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Home Parenteral Nutrition (HPN) Awareness Week

October 11-15, 2021

Overview

Home Parenteral Nutrition (HPN) Awareness Week is a week spearheaded by The Oley Foundation. It is held to help bring light to what it is like living with long-term PN, the reasons why people require it, and its benefits and the risks. Jump to nutrition FAQ here.

What is home parenteral nutrition (HPN)?

Home parenteral nutrition (HPN) is a liquid that provides nutrition directly into the body's large veins. It provides you (or your child) with all of the nutrients, fluids, vitamins, and minerals needed to fuel the body. Other names include total parenteral nutrition (TPN) and parenteral nutrition (PN). 

Watch this kid-friendly video on the basics of parenteral nutrition (PN).

Learn more about parenteral nutrition (PN) with these Transplant Unwrapped downloads. 

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Overview of Parenteral Nutrition (PN) for Adults

Part Two An Overview of PN.png

Handbook on Parenteral Nutrition (PN)

Updated October 2021 A Parent's Guide to CVC .png

Overview of Parenteral Nutrition (PN) for Parents

Learn more about parenteral nutrition (PN) with these patient stories. 

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Darryl

Living with Parenteral Nutrition (PN)

00:00 / 06:18
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Jamie

TPN After Intestinal Transplant

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Swapna

Living with TPN and Short Bowel Syndrome (SBS)

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Nancy

Living with TPN and Short Bowel Syndrome (SBS)

Total Parenteral Nutrition (TPN) Q & A with Dr. Segovia

Questions she answers includes:

  • What is TPN?

  • Why does a child or adult require TPN?

  • What is a central line?

  • What are complications associated with a central line?

  • What are complications associated with long-term TPN use?

  • Is an individual still able to eat by mouth while on TPN?

  • What is TPN failure and how does this qualify a person for intestinal transplant?

  • What are the benefits of tube feeds vs. TPN?

To stay up-to-date for the next part in this interview series, you can register here and become a Transplant Unwrapped Website member.

Section 1: Parenteral Nutrition (PN)

What is Parenteral Nutrition (PN)?


Parenteral nutrition (PN) is a liquid that provides nutrition directly into the body's large veins. It provides you (or your child) with all of the nutrients, fluids, vitamins, and minerals needed to fuel the body. Since PN is infused straight into the bloodstream, it bypasses the intestine. This is beneficial in short bowel patients and other gastrointestinal disorders where the intestine does not absorb efficiently. Other common terms for parenteral nutrition include: hyperalimentation (very old term), total parenteral nutrition (TPN), or home parenteral nutrition (HPN).




What are the components of PN?


PN supplies the primary nutrients your body needs to sustain life, including the three primary sources of energy for your body: 1. Carbohydrates: This gives your body calories and is the primary energy component in PN. It is usually in the PN as glucose. 2. Protein: Helps your body repair tissue, build muscle, fight infections, and carry nutrients through the body. 3. Fat: Another good source of calories and energy. It also is needed to absorb vitamins A, D, E, K, helps to protect your vital organs, and conserve heat. In addition to the three main components, PN also contains micronutrients your body needs to function: 1. Vitamins: Usually, you are required to add these to your PN solution when you are on home PN. These are what turn the PN solution yellow. Examples: vitamins A, D, E, and K. 2. Minerals: These may also be added to PN. Examples are zinc and copper. 3. Electrolytes: These are important for the function of most systems of the body. Examples: calcium, potassium, phosphorous, magnesium, and sodium. 4. Water: Water is important to keep you hydrated and your kidneys functioning. Medications: Sometimes you may add mediations to your PN solution. Commonly added medications include:

  • Insulin: This helps to control your blood sugar.
  • Anti-acid: This helps with stomach acid secretion.
  • Octreotide: This is an anti-secretory medication and can help decrease diarrhea.




What is important to monitor while on PN?


