why Patient Engagement is Important

Patients are the ones who the therapy is made for, so it’s important to get their input about what therapeutic benefits actually matter to them. Patients can help researchers better understand the challenges of these therapies and clinical care for their condition. Patients can also help improve study design and educational materials to make clinical trials more effective and help other patients with the condition better understand the risks and benefits of gene therapy.

Learn more about patient engagement efforts at the Foundation for the National Institutes of Health (FNIH) here: Patient Voices | FNIH

How Patients Contribute to Research

Patients can contribute to many aspects of research, including:

  • Helping researchers gain a new patient-centered perspective (1)
  • Improving the relevance of research to patient priorities (2)
  • Defining research questions or aims, selecting comparators, setting inclusion or exclusion criteria, and establishing study outcomes (3)
  • Tailoring interventions for specific populations or settings (3)
  • Improving patient educational materials and informed consent documents (2)
  • Anticipating barriers to recruitment, enrollment, and retention and helping provide solutions (3)
  • Providing input into when and how data is collected from study participants (3)
  • Improving dissemination and/or implementation of research findings (2)
  1. Partnering patients, caregivers, and basic scientists: An engagement model that fosters patient- and family-centered research culture
  2. Patient engagement: What partnering with patient in research is all about
  3. Patient Engagement In Research: Early Findings From The Patient-Centered Outcomes Research Institute

Challenges to Consider with Patient Engagement

There are some challenges to consider with patient engagement, including:

  • Additional costs to pay the patients or caregivers participating
  • Time issues for both patients and researchers
  • Patients feeling like tokens rather than valued team members or participants
  • Patient values leading to divergence from the research agenda
  • Concerns that patient contribution creates unfeasible study designs.
  • Concerns about equity and true representation as patients who engage with research teams may not be representative of the patient population (in terms of education level, SES, etc.)
  • Lack of clarity for resolving disputes between patients and researchers

Patient engagement: What partnering with patient in research is all about – ScienceDirect

Calculating Patient Engagement Costs

It is important to plan for patient engagement costs. Whether that is setting aside budget or calculating the financial value to show the funding party, here are a few things to consider:

Calculating ENPV

ENPV (expected net present value) can be used to predict how patient engagement can financially impact the project. Information about calculating ENPV can be found here.

Ways Patients Can Financially Impact Projects

Patients can offer insights and suggestions to avoid protocol amendments, improve enrollment, increase adherence to protocol, and improve retention rates, which can decrease costs and help streamline projects to next trial phases.

Costs Associated with Patient Engagement

Costs can differ depending on the project, the way researchers engage with patients, the stage of research, and the frequency in which they work with patients. Costs can include preliminary research, time spent educating or creating educational materials for patients, facilitator or facility fees, travel and lodging, compensatory fees for time, and time cost for researchers to work with patients instead of working on research.

Assessing the Financial Value of Patient Engagement: A Quantitative Approach from CTTI’s Patient Groups and Clinical Trials Project

How to Involve Patients and Caregivers in Research

Conditions Studied in SCGE Phase 2

What is Amyotrophic Lateral Sclerosis (ALS)?

ALS is a progressive neurodegenerative disease that affects nerve cells in the brain and spinal cord. A small portion of cases are genetic.

Relevant SCGE Project:

Advocacy Groups

 

What is Angelman Syndrome?

Angelman Syndrome is a neurogenetic disorder that causes delayed development, problems with speech and balance, intellectual disability, and, sometimes, seizures.

Relevant SCGE Project:

Advocacy Groups

Patient and Caregiver Perspectives

Parents’ Perspectives on Genetic Therapies for AS

  • 42 parents of children with AS were compared with 27 parents of a mixed etiology of other rare genetic disorders to their answers for an online survey about their perceptions of, and priorities for, genetic therapy
  • 95% of parents of children with AS said treatments that reduce symptoms associated with the condition were positive
  • 95% of parents of children with AS felt that genetic treatment trials to find a cure should be a priority for AS
  • When parents of children with AS would be willing to participate in gene therapy trials:
    • 48% would participate if the treatment had been trialed in mice but not humans
    • 26% would participate if the treatment has been trialed in humans but not trialed in those with AS
    • 19% would participate if the treatment has been trialed in humans with AS but was still in trial stages
    • 5% said they would wait until treatment was licensed
    • 2% would not want to consider genetic treatment
  • AS parent priorities for the focus of clinical trials were:
    • neurology/seizures
    • communication skills
    • motor skills/mobility
  • Parents specifically mentioned they did NOT want the trials to change their child’s happiness and personality

Parent perceptions, beliefs, and fears around genetic treatments and cures for children with Angelman syndrome – Adams – 2020 – American Journal of Medical Genetics Part A – Wiley Online Library

What is Autosomal Dominant Deafness?

