UVA Health System Dietetic Internship Capstone Project A Case Series of Pediatric Patients on Home Parenteral Nutrition for the ASPEN Sustain™ Database Sarah Horton April 29, 2013 Preceptor: Brandis Roman, MS, RD, CNSD
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UVA Health System Dietetic Internship Capstone Project
A Case Series of Pediatric Patients on Home Parenteral Nutrition for the ASPEN Sustain™ Database
Sarah Horton April 29, 2013
Preceptor: Brandis Roman, MS, RD, CNSD
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TABLE OF CONTENTS
ABSTRACT .................................................................................................................................................................... 3
LITERATURE REVIEW ............................................................................................................................................. 4 What is Parenteral Nutrition? ............................................................................................................................. 4 History of Total Parenteral Nutrition .............................................................................................................. 5 Indications for Use of TPN/HPN ...................................................................................................................... 6 Benefits of HPN ..................................................................................................................................................... 8 Risks and Safety Concerns ................................................................................................................................. 9
Central Line Associated Blood Infections & Sepsis ............................................................................. 9 Catheter Malfunction ..................................................................................................................................... 11 PNALD .............................................................................................................................................................. 12 Psychosocial Issues ........................................................................................................................................ 13
Purpose of Patient Registries ........................................................................................................................... 14 The SUSTAIN™ Database by ASPEN .................................................................................................. 15
PROJECT PURPOSE ................................................................................................................................................. 17
PROJECT METHODS ............................................................................................................................................... 18
PROJECT DEVELOPMENT OVERVIEW ........................................................................................................... 19 Anthropometrics ................................................................................................................................................... 19 Lab Values ............................................................................................................................................................. 19 Hospitalizations .................................................................................................................................................... 20 Case Descriptions ................................................................................................................................................ 21
DISCUSSION ............................................................................................................................................................... 24
CONCLUSION ............................................................................................................................................................. 28
REFERENCES ............................................................................................................................................................. 29
APPENDIX ................................................................................................................................................................... 31
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ABSTRACT
Home parenteral nutrition (HPN) is a required therapy used by patients with medical
conditions that prevent them from maintaining adequate nutrition status with enteral nutrition
alone. HPN has become increasingly common in children with life-threating malnutrition
stemming from short bowel syndrome or surgical treatments. Although HPN is beneficial in
preventing malnutrition, one of the main barriers in its use is a long list of safety concerns.
Patients on HPN are highly susceptible to central line associated bloodstream infections
(CLABSI), liver damage, and psychosocial issues, especially when long-term use is indicated.
SUSTAINTM, a web-based database created by The American Society of Parenteral and Enteral
Nutrition (ASPEN), collects data on patients and specific populations requiring HPN in the
United States. It is meant to monitor outcomes, allow benchmarking against other institutions,
and publish significant findings to share improvements in patient care.
This study will examine six pediatric patients who are followed by the pediatric GI team,
including a Registered Dietitian, at the University of Virginia Health System. This study will
collect baseline and follow up data from December 2012 to March 2013. The de-identified data
will then be shared with SUSTAINTM database. This study will use the data to assess several
major outcomes of the patients on HPN within our institution and will evaluate trends in areas
that could indicate further improvements in HPN management.
The results of this study suggest that CLABSI and catheter damage are the leading causes
of rehospitalization in the patients on HPN. Of the six original patients, one was able to transition
off of HPN. One patient underwent surgery to restore intestinal continuity. All of the patients
have experienced some weight maintenance or weight gain while on HPN. The data revealed that
the patients have been able to improve their nutritional status while on HPN, however, it should
be considered that HPN is associated with complications including catheter malfunctions, blood
stream infections, electrolyte abnormalities, and hyperglycemia.
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LITERATURE REVIEW
What is Parenteral Nutrition?
Total parenteral nutrition (TPN) has been one of the most important therapies for
optimizing nutrition in individuals who cannot consume food conventionally through the gut.
While oral or enteral nutrition is usually preferred, some patients require TPN due to conditions
that restrict the use of the gut. TPN provides most of the patient’s nutrition needs in their
simplest form. Carbohydrates, protein, fat, micronutrients, and electrolytes are delivered directly
into the central venous system through a catheter (1). TPN can be provided as a 2-in-1 solution
of amino acids and carbohydrates with electrolytes and a separate fat emulsion delivered
intravenously or as a 3-in-1 solution containing all of the components in one solution (2).
Home parenteral nutrition (HPN) is used in patients who require protracted
hospitalization and will need additional nutrition support after discharge. The duration of HPN
therapy will vary on the individual’s condition. Some may achieve enteral or oral autonomy after
healing or adaptation of the gastrointestinal tract, while others may be on HPN, providing either
full or partial nutrition, for life (1). Although not without complications, HPN has greatly
improved morbidity and mortality in these patients over time (3).
The process of initiating HPN starts during hospitalization. HPN can be very
complicated for patients and their families; therefore, a multidisciplinary team should help to
determine the suitability of the home environment for HPN and transition the patient from
hospitalization to home. This team includes the hospital nutrition support team, a social worker,
case manager, psychologist, pharmacist, and a home health care agency. The health care team,
including a registered dietitian, will determine appropriate amounts of calories, protein, fat,
micronutrients, and fluid in the HPN solution necessary to maintain normal functions and age-
appropriate growth, if applicable. Generally, patients reach goal TPN before being discharged (1).
Once the team and patient/caregiver have elected to start HPN, several decisions must be
made in terms of quality of life. For instance, cyclic HPN can enhance quality of life and allow
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for normal activities, including school, work, and therapies. Cyclic HPN is also recommended to
preserve liver function; however, glucose control can be complicated with cycled TPN and
patients must be able to tolerate large volumes over shorter infusion duration. The patient’s
biochemical data, anthropometrics, and bone mineralization should be evaluated often when
cycling TPN (1).
History of Total Parenteral Nutrition
The use of parenteral nutrition (PN) in general is a fairly new concept, with first major
developments occurring in the mid-20th century. TPN has become an increasingly common form
of nutrition support in children with both digestive and non-digestive conditions inhibiting the use
of the gut over the last three decades (3). After experiments in animals, PN was first used in
critically ill children (4). The first experiments in TPN use in infants, completed by Dr. Jonathan
Rhoades, Dr. Stanley Dudrick, and their colleagues, occurred at the Children’s Hospital of
Philadelphia in 1967 with a malnourished one-month-old infant with 95% of her small bowel
removed (5). The infant’s weight and head circumference increased while on TPN and she was
able to complete normal activities for her age. With this infant, Dudrick discovered that PN
formulas were deficient in vitamin D and essential fatty acids, and supplementation of these
nutrients was required (4).
