Case Study 2:

Amyotrophic Lateral Sclerosis

Karina Almanza
CSUN Dietetic Intern 2017-2018
Preceptor: Mary Rose Tadena
Cedars-Sinai Medical Center
 ALS
(Amyotrophic
A progressive neurological
Lateral Sclerosis)
disease that causes destruction
of the motor neurons of the
Also known as
nervous system, resulting in
Lou Gehrig’s
muscle weakness, twitching,
disease
and atrophy.
Voluntary Neurons

Movement of extremities
Swallowing
Talking
Breathing
Involuntary Neurons

Memory
Reasoning
Vision
Hearing
Heartbeat
Digestion
 No cure available

Progressive decrease in function and

eventually death within 3 to 5 years
after diagnosis
Suggestions from Studies

1. Generally, more fractionation, high-calorie, high-protein, normolipidic, high-fibre, nutrient

unchanged, adequate water supply and ideal consistency against dysphagia are characteristics
of the diet-oriented treatment.

2. Antioxidants, carotenes, fruits, and vegetables were associated with higher amyotrophic
lateral sclerosis function at baseline. Nutritional care of the patient with amyotrophic lateral
sclerosis should include promotion of foods high in antioxidants and carotenes, including
fruits and vegetables.
ALS Functional
Rating Scale
Revised
Assessed in Outpatient Setting
ALS Functional
Rating Scale
Revised
Assessed in Outpatient Setting
Dysphagia Intervention for ALS

Normal Eating Habits (ALS Severity Scale Rating 10-9)

➢ Hydration and maintenance. Fluid intake to at least 2 qts/day

Early Eating Problems (ALS Severity Scale Rating 8-7)

➢ Modification of consistency, avoidance of thin liquids, and use of easier to chew foods

Dietary Consistency Changes (ALS Severity Scale Rating 6-5)

➢ Change food consistency to Mechanical soft or Pureed

Tube Feeding (ALS Severity Scale Rating 4-3)

➢ Placed with signs of dehydration and dysphagia. Loss of bulbar and respiratory muscles.
➢ Long-term access: PEG

Nothing by Mouth (ALS Severity Scale Rating 2-1)

➢ TF is permanent; mechanical ventilation needed for saliva flow
Nutrition Intervention: PEG Placement

Indications for PEG:

❖ Nutritional assessment
❖ Emphasising the drop in 10% of body weight in the last 3 months
❖ Presence of dysphagia
❖ Respiratory function assessment, highlighting forced vital capacity (FVC) measurement
for nearly 50% of what was predicted

Limitation for PEG:

❖ Moderate respiratory risk

Instead → Insert radiologic gastrostomy, either between complications, during and after the
procedure, or in survival of patients
Calculating Energy Needs

Total Daily Energy Expenditure (TDEE)

1. Harris Benedict Equation for basal energy expenditure (BEE)
2. Calculated daily energy expenditure with ALS population specific
predictive formula
3. +/- calories for weight gain/ loss
Protein
1 g/kg
Fluids
Minimum of calories calculated
ALS: Total Daily Energy Expenditure

For Women For Men

Initial Assessment

Assessment:
RD consult received for TF. 74 yo M with ALS admitted for dysphagia. Met
pt at bedside s/p EGD PEG; wife present. Pt has had ongoing wt loss with
progressive dysphagia d/t inability to tolerate pureed and thickened liquids
diet. Wife was provided with nutrition education regarding TF and different
modes of TF. Suggest high fiber formula to assist with pt's bowel motility/
constipation. Current TF order by MD meets nutrient demands but lacks
fiber. For home, suggest formula higher in fiber.
Initial Assessment

Dx: Dysphagia Anthropometrics:

Height: 182.9 cm (6' 0.01")
PMHx: ALS, Kidney stones Weight: 72.6 kg (160 lb)
Food Intolerances/Allergies: BMI (Calculated): 21.69
No Known Food Allergies Desirable Weight: 178#
Diet history: Puree, thick liquids % Desirable Weight: 90%
Physical Appearance: pt on bi-pap Usual Weight: 176# (~4 months ago)
support %Usual Weight: 91%
%Weight change: 9% loss in 4 months.
Unintentional
 Wt Readings from Last 15
Initial Assessment Encounters:
05/09/18 72.6 kg (160 lb)
Weights 03/21/18 76.7 kg (169 lb 3.2 oz)
01/24/18 79.8 kg (176 lb)
01/10/18 80 kg (176 lb 6.4 oz)
12/13/17 83.9 kg (185 lb)
09/06/17 81.9 kg (180 lb 9.6 oz)
06/07/17 80.2 kg (176 lb 12.8 oz)
03/08/17 85.8 kg (189 lb 3.2 oz)
11/30/16 86.6 kg (191 lb)
09/14/16 88 kg (194 lb)
06/01/16 86.7 kg (191 lb 3.2 oz)
03/02/16 90 kg (198 lb 6.4 oz)
12/02/15 90.3 kg (199 lb)
09/02/15 88.9 kg (196 lb)
08/21/15 88.3 kg (194 lb 9.6 oz)
Initial Assessment