This will vary per center, however, it is usually important to record:

  • Weight
  • Fluid intake and output
  • Temperature
  • Urine-dipstick-for-sugar results or blood sugar monitor
During the first few weeks or months, you usually will need weekly bloodwork. If you have been on PN for a long time, the frequency of blood monitoring may decrease.




Why does someone require PN?


Parenteral nutrition is given to people with certain gastrointestinal disorders, such as short bowel syndrome, who are unable to absorb enough nutrients. PN is delivered directly into the bloodstream (bypassing the gastrointestinal tract) through a central venous catheter (CVC). Common reasons people require PN (this list is not all-inclusive, rather, a snapshot):

  • Crohn's and Ulcerative Colitis
  • Motility diosrders, such as chronic intestinal pseudo-obstruction (CIPO)
  • Cancer, particularly that of the GI tract
  • Short bowel syndrome from congenital conditions
  • Stroke
  • Cystic fibrosis
  • ALS
  • Parkinson's Disease
  • Ischemic bowel disease
  • Necrotizing Enterocolitis (NEC)




How is PN started and cycled?


Every individual is different, but generally, PN is started as an inpatient in the hospital. The reason for this is that your team needs to closely monitor you (or your child) very closely at the beginning to ensure that your electrolytes and other blood levels stay in balance. Since many people starting PN are malnourished, they also are at risk of something called refeeding syndrome (see PN complications). At first, most individuals will start PN on a 24-hour continuous cycle. This means the PN runs throughout the entire day.

  • Initially, your PN will not contain all of the calories and nutrients your body needs.
  • Gradually over a few days to weeks your team will increase the glucose (energy/calories) in the PN until the full nutritional requirements are met.
  • Once met, your team will likely start to 'cycle' your PN.
    • Note: Not all people will be able to cycle PN and may have to remain on a continuous infusion. Your team will determine the best option for you.
  • Cycling your PN means that they will decrease the amount of time that your PN runs over.
  • Typically it will go down in increments, such as from 24 hours to 18 hours to 16 hours and finally ending at a 12-hour cycle.
  • Most people like to do their 12-hour cycle overnight; therefore, they can be free from PN throughout the day.




What are the types of lipid formulations for PN?


1. Intralipid: This is a soybean oil lipid emulsion (SOLE). SOLEs contain high amounts of a plant-based cholesterol-like compound called phytosterols, found in vegetable oils. These have been found to contribute to the development of cholestasis and disruption of bile acid homeostasis. 2. Mixed-lipid emulsions: These lipid emulsions contain various amounts of plant-based oils, fish oils, and medium-chain triglycerides (MCTs). The most commonly known is SMOFlipid® which consists of: 30% soybean oil, 30% MCTs, 25% olive oil, 15% fish oil. SMOFlipid® is currently indicated for use in adults "as a source of calories and essential fatty acids for PN when oral or enteral nutrition is not possible, insufficient, or contraindicated." 3. Omegaven®: This is a fish-oil lipid emulsion (FOLE). It contains minimal phytosterols and is rich in omega-3 fatty acids. It is currently FDA approved for children with parenteral-nutrition associated cholestasis. Research is continuously ongoing on the best lipid formulation to help prevent parenteral-nutrition associated liver disease (PNALD). To stay up-to-date on nutrition news, visit the Oley website.




How is parenteral nutrition (PN) administered?


A catheter (small tube) is placed in one of your main blood vessels. Common areas include the neck and chest. If there are access issues, the catheter may be placed in the leg or groin The catheter is known as a central venous catheter (CVC) or central line, and delivers the PN solution directly into your bloodstream. Go to section two to read more about central venous catheters (CVCs). If you would like an overview of CVCs you can download this handout if you are a caregiver of a child or this hadnout for adults: Patient's Guide to CVCs




What is the difference between parenteral nutrition (PN) and enteral nutrition (tube feeds)?


Enteral nutrition, also known as tube feeding, is used when a person's digestive system works to some extent. The feeding tube is given directly into part of the digestive system. Parenteral nutrition (PN) bypasses the digestive system entirely and goes directly into the bloodstream, where the nutrients are absorbed. Individuals may be just on PN or enteral nutrition, or in some cases, an individual may be on both parenteral nutrition (PN) and enteral nutrition. To learn more about enteral nutrition visit this page.