HPN is also a fairly new practice in the United States. The first patient to receive TPN in
a home setting was a 36-year-old woman with extremely complicated ovarian cancer in 1968.
She was also treated by Dr. Rhoades and survived for 6 months on HPN. Just a few years later
in 1970, Dr. Rhoades used HPN in a patient with severe short bowel syndrome (SBS) to eliminate
her malnutrition and greatly improve her quality of life. She lived at home on HPN for 15 years.
In 1974, the first HPN manufacturing company was founded in Houston, Texas as a collaboration
between doctors, pharmacists, pharmaceutical companies, and medical device companies (4).
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During the latter half of the 20th century, many advances were made in HPN care. The
HPN manufacturing industry experienced a booming growth, as HPN became a standard practice
around the country. HPN also served as a great leap for home health care and outpatient therapy,
in general. Patients were able to survive up to 30 years on HPN. Infusion devices and catheters
greatly improved as technology expanded. Finally, on June 5, 1975, PN experienced its
organizational expansion with the establishment of ASPEN (4). The use of HPN has had a more
rapid expansion rate in the United States compared to other Western nations. This is mostly
attributed to the ability of insurance companies to subsidize the cost of treatment and equipment
for HPN for patients (6).
TPN has become increasingly common in children with life-threating malnutrition
stemming from SBS or surgical treatments. A 2009 survey by the National Center for Health
Statistics reported that of 36,000 patients on HPN, 33% of those were children (2). Starting
nutrition in some form is more critical in children than in adults. Adults can survive up to 90
days without nutrition, however infants can only withstand starvation for 4 days (23). Conditions
requiring HPN in children include many motility disorders, SBS with <500 mm of ganglionic
colon intact, microvillus inclusion disease, and chronic pseudo-obstructions. These children may
require permanent HPN, but some may only require intermittent periods of HPN. HPN is
commonly indicated through the teenage years to maximize linear growth throughout puberty
(23).
Indications for Use of TPN/HPN While oral and enteral nutrition are the preferred methods for nutrition therapy, there are
times when these modalities are not feasible for patients. TPN/HPN use is indicated in children
who are no longer able to fully meet nutrition requirements due to intestinal failure. This is
defined as, “a non-functioning small bowel, either due to the resection and leading to SBS or due
to impaired intestinal motility or absorption” (1). This can include conditions such as SBS,
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radiation enteropathy, pancreatitis, enterocutaneous fistula, and intestinal dismotility, as well as
conditions resulting from illness or trauma (2).
Certain kinds of cancer can also be indications for HPN. Similar SBS or motility
disorders, any cancer that results in a non-functioning GI tract can indicate a need for nutrition
support (9). However, because malnutrition is often a complication secondary to cancer,
particularly those resulting in mucositis and enteritis, and its treatments, some patients many
benefit from HPN. In patients requiring nutritional supplementation, HPN has been shown to
decrease mortality and improve quality of life. This is a very individualized option and is
recommended for shorter periods of time. The patient’s prognosis and overall quality of life
should be considered before initiation of HPN (10).
Duration of restricted oral intake is also an indication for the initiation of TPN/HPN. In
pediatric patients, 5 to 7 days without oral intake is an indication for inpatient TPN. For
premature infants or those with very low birth weight (<1000-1500 g), TPN may be needed as
additional support for growth. These infants may not have an optimally developed digestive
system and cannot tolerate enteral feeds. TPN should be initiated immediately in this population,
as many cannot last 24 hours without nutritional support (2). Guidelines for HPN are not as
concrete. Many suggest that HPN should be considered if it will be required for a period longer
than 90 days, but situations such as chemotherapy treatment and malnutrition related to cancer
may warrant HPN for a shorter period of time (1,10).
The child’s prognosis must also be taken into account when considering the initiating of
HPN. Their clinical condition should be generally stable to avoid complications. They must also
have highly motivated and involved parents or caregivers to help with the burdens of medical
care. HPN has many demands, both emotionally and technically. Adequate storage space must
be available for storing supplies. Parents or caregivers must also be supportive of the child
emotionally, help with transportation for medical care, and be present at appointments if any
changes are made to the home regimen (6). While the challenges must be considered in the
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initiation of HPN, there are resources available to help caregivers so the child may better benefit
from HPN therapy.
Benefits of HPN The major benefit HPN has provided is increased survival rate in children who previously
would have died from malnutrition. Because of HPN, children are able to maintain a relatively
normal life receiving nutrition support. Survival rates have greatly increased in children with
SBS due to HPN. Survival rates in children with less than 40 centimeters of small intestine have
increased from 42% in the 1980 to 94% today and to 97% in children with 40-80 centimeters of
small intestine (6). Providing PN in the home setting versus requiring the patient to be
hospitalized for the duration of parenteral therapy allows the patient to resume activities of daily
living (ADL), decreases chances for infection, and lowers health care costs (11).
Patients on HPN report an increased sense of security, as meeting nutritional needs is not
as much of a concern. HPN provides sufficient support to increase body weight, energy level,
strength, and ability to complete physical activities (9). Many studies report that coordination of
care between physicians, parents, home health care, and outpatient resources optimizes benefits
of HPN, especially with initial education and follow up training at home (12; 1).
HPN cycling can also optimize benefits in the pediatric population. Cycling helps to
establish glucose stability, to prevent pump malfunction and infusion issues, and to decrease
instances of parenteral nutrition-associated liver disease (PNALD), which is particularly
important as this population is already at a higher risk of developing PNALD compared to adults.
Cycling also offers the psychological benefits of a scheduled feeding regimen and improves daily
living, allowing for more normal school and social life (12).
Although there is some controversy surrounding the topic, long-term nutrition support in
cancer patients has provided benefits in terms of malnutrition and improving quality of life. HPN
has been shown to assist with the treatment and prevention of malnutrition, which reduces the
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complications from cancer treatment. Nutrition support through HPN can improve energy
balance, body composition, physical functioning ability, and overall quality of life. It also been
shown to increase the lifespan of patients. The use of HPN in cancer patients is very
individualized, and the best results were seen when HPN was combined with nutritional support
from enteral nutrition (10).
