medical nutrition therapy for prader willi syndrome l.
Download
Skip this Video
Loading SlideShow in 5 Seconds..
Medical Nutrition Therapy for Prader-Willi Syndrome PowerPoint Presentation
Download Presentation
Medical Nutrition Therapy for Prader-Willi Syndrome

Loading in 2 Seconds...

play fullscreen
1 / 34

Medical Nutrition Therapy for Prader-Willi Syndrome - PowerPoint PPT Presentation


  • 1337 Views
  • Uploaded on

Medical Nutrition Therapy for Prader-Willi Syndrome. By Bergen Holzworth CONCORDIA COLLEGE – MOORHEAD, MN. Objectives. Be able to describe Prader-Willi Syndrome Be familiar with the health effects associated with Prader-Willi Syndrome

loader
I am the owner, or an agent authorized to act on behalf of the owner, of the copyrighted work described.
capcha
Download Presentation

PowerPoint Slideshow about 'Medical Nutrition Therapy for Prader-Willi Syndrome' - jena


An Image/Link below is provided (as is) to download presentation

Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author.While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server.


- - - - - - - - - - - - - - - - - - - - - - - - - - E N D - - - - - - - - - - - - - - - - - - - - - - - - - -
Presentation Transcript
medical nutrition therapy for prader willi syndrome

Medical Nutrition Therapy for Prader-Willi Syndrome

By Bergen Holzworth

CONCORDIA COLLEGE – MOORHEAD, MN

objectives
Objectives
  • Be able to describe Prader-Willi Syndrome
  • Be familiar with the health effects associated with Prader-Willi Syndrome
  • To gain an understanding of the nutrition care process for patients with Prader-Willi Syndrome throughout their lifespan
  • Be able to identify ethical issues involved with nutritional care methods used in managing Prader-Willi Syndrome
background information
Background Information
  • Prader-Willi Syndrome (PWS) is a Endocrine Hypothalamic Disorder
  • It affects 1 in 10,000 to 25,000 persons born
  • There are approximately 400, 000 people living with PWS worldwide
  • PWS is a congenital chromosomal disorder affecting chromosome 15
  • There is no cure to the syndrome but it can be managed through strict diet restriction, behavior management, and exercise regimenting
  • Most common genetically identified cause of obesity
characteristics of pws
Characteristics of PWS
  • Compulsive Hyperphagia
    • Abnormally increased appetite
  • Polyphagia
    • Excessive eating
  • Short stature with small hands and feet
  • Mild retardation
  • Hypogonadism
    • production of sex hormones and germ cells (sperm and eggs) is below normal which means they are sexually sterile
characteristics continued
Characteristics Continued
  • Muscular Hypotonia
    • Low muscle tone
    • Decreased limb movement
    • Poor reflexes
  • Exhibit non-ambulatory behavior
    • Exercise and movement are avoided
    • Sedentary lifestyles are sought due to comfort
  • Reduced energy needs
    • Metabolism is slower than average
characteristics continued6
Characteristics Continued
  • Dental caries
  • Bizarre eating behaviors (gorging, hoarding, food stealing, eating inappropriate foods, etc.)
  • Poor sucking ability and failure to thrive in infancy
  • Varying degrees of obsessive compulsive behavior
  • Slow motor development
  • Obesity related diabetes in later childhood
  • Lack of emotional control
  • Drowsiness
diagnosis
Diagnosis
  • PWS is being diagnosed earlier and earlier now because the key symptom observed in infancy is failure to thrive and hypotonia. Babies born with these characteristics then must undergo genetic testing to determine if the syndrome is present
  • Early diagnosis means early intervention and health-related disorder and disease prevention
secondary health effects due to obesity
Secondary Health Effects Due to Obesity
  • Impaired pulmonary function
  • Sleep Apnea- breathing during sleep halts briefly but frequently (even in non-obese PWS individuals)
  • Scoliosis
  • Phlebitis- Inflammation of veins usually caused by clots
    • Chronic leg edema
  • Impaired cardiac function
  • Type II Diabetes
  • Decreased life span
etiology of pws
Etiology of PWS
  • The hypothalamus, which controls feelings of fullness, does not function correctly in PWS patients
  • Growth hormone releasing hormones (GHRH) are reduced by approximately 30% less than the average person
  • Levels of ghrelin, a potent appetite stimulating hormone, are raised and remain elevated even after meals
chromosomal
Chromosomal

