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Running head: FAILURE TO THRIVE 1

Failure to Thrive: Complications Associated with Mixed Connective Tissue Disorders

Abigail Smith

University of Kentucky

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Patient Profile

Age/Sex: Female/29 years old Height: 62” Weight: 46 kg (101 lbs)

BMI: 18.5 Ideal Body Weight: 50 kg % Ideal Body Weight: 92%

Marital Status: Single Ethnicity: Caucasian Religion: N/A

Occupation: N/A Education: N/A

Reason for Admission: Generalized pain, decreased oral intake, fatigue, hypoglycemia, emesis

x several days.

Past Medical History: Systemic lupus erythematous (SLE), CREST syndrome, C. diff positive,

vancomycin-resistant enterococci (VRE), chronic kidney disease (CKD), peptic ulcer disease

(PUD), hypertension (HTN), chronic steroid use, malnutrition, gastrocutaneous fistula s/p

revision

Family Medical History: N/A

Health History: Patient expresses emesis x several days and increasing difficulty swallowing.

She states when she eats, food immediately “comes back up.” Patient has an extensive medical

history and is familiar with medical procedures from previous experiences, states she would like

to have an EGD done and suspects she requires esophageal dilation to help with swallowing

difficulty.

Social History: Parents and grandmother are support system. Patient has daughter that her

parents adopted since patient was unable to care for a child. Patient has limited ambulation at

home and relies on parents and grandmother for assistance with shopping and meal

preparation/delivery.

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Failure to Thrive: Complications Associated with Mixed Connective Tissue Disorders

Literature Review

Introduction

There is shockingly little research regarding connective tissue disorders including

systemic lupus erythematous (SLE) and CREST syndrome. Connective tissue disorders result in

numerous complications that severely affect one’s ability to perform the activities of daily life

(ADLs) (Ortega-Hernandez & Shoenfeld, 2012). The etiology of connective tissue disorders is

unknown, and there is no definitive cure for these disorders (Mayo Clinic, 2016). Treatment

options are limited only seek to mask symptoms and associated complications (Ortega-

Hernandez & Shoenfeld, 2012). This review examines the most current literature regarding

connective tissue disorders, their complications, and treatment options.

Definition

Mixed connective tissue disease (MCTD) was first described in the 1970s as a disorder

with mixed features of SLE, scleroderma, polymyositis/dermatomyositis, and rheumatoid

arthritis together with the presence of specific antibodies (Ortega-Hernandez & Shoenfeld,

2012). SLE is defined as chronic inflammatory disease that occurs when the body’s immune

system attacks its own tissues and organs. It most commonly affects joints, skin, kidneys, blood

cells, brain, heart, and lungs (Mayo Clinic, 2016). Limited scleroderma, or CREST syndrome, is

defined as calcinosis – calcium deposits under the skin and in tissues – Raynaud’s phenomenon –

numbness associated with sensitivity to the cold – esophageal dysmotility, sclerodactyly –

thickened skin on the fingers – and telangiectasis – enlarged blood vessels (Johns Hopkins

Medicine, 2016). Together, these two diseases comprise, in large part, MCTD. The most

common clinical symptoms include Raynaud’s phenomenon, arthritis, swollen hands, sausage-

like fingers, and muscle weakness. These symptoms occur in 90% of patients and typically

develop in unison. However, MCTD may also be a more serious disease with the development of

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severe complications including pulmonary arterial hypertension, nephritis/chronic kidney

disease, vasculitis, gastrointestinal bleeding, and severe central nervous system involvement

(Ortega-Hernandez & Shoenfeld, 2012).