Risks and Safety Concerns
One of the main barriers to HPN use is a long list of safety concerns. Patients on HPN
are highly susceptible to infections via the catheter, liver damage, and psychosocial issues,
especially when long term HPN is required (1). In a study by Seidner et al. (8), most patients
experienced complications within the first 90 days on HPN. Of the 97 patients in the study, 33
experienced complications, 89% resulting in visits to the Emergency Room. The most common
problem was infection, followed by mechanical and metabolic issues (7). In terms of long-term
complications, PNALD has been reported in 50-66% of children receiving HPN, especially when
the child was born premature. This complication is generally seen more frequently in children
than adults due to immature liver function (8).
There is some evidence to suggest that more comprehensive patient education leads to
fewer complications. The study by Smith et al. (13) suggested that a comprehensive patient
education course on the prevention of depression and infection resulted in fewer cases of reactive
depression and rehospitalization due to infections compared to a control group (13). However,
complications do occur in these patients and should be addressed by their health care providers to
determine the best solution.
Central Line Associated Blood Infections & Sepsis
Central line associated bloodstream infection (CLABSI) is estimated to occur in 1.3-
26.2% of patients on TPN and is most common in children with SBS and in very low birth weight
infants (VLBW) (14, 2). These infections are hypothesized to occur due to the high susceptibility
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of TPN formulas to microbial growth given their high concentration of amino acids, dextrose, and
fat, which support and sustain bacteria growth. The central line is a direct access point to the
bloodstream. This line is accessed at least twice per day to begin and end TPN infusion. By this
route, environmental bacteria can directly enter the bloodstream, causing sepsis. Infection can be
exacerbated in patients on HPN because of immunosuppression associated with various disease
states. Malnutrition and disease states also initiate a stress response in the body that can cause
hyperglycemia. Dextrose infusion can further increase blood glucose, which makes the body
highly susceptible to bacterial infections (14). Although less common, fungal infections can also
lead to CLABSI and generally result in more instances of catheter removal than bacterial
infections (15).
Infection can also be related to the catheter placement. Bacteria can colonize at the
insertion site, which can be detrimental as the line feeds directly into the bloodstream. The
subclavian vein is considered the safest site as the catheter can be easily anchored and dressed.
Comparatively, jugular access is susceptible to more body movement, and femoral access is
highly susceptible to movement and contamination from fecal or urinary particles (14).
Patients and their caregivers must practice safe aseptic techniques when handling HPN to
minimize risk of bacterial infection. HPN supplies should be stored in proper conditions.
Dextrose and amino acid infusion sets should be changed every 72 hours and fat emulsions every
12 hours (2). This can be difficult to control outside of a hospital setting due to restricted and
unsterile storage space and irregular infusion times (14).
If a patient does experience a CLABSI, it can be detrimental. Acutely, the patient may
not be able to receive nutritional support safely, which, in extreme cases, can lead to significant
dehydration, electrolyte disarray, and eventual death (11). Hospital admission is usually required,
which can expose the patient to more pathogens and staff who may not be familiar with HPN
practices. The patient would start on intravenous antibiotics immediately, with subsequent
analysis of blood cultures to determine specific organisms and antimicrobial susceptibility. The
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patient may need catheter replacement if the infection is not cleared within 48 hours. If this
occurs, a peripheral line will need to be placed to continue intravenous antibiotic treatment;
gaining peripheral access can often be difficult, particularly in small and dehydrated children. For
the patient with a repeated need for central line removal and replacement due to recurrent
CLABSI, there is concern that eventually all central line sites will be used, and eventually the
patient will “run out” of potential sites, making HPN essentially impossible. This necessitates a
discussion of more aggressive intestinal failure therapy, such as intestinal transplant (14).
There is evidence to suggest that catheter lock therapy is effective in infection prevention.
Ethanol locks seem to be the most effective in reducing CLABSI in HPN patients (14). The
Centers for Disease Control and Prevention recommends using ethanol locks in patients on long
term HPN with a history of CLABSI, as they have been shown to reduce infections by 81% and
catheter replacements by 72% (2). Although ethanol locks show significant benefits in reducing
CLABSI, compliance can be an issue in HPN patients. Many stop using the lock when they are
feeling well (16). Other strategies for reducing infection include using a combination of enteral
and PN to stimulate gut immune function and prevent overfeeding to reduce hyperglycemia.
Some studies preliminarily suggest supplementing with omega-3 fatty acids and glutamine can
help to support immune function, although more research is required to prove the benefits of
these supplements (14).
Catheter Malfunction Catheter malfunctions are another common complication in patients receiving HPN.
Catheter occlusion occurs due to a blockage of the central line due to thrombosis, precipitation of
drugs or minerals, or deposition of intravenous lipids (17). They are more common in children,
as they have much smaller blood vessels, compared to adults, although its occurrence is quite
variable, ranging from 2-75%. When this occurs, fluids cannot be flushed through the line and
blood cannot be drawn out. An occlusion can be problematic to patients because they are often
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asymptomatic; furthermore, the occlusion can foster bacterial growth and cause pain, swelling,
and cramps, and loss of venous access. As previously mentioned, if central lines must chronically
be replaced due to catheter thrombosis or malfunction, eventually the patient will exhaust all
central line sites, which can indicate the need for an intestinal transplant in those with intestinal
failure. Occlusions can be treated using fibrinolytic agents or flush fluids, like heparin, and then
aspirating the occlusion (18).
Using aseptic technique at all times can prevent catheter occlusions and subsequent
infections. Most protocols suggest using a routine unfractioned heparin flush or saline flush to
prevent catheter thrombosis. Heparin is cautioned in children due to risk for heparin induced
thrombosis and bone demineralization. However, saline flushes have also been correlated with
catheter occlusions and infections, although this may be related to less frequent flushes. Some
studies also suggest using a drug, such as a low dose of warfarin, can help to prevent thrombosis
from occurring (18).
