Transcript File

REFEEDING SYNDROME:
NUTRITION MANAGEMENT IN THE
CLINICAL SETTING
SA MA NTHA WA LTERS
JANUARY 30, 2015
MAJOR CASE STUDY PRESENTATION
OBJECTIVES
• 1) To determine patients at risk for Refeeding
Syndrome and how to manage as a Clinical
Dietitian.
• 2) To identify the importance of a thorough
nutrition assessment in the clinical setting.
• 3) To develop an understanding of the genetic
disorder Neurofibromatosis Type 1.
THE PATIENT: AH
• 63 y/o AA female with
neurofibromatosis type 1
• Presented to JHH: per
request of her oncologist
with decreased PO intake
and weakness
• Admitted frequently to JHH
for years d/t complications
of NF1 and multiple facial
tumor resections.
SOCIAL AND FAMILY HISTORY
• Lives alone in one-story
apartment
• Recently lost a loved one
• Previous smoker 10
packs/year—quit 20 years
ago
• Denies EtOH or illicit drug use
• On disability d/t her medical
condition
PAST MEDICAL HISTORY
• Neurofibromatosis type I (initially dx 1955)
• Plexiform neurofibroma of the right face
(scalp, orbit, and face)
• Low grade malignant peripheral sheath
tumor
• Melanoma
• Migraines
• Pseudoseizures (4/2004)
PAST MEDICAL HISTORY
• Chronic pain
• Degenerative disc disease
• Asthma
• GERD
• Depression and anxiety (9/2006)
• Sleep apnea
• Hypertension
• Dissociative personality
disorder (9/2006)
PAST SURGICAL HISTORY
• 8/2011: R. parotidectomy, neck dissection, and
resection of a facial tumor in the orbital roof as
well as reconstruction with a free skin flap
transfer from her right upper thigh.
• 12/2011, 4/2011: additional debulking
procedure
• 8/2013: R. frontal extradural/intradural recurrent
facial plexiform NF tumor resection, R. eye
enucleation, orbital decompression, duraplasty,
titanium mesh cranioplasty, complex PRS
exposure/closure.
PAST SURGICAL HISTORY
• 5/2014: Rexeploration of R. frontotemporal
craniotomy for brain tumor resection.
• OSH: Multiple dermal neurofibroma
resections including left elbow, resection of
the R. frontal tumor (most recent 12/2009),
R. eyelid reconstruction (2006), melanoma
resection (2009) with wide excision and graft
HOME MEDICATIONS
• Lexapro
• Gabapentin
• Seroquel
• Mobic
• Vistaril
• Advair HFA
PRESENT MEDICAL PROBLEMS
• Neurofibromatosis type 1, plexiform neurofibroma
• Currently experiencing decreased PO intake and
weakness
• Management of medical conditions:
•
•
•
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•
•
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Melanoma
Migraines
HTN
Chronic pain
Degenerative disc disease
Asthma
Depression, Anxiety disorder, dissociative personality disorder
GERD
Discussion of
Neurofibromatosis
Type 1
WHAT IS IT?
• Neurofibromatosis: (type 1 and 2) the most
common neurologic tumor suppressor syndromes
and are a group of unrelated neurogenetic
conditions characterized by a predisposition to
develop multiple nerve sheath tumors.
• Neurofibromatosis Type 1 (NF1): the most common
of the two, with a birth incidence of ~1/3,000
individuals worldwide.
• an autosomal dominant neurocutaneous disorder that
predominately affects the skin, bone, and nervous system,
but complications vary, even within families with the same
germ line NF1 mutation.
HOW TO DIAGNOSE
• Made on clinical grounds when two or more of the
following are present:
• a first degree relative with NF1
• 6 or more café au lait patches (<0.5 cm in children and >1.5
cm in adults)
• axillary or groin freckling
• two or more iris Lisch nodules
• optic pathway glioma
• two or more cutaneous or subcutaneous neurofibromas or
one plexiform neurofibroma
• a distinctive bony dysplasia including sphenoid wing or
thinning of long bone cortex with or without pseudoarthrosis
PATHOPHYSIOLOGY: NF1
• Neurofibromatosis type 1 is a
classic single-gene disorder
with a high rate of new
mutations.
• The NF1 gene, located on
chromosome 17, provides
instructions for making a
protein called neurofibromin,
consisting of 2818 amino
acids.
PATHOPHYSIOLOGY: NF1
• This protein is produced in
many cells, including nerve cells
and specialized cells
surrounding nerves
(oligodendrocytes and
Schwann cells).
• Neurofibromin acts as a tumor
suppressor, meaning that it
keeps cells from growing and
dividing too rapidly or in an
uncontrolled way.
