Assisted nutrition: a difficult decision in patients with amyotrophic lateral sclerosis
Authors: Raymond Voltz
Journal: The Lancet Neurology (2015)

Management of patients with amyotrophic lateral sclerosis is a difficult task for the clinician. Difficulties start when making the correct diagnosis, which is often a protracted process because symptoms can start slowly, and the neurologist might refrain from making a diagnosis of amyotrophic lateral sclerosis for a long time owing to its poor prognosis. Once the diagnosis is established, besides traditional neurological care, palliative care skills are needed, which often are not in the forefront of neurological thinking. Amyotrophic lateral sclerosis affects not only neurological functions such as those of the motor system, but also survival functions such as breathing and eating. Finally, the clinician also has to resolve the issue of how patients will die and be cared for during the terminal phase.1Lulé D Nonnenmacher S Sorg S et al.Live and let die: existential decision processes in a fatal disease.J Neurol. 2014; 261: 518-525Crossref PubMed Scopus (40) Google Scholar, 2Voltz R Nübling G Lorenzl S Care of the dying neurologic patient.Handb Clin Neurol. 2013; 118: 141-145PubMed Google Scholar A diagnosis of amyotrophic lateral sclerosis is of course also difficult for the patients themselves and their families. Especially late in the disease, the fear of choking and the uncertainty of what will come next are most pressing. Where legally available, many patients opt for active euthanasia, showing the high psychological burden on patients and relatives.3Stutzki R Weber M Reiter-Theil S Simmen U Borasio GD Jox RJ Attitudes towards hastened death in ALS: a prospective study of patients and family caregivers.Amyotroph Lateral Scler Frontotemporal Degener. 2014; 15: 68-76Crossref PubMed Scopus (51) Google Scholar, 4Maessen M Veldink JH Onwuteaka-Philipsen BD et al.Euthanasia and physician-assisted suicide in amyotrophic lateral sclerosis: a prospective study.J Neurol. 2014; 261: 1894-1901Crossref PubMed Scopus (30) Google Scholar In such circumstances, clinical decisions about respiration and nutrition are highly emotionally charged. In-depth insight into the patient's view of amyotrophic lateral sclerosis is best provided by qualitative studies,5Greenaway LP Martin NH Lawrence V et al.Accepting or declining non-invasive ventilation or gastrostomy in amyotrophic lateral sclerosis: patients' perspectives.J Neurol. 2015; 262: 1002-1013Crossref PubMed Scopus (40) Google Scholar, 6Stavroulakis T Baird WO Baxter SK Walsh T Shaw PJ McDermott CJ The impact of gastrostomy in motor neurone disease: challenges and benefits from a patient and carer perspective.BMJ Support Palliat Care. 2014; (published online May 21.)https://doi.org/10.1136/bmjspcare-2013-000609Crossref Scopus (28) Google Scholar and for patients and clinicians to be able to make joint meaningful clinical decisions scientific data are desperately needed. One new small piece in the mosaic of clinical decision making in amyotrophic lateral sclerosis is presented in The Lancet Neurology by the ProGas Study Group,7ProGas Study GroupGastrostomy in patients with amyotrophic lateral sclerosis (ProGas): a prospective cohort study.Lancet Neurol. 2015; (published online May 29.)http://dx.doi.org/10.1016/S1474-4422(15)00104-0Google Scholar who followed up a large cohort of 330 patients with amyotrophic lateral sclerosis in the UK who underwent gastrostomy. The study, although not randomised, shows that, in the decision to do a gastrostomy, clinicians can choose among percutaneous endoscopic gastrostomy, radiologically inserted gastrostomy, or per-oral image-guided gastrostomy: the method used does not seem to affect the disease course, shown by the primary endpoint of mortality within 30 days (five [3%] of 163 patients died after percutaneous endoscopic gastrostomy, four [3%] of 121 after radiologically inserted gastrostomy, and three [7%] of 43 after per-oral image-guided gastrostomy; p=0·46), and by similar numbers of complications in each group. However, percutaneous endoscopic gastrostomy