• Empower you to care for your child at home. We will be there to support you.
• Enable your child to live a full life, despite their medical needs
• Optimize your child's health, development and well-being
• Promote intestinal adaptation, and increase your child's ability to digest and absorb nutrients
• Increase your child's intake of food and promote a healthy relationship with food
• Decrease or eliminate your child's need for PN
• Optimize surgical outcomes

Intestinal failure (IF) happens when the intestine cannot absorb enough nutrients and fluids from a regular diet to support growth and good health. This can happen when the intestine is too short or when it does not work well. Children with IF typically need intravenous nutrition (parenteral nutrition, PN) for some time to ensure appropriate growth and development.

There are several common causes of IF:

• Intestines that did not develop properly (atresia)
• Intestines that developed outside the body (gastroschisis)
• Inflammation and poor blood supply to the intestines (necrotizing enterocolitis)
• Twisting of the intestines (volvulus)
• Neuromuscular problems in the intestines (severe dysmotility)
  

These are most often identified or happen before birth or very early in life, and usually require surgery. IF can also develop in older children, but this is much less common.

As children grow, their intestine also grows. For most children with IF, this helps with the absorption of nutrients and fluids. Some strategies can help the intestine work better over time and minimize intestinal problems, and help decrease the need for PN. This is called intestinal rehabilitation (IR) and is the work of intestinal rehabilitation teams such as CHIRP.

We individualize treatment plans to meet the unique needs of each child, through a combination of medical management and surgery. Treatment plans may include:

• Intravenous nutrition (parenteral nutrition, PN)
• Intravenous fluids
• Tube feeding
• Diet modifications
• Medication
• Surgery
  

The goal of IR is to decrease or eliminate your child's need for PN and to increase their intake of food. This multi-step process happens gradually over time; this timeline is very different for every child.

When IR does not work for a child, intestinal transplant may be considered. When this is the case, we work closely with transplant teams in Canada.

Research shows that the development of IR teams has been the single most important factor associated with improving outcomes for children with IF.

Members of an IR team develop expertise in caring for children with IF and we continue to learn about new ways to best manage problems associated with IF. Because IF is rare, it is important to have a dedicated team who has experience in treating these conditions. Our team cares for all children with IF in BC and the Yukon, therefore gaining maximum experience to benefit the care of every child.

As a specialized team, we also share knowledge and experience with other teams from around the world. We participate in research to improve care and train professionals to meet the needs of children and families in the future.

NeoCHIRP

Babies with or at risk for IF are part of NeoCHIRP, which includes both neonatal (NICU) and CHIRP team members. The NeoCHIRP team meets weekly to discuss recommendations for IR, taking into consideration your baby's other care needs. During this time, we get to know you and your baby. This makes the transition to CHIRP much smoother if it is needed.

Transitioning from NeoCHIRP to CHIRP

If your child needs longer-term IR, they will be transferred to an inpatient unit at BC Children's Hospital once they are well enough to leave the NICU. Most often, they will be under the care of the CHIRP team.

You may find the change in your relationship with medical and nursing staff to be the biggest challenge after transfer from the NICU. Although you are encouraged to be present as much as possible during your NICU stay, their medical and nursing staff do most of the hands-on care. As your child transitions to the inpatient unit in preparation for discharge, a key goal is to develop the strong working relationship that is so important for your success at home. Therefore, it is important that you be present at the bedside at much as possible, learning what is normal or unusual for your child and what information we use to make decisions. We look forward to hearing your observations, your questions and your thoughts during daily rounds. We will discuss appropriate changes to your child's management and, together, we will make a care plan for the coming day. Families are very involved and provide important information about their child's progress. The more comfortable and confident you feel, the better we will work together when you are at home.

Some families have expressed a feeling of being "abandoned" as they move from the high-intensity environment of the NICU to the inpatient unit where they spend much more one-on-one time with their baby, although nurses are always nearby and available. It is important to understand that, although this transition may sometimes feel stressful, it is an important step in establishing your confidence at home.

At any time, if you have concerns about the care being provided or the level of support that you are receiving, speak to a CHIRP team member so that any issues can be addressed.

In hospital, we can make changes to your child's care plan every day if needed. Daily changes are not always possible at home. As your child becomes more stable and needs less frequent changes to their medical and nutritional management, we will start more actively planning for home.

If we expect that your child will need to go home on PN, we will start the discharge planning process as soon as possible to make sure there are no unnecessary delays, but it can sometimes take up to 12 weeks. There are many steps in this process:

• Making sure your child has an intravenous line that is suitable for home
• Learning the steps and procedures needed to safely, competently and confidently give PN at home
• Learning how to care for any other devices, such as feeding tubes or stomas
• Learning how to order supplies
• Learning who to contact in case of problems, questions or concerns
• Developing a system of record-keeping to track progress at home
• Connecting you with a pediatrician and other supports in the community, including in-home nursing
• Organizing funding for supplies and certain medications

In the days leading up to discharge, each caregiver will complete 24 to 48 hours of Care-by-Caregiver. This will be done in the hospital. During that time, you will be entirely responsible for your child's care, including giving PN, feeds and medications using your home supplies. A Home IV Therapy nurse will observe and support you during hook-up and unhook. However, other medical and nursing staff will not routinely check on you.

If you have any questions or concerns during Care-by-Caregiver, you can reach out to us or your bedside nurse. Care-by-Caregiver is not a test; asking for help does not mean that you have failed. The goal of Care-by-Caregiver is to ease you into the transition of going home.