Complications

MCTD may result in serious complications including joint and forearm manifestations,

muscle diseases, skin manifestations, lung manifestations, cardiovascular disease, hematological

manifestations, neurological disease, and, most frequently, gastrointestinal disease and renal

complications. Gastrointestinal involvement is seen in approximately 66-74% of patients. Of

these, esophageal dysfunction is the most prevalent GI manifestation (Ortega-Hernandez &

Shoenfeld, 2012). Weak/absent esophageal peristalsis (gastroparesis) is the most common

esophageal dysmotility dysfunction and causes dysphagia when it is symptomatic (Smout & Fox,

2012). Impairment of the distal esophagus and the lower esophageal sphincter are frequent as

well. Other consequences of esophageal dysmotility include gastroesophageal reflux, Barrett

esophagus, esophageal strictures, and occult aspiration (Nisa & Giger, 2011). MCTD is

associated with other GI manifestations including mesenteric vasculitis, colonic perforation,

protein-losing enteropathy, acute pancreatitis, hemoperitoneum, diarrhea, and chronic hepatitis.

Renal involvement is another of the major complications associated with MCTD. It is observed

in approximately 25% of patients and is often asymptomatic. When renal symptoms do arise,

glomerulonephritis and nephrolithiasis are the most common clinical manifestations (Ortega-

Hernandez & Shoenfeld, 2012).

Treatment

As the etiology of MCTD is unknown, treatment options are focused on masking

symptoms and complications. Therapy should be individualized on a patient-by-patient basis to

address the specific organs involved and the severity of the MCTD activity itself. Initially, it was

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believed that MCTD responded well to corticosteroid therapy. However, subsequent studies have

shown different results. In general, corticosteroids such as prednisone and methylprednisone are

the most commonly used immunosuppressant therapies. Antimalarial drugs, methotrexate, and

vasodilators have also been used to treat experimentally with varying degrees of success (Ortega-

Hernandez & Shoenfeld, 2012). Reglan may be used to treat esophageal dysmotility and

gastroparesis by stimulating peristalsis (Smout & Fox, 2012). Other medications, therapies, and

surgical interventions are used to treat complications on a case-by-case basis. In most patients

with MCTD, pain medications are typically required to control and/or mask disease-related pain


Conclusion

In closing, mixed connective tissue disorders are complicated in definition, etiology, and

treatment. The complications associated with MCTD are numerous and range from mild to

serious and life threatening. The current research on MCTD is extremely limited, which makes

these disorders even more difficult to treat. Given the diverse expanse of MCTD, additional

research is warranted to fully understand the etiology of the disorder so more effective

treatmentprotocols may be developed.

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Disease Background

Definitions:

Systemic lupus

erythematous (SLE):

Chronic inflammatory disease that occurs when the body’s immune

system attacks its own tissues and organs. May affect joints, skin,

kidneys, blood cells, brain, heart, and lungs. It is more common in

women between the ages of 15 and 40 (Mayo Clinic, 2016).

CREST syndrome: Limited scleroderma, the milder form of scleroderma. CREST stands for

calcinosis (calcium deposits under the skin and in tissues), Raynaud’s

phenomenon (numbness associated with sensitivity to the cold),

esophageal dysmotility, sclerodactyly (thickened skin on the fingers),

telangiectasis (enlarged blood vessels). It is most commonly seen in

Caucasian women (Johns Hopkins Medicine, 2016).

Gastrocutaneous

fistula:

Uncommon complication after PEG tube removal involving opening in

the stomach and disruption of the lining of the GI tract. Removing

necrotic tissue within the fistula tract may facilitate new tissue growth

and subsequent fistula closure (Tang, 2014).

Assessment: 29 year old female well-known to the Medicine Team due to frequent admissions

with lengthy stays due to her complicated medical history including mixed connective tissue

disorder (SLE and CREST). Although all the active problems stem back to the diagnoses of SLE

and CREST syndrome, symptoms are interconnected and it is difficult to address each

individually. The patient has been diagnosed with chronic kidney disease and hypertension,

complications frequently associated with mixed connective tissue disorder (Ortega-Hernandez &

Shoenfeld, 2012). Kidney failure is one of the leading causes of death among people with SLE

(May Clinic, 2016). This patient also struggles with recurrent infectious diseases including but

not limited to C. diff, VRE, and UTI related to frequent hospital stays, chronic/long-term

antibiotic use, and weakened immune system which are also secondary to her mixed connective

tissue disorder (Ortega-Hernandez & Shoenfeld, 2012). People with SLE are more vulnerable to

infection because both the disease and its treatments weaken the immune system (Mayo Clinic,

