The purpose of this study was to explore the unmet mental health and emotional palliative care needs of patients with Ehlers-Danlos Syndrome.
Background:
Ehlers-Danlos Syndrome (EDS) is defined as a group of 13 subtypes of incurable connective tissue disorders affecting an estimated 1:2,500 to 1:5,000 patients (Ehlers-Danlos Society, 2017). Presentation by subtype varies, but includes pain and progressive debility, with some subtypes also resulting in shortened life expectancies, vascular problems, and organ system complications (Pauker & Stoler, 2018).
Palliative care (PC) emphasizes quality of life, prioritizes symptom relief, and provides an
extra layer of support for patients and families in all stages of serious illness (Kelley & Morrison, 2015). PC is appropriate for any patient with a life-limiting illness, including EDS. Currently, there are eight identified domains included in palliative care: structure and processes of care, physical aspects, psychological aspects, social aspects, spiritual aspects, cultural aspects, care near the end of life, and ethical and legal aspects (Ahluwalia et al., 2018). Unfortunately, if a health care provider is not familiar with PC, then the patient is less likely to receive it (Kavalieratos et al., 2014). Importantly, PC has an undefined time limit (National Caregivers Library, 2017) and should begin at diagnosis of life-limiting illnesses.
PC in patients with rare diseases has not been well studied (Adams, Miller, & Grady, 2016). Depending upon the source, EDS is defined either as a rare disease or a common disease that is rarely diagnosed. Regardless of categorization of rare disease or rarely diagnosed common disease, palliative care needs have not been well documented in the EDS patient population. Some EDS providers recognize and encourage deliberate psychosocial support (Pauker & Stoler, 2018), but implementation of psychosocial support in patients with EDS is not well documented. As mental health and emotional needs are prioritized within the psychosocial domain of palliative care, it is appropriate to explore these palliative care needs of patients with EDS within the context of their EDS related mobility and pain issues.
Method:
This study was part of a larger descriptive, exploratory pilot needs assessment survey of all eight domains of palliative care in patients with Ehlers Danlos Syndrome. Participant recruitment was coordinated via International Society of Ehlers Danlos Syndrome and distributed by email list serv. Snowball sampling was encouraged. University IRB granted expedited review approval.
For the presented sub-component, the focus was only on unmet mental health and emotional needs. Thematic analysis of free texted responses (n=120) was used.
Results:
For the whole survey there were 317 clicks, 239 completed surveys with 30 additional partially completed surveys. For the descriptive question: “Do you have any emotional or mental needs that you feel aren’t well managed?”, 122 participants answered “yes”, 117 participants answered “no”. For participants who answered “yes”, there was a prompt to describe their unmet mental health or emotional needs, which resulted in 120 participants free-texting responses.
Thematic analysis identified patients experiencing psychological symptoms and disorders including anxiety, depression, and PTSD. Patients described hesitancy or refusal in talking with healthcare providers about their psychological symptoms. Patients used terminology such as “gas lighting” to describe interactions with healthcare providers and even family dismissing their mental health and emotional needs.
Implications:
In patients with EDS, there is a profound need to address emotional and mental health needs, both as part of formal palliative care services, but also with health care interactions beyond palliative care. Interdisciplinary collaborations and connections will be crucial in creating interventions to address unmet palliative care mental health and emotional needs in patients with EDS. Particular deliberate consideration given to home and remote support in light of the physical disability and geographic limitations many of these patients face will catalyze how patients with EDS can interact with providers in addressing unmet mental health and emotional needs.