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Dietary protein intake of Dutch people

with Neuromuscular Diseases

An observational study of patients with Neuromuscular diseases.

Authors: Josje Pouwer and Laure Snijders Company: HvA Lectorate Weight Management Institute: Amsterdam University of Applied Sciences Department: Nutrition and Dietetics

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Dietary protein intake of Dutch people

with Neuromuscular Diseases

An observational study of patients with Neuromuscular diseases.

Author: Josje Pouwer

Student number: 500744227

Email address: josje_pouwer@hotmail.com Autor: Laure Snijders

Student number: 500731361

Email address: lauresnijders@gmail.com

Project number: 2019102 Supervisor: Michael Tieland

Company: HvA Lectorate Weight Management Institute: Amsterdam University of Applied Sciences

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Acknowledgments

This thesis is written within our graduation project of the education Nutrition and Dietetics. We were working on the assignment of the Innovative Health Practice of the Amsterdam University of Applied Sciences, Faculty of Sports and Nutrition. Our research focused on people with a neuromuscular disease (NMD) and their nutritional intake, with a focus on proteins.

The purpose of this research is the missing guidelines for patients with an NM disease. Despite many promising studies in other populations, there is still little information about the added value of protein in a diet of patients with NMD. This hampers dieticians in the optimal treatment of patients with NMD. Research is clearly necessary and vitally important. For a dietary protein intake advice of a patient with NMD, research is needed on the habitual dietary protein intake. Therefore, we aim to examine the total protein intake, sources of protein as well as the distribution of protein sources over the day of Dutch patients with an NM disease. There are certain people we would like to thank for helping us during our research. Starting with Michael Tieland, practice supervisor from the Innovative Health Practice of the Amsterdam University of Applied Sciences, for giving us the chance to work on this interesting study and guiding us throughout the project. He was also our school supervisor from the Amsterdam University of Applied Sciences and gave us some short lectures about writing an introduction and discussion. These lectures helped us throughout the whole study process. Next we are grateful to the guidance of Jantine van den Helder, a researcher at the University of Amsterdam Applied Sciences. She helped us using and understanding the research methods.

Our thanks go to the associate dieticians of the National Network to cooperate with us. We have sent them a lot of emails and called some of them for information about the collection of the nutritional diaries. Furthermore, we would like to thank the patients with NMD who signed the informed consents for using their nutritional diaries and their personal data. Last, but certainly not least, we would like to thank everybody who helped us with their critical view and the thinking along with us.

Amsterdam, 6 Januari 2019

Josje Pouwer Laure Snijders

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Abstract

Introduction: Neuromuscular disease is a progressive muscle disease, whereby

patients suffer from a decrease in muscle mass. Although muscle diseases can hardly be cured, treatment is possible with adequate protein intake. However, there is no general nutritional guideline available for patients with NMD. Therefore, we first had to evaluate the daily dietary protein intake, distribution during the day and consumed protein sources of patients with NMD.

Method: Dietary intake of 105 patients with an NMD - 35 men and 70 women with a

minimum age of 18 years - was evaluated using 3-day nutritional diary. This observational study included the 15 most common types of NMD in the Netherlands. The study population was divided into three groups; ALS, PSMA, HSP, and PLS group (19 patients), MD group (65 patients), and remainder NM diseases (21 patients).

Results: Daily dietary protein intake averaged 71.5 +/- 17.9 g/day, and 1.07

g/kg-bw/day in the total study population. The average recommendation of the total study population was 0.84 g/kg-bw/day. The ALS, PSMA, HSP, and PLS group had an average of 79.5 g/day, MD group 71.2 g/day, and remainders 65.2 g/day and accounted for 17.3%, 16.1%, and 15.7% of their total energy intake. Dietary protein intake averaged during breakfast 12.3 g, during lunch 18.5 g, during dinner 30.7 g, and 11 g divided over snack times. The majority of consumed protein is from animal sources (63.5%). Dominated protein sources during the day are meat, cheese, and bread.

Conclusion: The average daily dietary protein intake of patients with an NMD was 71.5

g/day and represents 1.07 g/kg-bw/day. The intake is above the current recommendation of patients with NMD, but the requirement could be higher for an optimal protein muscle synthesis. Therefore, expanding the protein intake - with high-quality protein - during breakfast could improve the daily protein intake.

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Table of contents

1. Introduction ... 5

2. Methods ... 7

2.1 Data collection ... 7

2.2 Calculation of dietary protein intake ... 7

2.3 Statistical analysis ... 8

3. Results ... 9

3.1 Characteristics of the participants ... 9

3.2 Dietary intake during the day ... 9

4. Discussion ... 15 4.1 External analysis ... 15 4.1.1 Protein metabolism ... 17 4.2 Internal analysis ... 19 4.2.2 Body composition ... 20 4.3 Conclusion ... 21 5. Recommendation ... 23

5.1 Nutrition and dietetics ... 23

5.2 Follow-up studies ... 23

References ... 25

Appendix 1. Information letter and informed consent... 27

Appendix 2. Nutritional diary ... 31

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1. Introduction

Neuromuscular disease (NMD), also known as muscular disease, is a hereditary or non-hereditary disease that affects the musculoskeletal system. NMDs are rare but progressive and contain a lot of variations regarding the cause, symptoms, and progression of the disease (1). Relatively common NMDs are Amyotrophic Lateral Sclerosis (ALS), Myotonic Dystrophy (MD), and Duchenne Muscular Dystrophy (DMD). The number of people suffering from NMD is estimated to be around 200.000 in the Netherlands alone (2). Common symptoms are pain, weakness, mobility reduction and problems with communication, swallowing and personal care. Furthermore, a reduction in muscle mass and a deteriorated function of muscles occurs. The impact of NMD affects the quality of life, along with the medical problems, and early death (3).

One of the most important causes of reduced quality of lifeis the decrease in muscle mass and physical functioning. The decrease in muscle mass and physical functioning is caused by the NMD, an inadequate nutritional intake and low physical activity (4). Although muscle diseases can hardly be cured, treatment is possible. Treatment of muscle diseases, such as mobilizing and proper nutrition could help to inhibit muscle mass loss and possibly improve physical functioning. Research among frail elderly has shown positive effects of mobility and optimal protein intake on muscle mass and physical functioning (5). Physiologically, dietary protein stimulates muscle protein synthesis and inhibits muscle protein breakdown. Resulting in less decrease and sometimes even the preservation of muscle mass. Dietary protein also improves physical functioning (6-9). Therefore, the necessity of focusing on maintaining nutritional status, becomes more important for patients with an NMD.

The current recommended dietary allowance of a Dutch adult is 0.8 grams of protein per kilogram of body weight per day (g/kg-bw/day) (10). A lower intake of dietary protein could cause loss of muscle mass. Previously reported results showed a consumption of 1.2 g/kg-bw/day dietary protein intake could inhibit the loss of substantially lean body mass for older adults. Participants with an intake of 1.2 g/kg-bw/day lost significantly less lean mass (43% less) and appendicular lean mass (39% less) over the 3-y follow-up than those with an average intake of 0.8 g/kg-bw/day. (11). Older adults are also suffering the loss of functional muscle mass. Therefore, we expect that a higher intake of protein could have positive effects on the muscle mass in adults with NMD. An optimal protein intake, preferably in combination with physical activity, will inhibit the breakdown, stimulate muscle protein synthesis and could possibly increase muscle mass and physical function, which may increase the quality of life (12,13).