2016). From a nutritional standpoint, the main concern is diagnosed malnutrition. In this

instance, malnutrition is likely due to dysphagia, which is likely secondary to esophageal

dysmotility from CREST syndrome (Nisa & Giger, 2011). Weak and absent esophageal

peristalsis are frequently encountered esophageal motility disorders in CREST syndrome, which

may be associated with dysphagia (Smout & Fox, 2012). At this time, the attending physician

prefers to keep the patient on a PO diet to minimalize treatment interventions. If alternative

feeding routes are required, options will be limited. Percutaneous endoscopic gastrostomy (PEG)

tube is the most common route for enteral nutrition in patients requiring medium to long term

tube feeding and with impaired swallow function or dysphagia (Tang, 2014). The patient is not a

candidate for a PEG due to a gastrocutaneous fistula that developed after a previous PEG

placement. The location and extent of the fistula prevent a new PEG placement. The patient has

previously had an NGT, which she pulled out, citing discomfort.

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Diagnosis:

o Failure to thrive

o Dysphagia

o Malnutrition

o Systemic lupus erythematous (SLE)

o CREST syndrome

o Clostridium difficile (C. diff)

Pathophysiology: From a nutritional standpoint, the main concern with this patient is

malnutrition. In this case, malnutrition is caused by inability to obtain adequate nutrition due to

dysphagia. Dysphagia, or difficulty swallowing, is somewhat subjective in nature. The patient

complains of difficulty swallowing and states that “food comes back up” when she tries to eat.

This patient’s dysphagia is likely due to weak and/or absent esophageal peristalsis seen in

CREST syndrome esophageal dysmotility (Smout & Fox, 2012). An EGD and/or esophageal

biopsy is needed to confirm a dysphagia diagnosis (Nisa & Giger, 2012).

This patient’s CKD is likely lupus nephritis caused by her SLE. In lupus nephritis, the filters in

the kidneys are damaged which results in a loss of kidney function and subsequent

nephrolithiasis, also known as kidney stones (Mayo Clinic, 2016). The diagnosis of hypertension

is likely pulmonary arterial hypertension (PAH) related to CREST syndrome in which the

endothelial cells of blood vessels are injured and excessive connective tissue accumulates inside

the blood vessels causing narrowing (Johns Hopkins Medicine, 2016).

Etiology: All active diagnoses may be traced back to the patient’s mixed connective tissue

disorder (i.e. SLE and CREST). Both are chronic inflammatory autoimmune conditions in which

the body’s immune system attacks its own tissues and organs causing numerous complications.

The etiology of these autoimmune diseases is unknown, although heredity and environmental

factors are suspected causes. Women between the ages of 15 and 40 are more likely to be

diagnosed with SLE than any other population (Mayo Clinic, 2016). Caucasian women are the

most likely population to have a diagnosis of CREST syndrome (Johns Hopkins Medicine,

2016).

Treatment: Dysphagia is typically treated with an altered consistency diet as determined by a

speech therapist in a swallow evaluation. In some cases, alternative forms of nutrition (i.e.

enteral or parenteral nutrition) may be indicated (Nisa & Giger, 2012). At this time, the only

commonly practiced treatments for mixed connective tissue disorders include steroids and pain

management medications. Management of other complications is on an as-needed basis (Ortega-

Hernandez & Shoenfeld, 2012). For instance, lupus nephritis involving nephrolithiasis may

require surgical intervention depending on its severity (Mayo Clinic, 2016).

Nutrition Intervention: It will be beneficial to calculate calorie, protein, and fluid requirements

due to the pre-existing diagnosis of malnutrition. The primary team has concerns about the

patient’s intake due to her disease state and has ordered a calorie count to formally assess PO

intake. At this time, the patient is not agreeable to a modified diet, so the diet order stands for a

regular diet. Discuss with patient the importance of adequate nutrition and the meaning of the

“failure to thrive,” “dysphagia,” and “malnutrition” diagnoses as well as how other active

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problems play a role in nutrition. Determine patient preferences regarding nutrition

supplementation (i.e. Ensure and other supplements) and willingness to try alternative routes of

nutrition (i.e. enteral nutrition routes).

