Abstract
Article abstract Percutaneous endoscopic gastrostomy (PEG) has been proposed as symptomatic treatment of dysphagia in patients with ALS. Safety and factors related to survival after PEG were analyzed in 50 consecutive ALS patients. No major acute or long-term complications were observed. Stabilization or increase in weight were observed after PEG. Median survival after PEG was 185 days, with a worse outcome in patients with weight loss ≥10% healthy body weight and forced vital capacity <65%. PEG may be a useful option in the symptomatic treatment of dysphagia in ALS.
ALS is a neurodegenerative disease of adult life characterized by progressive weakness and atrophy of limb muscles, dysphagia, dysarthria, and hyperreflexia. Usually death occurs 1 to 3 years from onset as a consequence of respiratory failure, undernutrition, or aspiration pneumonia. Dysphagia is responsible for undernutrition and increased risk of aspiration pneumonia.1 In the early phases of ALS, dysphagia may be managed with compensatory strategies and qualitative and quantitative changes in the diet. In more advanced stages, when these strategies are no longer effective, enteral nutrition delivered via percutaneous endoscopic gastrostomy (PEG) represents a possible symptomatic treatment for dysphagia in ALS.1 However, only a few reports have analyzed the use of PEG in ALS.2-4 To determine the safety of PEG and the factors related to survival after its execution, we investigated a series of consecutive ALS patients who underwent PEG in the neurologic departments of three institutions in northern Italy.
Methods.
Patients.
From January 1993 to June 1998, PEG was proposed to a total of 68 ALS patients of 103 followed in the Departments of Neurology participating in the study. Of these, 51 accepted, but in 1 patient gastrostomy tube insertion was not possible due to a previous gastrectomy for a gastric ulcer. PEG was proposed to patients with moderate to severe dysphagia (8 patients) or weight loss (WL%) ≥5% with respect to healthy body weight (HBW),5 or both.
Since the occurrence of dysphagia, nutritional status was followed-up by a clinical nutritionist who proposed modifications of the diet and taught compensatory strategies. Body mass index (BMI) was calculated as follows: BMI = weight (kg)/height2 (m). WL% was calculated as follows: WL% = (1 − [weight at time of PEG/HBW]) × 100. HBW was determined as the usual weight before the onset of ALS as stated by the patient or one of the relatives. Respiratory status was analyzed as forced vital capacity percent of predicted (FVC%).
Intervention characteristics.
PEG was performed with the pull method using a commercially available gastrostomy kit. Local anesthesia was given in all patients but nine, who received general anesthesia because of highly compromised respiratory function. In the perioperative period, 2 grams IV ceftriaxone was given each day. Gastrostomy tube was not used in the first 24 hours from the intervention, after which time 500 mL water was given at a rate of 100 mL/h. Enteral nutrition was started on the second day. The patients or their caregivers, or both, underwent a training program on PEG feeding and management of minor complications.
Statistical methods.
Because a randomized control group was not acceptable for ethical reasons, the patients were compared with a group of ALS patients diagnosed during the 5-year period preceding the introduction of PEG in our institutions (1988–1992). Two controls were matched to each patient for age at diagnosis (±1 year), sex, type of onset of ALS (spinal or bulbar), and severity of disease as indicated by FVC% (±5%) and Norris score (±5 points) at diagnosis. Survival curves were calculated with the Kaplan-Meier method and compared with the log-rank test.5 Patients who underwent tracheostomy were considered censored at the time of ventilation. Multivariate analysis was performed with the Cox stepwise proportional hazards model.5 A p value <0.05 was considered significant.
Results.
The main demographic and clinical characteristics of the 50 patients (29 women and 21 men) who underwent PEG are reported in table 1.
Demographic, clinical, and nutritional characteristics of ALS patients
Safety.
PEG was generally well tolerated. No major acute complications were observed. There was no surgical mortality. The only long-term complication observed was the rupture of the feeding tube in one patient occurring 1 month after PEG; the tube was promptly replaced. In all patients, local pain after surgery was mild and required only treatment with nonsteroidal anti-inflammatory drugs for less than 24 hours.
Effects on nutritional factors.
In 25 of the 35 patients (71%) surviving more than 90 days there was an increase in weight (from 5.3 to 9.6%), usually occurring in the first 3 months, with a subsequent plateau. In all other patients WL% remained stable. All other nutritional factors (in particular serum proteins and electrolytes) remained within the normal range.