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6 Furthermore, to the level of protein intake, protein sources and the timing of their intake are considered important. Even though all protein sources have the capacity to stimulate muscle protein synthesis, synthetic rates can vary substantially following the consumption of different protein sources (6,14). Although both animal and plant-based proteins can provide the required essential amino acids, animal proteins have higher digestibility and absorption than plant-based proteins. Besides, a skewed protein intake pattern is less potent to stimulate muscle protein synthesis as opposed to protein intake which is evenly distributed. Insight in the intake and timing of consuming could be valuable information to locate protein intake inadequacies (15).

Despite many promising studies in other populations, there is still little information about the added value of an increased protein intake in a diet of patients with NMD. The few published articles describe a general low protein intake. In recently published international treatment guidelines for ALS, DMD, and SMA, it is stated that the diet must be complete with sufficient protein (16-18). However, nutritional guidelines, about the specific necessary protein intake for all NMDs, are still not available. This hampers dietetics in the optimal treatment of patients with NMD. Research is clearly needed and vitally important.

Therefore, we aim to examine the total protein intake, the sources of protein, as well as the distribution of protein over the day of Dutch patients with an NMD.

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2. Methods

2.1 Data collection

We used data of 105 patients - 35 men and 70 women with a minimum age of 18 years -with one of the 15 most common NMD. The included NMD’s are ALS, PMA, HSP and PLS, Arthrogryposis Multiplex Congenita (AMC), Congenital and metabolic muscular dystrophies, Hereditary polyneuropathies, Facioscapulohumeral Muscular Dystrophy (FSHD), Guillain Barré Syndrome (GBS)/CIDP, Myotonic Dystrophy (MD), Myasthenia, Myositis, and Spinal Muscular Atrophy (SMA).

The collected data during the research contains 3-day nutritional diaries of patients with an NM disease. Some of the data has been collected during previous research, concerning the nutritional intake of patients with MD. This data was collected in the period between 2013 and 2016. Remaining data is collected between the beginning of September and the end of November 2018. The data is collected by trained dieticians from Radboud UMC, Roessingh Revalidatie, Rijndam Revalidatie and, Revant Breda and Goes. The dieticians asked their own patients for the participation of this research. Through a written informed consent, patients gave their permission (appendix 1). The 3-day nutritional diaries are collected of minimal two weekdays and one weekend day (appendix 2). The nutritional diaries were digitally produced and received by email. The ones written down on paper have been picked up, sent by post or scanned and have been sent by email. Patients were excluded when the NM disease was not among the 15 most common and when nutritional diaries were illegible, incomplete for a minimum of 3 days or patients were under 18 years old. In total, we excluded 8 nutritional diaries.

Besides the nutritional information, personal- and medical data is collected of the patients. The personal information includes gender, age, body weight (kg), height (m) and BMI (kg/m²). The medical information contains the specific NMD and mobility of the patient. Both information - personal and medical - is measured by dieticians.

2.2 Calculation of dietary protein intake

Patients completed the 3-day nutritional diaries after the given instructions regarding documentation of the nutritional diary by trained dieticians. Instructions concern food sources, estimation of the portion size using household measures and the timing of the intake. The collected data is cross-checked by dieticians and students for completeness and obtained additional information to optimize dietary intake assessment.

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8 The collected data from the dietary intake were coded in a Microsoft Excel file. The coded information contains food intake, amount and mealtime. Portion sizes were documented in household measures with the Dutch food measurements codebook (19). Energy intake and protein intake is calculated by Nevotabel (20). The coded data collection was calculated using an SPSS Syntax. The Syntax calculates the energy and protein intake (g/day) per patient and mealtime, i.e., breakfast, lunch, dinner, and between meals (snacks). Total protein intake from vegetable and animal was also calculated by using the SPSS Syntax. These determinants were calculated to provide a total view of the protein intake - both source as mealtime.

Daily dietary protein intake was compared with the standard recommendation for healthy adults with a BMI > 18,5 kg/m², of 0.8 g/kg-bw/day. In the case of underweight, patients with a BMI <= 18,5 kg/m², the intake was compared to the recommendation of 1.2 g/kg-bw/day (21,22).

Besides the compared daily dietary protein intake with the standard recommendation, we divided the study population into several groups to compare the protein intake. Groups we compare were patients with MD, patients with ALS, PMA, HSP or PLS, and patients with the remainder NM diseases. This division was based on the number of obtained nutritional diaries per disease.

2.3 Statistical analysis

Data analyses were performed using the IBM SPSS Statistical software package (version 24). Descriptive statistics were used to present the nutrient intake, with a focus on proteins. Descriptive analyses were used to derive the mean and standard deviations of baseline characteristics. Baseline characteristics of the population were presented on the basis of their age, gender, and BMI-score. The descriptive analyses provide the protein intake in grams per kg per day per type of NMD. One-way ANOVA was used to compare differences in protein intake between different types of NMDs. In case of a significant difference (p < 0.05) in the intake, Bonferroni’s post hoc test was used to locate these differences.

The one sample T-test was used to compare the daily dietary intake with the average recommended intake. The recommended intake of 0.8 g/kg-bw/day or in case of underweight with 1.2 g/kg-bw/day of patients with an NMD.

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3. Results

3.1 Characteristics of the participants

The study includes the 15 most common types of NMD. The majority of the study population were MD patients (65 patients) and patients with ALS, PSMA, HSP or PLS (19 patients). Remaining 21 patients with an NM disease were merged in one group. Descriptive characteristics of the study population are presented in Table 1. The study population included 70 women (66.7%) and 35 men (33.3%). The mean age was 53.1 +/- 14.7 years. The mean BMI-score was 24.4 +/- 4.9 kg/m².

In the ALS, PSMA, HSP and PLS group, MD group and the remainders, was the majority of women (53-71%). The BMI-score was almost equal in all population groups (24.4 - 24.5 kg/m²).

3.2 Dietary intake during the day

The nutritional intake of the 105 patients with an NMD is shown in Table 1. The average intake was 1759 kcal +/- 446 kcal, with a minimum energy intake of 730 kcal and maximum intake of 2842 kcal. The dietary protein intake of patients with NMD showed a mean intake of 71.5 +/- 17.9 g/day. Dietary protein intake, expressed in bw/day, is 1.07 +/- 0.37 g/kg-bw/day in patients with an NMD. The ALS, PSMA, HSP and PLS group had with 79.6 g/day the highest intake of dietary protein of the three groups. Dietary protein intake in g/day was significantly different between the three groups (P=0.04). Dietary protein intake in g/kg/bw/day was not significantly different between the three groups (P=0.26). This could be caused by the highest intake of dietary preparations (Figure 1). In the ALS, PSMA, HSP and PLS group the intake of dietary preparations ensured 8% of the average total daily protein intake. In the MD group and the remainders was the intake of dietary preparations 3% and 2%.

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Table 1. Study population characteristics and nutritional intake.

Values are means +- SD; BMI: body mass index; en%: energy percentage; g/kg-bw/day: gram per kilogram bodyweight per day; ALS, PSMA, HSP and PLS: Amyotrophic Lateral Sclerosis, Progressive Spinal Muscular Atrophy, Hereditary Spastic Paraplegia and Primary Lateral Sclerosis; MD: Myotonic Dystrophy; Remainders: patients with Arthrogryposis Multiplex Congenita (AMC), Congenital and metabolic muscular dystrophies, Hereditary polyneuropathies, Facioscapulohumeral Muscular Dystrophy (FSHD), Guillain Barré Syndrome (GBS)/CIDP, Myasthenia, Myositis, or Spinal Muscular Atrophy (SMA).

