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LUCILE PACKARD CHILDREN'S HOSPITAL - HOUSESTAFF DIRECTORY - (650) 497-8000

LUCILE PACKARD CHILDREN'S HOSPITAL - HOUSESTAFF DIRECTORY - (650) 497-8000

LUCILE PACKARD CHILDREN'S HOSPITAL - HOUSESTAFF DIRECTORY - (650) 497-8000

LUCILE PACKARD CHILDREN'S HOSPITAL - HOUSESTAFF DIRECTORY - (650) 497-8000

LUCILE PACKARD CHILDREN'S HOSPITAL - HOUSESTAFF DIRECTORY - (650) 497-8000

LUCILE PACKARD CHILDREN'S HOSPITAL - HOUSESTAFF DIRECTORY - (650) 497-8000

LUCILE PACKARD CHILDREN'S HOSPITAL - HOUSESTAFF DIRECTORY - (650) 497-8000

LUCILE PACKARD CHILDREN'S HOSPITAL - HOUSESTAFF DIRECTORY - (650) 497-8000

LUCILE PACKARD CHILDREN'S HOSPITAL - HOUSESTAFF DIRECTORY - (650) 497-8000

LPCH Fellows Guide Phone prefixes If 1, 3, 4, 5 then dial 72x-xxxx If 6 then dial 73x-xxxx If 7,8 then dial 49x-xxxx *67 blocks your number for outgoing calls

Phone calls

1) From the Operator a. Ask who the GI attending is for the patient

(ask them to call that MD if they are available)

b. If during rounds, etc – ask if urgent. If not urgent, operator can page you the parent number to call back (the operator will inform the family that you will call them back)

c. If outside MD, wanting to transfer the patient – try to reroute through the transfer center

2) From the Transfer Center (aka Dispatch) a. Calls will come from outside hospitals to

Dispatch – sometimes they speak first with the attending, sometimes the call comes first to the fellow

b. Use this line to contact outside hospitals, MDs, ERs etc if a transfer is at all possible

c. They will connect you to bed control, figure out insurance, get nursing signout, etc

3) From parents

a. Create Epic phone encounter (and forward to the primary GI MD)

b. Call the ED to warn them a kid is coming (you can direct care, labs, etc)

c. Key Reasons for admission i. Fever with Central Line

1. All patients must go to ER – local or LPCH

2. CBCD, CRP, Blood culture, Chem 15

3. Vanco and Ceftaz (check prior resistant bugs, fungal?)

ii. Fever with a transplant 1. If <3months post transplant,

likely admit 2. If >3months and clear source,

consider CBC, LFTs at PMD or local lab and follow closely

3. If pretransplant (i.e. BA), likely admit re: cholangitis, remember to get glucose and coag panel

4) Foreign Body Calls a. Urgency

i. EMERGENT for all esophageal batteries

ii. Urgent procedures for esophageal foreign bodies and GI bleeders (severe bleeders need PICU stabilization first!)

1. May try to schedule in APU/OR next day…but involve anesthesia early

iii. Non urgent for foreign body in stomach >1month

b. Ask ER to repeat Xray if needed c. Ask NPO STATUS, allergies, recent illness d. Call OR front desk (given details of case,

they create a pre-cert) e. Call Anesthesia (OR front desk will give

you pager) f. Head to ER to get brief H&P and consent g. Go to APU to set up tower, get supplies

(emergency cart) Admissions

1) From ER a. Tell ER who is the attending, green team,

etc b. Sign-out to residents

2) Direct admission a. From Clinic or Home

i. Call Bed Control – name, MR, DOB, attending, referring MD, length of stay, reason for admission, isolation??

ii. (Try to dictate your clinic note as an H&P)

iii. If stable, parent/child walk over to admitting

iv. Sign-out to Green Team

Outpatient (or Follow-up) Appointments

1) Email medicinespecialities@stanfordchildrens.org – give name, DOB, provider, urgency etc (the schedulers will contact family to confirm appointment)

a. If URGENT, Cc Hope Gumagay, Jessica King, Megan Christofferson

2) Call front desk (to add someone quickly to your clinic afternoon)

SSU – Short Stay Unit (Infusion Center)

1) What they do a. Lab draws from PICCs, central lines b. Meds – infliximab, pentamidine, cidofivir,

alteplase, IVIG, transfusions 2) Calls

a. OK to start b. Asking to place orders

i. Ask who the attending is…if they are here they can write orders

ii. Go to APU schedule in Epic and find patient

iii. Add order set Procedures

1) Pre-op H&P clinic visit, consent 2) Precertification

a. Outpatients to Italo b. Inpatients case request order in Epic c. Remember complete medical history

d. You will be given day of procedure (never tell patient TIME)

i. Patients get call ~4pm day before procedure with details of NPO, place/time to arrive

ii. Cleanout instructions (GI nurses can do in clinic or they call family)

3) Pre op Orders (enter night before) a. “Pre op Admit (Outpatient)”

i. Find correct CSN – can look at providers schedule

ii. Check lidocaine-tetracaine patch, NS lock, ?Urine preg

iii. Add labs you may need drawn (will need ICD9 code)

iv. Liver biopsy 1. If Roux-limb – CIPRO Q12

x24 hours (first dose at start of procedure)

2. Hold ASA/Dextran/Persantine

3. If splenomegaly, check plts (give if on ASA or plts<50)

4. Add Misc Test (write in EBV qualitative PCR)

5. Add CMV qualitative PCR 4) H&P update (if clinic visit >24hour prior to

procedure) a. Update in Epic at Ford Family Surgery

Center 5) Procedure paperwork

a. Path form (write GOLD if transplant liver biopsy, results by next day noon, if weekend write ULTRA for next day results)

b. EGD/colo form (write down scope, LES, findings)

c. Liver Bx form (write down pre HR, plt, INR, HCT)

i. Needle Gauge - 18 gauge for babies. 16 gauge for bigger kids.

d. Discharge Instructions 6) Post op Orders

a. “Post Op Same Day Surgery” b. “Liver Biopsy Post Procedure”

i. Enter your name and pager in transfer order (MD/RN)

ii. Choose 4hr HCT iii. Edit diet order to regular

PEG placement notes:

1) No PEG in patients with prior abdominal surgery 2) Sizing: little 1-2y/o 18Fr 3) Pre-op and post-op Abx (Ancef) 4) Pre-op labs 5) Admit orders

a. Open to gravity x 3hrs b. Clamp x 4hrs c. Pedialyte to full rate d. Then change over to home formula (or

slower if necessary) e. Tylenol ATC x 24 hours and the PRN

Attending On Call Preference: • Bass: Text first. If no answer, page. Then if no

answer, call cell. • Berquist: Text or call cell during the day. After

10pm call home phone. • Burgis: Text during the day. At night page or call

cell. • Castillo: Call cell. • Cox: Text first. If no answer, then call cell. Then if

no answer, page. • Hurwitz: Text first. If no answer, call cell. • Kerner: Text or call during the day. At night page.

If no answer, call cell. • Khavari: Text first. Then if no answer, page. • Park: Text first. If no answer, call cell. • Yeh: Text first. If no answer, call cell.

Current Practice Guidelines Variceal Bleeding

• IV access (multiple peripheral lines, Broviacs are too narrow for resuscitation)

• Type & cross (always stay ahead of the game, order the next unit while one is already being transferred)

• Check Hct Q4h, coags, and platelets • Start octreotide and protonix drips • Blood cultures & antibiotics • If you decide to scope, have a Blakemore tube handy.

