The document discusses nutrition considerations for a pediatric patient undergoing bone marrow transplantation for sickle cell disease. It outlines the patient's diagnosis and treatment plan, including high calorie and protein needs due to increased metabolic demands. Due to chemotherapy side effects like mucositis and nausea, optimal oral intake is difficult and nutrition support is important. Enteral nutrition is preferred over total parenteral nutrition when possible due to gut health benefits. The case study focuses on a 3-year old male patient admitted for umbilical cord blood transplantation and his nutrition care plan.

1. Nutrition & Pediatric BMT
Patients: A Case Study
Stacy Kim
Dietetic Intern, Class of 2013-2014
University of Maryland, College Park
January 31, 2013

2. Sickle Cell Disease
 Autosomal recessive genetic disorder
 Crescent-shaped RBCs due to abnormal hemoglobin in sickle cells
 Cells can get “stuck” in blood vessels  reduced blood flow 
pain, infection, organ damage
 RBCs have shortened lifespan  chronic hemolytic anemia
 Other complications include HTN, stroke

3. Sickle Cell Disease & Nutrition
 Growth retardation and wasting common
Hypophagia
Increased RMR
Increased metabolic demands especially with sickle cell-related
complications
 Wasting can lead to increased hospitalization and poorer clinical
outcomes

4. Stem Cell Transplantation
 Hematopoietic stem cells from bone marrow, peripheral blood,
umbilical cord blood
 Prep regimens to prevent formation of sickle cells + make room in
bone marrow for new stem cells
 Complications: GVHD, infection, mucositis, HTN, electrolyte
imbalances

5. UCB Transplantation
 More readily available source of stem cells
 Lower occurrence of GVHD
 However, delayed time to engraftment  increased risk for
infection
 Side effects of chemotherapy: nausea, vomiting, mucositis,
anorexia, taste changes, malabsorption
Suboptimal oral intake  potential malnutrition

6. Mucositis
 Adverse effect of high-dose chemotherapy which act on cells with
high turnover rates
 Compromised integrity of mucosal epithelia that line entire GIT,
usually ~7-10 days after chemo starts, healing 14-21 days
 Can lead to sepsis, ulceration, bleeding, malabsorption, diarrhea,
and pain
 Inadequate oral fluid and food intake  dehydration, malnutrition
 Adequate nutrition support is important

7. Nutrition Support: Enteral Nutrition
 EN preferable
Maintain normal gut function
Provide nutrients not available through TPN
Decreased risk of infection
Maintain gut-associated immune function
Cost containment

8. Nutrition Support: Enteral Nutrition
 Risks: vomiting, tube dislodgement, excess discomfort due to
mucositis, occlusion of tube, potential bleeding from
placement/replacement secondary to thrombocytopenia
 However, NG tubes have been used in pediatric patients following
BMT with good outcomes

9. Nutrition Support: Enteral Nutrition
 PEG tubes
Risks: localized inflammation, infection, insertion site bleeding,
feeding intolerance
Demonstrated optimization of nutrition and successful weight
maintenance in pediatric+adult BMT patients
Due to immunosuppressive therapy for GVHD, extremely difficult
decision whether complication of PEG tube placement outweighs
benefits in high-risk patients
Study suggests that ANC should be considered before PEG
placement + avoid placing during neutropenic episodes

10. Nutrition Support: TPN
 Easier to administer as patients already have central venous access
for transplantation procedure
 In adults, often argued that EN before PN
 However, normal development and maturation in pediatrics is so
important that there is less debate

11. Nutrition Support: Perceptions
 “I already had a port in for chemo so we used that with the TPN”
 “Having a tube up her nose was a lot of hassle. TPN was more
convenient”
 “TPN made life a little more ‘normal’”
 “Don’t want things down my nose or throat”
 “Tube feeding sounds disgusting and uncomfortable”
 “Chose TF because it helped keep digestive system active, making it
easier to adjust back to food”
 “TF is helpful in giving meds and not as hard on liver”

12. Glutamine
 Shown to reduce severity of mucositis in children receiving chemo
 Glutamine group had reduction in mean # of days of IV narcotics use and
TPN versus glycine
 Decreased length of hospitalization  potential $$ savings

13. “TA”
 3 year old male with sickle cell disease
FT via NSVD; breastfed
 Admitted January 22 for umbilical cord blood transplant
 PMH: h/o dactylitis x2 episodes (2011), veno-occlusive crisis (2011),
E. Coli (2011), PNA (2012), stroke (2012)
 Sx hx: liver biopy (2011), central line placement (2014)