NF1 RELATED TO DEPRESSION
• Symptom severity varies widely among individuals
• Hallmark manifestation: nerve sheath tumors (including
neurofibromas and schwannnomas)
• These benign tumors can occur anywhere in the body,
and often cause significant morbidity including
disfiguring cutaneous tumors (NF1).
• May also have or develop malignant tumors and
nontumor manifestations that affect the nervous system,
eyes, and skin.
NF1 RELATED TO DEPRESSION
Emotional functioning of patients with
neurofibromatosis tumor suppressive syndrome
(Genetics in Medicine, 2012)
• 1/3 of patients in study with NF1 met criteria for at least one
psychiatric disorder.
• Multiple studies have found that NF1 appears to be associated
with reduced emotional functioning.
• Levels of anxiety and depressive symptoms in NF samples were
found to be higher than those with other chronic diseases,
including CAD and CA.
NF1 RELATED TO DEPRESSION
Depression among adults with neurofibromatosis type 1:
prevalence and impact of quality of life
(Clinical Genetics, 2015)
• NF1 carries significant psychosocial burden for affected individuals
• Aspects of NF1 that are especially challenging:
• Unpredictable nature of the disease
• Variability in severity of symptoms and medical complications
• Vulnerability to stigmatization due to highly visible and often cosmetically
disfiguring features
• NF1 patients are at increased risk for experiencing social and
emotional difficulties, including anxiety, depression, low self-esteem
and/or body image, social withdrawal, difficulty forming
interpersonal relationships, behavioral problems, and difficulties in
school.
• Depression in particular seems to be common among NF1
individuals. (over 55% of study participants had a significant level of
depressive symptoms)
Hospital
Course
HOSPITAL COURSE
• Day 1: Admitted to JHH
-Clinical Nutrition consulted for positive nutrition
screen: "poor appetite/oral intake for 5 days PTA”
-Diet order: Cardiac Diet, no restrictions
• Day 2:
-Critical Action Note: Large number of
ketones in urine
-Patient received 500mg Thiamine
-Patient received 40mEq KCl
-Dexamethasone 4mg q6h
-Clinical Nutrition visit
-PT visit
Nutrition
Assessment
NOTEWORTHY LABS
Lab
Day 2 Values (12/12)
Normal Range
Sodium
144
135-148 mEq/L
K-Serum
3.2 (L)
3.5-5.1 mEq/L
Chloride
106
96-106 mEq/L
CO2
20 (L)
21-31 mEq/L
Glucose
90
60-99 mg/dL
BUN
23 (H)
7-22 mg/dL
Creatinine
0.7
0.5-1.2 mg/dL
Albumin
4.0
3.5-5.3 g/dL
C-Reactive Protein
0.5
0.5 mg/dL
Mg
1.8
1.6-2.4 mg/dL
Phos
3.5
2.7-4.5 mg/dL
NOTEWORTHY LABS
• HgA1C: 5.6
• Urine Analysis: Large Ketones –(Day 2)
• Vitamin D: 6 ng/mL (L)
• H/H: 16.9/49.9 (H/H) –(Day 1)
CURRENT MEDICATIONS
Order Name
Order Summary Line
Docusate Enteral
100 mg PO bid,
routine
500 mg in NS Inj 100
ml IV q8h Routine
Thiamine Inj
Dexamethasone Inj
4 mg IV q6h
**Patient received KCl on Day 1, Approximately 800mL IVF
ANTHROPOMETRICS
•
•
•
•
•
Reference Height: 147.3 cm (4’10”)
Reference Weight: 58.4 kg (128.75 lbs)
Reference BMI: 26.916  Overweight
IBW: 45.5 kg
% IBW: 128.4%
UBW: 64.5 kg
% UBW: 95% (mild malnutrition)
• 10% unintentional weight loss in 6 weeks  severe
PHYSICAL EXAM
Café au lait spots
Slim legs
Noticeable hair loss
Muscle wasting present on arms
Poor Dentition
Dry Mouth
Skin graft on right side of her face, covering her right
eye
• No edema present
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INTAKE/DIGESTIVE PROBLEMS
• Endentulous (only has 2 front teeth; since 2011)
• Difficulty chewing
• Episodes of emesis
• “I don’t want to vomit, so I eat less.”