Study population Characteristics

Mean SD Mean SD Mean SD Mean SD

Age (years) 53,1 14,7 62,3 11,1 52,9 13,5 45,2 17,9 Women 51,8 14 61,3 9,9 51,9 13,5 45 15,9 Men 55,7 15,9 63,4 12,8 55,2 13,1 45,7 24,1 Seks, women % 66,7 - 53 - 69 - 71 -Weight (kg) 71,1 17,4 73,3 14,1 70,5 18,5 70,8 17 Women 66,3 15,4 67,2 14,8 65,3 15 68,3 17,6 Men 80,7 17,3 80,2 10,1 81,9 20,7 77,1 14,8 Height (m) 1,7 0,09 1,73 0,08 1,69 0,09 1,71 0,09 Women 1,66 0,07 1,68 0,05 1,65 0,07 1,67 0,08 Men 1,79 0,08 1,77 0,07 1,79 0,08 1,81 0,04 BMI (kg/m²) 24,4 4,9 24,5 3,9 24,4 4,9 24,4 5,7 Women 24 5,1 23,6 4,8 23,9 4,8 24,7 6,1 Men 25,2 4,5 25,5 2,6 25,5 5,2 23,7 4,9

Nutitional intake Mean SD Mean SD Mean SD Mean SD

Energy intake (kcal) 1759 446 1841 519 1767 392 1660 530

Women 1730 436 1844 643 1747 356 1602 497

Men 1817 465 1838 377 1811 471 1807 630

Protein intake (g/day) 71,5 17,9 79,6 18,7 71,2 15,3 65,2 22,3

Women 70,7 18,8 82,3 22,1 70,8 15,4 62,8 22,8

Men 73,1 15,9 76,5 14,7 72,2 15,3 71,4 21,4

Protein intake (en%) 16,3 - 17,3 - 16,1 - 15,7

-Women 16,3 - 17,9 - 16,2 - 15,7 -Men 16,1 - 16,6 - 15,9 - 15,8 -Protein (g/kg-bw/day) 1,07 0,37 1,14 0,42 1,08 0,37 0,96 0,34 Women 1,12 0,39 1,29 0,49 1,14 0,37 0,95 0,3 Men 0,96 0,32 0,97 0,23 0,94 0,34 0,98 0,44 Plant protein (g) 26,1 8,4 24,6 10,4 27,5 7,9 22,9 7,2 Women 25,2 8,5 23,4 14 26,8 7,3 21,7 6,9 Men 27,8 7,9 26 4,2 29,1 9,2 26,1 7,9 Plant protein (%) 36,5 - 30,9 - 38,6 - 35,1 -Women 35,6 - 28,4 - 37,9 - 34,6 -Men 38 - 34 - 40,3 - 36,6 -Animal protein (g) 45,4 15,7 54,8 17,2 43,7 13,5 42,3 18,4 Women 45,4 16,4 58,7 18,6 52,9 13,5 41 19,6 Men 45,4 14,5 50,5 15,4 43,2 13,8 45,3 16,5 Animal protein (%) 63,5 - 68,8 - 61,4 - 64,9 -Women 64,2 - 71,3 - 74,7 - 65,3 -Men 62,1 - 66 - 59,8 - 63,4 -Remainders (n = 21) MD (n = 65)

ALS, PSMA, HSP and PLS (n = 19) Total (n = 105)

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Figure 1. Contribution of plant-based, animal and composite protein sources to total daily protein intake

in ALS, PSMA, HSP, and PLS (A), MD (B) and remainders (C).

Bread 17%

Nuts, seeds and snacks 5% Dietary preparations

3% Pastry, cake & buscuit

4% Cereal products 3% Vegetables 1%

Other vegetable sources 9% Composite sources 2% Eggs 3% Fish 3% Milk and milk products

6% Cheese

18% Meat, meat products and

poultry 26% (B) MD: PROTEIN SOURCES PLANT-BASED ANIMAL COMPOSITE Bread 21%

Nuts, seeds and snacks 6% Dietary preparations

2% Pastry, cake & buscuit

5% Cereal products 2% Vegetables 1% Other vegetable sources

8% Composite sources 4% Eggs 3% Fish 1% Milk and milk products

8% Cheese

19%

Meat, meat products and poultry

20%

(C) REMAINDERS: PROTEIN SOURCES

PLANT-BASED

ANIMAL

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12 Furthermore, 29.5% of the patients with a healthy BMI-score - with a protein recommendation of 0.8 g/kg-bw/day - did not achieve the recommendation. The protein recommendation of 1.2 g/kg-bw/day, was not achieved by 10% in the group of patients with a BMI-score below 18.5 kg/m² (Figure 2). However, the average protein intake of 1.07 g/kg-bw/day was significantly(p=0.000)higher than the average recommended intake of 0.84 g/kg-bw/day in the study population. Hereby, the average protein intake per kilogram body weight was compared with the average recommendation. The recommended intake of 0.8 g/kg-bw/day for people with a healthy BMI, and the recommendation of 1.2 g/kg-g/kg-bw/day for people with a BMI-score under 18.5 kg/m².

The distribution of the daily dietary protein intake is presented in Figure 3. Mealtimes were divided in breakfast, lunch, dinner and snack times (in between meals). Remarkable is the constant higher intake of proteins in the ALS, PSMA, HSP and PLS group. The average intake during breakfast was 16.9% (12.3 g protein) of the total protein intake, during lunch 25.5% (18.5 g protein) and during dinner 42.2% (30.7 g protein). Remaining protein intake of 11 g was divided over snack times.

During breakfast, the intake of protein was the smallest and the protein sources were almost equally divided, 48.8% was from plant-based protein and 51.2% from animal protein. During lunch, the protein intake was a little bit more. The protein intake during lunch contained a dividing in protein sources of 41.1% plant-based protein and 58.9% animal protein. During dinner, the consumption of protein was the highest, mainly from animal sources. Total protein intake of dinner contains 74.6% animal proteins, with meat as a dominant source, and 25.4% was plant-based protein.

The protein intake during the day was mainly from animal proteins (63.5%). The biggest sources were meat, cheese and bread products (Figure 1).

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Figure 2. The distribution of protein intake per BMI group. The group with a BMI <= 18.5 kg/m² has a

recommendation of 1.2 g/kg-bw/day. The group with a BMI > 18.5 kg/m² has a recommendation of 0.8 g/kg-bw/day. Based on the recommendations for the average Dutch people and underweight people (BMI <= 18.5 kg/m²) (21,22).

Figure 3. Daily protein intake in grams distributed per mealtime in ALS, PSMA, HSP and PLS group,

MD group and remainders. Values are an average intake per mealtime +/- SD. Snack 1 represents protein intake between breakfast and lunch. Snack 2 represents protein intake between lunch and dinner, and snack 3 represents protein intake after dinner.