14. Diet History
 Good appetite and intake pta
 Favorite foods: omelets with tomatoes, white rice, spaghetti with
meat sauce, sometimes vegetable soup
 Dislikes Pediasure
 NKFA
 Home Diet: Regular
 Current diet: Low Bacteria, 1-3 years
Working with kitchen to provide favorite foods

15. Admission
 Admission weight: 18.0 kg
75-90th %ile
 Height: 102 cm
50-75th %ile
 BMI: 17.3
85-90th %ile

19. Medications
Aprepitant Anti-emetic Diarrhea, constipation, loss of appetite
Cyclosporine + D5 GVHD prophylaxis Diarrhea, heartburn, gas
Fludarabine + NS Chemotherapy Diarrhea, constipation, loss of appetite, nausea, vomiting, mouth sores
Furosemide Diuretic Upset stomach, vomiting, constipation
Levetiracetam Anticonvulsant Diarrhea, constipation, vomiting, loss of appetite
Melphalan + NS Chemotherapy Nausea, vomiting, loss of appetite or weight, sores in mouth and throat
Mycophenolate mofetil + D5 GVHD prophylaxis Constipation, stomach pain, nausea, vomiting
Ondansetron Anti-emetic Diarrhea, constipation
Ranitidine Treat ulcers, GERD Diarrhea, constipation, nausea, vomiting, stomach pain
Bactrim Antibiotic Diarrhea, nausea, loss of appetite
Thiotepa + NS Chemotherapy Nausea, vomiting, stomach pain, loss of appetite
Ursodiol Dissolve gallstones Diarrhea, constipation, upset stomach, indigestion
Valacyclovir Antiviral Diarrhea, constipation, vomiting, upset stomach
Voriconazole Antifungal Diarrhea, vomiting, dry mouth
PRN acetaminophen Pain relief n/a
PRN diphenhydramine Sleep Constipation, nausea, vomiting, loss of appetite
PRN heparin flush Anticoagulant n/a
PRN Miralax Laxative Diarrhea, nausea

20. Labs
DATE 1/22 1/23 1/24 1/25 1/26 1/27 1/28 1/29 1/30
WEIGHT 18.0 18.1 17.9 - 18.3 17.7 17.7 17.5 17.7
Na 135 138 136 137 134 135 134 136 135
K+ 4.0 3.9 3.9 3.6 3.8 3.9 3.8 3.5 3.6
Cl 102 100 99 103 102 98 101 104 103
CO2 24 25 24 21 24 23 23 23 24
Glucose 104 88 84 81 97 79 90 91 88
BUN 8 10 10 11 9 10 10 9 11
Cr 0.4 0.4 0.4 0.3 0.3 0.4 0.3 0.3 0.3
Ca 8.7 9.0 8.8 8.9 8.9 9.1 9.0 8.5 8.6
Phos - 4.7 5.1 5.5 5.5 5.3 5.7 5.3 5.3
Mg - 2.1 1.7 1.9 1.8 2.0 1.9 1.7 1.7
Alb 3.8 3.8 - - - 3.7 - - 3.5
Bili 0.5 0.5 - - - 0.6 - - 0.9
ALT
AST
21 26 - - - 22 - - 20
46 44 - - - 43 - - 32
TG - - - - - 77 - - -
HGB 9.3 9.0 8.3 11.3 11.4 11.3 10.8 10.3 10.2
HCT 26.7 26.4 24.4 32.6 32.7 32.8 31.0 29.5 29.5

21. Nutrition Diagnosis
 Predicted suboptimal energy intake (NI-1.6) related to BMT as
evidenced by kcal needs increased 140-160% of BMR and protein
needs are 2.0-2.5 g/kg.

22. Estimated Needs
 Energy: 70-80 kcal/kg
BMR x 1.4-1.6
 Protein: 2.0-2.5 g/kg
 Fluids: 1905 ml/day
Holliday-Segar method

23. Nutrition Intervention
 Continue low bacterial diet for age.
 Consider providing 240 ml Pediasure daily to help meet needs during
hospitalization.
 BMP, Mg, Phos daily
 LFTs and TG weekly
 Weigh daily to monitor for weight stability during admission
 Follow for regular bowel movements as prep regimen is initiated

24. Follow-up
 Patient is being followed per low-risk protocol (<7 days)
 Per medical chart, TA’s po intake had decreased. Pediasure TID
ordered.
 If po intake does not improve and mother continues to decline EN,
TPN will be initiated per RD recommendations.

25. References
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support in pediatric oncology patients and parents. Journal of Pediatric Oncology Nursing. 2013: 30: 90-98
 Reid M. Nutrition and sickle cell disease. Comptes Rendus Biologies. 2013: 336: 150-163.
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 Wedrychowicz A, Spodaryk M, Krasowska-Kwiecieri A, Gozdzik J. Total parenteral nutrition in children and
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