Prognosis: Due to the extensive problem list and complicated disease background, the patient’s

prognosis is poor. She has had frequent hospital admissions for mixed connective tissue disease-

associated complications and pain management. Most recently, the attending physician has

assigned a diagnosis of “failure to thrive.” Both SLE and CREST syndrome are chronic

inflammatory autoimmune diseases for which no cure exists and treatment are limited. Although

the generalized pain, hypoglycemia, and emesis that caused the current hospital admission may

be controlled with medication and nutrition intervention, this patient will continue to experience

numerous complications related to her mixed connective tissue disorder for the rest of her life


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Current Admission

Active Problems/Diagnoses:

Present Upon Hospital Admission:

o Failure to thrive

o Dysphagia

o Malnutrition


o CREST syndrome


Acquired/Developed During Hospital Admission:

o Urinary tract infection (UTI)

o Nephrolithiasis

o Bacterium pseudomonas

Diagnostic Procedures: Purpose:

Clostridium Difficile Culture Procedure indicated due to diarrhea and medication history.

Lab results positive for C. diff, antibiotics started.

CT Abdomen/Pelvis WO Procedure indicated due to abdominal pain and suspected

kidney stones. Kidney stones confirmed. All other findings

appear normal.

CR Retrograde Urogram Procedure indicated to analyze kidney stones. Bilateral

ureteral stones for analysis; integrated crystallographic

analysis of urinary calculi. Specimen consists of several

irregularly shaped calculus fragments, weight a total of 27.4

mg.

CT Chest PE Protocol WO Procedure to rule out pulmonary embolism. No convincing

evidence to suggest pulmonary artery thromboembolism.

Esophagus is dilated and demonstrates and air-fluid level

likely secondary to esophageal dysmotility. Upper abdomen

shows diffuse hepatic steatosis. There is diffuse bronchial

wall thickening present. There is an addition of a 1.6 x 1.473

mm cavitary lesion seen within the R upper lobe. Multiple

calcifications identified within bilateral breast tissue.

EKG/ECG Procedure indicated due to cough. Sinus tachycardia, aberrant

complex, possibly supraventricular, probable L atrial

abnormality.

CR Abdomen 1 Vw Port Procedure indicated due to flank pain. Scattered calcifications

on the R kidney represent R renal calculi. Faint calcifications

on the L kidney may represent L renal calculi.

Nonobstructive bowel gas pattern with moderate colonic stool

burden.

CR Chest 1 Vw Port Procedure indicated due to cough. Minimal L basilar

atelectasis noted. All other findings appear normal.

CR Esophagram Procedure indicated due to dysphagia. Absence of the

primary and secondary esophageal contractions with

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esophageal dilation up to 2 cm from the gastroesophageal

junction, which is narrowed to less than 1 cm in diameter.

Marked delayed esophageal emptying, which only occurred

when the patient was in the fully upright position with

repeated dry swallowing. No gastroesophageal reflux was

elicited.

CR Swallowing Funct Video/

Modified Barium Swallow

Study

Procedure indicated due to dysphagia. There is no evidence

of laryngeal penetration or tracheal aspiration with any

consistency. Normal transit time is observed.

Esophagogastroduodenoscopy/

Esophageal Biopsy

Procedure indicated due to dysphagia. Diagnosed esophageal

ulcer through fragment of granulation tissue with food

particles consistent with ulcerative esophageal mucosa with

chronic inflammation. Special fungal stains are negative.

Treatments: Purpose/Outcome:

Cystoscopy Stent Insertion Procedure indicated due to the presence of kidney

stones. Stent placed to unblock the kidney.

Cystoscopy Ultrasonic Lithotripsy Procedure indicated due to the presence of kidney

stones. Noninvasive procedure used to break up

large kidney stones into smaller particles to allow

them to pass.

XA CVC Tunneled 2 Cath WO Pump/Port Procedure for placement of a tunneled central line

for use in long-term venous access for therapy.

Tunneled central line was successfully placed with

no complications.

