Outline Nutrition Interventions for ROHHAD

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S

Nutrition Interventions for ROHHAD

Ashley Berrett Advanced Pediatric Case Study

Outline

S  Discuss pathophysiology and clinical presentation of ROHHAD.

S  Introduce case study patient.

S  Detail nutrition interventions taken as part of medical management of ROHHAD.

What is ROHHAD? Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation

Patwari PP, Rand CM, Berry-Kravis EM, et al. Monozygotic twins discordant for ROHHAD phenotype. Pediatrics. 2011; 128(3): 711-715.

Patwari PP, Wolfe LF. Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation: review and update. Curr Opin Pediatr. 2014; 26: 487-492.

Photo credit http://haveyroo.blogspot.com/2009/02/comparison-photos-rohhad-and.html

Rapid-Onset Obesity

June 2006 July 2006

These photographs DO NOT reflect actual case study patient.

Hypothalamic Dysfunction

Hypothalamus

Pituitary Gland

Adrenal glands, thyroid gland, ovaries, and testes

Body temperature, growth, salt and water balance, sleep, weight, appetite, and more

controls

Albert RK, Bowman MA, Braunstein GD, et al. The Merck Manual of Diagnosis and Therapy. 19th ed. Whitehouse Station, NJ: Merck Sharp & Dohme Corp; 2011

Photo credit http://www.merckmanuals.com/home/hormonal_and_metabolic_disorders/pituitary_gland_disorders/overview_of_the_pituitary_gland.html

releases hormones to

detects changes in

Hypothalamic Dysfunction

S  Water imbalance S  Dysregulation of antidiuretic hormone (ADH)

S  Hypernatremia or hyponatremia

S  Hypothyroidism

S  Growth hormone deficiency

S  Altered onset of puberty


John CC, Day MW. Central Neurogenic Diabetes Insipidus, Syndrome of Inappropriate Secretion of Antidiuretic Hormone, and Cerebral Salt-Wasting Syndrome in Traumatic Brain Injury. Crit Care Nurse. 2012; 32(2):1-7.

Pronsky ZM, Crowe JP. Food-Medication Interactions. 17th ed. Birchrunville, PA: Food Medications Interactions; 2012.

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Hypoventilation

S  Develops either with or after onset of obesity.

S  Consists of obstructive sleep apnea and/or central hypoventilation.


Dhondt K, Verloo P, Verhelst H, et al. Hypocretin-1 deficiency in a girl with ROHHAD syndrome. Pediatrics. 2013; 132(3): 788-792.

Autonomic Dysregulation

S  Decreased heart rate

S  Opthalmologic abnormalities

S  Altered thermoregulation

S  Gastrointestinal dysmotility

S  Altered pain perception



Autonomic Nervous System

Receives input in the body and regulates physiologic processes. Controls BP, HR, body temp, weight, digestion, metabolism, fluid and electrolyte balance, sweating, urination, and more.

ROHHAD Symptoms

S

Case Study Patient

Patient Profile

S  16 y/o Caucasian female

S  Resident at skilled nursing facility for 10 years, multiple discharges and readmissions for medical procedures

S  Attended school at out-of-house facility

S  In custody of the Division of Child and Family Services

S  Parents have visitation rights

ROHHAD Diagnosis

S  Initial admit at skilled nursing facility (age 6) S  Admit dx: excessive wt gain of unknown etiology (similar to Prader-Willi

syndrome), obstructive sleep apnea, vent dependent at night S  Nutrition note: “Resident at severe nutritional risk r/t excessive weight gain of

unknown etiology, and seemingly unsatiable appetite. Resident is at 209% IBW with a BMI of 32. Physician stated in pediatric rounds that it is thought that weight gain may be r/t central endocrine abnormality but is yet to be diagnosed…”

S  8/30: Discharged to acute care hospitalfor hypoventilation, bradycardia, and low body temperature (BP 77/47, pulse 44, temp 35.8, RR 16). Diagnosed with ROHHAD. Pacemaker placed.

S  9/30: Readmitted to skilled nursing facility.

Cassidy SB, Driscoll DJ. Prader-willi syndrome. Eur J Hum Genet. 2009; 17: 3-13.

Medical History

S  Central hypoventilation with respiratory failure and trach-vent dependency

S  Pituitary insufficiency with thyroid replacement and growth hormone

S  Behavioral problems

S  Developmental delay

S  Obstructive sleep apnea

S  Chronic lung disease with pulmonary fibrosis

S  Central temperature instability

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Medical Treatment

S  Rapid Onset Obesity: Dietary interventions for weight management.

S  Hypothalamic Dysfunction: Fluid goals, antidiuretics, growth hormone replacement, and thyroid medications.

S  Hypoventilation: Tracheostomy and mechanical ventilation.

S  Autonomic Dysregulation: Cardiac pacemaker, bowel medications, darkened room, and close monitoring of vital signs.

