Third Edition, 2010

Nutrition Interventionsfor Children with SpecialHealth Care Needs

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ProductManufacturer

Energy (kcal/oz)

Comments

Specialized Products

NeoCate One +

( Powder) and

Neocate E028 Splash (liquid) and

Neocate Junior

Nutricia

30 Elemental: Impaired GI function, cow and soy milk allergy, multiple food protein intolerance. Protein from L-amino acids; fat from coconut, canola and high-oleic safflower oils; carbohydrate from corn syrup solids(powder), maltodextrin, and sucrose (liquid)

Pepdite JuniorNutricia

30 Semi-Elemental: Impaired GI function, malabsorption. Protein from hydrolyzed pork and soy (free amino acids and non-dairy hydrolysates); fat from coconut oil, canola and high-oleic safflower oils; carbohydrate from corn syrup solids

Peptamen JuniorPeptamen Junior with Prebio Peptamen Junior Fiber (with Prebio)Nestle

30 Semi-Elemental: Impaired GI function; protein from hydrolyzed whey; fat from MCT, soy, canola oils; carbohydrate from maltodextrin, cornstarch; Fiber from oligofructose, pea fiber (fiber version only); prebiotic is inulin; Peptamen Junior versions: unflavored, vanilla, chocolate, strawberry. All others: Vanilla

Peptamen Junior 1.5 with PrebioNestle

45 Semi-Elemental: Impaired GI function; protein from hydrolyzed whey; fat from MCT, soy, canola and tuna oil; carbohydrate from maltodextrin, and cornstarch; fiber from oligofructose; prebiotic inulin

Vivonex PediatricNestle

24 Elemental: Impaired GI function. Protein from L-amino acids; fat from MCT, soy oils; carbohydrate from maltodextrin, modified corn starch

ElecareAbbott

20-30 Elemental: Impaired GI function, allergy. Protein from L-amino acids; fat from high-oleic safflower, MCT oil and soy oil; carbohydrate from corn syrup solids

EleCare Unflavored and EleCare Unflavored with DHA/ARA appropriate for infants; both of these products and vanilla flavored version appropriate for children 1 year and older

Vital jr.Abbott

30 Semi-Elemental: Impaired GI function. Protein from Whey protein hydrolysate and sodium casienate; fat from interesterified canola and MCT oil; carbohydrate from maltodextrin and sucrose; fiber from fructooligosaccharides (3 g/L). Flavors: vanilla and strawberry

LipistartVitaflo

22-30 Fat malabsorption, long chain fatty acid oxidation disorders, chylothorax; 40% calories from fat ; 30% MCT, 10% LCT. Fractionated coconut oil, soy oil, AA rich oil derived from fungal sources, whey protein isolate; has adequate essential fatty acid; DHA/ARA; for children 12 months to 10 years of age

i:::r:·:::r:·:::r:·:::r:·:::r:·:::r:·:::r:·:::r:·:::r:·:::r:·:::NMJtlii~~·:~r~sr~~~:~~i~I :·:::r:·:::r:·:::r:·:::r:·:::r:·:::r:·:::r:·:::r:·:::r:·::::u Dale of Service: Authored By: Result Status: Documen1 Type:

Nutrjliow Brjef Note

4/25/2013 16:09 PDT Davis.Cheryl E,RD CD (4/25/2013 16:14 PDT) Aulh (Verified) Nutrition Progress No1e - Direct Entry

Attended mul1idisciplinary rounds with team 1his morning. Serum ammonia is s1able a162 mcmol/L wi1h trial of 20 kcal/oz Pregestimil (compared to 15 kcal/oz Pregestimil + 5 kcal/oz Polycose to decrease prolein provision). PO intake continues to be good, taking > 100 kcal/kg ad lib.

Home Care order 1or Pregestimil provided lo Seattle Children's Home Care; au1horiza1ion pending. Provided 2 sample cans of Preges1imil to bedside and reviewed preparation with family (packed formula scoops versus standard unpacked scoops). Jianhua will return for labs on Monday and clinic on Thursday.