Factors influencing survival after PEG.
One patient (2%) died within 1 month. The median survival time after PEG was 185 days. Death was always related to respiratory failure. In univariate analysis, factors influencing the outcome after PEG were as follows: WL% at the time of PEG (median survival: ≥10%, 116 days; <10%, 305 days; p = 0.008) and FVC% (median survival: <65%, 138 days; ≥65%, 241 days; p = 0.04). BMI, serum albumin and chloride, age at time of PEG, sex, type of ALS (bulbar versus spinal), time between onset and PEG, treatment with riluzole, manual muscle testing score, and Norris score at time of PEG were not significant. The results of the multivariate model are reported in table 2.
Outcome after PEG: Multivariate analysis (Cox model)
Effect of PEG on overall survival.
No significant difference in overall survival was found between patients and controls (median survival time: 915 days for patients versus 760 days for controls, p = 0.09). In univariate analysis, factors influencing survival were as follows: type of ALS (p = 0.0012), FVC% at diagnosis (p = 0.0013), and age at onset (p = 0.0001). In multivariate analysis, PEG was also significantly related to survival, both in the whole series and in bulbar-onset patients but not in spinal-onset patients (table 3).
Factors influencing ALS survival: Multivariate analysis (Cox model)*
Discussion.
Management of dysphagia represents a major problem in ALS. Normal swallowing is critical for adequate nutrition; an impairment of this function can cause undernutrition and an increased risk of aspiration pneumonia.6 Moreover, difficulty in swallowing worsens the quality of life of ALS patients.7
PEG has been successfully used to treat dysphagia in various disorders, but only scanty data exist about the use of PEG in patients affected by ALS.2-4
According to our data, PEG is a well-tolerated and safe procedure with very low acute and long-term morbidity and negligible surgical mortality. In our series, survival after PEG was significantly related to respiratory status and WL% at the time of PEG, whereas nutritional factors were not significantly related to survival, probably because most of our patients had still relatively normal nutritional laboratory values at the time of PEG. Because some patients were still overweight at the time of PEG, although they had lost more than 10% of their HBW, BMI was not significantly related to survival.
A possible effect of PEG on overall ALS survival was observed, although it was not significant in univariate analysis; nevertheless, when considering PEG, the primary goal should be the improvement of nutritional status rather then life prolongation.
No consensus has yet been reached about the best timing to perform PEG in ALS patients. Usually the decision to perform PEG is made mostly on a clinical basis after considering nutritional status, hydration, clinical indices of aspiration, need for modifications of consistency of foods, and attitude of patients toward foods and meals.7,8 According to our observations, WL% is the most important objective factor related to nutritional status that should be taken into account when considering PEG. However, respiratory status should be considered because the tolerance of gastrostomy may depend on adequate vital capacity,9 and, as observed in our series, an impairment of FVC% is related to a worse outcome.
PEG represents a good option in the symptomatic treatment of ALS. It is effective in the management of undernutrition of ALS patients with severe dysphagia, with minimal morbidity and virtually no acute mortality. However, further studies on larger series are necessary to settle generally acceptable guidelines for establishing the best timing for PEG execution.
Appendix
Participating persons and centers of the ALS Percutaneous Endoscopic Gastrostomy Study Group: A. Chiò, MD, A. Cucatto, MD, A.A. Terreni, MD, A. Calvo, MD, D. Schiffer, MD, Department of Neuroscience, University of Turin, Italy; E. Finocchiaro, MD, M.C. Da Pont, MD, M. Rivetti, RD, P. Onorati, RD, Department of Clinical Nutrition, San Giovanni Hospital, Turin, Italy; G. Saracco, MD, A. Ferrari, MD, Department of Gastroenterology, University of Turin, Italy; P. Meineri, MD, E. Grasso, MD, Department of Neurology, Santa Croce Hospital, Cuneo, Italy; E. Bottacchi, MD, M. Corso, MD, General Regional Hospital, Aosta, Italy.
Acknowledgments
Acknowledgment
The authors thank Antonio Migheli, MD, PhD, for his help in the revision of the manuscript.
Footnotes
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↵*See the Appendix for a list of participating persons and centers of the ALS Percutaneous Endoscopic Gastrostomy Study Group.
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Presented in part at the 50th annual meeting of the American Academy of Neurology; Minneapolis, MN; April 1998.
- Received September 24, 1998.
- Accepted April 10, 1999.
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