I/O: Incomplete; in GI Lab

Skin: No Pressure Ulcer Documented.
Impaired Skin Integrity-Wound Right Posterior Leg
Edema: none documented
Labs include: none collected
Meds include: Vitamin D3; glycopyrrolate (robinul); ondansetron (zofran);
polyethylene glycol 3350 (glycolax)
GI: Dysphagia s/p G-tube
Current diet Rx: No active orders
Initial Assessment

Estimated Needs based on 160# (72.7 kg) admit weight (as of 5/9):
Harris Benedict Equation for basal energy expenditure (BEE): 1480 kcal
ALS Functional Rating Scale-6 score (speech, handwriting, dressing/hygiene,
turning in bed, walking, dyspnea) total: 5
Calculated daily energy expenditure with ALS population specific predictive
formula: 1592 kcal to maintain weight; >1842 kcals (weight gain plus 250 kcal)
Protein goal 1 g/kg: 73 g
Fluids: at least 1842 ml/D (1 ml/kg)
PES Statement

Nutrition Diagnosis/Problem:
1. Difficulty swallowing related to ALS progression as
evidenced by TF (G-tube) placement.
Initial Assessment

Interventions:
1. Coordination of care: Nutrition care plan communicated with team. See
recommendations box at top of note.
2. Nutrition Education: explained rationale for tube feeding to wife/CG. CG
receptive to education and nutrition plan; expect good compliance. RD to
continue to follow for discharge planning needs as needed. Nutrition
discharge planning needs: bolus GT feeds for home.
Initial Assessment

Monitor/Evaluate:
1. Nutrition support to meet +/-10% of energy and protein goals.
New goal.
2. Lab: WNL (POC gluc < 180, Chemistries WNL). New goal.
3. GI: 1-3BM Q1-3 days (or 500mL max daily). New goal.
4. Residuals < 200 ml; abdomen non-distended, no abdominal
pain. New goal.
Initial Assessment

Nutrition priority level is 3; RD to follow up within 5 days.

Current TF Rx: Nutren 1.5 kcal/ml Bolus 250 ml 5x/day

Vol 1250 mL Water 955 mL

Energy 1875 kcal (102% goal) Na 70 mEq
Protein 85 g (116% goal) K 76.9 mEq
Carbs 220 g Phos 47.5 mmol
Fiber 0 g Vit C 375 mg
Fat 75 g Zinc 25 mg
Vit/Min >125% RDA
Water deficit for 1 mL/kcal maintenance energy need
is 887 mL
Initial Assessment

Recommendations for Physicians:

1. Nutren 1.5 kcal, 250 ml bolus 5x daily as ordered, while in the hospital
2. TF at home: Consider Jevity 1.5 or equivalent, 1 can (~250 ml) 5x daily
administered as bolus feeds in G-Tube
3. Free water flushes 200 ml 5x daily in between bolus feeds

Karina Almanza, Dietetic Student

Mary Rose Tadena, RD, CNSC ext. 42783
Questions?
Thank you!
1. Salvioni, C., Stanich, P., Almeida, C., Oliveira, A., Dos Santos Salvioni, C., & Bulle Oliveira, P. (2014). Nutritional
care in motor neurone disease/amyotrophic lateral sclerosis. Arquivos De Neuro-Psiquiatria, 72(2), 157-163.
2. Wills, Hubbard, Macklin, Glass, Tandan, Simpson, . . . Cudkowicz. (2014). Hypercaloric enteral nutrition in patients
with amyotrophic lateral sclerosis: A randomised, double-blind, placebo-controlled phase 2 trial. The Lancet,
383(9934), 2065-2072.
3. Nieves, J., Gennings, C., Factor-Litvak, P., Hupf, J., Singleton, J., Sharf, V., . . . Mitsumoto, H. (2016). Association
Between Dietary Intake and Function in Amyotrophic Lateral Sclerosis. JAMA Neurology, 73(12), 1425-1432.
4. Kasarskis, Edward J., Mendiondo, Marta S., Matthews, Dwight E., Mitsumoto, Hiroshi, Tandan, Rup, Simmons,
Zachary, . . . Kryscio, Richard J. (2014). Estimating daily energy expenditure in individuals with amyotrophic lateral
sclerosis. American Journal of Clinical Nutrition,99(4), 792-803.
5. Price, S., & Wilson, L. (1986). Pathophysiology: Clinical concepts of disease processes (3rd ed.). New York: McGraw-
Hill.
6. Nahikian-Nelms, M. (2011). Nutrition therapy and pathophysiology (2nd ed.). Belmont, CA: Wadsworth, Cengage
Learning.
7. Mahan, LK, Escott-Stump, S, Raymond, J. (2011) Krause’s Food and the Nutrition Care Process (13th edition).
Saunders.

References