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4. Discussion

This study provides information on daily dietary protein intake, the distribution of protein intake during the day and the protein sources. The NMD population showed an average intake of 71.5 g/day, accounting for 16.3% of the total daily energy intake. Per kg/bw was the daily protein intake 1.07 g. The main source of protein during the main meals was from animal sources. The distribution of plant-based versus animal was at breakfast 49-51%, at lunch 41-59%, and at dinner 25-75%. 29.5% of the patients with a protein recommendation of 0.8 g/kg-bw/day did not achieve their recommendation. 10% of the patients with underweight and a protein recommendation of 1.2 g/kg-bw/day did not achieve their recommendation. The difference between the average recommended protein intake of 0.84 g/kg-bw/day and the actual average protein intake of 1.07 g/kg-bw/day was significantly (P=0.000) higher. The distribution of the average protein intake was at breakfast 12.3 g, at lunch 18.5 g, and at dinner 30.7 g.

4.1 External analysis

Even though muscle diseases can hardly be cured, treatment is possible. It is well-known that a decrease in muscle mass and physical functioning is caused by an inadequate nutritional intake and low physical activity. Research among athletes, fragile elderly, patients with diabetes and cancer has shown positive effects of physical effort and optimal protein intake of 1.2-1.5 g/kg-bw/day on muscle mass and physical functioning (5,6). The average intake in the NMD population was 1.07 g/kg-bw/day. In this study, the ALS, PSMA, HSP and PLS group had the average daily protein intake of 1.14 g/kg-bw/day. Compared with the MD group and the remainders, the ALS, PSMA, HSP and PLS group had a higher protein intake. This could be deduced from a higher consumption of dietary preparations. The ALS, PSMA, HSP and PLS group consumed 8% of their daily dietary protein intake from dietary preparations. Where the MD group and the remainders consumed 3% and 2% of their daily dietary protein intake from dietary preparations. Dietary preparations are often used when a (sick) person cannot eat or drink sufficiently. This can be caused by swallowing problems, stomach/bowel problems and/or reduced appetite (23-25). These problems can occur in all the NMD groups, but it could be more common, or many participants were in the progressive phase in the ALS, PSMA, HSP and PLS group. To achieve a higher protein intake - and expanding the current diet is not possible because of the eating problems - dietary preparations could be a solution. A proper dietary protein intake and physical activity stimulates muscle protein synthesis and could inhibit muscle protein breakdown. Even though physical functioning does not always apply to the possibilities for patients with NMD, a treatment such as mobilizing in combination with a proper nutrition diet could contribute to

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16 inhibiting the decrease of muscle mass. If the dietary protein intake and physical functioning not improve, the quality of life will presumably not improve for patients with an NMD.

Today there are guidelines for the protein intake of several population groups, except for the entire group of patients with an NMD. The protein requirement for healthy people is 0.8 bw/day and for people with underweight is the recommendation of proteins 1.2-1.5 g/kg-bw/day (21,22). Although, there are guidelines for patients with ALS, Duchenne, and patients with swallow disorders due to a neurological disorder. The daily dietary protein intake for patients with ALS is set on 1.0-1.2 g/kg-bw/day and for optimizing nutritional status is the intake 1.2-1.5 g/kg-bw/day (23). Even though muscle gaining is not always possible, an increased protein intake is advised to inhibit the decrease in muscle mass and weight. For patients with Duchenne, the recommended daily dietary intake of proteins is 10-15% of the daily energy intake (24). This is a similar recommendation of daily dietary protein intake for people without a disease. A similar protein intake of 0.8-1.0 g/kg-bw/day applies also to patients with a swallowing disorder (25). So, there are several guidelines, with different recommendations for the daily dietary protein intake for patients with an NMD. In some cases, increased, but sometimes just the same recommendation as for healthy people. This is confusing and it would not be if there was just one guideline for the whole NMD group.

The results of this study show an average protein intake of 1.07 g/kg-bw/day. The average protein intake of patients with NMD is higher in comparison with the current recommended daily dietary protein intake of people without a disease. However, 30% of the patients with NMD - with a BMI-score >18,5 kg/m² - did not achieve the recommendation of 0.8 g/kg-bw/day. For the group of patients with a BMI-score <=18.5 kg/m² and the recommendation of 1.2 g/kg-bw/day, 10% of the patients did not achieve the recommendation. Also, this study showed an average protein intake of 16.3% of the total energy per day. In the ALS, PSMA, HSP and PLS group contributed the average intake for 17.3%, in the MD group 16.1% and in the remainders 15.7% of the total energy intake. Averaged energy intake from proteins of the average Dutch population is 15-16% in adults. So, the average intake of NMD patients did not deviate with the average Dutch population. This means the patients with an NMD do not have an adjusted diet for their disease yet. An adjusted diet is desired for a higher protein intake and a more optimal protein synthesis (11,12). So, we need a guideline with a higher recommendation for the daily dietary protein intake for patients with NMD to increase their protein intake.

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4.1.1 Protein metabolism

Although the protein intake for patients with NMD seems high enough in comparison to the current recommended daily dietary protein intake for patients with NMD (1.07-0.84 g/kg-bw/day), the protein metabolism is changing during sickness and aging (26,27). During sickness, muscle protein synthesis rates are lower with the same stimuli. This is called anabolic resistance. Anabolic resistance means a reduced capacity to form muscle protein with a certain amount of protein intake. It is caused by different things. One of them is caused by the increase of splanchnic sequestration, whereby amino acids following feeding are more absorbed by intestines and liver. This indicates a decrease of the amino acid availability for muscles. Another factor for the anabolic resistance is the insulin resistance, which limits amino acids uptake into muscles and hinders the supply of muscle protein. The third factor is the reduced response to amino acids with anabolic qualities, like leucine. Altogether this indicates that an increase in protein intake is necessary for optimal muscle protein synthesis (28).

Besides the changed metabolism of proteins in patients with NMD, there are factors we need to evaluate for an improvement in protein intake and synthesis. Factors we need to evaluate, are the sources of the daily dietary protein intake and the timing of meals. This could be valuable information to allocate protein intake inadequacies (15). Even though all protein sources have the capacity to stimulate muscle protein synthesis, synthetic rates can vary substantially following the consumption of different protein sources. Both animal and plant-based proteins could provide the required essential amino acids, but animal proteins have in general a higher rate of the amino acids and are likely attributed to the faster digestion and absorption. Besides animal proteins, proteins in products of soy contain also all of the essential amino acids (29). Mainly leucine, an essential amino acid, seems to be a powerful stimulator for muscle protein synthesis. Leucine plays a role in stimulation, translation, initiation and muscle gaining (30). Therefore, the intake of the high quality of protein is important to stimulate muscle protein synthesis which ensures muscle retention. In this study, we see that the biggest protein source is from animal products. The ratio between animal protein and plant-based proteins during the day is 58.9% versus 41.1%. During the day, the ratio plant-based and animal protein is quite balanced, except during dinner. During dinner, the animal protein source is higher, the intake of protein is for 75% from an animal source. So, the average intake of essential amino acids is at a good rate, because of the current balance between animal and plant-based proteins. But, if a patient with NMD is vegetarian or vegan, supplementation of leucine could be considered.