IBD

• Initial Labs: CBC, ESR, CRP, Ch23, coags, (Kerner also likes zinc levels). Place PICC if you are suspicious for IBD

• Stool studies: Guaiac, stool cultures, C. diff, O+P, cryptosporidium, consider fecal A1AT, calprotectin

• Imaging: UGI/SBFT, CT/MR enterography • Immediately after colo: order TPMT activity, consider

ordering Prometheus panel (especially if you’re on with Berquist)

• Inpatient management: Solumedrol, NPO/TPN/IL, or low-residual diet or elemental diet, +/- Vit K

• Outpatient steroid management: Prednisone (2 mg/kg/day up to 60 mg QD, then taper), budesonide (entocort), cort foam

• Immunomodulators: 6MP (1.5 mg/kg/day for normal TPMT activity, check CBC and Ch23 in 2, 4, Q8wks) +/- ASA (PO/PR), methotrexate (15 mg/m2 PO/SQ Qweek)

• Biologics: Remicade (5-10 mg/kg, wks 0, 2, 6, Q8wks), Humira (Q2wks)

• Other supplements: Folate, MVI, Ca (Viactiv chocolate chewables), Vit D, PPI with steroids

Model IBD Care—a Guideline for Consistent Reliable Care: diagnostic and therapeutic interventions that are appropriate and recommended for a very large percentage of children and adolescents with Crohn’s disease and ulcerative colitis.1

Complete diagnostic and initial evaluation:

• CBC, ESR, and serum albumin • esophagogastroduodenoscopy with biopsy and

colonoscopy with biopsy • imaging of the small intestine (upper GI and small

bowel series; or CT scan with oral and IV contrast; or MRI with contrast; or capsule endoscopy)2

• other studies as indicated

_______ 1 The guidance in this document does not indicate an exclusive course of treatment or serve as a standard of medical care. Variations, taking into account individual circumstances, may be appropriate. 2 In patients with left-sided ulcerative colitis (distal to the splenic flexure) in whom the terminal ileum is normal on colonoscopy, not performing small bowel imaging and/or esophagogastroduodenoscopy is also consistent with the ImproveCareNow Model of Care. N213 Given Courtyard | UVM College of Medicine | Burlington, VT 05405-0068 | info@improvecarenow.org | www.improvecarenow.org

Extent of disease: documentation of disease location (esophagus, stomach, duodenum, jejunum, ileum, right colon, transverse colon, left colon, rectum, perineum)

Crohn’s disease phenotype: based on the Montreal classification (non-stricturing, non-penetrating; penetrating; or stricturing)

Severity: Physician Global Assessment (Quiescent, Mild, Moderate, Severe)

Visit frequency: it is recommended that each patient be examined and evaluated at least once every 6 months (≤ 200 days)

Treatment with 5-ASA: When using the following medications, use the recommended doses:

1. Mesalamine 80 (60-100) mg/kg/day up to 4.8 g/day for active colitis.

2. Mesalamine at least 30 (30-100) mg/kg/day up to 4.8 g/day for maintenance of quiescent or inactive colonic disease.

3. Sulfasalazine 70 (50-80) mg/kg/day up to 4 g/day for active colitis.

4. Sulfasalazine at least 25 (25-80) mg/kg/day up to 4 g/day for maintenance of quiescent or inactive colonic disease.

Treatment with prednisone:

1. Prednisone is indicated for induction of remission. Long-term treatment with prednisone can induce significant adverse effects and has not been shown to be effective for maintenance of remission.

2. To induce remission the dose of prednisone is 1 mg/kg/d, rounding up to the nearest 5 mg, up to 40 to 60 mg per day, PO for 1 to 4 weeks.

3. Taper prednisone and discontinue it within 16 weeks after treatment was begun.

a. Prednisone resistance is defined as an inadequate improvement after 2 to 4 weeks of treatment with prednisone.

b. Prednisone dependence is present when a patient, who initially improves in response to prednisone treatment, develops a recurrence when the dose is being tapered or within 6 months after prednisone is discontinued.

Treatment with thiopurines:

1. Prior to initiation of a thiopurine, determine thiopurine methyltransferase (TPMT) genotype or phenotype.

2. Choose a starting dose of azathioprine or 6-mercaptopurine (6MP) based on TPMT. If there is:

a. absent or very low TPMT activity, do not use a thiopurine.

b. intermediate TPMT activity, start azathioprine at 1.0 to 1.5 mg/kg/day or 6MP 0.5 to 0.75mg/kg/day.

c. normal to high TPMT activity, start azathioprine at 2.0 to 3.0 mg/kg/day or 6MP 1.0 to 1.5 mg/kg/day.

3. For a maintenance dose of thiopurine use either at least the starting dose as defined above, or base the dose on blood concentrations of thiopurine metabolites or evidence of toxicity.

4. Monitor CBC and ALT for evidence of toxicity. 5. For patients treated with a thiopurine, when disease

is moderately or severely active it is recommended that the 6-TGN level be measured (if not done in the previous 90 days).

Treatment with methotrexate:

1. For induction of remission the recommended dose of methotrexate is 15 mg/m2, up to 25 mg, IM, subcutaneous or oral once a week.

2. For maintenance of remission the recommended dose of methotrexate is 10 to 15 mg/m2, up to 15 to 25 mg, IM, subcutaneous or oral once a week.

3. Folic acid supplementation is recommended in a dose of 400 micrograms or 1 mg per day.

4. Monitor CBC and ALT for evidence of toxicity.

Treatment with infliximab: 1. It is recommended that a skin test (PPD) and/or a

chest radiograph for tuberculosis be obtained before initiation of infliximab therapy.

2. For induction of remission it is recommended that infliximab 5 mg/kg IV (or rounding up to the nearest 100mg) be used as an initial dose, with repeat doses of 5 mg/kg IV 2 and 6 weeks later (0, 2, 6 weeks).

3. For initial maintenance of remission it is recommended that infliximab 5 mg/kg IV (or rounding up to the nearest 100 mg) be given every 8 weeks.

4. For patients treated with infliximab, when disease is moderately or severely active it is recommended that the infliximab trough level be measured (if not done in the previous 180 days).

Nutritional and Growth Assessment

Status Definition Nutritional status at risk

Weight percentile changed lower by one isobar or Weight stable (no gain) or 1% to 9% loss (involuntary) Body mass index <10th percentile for age (Adjust for prednisone treatment)

Nutritional failure

Weight percentile changed lower by two isobars or Weight loss ≥ 10% Body mass index <3rd percentile for age (Adjust for prednisone treatment)

Nutritional status satisfactory

Not at risk or failure

Growth status at risk

Height percentile changed lower by one isobar or Height percentile <10th percentile for age or Height velocity <10th percentile for age

Growth failure

Height percentile changed lower by two isobars or Height percentile <3rd percentile for age or Height velocity <3rd percentile for age

Growth satisfactory

Not at risk or failure

Pediatric Acute Liver Failure (PALF) Definition: (1) no known evidence of chronic liver disease,

(2) biochemical evidence of acute liver injury (elevated AST/ALT), and (3) hepatic-based coagulopathy defined as INR ≥ 1.5 not corrected by vitamin K with hepatic encephalopathy or INR ≥ 2.0

50% of children with PALF wither die or need liver transplant! If listed, highest priority of status 1A.

Physical exam: Hepatic encephalopathy grades: I –

altered sleep/wake cycle; II – confusion, asterixis; III – unresponsive to verbal, hyperreflexia; IV – coma

Signs of chronic liver disease: big spleen, palmer erythema; Big tender liver suggests acute process; small, nodular liver suggests cirrhosis

Labs: Need HIV and 2 documented blood types for stat transplant listing • Glucose, Chem-22, CBC, ammonia, INR, Factors

V, VII, VIII before FFP if possible. Factors helpful in distinguishing DIC from ALF. V, VII made in liver, VIII made in endothelial cells, so in DIC ALL will be low, in ALF only V/VII will be low.