PNALD PNALD, a common long-term complication associated with HPN and occurring in 30-
70% of HPN cases (2) is also referred to as cholestasis, PN-associated liver injury, and intestinal
failure-associated liver disease. It is especially common in premature infants who do not have a
mature hepatobiliary system to filter toxins. The exact cause of PNALD is not known and is
likely multi-factorial. Immature liver function, inflammation, infections, oxidative stress, nutrient
deficiencies, and contaminants are all linked to increased risk of PNALD (8). The high
concentration of dextrose and fat in the PN formula is theorized to stimulate high insulin levels
and to promote fatty liver by increasing fatty acid synthesis (2). This can progress to cholestasis,
to cirrhosis, and finally, to liver failure if not treated. PNALD is diagnosed based on abnormal
levels on a hepatic panel: aspartate aminotransferase (AST), alanine transferase (ALT), alkaline
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phosphate, and total and conjugated bilirubin (8). The levels should be checked regularly to
monitor for evolving or worsening PNALD.
PNALD can be prevented or reversed if caught early enough with the initiation of enteral
nutrition and discontinuation of HPN; however, for patients with conditions such as SBS, this is
not an option (8). Other options for treatment include cycling HPN to 12 to 20 hours a day
instead of 24 hours a day, as fasting allows for fatty acid mobilization. This technique may slow
the progression of PNALD, but there is no evidence to show prevention entirely (2, 8).
Other nutritional therapies have been used to treat PNALD, such as supplementing
omega-3 polyunsaturated fatty acids enterally or using fish oil based intravenous fat emulsion
(IVFE), such as Omegaven®. While effective in reversing cholestasis, Omegaven® is not
available in the United States for routine use, outside of compassionate use and clinical trial
protocols. Omega-3 based lipid formulas are believed to decrease inflammation compared to
omega-6 formulas. Products like Omegaven produce less proinflammatory metabolites, which
act as substrates for the production of arachidonic acid and prostaglandins (24). As these
methods are not well researched, the best technique to prevent PNALD is to use enteral nutrition
support whenever possible (8).
Psychosocial Issues HPN is associated with psychological concerns, especially in children, due to the major
disruption in everyday living. HPN is time-consuming, requires many physical demands, and is
associated with frequent hospitalizations, which can greatly decrease quality of life. Depression
is a major risk in patients of all ages on HPN (1). Children often report feelings of sadness,
loneliness, and social isolation because of their disease and need for HPN. They can also
experience feelings of loneliness and difference from missing the normal meal experience. If
HPN was initiated early in life, they may never experience the taste and texture of food (19).
HPN can be very difficult on families due to the large time commitment and financial cost.
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Parents should work with a social worker, psychologist, or support group to meet their
psychological needs and to best help their child (6).
Patients are required to complete psychological screening before initiation of HPN
because of the extreme psychological burden. HPN in adults has been correlated with increased
substance abuse and social issues. This can lead to poor compliance once at home, increased
complications, and increased mortality. Psychologist or psychiatrist intervention should be
considered in the treatment plan if issues are identified before initiation of HPN (1).
Purpose of Patient Registries
Patient registries are defined by the Agency for Healthcare Research and Quality as (20):
“An organized system that uses observational study methods to collect uniform
data (clinical and other) to evaluate specified outcomes for a population defined
by a particular disease, condition, or exposure and that serves one or more
predetermined scientific, clinical, or policy purpose,”
Data can be collected retrospectively or prospectively to evaluate the epidemiology or treatment
of a disease or outcome of a procedure. They are extremely useful for conducting ongoing
research over multiple states or regions and providing information on the outcomes or
management techniques used by other institutions (21).
The concept of databases has been around for centuries; however the use of computers
has rapidly expanded their use. The Cook County Hospital and the Mayo Clinic created the first
reported computerized registries in the 1970s (19). Many institutions have established their own
registries as a method to keep track of their specific patient groups and outcomes. This is also an
excellent technique for monitoring quality improvement efforts (20). Registries may have
different formats and criteria for patients, but all are required to operate under the Health
Insurance Portability and Accountability Act’s policies and security rules in the United States to
protect the privacy of the patients registered (19).
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The first registry specifically focusing on patients on HPN was created in 1976 by the
American College of Surgeons and the National Institutes of Health. The registry had limited
amounts of data due to aggregate data and exclusion of some patients who were actually on HPN,
as the participation in the registry was voluntary. This registry merged with another HPN registry
monitoring patients in the United States and Canada in 1984 to form the North American Home
Parenteral and Enteral Patient Registry. This registry collected data until 1992 and was
discontinued due to problems obtaining uniform data due to a wide variety of electronic
collection systems. Several other retrospective HPN registries have been reported since;
however, they have small populations and limited information on outcomes (20).
The SUSTAIN™ Database by ASPEN
As the United States is a major user of HPN, the need for a current, well-managed HPN
database in has been identified. Many other countries, such as Spain and New Zealand, have
advanced HPN registries. Although the United States has higher usage rates of HPN, no
systematic approach for collecting data on usage and outcomes exists. This was an indicator to
ASPEN to create a database to collect information on the demographics, practice, and
improvements of patients on HPN (20).
SUSTAIN™, a web-based database, was created by ASPEN in 2011 to collect data on
patients and specific populations requiring HPN in the United States. The database is meant to
monitor outcomes, allows benchmarking against other institutions, and publishes significant
findings to share improvements in patient care. The database will be available to all health
systems and institutions that provide HPN to include patients. The first step of the SUSTAIN™
creation process will be to collect data on patients receiving HPN in the United States.
Researchers will subsequently analyze the data on these patients and their outcomes and publish
the findings to improve the quality of care for these patients. Various data points (Table 1) will
be collected at baseline and during follow up (20).
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Data collection elements were validated using a tool developed by the Home Parenteral
Nutrition Database Taskforce. The taskforce identified data collection domains and compiled
them into a survey. They were sent to trial hospital-based and home-based clinicians. Using
feedback from those clinicians, items were added and deleted from the data collection forms in
hopes to capture as many patients on HPN as possible.
All costs of the registry will be absorbed by SUSTAIN™. The participating institutions
will not have to pay to be a part of or use the registry, unlike many others. ASPEN hopes to
capture 100% of patients on HPN to generate periodic reports with the good of improving patient
(20).
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PROJECT PURPOSE The purpose of this project is to evaluate pediatric HPN patients seen at The University
of Virginia Health System, in order to include relevant information in the SUSTAIN™ database.