Furthermore, the mealtime is considered important. Approximately 10 grams of essential amino acids per meal time are required in adults for a good anabolic response (28). To get this amount of essential amino acids, 20 grams of high-quality protein -mainly animal

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18 proteins, but also soy proteins- per meal are needed, or 25-30 grams of average dietary protein (31). In our study, we observed an unequal protein intake distribution per day. Averaged total of protein intake was 71.5 g/day in patients with NMD. The groups in this study had an almost similar distribution of dietary protein intake during the day. Mainly proteins were consumed at dinner, with an average of 30.8 g protein. The smallest amount of proteins was consumed during breakfast, with an average of 12.3 g protein. During lunchtime, the protein intake had an average of 18.5 g. The absolute amount of protein at breakfast and lunch was inadequate to maximally stimulate muscle protein synthesis. Therefore, we assume, to increase the protein intake mainly during breakfast. An increased protein intake during lunchtime could also be valuable for a section of patients with NMD. The increased protein intake during bread meals, preferably with animal sources or soy, could stimulate the muscle protein synthesis for possible muscle retention.

In comparison with the research of protein intake of older adults, there were a few differences (5). The differences were in the distribution of protein intake per mealtime. Older people consumed more often hot meals during lunchtime, in comparison with patients with NMD. However, a similarity of both study populations was that bread meals contain less protein than hot meals. This study also reported that older adults had lost less muscle mass when consuming 1.2 g/kg-bw/day of dietary protein, compared with 0.8 g/kg-bw/day. We assume this applies to more populations. To achieve a daily dietary protein intake of 1.2 g/kg-bw/day the daily intake has to increase with 9.6 g protein, assumed to the average weight of 71.5 kg in the study population. For a more optimal protein muscle synthesis, we recommend an increased protein intake during bread meals. Breakfast is often a smaller meal and contains fewer proteins. Therefore, we suggest a similar advice as applied to older adults in previous research, to increase the protein intake during breakfast to 20 g with for example a dairy product. This dairy product could be from soy- or animal products, to increase the intake of essential amino acids for a more optimal protein synthesis.

The daily protein distribution per meal time was almost equal between the ALS, PSMA, HSP and PLS group, the MD group, and remainders. However, the difference was significantly (p=0.038) between the total intake of daily protein intake per group. The daily dietary protein intake in the ALS, PSMA, HSP and PLS group was 79.6 g, the MD group had an average intake of 71.1 g, and the remainders had an average of 62.8 g. Although, when compared the protein intake per kilogram body weight, the difference was not significantly (p=0.262) in this study. In groups with a higher intake of protein, patients had a higher body weight. So, there was a significant difference in protein intake in grams per day between the three groups, but not per g/kg-bw/day.

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4.2 Internal analysis

In this study, we studied the daily dietary protein intake of patients with an NMD. A strong point of this study is the collection of nutritional diaries of patients with NMD. Nutritional diaries were collected by a well-characterized selected NMD population. During the research, the 15 most common types of NMD were selected to ensure a clear demarcation for the population. The selection eliminates rare types of NMD’s and showed an average and more representative picture. Also, the patients with an NM disease were from several facility institutions across the country. Centers like hospitals, rehabilitation centers, and dieticians’ practices. Therefore, the study population is a more representative selection for Dutch patients with NMD. For a high response of the research, we had a recognizable and reliable sender. The reliable sender was the dietician of the patients. The dieticians made several phone calls to enlarge the number of responses. We also used a well-formed layout for the nutritional diary and the research had a good relevance for patients with NMD. The collection of 105 nutritional diaries was the maximum achievable quantity of the daily dietary protein intake of patients with NMD, collected by the dieticians. Compared with other studies, it is a large amount of nutritional assessments. Other previous studies report a much lower amount of nutritional assessment, between 22 and 40 patients with NMD (32,33). So, our study ensured a good demarcation of the study population of patients with NMD.

Although a large number of nutritional diaries, some types of NMD are less common in our study. Nevertheless, we suspect that there would be no big differences in nutritional intake in the less common types of NMD. Whereby this research, the first insights shows of the protein intake of patients with NMD. The study population was divided into groups, whose protein intake was compared and indicated eventual differences between the groups. The collected data could be used for interventions and help for further research of basic nutritional care.

Another strength of the collected data is the used method. The used method of data collection is through 3-days nutritional diaries. The 3-day nutritional diary is a valid measurement because it reflects the daily dietary nutritional intake. Through the use of minimal two weekdays and one weekend day, it is a relatively more valid method than a 24 hour-recall method. A 3-day nutritional diary, in comparison with a 7-day nutritional diary, provides less accurate information. However, the number of participants would probably be less through the larger time investment. A 3-day nutritional diary was the best option for our study. A written survey, with an accompanying letter, was sent to a part of the Dutch NMD population. A disadvantage of a written survey is the mistakes a respondent could make, therefore is a good layout necessary. We used a nutritional diary with an instruction form to exclude this disadvantage (34).

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20 Besides the valid method of data collecting, the nutritional diaries were collected by trained dieticians. They are experienced in conducting nutritional diaries. Although all patients received the same nutritional diary to fill out, with the same instruction form, the diaries were less accurate as required. In some diaries the type of yoghurt or bread, or the amount of the product, was missing. For this reason, we used the four eyes principle. The data were registered separately and completely checked by someone else. The check ensured fewer mistakes and increased the reliability of the research. The reliability of this study is also increased by using equal household measurements. Hereby is a continuation of the data input guaranteed (34).

4.2.2 Body composition

Patients with an NMD are faced with changes in their body composition. They have progressive weakness and wasting of skeletal muscle, reduced fat-free mass, and increased fat mass compared to healthy people (35). For the estimation of the protein requirements of the patients, we separate the total research population into two groups. The division was based on the body mass index (BMI). BMI describes the ratio between body- height and weight, in kilograms per meter squared (kg/m²). The BMI-score <=18.5 kg/m² for underweight and >18.5 kg/m² for a healthy weight, provides a different protein requirement of both groups. The BMI method is easy to apply and is a cheap method but does not define the body composition and any process of muscle mass loss of patients with NMD. Nevertheless, the use of the BMI method was beside a cheap method, also the least time-consuming method and for this research the most realistic method to distinguish patients with NMD (22).

For a more accurate view of the disease progression and changes in body composition, a further research has to involve a method like skinfold measurements or the Dual-energy x-ray absorptiometry (DEXA). These methods provide the fat-free mass and fat mass of the body and will provide more useful information about the progression of the disease. Another safe and useful, but also more intensive method to analyze the progression of NMD is to perform a muscle biopsy. Muscle biopsy is not common practice, but it could give more information about the muscle mass, by researching the muscle tissue structure (36).

These methods will provide the progression in body composition and will be interesting for a follow-up research to make eventually a nutritional guideline for patients with an NMD. The nutritional guideline will contain a recommendation of daily protein intake. With this study, we could only suggest a higher daily protein intake. Which could help to inhibit the decrease of muscle mass.

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4.3 Conclusion

In summary, patients with an NMD are dealing with a progressive muscle disease. Even though muscle gaining is hardly possible, an improved protein intake could help to inhibit the muscle breakdown or even to retain the muscle mass. Therefore, an equal distribution of proteins during the day is important for an optimal protein synthesis. The current protein intake of patients with an NM disease is 1.07 g/kg-bw/day, which is above the recommended daily protein intake of 0.8 g/kg-bw/day. However, 1.07 g/kg-bw/day is below the requirement of 1.2 g/kg-bw/day protein intake for muscle retention in older adults and patients with a disease. Therefore, we require also for patients with an NM disease, an increased protein intake, and a more equal distribution during the day. An increased intake and more equal distribution of proteins during the day could be achieved by increasing the protein intake during breakfast to a minimum of 20 g high-quality proteins.