Etiology: 50% indeterminate, acetaminophen toxicity

(14%), metabolic (10%), autoimmune (6%), infectious (6%), drug toxicity (5%), and other (11%)

Drug history: acetaminophen level, urine toxicology screen, herbals • Tylenol: one time toxic dose: 250 mg/kg child, 7.5-

12 grams adult; chronic use: 150 mg/kg over 2 days

o NAC protocol for Tylenol: 140 mg/kg load, 17 doses of 70 mg/kg Q4 PO/IV

Ischemic: ECHO, US with Doppler for hepatic vein issues Metabolic: older children: ceruloplasmin for Wilson’s,

alpha-1 antitrypsin phenotype; younger children: urine succinylacetone for tyrosinemia, ferritin for neonatal hemochromatosis/hemophagocytic lymphohistiocytosis, serum amino acids, urine reducing substances for galactosemia, acylcarnitine profile for fatty acid oxidation defects, lactate/pyruvate ratio for mitochondriophaties • Wilson’s disease: high bilirubins (>20) and mixed

direct/indirect due to hemolysis, low alkaline phosphatase and phosphorus due to renal injury from Cu. Dx: Kaiser-Fleisher rings, low ceruloplasmin, high 24 hour Cu urine content

• Neonatal hemochromatosis: at least 2nd pregnancy, low glucose and high INR at birth with normal AST/ALT. Dx: high ferritin (>1000), excess Fe in extrahepatic organs (pancreas/heart on MRI or salivary glands on buccal biopsy). Tx: exchange transfusion, IVIG but often need liver transplantation

Infection: Hepatitis A/B/C, EBV, CMV, consider herpes/HHV, enterovirus, parvovirus, myco, adeno, TORCH Autoimmune: ANA, anti-smooth muscle antibody, anti-liver kidney microsomal antibody. Tx: 2 mg/kg IV Management: FEN/Renal: Need access for glucose, blood products, so

central line is key. Q2-4 glucoses. Aim for GIR 5-7

mg/kg/min [%D x cc/hr ÷ 6 x wt (kg)]. Watch for Na/ammonia/fluid overload from FFP. Consider CVVH early if urine output dropping off, will help with ICP issues and ammonia.

Liver/Heme: Goal to keep INF < 2.5-3.0 to reduce risk of

bleeding, FFP dose 10 ml/kg Q4. IV vitamin K for 3 days. Falling enzymes in a child with low glucose of high INR is a sign of liver death and not recovery! NAC shown in adults to help in early ALF for ALL etiologies of ALF, so use it! 150 mg/kg/day IV drip. Often too coagulopathic to perform liver bx safely.

Neuro: Watch ammonia’s and treat with either lactulose ( ̴

1 ml/kg/dose up to QID, must stool out to work) or neomycin/rifaximin (400 mg PO Q8). NO benzodiazepines (ativan/versed/Benadryl) because they are metabolized by the liver and they won’t wake up! Use fentanyl/remifentanyl/propofol for sedation. Watch for cerebral edema and consider head CT with big changes in mental status ot look for bleeding, edma. Herniation is how most children die.

ID: Broad spectrum antibiotics with the first sign of fever.

Pediatric Liver/Intestinal Transplant Baseline questions for parents: type of tx, underlying

diagnosis, sick contacts, viral symptoms, appearance of child, trustworthiness of the family, immunosuppression, presence of spleen, recent hospitalizations.

Fever: ≥ 100.4 or 38 – worry about infection, rejection,

drug effect. • If have central line: needs admission for 48 hour

rule out. Draw CBC, CRP, blood culture from each port, chem-23, EBV/CMV PCR. Consider viral DFA, CXR, urine culture, stool studies based on symptoms, age. Tx: broad spectrum antibiotics (Vancomycin, cephalosporin).

• If no central line: ask about sick contacts, viral symptoms, appearance of child, trustworthiness of the family, immunosuppression, presence of spleen, past history of bad infections. When in doubt, send them to the ED for evaluation.

Vomiting/Diarrhea – worry about infection, rejection, obstruction, feeding intolerance.

• If intestinal transplant, likely will need to go to ED because they get into trouble quickly. If has stoma, ask about output and how compares to baseline (normal is 40 cc/kg/day). Draw CBC, CRP, chem-23, EBV/CMV PCR, stool studies (bacterial cx, viral cx, C. diff, cryptosporidium, rotavirus, ova/parasite, isospora/cyclospora). If has low albumin, send alpha-1 antitrypsin of stool for

protein losing enteropathy. Consider AXR/abdominal CT for signs of obstruction. Tx: consider NS bolus, 5% albumin, correcting electrolytes (K, Mg, HCO3), admission.

• If liver transplant, basic pediatric guidelines for dehydration, but watch immunosuppression levels as they can go either low or high.

GI/stomal bleeding

• See GI bleeding sheet for stabilization, if localized stoma bleeding, apply pressure, consider Surgicel, silver nitrate, but call surgery for stitch.

Hyperkalemia – seen with tacrolimus toxicity, acidosis, renal insufficiency, spironolactone

• Level 5-5.9: stop K in IVF’s/TPN, give NS bolus, EKG, if HCO3 < 20, give HCO3

• Level 6-6.5: above plus IV lasix 1mg/kg • Level ≥ 6.5: above plus EKG changes (peaked T

waves), kayexalate, insulin/glucose, NaHCO3, calcium gluconate

Hypertension – seen with tacrolimus, steroids, volume overload, pain, renal insufficiency

• Lower immunosuppression if tolerated, treat volume overload with diuretics, manage pain

• Amlodipine: > 6 years of age start at 2.5 mg, < 6 years, consult renal

Common medications used in transplant • Tacrolimus/cyclosporine (calcineurin inhibitors

CCI): 1st line maintenance immunosuppression. Side effects: hemolytic anemia, headache, hypertension, tremors, low Mg, high K, high glucose

o Cyclosporine – not used as much. Side effects: same as with tacro + hirsutism. Should be on low dose prednisone if cyclosporine is only immunosuppression.

• Sirolimus: used as 2nd line immunosuppressive agent, has some EBV activity. Side effects: mouth sores, hypertension.

• Cellcept: used as 2nd line immunosuppressive agent. Side effect: diarrhea, abdominal pain, pancytopenia.

• Valganciclovir: used as EBV prophlaxis 1st year out, Qday (15 mg/kg, max 450 mg) for prophlaxis, BID if have + EBV PCR. Side effects: pancytopenia.

• Septra: used as PCP prophlaxis 1st year out. Side effects: may increase LFT’s and then switch to inhaled/IV pentamidine which is Qmonth.

• Cytogram: proven to treat CMV but we also use for EBV. 100 mg/kg/dose IV qweek. Side effects: infusion reaction, but don’t need to pre-treat.

• Antithymocyte globulin: used in induction when CCI cannot or to treat bad rejection. 1.5 mg/kg/day for 7-14 days. Watch for infusion reaction, pre-treat with Tylenol (10 mg/kg), Benadryl (1 mg/kg),

methylprednisolone (2 mg/kg). Side effects: leucopenia (reduce dose by 50% or hold), pancreatitis, increased liver enzymes.

• Methylprednisolone: used in induction and rejection. "Recycle" = Day 1: 10 mg/kg up to 1 gram, then each day one dose of 5 mg/kg, 4 mg/kg, 3 mg/kg, 2 mg/kg, 1 mg/kg (hold). Side effects: hypertension, high glucose, mood swings. Give acid blockade and nystatin.