The children in the study will be assessed based on anthropometrics, nutritional status, and
incidence of complications, using data collection tools provided by SUSTAIN™. This project
will specifically examine and analyze the patients’ growth, caloric intake, and rate of
complications from HPN. Another dietitian will ultimately enter the data into the SUSTAIN™
database, where it can be shared with other institutions around the country.
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PROJECT METHODS This study will examine six pediatric patients, ages 10 months to 4 years, who receive
some amount of HPN. Of the six patients, one child is completely dependent on HPN. Two of
the children also receive gastric tube feeds and five can also take in food orally. The patients in
the study have a variety of diagnoses, including: SBS, non-short gut related malabsorption, and
gastromotility/pseudo-obstruction disorders (Figure 1). All of the patients are on cyclic HPN,
ranging from 8 hours to 20 hours. The patients also vary on their total duration of HPN during
the week. One patient receives HPN only three nights per week, whereas the others receive HPN
daily. While one patient will be on short term HPN, the duration of HPN for the remaining
patients will vary.
The six patients in this study are followed by the pediatric GI team, which includes a
Registered Dietitian, at the University of Virginia Health System (UVAHS). Baseline (Appendix
A) and follow up (Appendix B) data will be collected December 2012 through March 2013
(Table 2) using the data collection tools provided by SUSTAIN™. Baseline data will be defined
as the patient’s current status on HPN at the time of initiation of data collection. Subsequent
follow up data collection will be completed every time the patient is seen in the GI clinic or is
readmitted to the hospital. The de-identified data will then be shared with SUSTAIN™ database
as part of a separate Nutrition Services-sponsored, Institutional Review Board approved protocol
that also includes adult HPN patients.
This study will use the data to assess several major outcomes of the patients on HPN
including patient demographics, height and weight percentiles for age over time, percent of
calories per day delivered through enteral and/or parenteral nutrition, rates of infections, and rates
of hospitalizations. The goal of the study is to evaluate the effectiveness of HPN, as a therapy for
children who are unable to meet all of their nutritional needs orally or enterally. Secondly, the
study will evaluate any trends in areas that could require further improvements in HPN
management.
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PROJECT DEVELOPMENT OVERVIEW
Six patients were followed during the study time period. The patient demographic
characteristics at the time of study are summarized in Table 3. The patient’s goals while
completing HPN therapy are shown in Figure 2. Baseline data collection was completed at the
first patient encounter after December 1, 2012. Follow up data was collected when the patients
were seen in GI clinic or admitted to the hospital through March 15, 2013. All patients received
HPN while followed. The percentage of calories from HPN, enteral nutrition, and/or oral intake
is outlined in Figure 3.
Anthropometrics
Five of the six patients were able to gain weight while on HPN during the study time
period. One patient only had one weight recorded. By the end of the study period, three patients
were able to maintain their weight or continue to gain weight (Figure 6). Five patients increased
in height during the study time period. One patient only had one height recorded. Four patients
maintained or continued to grow in height by the end of the study period (Figure 7). Four of the
six patients saw an increase in weight for height percentile. One patient only had one weight
recorded. By the end of the study period, two of the six patients were able to maintain the
increase in weight for height percentile (Figure 8). Two of the six patients saw an increase in
their BMI percentile during the study period. Three patients only had one BMI percentile
recorded. By the end of the study, only one patient was able to maintain an increase in BMI
percentile (Figure 9).
Lab Values Liver function lab values were trended over the study period. Two patients experienced
an upward trend in direct bilirubin, two patients experienced a downward trend, and two had only
one lab value measured (Figure 10). Of the six patients, one saw an upward trend in AST, three
patients saw a downward trend in AST, and two patients did not have a lab value measured
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(Figure 11). In terms of ALT, four patients saw a downward trend in ALT, one patient had only
one value measured, and one patient did not have ALT measured (Figure 12).
Hospitalizations
Number of hospitalizations per patient ranged from four to zero admissions during the
study time period. Clinic encounters ranged from three to one appointment per patient. Patient
encounters during the study time period are summarized in Figure 4. The main reasons for
hospitalization among the population were catheter damage, CLABSI, or for another reason
including intractable diarrhea and vomiting and urinary tract infection (UTI). Reasons for
hospitalizations during the study time period are summarized in Figure 5.
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Case Descriptions Patient 1 is a 2 year-old male with a complex medical history including a chromosomal
abnormality, static encephalopathy, and SBS secondary to mid gut volvulus. He has an estimated
20-40 cm of small bowel remaining and a high-grade obstruction at the duodenal colonic
anastomosis, resulting in high gastrostomy tube output. The patient has a previous history of
PNALD, now improved with initiation of fish oil lipid formulation, Omegaven®.
During the data collect time period, Patient 1 was admitted to the general medicine unit at
UVAHS on three occasions for central line breakages. On the last admission his Hickman
tunneled catheter was replaced with a Port-A-Cath®. Admissions were complicated by bacterial
overgrowth from his G-tube. Metabolic issues during admissions included hypoglycemia,
hypokalemia, and fluid and electrolytes imbalance. Patient 1 is visually impaired, but has no
psychological issues, is independently mobile, and completes appropriate ADL for age. A parent
or a hired skilled nurse completes HPN care and administration.
Patient 2 is a 2 year-old-male diagnosed with SBS with approximately 75 cm of small
bowel remaining secondary to gastroschisis and associated ischemic bowel. The patient is also
noted for sensory modulation dysfunction and developmental delay resulting in visual and
dexterity impairments.
Patient 2 had multiple admissions during the study time period for CLABSI, as well as
non-catheter related sepsis, related to bacterial overgrowth. The patient remains on antibiotics to
manage this issue. Metabolic issues during admission included fluid and electrolyte imbalance,
anemia, and hypoglycemia. Despite developmental concerns, Patient 2 has no psychological
issues, is independently mobile, and completes appropriate ADL for age. A parent or grandparent
completes HPN care and administration.
Patient 3 is a 2 year-old female born with duodenal and jejunal atresia and malrotation.
After undergoing an exploratory laparotomy, she was found to have severe midgut necrosis and
SBS with an estimated 10-20 cm of small bowel remaining. Since this procedure, her main source
22
of nutrition has been from PN, only taking food and beverages for comfort. She does not suffer
from any physical or developmental impairment.
