Tasks and interfaces in primary and specialized palliative care for Duchenne muscular dystrophy – A patients’ perspective

Janisch, Maria; Boehme, Kristin; Thiele, Simone; Bock, Annette; Kirschner, Janbernd; Schara, Ulrike; Walter, Maggie C.; Nolte-Buchholtz, Silke; Hagen, Maja von der

doi:10.1016/j.nmd.2020.09.031

Cited by 9 publications

(15 citation statements)

References 34 publications

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“…At the same time, parents report significant discomfort regarding discussions about death with their children with NMD. However, the same study showed a positive association between the frequency of these discussions and advanced care planning [44].…”

Section: Palliative Care In Neuromuscular Diseasementioning

confidence: 82%

“…Although respiratory manifestations are often the primary drivers of symptoms towards the end of life, individuals with NMD often experience systemic complications including malnutrition, cardiac dysfunction [43], and scoliosis. In addition, patients often report significant musculoskeletal pain, fatigue, depressive mood, constipation, and dyspnea [44]. Despite the heterogeneous etiologies of these disorders, respiratory muscle training, mucociliary clearance, controlled ventilation, and nutritional support form the backbone of supportive care for patients with NMD.…”

Section: Neuromuscular Disease Clinical Coursementioning

confidence: 99%

“…Patients and family members with DMD report uncertainty over the trajectory and management of NMD, particularly regarding the timing of discussions of end-of-life planning or how to connect to palliative care resources [44]. At the same time, parents report significant discomfort regarding discussions about death with their children with NMD.…”

Section: Palliative Care In Neuromuscular Diseasementioning

confidence: 99%

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Palliative Care in Pediatric Pulmonology

et al. 2021

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Children with End Stage Lung Disease (ESLD) are part of the growing population of individuals with life-limiting conditions of childhood. These patients present with a diverse set of pulmonary, cardiovascular, neuromuscular, and developmental conditions. This paper first examines five cases of children with cystic fibrosis, bronchopulmonary dysplasia, neuromuscular disease, pulmonary hypertension, and lung transplantation from Texas Children’s Hospital. We discuss the expected clinical course of each condition, then review the integration of primary and specialized palliative care into the management of each diagnosis. This paper then reviews the management of two children with end staged lung disease at Hospital Civil de Guadalajara, providing an additional perspective for approaching palliative care in low-income countries.

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Section: Palliative Care In Neuromuscular Diseasementioning

confidence: 82%

Section: Neuromuscular Disease Clinical Coursementioning

confidence: 99%

Section: Palliative Care In Neuromuscular Diseasementioning

confidence: 99%

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Palliative Care in Pediatric Pulmonology

et al. 2021

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“…Ideally, comprehensive ALS-care should be coordinated by a fixed, easily accessible contact partner who coordinates all aspects of care throughout the whole disease course [ 19 ], e.g., a case manager. This is not only supposed to lower health costs [ 80 ], but also to provide emotional relief and a feeling of trust for patients and caregivers [ 64 , 81 ].…”

Section: Discussionmentioning

confidence: 99%

Caregivers’ View of Socio-Medical Care in the Terminal Phase of Amyotrophic Lateral Sclerosis—How Can We Improve Holistic Care in ALS?

Linse

Aust

Günther

et al. 2022

JCM

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Multidimensional socio-medical care with an early integration of palliative principles is strongly recommended in amyotrophic lateral sclerosis (ALS), but provided inconsistently. We conducted telephone interviews with 49 former caregivers of deceased ALS patients to examine their experience of care in the terminal phase including caregiver burden. Patients who received specialized palliative care (45% of patients) were more likely to die at home (p = 0.004) and without burdening symptoms (p = 0.021). The majority of caregivers (86%) reported deficits in socio-medical care. Most frequently mentioned were problems receiving medical aids (45%) and a lack of caregiver support (35%). A higher level of deficits experienced by caregivers was associated with negative health outcomes on the side of the caregivers (reported by 57% of them; p = 0.002) and stronger caregiver burden (p = 0.004). To provide good quality of dying to patients and reduce the burden on caregivers, multidimensional—including palliative—care in ALS urgently needs to be strengthened in the healthcare structures.

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“…Palliative care engagement is recommended throughout the lifespan of these individuals 1 ; however, practices surrounding these topics and palliative care team involvement in DMD is highly variable across centers. The few published studies consistently showed underutilization of palliative care services in DMD 3‐8 . The reasons for this are multilayered, and may include limited resources, time constraints, lack of awareness among providers or families, and lack of willingness of patients and their families to engage with these services.…”

mentioning

confidence: 99%