• OKT3: for severe steroid resistant rejection. < 30 kg = 2.5 mg IV for 14 days, > 30 kg = 5 mg. Pre-treat with Tylenol (10 mg/kg), Benadryl (1 mg/kg), methylprednisolone (2 mg/kg). First dose in PICU due to anaphylaxis.

Pediatric and Adult Intestinal Transplant Protocol (June 2012)

I. PRE TRANSPLANT

A. No donor pretreatment B. Draw pre-transplant blood culture (all lumens) C. HLA Antibody Screen with T-Cell/B-Cell flow cytometry

to be sent at listing then 1 month after blood transfusions if >1yo in pre-transplant patients

II. IMMUNOSUPPRESSION

A. Induction- Timing of induction agent to be at surgeon’s discretion

1. Isolated a.CAMPATH 0.4 mg/kg given over 2 hrs (max dose 30 mg) with pre medications:

i. Solu-Cortef 5mg/kg (max 100mg) ii. Tylenol 15 mg/kg (Adults: 500 mg) iii. Benadryl 1 mg/kg (Adults: 50 mg)

2. Combined/Multi-visceral b.Thymoglobulin 1.5-2mg/kg

i. Solu-Cortef 5mg/kg (max 100mg) ii. Tylenol 15mg/kg (Adults: 500mg) iii.Benadryl 1mg/kg (Adults: 50mg)

B. Post-Transplant 1. Prograf: 0.05 mg/kg IV over 24hrs

**DESIGNATE 1 LUMEN TO BE USED FOR IV PROGRAF AND REMAIN UNUSED FOR LAB DRAWS THROUGHOUTADMISSION, ENSURE CLEARLY LABELED FOR NURSING STAFF TO PREVENT FK LEVEL ERRORS** a. Start Prograf within 72 hours of the transplant at

the surgeons' discretion. b. Goal level 12-15 mg/ml

c. When appropriate, start oral/enteral at 0.2-0.4 mg/kg BID-Adjust to maintain target level 10-12 for 3 month then 8-10 until 1 year post op

2. FOR ISOLATED ONLY: Prednisone/Solumedrol: 1mg/kg/day (max 20 mg/day)

3. FOR COMBINED ONLY: Thymoglobulin 1.5-2mg/kg x 2-3 doses depending on response and patient status

4. Consider starting second agent at three weeks post op if isolated or if episode of rejection within first 3 months of transplant.

5. Please send DSA on POD 1 then yearly/PRN rejection 6. Cylex studies weekly in initial post op period

III. ANTIBIOTICS/ANTIVIRALS A. Zosyn x 1-2 weeks (If previously infected and treated with

Zosyn, use Meropenem) B. Anidulafungin

1. Peds: 3mg/kg IV one time first dose, then 1.5 mg/kg IV daily.

2. Adults: 200mg IV first dose, then 100mg qdx14d C. Gancyclovir (DHPG):

1. Peds: 5 mg/kg q12h until diet advanced, then switch to oral Valgancylovir 15mg/kg po BID

2. Adult: DHPG 5mg/kg until diet advanced, then switch to Valgancyclovir 900mg daily

a. monitor WBCs-may need to be decreased to 450mg daily

D. Cytogam (Peds only): First dose within first week; 150 mg/kg/dose weeks 0/ 2/4/6/8 then 100mg/kg/dose weeks 12/16

E. PCP Prophylaxis: 1. Peds: Pentamidine monthly x 2-3 months then transition

to Septra 5mg/kg(max 80mg or SS tab) daily Mon/Wed/Fri.

2. Adults: Septra 1 SS tablet Mon/Wed/Fri

IV. ANTICOAGULANTS

A. Peds: None B. Adults: Case by case

V. GI MEDS/ENTERAL FEEDS A. Pediatrics:

1. Protonix 1 mg/kg BID IV; follow gastric pH daily (goal 6-8); start Protonix drip if unable to reach within 24hrs.

2. Feed initiation: a. Pedialyte: start POD 3-evaluate tolerance before

advance to formula b. Pediatric Vivonex x 4wks. Start with dilute

strength (1/2 to start). c. Continue TPN until enteral/oral feeds provide 50%

nutritional goal 3. Imodium 0.5-2 mg/ kg/d (capsules added to feed)

started once ostomy output 35-50ml/kg/day AND no rejection

B. Adults: 1. Protonix 40 mg/d IV, or PPI PO 2. Feed initiation: Tolerex 1/4-1/2 strength x 2 weeks 3. Imodium – Start with 4mg TID added to feeds once

ostomy output >1L/day AND no rejection

VI. SURVEILLANCE A. Close monitoring of ostomy output-typically 30-

50ml/kg/day or 1 liter for adults; increased output concerning for rejection vs infection; if >20ml/kg in 8hrs consider holding feeds, assess fluid status

B. Enteric biopsies SEND ULTRASTAT 1. 2-3x week x 1st month, 1x week x 2 months then PRN

thereafter C. Stool pH and stool reducing substances daily to monitor

absorption

D. EBV-PCR / CMV – PCR q 2 weeks first month, then q month x 1yr

E. Immune function test (Cylex) as needed when concern for infection/rejection

V. REJECTION

A. Mild: Solu-Medrol bolus 10mg/kg (max 1 gm) 1-3 boluses B. Moderate:

1. Solu-Medrol bolus 10 mg/kg (max 1 gm) 2. Steroid recycle: (max 1gm)

a. Day 1: 5 mg/kg b. Day 2 4 mg/kg c. Day 3: 3 mg/kg d. Day 4: 2 mg/kg e. Day 5: 1 mg/kg (continue this dose until

appropriate to wean C. Severe: Thymoglobulin 2mg/kg/dose x 2-5 doses (Duration

to depend upon clinical response) 1. PreMed with Tylenol/Benadryl and Solu-Cortef 2. Solu-Cortef dosing:

a. Peds: 5mg/kg(max 100mg) first dose then 3mg/kg(max 75mg) for subsequent doses

b. Adult: 125mg per dose D. For all grades of rejection:

1. increase Prograf dose (Target level 10-12) 2. re-biopsy at 48 hrs to assess treatment efficacy 3. consider biopsies of the PROXIMAL graft if clinical

picture suggests rejection but ileal biopsies are negative 4. for Thymo -resistant rejection, consider REMICADE

5mg/kg/wk x 4 wks

GI and Transplant Medication Cheat Sheet

Reflux

Zantac 2-4mg/kg/day divided BID (suspension 15mg/ml)

Prilosec (omeprazole)

1mg/kg/dose daily or BID (suspension 2mg/ml)

Prevacid (lansoprazole)

1mg/kg/dose daily or BID (solutab 15mg and 30mg)

Protonix (pantoprazole)

1-2mg/kg/day IV daily or BID Continuous drip 2mg/kg/day divided over 24hours or 0.1mg/kg/hour

Nexium (esomeprazole)

<20kg 10mg daily, >20kg 10-20mg daily

Reglan 0.1-0.2 mg/kg/dose up to QID Erythromycin 20mg/kg/day divided TID, low dose 3-

5mg/kg/dose TID Constipation

Milk of Magnesia

<2 years: 0.5ml/kg/dose 2-5 years: 5-15ml daily or divided 6-12 years: 15-30ml daily or divided >12years: 30-60ml daily or divided

Miralax 0.5-1 gram/kg/day starting dose Lactulose 1-3ml/kg/day divided BID Magnesium citrate

1 oz per year of age or 0.5oz per kg up to 10oz Can give 3 days in a row

Dulcolax 3-12 years 5-10mg PO or PR daily >12 years: 5-15mg PO (max 30)

