Stages of Hypophagia

One of the the major characteristics - if not the main one - is the issue around eating, appetite, and the fact that there seems to be no "on/off" switch to regulate the appetite.  It is ironic that a newborn babe is virtually unable to feed, yet this turns completely around to become a child who simply cannot stop feeding.

Most of us understand that this is a genetic condition and something that cannot be cured, but it can be managed.  I found the following chart in a paper entitled Clinical Characteristics of the Nutritional Phases and thought it was really interesting to track these phases with the stages that my daughter went through.  Now that she is in her early 30s, she is, generally speaking, a lot more flexible around food and will accept changes if there is a good reason for them.  It's been a long time coming, I have to say.  We still lock food cupboards and the fridge and she can't really be trusted around food that is left out, and she's still very opportunistic (will take every opportunity if it's left open to her), but she's clearly not the teenager that we had huge challenges with.

Of course, everyone is different and there is a huge scale of difference where the person you know with PWS might fit in.  The reason I'm sharing this Table with you is simply because when my daughter was growing up, other people could just not understand her obsession with food, her behaviour around food, and the very real consequences it had - not just for her, but for our whole family.  This Table is pretty clear in its definition and this is a good thing to share with those who just don't 'get it'.

AMERICAN JOURNAL OF MEDICAL GENETICS PART A

TABLE I. Clinical Characteristics of the Nutritional Phases

Phase 0      

• Decreased fetal movements and lower birth weight
• Full-term birth weight and BMI are about 15–20% less than the siblings
• Typically normal gestational age
• 85% have decreased fetal movements

Phase 1a    

• Hypotonia with difficulty feeding (0–9 months)
• Weak, uncoordinated suck. Usually cannot breastfeed
• Needs assistance with feeding either through feeding tubes (nasal/oral gastric tube or gastrostomy tube) or orally with special, widened nipples. Many would die without assisted feeding
• Oral feeds are very slow
• Severely decreased appetite. Shows little or no evidence of being hungry
• Does not cry for food or get excited at feeding time
• If feeding just occurred when baby ‘‘acted hungry’’ then would have severe ‘‘failure-to-thrive’’ Weak cry

Phase 1b   

• No difficulty feeding and growing appropriately on growth curve (9–25 months)
• No longer needs assisted feeding
• Growing steadily along growth curve with normal feeding Normal appetite

Phase 2a    

• Weight increasing without an increase in appetite or excessive calories (2.1–4.5 years) Infant starts crossing growth curve centile lines
• No increase in appetite
• Appetite appropriate for age
• Will become obese if given the recommended daily allowance (RDA) for calories or if eating a ‘‘typical’’ toddler
• diet of 70% carbohydrates
• Typically needs to be restricted to 60–80% of RDA to prevent obesity

Phase 2b   

• Weight increasing with an increase in appetite (4.5–8 years)
• Increased interest in food. Frequently asking ‘‘food related’’ questions
• Preoccupied with food. Very concerned about the next meal/snack (e.g., ‘‘Did you remember to pack my lunch?’’)
• Increased appetite
• Will eat more food than a typical child if allowed
• Will eat food within their line of sight if unattended
• Will become obese if allowed to eat what they want
• Can be fairly easily redirected about food
• Can feel full
• Will stop eating voluntarily

Phase 3      

• Hyperphagic, rarely feels full (8 years - adulthood)
• Constantly thinking about food
• While eating one meal they are already thinking about the next meal
• Will awaken from sleep early thinking about food
• Will continue eating if portion size is not limited
• Rarely (truly) feels full
• Will steal food or money to pay for food
• Can eat food from garbage and other unsavory/inedible sources (e.g., dog food, frozen food, crayons, etc.)
• Typically are not truthful about what they have eaten (i.e. amount and types of food)
• Will gain considerable amount of weight over a short period of time if not supervised (e.g., some individuals are known to have gained up to 20 pounds in one weekend)
• Food typically needs to be locked up. Frequently the child will ask the parent to lock the food if the parent has forgotten Will break into neighbors’ houses for food
• Temper tantrums and ‘‘meltdowns’’ frequently related to food
• Needs to be placed on a diet that is approximately 50–70% of the RDA to maintain a healthy weight

Phase 4      

• Appetite is no longer insatiable (adulthood)
• Appetite may still be increased or may be normal or less than normal
• Previously in phase 3, but now a noticeable improvement in their appetite control
• Can feel full
• Appetite can fluctuate in this phase, but the key component is noticeable improvement in control of appetite compared to when they were younger
• Not as preoccupied with food
• Absence of major temper tantrums and ‘‘meltdowns’’ related to food
• Onset in adulthood. Could be as early as 20s or as late as 40–50s
• Most adults have not gone into this phase and maybe some (most?) never will

 If you would like to read the full paper, contact me and I will send it to you.:

Nutritional Phases in Prader–Willi Syndrome

Jennifer L. Miller,¹ Christy H. Lynn,¹ Danielle C. Driscoll,¹ Anthony P. Goldstone,^1,2 June-Anne Gold,³

Virginia Kimonis,³ Elisabeth Dykens,⁴ Merlin G. Butler,⁵ Jonathan J. Shuster,⁶ and Daniel J. Driscoll^1,7*

1

Department of Pediatrics, College of Medicine, University of Florida, Gainesville, Florida

2

Department of Imaging Services, Hammersmith Hospital, London, England

3

University of California Irvine, Irvine, California

4

Vanderbilt Kennedy Center, Vanderbilt University, Nashville, Tennessee

5

Departments of Psychiatry and Behavioral Sciences and Pediatrics, Kansas University Medical Center, Kansas City, Kansas

6

Department of Health Outcomes and Policy, University of Florida, Gainesville, Florida

7

Center for Epigenetics, College of Medicine, University of Florida, Gainesville, Florida

Received 11 September 2010; Accepted 25 January 2011