**4. Nutritional therapy**

Patients with symptoms of dysphagia limiting their intake of foods and liquids, hospitalized or at home, should be considered those at high risk of experiencing nutritional deficiencies and consequently should be treated.

Appropriate nutrition and hydration in patients with dysphagia are based on a complex balance between preparation, intake and absorption of foods and drinks (Steele & Lieshout, 2004).

When diagnosing the cause and severity of dysphagia, healthcare professionals can determine the texture of foods and the thickness of fluids for a safer swallowing by dysphagic patients, since the consistency of the diet should be individualized according to the type and extent of dysfunction. In case the recipe is not followed, the subject may face serious consequences for health (Silva et al, 2010; Macedo & Furquim, 2000).

Table 1 shows an example of a modified diet with restriction of "thin liquids" (1-50 cP) and solids for subjects with dysphagia and swallowing impairment.

Nutritional Care in Amyotrophic Lateral Sclerosis:

of liquids (Silva et al, 2010)

adjustment of the correct consistency.

standard (Silva et al., 2006a).

2006b). Studies like this are still scarce for this population.

An Alternative for the Maximization of the Nutritional State 603

Changes in viscosity of foods and fluids can be achieved with the help of commercial thickeners. The choice of thickening agent is critical to achieve a homogeneous and lasting consistency. The thickeners should interfere as little as possible with the sensory properties

Several agents can be used as food thickeners. Such thickeners are mostly composed of polysaccharides (carbohydrates), such as gums and starches, in addition to pectins and cellulose derivatives. Among them, the modified starch is one of the most used, since the starch physically or chemically treated improves the properties of thickening, cohesion, stability, gelatinization, luster and taste of the natural starch. In addition, they can also maximize the nutritional and water intake, facilitating a wide variety of textures (Silva & Ikeda, 2009). Therefore, these modified starch-containing thickeners can be used to prevent dehydration of subjects with dysphagia (Ada, 2002). However, the commercial thickeners are very expensive (approximately R\$ 40.00 BRL/200 g), which limits the purchase and

It is known that the intake may be maximized by adjusting the consistency of foods through simple and low-cost techniques, without using commercial thickeners that are very expensive (Silva & Ikeda, 2009; Whelan, 2001). Thickening of foods by using the own foods in several preparations so as to adjust the correct consistency is still unknown by many patients, caregivers and healthcare professionals, limiting the food intake, resulting in high rates of malnutrition, dehydration and pulmonary aspiration, and increasing the risk of death32. These techniques are designed for this population, especially regarding the amount of food in household measures necessary to achieve optimal viscosity, according to the ADA

In 2006, researches were conducted in order to develop a guide with recent literature survey, standardized preparations for patients with dysphagia, viscosities adjusted according to the ADA, chemical composition and photographic record for healthcare professionals, caregivers and patients with dysphagia, for a safe dietary intake (Silva et al.,

The poor knowledge of the fundamental physical characteristics of the consistency of the preparations is considered a limiting factor to adjust the viscosity, which does not ensure a safe intake. Figure 1 shows a photographic representation of a preparation of heart of palm cream with the consistency of pudding. Its main characteristic is the formation of a heavy cake, in

which there is low adherence on the spoon surface, forming no continuous filaments.

Fig. 1. Photographic representation of a preparation (heart of palm cream) with pudding

consistency. pH = 3.73; viscosity = 2000 cP; amount of water for dilution = 0 mL


*\** Adapted from Peres, Manzano and Silva (2007).

Table 3. Modified diet with restriction of "thin liquids" (1-50 cP) and "solids" for subjects with dysphagia and swallowing impairment. Features: Soft, wet and liquefied foods. Liquid foods are all thickened. The example menu contains approximately 2,000 kilocalories.

*Breakfast:* Dried milk porridge

*Lunch:*

Mashed banana

(liquefied)

*Snack:* Juice of fruits (papaya,

*Dinner:* Vegetable broth

*Snack:* Thickened papaya juice Water: 30 mL

Spaghetti and basil soup

Thickened orange juice

banana and apple)

Lemon Mousse

*\** Adapted from Peres, Manzano and Silva (2007).

*Supper:* Maize porridge Milk: 100 mL

**Meal Food Ingredients (Servings) Viscosity** 

Milk: 100 mL Dried Milk: 25 g 1 unit – 90 g

spoon).

