Table 5 Areas for future research

1

Develop best care practices for healthcare providers specific to the needs at end-of-life

2

Examine how cultural and ethnic beliefs as well as local, state, provincial, and or national regulations affect the provision and reception of palliative care services within the EB population both globally and during individual visits with the medical provider(s)

3

Develop best care practices for healthcare providers in areas where access to interdisciplinary team and/or medical resources are limited due to things such as geography, economic conditions, and geopolitical tensions

4

Develop best care palliative care practices for healthcare providers that are specific to EB subtype

5

Develop best care palliative care practices for healthcare providers that are age group-specific (e.g., infancy, toddlerhood, childhood, adolescence, young adulthood, adulthood, older adulthood)

6

Evaluate the generalizability of the palliative care literature and, specifically, EoL comfort care literature related to people with other potentially life-limiting conditions such as cardiovascular diseases, cancer, chronic respiratory diseases, diabetes, and others to people living with EB

7

Further exploration of the experience of grief in people living with EB, as well as their families, carers, and healthcare providers and should pay special attention to examining EB-related grief across cultures and ethnicities

8

Develop additional pharmacologic treatments and nonpharmacologic skills to reduce pain and itch without increasing negative side-effects

9

Examine the potential satisfaction differences between those living with EB, their caregivers, and their treatment team(s)

10

Develop surveys that a) are EB-specific, wider-reaching, consider mental health conditions, and that measure QoL in small children to better understand the needs of people living with EB and b) differentiate between individual versus assumed independence, participation, or other values that are needed to support individualized care and ensure inclusive healthcare experiences

11

Explore the challenges and process of, and bioethics related to, medical futility and the decisions to provide, alter, or withhold specific aspects of care, especially at end of life, including terminating life-sustaining therapies (e.g., artificial nutrition) as well as the use of comfort care, palliative sedation, and the Groningen Protocol/euthanasia, especially in cases of the most severe subtypes of EB such as severe JEB subtype