Occupational Therapy Patient originally agreeable to OT, participated in 2

sessions with little progress. Later refused OT.

Discharged due to lack of progress/plateau and

patient refusal, no further therapy planned.

Speech Therapy Modified Barium Swallow Study – Oropharyngeal

swallowing within normal limits. No further speech

therapy indicated.

Physical Therapy Patient refused PT, stating she does better on her

own. Discharged from PT, no further therapy

planned.

Pain Management Consulted as pain management is outside the scope

of primary team. Assessed pain and treated

accordingly.


Medications: Purpose & Drug-Nutrient Interactions:

Phenergan Used to treat nausea and vomiting. Do not take with alcohol, may cause

uncontrollable movements, agitation, seizures, severe dizziness or fainting,

coma, very deep sleep, irregular heart beats, and high or low body temperature.

Prednisone Used to treat inflammation caused by mixed connective tissue disorder.

Dietary sodium restriction and potassium supplementation may be advisable as

this medication may cause fluid retention.

Zofran Used to treat nausea/vomiting. Does not have any known drug-nutrient

interactions.

Oxycodone Used for pain control/management. Do not take with alcohol, may cause

drowsiness, dizziness, lightheadedness, difficulty concentrating, and

impairment in thinking and judgment. In severe cases, low blood pressure,

respiratory distress, fainting, coma, or even death may occur. Avoid or limit

consumption of grapefruit and grapefruit juice, which can significantly

increase blood levels.

Dilaudid Used for pain control/management. Do not take with alcohol, may cause

drowsiness, dizziness, lightheadedness, difficulty concentrating, and

impairment in thinking and judgment. In severe cases, low blood pressure,

respiratory distress, fainting, coma, or even death may occur.

Reglan Used to treat gastroparesis. Increases muscle contraction in the upper digestive

tract to increase the rate at which the stomach empties into the intestine. Do not

take with alcohol, may cause dizziness, drowsiness, and difficulty

concentrating.

Ativan Used to treat anxiety. Do not take with alcohol, may cause dizziness,

drowsiness, and difficulty concentrating.

Megace Used to stimulate appetite. No known drug-nutrient interactions.

Benadryl Used to treat/control pain. Do not take with alcohol, may cause drowsiness,

dizziness, and in severe cases, serious liver damage.

Lovenox Used to treat/prevent deep vein thrombosis blood clots, which lead to

pulmonary embolism.

Tylenol Used to treat/control pain. Do not take with alcohol, may cause serious liver

damage.

TUMs Used to treat acid reflux/upset stomach. No known drug-nutrient interactions.

*NOT INDICATED IN PATIENTS WITH A HISTORY OF KIDNEY

STONES.

Remeron Used to treat major depressive disorder. Do not take with alcohol, may cause

dizziness, drowsiness, and difficulty concentrating.

Protonix Used to decrease stomach acid production and treat esophagitis. No known

drug-nutrient interactions.

Plaquenil Used to treat symptoms of systemic lupus erythematous. No known drug-

nutrient interactions.

Vancomycin Used to treat C. diff and UTI. No known drug-nutrient interactions.


Nutrition Care Process

Nutrition Assessment:

Active Problems/Diagnoses:

o Failure to thrive

o Dysphagia

o Malnutrition


o CREST syndrome


o Urinary tract infection (UTI)

o Nephrolithiasis

o Bacterium pseudomonas

Nutrition-Related Problems:

o Inadequate intake related to dysphagia.

o Malnutrition related to inadequate intake.

o Frequent loose, watery bowel movements related to positive C. diff culture. Monitor for

dehydration and electrolyte status due to diarrhea.