While more surgery is planned, she underwent an end-to-end anastomosis of 3 cm of
duodenum/jejunum to 7 cm of ileum during the study period. The patient post-operatively
experienced a urinary tract infection (UTI). Prior to surgery, the patient was noted to have
angular chelosis and other signs of vitamin and mineral deficiencies due to national shortage of
mineral component for HPN. Other metabolic issues experienced include hyperglycemia, fluid
and electrolytes disturbances, and anemia related to iron deficiency. Patient 3 has no
psychological issues, is independently mobile, and completes appropriate ADL for age. A parent
completes HPN care and administration.
Patient 4 is a 2 year-old male diagnosed with cystic fibrosis suffering from intractable
vomiting and diarrhea. After hospitalization, he was discharged on HPN to complete two to three
weeks of supplemental parenteral nutrition in addition to ad lib oral intake to maximize
nutritional restitution. After two weeks on HPN, the patient was able to substantially increase his
weight and HPN was discontinued. No metabolic issues were reported. The patient was not
noted to have any physical or mental impairment and no psychological issues.
Patient 5 is a 3 year-old male with a complex past medical history including
mitochondrial myopathy and neurogenic bladder resulting in gastrointestinal dismotility and
chronic constipation. The patient’s HPN schedule consists of two consecutive days of HPN, with
one-day break. He is allowed to consume food and beverage ad lib. The patient’s course during
the study time period consisted of one admission for a UTI. Fluid and electrolyte imbalance was
the only metabolic issue experienced. The patient was not noted to have any physical or mental
impairment and no psychological issues. He is independently mobile and completes appropriate
ADL for age. A parent completes HPN care and administration.
Patient 6 is a 3 year-old male who received the majority of his care from an outside
institution and recently has had care transferred to UVAHS. He was born with gastroschisis and
23
a midgut volvus resulting in profound SBS, with an estimated 30 cm of small bowel remaining.
During infancy, he developed severe PNALD and was treated with Omegaven®, which resolved
the issue. His history also includes several bowel-lengthening procedures. Ultimately, he will
remain on HPN for weight gain with the hope of further surgery to establish GI anatomy. This
patient entered the study later; therefore his course during the study time period has been
unremarkable. He does not suffer from any physical or developmental impairment and no
psychological issues. A parent completes HPN care and administration. He is independently
mobile and completes appropriate ADL for age.
24
DISCUSSION
To evaluate improvements in nutritional status, the study observed trends in the change of
percentile for weight for age, height for age, height for weight, and BMI for age. We correlated
an improvement in nutritional status to an increase in these percentiles, as an increase would
indicate the child is receiving enough energy to promote growth. We observed that generally all
patients saw an increase in weight for height percentile and most were able to maintain their
weight or continue to grow. Although weight loss was observed in two patients, the losses were
minimal and can most likely be attributed to increased energy needs from growth or an illness
requiring higher calorie and protein needs.
Similar trends were seen in terms of height for age and height for weight percentile.
Generally all patients had an increase in height for age percentile, and most were able to maintain
their height or continue to grow. Decreases in height for age percentile were most likely due to
discrepancies in height measuring equipment, as it is unlikely that the patients actually decreased
in height over time. Change in height for weight percentile was also followed a somewhat similar
pattern. Most patients saw an increase in height for weight percentile, although less than half
were able to maintain this increase. Again, this may have been due to measurement errors or
periodic changes in energy needs, which did not support proportional weight gain.
Trends in BMI percentile for age increased in most of the patients over time, however
only two patients were able to maintain the increase. This may have been due to similar factors
like measurement error or increased energy needs. Other factors may have included slightly
disproportional weight gain for age and height or changes in fat and muscle distribution.
Overall, the changes in anthropometrics are what we hoped to seen in patients on HPN.
Most of the patients saw an increase in height and weight, showing an overall improvement in
nutritional status, which was the purpose of the initiation HPN therapy. This is concurrent with
research suggesting that HPN can be an excellent therapy, by itself or in combination with other
25
forms of nutrition, for improving nutritional status in patients who cannot otherwise meet their
nutritional needs.
The study observed varied lab results. Overall, most patients had normal values for their
liver function tests. Total bilirubin was generally within the normal limits of 0.3 to 1.9 mg/dL in
all patients with this lab measured. However all were observed to have increasing levels of total
bilirubin by the end of the study period. Increasing bilirubin, beyond normal levels, is indicative
of liver damage. This is expected in HPN patients, as HPN is known to cause damage to the liver
due to content of dextrose and fat in the HPN formula.
Similarly, AST and ALT are indicators of liver function. All patients, except one, were
observed to have AST levels within the normal limits of 8 to 48 u/L. Likewise, the patients’ ALT
levels were all within the normal range of 7 to 55 u/L, excluding one patient. This indicates, with
the exception of one patient, all patients had generally good liver function while on HPN.
However, it should be considered that the study duration is short and the patients have been on
HPN for varied amounts of time. It is important to measure liver function tests in these patients
often and make adjustments in dextrose and fat concentration or duration of HPN as necessary.
The patient who did have abnormally high levels of AST and ALT has already been
diagnosed with PNALD and is undergoing treatment with Omegaven®. This omega-3 based fat
emulsion is still not widely offered in the United States. However, it has shown to be beneficial
in this patient, who has been on TPN/HPN for over two years. Overall, his PNALD has
improved, which can be seen by the general downward trend in his liver function lab values.
All of the study patients, except one, were hospitalized at least once at some point during
the study period. This is a very high rate of complications requiring hospital admission. Most
hospitalizations were related to catheter damage, CLABSI, or another issue including UTI,
diarrhea, and vomiting. The patients with CLABSI and catheter damage were hospitalized the
most frequently and required more follow up appointments in clinic following discharge.
26
CLABSI is not unexpected, as it is the most common HPN complication in children with
short bowel. It is also exacerbated with malnutrition, as the body is highly susceptible to
hyperglycemia. The patients who experienced CLABSI were all undernourished at some point in
the study. This is concerning in our population as their high energy needs are already
exacerbated due to malnutrition. It is also concerning as this is a potentially preventable
complication. Using aseptic technique at all times can prevent both catheter occlusions and
subsequent infections.
Catheter damage was also common in population. This is also a common complication
with HPN. These results are concerning in our population as too many catheter replacements can
cause loss of access for HPN altogether. One of our patients who experienced chronic catheter
damage was able to have a Port-A-Cath® placed.