Paternal and or maternal genes of chromosome 15 are defective, absent, or silenced in PWS

three phases of pws
Three Phases of PWS
  • 1.) Hypotonic Stage (prenatal-infancy)
  • 2.) Hyperphagic Stage (childhood)
  • 3.) Adolescence and Adulthood
intervention in infancy hypotonic phase
Intervention in Infancy (hypotonic phase)
  • In infancy PWS is characterized as failure to thrive, because infants lack sucking reflexes
  • Intervention is needed in order for infants to reach appropriate weight ranges
    • Kcal needs are determined according to infant’s length in cm
  • Feeding interventions
    • Special nipples
    • Tube feedings
  • Occupational and physical therapy often needed
  • Physical status is monitored closely
  • Growth hormone therapy
infancy
Infancy
  • Hypotonia in infancy makes it difficult for babies to attain adequate calories, but by approximately 12 months to 36 months hyperphagia begins causing a massive influx in body weight. When hyperphagia becomes apparent the nutritional intervention methods need to be drastically changed.
intervention in early childhood
Intervention in Early Childhood
  • Hyperphagia begins between ages 1-6
    • Weight control and management
    • Vitamin supplementation if needed
  • Children exhibit compulsive eating and lack satiety
    • Hypothalamic abnormalities reduce physiological sensations
  • GHRH is reduced by 30% so children are shorter in stature
    • Growth hormones are needed to avert early onset obesity which, in PWS patients on average occurs around age 2
  • Educational and behavioral interventions are also needed for cognitive and social disabilities
adolescence and adulthood
Adolescence and Adulthood
  • The average individual with PWS has 40-60% body fat by later adolescence and adulthood (2-3 X more than gen. pop.)
  • Unless weight has been strictly managed throughout childhood weight-related disorders and diseases have manifested themselves
    • Reduced or inhibited glucose tolerance due to diabetes
      • 25% of adults with PWS have type II diabetes
  • Nutritional intervention must be taken by a behavioral, medical, and an educational perspective
    • Weight management plans are highly variable and depend up upon the individual and their support groups
growth hormone therapy
Growth Hormone Therapy
  • The only scientifically proven intervention to have effects is through use of Growth Hormone therapy
    • Increases overall height
    • Nearly 2/3 of people with PWS receive therapy
    • Decreases overall body fat
    • Raises resting energy expenditure (REE)
      • Harris-Benedict Equations (calories/day):Male: (66.5 + 13.8 X weight Kg) + (5.0 X height cm) - (6.8 X age)Female: (665.1 + 9.6 X weight Kg) + (1.8 X height cm) - (4.7 X age)
    • Improves lean muscle mass, tone and strength
    • Lowers the BMI
care process considerations
Care Process Considerations
  • Because there is no cure to PWS the only way to manage weight is by strict diet control through educational, behavioral and nutritional methods
  • In addition intervention management techniques regarding PWS are facilitated by caregivers and usually must be monitored throughout the life-span
  • Managing food intake is primary goal in order to reduce the risk of secondary health effects related to obesity
importance of intervention
Importance of Intervention
  • Without intervention acute hyperphagia eventually leads to obesity which reduces life expectancy from 20 to 30 years.
  • In addition it also causes many other health problems
    • respiratory failure
    • diabetes mellitus
    • arteriosclerosis
    • congestive heart failure, (number one cause of mortality in PWS patients)
nutrition care process for infants with pws
Nutrition Care Process for Infants with PWS
  • Hypotonia inhibits infant’s ability to suck and limits the overall caloric intake
  • PWS infants are prone to being underweight already, as they tend to be premature by 2-3 weeks
  • Adaptive bottles and nipples are needed to assist in increasing feeding consumption
  • Thickening agents also prove to be beneficial
    • Examples: Instant Carobel® or Karicare Food Thickener®
    • Thickeners can increase Kcals /oz. up to 18% which may result less formula consumed
  • Enteral tube feedings using nasogastric tubes are utilized when less invasive methods fail
nutrition care process for pws children and adults
Nutrition Care Process for PWS Children and Adults
  • When hyperphagia becomes evident (age 1-3 years) the primary concern is weight management to avoid obesity and the secondary health effects that accompany it
  • Extreme measures for monitoring and restricting food intake must be taken to eliminate compulsive binge eating
    • Access to food must be totally eliminated
      • Cupboards and refrigerator must be locked up
    • Regimented diet and exercise must be implemented
    • Methods of obtaining food must be totally restricted
      • Access to money or friends’ homes
nutrition care process for pws adolescents and adults
Nutrition Care Process for PWS Adolescents and Adults
  • Diet adherence must be rendered by some sort of caregiver due to the compulsive nature and limited cognitive ability of individuals with PWS
  • Based upon family studies the most successful techniques were ones in which entire families participated in the same types of behavior as the member with PWS (unless absent)
    • Smaller portions at meal or snack times
      • Half size portions so that seconds can be had
    • Strictly scheduled meal times
    • Low-fat/ low-calorie foods consumed for everyone
    • Regular family activities and exercise
nutritional needs for pws
Nutritional Needs for PWS
  • Recommended caloric intake is
  • 1,000-1,200 Kcals per day
  • Calorie intake needs for people with PWS is 40% less than the average person
  • Low-calorie, Low-fat foods should be emphasized
