Table 1 Characteristics of the studies included in the systematic review (n = 8)

Gysels MH, et al., UK, 2011

Qualitative study, with a semi-structured interview

48 patients experiencing daily problems of dyspnea (10 ALS, 10 cancer, 10 heart failure, and 18 COPD patients)

None

None

Individual experiences of breathlessness

-A semi-structured interview face-to-face was performed to understand the lived experience of breathlessness.

-Nine of the 10 ALS patients were using non-invasive positive pressure ventilation and three of them reported difficulties using it, but for those who had incorporated it into their daily routines, this device was the solution for their daily breathing problems.

-Breathlessness affects patients in different ways across different conditions, meaning that its management should be refined and interventions tailored to specific patient groups.

Simon ST, et al., UK, 2016

Qualitative study with Interviews

51 patients suffering from dyspnea (15 chronic heart failure, 14 COPD, 13 lung cancer, 9 ALS) recruited from five outpatient clinics

None

None

Strategies for relieving episodic breathlessness

-The six main strategies described by patients for relieving episodic breathlessness were: reduction of physical exertion, cognitive and psychological strategies, breathing techniques and positions, air and oxygen, drugs and medical devices, environmental and other strategies.

-Strategies did not differ between disease groups but between individual patients and were related to the trigger of the dyspnea episode. The identified strategies were largely easy to apply and can be practical aids in the daily care of patients suffering from episodic breathlessness.

-Not all recommended strategies (such as leaning forward, breathing techniques, or a draft of cold air) are helpful for all patients demonstrating that any support for patients should be individualized and tailored by the patient’s own experiences.

Veronese S, et al., Italy; 2017

Phase II non-blinded randomized controlled trial – parallel arm design

50 Adult patients severely affected by ALS (n = 16), multiple sclerosis (n = 18), Parkinson’s disease (n = 16). Informal family carers of the patients were also enrolled if they wished.

Immediate referral to PALC service (fast track) (n = 25)

16-week wait to referral to the PALC service (standard track) (n = 25)

Main outcomes: QOL; Burden of the carers. Secondary outcomes: Physical symptoms, including shortness of breath; social issues; psychological issues; spiritual issues; disability issues.

At baseline, there were no differences between groups.

After 16 weeks, fast track participants scored significant improvement in QOL and breathlessness.

The mortality was equal in the two arms.

Caregiver’s burden was not affected by the service.

Tiirola A, et al., Finland, 2017

Retrospective, longitudinal, cohort study

67 patients with non-malignant disease who died during the period of 2004 to 2013 (32 with ALS)

None

None

Prevalence of symptoms; Prescription of opioids, NIV, and oxygen (comparison between ALS patients and people with other diseases)

-Dyspnea was the most common symptom reported and it increased from admission to the last day of life, but there were no significant differences in the prevalence of symptoms between ALS patients and people with other non-malignant diseases.

-During the last 24 h, as-needed opioids were prescribed to nearly all patients (98%), and 75% of them were prescribed regular opioids.

-More than one third of patients with ALS used NIV during the last 24 h of life, whereas oxygen was more often given to patients with other diseases.

Morélot-Panzini C, et al., France, 2018

Prospective, longitudinal cohort study

41 ALS adult patients with chronic respiratory failure and indication for NIV

None

None

Effects of NIV on dyspnea

-Patients were allowed to select descriptors of dyspnea in sensory (e.g. “I feel air hunger”; “I am smothering”; “my chest and lungs feel tight or constrictor”; “my breathing requires muscle work or effort”; “my breathing requires mental effort or concentration”; “I am breathing a lot”; “not enough air, smothering or hunger for air”) and affective (depressed, anxious, frustrated, angry) dimensions.

-At inclusion 36.6% of the patients considered “lying supine” the worst dyspnoeic episode within the last 15 days, which is bound to have an extremely severe impact on daily life, making normal sleep impossible. But the 27 patients who attended the 1-month follow-up visit and who had used NIV had satisfactory clinical improvement because the most unpleasant breathing episode occurring in the preceding two weeks was “walking a few steps” (25.9%) or “talking or eating” (14.8%).

-The positive effects of NIV on dyspnea do not carry over to periods of unassisted breathing, an empirically intuitive notion that has however not been precisely documented before.

-This study supports the existence of dissociation between the sensorial and affective dimensions of dyspnea.

Eljas Ahlberg E, et al., Sweden, 2021

Retrospective comparative, registry study

825 patients with ALS as the main cause of death

None

3300 Patients with cancer as the main cause of death

Symptom assessment; Prescription of as-needed drugs;

Communication about transition to end of life care.

-Patients with ALS receive poorer end-of-life care than patients dying from cancer, in terms of validated symptom assessments and prescription of as-needed drugs.

-About 80% of the patients in both groups had no symptom assessment (other than pain) within the last week of life.

-More patients with ALS than with cancer had dyspnea and anxiety in the last week of life.

-There was no significant difference in communication about end-of-life between the two groups.

-Patients with ALS were less likely to have support from a specialized PALC team than patients with cancer.

Mehta AK, et al., USA, 2021

Retrospective comparative chart review

24 adult ALS patients admitted to two tertiary care academic hospitals from 2013 to 2018

9 patients seen by the inpatient PALC service (PALC group)

15 patients not seen by the inpatient PALC consult team (non PALC group)

Goals of Care; ACP; Symptom control;

survival benefit

-This study supports the benefit of inpatient PALC consultations for ALS patients admitted to the hospital for non-elective reasons.

-PALC consultations were associated with significantly increased Goals of Care, which includes the discussion of tracheostomy and transition to comfort care, thus ensuring that the patient’s wishes are followed during unexpected, acute changes.

-Goals of Care recorded during admission were 89% in the PALC group and 32% in non-PALC group.

-The PALC group were more significantly likely to have Goals of Care and ACP forms documented in their medical records at discharge time.

-Symptom management was performed by PALC in all 9 patients from the PALC group. Four of these were treated for dyspnea with opioids, benzodiazepines, nebulizers, and supplemental oxygen. Anxiety related to dyspnea was treated with benzodiazepines.

-The higher death in the PALC group at the end of the study was not significantly different from the non-PALC group.

Sennfält S, et al., Sweden, 2023 (ahead 2022)

Retrospective cohort study

Main cohort: 93 ALS patients diagnosed in 2016 and followed at the ALS Research Centre, Stockholm, deceased in 2018–2020. Complementary cohort: 2224 ALS patients from all of Sweden deceased in 2011–2020.

None

None

Use of NIV during the last 12 months of life; Prevalence of symptoms

-There was a gradual increase of patients on regular NIV during the last 12 months of life, reaching about 50% at the time of death in both ALS patients with spinal and bulbar onset.

-In the week before death, 57 of the 61 ALS patients with anticipated death (death as a culmination of a slow decline) from the main cohort, and 21 of the 29 patients with precipitous death (rapid and unexpected clinical worsening) had dyspnea. For most patients, dyspnea was managed effectively, but in 29.8% of patients from the first group, dyspnea was only partially relieved or not relived at all.

-The lower prevalence of dyspnea in the precipitous death group was not statistically significant.