In general, hospitalizations are problematic in this population because they are very
susceptible to infections. Being in a highly infectious environment puts them at risk for other
complications. Hospitalization also requires HPN to be discontinued, which can leave the child
without nutrition for significant portions of time. Lastly, admissions also add to the high cost of
HPN, creating stress for the child’s caretaker.
In summary, nutrition status was shown to improve in the majority of patients in terms of
height and weight percentile, however, as complications were common, HPN is not the best long-
term source of nutrition to maintain growth and development. It is our hope that most of the
patients will be on HPN for a relatively short period of time to boost their nutritional status and
promote growth until they are strong enough to undergo surgery to reestablish the use of the
intestinal tract. Until then, we must monitor them carefully to prevent further complications.
Further research is necessary to find the best way to administer HPN to decrease possible
problems. While preliminary research has been done regarding omega-3 fatty acids lipid
solutions, this is something that needs further development before it can be recommended to all
patients. More research is also necessary to determine the best way to administer HPN to prevent
27
infections. Ultimately, a safe solution to provide nutrition to children who cannot use their GI
tract needs to be found. HPN is a good short-term solution however it is not indicated for long-
term use.
28
CONCLUSION
This study was able to provide useful information to the SUSTAIN™ on six pediatric
patients on HPN. The data will be used to study tendencies and outcomes of pediatric patients on
this nutrition therapy. Our hospital examined trends in our patients and observed an overall
improvement in nutritional status while on HPN by examined weight for age and height for age
percentiles. However, we also observed many complications, particularly catheter damage and
CLABSI, indicating HPN is not an ideal therapy for long-term nutrition support. Further cases
should be examined over a longer period of time to determine best practices for HPN use.
29
REFERENCES
1. Kumpf VJ, Tillman EM. Home Parenteral Nutrition: Safe Transition from Hospital to Home. Nutrition in Clinical Practice. 2012; 27(6): 749-57. 2. Gargasz A. Neonatal and Pediatric Parenteral Nutrition. American Association of Critical-Care Nurses. 2012; 23(4): 451-64. 3. Colomb V, Dabbas-Tyan M, Taupin P, Talbotec C, Jan, YRD, De Potter S, Gorski-Colins AM, Lamor M, Herreman K, Corriol O, Landais P, Ricour C, Goulet O. Long-term outcome of children receiving home parenteral nutrition: a 20-year single-center experience in 302 patients. Journal of Pediatric Gastroenterology and Nutrition. 2006; 44(6): 347-53. 4. Dudrick SJ. A 45-Year Obsession and Passionate Pursuit of Optimal Nutrition Support: Puppies, Pediatrics, Surgery, Geriatrics, Home TPN, A.S.P.E.N., Et Cetera. Journal of Parenteral and Enteral Nutrition. 2005; 29(4): 272-87. 5. Dudrick SJ. History of Parenteral Nutrition. Journal of the American College of Nutrition. 2009; 28(3): 243-51. 6. Puntis JWL. Home Parenteral Nutrition. Archive of Disease in Childhood. 1995; 72(2): 186-90. 7. Seidner D, Hamilton C, Stafford J, Steiger E. Examination of Factors That Lead to Complications for New Home Parenteral Nutrition Patients. Journal of Infusion Nursing. 2006; 29(2): 74-80. 8. Tillman ET. Review and Clinical Update on Parenteral Nutrition-Associated Liver Disease. Nutrition in Clinical Practice. 2012:1-10. 9. Chermesh I, Mashiach T, Amit A, Hiam N, Papier I, Efergan R, Lachter J, Eliakim R. Home parenteral nutrition (HTPN) for incurable patients with cancer with gastrointestinal obstruction: do the benefits outweigh the risks? Medical Oncology. 2011; 28 (1): 83-88. 10. Agostino P, Morassutti I, Rosti G. Nutritional intervention for improving treatment tolerance in cancer patients. Current Opinion in Oncology. 2001; 23: 322-30. 11. Hojsak I, Strizic H, Misak Z, Rimac I, Bukovina G, Prlic H, Kolacek S. Central venous catheter related sepsis in children on parenteral nutrition: A 21-year single-center experience. Clinical Nutrition. 2012; 31(5): 672-75. 12. Norman JL, Crill CM. Optimizing the transition to home parenteral nutrition in pediatric patients. Nutrition in Clinical Practice. 2011; 26(3): 273-85. 13. Smith CE, Curtas S, Kleinbeck SVM; Werkowitch M, Mosier M, Seidner DL, Steiger E. Clinical Trial of Interactive and Videotaped Educational Interventions Reduce Infection, Reactive Depression, and Rehospitalizations for Sepsis in Patients on Home Parenteral Nutrition. Journal of Parenteral and Enteral Nutrition. 2003; 27(2): 137-47. 14. Opilla MT. Epidemiology of bloodstream infection associated with parenteral nutrition. American Journal of Infection Control. 2008; 36(2): 3-5.
30
15. Santarpia L, Alfonsi L, Tiseo D, Creti R, Baldassarri L, Pasanisi F, Contaldo F. Central Venous Catheter Infections and Antibiotic Therapy During Long-Term Home Parenteral Nutrition: An 11-Year Follow-Up Study. Journal of Parenteral and Enteral Nutrition. 2011; 35(5): 581-87. 16. Opilla MT, Kirby, Donald F, Edmond MB. Use of Ethanol Lock Therapy to Reduce the Incidence of Catheter Related Bloodstream Infections in Home Parenteral Nutrition Patients. Journal of Parenteral and Enteral Nutrition. 2007; 31(4): 302-305. 17. Moreno JM; Valero MA; Gomis P, Leon-Sanz M. Central venous catheter occlusion in home parenteral nutrition patients. Clinical Nutrition. 1998; 17(1): 35-36. 18. van Ommen C, Heleen, Tabbers, Merit M. Catheter-related thrombosis in children with intestinal failure and long-term parenteral nutrition: How to treat and to prevent? Thrombosis Research. 2010; 126(6): 465-70. 19. Johnson T, Sexton E. Managing children and adolescents on parenteral nutrition: challenges for the nutritional support team. Proceedings of the Nutrition Society. 2006: 217-21. 20. Guenter P, Robinson L, DiMaria-Ghalili RA, Lyman B, Steiger E, Winkler MF. Development of Sustain™: A.S.P.E.N.'s National Patient Registry for Nutrition Care. Journal of Parenteral and Enteral Nutrition. 2012; 36(2): 399-406. 21. Drolet BC, Johnson KB. Categorizing the world of registries. Journal of Biomedical Informatics. 2008; 41(6): 1009-20. 22. Dudrick SJ. A 45-Year Obsession and Passionate Pursuit of Optimal Nutrition Support: Puppies, Pediatrics, Surgery, Geriatrics, Home TPN, A.S.P.E.N., Et Cetera. Journal of Parenteral and Enteral Nutrition. 2005; 29(4): 272-87. 23. Johnson T, Sexton E. Managing children and adolescents on parenteral nutrition: challenges from the nutritional support team. Proceedings of the Nutrition Society. 2006; 65: 217-21. 24. Burns DL, McGill BM. Reversal of Parenteral Nutrition−Associated Liver Disease With a Fish Oil−Based Lipid Emulsion (Omegaven) in an Adult Dependent on Home Parenteral Nutrition. Journal of Parenteral and Enteral Nutrition. 2012; 10(20): 1-7.