100 g

Table 3. Modified diet with restriction of "thin liquids" (1-50 cP) and "solids" for subjects with dysphagia and swallowing impairment. Features: Soft, wet and liquefied foods. Liquid foods are all thickened. The example menu contains approximately 2,000 kilocalories.

Orange juice: 200 mL Thickener: 10 g

Cold fluid milk (10o)

(200 mL), papaya (100 g), banana (90 g), apple (50 g)

Water (2 L), turnips (2 units), carrots (2 units), garlic clove (1 unit), onion (1 unit), arracacha (1 unit), bunch of watercress (1 unit), basil (to taste), salt (to taste), and a drizzle of olive oil, raw large potato (1 unit)

Maize bran: 25 g 840

Papaya: 170 g 870

Spaghetti (125 g), vegetable oil (2 tablespoons), onion (1 unit), mashed garlic cloves (2 units), nut (60 g), chicken bouillon (70 mL), fresh basil leaves (30 g), salt to taste, grated cheese (1 dessert **(cP)** 

910

2.900

2.440

320

1.090

4.680

8.000

Changes in viscosity of foods and fluids can be achieved with the help of commercial thickeners. The choice of thickening agent is critical to achieve a homogeneous and lasting consistency. The thickeners should interfere as little as possible with the sensory properties of liquids (Silva et al, 2010)

Several agents can be used as food thickeners. Such thickeners are mostly composed of polysaccharides (carbohydrates), such as gums and starches, in addition to pectins and cellulose derivatives. Among them, the modified starch is one of the most used, since the starch physically or chemically treated improves the properties of thickening, cohesion, stability, gelatinization, luster and taste of the natural starch. In addition, they can also maximize the nutritional and water intake, facilitating a wide variety of textures (Silva & Ikeda, 2009). Therefore, these modified starch-containing thickeners can be used to prevent dehydration of subjects with dysphagia (Ada, 2002). However, the commercial thickeners are very expensive (approximately R\$ 40.00 BRL/200 g), which limits the purchase and adjustment of the correct consistency.

It is known that the intake may be maximized by adjusting the consistency of foods through simple and low-cost techniques, without using commercial thickeners that are very expensive (Silva & Ikeda, 2009; Whelan, 2001). Thickening of foods by using the own foods in several preparations so as to adjust the correct consistency is still unknown by many patients, caregivers and healthcare professionals, limiting the food intake, resulting in high rates of malnutrition, dehydration and pulmonary aspiration, and increasing the risk of death32. These techniques are designed for this population, especially regarding the amount of food in household measures necessary to achieve optimal viscosity, according to the ADA standard (Silva et al., 2006a).

In 2006, researches were conducted in order to develop a guide with recent literature survey, standardized preparations for patients with dysphagia, viscosities adjusted according to the ADA, chemical composition and photographic record for healthcare professionals, caregivers and patients with dysphagia, for a safe dietary intake (Silva et al., 2006b). Studies like this are still scarce for this population.

The poor knowledge of the fundamental physical characteristics of the consistency of the preparations is considered a limiting factor to adjust the viscosity, which does not ensure a safe intake. Figure 1 shows a photographic representation of a preparation of heart of palm cream with the consistency of pudding. Its main characteristic is the formation of a heavy cake, in which there is low adherence on the spoon surface, forming no continuous filaments.

Fig. 1. Photographic representation of a preparation (heart of palm cream) with pudding consistency. pH = 3.73; viscosity = 2000 cP; amount of water for dilution = 0 mL

Nutritional Care in Amyotrophic Lateral Sclerosis:

recipe with pudding viscosity = 107.14 mL

(Santoro, 2008)

2003)

audiologist.

replication (Nguyen et al., 2006).