Anthropometrics

10/12 Assessment

Weight 69.7kg (153.3lb) 89th%ile

Height 157.5cm (62in) 24th%ile

BMI 28.1 93rd%ile (overweight)

IBW 53-60kg

%IBW 116-132% Overweight

UBW 69-72kg

Weight Changes Stable x 1 mos -5.4% x 3 mos -7.1% x 6 mos

Non-significant, beneficial weight loss

2 to 20 years: GirlsStature Weight-for-age percentiles-for-age and

NAME

RECORD #

WEIGHT

WEIGHT

STATURE

STATURE

kg10

15

20

25

30

35

80

85

90

95

100

105

110

115

120

125

130

135

140

145

150

155

cm

150

155

160

165

170

175

180

185

190

kg10

15

20

25

30

35

105

45

50

55

60

65

70

75

80

85

90

95

100

2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20

12 13 14 15 16 17 18 19 20

AGE (YEARS)

AGE (YEARS)

40

160

cm 113 4 5 6 7 8 9 10

90

75

50

25

10

90

75

50

25

10

97

3

97

3

lb

30

40

50

60

70

80

lb

30

40

50

60

70

80

90

100

110

120

130

140

150

160

170

180

190

200

210

220

230

Date

Mother’s Stature Father’s Stature

Age Weight Stature BMI*

62

42

44

46

48

60

58

52

54

56

in

30

32

34

36

38

40

50

74

76

72

70

68

66

64

62

60

in

SOURCE: Developed b(2000).

y the National Center for Health Statistics in collaboration withthe National Center for Chronic Disease Prevention and Health Promotionhttp://www.cdc.gov/growthcharts

Published May 30, 2000 (modified 11/21/00).

Grow

th Charts

Grow

th Charts

2 to 20 years: GirlsBody mass index-for-age percentiles

NAME

RECORD #

SOURCE: Developed b(2000).

y the National Center for Health Statistics in collaboration withthe National Center for Chronic Disease Prevention and Health Promotionhttp://www.cdc.gov/growthcharts

2 543 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20

26

24

22

20

18

16

14

12

kg/m2

28

26

24

22

20

18

16

14

12

kg/m2

30

32

34

BMI

BMI

AGE (YEARS)

13

15

17

19

21

23

25

27

13

15

17

19

21

23

25

27

29

31

33

35

Date Age Weight Stature BMI* Comments

95

90

85

75

50

10

25

5

Published May 30, 2000 (modified 10/16/00).

BMI-for-Age

Clinical Observations

S  Appeared overweight but not obese

S  Energetic demeanor

S  Behavioral disturbances

S  History of polyphagia and polydipsia

Nutrition Care Plan

S  PES (2/20): Involuntary weight gain r/t insatiable appetite, ROHHAD AEB hx significant weight gain x 1, 3, and 6 months.

S  Goals: Wt stability or gradual wt loss to previous UBW of 68-70kg. Prevention of skin breakdown, adequate hydration, and energy for daily activities and therapies.

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Dietary History

Date Diet Order Fluid Order Reason for Change

6/14 900 kcals Admit, restrict d/t hyperphagia

10/4 Regular pediatric Ex: 2500mL Changed d/t wt loss

8/27 1200 kcals Ex: 4500mL ROHHAD dx

9/12 1200 kcals Snacks TID

Ex: 2750mL Current diet order

S  PO intake = 100%

S  Estimated needs (calculated using IBW 57kg): 1150-1440 kcals/day (20-25kcals/kg), 57-70g protein (1-1.2g/kg), 2750ml/day (to maintain fluid balance)

Analysis of Dietary Interventions

S  Dietary interventions have been successful. S  Current weight 69.7kg, meeting goal of 68-70kg. S  Diet + multivitamin adequate to meet nutritional needs.

S  Update PES statement: Overweight (NC-3.3.1) r/t ROHHAD AEB BMI 28.1 at 93rd%ile BMI-for-age, hx of significant wt gain.

S  Continue current POC.

S  Monitor weight, nutrition-related labs, skin status, PO intake, tolerance to diet order, and fluid balance and make recommendations PRN.

Prognosis & Conclusion

S  High risk for sudden mortality.

S  No treatment, but complications can be managed through symptom-targeted interventions.

S  The patient is likely one of the oldest known living patients with ROHHAD.


References

S  Patwari PP, Rand CM, Berry-Kravis EM, et al. Monozygotic twins discordant for ROHHAD phenotype. Pediatrics. 2011; 128(3): 711-715.

S  Patwari PP, Wolfe LF. Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation: review and update. Curr Opin Pediatr. 2014; 26: 487-492.

S  Albert RK, Bowman MA, Braunstein GD, et al. The Merck Manual of Diagnosis and Therapy. 19th ed. Whitehouse Station, NJ: Merck Sharp & Dohme Corp; 2011.

S  John CC, Day MW. Central neurogenic diabetes insipidus, syndrome of inappropriate secretion of antidiuretic hormone, and cerebral salt-wasting syndrome in traumatic brain injury. Crit Care Nurse. 2012; 32(2):1-7.

S  Cassidy SB, Driscoll DJ. Prader-willi syndrome. Eur J Hum Genet. 2009; 17: 3-13.

S  Pronsky ZM, Crowe JP. Food-Medication Interactions. 17th ed. Birchrunville, PA: Food Medications Interactions; 2012.

S  Dhondt K, Verloo P, Verhelst H, et al. Hypocretin-1 deficiency in a girl with ROHHAD syndrome. Pediatrics. 2013; 132(3): 788-792.

Documents

Outline Nutrition Interventions for ROHHAD