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5. Recommendation

5.1 Nutrition and dietetics

For patients with an NMD, it is important to pursue a proper diet, with an adequate daily protein intake. An adequate protein intake could help to inhibit the muscle protein breakdown. A dietician has the main role to monitor the protein intake of patients with an NMD and, if necessary, stimulate the protein intake to 1.2 g/kg-bw/day. To achieve the daily protein intake of 1.2 g/kg-bw/day, the intake has to increase with 9.6 g protein, assumed to the average weight of 71.5 kg and the average intake of the study population. An increased protein intake during breakfast could be achieved by expanding breakfast with for example a dairy product from soy- or animal source. The increased protein consumption will stimulate protein synthesis.

If the current diet of patients with an NMD could not be increased by proteins from food, dietary preparations could be considered. Dietary preparations like nutritional drinks with additional proteins. In this study, the ALS, PSMA, HSP, and PLS group had a higher average protein intake. The higher protein intake could be caused by the consumption of dietary preparations. For an improvement in the protein distribution during the day, consumption of dietary preparations in the morning could be an option.

5.2 Follow-up studies

The collected data of this observational study could be used for care interventions. These interventions could help improve the quality of life for patients with NMD. For example, an intervention program could be developed in combination with a physical therapist and a dietician. The physical therapist could help improve the mobility of patients with NMD, which will help in daily activities. The dietician will help with the adequate nutritional intake for a more optimal protein synthesis. This multidisciplinary intervention will show the importance of an adequate nutritional intake in combination with physical activity to maintain the muscle mass. This study could help with developing the nutritional part, but a study in which way the physical part could help is needed.

The recommended intake of dietary protein of the average Dutch is 0.8 g/kg-bw/day. However, the protein metabolism changes during sickness and aging. For this reason, a higher protein intake is desirable. For older adults, the recommended daily dietary protein intake is already higher. The recommended protein intake is 1.2-1.5 g/kg-bw/day for older adults and patients during sickness. Further research has to determine if a similar recommendation is required for patients with an NMD. To determine the daily requirement of proteins for patients with an NMD, the research has to contain various measurement moments. For example, it would be interesting to compare the differences in body composition in two study groups. A

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24 study group with a protein intake of 1.2 g/kg-bw/day and control group with a protein intake of 0.8 g/kg-bw/day. Besides the adequate total consumption of proteins, the distribution during the day is important for protein synthesis. The protein synthesis is optimal by an equal distribution of protein intake during three main meals. Each meal has to contain a minimum of 20 g high-quality protein for optimal synthesis. Thereby, various measurement moments with a muscle biopsy or DXA-scan will determine the change in body composition of patients with NMD. For the changes in body composition, it is important to consider the stage of progression of the disease. The stage of progression affects the mobility of patients with NMD. This research has to prove if there is a possibility to maintain muscle mass or decrease the loss of muscle mass. If the decrease in muscle mass would be less with an intake of 1.2 g/kg-bw/day, there will be more proof to change the current guideline for patients with an NMD.

Another interesting follow-up research would contain the daily dietary protein intake of children with an NMD. NMDs often occur at a young age. Therefore, we assume it could be helpful to start as soon as possible with a proper diet, to limit the loss of muscle mass at a young age. So, another observational study to the nutritional intake of children with an NMD could provide information to create a guideline for patients under the age of 18.

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(12) Beelen M, Tieland M, Gijsen AP, Vandereyt H, Kies AK, Kuipers H, et al. Coingestion of carbohydrate and protein hydrolysate stimulates muscle protein synthesis during

exercise in young men, with no further increase during subsequent overnight recovery. Journal of Nutrition 2008 Jan 1,;138(11):2198-204.

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(14) Gorissen SHM, Rémond D, van Loon LJC. The muscle protein synthetic response to food ingestion. Meat Science 2015 Nov;109:96-100.

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(16) Lopes da Silva, H F, de Araújo Brito, A N, Silva de Freitas, E P, Teixeira Dourado Júnior, M E, Maurício de Sena-Evangelista, K C, Leite-Lais L. Dietary intake and zinc status in amyotrophic lateral sclerosis patients. Nutrición Hospitalaria

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(17) Motlagh B, MacDonald JR, Tarnopolsky MA. Nutritional inadequacy in adults with muscular dystrophy. Muscle & Nerve 2005 Jun;31(6):713-718.

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26 Nutritional Status and Nutrient Intake Challenges in Children With Spinal Muscular Atrophy. Pediatric Neurology 2016;57:80-83.

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(28) Weijs, Peter J.M.|Wolfe, Robert R. Exploration of the protein requirement during weight loss in obese older adults. Clinical Nutrition 2015;35(2):394-398.

(29) Michelfelder AJ, MD. Soy: A Complete Source of Protein. American Family Physician 2009;79(1):43-47.

(30) Deutz, Nicolaas E.P.|Pereira, Suzette L.|Hays, Nicholas P.|Oliver, Jeffery S.|Edens, Neile K.|Evans, Chris M.|Wolfe, Robert R. Effect of β-hydroxy-β-methylbutyrate (HMB) on lean body mass during 10 days of bed rest in older adults. Clinical Nutrition

2013;32(5):704-712.

(31) Verreijen AM, Verlaan S, Engberink MF, Swinkels S, Bosch J, Weijs PJM. A high whey protein-, leucine-, and vitamin D-enriched supplement preserves muscle mass during intentional weight loss in obese older adults: a double-blind randomized controlled trial. American Journal of Clinical Nutrition 2015;101(2):279-286.

(32) Ramelli GP, Aloysius A, King C, Davis T, Muntoni F. Gastrostomy placement in paediatric patients with neuromuscular disorders: indications and outcome. Developmental medicine and child neurology 2007 May;49(5):367-371.

(33) Barja S, Regina. Clinical assessment underestimates fat mass and overestimates resting energy expenditure in children with neuromuscular diseases. Clinical Nutrition ESPEN 2016;15:11-15.

(34) Gleason, Philip M., PhD|Harris, Jeffrey, DrPH, RD|Sheean, Patricia M., PhD, RD|Boushey, Carol J., PhD, MPH, RD|Bruemmer, Barbara, PhD, RD. Publishing Nutrition Research: Validity, Reliability, and Diagnostic Test Assessment in Nutrition-Related Research. Journal of the American Dietetic Association 2010;110(3):409-419. (35) McCrory MA, Kim HR, Wright NC, Lovelady CA, Aitkens S, Kilmer DD. Energy

expenditure, physical activity, and body composition of ambulatory adults with hereditary neuromuscular disease. The American journal of clinical nutrition 1998 Jun;67(6):1162-1169.

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Appendix 1. Information letter and informed consent

Proefpersoneninformatie voor deelname aan onderzoek Voeding en spierziekten: Focus op eiwitinname

Inleiding

Geachte heer/mevrouw,

Wij vragen u vriendelijk om mee te doen aan een wetenschappelijk onderzoek. Meedoen is vrijwillig. Om mee te doen is wel uw schriftelijke toestemming nodig.

Dit onderzoek wordt uitgevoerd door de Hogeschool van Amsterdam. De toetsingscommissie van de METc van VUmc heeft beoordeeld dat dit onderzoek niet onder de Wet medisch-wetenschappelijk onderzoek met mensen (WMO) valt.

Voordat u beslist of u wilt meedoen aan dit onderzoek, krijgt u uitleg over wat het onderzoek inhoudt. Lees deze informatie rustig door en vraag de onderzoeker uitleg als u vragen heeft. U kunt er ook over praten met uw partner, vrienden of familie.