Autosomal Dominant Deafness is a type of genetic hearing loss where the mutated gene is inherited from one of the parents.

Relevant SCGE Project:

Advocacy Groups

 

What is Best Disease?

Best Disease is a genetic disease that causes the macula, which is the central part of the retina, to degrade, causing problems with central vision.

Relevant SCGE Project:

Advocacy Groups

What is Friedreich’s Ataxia?

Friedreich’s Ataxia (FA) is a genetic disease that causes progressive damage to the nervous system and movement problems.

Relevant SCGE Project:

Advocacy Groups

Patient and Caregiver Perspectives

Perspectives of the Friedreich’s Ataxia Community on Gene Therapy Clinical Trials 

  • 137 records analyzed, participants recruited via email from the Friedreich’s Ataxia Global Patient Registry (FAGPR)
  • Patients care about quality of life and neurologic symptoms, such as coordination and walking
  • Researchers found that while a majority of the sample reported feeling knowledgeable about gene therapy prior to the survey, with an increase in knowledge after reading the educational materials provided from researchers,  the respondents ambivalence about immunomodulation and the vector dose seemed to indicate a lack of understanding about these topics. Patients’ idea of being knowledgeable about gene therapy may differ from researchers’ idea of knowledgeable. Or people may overestimate their knowledge.

Perspectives of the Friedreich’s Ataxia Community on Gene Therapy Clinical Trials – ScienceDirect

What is H1-4 Syndrome?

H1-4 Syndrome, sometimes called HIST1H1E syndrome, is a rage genetic condition characterized by intellectual disability and a distinctive facial gestalt.

Relevant SCGE Project:

Advocacy Groups

What is Hereditary Tyrosinemia Type 1 (HT1)?

Hereditary Tyrosinemia Type 1 is a genetic disorder characterized by elevated blood levels of the amino acid tyrosine (a building block of most proteins), that is caused by a shortage of an enzyme that is required for the process of breaking down tyrosine.

Relevant SCGE Project:

Advocacy Groups

What is Huntington’s Disease (HD)?

Huntington’s Disease is a genetic disorder that causes nerve cells in parts of the brain, including the part that controls voluntary movement, to gradually break down and die.

Relevant SCGE Projects:

Advocacy Groups

 

What is Leber Congenital Amaurosis (LCA)?

Leber Congenital Amaurosis is a genetic eye disorder that can include symptoms of blindness at birth, strabismus, nystagmus, photophobia, cataracts, and keratoconus.

Relevant SCGE Project:

Advocacy Groups

What is Mucopolysaccharidosis Type 1 (MPSI)?

Mucopolysaccharidosis Type 1 is a genetic condition characterized by an abnormal build-up of various toxic materials in the body’s cells because the body is not producing enough of an enzyme that breaks down the materials.

Relevant SCGE Project:

Advocacy Groups

What are Organic Acidemias?

Organic Acidemias are a class of inborn errors of metabolism characterized by the accumulation of abnormal organic acid metabolites and increased excretion of organic acids in urine. 

Relevant SCGE Project:

Advocacy Groups

What is Phenylketonuria (PKU)?

Phenylketonuria is a genetic disorder that causes an amino acid called phenylalanine to build up in the body because the body doesn’t produce enough (or any) of an enzyme needed to break down phenylalanine.