• Diarrhea
• Early satiety
• Food allergy: bananas
NUTRITION HISTORY
• No vitamins/minerals/supplements
• Decreased appetite and poor PO
intake
• Tray observed at bedside: skewed
sense of intake
• Unaware of usual PO intake
• Grocery shopping once a month with nephews
• Throws foods away quite often at home
NUTRITION HISTORY
• Difficulty preparing foods s/p
eye enucleation surgery in 2013
• Does not feel comfortable
cooking foods in oven or on
stovetop d/t vision loss
• Poor dentition prefers pureed
or soft foods
• Pureeing foods on her own takes
too long, so she often does not do
so
NUTRITION HISTORY DISCUSSION
• Spoke with patient about foods that she
gets when she grocery shops
• Discussed appropriate foods, including:
• Foods she can prepare on her own
• Softer consistency foods
• Spoke with AH about Meals on Wheels, and
she was very interested
• Small, frequent meals (~5-6/day) d/t poor
appetite, high-protein foods to prevent loss
of muscle mass
NUTRITION ORDERS
• Cardiac Diet
• No Restrictions
• Appropriate patient
for Room Service
ESTIMATED NEEDS
• 1050-1370 kcal/day
• Based on (Mifflin) REE: 1.0-1.3
• 18-23 kcal/kg
• 65-75 gm/day (1.1-1.3 gm/kg)
• Based on estimated weight of 59.5 kg
Energy needs initially conservative 2/2 potential increased
risk for refeeding (will aim for 15-20 kcal/kg per ASPEN
guidelines, advance when appropriate)
Admission weight likely decreased 2/2 dehydration (dry
mouth mucosa, poor PO intake, H/H elevated)
PES STATEMENT
Inadequate oral intake (NI 2.1) related to limited
ability for food preparation/cooking and grocery
shopping 2/2 vision loss; decreased appetite, and
poor dentition as evidenced by diet history per
patient report, severe unintentional weight loss (10%
in 6 weeks), ketones in urine.
GOALS
• PO intake equal or greater than 75% of
estimated needs
• Prevent further weight loss
• Resources available to assist with
adequate nutrition at home
• Preserve lean body mass
RECOMMENDATIONS
• SUGGEST:
• Change Cardiac Diet to soft consistency
• Agree with Social Work consult (will also ask re: living
situation, food handling, etc.)
• MVI + mineral and Vitamin D repletion
• Ensure Muscle (vanilla) TID, provides: 250 kcal, 13 gm protein
per 8 fl. oz.
• Consider Calorie Count in alternative nutrition and PO
option should fail
RECOMMENDATIONS
• Please monitor Phos, Mg, and K levels daily—until
stable, replete prn
• Please monitor weight for rapid weight gain
• >65 kg would be excessive weight--since >10% weight
increase could reflect volume overload
• Patient encouraged to contact RD with
questions/concerns regarding diet
• Will send snack (yogurt, fruit) as per patient
preference
Refeeding
Syndrome
ETIOLOGY
• Broadly encompasses a severe
electrolyte disturbance and metabolic
abnormalities in undernourished
patients and undergoing refeeding
whether orally, enterally, or parenterally.
• reflects the change from catabolic to
anabolic metabolism
VIDEO: PATHOPHYSIOLOGY
• Starvation can be categorized into different
levels of severity, but is overall defined as a
catabolic state where the body depends
on fat and protein metabolism rather than
carbohydrate utilization.
• https://www.youtube.com/watch?v=wWTw
AclznRw
PATHOPHYSIOLOGY
• Intracellular ions, specifically K, Phos, and Mg
are lost over time from the body during
starvation, although their measured
concentrations may remain falsely normal
mainly because of concurrent loss of total body
water that is also occurring during starvation.
• Sometimes fluid retention occurs, causing
increases in extracellular fluid volume.
• Reactivation of carbohydrate-dependent
metabolic pathways increases demand for
thiamine, which is a cofactor required for
cellular enzymatic reactions.
REVIEW OF LITERATURE
Refeeding Syndrome: Treatment considerations based on
collective analysis of literature case reports
(Nutrition, 2010)
• Conditions that increase the risk for RFS
• Depression in the elderly
• Clinical considerations in management of refeeding
syndrome based on reported cases
• Diagnosing RFS can be an arduous task for the physician
• Clinicians must be educated to recognize this syndrome
• Co-morbid conditions can complicate the timely
recognition of RFS
• Slow nutritional supplementation is an essential
component in the prevention of refeeding complications
INFLUENCE ON NUTRITIONAL STATUS
• Rate of feeding (low kcal to start)
• Hypophosphatemia
• Hyperglycemia
• Hypomagnesaemia
• Hypokalemia
• Fluid overload
• Vitamin deficiency (thiamine)
• Trace element deficiency
MANAGEMENT OF RFS
• Prevention and management includes:
• Identifying individuals at risk
• Thoroughly monitoring nutritional intake
• Nutrition Hx
• Start low and slow (15-20 kcal/kg)
• Fluid balance, sodium balance, and renal
function
• Fluid balance adjusted as needed to achieve adequate
hydration
• Daily weights measured to prevent fluid overload
MANAGEMENT OF RFS
• Prevention and management includes:
• Electrolyte Correction
• Repleted as needed; daily monitoring of K, Phos, and Mg
until stable.