PR < 2 years 5mg, 2-11 years up to 10mg Golytely Goal 4L or clear stools, Start 75-150ml/hr thru

NGT, increase by 75ml/hr to goal 400ml/hr (lower in small kids ~250ml/hr)

Miscellaneous

Periactin 0.25mg/kg/day divided BID (suspension 2mg/5ml) Start with first dose at bedtime, titrate up 2-6 years 2mg at bedtime, then BID 7-14 years 4mg at bedtime, then BID

Loperamide 1-3mg/kg/day (higher doses in short gut) – up to 2 cap TID - QID

Glutamine 0.4-0.5mg/kg/day divided TID Imodium 0.5-2mg/kg/day Liver Failure

Octreotide 1-2mcg/kg/hour gtt (max 50 mcg/hr) or 1 mcg/kg/dose IV/subQ BID

Rifaximin 10mg/kg/dose bid (up to TID) (max 400 mg/dose) Adults 550 mg PO BID

Lactulose 1-3ml/kg/day divided BID NAC (for Tylenol ingestion)

150mg/kg (max 15gm) IV over 60 minutes 50mg/kg (max 5gm) IV over 4hours 100mg/kg (max 10gm) over 16hours, cont till normal coags

Cholestasis

Actigall 10mg/kg/dose PO BID or TID Phenobarbital 5mg/kg/day divided BID x 3-5days

before HIDA Rifampin (for pruritis) 5 mg/kg/dose BID up to max 10

mg/kg/dose BID (max 300 mg/day or 600 mg/day)

Cholestyramine 240 mg/kg/day divided TID ≤ 10 yrs: max 4 gm/day > 10 yrs : 8 gm/day

AquADEK Vit A 5751 IU, Vit D 400 IU, Vit E 50 IU, Vit K 0.1mg

Fat soluble Vitamins in Cholestatic Kids

Vitamin A 5-25,000 IU/day

Vitamin E (Aqua-E or Liqui-E)

15-25 U/kg/day of water, miscible (Liqui-E)

Vitamin D (goal level 30)

4000-8000 IU/day

Vitamin K < 2 years: 2.5mg 2 - 5 years: 5mg > 5 years: 10mg

IBD Meds

Pentasa (released in small bowel)

50mg/kg/day divided qid or bid (max 1 gram per dose)

Asacol (released after TI) 50mg/kg/day divided qid or bid (max 1 gram per dose)

Canasa suppository 500mg PR at bedtime (retained 1-3hours)

Rowasa enema 4gm per 60ml PR at bedtime (retained overnight)

Colazal (Balsalazide) 750mg – 2.25gm PO tid Sulfasalazine (give with folate)

30-50mg/kg/day divided bid-tid (max 2 grams per day)

6-Mercaptopurine 1-1.5mg/kg/dose daily (for normal enzyme level) 0.5mg/kg/dose daily

Methotrexate (give with folate)

15 mg/M2 per week (up to max 25mg per week)

Infliximab Start 5mg/kg/dose IV (week 0, 2, 6 then Q8weeks)

Allopurinol/6-MP Reduce 6-MP dose by 25% if used w/allopurinol. Allopurinol will decrease metabolism of 6-MP.

AZA 1-3 mg/kg/day Humira Lialda Treatment of UC: 2.4-4.8 gm once

daily for up to 8 weeks Maintenance remission of UC: 2.4 gm once daily

Cortifoam (10%) Insert 1 application 1-2 times/day x 2-3 weeks

Crohn’s disease (moderate-severe; refractory)

Humira Induction: <40 kg: 80 mg on week 0 followed by 40 mg on week 2 ≥ 40 kg: 160 mg on week 0 followed by 80 mg on week 2 Maintenance dose: start on week 4 (2 weeks after induction) <40 kg: 10 or 20 mg every other week , if needed dose may be ↑by ∆ weekly dosing ≥ 40 kg: 20 mg or 40 mg every other week, if needed dose may be ↑by ∆ weekly dosing

CLAS

SIFI

CATI

ON

AN

D DO

SIN

G O

F LA

XATI

VES

LAXA

TIVE

FD

A IN

DICA

TIO

N

PEDI

ATRI

C D

OSA

GE

ADU

LT D

OSA

GE

MEC

HAN

ISM

OF

ACTI

ON

BU

LK-P

RODU

CIN

G

Psyl

lium

(M

etam

ucil®

) Tr

eatm

ent o

f oc

casio

nal

cons

tipat

ion

Ora

l: Ag

es 6

-11:

1.2

5-15

g da

ily in

div

ided

dos

es

Ora

l: 2.

5-30

g da

ily in

di

vide

d do

ses

Hold

s wat

er in

st

ool,

mec

hani

cal

dist

entio

n st

imul

ates

pe

rista

lsis

Met

hylc

ellu

lose

(C

itruc

el®)

Ad

junc

t in

trea

tmen

t of

cons

tipat

ion

Ora

l: Ca

plet

s (50

0mg)

: Ag

es 6

-12:

1 c

aple

t up

to 6

tim

es/d

ay w

/8 o

z of

wat

er

Pow

der:

Ages

6-1

2: 1

gm

in 4

oz

wat

er 1

-3x/

day

Ora

l: Ca

plet

s (50

0 m

g):

2-4

capl

ets u

p to

1-

3x/d

ay w

/8oz

of

wat

er

Pow

der:

2 gm

in 8

oz w

ater

1-

3x/d

ay

Calc

ium

po

lyca

rbop

hil

(Fib

erCo

n®)

Trea

tmen

t of

cons

tipat

ion

or

diar

rhea

Ora

l: Ag

es 6

-12:

625

mg

1-4x

/day

Ora

l: 12

50 m

g 1-

4xda

y

HYPE

ROSM

OTI

C

Glyc

erin

(Fle

et®

Supp

osito

ry)

Trea

tmen

t of

cons

tipat

ion

Rect

al (s

uppo

sito

ry):

Age

< 6:

1 in

fant

su

ppos

itory

, 1-2

x/da

y as

nee

ded

Rect

al

(sup

posi

tory

): 1

adul

t su

ppos

itory

1-

2x/d

ay a

s nee

ded

Osm

otic

effe

ct

draw

s wat

er in

to

inte

stin

es

stim

ulat

ing

peris

talis

and

/or

bow

el m

ovem

ent

Lact

ulos

e (K

rista

lose

®,

Cons

tulo

se®,

Ge

nerla

c®)

Trea

tmen

t of

chro

nic

cons

tipat

ion

Ora

l: 5

g/da

y (7

/5 m

l) O

ral:

10-2

0 g/

day

(15-

30 m

l) in

crea

sed

to 6

0 m

l/day

in 1

-2

divi

ded

dose

s if

need

ed

Poly

ethy

lene

gl

ycol

(Mira

lax®

, Gl

ycol

ax®)

Trea

tmen

t of

occa

siona

l co

nstip

atio

n in

ad

ults

(U

nlab

eled

use

in

child

ren)

Ora

l: Ag

e >

6 m

onth

s: 0

.5-

1.5

g/kg

N

ot to

exc

eed

17

g/da

y, d

o no

t use

> 2

w

eeks

unl

ess d

irect

ed

by h

ealth

care

pro

vide

r

Ora

l: 17

g d

issol

ved

in

4-8

oz b

ever

age

daily

Do

not

use

> 1

w

eek

unle

ss

dire

cted

by

heal

thca

re

prov

ider

LUBR

ICAN

TS

Min

eral

oil

Tem

pora

ry re

lief

of o

ccas

iona

l co

nstip

atio

n,

relie

f of f

ecal

im

pact

ion

Ora

l: Ag

es 6

-11:

5-1

5 m

L at

be

dtim

e Re

ctal

(ene

ma)

: Ag

es 2

-11:

½ b

ottle

(6

6.5

mL)

x 1

Ora

l: 15

-45

mL/

day

Rect

al (e

nem

a):

1 bo

ttle

(133

mL)

as

sing

le d

ose

Lubr

icat

es th

e in

test

ine

and

coat

s and

soft

ens

stoo

l

EMO

LLIE

NTS

Docu

sate

Sod

ium

(C

olac

e®)

Trea

tmen

t of

cons

tipat

ion

and

cons

tipat

ion

care

Ora

l: Ag

es <

3: 1

0-40

m

g/da

y Ag

es 3

-6: 2

0-60

m

g/da

y Ag

es 6

-12:

40-

120

mg/

day

Ages

12+

: 50-

200

mg/

day

Ora

l:

50-3

00 m

g/da

y Pr

even

ts

hard

enin

g of

the

stoo

l by

addi

ng

moi

stur

e

SALI

NE

Mag

nesiu

m

Citr

ate

(Citr

oma®

)

Evac

uatio

n of

bo

wel

prio

r to

cert

ain

surg

ical

an

d di

agno

stic

pr

oced

ures

or

over

dose

sit

uatio

ns

Ora

l: Ag

e <

6: 2

-4 m

l/kg

as

singl

e do

se

Age

6-12

: 100

-150

ml a

s sin

gle

dose

Ora

l: 15

0-30

0 m

l as

singl

e do

se

Draw

s wat

er

into

inte

stin

es,

incr

ease

d pr

essu

re

prom

otes

in

test

inal

m

otili

ty

Mag

nesiu

m

Hydr

oxid

e (P

hilli

ps’®

Milk

of

Mag

nesia

)

Shor

t-te

rm

trea

tmen

t of

occa

siona

l co

nstip

atio

n

Ora

l: Li

quid

(400

mg/

5 m

L):

Ages

2-5

: 5-1

5 m

l at

bedt

ime

or in

div

ided

do

ses

Ages

6-1

1: 1

5-30

ml a

t be

dtim

e or

in d

ivid

ed

dose

s

Ora

l: Li

quid

(400

mg/

5 m

L):

30-6

0 m

l at

bedt

ime

or in

di

vide

d do

ses

Sodi

um

Phos

phat

es

(Fle

et®

Enem

a)

Shor

t-te

rm

trea

tmen

t of

cons

tipat

ion

and

to e

vacu

ate

the

colo

n fo

r rec

tal

and

bow

el e

xam

s

Rect

al:

Ages

2-4

: ½ c

onte

nts o

f 2.

25 o

z ped

iatr

ic e

nem

a Ag

es 5

-12:

2.2

5 oz

pe

diat

ric e

nem

a, m

ay

repe

at

Rect

al:

4.5

oz e

nem

a as

sin

gle

dose

, may

re

peat

STIM

ULA

NT

Senn

a (S

enok

ot®)

Tr

eatm

ent o

f co

nstip

atio

n or

us

ed a

s bow

el

prep

for

proc

edur

e

Ora

l: Sy

rup

(8.8

mg/

5 m

L):

Ages

2-6

: ½ -

¾ ts

p/da

y (m

ax ¾

tsp

2x/d

ay)

Ages

6-1

2: 1

-1 ½

tsp/

day

(max

1 ½

tsp

2x/d

ay)

Tabl

et (8

.6 m

g):

Ages

2-5

: ½ ta

b/da

y (m

ax 1

tab/

day)

Ag

es 6

-12:

1-2

tabs

/day

(m

ax 3

tabs

/day

)

Ora

l: Sy

rup

(8.8

mg/

5 m

L):

10-1

5 m

L (m

ax 3

0 m

L) a

t bed

time

Tabl

et (8

.6 m

g):

2 ta

bs/d

ay, m

ax 4

ta

bs/d

ay

Dire

ct a

ctio

n on

in

test

inal

muc

osa;

st

imul

ates

con

trac

tion

of c

olon

mus

cles

Bisa

cody

l (D

ulco

lax®

) Tr

eatm

ent o

f co

nstip

atio

n or

us

ed a

s bow

el

prep

for

proc

edur

e

Ora

l:

Ages

6-1

1: 5

mg/

day

Rect

al (s

uppo

sito

ry):

Ages

6-1

1: 5

mg/

day

Ora

l:

5-15

mg/

day

(max

30

mg/

day)

Re

ctal

(s

uppo

sito

ry):

10 m

g/da

y Ca

stor

oil

Trea

tmen

t of

cons

tipat

ion

or

used

as b

owel

pr

ep fo

r pr

oced

ure

Ora

l: Ag

es <

2: 1

-2 m

L sin

gle

dose

(max

5 m

L)

Ages

2-1

2: 5

-15

mL

singl

e do

se

Ora

l: 15

-60

mL

singl

e do

se

Dire

ct a

ctio

n on

in

test

inal

muc

osa;

st

imul

ates

con

trac

tion

of sm

all a

nd la

rge

inte

stin

e

INIT

IATI

NG

AN

D AD

VAN

CIN

G E

NTE

RAL

NU

TRIT

ION

TY

PE

AGE

INIT

IAL

INFU

SIO

N

ADVA

NCE

MEN

T G

OAL

Co

ntin

uous

0-

12 m

os

1-2

mL/

kg/h

r 1-

2 m

L/kg

eve

ry 2

-8 h

r 6

mL/

kg/h

r 1-

6 yr

s 1

mL/

kg/h

r 1

mL/

kg e

very

2-8

hr

4-5

mL/

kg/h

r >

6 yr

s 25

mL/

hr

25 m

L ev

ery

2-8

hr

100-

150

mL/

hr

Bolu

s 0-

12 m

os

10-6

0 m

L/2-

3 hr

10

-60

mL/

feed

ing

90-1

80 m

L/4-

5 hr

1-

6 yr

s 30

-90

mL/

2-3

hr

30-9

0 m

L/fe

edin

g 15

0-30

0 m

L/4-

5 hr

>

6 yr

s 60

-120

mL/

2-3

hr

60-9

0 m

L/fe

edin

g 24

0-48

0 m

L/4-

5 hr

Cy

clic

0-

12 m

os

1-2

mL/

kg/h

r 1-

2 m

L/kg

/2 h

r 60

-90

mL/

hr 1

2-18

hr/

d 1-

6 yr

s 1

mL/

kg/h

r 1

mL/

kg/2

hr

75-1

25 m

L/hr

8-1

6 hr

/d

> 6

yrs

25 m

L/hr

25

mL/

kg/2

hr

100-

175

mL/

hr 8

-16

hr/d

Ad

apte

d fr

om D

avis

A. P

edia

tric

s. In

Con

tem

pora

ry N

utrit

ion

Supp

ort P

ract

ice;

199

8; C

h 26

; 358

.

GRO

WTH

VEL

OCI

TY

AGE

WEI

GHT

(g/d

ay)

LEN

GTH

(cm

/mo)

<

3 m

o 25

-35

2.6-

3.5

3-6

mo

15-2

1 1.

6-2.

5 6-

12 m

o 10

-13

1.2-

1.7

1-3

yr

4-10

0.

7-1.

1 4-

6 yr

5-

8 0.

5-0.

8 7-

10 y

r 5-

12

0.4-

0.6

Adap

ted

from

Fom

on S

J, Ha

schk

e F,

et a

l. Bo

dy C

ompo

sitio

n of

refe

renc

e ch

ildre

n fr

om b

rith

to a

ge 1

0 ye

ars.