1. Doel van het onderzoek

Het doel van dit onderzoek is het verzamelen en analyseren van de huidige voedingsconsumptie van de doelgroep, met een focus op de eiwitinname. Dit doen we door een 2/3-daags voedingsdagboek in te vullen van mensen met een spierziekte. De eiwit en energie-inname zal eerst worden geregistreerd en daarna geanalyseerd. Het invoeren en verwerken van de voedingsconsumptie zal gedaan worden door studenten van de Hogeschool van Amsterdam en zij zullen de resultaten gebruiken voor hun afstudeeronderzoek.

Het onderzoek wordt uitgevoerd door een onderzoeker van de Hogeschool van Amsterdam en worden gepubliceerd in een nationaal en internationaal tijdschrift. Tevens wordt een landelijk netwerk geformeerd die mee schrijft aan een projectvoorstel omtrent de behoefte en rol van eiwitinname bij mensen met een spierziekte.

2. Wat meedoen inhoudt

U doet mee aan een deelonderzoek binnen het project ‘Voeding en spierziekten: focus op eiwitinname’. Uit verschillende onderzoeken is gebleken dat eiwitten een rol kunnen spelen in het behoud van de spiermassa en er eventueel zelfs voor kunnen zorgen dat er spieropbouw plaatsvindt. Echter is er nog weinig informatie bekend over de rol van eiwit in de voeding bij spierziekten. Om hier informatie over te verzamelen vragen wij u een 2/3-daags voedingsdagboek in te vullen. Hierbij vult u in wat u de afgelopen 2/3 dagen zowel gegeten als gedronken heeft. Omdat we graag uw eiwitinname willen berekenen zijn ook de portiegroottes van belang en het tijdstip dat u de voeding geconsumeerd heeft. Op die manier krijgen we inzicht over wat u heeft gegeten, wanneer en hoeveel.

3. Mogelijke voor- en nadelen

U heeft geen voordeel van meedoen aan dit onderzoek.

Nadelen kunnen zijn: Als er nog geen eetdagboek bij u is afgenomen/door u is ingevuld, zal dit eenmalig ongeveer 60 minuten van uw tijd kosten verdeeld over de 2/3 dagen.

4. Als u niet wilt meedoen of wilt stoppen met het onderzoek U beslist zelf of u meedoet aan het onderzoek. Deelname is vrijwillig.

Doet u mee aan het onderzoek? Dan kunt u zich altijd bedenken. U mag tijdens het onderzoek stoppen. U hoeft niet te zeggen waarom u stopt. Wel moet u dit direct melden aan de onderzoeker. De gegevens die tot dat moment zijn verzameld, worden gebruikt voor het onderzoek. Hierdoor kunnen de studenten wel uw gegevens gebruiken voor hun afstudeeronderzoek.

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28 5. Gebruik en bewaren van uw gegevens

Voor dit onderzoek is het nodig dat uw persoonsgegevens worden verzameld en gebruikt. Tijdens een gesprek met de diëtist zal informatie verzameld worden over wat u de afgelopen 2/3 dagen geconsumeerd heeft. Daarnaast is uw medische diagnose van belang voor het onderzoek. Elk proefpersoon krijgt een code die op zijn/haar gegevens (gecodeerd), Uw naam wordt verder in het onderzoek niet meer gebruikt en is alleen bekent bij uw eigen diëtist. Uw gegevens

Al uw gegevens blijven vertrouwelijk. Alleen de diëtist heeft u persoonsgegevens en zal deze gecodeerd aan de onderzoeker doorsturen. De onderzoeker heeft dan alleen een code met voedingsgegevens. U kunt de onderzoeker vragen om een elektronische kopie van uw gegevens die u heeft aangeleverd of die direct bij u gemeten zijn. De onderzoeksgegevens zijn bij publicatie in een (wetenschappelijk) tijdschrift daardoor niet te herleiden naar u. Aan het eind van het onderzoek zullen de onderzoeksresultaten gedeeld worden met uw diëtist. Als u de resultaten graag in wil zien is dat mogelijk. Vraag hiervoor de resultaten op bij uw diëtist. De resultaten zullen eind januari 2019 bekend zijn.

Als u de toestemmingsverklaring ondertekent, geeft u toestemming voor het verzamelen, bewaren en inzien van uw medische en persoonsgegevens.

De onderzoeker bewaart uw gegevens 10 jaar. Daarna worden de persoonsgegevens vernietigd.

Indien u ontevreden bent over hoe wordt omgegaan met uw privacy dan kunt u een klacht indienen bij de Functionaris gegevensbescherming van de Hogeschool van Amsterdam. Roeland Reijers, u kunt contact opnemen via de email: functionarisgegevensbescherming@hva.nl. Ook kunt u terecht bij de Autoriteit Persoonsgegevens.

Aanvullend gebruik gegevens

Wij willen uw gegevens graag bewaren. Misschien kunnen we daar later extra onderzoek mee doen. Het gaat dan om verder onderzoek naar de rol van eiwitten bij mensen met een spierziekte. Op het toestemmingsformulier kunt u aangeven of u hiermee akkoord gaat. U kunt deze toestemming altijd weer intrekken. Uw persoonsgegevens worden dan vernietigd. 6. Vergoeding voor meedoen

Voor het meedoen aan dit onderzoek krijgt u geen onkostenvergoeding. 7. Heeft u vragen?

Bij vragen kunt u contact opnemen met de studenten Voeding en Diëtetiek van de Hogeschool van Amsterdam. Alle contactgegevens staan in Bijlage A op de volgende pagina.

Bijlagen:

A. Contactgegevens onderzoeksteam. (Niet in scriptie opgenomen wegens privacy) Indien de proefpersoon een kind is ontvangt u als ouders/voogden:

- Formulier: Proefpersoneninformatie voor deelname aan onderzoek; informatie voor ouders/voogden en kind.

- Toestemmingsverklaring voor ouders/voogden van proefpersoon 0-11 jaar of Toestemmingsverklaring voor kind 12-15 jaar en ouders/voogden van proefpersoon.

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Toestemmingsformulier proefpersoon

Voeding en spierziekten: focus op eiwitinname

- Ik heb de informatiebrief gelezen. Ook kon ik vragen stellen. Mijn vragen zijn voldoende beantwoord. Ik had genoeg tijd om te beslissen of ik meedoe.

- Ik weet dat meedoen vrijwillig is. Ook weet ik dat ik op ieder moment kan beslissen om toch niet mee te doen of te stoppen met het onderzoek. Daarvoor hoef ik geen reden te

geven.

- Ik geef toestemming voor het verzamelen en gebruiken van mijn gegevens op de manier en voor de doelen die in de informatiebrief staan. Ik geef toestemming om mijn gegevens nog 10 jaar na dit onderzoek te bewaren.

- Ik wil meedoen aan dit onderzoek.

□ Ik geef toestemming om mijn gegevens nog na dit onderzoek te bewaren. Mogelijk kan dit later nog voor meer onderzoek worden gebruikt, zoals in de informatiebrief staat. □ Ik geef toestemming om mij na dit onderzoek opnieuw te benaderen voor een vervolgonderzoek

Naam proefpersoon:

………..……… Handtekening: ……….…… Datum: __ / __ / ____

--- Ik verklaar dat ik deze proefpersoon volledig heb geïnformeerd over het genoemde onderzoek.