• Acid-Base Correction
• High plasma anion gap metabolic acidosis such as lactic
acidosis can occur in thiamine deficiency
• Vitamin Supplementation
• Before feeding starts, high dose (200-300mg) thiamine
daily
• Also monitor for serum ferritin, vitamin B12, and folate
concentrations
Continued
Hospital
Course
HOSPITAL COURSE
• Day 3:
-Nursing Note: change in patient condition (unable to
state correct year, though it was 2020)
• Day 4:
-OT visit
• Day 5:
-Neuro Note: repleted K overnight for hypokalemia
-D/c Summary: suggestions for continued OT/PT 4x
a week
-Clinical Nutrition f/u visit
-SW visit with d/c recs
-AH was d/c to sub-acute rehab and resumed
outpatient care with JHH doctor
NUTRITION FOLLOW-UP
• Day 5
• 3-Day Calorie Count
• Day 1: Met goals
• Day 2: Some records missing
• Day 3: Only had B/L recorded, but increased cal/pro
• Conservative estimated needs 2/2 refeeding risk—increased
to 1250 kcal/day
• K: 3.2 (L)
• Phos: 2.3 (L)
• H/H: 12.8/38.2(WNL)
NUTRITION FOLLOW-UP
• Weight change (increase of 2.5 kg)
• Reference: 58.4 kg
• Current: 60.9 kg
• LOS I/O: +2.5 Liters
• Diet Change:
• Cardiac Diet w/ soft consistency
• Ensure Muscle (vanilla) TID
• Added meds:
• MVI + minerals (1/day)
• Ergocalciferol Enteral (5000IU q7days)
• Recommend: Ensure Muscle to Ensure Complete (additional
100 kcal), replete Phos, continue to monitor Phos and K until
stable
Importance of
Clinical Nutrition
Assessment
INVESTIGATING
• Working patients up
•
•
•
•
Disease state
Weight history
Labs
PMHx, PSHx
• Physical assessment
•
•
•
•
Wasting
Dentition
Wounds
Nails/Hair/Skin
• Nutrition/Social History
• What they eat, how much, how often
• Who shops/prepares the food
• UBW, Intake/Digestive Problems
Importance of
Multidisciplinary
Team
Communication
COMMUNICATING TO THE TEAM
• After Nutrition Assessment:
• Make team aware of refeeding risk
and prevention/management
recommendations
• Diet order changed d/t poor
dentition
• Nursing to encourage PO intake
• Calorie Count to monitor intake
• Social work for help with d/c plans
(food availability, Meals on Wheels,
etc.)
REFERENCES
• Cohen J.S., Levy H.P., Sloan J., Dariotis J., Biesecker B.B. Depression among
adults with neurofibromatosis type 1: prevalence and impact on quality of life.
Clin Genet. 2015; 1-6.
• Wang D.L., Smith K.B., Esparza S., Leigh F.A., Muzikansky A., Park E.R., Plotkin
S.R. Emotional functioning of patients with neurofibromatosis tumor suppressor
syndrome. Genet Med. 2012; (12): 977-982.
• Ferner R.E., Gutmann D.H. Neurofibromatosis type 1 (NF1): diagnosis and
management. Handb Clin Neurol. 2013; (53) 115:939-55
• Laycock-van Spyk S., Thomas N., Cooper D.N., Upadhyaya M.
Neurofibromatosis type 1-associated tumours: Their somatic mutational
spectrum and pathogenesis. Hum Genomics. 2011 Oct; 5(6):623-90.
• Boateng A.A., Sriram K., Meguid M.M., Crook M. Refeeding syndrome:
Treatment considerations based on collective analysis of literature case
reports. Nutrition. 2010 Feb; 26(2):156-67.
REFERENCES
• Crook, M.A. Refeeding syndrome: Problems with definition and management.
Nutrition. 2014; Volume 30(11): 1448-1455.
• Viana Lde A., Burgos M.G., Silva Rde A. Refeeding syndrome: clinical and
nutritional relevance. Arq Bras Cir Dig. 2012 Jan-Mar; 25(1):56-59.
• Khan L.U.R., Ahmed J., Khan S., and MacFie J. Refeeding Syndrome: A
Literature Review. Gastroenterology Research and Practice. 2011; Volume
2011. 6 pages.
• Maiorana A., Vergine G., Coletti V., Luciani M., Rizzo C., Emma F., Dionisi-Vici
C. Acute thiamine deficiency and refeeding syndrome: Similar findings but
different pathogenesis. Nutrition. 2014 Jul-Aug; 30(7-8): 948-952.
•
Einat Hershkowitz, Alon Reshef, Olga Munich, Bracha Yosefi, Arie Markel.
Thiamine Deficiency in Self-Induced Refeeding Syndrome, an Undetected
and Potentially Lethal Condition. Case Reports in Medicine. Volume 2014.
2014; 6 pages.