-Cheryl Davis, RD, CD, CNSC 469-2712

Seattle Children's Hospital

PO Box 5371

Seat11e, Washington 98105-0371

NAME: XIE, J IANHUA DRACO DOB:

MRN:

9/16/2012

1275567

Print Date:

RRID: 9/2312015 13:52 PDT

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!eallie Children'5 HOSPITAL· RESEARCH' FOUNDATION

4800 Sandpoint Way NE, Seattle, WA 98105

Continuing Care Instruction/Discharge Summary

Patient Information

Name: XIE, JIANHUA DRACO

Med Rec#: 1275567

Admit Date: 4/9/2013 19:37:00 Discharge Date: 04/25/2013

Discharge Diagnosis Primary Diagnosis: end-stage liver disease

Surgical Procedures

Procedure(s) performed during this visit: Liver Biopsy Laparoscopic

Surgical Procedures Completed:

Last Vital Signs

Weight: 7.815 kg (04/23/2013 07:00 pm) Height: 61 em (04/22/2013 02:00 pm) Temperature: 36.7 DegC (04/25/2013 01:48 pm) Pulse Rate: 120 bpm (04/25/2013 01:48 pm)

Heart Rate Monitored: 121 bpm (04/23/2013 01:00 pm) SP02: 97 % (04/25/2013 01:48 pm) Respiratory Rate: 40 br/min (04/25/2013 01:48 pm)

Blood Pressure: 87/35 (04/25/2013 01:48 pm) Head Circumference: 42 cm (04/22/2013 02:00 pm) Pain Score: 0 (FLACC Score)

Vaccine(s) administered during this visit: Pentacel (04/23/2013), Recombivax HB Pediatric/Adolescent 5 mcg/0.5 mL intramuscular suspension (04/23/2013), Prevnar 13 (04/23/2013)'

Name XIE. JIANHUA DRACO MRN 1275567 Page:1

rotavirus vaccine pentavalent oral suspension (RV5) (04/24/2013)

Patient and Family Education

liver Biopsy

Call Gastroenterology Clinic at 206-987-2521 (Monday-Friday) or the Gastroenterology Resident on-call at 206-987-2000 (Evenings/Nights/Weekends) for any of the following:

• A fever higher than 100.4 F (38 C) • General malaise (feeling sick, tired or weak) • Yellow-colored skin or eyes (jaundice) • Nausea, vomiting, diarrhea • Blood in stool • Swelling in hands or feet due to excess fluid (edema) or excess fluid build up in stomach area (ascites)

• Stomach pain • Sudden change in biliary drain output (if drain present) • Unable to take medicines or pain not controlled by medicines

• Any questions or concerns

Diet:

• Progestimil 20cal/oz as tolerating.

Activity:

• As tolerating per home routine

Teaching and Self-Care Tips for Home:

• No aspirin-like medications (Ibuprofen, Motrin, Aleve) • Keep incision clean and dry. • May wash incision with mild soap and water, but do not soak or submerge incision for 2 days • Okay to shower day after procedure • Have your child and all household members wash hands often to help stop the spread of germs

Discharge Diet: Resume usual diet unless otherwise instructed

Name. XIE, JIANHUA DRACO MRN 1275567 Page:2

Activity: Resume pre-admission activity level unless otherwise instructed

Meas: per LJlscnarge ana pre~cr IIJ1IUIl Kecuru

Service Requested

Care Coordination, Home

Infusion/Enteral Therapy

With:

Outpatient Labs on Monday

and Thursday

Comments:

With:

Julie Wen

Comments:

Call for Appointment

With:

Requested Services

Details

Agency Name: Seattle Childrens Home Care, Agency Phone Number:

425-482-4000, Requested Therapies: Enteral Feeds, Parents Aware

Ordered By: Morrill, Gregory, RN

Follow Up

Address: When:

Address: When:

22635 NE Marketplace Dr, Suite Within 1 to 3 days

120 Redmond, WA 98053 (425) 898-7408 Business (1)

Address: When:

SCH Transplant Liver/Bowel MB.9.420 - Transplant Services, 5/2/2013

Name XIE, JIANHUA DRACO

4800 Sand Point Way NE Seattle, 16:00:00 WA 98105

MRN.1275567 Page:3

(206) 987-1800 Business (1)

Comments:

Future appointments: DICK, A - DAVIS, C - 1wk rtc; pre liver txp (05/02/13 04:00 pm, Transplant Clinic)

Important Phone Numbers

Children's Hospital Main Line: 206-987-2000 (or visit http://ww\\.seattlechildrens.org for more

in formation ) For other questions call:

Poison Control: 1-800-222-1222 (or visit http://www.1-800-222-1222.info for more information)

Toll-free Family Phone Interpreting Line: 1-866-583-1527

Call Children's Hospital in your own language. Not for medical emergencies.