insertion was the most burdensome for the patient. The results of the study also show that, in most instances, artificial nutrition does not achieve the outcomes that clinicians would expect: little weight gain, if any, was achieved and the quality of life of patients was not really changed. The authors suggest that if artificial nutrition is considered, gastrostomy should be done early. They further suggest not waiting for weight loss of 10% from diagnosis, as is currently recommended,8Andersen PM Borasio GD Dengler R et al.EFNS task force on management of amyotrophic lateral sclerosis: guidelines for diagnosing and clinical care of patients and relatives.Europ J Neurol. 2005; 12: 921-938Crossref PubMed Scopus (238) Google Scholar but rather a weight loss of 5%. Other studies have argued that gastrostomy is safe even in patients with low forced vital capacity, but the procedure still carries risks.9Sarfaty M Nefussy B Gross D Shapira Y Vaisman N Drory VE Outcome of percutaneous endoscopic gastrostomy insertion in patients with amyotrophic lateral sclerosis in relation to respiratory dysfunction.Amyotroph Lateral Scler Frontotemporal Degener. 2013; 14: 528-532Crossref PubMed Scopus (25) Google Scholar Clinicians should speak about this decision with patients and their families as early as possible during the disease course. However, for this clinical discussion we need more than just technical data; we have to ask whether we do any good for patients by placing a gastrostomy. When is it really medically indicated? The study by the ProGas Study Group7ProGas Study GroupGastrostomy in patients with amyotrophic lateral sclerosis (ProGas): a prospective cohort study.Lancet Neurol. 2015; (published online May 29.)http://dx.doi.org/10.1016/S1474-4422(15)00104-0Google Scholar shows only little effect, but the study had no control group and does not show what happened to the patients who did not receive gastrostomy. Additionally, the investigators did not assess whether placement of gastrostomy has beneficial effects later in the disease course by making it easier for patients to receive drugs for symptom control when they can no longer swallow, and preventing parenteral administration of these drugs becoming necessary. It is also not clear, once artificial nutrition is started, how difficult it is to stop (leaving the gastrostomy in place) if no effect has been shown—yet another ethically difficult decision. How much is really gained in terms of life expectancy and quality of life by gastrostomy?10Dorst J Dupuis L Petri S et al.Percutaneous endoscopic gastrostomy in amyotrophic lateral sclerosis: a prospective observational study.J Neurol. 2015; 262: 849-858Crossref PubMed Scopus (59) Google Scholar A similar discussion is ongoing for patients with dementia.11Moran C O'Mahony S When is feeding via a percutaneous endoscopic gastrostomy indicated?.Curr Opin Gastroenterol. 2015; 31: 137-142Crossref PubMed Scopus (20) Google Scholar For all these questions, which are of high clinical and ethical relevance, we need many more clinical studies, be they qualitative or quantitative, prospective cohort studies such as ProGas7ProGas Study GroupGastrostomy in patients with amyotrophic lateral sclerosis (ProGas): a prospective cohort study.Lancet Neurol. 2015; (published online May 29.)http://dx.doi.org/10.1016/S1474-4422(15)00104-0Google Scholar or, even better, randomised controlled trials. Funders of amyotrophic lateral sclerosis research have to be urged to look, in addition to molecular studies, at these clinical questions, which are very relevant to patients and their families. I declare no competing interests. Gastrostomy in patients with amyotrophic lateral sclerosis (ProGas): a prospective cohort studyThe three methods of gastrostomy seemed to be as safe as each other in relation to survival and procedural complications. In the absence of data from randomised trials, our findings could inform clinicians and patients in reaching decisions about gastrostomy and will stimulate further research into the nutritional management in patients with amyotrophic lateral sclerosis. Full-Text PDF Open Access