MCTD complications

Running head: FAILURE TO THRIVE 1
Failure to Thrive: Complications Associated with Mixed Connective Tissue Disorders
Abigail Smith
University of Kentucky

FAILURE TO THRIVE 2
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.

FAILURE TO THRIVE 3
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

FAILURE TO THRIVE 4
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

FAILURE TO THRIVE 5
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.

FAILURE TO THRIVE 6
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.

FAILURE TO THRIVE 7
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

FAILURE TO THRIVE 8
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

FAILURE TO THRIVE 9
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

FAILURE TO THRIVE 10
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

MCTD complications

Recommended

Recommended

More Related Content

What's hot

What's hot (20)

Similar to MCTD complications

Similar to MCTD complications (20)

MCTD complications