Nutrition & Pediatric BMT

Patients: A Case Study

Stacy Kim

Dietetic Intern, Class of 2013-2014

University of Maryland, College Park

January 31, 2013

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

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

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

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

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

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

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

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

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

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”

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

“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)

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

Admission

Admission weight: 18.0 kg

75-90th %ile

Height: 102 cm

50-75th %ile

BMI: 17.3

85-90th %ile

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

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

ALTAST

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

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.

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

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

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.

References

Aquino VM, Harvey AR, Garvin JH, Godder KT, Nieder ML, Adams RH, Jackson GB, Sandler ES. A double-blind randomized placebo-controlled study of oral glutamine in the prevention of mucositis in children undergoing hematopoietic stem cell transplantation: a pediatric blood and marrow transplant consortium study. Bone Marrow Transplantation. 2005: 36: 611-616.

Kaur S, Ceballos C, Bao R, Pittman N, Benkov K. Percutaneous endoscopic gastrostomy tubes in pediatric bone marrow transplant patients. Journal of Pediatric Gastroenterology and Nutrition. 2013: 56(3): 300-303.

Montgomery M, Belongia M, Mulberry MH, Schulta C, Phillips S, Simpson PM, Nugent ML. Perceptions of nutrition 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.

Storey B. The role of oral glutamine in pediatric bone marrow transplant. Journal of Pediatric Oncology Nursing. 2007: 24 (1): 41-45.

Thompson LM, Ceja ME, Yang SP. Stem cell transplantation for treatment of sickle cell disease: Bone marrow versus cord blood transplant. American Journal of Health-System Pharmacy. 2012: 69: 1295-1302.

Wedrychowicz A, Spodaryk M, Krasowska-Kwiecieri A, Gozdzik J. Total parenteral nutrition in children and adolescents treated with high-dose chemotherapy followed by autologous haematopoietic transplants. British Journal of Nutrition. 2010: 103: 899-906.