Name XIE. JIANHUA DRACO MRN 1275567 Page:4

Amil 7~ 7nn

Discharge Home Medication List

Name: XIE, JIANHUA DRACO Birthdate: 09/16/2012 MRN: 1275567

I Scheduled Medications

cholecalciferol (cholecalciferol 1000 inti units/drop oral solution) 1.000 unit(s) by mouth once a day Last Dose Given: ---'-+-''-r''"'f'------L---'-'-''----''Y\

Next Dose to be Given at: ---+-I---J-:~--

multivitamin with minerals (SourceCF Pediatric Drops oral liquid)

Provider: Pokorney, Rachel Jane, MD

. '\ i .-, ,- r'i. "-Last Dose Given: 'v ~ L"::2 \.j U,Y) \ I mL by mouth once a day

Next Dose to be Given at: --,k\~\r-'+l-"\cb1'\~' __ I

spironolactone (spironolactone 5 mg/mL oral susp (SCH Provider: Pokorney. Rachel Jane, MD

compound» \ C'\ 12 mg by mouth 2 times a day Last Dose Given. l.\ )~~ '\l\r(\.

Next Dose l\l he Given at ~\~0 rr-p i/1("\ .. Comments: Target Dose. spllonolactone 5 mg/mL Ulal ,usp (SCH compound) I 5 mg/kg -l-12512013 IO.-l-3 51

Provider: Pokorney, "

ursodiol (ursodiol susp 25 mg/mL (SCH compd» ::10 mg by mlluth 3 times a day Las t Do se Give n: --'--'t--"!,-=,I---~~' '1'- \.

Next Dose to be Given at: ---+-t---?-~~--jr±;/'\ 1/1"· ..... · ....

.. End of /{eport""

Stop taking medications not listed here. Dispose of unused quantities responsibly.

BRI:\G THIS LIST TO YOCR :\EXT \ISIT 'YITH YOCR DOCTOR OR OTHER HEALTH CARE PROVIDER

Documenter Signature Time

Printed:041251I3 @16:33 by Ab,alon,oll. Angela T. RN

11111111111111111111111111111111111 Page' I uf I

Fllml :'\umber YOllS

Dale of Service: Authored By: Result Status: Documen1 Type:

DISCHARGE Summary

DISCHARGE SUMMARY XIE, JIANHUA DRACO

DOB: 09/16/2012 M -MR#: 01-27-55-67

4/25/2013 00:00 PDT Hsu,Evelyn Kanyu,MD (5/14/2013 17:42 PDT) Modified ln1erim/Discharge/Expira1ion Summary

DATE OF ADMISSION: 04109/2013 SUMMARY DATE: 04/25/2013

SENIOR RESIDENT: Heather Henne, MD

PRINCIPAL DIAGNOSIS: End-stage liver disease of unknown etiology.

DISCHARGE MEDICATIONS: 1. Spironolactone 12 mg p.o. 2 times a day. 2. Ursodiol 80 mg p.o. 3 times a day. 3. Cholecalciferol 1000 units p.o. once a day. 4. Multivitamin with minerals 1 ml p.o. once a day.

Jianhua will follow up in Transplant Clinic with Dr. Davis on 51212013. He will also get labs drawn every Monday and Thursday starting Monday 4/29/2013.

HISTORY OF PRESENT ILLNESS: Jianhua is a 7-month-old, previously healthy male who presented with about 1 week of bruising, pruritus and a few days of loose stools and spitting up, and was found to have significant fibrosis and cirrhosis on liver biopsy consistent with end-stage liver disease.

ADMISSION PHYSICAL EXAMINATION: Afebrile with normal vital signs. Ecchymosis noted on left cheek. Slight scleral icterus bilaterally. Grade 3/6 systolic ejection murmur noted at the left lower sternal border. Lungs clear to auscultation bilaterally. Abdomen full but soft and nontender. Liver edge palpable 3-4 cm below costal

margin, no splenomegaly. No scrotal edema or pitting edema of the lower extremities, but mild periorbital edema. Extremities warm and well perfused with normal capillary refill.