31
APPENDIX Table 1. Data Elements Collected at Baseline and Follow-Up
Baseline Follow-Up
• Demographics • Diagnoses and reason for parenteral
nutrition (PN) • Current PN formula • Nutrition status • Psychosocial status • Functional status • Patient teaching
• Demographics • Diagnoses and ongoing reason for
continuing PN • Current PN formula • Nutrition status • Morbidity data (including
complications) • Rehospitalization data • Mortality data • Community resources
32
Table 2. Major Project Timeline October Begin research for Lit Review November 12 Draft of Lit Review, Purpose, Methods, and References due to
Brandis December 3 Proposal Due
Items Due: Literature Review
Purpose Methods References
December 1 Start data collection for Initial Visits & Follow Ups (as available) January (when Proposal returned)
Finalize Lit Review and Methods Sections and continue data collection. Begin Project Presentation.
January 28 Final Lit Review/Methods and drafted Project Presentation to Brandis
February 11 Interim Report Due Items Due: Final and revised version of Lit Review Final and revised version of the project methods Interim progress report
February (when Interim report returned)
Continue Project Presentation and finish data collection
February 15 Abstract due to VDA March 25 Draft of final paper (or as much as completed depending on
patients) due to Brandis March-April Continue following patients, finalizing paper, and preparing for
VDA (if accepted) April 5 Final Paper to Brandis April 29 Final Project Due
Items Due: Title Abstract Lit Review Methods Project Presentation References
May VDA Conference in Richmond
33
Table 3. Patient Demographics
Patient 1 Patient 2 Patient 3 Patient 4 Patient 5 Patient 6
Age (years)
2 2.8 2.5 2 3 3.8
Gender Male Male Female Male Male Male Ethnic
Category Hispanic/
Latino Non-
Hispanic/ Latino
Non-Hispanic/
Latino
Non-Hispanic/
Latino
Non-Hispanic/
Latino
Non-Hispanic/ Latino
Race White White African American
White White White
Insurance Type
Medicaid Medicaid Medicaid Private Insurance
Medicaid Private Insurance
Primary Caregiver
Parent/ Visiting Nurse
Parent/ Grandparents
Parent Parent Parent Parent
34
Figure 1: Underlying Diagnoses
Short Bowel Syndrome
Non-Short Gut Related Malabsorption
Gastromotility/Pseudo-obstruction disorder
0
1
2
3
4
5
6
7
Weight Gain Resolution of Issue and Discontinuation of HPN
Future surgery and re-establishment of GI
anatomy
Num
ber
of P
artic
ipan
ts
Figure 2: Goals of HomeParenteral Nutrition Therapy
35
0
10
20
30
40
50
60
70
80
90
100
Patient 1 Patient 2 Patient 3 Patient 4 Patient 5 Patient 6
Perc
enta
ge o
f Tot
al C
alor
ies
Figure 3: Percent Calories from Parenteral Nutrition, Enteral Nutrition, and Oral Intake
Parenteral Nutrition Enteral Nutrition Oral Intake
0
1
2
3
4
5
Patient 1 Patient 2 Patient 3 Patient 4 Patient 5 Patient 6
Num
ber
of E
ncou
nter
s
Figure 4: Frequency and Type of Encounter during Data Collection Period
Hospitalization Clinic Appointment
36
0
0.5
1
1.5
2
2.5
3
3.5
Catheter Damage CLABSI Sepsis not Related to Catheter
Surgery Other
Fequ
ency
of A
dmis
sion
Figure 5: Reasons for Hospitalization
0
2
4
6
8
10
12
14
16
1 2 3 4 5 6
Weight (Kilogram
)
Encounter
Figure 6: Patient Weight Over Time
Patient 1
Patient 2
Patient 3
Patient 4
Patient 5
Patient 6
37
70
75
80
85
90
95
100
1 2 3 4 5 6
Height (cm
)
Encounter
Figure 7: Patient Height Over Time
Patient 1
Patient 2
Patient 3
Patient 4
Patient 5
Patient 6
0
20
40
60
80
100
1 2 3 4 5
Wei
ght f
or L
engt
h Pe
rcen
tile
Encounter
Figure 8: Change in Weight for Length Percentile Over Time
Patient 1
Patient 2
Patient 3
Patient 4
Patient 5
Patient 6
38
0 10 20 30 40 50 60 70 80 90
100
1 2 3
BM
I Per
cent
ile
Encounter
Figure 9: Change in BMI Percentile Over Time
Patient 1
Patient 2
Patient 3
Patient 4
Patient 5
Patient 6
0
0.2
0.4
0.6
0.8
1
1.2
1 2 3 4 5 6 7
Dir
ect B
iliru
bin
(mg/
dL)
Encounter
Figure 10: Trends in Direct Bilirubin over Time
Patient 1
Patient 2
Patient 3
Patient 4
Patient 5
Patient 6
39
0
10
20
30
40
50
60
70
80
90
1 2 3 4 5 6 7
AST
(U/L
)
Encounter
Figure 11: Trends in AST over Time
Patient 1
Patient 2
Patient 3
Patient 4
Patient 5
Patient 6
0
20
40
60
80
100
120
140
160
1 2 3 4 5 6 7
ALT
(U/L
)
Encounter
Figure 12: Trends in ALT over Time
Patient 1
Patient 2
Patient 3
Patient 4
Patient 5
Patient 6
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