Als er tijdens het onderzoek informatie bekend wordt die de toestemming van de

proefpersoon zou kunnen beïnvloeden, dan breng ik hem/haar daarvan tijdig op de hoogte. Naam onderzoeker (of diens vertegenwoordiger): ………..

Handtekening: ……….…… Datum: __ / __ / ____

De proefpersoon krijgt een volledige informatiebrief mee, samen met een kopie van het getekende toestemmingsformulier.

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Appendix 2. Nutritional diary

Deelnemer nummer:………

Voedingsdagboek

Voeding en spierziekten

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32

Voor u ligt een voedingsdagboek waar u voor gedurende 3 dagen in bijhoudt

wat u eet en drinkt.

Waar u op moet letten

1. De 3 dagen bestaat uit: 2 doordeweekse dagen en 1 weekenddag

2. Het is handig om meteen op te schrijven wat u eet, dan is de kans dat u iets

vergeet kleiner.

3. Schrijf alles op, ook kleine snacks zoals een biscuitje, fruit en een handje

pinda’s. En vergeet niet op te schrijven wat u hebt gedronken.

4. Het is zeer belangrijk dat u noteert hoeveel u van iets eet, bijvoorbeeld:

 1 stuk fruit

 1 snee brood

 3 grote aardappelen

 1 schaaltje vla

U kunt ook aangeven hoeveel gram of milliliter u hebt gegeten en gedronken,

dit kunt u doen door de verschillende producten te wegen voordat u het

opschrijft.

5. Omschrijf zo nauwkeurig mogelijk welk soort u gebruikt, bijvoorbeeld:

- Melk: Volle, halfvolle, mager, karnemelk, bananenmelk

- Brood; wit, bruin, volkoren, krenten, etc.;

- Halvarine/ Margarine: Dieet-, light-, Pro-activ.

6. Omschrijf ook hoe u iets eet/ drinkt, bijvoorbeeld;

- Koffie; met of zonder melk/ suiker

- Aardappelen: gekookt, gebakken, gefrituurd

7. Als er niet voldoende schrijfruimte is ga dan verder op de onderkant of

achterzijde van de pagina. Geef hier dan wel aan bij welke dag en welk

eetmoment het hoort.

Om een zo goed mogelijk beeld te krijgen van uw voedingspatroon is het

belangrijk om zo NAUWKEURIG en EERLIJK mogelijk uw voeding te noteren.

Hierdoor krijgen de onderzoekers een zo juist mogelijk beeld van uw

voedingsinname

Voorbeeld lunch

Voedingsmiddel

Soort en/of

merk:

Hoeveel:

Brood

Bruin

2 sneetjes

Halvarine

Becel light

Dun besmeerd

Kaas

Goudse 48+

kaas

1 plakje

Jam

Light

Normaal

besmeerd

(34)

Het noteren van de warme maaltijd wordt vaak moeilijk gevonden. Daarom volgt

hieronder een voorbeeld.

Voorbeeld warme maaltijd; FOUT

Voedingsmiddel:

Soort en/of

merk:

Hoeveel:

Tagliatelle bolognaise

1 bord

Spinazie a la crème en Parmezaanse kaas

Wijn

2 glazen

Voorbeeld warme maaltijd; GOED

Voedingsmiddel:

Soort en/of

merk:

Hoeveel:

Tagliatelle

3 opscheplepels

Gehakt

Half-om-half

75 gram

Tomaat

2 stuks

Ui

½

Spinazie

A la crème

2 opscheplepels

Kaas

Parmezaanse

4 eetlepels

Wijn

Rood

2 glazen

Bereidingsvet:

Vloeibaar bak- en braadproduct

(35)

34

Weekend of weekdag:………….

DAG 1

Datum :………...

Ontbijt

Tijdstip:……...uur

Voedingsmiddel Soort en/of merk Hoeveel

In de loop van de morgen Tijdstip:……...uur

(36)

Lunch Tijdstip:……...uur

Voedingsmiddel Soort en/of merk Hoeveel

In de loop van de middag Tijdstip:……...uur

(37)

36

Avond maaltijd

Tijdstip:……...uur

Voedingsmiddel Soort en/of merk Hoeveel

(38)

In de loop van de avond Tijdstip:……...uur

Voedingsmiddel Soort en/of merk Hoeveel

Eventuele opmerkingen: ……… ……… ……… ……… ……… ………

(39)

38

Weekend of weekdag:……….

DAG 2

Datum :………...

Ontbijt

Tijdstip:……...uur

Voedingsmiddel Soort en/of merk Hoeveel

In de loop van de morgen Tijdstip:……...uur

(40)

Lunch Tijdstip:……...uur

Voedingsmiddel Soort en/of merk Hoeveel

In de loop van de middag Tijdstip:……...uur

(41)

40

Avond maaltijd

Tijdstip:……...uur

Voedingsmiddel Soort en/of merk Hoeveel

(42)

In de loop van de avond Tijdstip:……...uur

Voedingsmiddel Soort en/of merk Hoeveel

Eventuele opmerkingen: ……… ……… ……… ……… ……… ………

(43)

42

Weekend of weekdag:……….

DAG 3

Datum :………...

Ontbijt

Tijdstip:……...uur

Voedingsmiddel Soort en/of merk Hoeveel

In de loop van de morgen Tijdstip:……...uur

(44)

Lunch Tijdstip:……...uur

Voedingsmiddel Soort en/of merk Hoeveel

In de loop van de middag Tijdstip:……...uur

(45)

44

Avond maaltijd

Tijdstip:……...uur

Voedingsmiddel Soort en/of merk Hoeveel

(46)

In de loop van de avond Tijdstip:……...uur

Voedingsmiddel Soort en/of merk Hoeveel

Eventuele opmerkingen: ……… ……… ……… ……… ……… ………

(47)
(48)

Assessment form

Beoordeling afstudeerproject: scriptie, eindassessment en proces

Naam student: Josje Pouwer en Laure Snijders

Titel en nummer AO:

Dietary protein intake of Dutch people with Neuromuscular Diseases

2019102

Opdrachtgever: HvA Lectoraat Gewichtsmanagement

Naam examinator:

M van der Werf

Naam docentbegeleid er: M Tieland Datum: 6 januari 2019 1. Beoordeling scriptie Criterium B onvoldoend e

voldoende goed Doce

nt Exa m. o/v/g o/v/ g Randvoorwaard elijke eisen (weging 0%): zie ook Taal- en schrijfwijzer (MijnHvA) en Handleiding Afstudeerproject 2 Niet op tijd ingeleverd

Op tijd ingeleverd Op tijd ingeleverd Leesbaarhei d Leesbaarheid Leesbaarhei d De afstudeeropd racht is een aaneenschak eling van stukken tekst, het geheel loopt niet. Een eigen schrijfstijl is niet herkenbaar. Er zijn veel grammaticale en spelfouten. De formulering is persoonlijk, subjectief, vaag en wijdlopig. De afstudeeropdracht leest redelijk vlot, er zijn weinig

grammaticale en spelfouten. De formulering is bondig, hier en daar wat algemeen en niet overal even formeel. Het verhaal is een lopend geheel. De afstudeeropdr acht is vlot leesbaar en in correct Nederlands of Engels geschreven: de formulering is bondig, concreet en formeel. De rode draad in het verhaal is duidelijk.

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