ADMITTING LABORATORIES/STUDIES: Normal venous blood gas, low serum sodium at 134, but the remaining electrolytes were normal. AST 107, ALT 91, alkaline phosphatase 1270. White count of 27, platelets 146, hematocrit 24.8, and then 20.6 on blood draw 2 hours later. INR 5.6, PTT 75, fibrinogen 60. Bilirubin conjugated 4.0 and unconjugated 10.3, ammonia 40. Normal urine.

Seattle Children's Hospital PO Box 5371

Seat11e, Washington 98105-0371

NAME: XIE, J IANHUA DRACO DOB: 9/16/2012

MRN: 1275567

Print Date:

RRID: 9/2312015 13:52 PDT

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Abdominal ultrasound significant for moderate intraperitoneal free fluid, mild diffuse gallbladder wall thickening, mild splenomegaly and trace bilateral pelviectasis_

HOSPITAL COURSE BY SYSTEMS: 1 _ FLUIDS, ELECTROLYTES, NUTRITION: Jianhua was started on Pregestimil p_o_ ad lib, and for some time was given Polycose 5 kcal/ounce to make up a total of 20 kcal/ounce_ This was for the purpose of reducing protein in Jianhua's diet for fear of hyperammonemia. The Polycose was later removed, and Jianhua was discharged on Pregestimil only, 20 kcal/ounce. He was started on spironolactone for portal hypertension, and this dose was titrated during admission to discharge dosing of 12 mg twice daily. He was also started oncholecalciferol and SourceCF multivitamin, given likely malnutrition with liver failure_ 2. GASTROINTESTINAL: Jianhua's liver enzymes were significantly elevated during admission but then trended downward. A biopsy on 411812013 revealed cirrhosis with marked bile duct proliferation and variable cholestasis. There was ongoing hepatocytic injury as well. Unfortunately, this biopsy did not reveal etiology of Jianhua's liver disease. He was placed on the transplant list on 4122/2013. His ammonia reached a maximum serum level of 123, but then decreased and was stably in the 60s by the day of discharge. A number of labs were drawn to rule out various causes of liver failure, and all of these have been normal or negative. 3. CARDIORESPIRATORY: Stable, no issues. 4. INFECTIOUS DISEASE: Jianhua completed a 5-day course of Zosyn, as well as 3 days of micafungin, for possible peritonitis given his ascites. His blood, stool and urine cultures remained negative. He also had an echocardiogram that was negative for any vegetations. 5. HEMATOLOGY: Due to his coagulopathy secondary to liver failure, Jianhua received transfusions of cryoprecipitate, packed red blood cells, and fibrinogen on hospital days 2, 3, and 5, respectively. He also received a transfusion of packed red blood cells on day #3 of admission. His INR crept upward to 2.5 on the day of admission, which is below transfusion threshold INR of 4, and it was felt that he would not meet this threshold before receiving his liver transplant.

DISCHARGE PHYSICAL EXAMINATION: Jianhua appeared well and in no apparent distress. He continued to have a distended abdomen, but it was soft and nontender. His spleen was palpable 4 cm below the costal margin, and his liver was nonpalpable. He had trace scleral icterus bilaterally, but no other jaundice, and his pruritus had improved.

Electronically Authenticated by Evelyn K Hsu, MD 05/1412013 05:42 P

Evelyn K Hsu, MD , Attending Physician, Gastroenterology Reviewed by Christopher C Denton, MD' 04126/2013 06:02 P

Dictated By Christopher C Denton, MD' , Resident Physician

CCD/jkj Doc #2953012 d: 04/26/2013 12:03 P 1: 04/2612013 01 :04 P (1568335-)

Seattle Children's Hospital

PO Box 5371

Seat11e, Washington 98105-0371

NAME: XIE, J IANHUA DRACO

DOB: 9/16/2012 Print Date:

MRN: 1275567 RRID:

9/2312015 13:52 PDT

25788848

289 of 2081

cc: cc Pediatric Assoc Redmo Julie E Wen, MD

Seattle Children's Hospital

PO Box 5371

Seat11e, Washington 98105-0371

NAME: XIE, J IANHUA DRACO

DOB: 9/16/2012

MRN: 1275567

Print Date:

RRID:

9/2312015 13:52 PDT

25788848

290 of 2081