An Alternative for the Maximization of the Nutritional State 605

life and reduction of potential complications, through education/health promotion programs, including specialized procedures and orientation programs for caregivers

Fig. 4. Photographic representation of a preparation (heart of palm cream) with thin consistency. pH = 5.36; viscosity = 46 cP; amount of water for dilution in 100 mL of the

Periodic and appropriate reassessments of the swallowing condition are critical aspects for the prevention/recovery from malnutrition. One study assessed the adequacy of the diet of elderlies admitted to nursing homes, where 91% of the patients had diets with a consistency below what they could tolerate safely. Both the nutritional status and the quality of life may be affected when patients are maintained on diets with inappropriate viscosity (Souza et al.,

Patients with dysphagia may experience satiety quickly when they are given an extremely concentrated meal. Instead of providing three meals a day, these patients should receive smaller and more frequent portions (Silva et al., 2003). Of note, for patients with dysphagia, difficulty in performing the swallowing movements worsens when they are most tired. This is especially important for patients with diseases like Parkinson's, for which the medication effect can be reduced during the day, further reducing the patient's ability to swallow (Sachdev, 2005). The correct positioning of the patient may be of great help during meals, but it is important to follow the instructions of a speech therapist &

If there is a high risk of aspiration or oral intake is insufficient to maintain the good nutritional status, the possibility of an alternative nutritional support must be considered. A soft and well tolerable tube can be inserted and radiologically guided. Percutaneous endoscopic gastrostomy is performed by inserting a gastrostomy tube into the stomach through a percutaneous abdominal route guided by the endoscopist and, if available,

Therefore, the guidance on an individualized diet, precautions on the risk of aspiration, and appropriate choice regarding the route of access for feeding, help to prevent malnutrition in patients with dysphagia, where the care of a multidisciplinary team is required for the patient's welfare, as well as for a better quality of life. Nonetheless, the absence of detailed descriptions on the procedures for nutritional therapy makes unfeasible their efficient

surgical gastrostomy is preferable (Ickenstein, 2003; Nguyen et al., 2006).

Figure 2 shows a photographic representation of the same preparation with the consistency of honey, in which there is a formation of continuous filament with the base of the spoon forming a characteristic "V".

Fig. 2. Photographic representation of a preparation (heart of palm cream) with honey consistency. pH = 3.69; viscosity = 1080 cP; amount of water for dilution in 100 mL of the recipe with pudding viscosity = 14.23 mL

In the photographic representation of the nectar consistency (Figure 3), there is a formation of continuous filament thinner than the previous one, without a characteristic "V" at the base of the spoon.

Fig. 3. Photographic representation of a preparation (heart of palm cream) with nectar consistency. pH = 3.71; viscosity = 240 cP; amount of water for dilution in 100 mL of the recipe with pudding viscosity = 42.88 mL

Figure 4 shows a photographic representation of the thin consistency of the same preparation; as the name implies, there is no formation of continuous filament, but only drops that fall from the spoon.

These alternatives are considered simple, low-cost and safe, and are extremely important to ensure a better quality of life for patients without dysphagia, without limiting the need for commercial thickeners, but guidelines concerning how to follow a correct preparation are still necessary. Currently, there are discussions on the improvement of the quality of

Figure 2 shows a photographic representation of the same preparation with the consistency of honey, in which there is a formation of continuous filament with the base of the spoon

Fig. 2. Photographic representation of a preparation (heart of palm cream) with honey consistency. pH = 3.69; viscosity = 1080 cP; amount of water for dilution in 100 mL of the

Fig. 3. Photographic representation of a preparation (heart of palm cream) with nectar consistency. pH = 3.71; viscosity = 240 cP; amount of water for dilution in 100 mL of the

Figure 4 shows a photographic representation of the thin consistency of the same preparation; as the name implies, there is no formation of continuous filament, but only

These alternatives are considered simple, low-cost and safe, and are extremely important to ensure a better quality of life for patients without dysphagia, without limiting the need for commercial thickeners, but guidelines concerning how to follow a correct preparation are still necessary. Currently, there are discussions on the improvement of the quality of

In the photographic representation of the nectar consistency (Figure 3), there is a formation of continuous filament thinner than the previous one, without a characteristic "V" at the base

forming a characteristic "V".

recipe with pudding viscosity = 14.23 mL

recipe with pudding viscosity = 42.88 mL

drops that fall from the spoon.

of the spoon.

life and reduction of potential complications, through education/health promotion programs, including specialized procedures and orientation programs for caregivers (Santoro, 2008)

Fig. 4. Photographic representation of a preparation (heart of palm cream) with thin consistency. pH = 5.36; viscosity = 46 cP; amount of water for dilution in 100 mL of the recipe with pudding viscosity = 107.14 mL