Section 3: PN Complications

What are complications associated with PN?


PN complications can be broken up into 3 categories: 1. Metabolic complications. 2. Catheter-related (mechanical) complications. 3. Infection-related complications (sepsis). The complications can also be considered short-term or long-term. Common Short-Term Complications:

  • Volume overload
  • Hyperglycemia (high blood sugar) or Hypoglycemia (low blood sugar)
  • Refeeding Syndrome (hypophosphatemia (low phosphorous), hypomagnesemia (low magnesium), hypokalemia (low potassium)
  • Other electrolyte imbalances
Common Long-Term Complications
  • Hepatic cholestasis (impaired bile formation or flow)
  • Hepatic steatosis. (fatty liver)
  • Cirrhosis and End-Stage Liver Disease




Hyperglycemia and Hypoglycemia


PN contains large amounts of glucose. This places individuals on PN at risk of developing hyperglycemia (too much glucose in the blood) or hypoglycemia (too little glucose in the blood). Hyperglycemia (High Blood Sugar)

  • Can result from the high amount of glucose infused over a short period
  • Generally, you will be required to use a urine dipstick test or glucose monitor to assess the levels of glucose (sugar) in your blood to determine if the level is too high.
  • Treatment: Your team may choose to add insulin to your PN or require you to give yourself insulin. Insulin is the hormone in your body that regulates sugar levels, signaling to cells to take in the glucose and store energy for later use.
Hypoglycemia (Low Blood Sugar)
  • This is when your blood sugar is too low.
  • This can occur when your PN is stopped too abruptly.
  • Signs and Symptoms of Hypoglycemia
    • Feeling shaky
    • Sweating, clamminess
    • Confusion
    • Fast heartbeat
    • Feeling lightheaded or dizzy
  • If you are having trouble with hypoglycemia, your team may choose to place your PN regimen on a taper schedule.
    • This means that the infusion starts off slowly over the first hour or two of your infusion, gradually increasing the infusion until it reaches full speed.
    • The hour or two before your PN cycle is going to end, you may also need to taper-down.
    • The tapers make it so your body is not shocked by massive amounts of glucose, which could cause the release of too much insulin, resulting in low blood sugar (hypoglycemia).




Refeeding Syndrome


Refeeding syndrome can occur upon initiation of PN if an individual is severely malnourished. When the body is in the starved state without carbohydrates, it uses protein and fat stores for energy. When PN is introduced and initiates carbohydrate metabolism, insulin production increases. This causes a movement of electrolytes into cells, particularly phosphate, leading to serious electrolyte and fluid disturbances in the body. It is a very serious situation and can be life-threatening if not treated. Fluid can shift into the lungs which causes serious breathing difficulties.

A hallmark of refeeding syndrome is hypophosphatemia (low phosphate levels), but concentrations of many electrolytes, such as potassium and magnesium, can become low.




Parenteral Nutrition Associated Liver Disease (PNALD) in Adults


Parenteral Nutrition Associated Liver Disease (PNALD) refers to liver dysfunction that results from the use of parenteral nutrition (PN) in individuals with a nonfunctioning intestine (intestinal failure). There are three main types of PNALD: 1. Steatosis (fatty liver): This is a benign condition but can progress to fibrosis and cirrhosis. Cirrhosis is scarring of the liver and is generally permanent. The portions of the liver that become cirrhotic are no longer functional. 2. Cholestasis: This is when the flow of bile from your liver is reduced or blocked. 3. Cholelithiasis (gallstones or gallbladder sludge): Commonly seen in short bowel patients on PN due to reduced concentration of bile acids from altered intestinal-liver (enterohepatic) circulation. Also, with limited oral intake, the gallbladder contracts less, leading to more concentrated bile and increased risk for developing stones. Steatosis and cholestasis can progress to end-stage liver disease and cirrhosis (scarring of the liver, no longer functional). Factors affecting PNALD:

  • Long-term PN use, more prolonged use = higher risk
  • Infusion of high amounts of lipids or dextrose = higher risk
  • Type of lipid emulsion, SMOFlipid®, and Omegaven® is shown to have better outcomes
Diagnosis: Diagnosis is based on bloodwork which assesses liver function tests such as alanine transaminase (ALT), aspartate transaminase (AST), alkaline phosphatase (ALP). bilirubin). A liver or gallbladder ultrasound may be used to visualize the liver or look for stones. Either a magnetic resonance elastogram or an ultrasound elastogram may be performed to assess for the presence of fibrosis (scarring). The gold standard is a liver biopsy to assess for fibrosis and cirrhosis. Treatment: The best option is to get you off of PN. If this is not possible, your team may be able to make adjustments to your PN solution, such as decreasing the amount of glucose or lipids. Also, your team may recommend the use of a different lipid formulation (see SMOFlipid® and Omegaven® for more information). If you have progressed to end-stage liver disease, a liver or multivisceral transplant may be recommended.




Parenteral Nutrition Associated Liver Disease (PNALD) in Children


Parenteral Nutrition Associated Liver Disease (PNALD) refers to liver dysfunction that results from the use of parenteral nutrition (PN) in indivduals with a nonfunctioning intestine (intestinal failure). Risk factors for development of PNALD in a child includes:

  • Premature birth
  • Disruption of the intestinal-liver (enterohepatic) circulation of bile acids
  • Slow intestinal motility resulting in bacterial overgrowth
  • High parenteral protein, fat, and/or energy intake
  • Frequent line infections
  • Absence of oral feeds (inc. risk for biliary sludge)
  • Continuous PN infusion can lead to too much insulin in the blood and is accosiated with cholestatic liver disease in children
What are the possible forms of PNALD? 1. Steatosis (more common in adults): his is a benign condition but can progress to fibrosis and cirrhosis. Cirrhosis is scarring of the liver and is generally permanent. The portions of the liver that become cirrhotic are no longer functional. 2. Cholestasis (common in children): This is when the flow of bile from your child's liver is reduced or blocked. 3. Cholelithiasis (gallstones, gallbladder sludge): Commonly seen in short bowel patients on PN due to reduced concentration of bile acids from altered intestinal-liver (enterohepatic) circulation. Also, with limited oral intake, the gallbladder contracts less, leading to more concentrated bile and increased risk for developing stones. Diagnosis: Diagnosis is based on bloodwork which assesses liver function tests such as alanine transaminase (ALT), aspartate transaminase (AST), alkaline phosphatase (ALP). bilirubin). A liver or gallbladder ultrasound may be used to visualize the liver or look for stones. Either a magnetic resonance elastogram or an ultrasound elastogram may be performed to assess for the presence of fibrosis (scarring). The gold standard is a liver biopsy to assess for fibrosis and cirrhosis, but often this is not performed in a young child. Treatment: The best option is to get your child off of PN. If this is not possible, your child's team may be able to make adjustments to their PN solution, such as decreasing the amount of glucose or lipids. Also, your child's team may recommend the use of a different lipid formulation (see SMOFlipid® and Omegaven® for more information). If your child has progressed to end-stage liver disease, a liver or multivisceral transplant may be recommended.




Central Venous Catheter Occlusion or Thrombosis


Central Venous Catheter Thrombosis (CVT) occurs when a blood clot forms in a central line (the line that goes into your (or your child's) vein for infusing PN). You (or your child) can also have an occlusion in the central line from blood, a medication, or PN fluid precipitate (such as a lipid component). Signs and Symptoms:

  • Facial swelling
  • Swelling of the arm on the same side of the body as the catheter
  • Distended neck veins
If you are caring for a child, you should look for:
  • Any distress or breathlessness while infusing
  • Swelling in the neck or limbs
  • Stiffness or difficulty when flushing the line
  • Fluid coming out around the exit site of the line
How to help prevent this from happening?
  • Flush with 0.9% NS after every use of the line
  • If you do not use the line on a regular basis, flush the line at least once per week
  • If you start to feel your line becoming difficult to push, consult your team so they can get you a special solution a nurse can place in your line to help it flow better
Diagnosis: Your team may or may not choose to order a test to assess for a blood clot depending on the situation. If a test is ordered, it may be a venogram, MRI angiography, or Doppler ultrasound. All of these tests are able to view the flow of blood through your blood vessels. Treatment: Treatment will depend on the severity of your clot. In some cases, an anti-thrombolytic agent can be used. These medications are 'clot busters' and are placed directly into your line to break-up the clot. You may also be placed on some form of anticoagulation or blood thinner to help prevent future clots. In some cases, the central line must be removed and replaced.




Infection


Infection is one of the most common complications associated with PN use. Aseptic (sterile) technique is essential when setting up PN and doing anything with your (or your child's) central line. The most common organisms that lead to central line infections include:

  • Gram-positive organisms
  • Coagulase-negative staphylococci
  • Staphylococcus aureus
Signs and Symptoms: (oftentimes correlates with PN infusion)
  • Fever
  • Chills
  • Muscle aches and pains
  • Fatigue and lethargy
Diagnosis: Diagnosis is usually based on blood cultures. Blood cultures can take days to come back, so your (or your child's) team will often initiate a broad-spectrum course of antibiotics if there is clinical suspicion of a bloodstream infection. Treatment: Your team will determine the best antibiotic and antimicrobial treatment for you. Depending on the organism causing the infection often determines if your line will need to be removed and replaced. For example, if you have a fungal infection, gram-negative bacilli, valvular heart disease, multiple organisms, or endocarditis, the line is usually removed. Each case is individualized, and your team will determine the best course for you. Prevention:
  • Wash your hands before and after handling your PN or CVC.
  • Always scrub your catheter hubs for 15 seconds before and after each use.
  • Always cover your central line when taking a shower or getting wet.
  • Never let the tip of your PN tubing touch anything other than the connection with your catheter.
  • Perform cap changes about every 3 days.
  • When your catheter is not in use, be sure it is covered with a cap.
  • Remain completely sterile (completely free from bacteria) while doing dressing changes.





Section 4: Additional Resources

Helpful Website: The Oley Foundation


The Oley Foundation: Visit The Oley Foundation for more information on parenteral and enteral nutrition.




Helpful Website: American Society for Parenteral and Enteral Nutrition (ASPEN)


American Society for Parenteral and Enteral Nutrition (ASPEN): Visit this website for a plethora of information on parenteral and enteral nutrition.




Helpful Guide from Toronto General: Taking Care of PN


Taking Care of Your Home Parenteral Nutrition: A useful guide created by Toronto General.




Page References


References here.





Section 2: Central Lines

What are the types of central venous catheters (CVCs) or central lines?


Peripherally Inserted Central Catheter (PICC):

  • This is a type of catheter (small, flexible plastic tube) inserted into a large vein in your upper arm.
  • The tip of the catheter ends just above the heart.
  • PICCs can be used for many months.
Tunneled Central Venous Catheter (CVC):
  • This type of catheter (small, flexible plastic tube) is inserted into a large vein in your chest, groin, or neck.
  • It usually is tunneled under your skin.
  • Most commonly, for long term PN use, the CVC will be placed in the chest.
  • This type of CVC can be used for years.
  • The following are types of tunneled CVC: Hickman®, and Broviac®.
Implanted Port
  • A particular type of tunneled CVC in which there is no exit point.
  • A round metal disc with a soft rubber top is placed just under the skin, and a catheter is tunneled to reach just above the heart.
  • To access, you must use a special needle poked into the disk under your skin.
Occasionally, a special, less concentrated PN form may be given into a vein through a peripheral line (cannula) in your arm. This is only used for a short period, usually as a bridge until a central line can be inserted. To see visuals of the types of catheters, please view this article.