Am

J Cl

in N

utr J

198

2;35

:116

9.

ENER

GY R

EQU

IREM

ENTS

IN C

HILD

REN

WIT

H DE

VELO

PMEN

TAL

DISA

BILT

IES

CLIN

ICAL

CO

NDI

TIO

N

CALO

RIE

REQ

UIR

EMEN

T Ce

rebr

al P

alsy

(age

5-1

1 yr

s)

13.9

kca

l/cm

hei

ght w

ith m

ild to

mod

erat

e ac

tivity

11

.1 k

cal/c

m h

eigh

t with

seve

re p

hysic

al re

stric

tions

At

heto

id C

ereb

ral P

alsy

U

p to

600

0 kc

al/d

(ado

lesc

ence

) Do

wn

Synd

rom

e Bo

ys (a

ge 5

-12

yrs)

16

.1 k

cal/c

m h

eigh

t Gi

rls (a

ge 5

-12

yrs)

14

.3 k

cal/c

m h

eigh

t M

yelo

men

ingo

cele

(Spi

na B

ifida

) 9-

11 k

cal/c

m h

eigh

t for

mai

nten

ance

7

kcal

/cm

for w

eigh

t los

s Ap

prox

imat

ely

50%

RDA

for a

ge a

fter

infa

ncy

Prad

er-W

illi S

yndr

ome

10-1

1 kc

al/c

m h

eigh

t for

mai

nten

ance

8.5

kcal

/cm

hei

ght f

or w

eigh

t los

s

1.

Prot

ein

requ

irem

ents

– M

ay b

e m

et b

y pr

ovid

ing

RDA

for a

ge

2.

Flui

d re

quire

men

ts –

Att

entio

n to

flui

d ne

eds i

s cru

cial

in th

ese

patie

nts b

ecau

se m

any

do n

ot h

ave,

or c

anno

t exp

ress

, a th

irst s

ensa

tion

3.

Form

ula

choi

ce g

uide

line

– If

wei

ght a

ge is

≤ 1

0 ye

ars,

use

pedi

atric

form

ula;

if w

eigh

t ag

e is

> 10

yea

rs, m

ay u

se a

dult

form

ula

Ek

vall

SW, B

andi

ni L

, Ekv

all V

: Obe

sity.

In E

dval

l SW

(ed)

: Ped

iatr

ic N

utrit

ion

in C

hron

ic D

iseas

es a

nd D

evel

opm

enta

l Di

sord

ers,

Oxf

ord

Uni

vers

ity P

ress

, 199

3; 1

68.

Davi

s A. P

edia

tric

s. In

Con

tem

pora

ry N

utrit

ion

Supp

ort P

ract

ice;

199

8; C

h 26

; 356

.

CATC

H-U

P GR

OW

TH R

EQU

IREM

ENTS

Catc

h-U

p Gr

owth

Re

quire

men

t (k

cal/k

g/d)

= RD

A Ca

lorie

s for

Age

(k

cal/k

g/d)

X

Idea

l Wei

ght f

or

Heig

ht (k

g)

Actu

al W

eigh

t (kg

)

Prot

ein

Requ

irem

ent

(g/k

g/d)

=

RDA

Prot

ein

for A

ge

(g/k

g/d)

X

Idea

l Wei

ght f

or

Heig

ht (k

g)

Actu

al W

eigh

t (kg

)

1.

Plot

the

child

’s h

eigh

t and

wei

ght o

n th

e N

CHS

grow

th c

hart

s 2.

De

term

ine

the

child

’s re

com

men

ded

calo

ries p

er k

g fo

r thi

s chi

ld’s

age

3.

De

term

ine

the

idea

l wei

ght (

50th

per

cent

ile) f

or th

is ch

ild’s

hei

ght

4.

Mul

tiply

the

valu

e ob

tain

ed in

(2) b

y th

e va

lue

obta

ined

in (3

) 5.

Di

vide

the

valu

e ob

tain

ed in

(4) b

y ac

tual

wei

ght

For t

he p

rote

in e

quat

ion,

follo

w th

e sa

me

step

s, bu

t in

(2),

subs

titut

e pr

otei

n fo

r cal

orie

s Ad

apte

d fr

om K

M C

orra

les a

nd S

L U

tter

, Fai

lure

to T

hriv

e. In

Sam

our P

Q, H

elm

KK,

and

Lan

g CE

. Han

dboo

k of

Ped

iatr

ic N

utrit

ion,

2nd

ed.

Asp

en P

ublis

hers

. 199

9;40

6.

LPC

H N

on-N

ICU

TPN

GU

IDE

A

dditi

onal

TPN

Add

itive

s 1.

Ped

iatri

c Tr

ace

elem

ents

(Cu,

Mn,

Zn,

Cr)

= 0.

2mL/

kg, m

ax 5

mL

•

Hol

d if

Dbi

li >2

, sev

ere

rena

l dise

ase,

or o

n lo

ng te

rm T

PN

• If

hold

ing

add

200m

cg/k

g/da

y of

Zin

c

• If

hold

ing

add

10m

cg o

f cop

per

2. P

edia

tric

Mul

ti V

itam

in =

5mL

up to

11

yrs a

nd 1

0mL

if >1

1 yr

s

3. S

elen

ium

sho

uld

be a

dded

on

day

31 a

t 2m

cg/k

g/da

y fo

r lon

g-te

rm T

PN

4. H

epar

in: 0

.5un

its/m

l for

0-1

yr a

nd 1

unit/

mL

if >1

yr (i

f nor

mal

coa

gula

tion

prof

ile)

Fl

uid

Goa

ls

Base

d on

4-2

-1 ru

le

• N

ote

TPN

ord

er e

ntry

pro

gram

ask

s fo

r flu

id a

s ml/k

g/da

y

TPN

ord

er e

ntry

allo

ws e

ntry

of n

on T

PN fl

uid

volu

mes

(i.e

. drip

s, m

eds o

r ent

eral

fe

eds)

. If t

hese

are

ent

ered

, the

pro

gram

will

take

thes

e in

to c

onsi

dera

tion

to in

sure

pa

tient

doe

s not

exc

eed

ente

red

fluid

goa

l whe

n m

akin

g TP

N.

TPN

is a

med

icat

ion

with

pot

entia

l for

har

m if

ord

ered

or u

sed

inco

rrec

tly. T

his

reso

urce

shou

ld se

rve

as a

gui

de fo

r the

initi

atio

n an

d ad

just

men

t of T

PN. I

t is n

ot

all e

ncom

pass

ing

and

shou

ld b

e us

ed in

con

junc

tion

with

ong

oing

ass

essm

ent o

f the

pat

ient

’s s

tatu

s and

with

inpu

t fro

m th

e LP

CH N

utrit

ion

Supp

ort T

eam

.

Infu

sion

Tim

e

• In

itial

ly st

art w

ith 2

4 ho

ur in

fusi

ons

•

For p

atie

nts t

hat w

ill b

e on

long

term

TPN

, it c

an b

e cy

cled

to ru

n fo

r 12-

18 h

ours

o

D

ecre

ase

cycl

e le

ngth

2 h

ours

/day

for 0

-6 y

rs a

nd b

y 4

hrs/

day

for >

6 yr

s

o

Ram

p up

and

dow

n in

fusi

on ra

te o

ver 2

hou

rs w

hen

usin

g D

12.5

% o

r hig

her

o

Che

ck g

luco

se o

ne h

our i

nto

max

imum

rate

and

one

hou

r afte

r inf

usio

n w

hen

adju

stin

g cy

cle

leng

ths

o

Whe

n ou

tsid

e of

LPC

H, c

ycle

leng

th c

alcu

lato

r ava

ilabl

e at

ww

w.p

eds.s

tanf

ord.

edu

unde

r “Li

nks”

and

“Pa

tient

Car

e To

ols”

M

onito

ring

1.