Periodic and appropriate reassessments of the swallowing condition are critical aspects for the prevention/recovery from malnutrition. One study assessed the adequacy of the diet of elderlies admitted to nursing homes, where 91% of the patients had diets with a consistency below what they could tolerate safely. Both the nutritional status and the quality of life may be affected when patients are maintained on diets with inappropriate viscosity (Souza et al., 2003)

Patients with dysphagia may experience satiety quickly when they are given an extremely concentrated meal. Instead of providing three meals a day, these patients should receive smaller and more frequent portions (Silva et al., 2003). Of note, for patients with dysphagia, difficulty in performing the swallowing movements worsens when they are most tired. This is especially important for patients with diseases like Parkinson's, for which the medication effect can be reduced during the day, further reducing the patient's ability to swallow (Sachdev, 2005). The correct positioning of the patient may be of great help during meals, but it is important to follow the instructions of a speech therapist & audiologist.

If there is a high risk of aspiration or oral intake is insufficient to maintain the good nutritional status, the possibility of an alternative nutritional support must be considered. A soft and well tolerable tube can be inserted and radiologically guided. Percutaneous endoscopic gastrostomy is performed by inserting a gastrostomy tube into the stomach through a percutaneous abdominal route guided by the endoscopist and, if available, surgical gastrostomy is preferable (Ickenstein, 2003; Nguyen et al., 2006).

Therefore, the guidance on an individualized diet, precautions on the risk of aspiration, and appropriate choice regarding the route of access for feeding, help to prevent malnutrition in patients with dysphagia, where the care of a multidisciplinary team is required for the patient's welfare, as well as for a better quality of life. Nonetheless, the absence of detailed descriptions on the procedures for nutritional therapy makes unfeasible their efficient replication (Nguyen et al., 2006).

Nutritional Care in Amyotrophic Lateral Sclerosis:

improve the quality of life of subjects with ALS.

*Postgraduate Medicine*, Vol. 71, pp 46-63

*Neurology*, Vol. 244, pp.S11-S7. Supl. 4.

*theNeurological Sciences*, Vol 64, pp. 65–71

Springer. DOI 10.1007/978-1-4419-1788-1\_39.

**5. Conclusions** 

**6. References** 

pp 115-119

*(In Portuguese)*

31-64. *(In Portuguese*)

330-342

116-120

An Alternative for the Maximization of the Nutritional State 607

This chapter was conducted to support the hypothesis of the thesis and gathers scientific information listing the main practices for assessment, from the nutritional point of view, in patients with ALS. The relevant literature available for consultation is limited. Studies on food intake, specific techniques for assessment of nutritional status, and the use of supplements are scarce. However, the follow-up of nutritional status by monitoring the anthropometric evolution, body composition and clinical signs, such as dysphagia, may

ADA. (2002). National Dysphagia Diet Task Force. *National Dysphagia Diet: Standardization* 

Albert, SM., Murphy, PL., Del Bene, M., Rowland, LP., Mitsumoto, H. (2001). Incidence and

Barros, PB., Manzano, FM., Silva, LBC. (2006). *Manual de Técnicas e Receitas para Espessamento* 

Blackburn, GL., Havey, KB. (1982). Nutritional assessment as a routine in clinical medicine*.* 

Blackburn, GL., Thorrnton, PA. (1979). Nutrition assessment of the hospitalized patient.

Borasio, GD., Voltz, R. (1997). Palliative care in amyotrophic lateral sclerosis. *Journal of* 

Calia, LC., Annes, M. (2003). Afecções neurológicas periféricas. In: Levy, JA.; Oliveira, AS.

Campbell, MJ., Enderby, P. (1984). Management of motor neurone disease. *Journal of* 

Carvalho-Silva, LBC. (2011). Anthropometric wrist and arm circumference and their

Chiappetta, ALML. (2005). *Disfagia Orofaríngea em Pacientes com Doença do Neurônio* 

Chiappetta, ALML., Oda, AL. (2004). Doenças neuromusculares. In: Ferreira, L. P.,

Chumlea, MAC., Roche, AF., Steinbaugh, ML. (1958). Estimating stature from knee height

Escola Paulista de Medicina, Universidade Federal de São Paulo.