Anthropometrics:

Height: 62” Weight (kg): 46 kg Weight (lb): 101 lbs

BMI: 18.5 IBW: 50 kg %IBW: 92%

Biochemical Labs:

Day 1:

Day 3:

Day 5:

Day 8:


Day 10:

Day 12:

Day 16:

Day 19:

Day 23:

Day 30:

Day 37:

Estimated Nutritional Needs:

Energy (kcal): 1500 Calculation: 33 kcal/kg BW

Protein (g): 100 Calculation: 2.2 g/kg BW

Water (ml): 1500 Calculation: 33 ml/kg BW

Diet History:

Days 1-11 Regular Diet

Days 12-18 Pureed diet

Day 19 TPN Day 1: 1200 ml/day with 100 g PRO, 400 kcal CHO, 300 kcal lipids

Days 20-22 TPN Day 2: 1200 ml/day with 100 g PRO, 800 kcal CHO, 300 kcal lipids

Days 23-37 TPN Revised: 1200 ml/day with 100 g PRO, 970 kcal CHO, 300 kcal lipids MWF


Social Nutrition-Related Factors: Patient admitted to hospital frequently with extended lengths

of stay (>40 days). At home, patient requires ambulatory assistance and relies on

parents/grandma for assistance with shopping and meal delivery.

Previous Diet Instruction: Patient previously with PEG that developed gastrocutaneous fistula

that has since undergone revision. Patient is not a candidate for another PEG due to the location

and extent of previous fistula. Patient previously with NGT and refuses placement at this time

stating it is too uncomfortable. Patient is familiar with a variety of nutritional supplements

including Ensure, Ensure Plus, and Ensure Clear. States she does not enjoy the flavor but will try

to consume them, as she knows she needs nutritional support.

Current Intake: Patient reports minimal PO intake due to difficulty swallowing and decreased

appetite. Calorie count ordered by primary team to assess intake but discontinued after Day 1 due

to issues with nursing staff compliance. Intake as reported by patient is not at all sufficient to

meet calorie and protein needs.

Level of Risk: Nutrition Care Level 1 – High Risk due to past medical history and active

problems/diagnoses including an existing malnutrition diagnosis.

Nutrition Diagnoses:

Initial: Inadequate protein/energy intake related to decreased appetite, difficulty

swallowing evidenced by patient report of poor PO intake.

Initial: Malnutrition related to inadequate protein/energy intake as evidenced by BMI of

18.5 and 92% IBW at admission.

Day 10: Inadequate protein/energy intake related to dysphagia as evidenced by low PO

intake x several weeks, hypoglycemia.

Day 23: Altered nutrient related laboratory values related to hepatic steatosis as evidenced

by Alk Phos 252, ALT 64, AST 145, TG 345.

Nutrition Intervention:

Initial: Continue regular diet. Encourage PO intake to meet nutritional needs. Will send

chocolate Ensure Plus TID with meals to increase kcal/protein intake. Monitor for results of

EGD.

Monitoring/Evaluation/Follow-Up:

Day 3 Intervention Continue regular diet. Encourage PO intake to meet

nutritional needs. Continue chocolate Ensure Plus TID with

meals to increase kcal/protein intake. Will send Magic Cup

TID to increase kcal/protein intake. Will send ProMod, take

30 ml/day in water, Sprite, or juice to increase protein

intake.

Day 5 Progress Note Revisited the idea of NGT with patient. Patient still refuses,

stating NGT is too uncomfortable. Patient not a candidate

for PEG due to previous gastrocutaneous fistula.

Intervention Continue regular diet. Encourage PO intake to meet

nutritional needs. Continue chocolate Ensure Plus TID,


Magic Cup TID, ProMod.

Day 8 Intervention Continue regular diet. Encourage PO intake to meet



Day 10 Progress Note Recommended pureed diet secondary to dysphagia but

patient refused. Discussed the possibility of/indications for

TPN with primary team. Attending prefers to keep patient

on PO diet at this time but realizes the possible need for

TPN in the future. Spoke with patient’s insurance provider,

who stated that the patient meets the diagnostic criteria and

TPN would be covered by insurance if implemented.

Intervention Continue regular diet for now and encourage PO intake to

meet nutritional needs. Continue chocolate Ensure Plus TID,


Day 12 Progress Note Patient willing to try pureed diet at this time. Change diet

consistency to pureed secondary to dysphagia.

Intervention Begin pureed diet, encourage PO intake to meet nutritional

needs. Continue chocolate Ensure Plus TID, Magic Cup

TID, ProMod to increase intake.