In

itiat

e “T

PN M

onito

ring”

lab

orde

r set

in C

erne

r •

Prio

r to

orde

ring

TPN

= C

BC, M

etC

, Mg,

Pho

s, tri

glyc

erid

e , D

-bili

, GG

T, C

oags

, UA

o

C

XR

to in

sure

pro

per p

lace

men

t of c

entra

l lin

e

o

Do

NO

T or

der a

Che

m 2

3

• TP

N d

ays 1

-4 =

Dai

ly re

nal f

unct

ion

pane

l, M

g, tr

igly

cerid

e, a

nd U

A

• Lo

ng T

erm

inpa

tient

mon

itorin

g

o

qMon

day

= C

BC, M

etC

, Mg,

PO

4, D

-bili

, GG

T, tr

igly

cerid

e

o

qThu

rsda

y =

Ren

al fu

nctio

n pa

nel,

mag

nesi

um, t

rigly

cerid

e

2.

Cha

nge

in st

atus

of p

atie

nt/c

are

•

If pa

tient

stat

us, m

edic

atio

ns, o

r car

e pl

an c

hang

es w

ith p

oten

tial i

mpa

cts t

o el

ectro

lyte

s or l

iver

func

tion,

con

side

r mor

e fre

quen

t lab

s 3.

N

utrit

ion

Supp

ort S

ervi

ce is

aut

omat

ical

ly c

onsu

lted

for p

atie

nts o

n TP

N. C

heck

for “

Nut

ritio

n A

sses

smen

ts”

in C

are

Form

Not

es (i

npat

ient

) or i

n An

cilla

ry D

ocum

ents

(out

patie

nt) s

ectio

ns o

f Clin

ical

Doc

umen

ts.

Peri

pher

al L

ines

M

axim

ums:

Dex

trose

D12

.5%

, Pro

tein

3.5

%, P

otas

sium

40

mEq

/L, C

alci

um 3

g/L

, Osm

olar

ity 9

50 m

Osm

L

ong

Ter

m T

PN P

atie

nts

Atte

mpt

to o

btai

n ho

me

reci

pe (f

rom

thei

r pha

rmac

y or

on

thei

r hom

e ba

g of

TPN

). If

no re

cipe

is a

vaila

ble

prio

r to

LPCH

TPN

pha

rmac

y cl

osin

g fo

r the

day

, co

nsul

t with

the

fello

w to

det

erm

ine

an a

ppro

pria

te d

extr

ose

and

elec

trol

yte

solu

tion

until

TPN

can

be

mad

e.

---

TPN

is a

med

icat

ion

with

pot

entia

l for

har

m if

ord

ered

or u

sed

inco

rrec

tly. T

his

reso

urce

shou

ld se

rve

as a

gui

de fo

r the

initi

atio

n an

d ad

just

men

t of T

PN. I

t is n

ot

all e

ncom

pass

ing

and

shou

ld b

e us

ed in

con

junc

tion

with

ong

oing

ass

essm

ent o

f the

pat

ient

’s s

tatu

s and

with

inpu

t fro

m th

e LP

CH N

utrit

ion

Supp

ort T

eam

.

Guidelines for Omegaven for LPCH patients Updated 6/14 1-Consult Intestinal Rehab/GI- GI fellow on service will do initial consult. If Dr. Kerner, Dr. Castillo or Dr. Berquist is on service, they do consult with the fellow. If another GI attending is on service, Dr. Castillo will work with the GI fellow on service to gather information and present to the Nutrition Support Team. Eligibility will be discussed at Nutrition Support Team (NST) rounds (Wed 10-12) with team (Dr Kerner PI, Bob Poole, pharmD, Colleen Nespor- NST CNS). Historical and physical information, diagnosis, imaging studies, biopsies, and other available specialized tests will be reviewed by the multidisciplinary team experienced in the diagnosis and treatment of parenteral nutrition associated liver disease. We will discuss appropriatesness of consult, verify protocol requirements are met, discuss finances and any other issues. 2-Insurance Authorization- We cannot administer or consent without a funding source for when patient ultimately is discharged home on PN/Omegaven. Have patient’s case manager contact Colleen pg 28058 or office 6-8097. 3-Labs- With the sheer volume of necessary blood this will most likely take multiple draws over several days on the littlest ones.

SERUM Chemistry Panel Liver Function Panel CBC w/diff C-reactive Protein Essential Fatty Acid Profile Free Fatty Acids Lipid Profile/Panel PT/PTT/INR Vitamin A level Retinol Binding Protein Vitamin E level Vitamin D level (25-hydroxy-VitD) Alpha-1-Antitrypsin level and phenotype URINE Urine Glucose Urine Ketones

4-Criteria INCLUSION CRITERIA:

• TPN dependent (unable to meet nutritional needs solely by enteral nutrition).

• Must have parenteral nutrition associated liver disease. Other causes of liver disease should be excluded. A liver bx is not needed.

• Direct bilirubin > 3.0 mg/dL. • Signed patient informed consent.

• Insurance for patient must approve funding of the Omegaven for outpatient care.

The patient must have failed standard therapies to prevent the progression of his/her liver disease including:

• Surgical treatment • Cyclic PN • Avoiding overfeeding • Reduction/removal of copper and manganese from

PN • Advancement of enteral feeding • Use of ursodiol (i.e., Actigall) • Lipid minimization

EXCLUSION CRITERIA:

• Pregnancy. • Other causes of chronic liver disease (Hepatitis C,

cystic fibrosis, biliary atresia, and alpha-1-antitrypsin deficiency,).

• Signs of advanced liver disease, including cirrhosis on biopsy, varices, ascites.

• The patient is enrolled in any other clinical trial involving an investigational agent (unless approved by the designated physicians on the multidisciplinary team).

• The parent or guardian or child is unwilling to provide consent or assent.

5-Consent- Attending gastroenterologist who did consult will obtain consent from parents, send one copy to medical

records and forward one copy to Colleen for data collection. Available in English and Spanish. 6-Begin Omegaven ADMINISTRATION

• Begin at 0.5 g/kg/d over 12-24 hrs, for 2 days. • Increase dose to 1 g/kg/d after 2 days. • Given through central or peripheral line. • Compatible to “Y-in” with TPN. • May infuse through 1.2 micron in line filter safely. • Consider dose reduction by 25% if

hypertriglyceridemia develops (TG > 200, checked 4 hrs after stopping infusion; no confounding reason for TG elevation such as drugs or renal disease).

• If additional fat needed, give enterally. OK to give additional conventional Intralipid® if enteral not tolerated.

• Pt will require GI follow up for up to 3 months after stopping OmegavenTM.

• Omegaven will be administered over 12-24 hours. If over 24 hours, syringes must be changed out after 12 hours for infection control.

• If patient becomes an inpatient after being home with Omegaven they will use ‘home supply’ for hospital administration.

CONTRAINDICATIONS

• Impaired lipid metabolism • Severe hemorrhagic disorders

• Unstable diabetes mellitus • Collapse and shock • Stroke/Embolism • Recent cardiac infarction • Undefined coma status

SIDE EFFECTS

• Prolonged bleeding time • Inhibition of platelet aggregation • Fishy taste (rare) • Hyperglycemia (reduce or stop infusion) • Otherwise same as Intralipid®

MONITORING LABS (weekly)

• Essential Fatty Acid Profile • Liver Function Panel