*Reabilitação em doenças neurológicas – guia terapêutico prático.* São Paulo: Atheneu, pp.

derivations: application to amyotrophic lateral sclerosis. In *Handbook of Anthropometry:Physical Measuresof Human Form in Health and Disease*. New York:

*Motor/Esclerose Lateral Amiotrófica*. São Paulo. 124. Tese (Doutor em Ciências) –

Benefilopes, D. M., Limongi, S. C. Ed. *Tratado de fonoaudiologia*. São Paulo: Roca, pp.

for persons 60 to 90 years of age. *Journal of American Geriatrics Society*, Vol. 33, pp.

predictors of GEP placement in ALS/MND. *Journal of Neurological Sciences*, Vol.191,

*de Alimentos: utilização de diferentes amidos espessantes*, São Paulo: Cescorf pp 10-68.

*for Optimal Care*. The American Dietetics Association. pp 47

*Medicine Clinical Nutrition of American*, Vol 63, pp. 1103-1115

### **4.1 Nutritional support**

Nutritional support may delay the weight loss and muscle atrophy. Researchers have shown the weight loss associated with bulbar changes (dysphagia and breathing) require early and specific nutritional support (Kasarskis *et al*., 1996; Slowie *et al*., 1983).

Constant muscle atrophy, characteristic of progressive diseases, may mask the increased metabolic demand. The increased baseline energy expenditure of patients with ALS occurs since the energies are focused on the maintenance of pulmonary ventilation (Stanich *et al*., 2004; Kasarskis *et al*., 1996; Nau *et al*., 1995; Shimizu; Hayashi; Tanabe, 1991).

In a study of ALS patients, under the oral nutritional supplementation program, there was a progressive decrease in body mass index (BMI) in patients with progressive bulbar palsy and preservation of such variables in ALS patients. The lean mass/fat mass ratio was maintained during the study for both groups. The nutritional status classification has not changed for 70% of the patients. The results showed that supplementation prevented the worsening of nutritional status, but was unable to correct the overall averages of adequacy (Stanich et al., 2004).

In clinical practice, the use of supplements of vitamins, especially vitamin E, is common. The supplementation of this vitamin, with quantity still not defined, is expected to improve the nutritional profile of subjects with ALS (Borasio; Voltz, 1997). Oral supplementation with creatine monohydrate at 3g/day showed no improvement of nutritional status in ALS. However, the energy and protein supplementation is used by many professionals, and has proven to be efficient in the nutritional status of subjects with ALS (Rio; Cawadias, 2007; Heffernan et al., 2004).

Silva et al., (2010) evaluated the efficacy of oral supplementation with milk whey proteins and modified starch (70%WPI:30%MS), on nutritional and functional parameters of patients with ALS. Sixteen patients were randomized to two groups, treatment (70%WPI:30%MS) and control (maltodextrin). They underwent prospective nutritional, respiratory and functional assessment for 4 months. Patients in the treatment group presented weight gain, increased BMI, increased arm muscle area and circumference, higher albumin, white blood cell and total lymphocyte counts, and reduced creatine-kinase, aspartate aminotransferase and alanine aminotransferase. In the control group, biochemical measures did not change, but weight and BMI declined. The results indicate that the agglomerate 70%WPI:30%MS may be useful in the nutritional therapy of patients with ALS.

### **4.2 Alternative feeding in ALS**

Different authors report the need for alternative routes of nutrition from the following criteria: vital capacity of approximately 50% of the expected value, presence of moderate to severe dysphagia and 10% reduction in body weight over the past three months. (Stanich *et al*., 2004; Mitsumoto *et al*., 2003; Albert *et al*., 2001; Silani; Kasarskis; Yanagisawa, 1998; Lisbeth et al., 1994).

Percutaneous endoscopic gastrostomy (PEG) is an option for the symptomatic treatment of patients with ALS (Miller et al., 1999).

When comparing the use of enteral nutrition via nasogastric tube and percutaneous endoscopic gastrostomy (PEG) in patients with ALS, there is a significant difference in the body mass index (BMI) of patients with PEG compared to those with a nasogastric tube, as well as a better social acceptance and, consequently, quality of life of the patients studied, supporting the use of this technique when oral intake is not safe (Mazzini et al., 1995).