Day 16 Intervention Continue pureed diet, encourage PO intake to meet



Day 19 Progress Note Attending recognizes the need for TPN at this time and

consulted RD for TPN recommendations.

Intervention Begin TPN. [TPN calculations in Appendix.] Patient at risk

for refeeding syndrome; monitor K, Mg, Phos x 2 days and

replace as needed. Check baseline triglycerides (TG).

Day 23 Progress Note TPN Day 4. Elevated liver enzymes secondary to hepatic

steatosis – modify TPN order to cycle lipids.

Intervention [TPN calculation in Appendix.] Cycle lipids MWF. Increase

CHO kcal to 970/day. Check TG.

Day 30 Progress Note TPN Day 11. Patient states she is “tired of being poked at.”

Morale is low.

Intervention Continue current rate of parenteral nutrition.

Day 37 Progress Note TPN Day 18. Patient to be discharged home, will stop TPN

before discharge. Patient will restart pureed diet at home.

Insurance did not approve home supplements (i.e. Ensure

Plus). Goals of Care: Discharge home with pain

medications, pureed diet, and adaptive equipment. Patient’s

family on board.

Intervention: Discontinue TPN before discharge. Begin pureed diet at

home, supplement with Ensure Plus as able.


ADIME Note [Initial Assessment]:

Assessment: 29 year old female presents with generalized pain, decreased PO intake, fatigue,

hypoglycemia, and emesis x several days. PMHx SLE, CREST, C. diff, VRE, CKD, PUD, HTN,

chronic steroid use, malnutrition, gastrocutaneous fistula s/p resection.

Height: 62” Weight (kg): 46 kg Weight (lb): 101 lbs

BMI: 18.5 IBW: 50 kg %IBW: 92%

Estimated Energy Needs: 1500 kcal Calculation: 33 kcal/kg BW

Estimated Protein Needs: 100 g Calculation: 2.2 g/kg BW

Estimated Fluid Needs: 1500 ml Calculation: 33 ml/kg BW

Labs:

Medications: Reglan, Lovenox, IVF

Diagnosis:

Inadequate protein/energy intake related to decreased appetite, difficulty swallowing evidenced

by patient report of poor PO intake.

Malnutrition related to inadequate protein/energy intake as evidenced by BMI of 18.5 and 92%

IBW at admission.

Intervention:

Goal: 50-100% intake of meals/snacks/supplements

Interventions:

1. Continue regular diet. Encourage PO intake to meet nutritional needs.

2. Will send chocolate Ensure Plus TID with meals to increase kcal/protein intake.

3. Nutrition Care Level 1 (High Risk)

Monitoring & Evaluation: Monitor patient every 2-4 days based on high-risk status protocol.

Monitor PO intake per patient and RN report. Watch for results of EGD and adjust diet order

accordingly.


Summary

In conclusion, mixed connective tissues disorder may present as serious clinical

manifestations such as esophageal dysmotility and dysphagia, chronic kidney disease, pulmonary

hypertension, and heart, lung, and neurological involvement. Due to the complicated nature of

MCTD and the unknown etiology, it is very difficult to treat. Current treatment therapies seek to

alleviate pain and address the symptoms and complications associated with MCTD. However,

the disease and its complications take a toll on the body and the prognosis is typically poor.

In the case examined, the patient had both the systemic lupus erythematous and CREST

syndrome components of MCTD. She experienced severe nutrition-related complications

associated with MCTD including esophageal dysmotility, kidney involvement, pulmonary

hypertension, and weakened immune system leading to susceptibility to hospital-acquired

infections. The patient’s esophageal dysmotility presented as gastroparesis and dysphagia, which

in turn led to malnutrition and finally a diagnosis of “failure to thrive.” The patient also faced an

additional nutrion-related barrier in the form of a gastrocutaneous fistula from a previous PEG,

which limited options for an alternative nutrition route. Eventually, TPN was used to provide the

patient with nutrition in the hopes of reversing her malnutrition status and addressing her “failure

to thrive.” Unfortunately, the patient continued to struggle with other MCTD complications

including nephrolithiasis, pulmonary hypertension, and difficulty with ambulation. The patient

was discharged home with palliative care. The attending physician maintains that the prognosis

is poor due to the complicated and aggressive nature of the disease as well as the patient’s lack of

compliance and diminishing will to live.