Relevant SCGE Projects:

Advocacy Groups

Patient and Caregiver Perspectives

Gene therapy for PKU -Perspectives of patients, caregivers, and advocates

  • Qualitative survey of adult patients, caregivers, and advocates from US, Ireland, Germany, and Spain on impact of living with PKU and perceptions of gene therapy
    • 33 people total: 24 patients, 5 caregivers, 4 advocates
  • Interviews were over the telephone with standardized discussion guide and lasted up to 60 minutes
  • Interviewers classified each participant by their level of knowledge regarding gene therapy as either: low (little or no prior awareness); moderate (awareness of gene therapy as a concept in PKU); or high (working knowledge of gene therapy, e.g., vectors).
    • 10/33 had high knowledge
    • 18/33 had moderate knowledge
    • 5/33 had low knowledge
  • Participants’ experiences of living with PKU were mostly negative, with mentions of frustrations with current management of symptoms
  • There was a mix of positive and negative perceptions of gene therapy
  • 7/10 participants with high knowledge of gene therapy reported high levels of concern about gene therapy
  • For people who had negative perceptions of gene therapies, there was a reluctance to be an early adopter of gene therapy because of the uncertainty of risks and potential side effects
  • Researchers suggest that there is a need for education about the risks and benefits of gene therapy

Starting the conversation on gene therapy for phenylketonuria: Current perspectives of patients, caregivers, and advocates – PMC (nih.gov)

What is Prion Disease?

Prion Disease is a group of fatal neurodegenerative diseases caused by misfolded proteins and known by various names including as Creutzfeldt-Jakob disease, fatal familial insomnia, and Gerstmann-Sträussler-Scheinker disease.

Relevant SCGE Project:

Advocacy Groups

What is Rett Syndrome?

Rett Syndrome is a genetic neurological disorder that leads to severe impairments, in particular impacting speech, purposeful hand use, and coordination.

Relevant SCGE Project:

Advocacy Groups

What is Sickle Cell Disease (SCD)?

Sickle Cell Disease is a group of genetic red blood cell disorders that affect hemoglobin, the protein that carries oxygen through the body. Instead of flexible, disc-shaped red blood cells, the red blood cells become crescent (“sickle”) shaped and do not bend or move easily, and can block blood flow to the rest of the body.

Relevant SCGE Project:

Advocacy Groups

Patient and Caregiver Perspectives

SCD Impact on Daily Life and Treatment Goals (1)

  • 2145 patients from 16 countries
  • SCD impact on daily life
    • 62% avoid intense physical activity
    • 41% are impacted in family and social life activities
    • 38% say it impacts household activities
    • 60% say SCD has high impact on emotional wellbeing
    • 66% of patients agreed or strongly agreed that their treatment effectively managed their symptoms
  • Treatment goals:
    • improve quality of life
    • prevent worsening of SCD
    • reduce pain crises
    • improve symptoms
    • improve long-term survival

According to Patient-centered outcome research on clinical trial considerations for SCD, investigators often overlooked:(2)

  • patient reported outcomes
  • treatment satisfaction
  • caregiver/parent burden
  • work time off
  • transportation costs
  • out-of-pocket costs

When Involving Patients in Clinical Trials or Therapies, Educational Materials should:(3)

  • address lack of trust in healthcare system
  • consider cultural beliefs
  • address fears related to potential side effects
  • use visual illustrations to help people learn about and evaluate their interest in gene therapies

1.American Journal of Hematology | Blood Research Journal | Wiley Online Library

2. Clinical trial considerations in sickle cell disease: patient-reported outcomes, data elements, and the stakeholder engagement framework – PMC (nih.gov)

3. Patient Perspectives on Gene Transfer Therapy for Sickle Cell Disease – PMC (nih.gov)

What is Spinal Muscular Atrophy (SMA)?

Spinal Muscular Atrophy is a genetic motor neuron disease that affects the central nervous system, peripheral nervous system, and voluntary muscle movement.

Relevant SCGE Project:

Advocacy Groups

Patient and Caregiver Perspectives

Treatment Preference for Patients with SMA 

  • The study looked at patients ages less than 1- 67 years old. 65 responses were self-reported, and 36 were caregiver-reported
  • Improvement in motor and breathing function was highly valued
  • Oral medication or one-time infusions were strongly preferred over repeated injections

Treatment preference among patients with spinal muscular atrophy (SMA): a discrete choice experiment | Orphanet Journal of Rare Diseases | Full Text (biomedcentral.com)

What are Urea Cycle Disorders?

Urea Cycle Disorders are genetic diseases that affect how the body removes the waste that is made from breaking down protein.

Relevant SCGE Project:

Advocacy Groups