nutrition care process for pws adolescents and adults24
Nutrition Care Process for PWS Adolescents and Adults
  • Despite pharmacologic innovation there is no known medical or pharmacological treatment for hyperphagia
    • Appetite suppressants, antiabsorptives, and pyschotropics have all proven ineffective or have even shown to exacerbate the overeating
  • Surgeries such as gastric or intestinal bypass have no effect on hyperphagia and therefore are not recommended
  • New research is examining the effects that ghrelin inhibiting drugs have on hyperphagia but there is no conclusive evidence yet
behavioral and cognitive intervention methods
Behavioral and Cognitive Intervention Methods
  • Slow motor and cognitive development make it hard to intervene using behavioral or analytically reasonable methods to a person with PWS until late adolescence or adulthood
  • Research and studies show that behavioral methods can be successful as long as they are strictly regimented with little to no room for exceptions
  • Positive reinforcement using non-food items has shown to help in compromising about food and diet
  • PWS patients are capable of making healthier food choices but without supervision they are still prone to overeat or choose preferably tasting high carbohydrate foods
invasive surgeries
Invasive Surgeries
  • Surgery is a rather uncommon treatment for weight-management in PWS patients because it has no effect on hyperphagia, but it is sometimes utilized
  • Gastric restrictive surgeries
    • Bariatric Surgery
      • Gastric banding
      • Gastric bypass
    • Trunkal Vagotomy
    • Jejunoileal bypass
  • Weight loss is proven to be initially successful but because patients were unable to comply long-term with dietary needs of bariatric patients, weight and obesity reoccurred in almost all patients
ethical dilemmas
Ethical Dilemmas
  • Should invasive weight-management procedures be allowed even though it does not treat the symptom of hyperphagia?
    • The majority of health professionals are in agreement that nutritional, behavioral and educational interventions, where the families of PWS patients are directly involved in care, are the most effective long-term weight-management techniques.
ethical dilemmas28
Ethical Dilemmas
  • Much primetime attention has been brought up regarding how families living with a PWS patient treat and care for that member. Is it really okay to lock up refrigerators, cupboards and to hide food from the member of the family with PWS?
    • Although the media may portray these situations as being unjust or unethical, the fact is that PWS patients have insatiable compulsions to eat and will eat anything and everything if it is in physical reach. If the only way to control the weight of a person with PWS is to lock up or hide all the food, then those are the measures that must be taken.
importance of dietitians
Importance of Dietitians
  • Although PWS has not become more prevalent more diagnosis‘s are seen because it is being identified earlier
  • Dietitians need to be able to assist in early interventions starting at infancy and throughout adolescence and adulthood
  • It is key to work with the family and or primary caregivers to educate them, implement dietary restrictions and guidelines, and to set nutritional goals and objectives
  • Although dietitians are needed in the realm of nutritional intervention methods, the behavioral interventions are better suited for psychologists, psychiatrists or counselors
in summary
In Summary
  • Prader-Willi Syndrome is a congenital chromosomal disorder affecting the hypothalamic region of the brain, causing hyperphagia and many other characteristic abnormalities
  • Interventions vary depending on the age related stage
    • Hypotonic stage in infancy- nutritional goal is to achieve adequate weight based on length
    • Hyperphagic stage (childhood)- hyperphagia is evident: goal is then weight-management
    • Adolescence and adulthood- focus is weight and behavioral management through education
  • There is no cure to PWS, but through proper nutritional and intervention methods weight can be healthy and stable
references
References
  • Bittel, D. C., & Butler, M. G. (2009). Prader–Willi syndrome. In Larry R. Squire (Ed.), Encyclopedia of neuroscience (pp. 873-883). Oxford: Academic Press.
  • Dykens, E., & Shah, B. (2003, February). Psychiatric Disorders in Prader-Willi Syndrome:
  • Epidemiology and Management. CNS Drugs, 17(3), 167-178. Retrieved September 22,2009, from Academic Search Premier database.
  • Goldberg, D. L., Garrett, C. L., Riper, C. V., & Warzak, W. J. (2002). Coping with prader-willi syndrome. Journal of the American Dietetic Association, 102(4), 537-542.
references33
References
  • Höybye, C. (2004). Endocrine and metabolic aspects of adult Prader–Willi syndrome with special emphasis on the effect of growth hormone treatment. Growth Hormone & IGF Research, 14(1), 1-15.
  • Kundert, D. K. (2008). Prader-willi syndrome. School Psychology Quarterly, 23(2), 246-257.
  • Lin, H., Lin, S., Tsai, L., Chao, M., Chen, M., Chuang, C., et al. (2008). Effects of growth hormone treatment on height, weight, and obesity in taiwanese patients with prader-willi syndrome. Journal of the Chinese Medical Association, 71(6), 305-309.
references34
References
  • Papavramidis, S., Kotidis, E., & Gamvros, O. (2006). Prader-Willi syndrome- associated obesity Treated by biliopancreatic diversion with duodenal switch. Journal of Pediatric Surgery, 41, 1153-1158.
  • Peckenpaugh, N. (2003). Nutrition Essentials and Diet Therapy: Ninth Edition. St. Louis, MO: Elsevier Science.
  • Rolfes, R. R., Pinna, K., Whitney, E. (2006). Understanding Normal and Clinical Nutrition. Belmont, CA: Thomson Wadsworth.
  • Zanella, S., Tauber, M., & Muscatelli, F. (2009). Breathing deficits of the prader-willi syndrome.Respiratory Physiology & Neurobiology, 168(1-2), 119-124.