I found this case very interesting due to the complexity of MCTD and its associated

complications. The patient’s lengthy hospital admission allowed me to get to know her on a


personal level, follow her progress, discuss the case with the attending physician, and make

several nutrition interventions along the way. As the physician also recognized, the patient

seemed very defeated from the beginning and her compliance and hopefulness diminshed

throughout the length of her hospital admission. I believe this patient would have benefitted

greatly from alternative medicine routes or experimental treatments as the currently accepted

protocols for treating MCTD are limited and do not seek to address the underlying disease, only

to mask the symptoms. Given the current research and the case examined, I believe additional

research on MCTD is warranted in order to determine its etiology and develop more effective

treatment protocols and improve patient prognosis.


References

Johns Hopkins Medicine. (2016). The Johns Hopkins Scleroderma Center. Retrieved July 3,

2016, from http://www.hopkinsscleroderma.org/scleroderma/types-scleroderma/

Mayo Clinic. (2016). Lupus. Retrieved July 3, 2016, from http://www.mayoclinic.org/diseases-

conditions/lupus/basics/definition/con-20019676

National Institute of Diabetes and Digestive and Kidney Diseases. (2016). Hypoglycemia.

Retrieved July 1, 2016, from https://www.niddk.nih.gov/health-information/health-

topics/Diabetes/hypoglycemia/Pages/index.aspx

Nisa, L., & Giger, R. (2011). Dysphagia, oral telangiectasia, and raynaud syndrome.

Otolaryngology – Head and Neck Surgery, 146(4), 676-677.

doi:10.1177/0194599811431060

Ortega-Hernandez, O., & Shoenfeld, Y. (2012). Mixed connective tissue disease: An overview of

clinical manifestations, diagnosis and treatment. Best Practice & Research Clinical

Rheumatology, 26(1), 61-72. doi:10.1016/j.berh.2012.01.009

Smout, A., & Fox, M. (2012). Weak and absent peristalsis. Neurogastroenterology & Motility,

24(1), 40-47. doi:10.1111/j.1365-2982.2011.01831.x

Tang, S. (2014). Endoscopic management of gastrocutaneous fistula using clipping, suturing, and

plugging methods. Video Journal and Encylopedia of GI Endoscopy, 2(2), 55-60.

doi:10.1016/j.vjgien.2014.03.001


Appendix

Day 19 TPN Calculations:

Estimated Energy Needs: 1500 kcal Calculation: 33 kcal/kg BW

Estimated Protein Needs: 100 g Calculation: 2.2 g/kg BW

Estimated Fluid Needs: 1500 ml Calculation: 33 ml/kg BW

Maximum Glucose Utilization Rate:

46 kg x 5 x 24 hr/day x 60 min/hr ÷ 1000 x 3.4 kcal/g CHO = 1126.08 CHO kcal/day

TPN Day 1: 100 g PRO

300 kcal lipids

400 kcal CHO

1100 kcal

TPN Day 2: 100 g PRO

(Goal) 300 kcal lipids

800 kcal CHO

1100 kcal

Monitor K, Mg, Phos x 2 days and replace prn 2/2 refeeding risk.

Check baseline TG. Keep below 400 while on TPN.

Day 23 TPN Revisions:

Biochemical Labs: Alk Phos 252, ALT 64, AST 145, TG 345

Elevated liver enzymes – cycle lipids every other day (MWF) 2/2 hepatic steatosis.

4 days without lipids x 300 kcal lipids/day = 1200 kcal/week deficit ÷ 7 days/week = ~170

kcal/day deficit without lipids

800 kcal CHO + 170 kcal = 970 kcal CHO

MWF TPN: 100 g PRO

300 kcal lipids

970 kcal CHO

1670 kcal

Revised 100 g PRO

TPN: 970 kcal CHO

1370 kcal

FAILURE TO THRIVE-3

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