Preparing for your visit

The BC Children's Hospital Support Services has a Sibling Support Centre available for children who must be on campus while their sibling is in care or receiving care. A first-come, first-served booking is required. Please call (604) 875-3594 or book online. Visit the Sibling Support Centre webpage for more details.

Planning your visit will help you get the most out of your appointment.

What to bring

• Your child's BC Medical Card
• Your child's binder
• Your child's intake / output records (if separate from your binder)
• A food record
• Your child's medications (preferably the medication itself or a picture of the label)
• Your emergency kits (PICC, stoma, …)
• Your child's feeds and feeding supplies
• Any questions or concerns that you would like to discuss
• Taurolin or Heparin, for locking the PICC line after bloodwork
• A urine sample (if requested)

Parking

Parking at BC Children's can be difficult, especially during midweek clinic days. Give yourself plenty of time to find parking and walk across the large campus. You may wish to take advantage of the FREE valet service. This service is located by the TACC building main entrance (near Emergency).

If you are running late or having trouble with parking please contact the program secretary at (604) 875-2345, local 7464.

In-person clinic visits

Clinic visits are typically an hour long. You will see a doctor, a nurse and a dietitian at the same time. Depending on your family's needs, you may see our social worker with the entire team or privately after your clinic visit. Depending on your child's needs, you may also see your surgeon, the IV team, the ostomy nurse, the g-tube nurse or an occupational therapist.

• If your child is known to CHIRP: We will review your child's overall status, growth, bloodwork, medications and nutrition. The doctor will examine your child. We might adjust their care plan to support growth, to help with the transition from PN to an oral diet or tube feeds, to help manage symptoms, or if any issues come up. We will discuss these changes with you.
• If your child is new to CHIRP: We will ask about your child's medical and surgical history, growth history, feeding and nutrition history, mealtime routines and behaviour at mealtimes. We will discuss your goals and concerns, and we will answer your questions. The doctor will examine your child. We might adjust your child's care plan to support growth, to help with the transition from PN to an oral diet or tube feeds, to help manage symptoms, or if any issues come up. We will discuss these changes with you.

We do our best to follow your scheduled appointment time, but some clinic visits may go longer than expected. Please have a plan for family members at home if this should happen or for keeping your child entertained if you should have to wait. If possible, it is best to have the person most familiar with the day-to-day care attend the clinic visit, so that the team is able to best address any concerns and discuss an appropriate plan.

Virtual clinic visits

If your child is medically stable, we may do some clinic visits virtually. However, in-person assessments are very important, especially after you first go home and when your child is weaning off PN. You will need to come for regular in-person clinic visits.

• Please weigh your child before your clinic visit.
• Log into your clinic visit using the Zoom link provided by the Complex Feeding and Nutrition clerk. You will be asked to wait in a virtual meeting room. We will let you into the meeting at your scheduled appointment time.
• You will meet with the team for your clinic visit. Depending on your child and family's needs, your clinic visit may include a discussion with the team and a private discussion with the social worker. If your child needs a medical examination by a physician, we may ask you to come for an in-person clinic visit in follow-up. If needed, we may ask you to take your child for bloodwork or we may refer them for additional testing.
• After your clinic visit, the Complex Feeding and Nutrition clerk will contact you to schedule your follow-up appointment.
• We do our best to follow your scheduled appointment time, but some clinic visits may go longer than expected. We will notify you if we are expecting to be delayed.

Follow-up

If your child is on PN at home, we will see you for an in-person clinic visit 1 to 2 weeks after discharge. We will then see you regularly in clinic depending on your child's needs. This can range from once a week to once every 3 months. After your child weans off PN, we will see you less often – once every few months to once a year. How often you will need to come to clinic will depend on your child's needs.

We may ask you to take your child for bloodwork or for further investigations (such as x-rays) after your clinic visit. We will discuss these tests with you.

If your child has a central line

• If bloodwork is done as part of an in-person clinic visit, their bloodwork will be done in the Medical Day Unit (MDU). We will organize an appointment with MDU and will order Taurolin or Heparin for locking after bloodwork.
• If you live locally and your child is having bloodwork outside of a clinic appointment, you must call MDU at (604) 875-2345, local 3680, to schedule an appointment. Bring your own supply of Taurolin or Heparin for locking after bloodwork.
• If you do not live locally and your child is unwell or if you were seen for a virtual clinic visit, we may ask you to take your child for bloodwork in your community. We will give you more instructions.

If your child does not have a central line

• If your child does not have a central line, they may go to any outpatient lab for bloodwork.

• BC Children's Hospital Foundation. Meet the CHIRP Team.
• BC Children's Hospital Foundation. HPN Awareness Week: Abigail.
• BC Children's Hospital Foundation. HPN Awareness Week: Zion.
• BC Children's Hospital Foundation. HPN Awareness Week: Bridgette.

Resources for caregivers

• Oley Foundation
• Canadian Patient Parenteral and Enteral Nutrition Alliance (CPPENA)

Mouth: The digestion process begins in the mouth. Good chewing breaks down food into smaller pieces and helps prepare it for digestion and absorption. Enzymes in the saliva start breaking down carbohydrates and fats.

Stomach: After being swallowed, food travels down through the pharynx and the esophagus, into the stomach. The stomach holds food while it is being churned and mixed with stomach acid. Enzymes start breaking down proteins. When food is broken down into a liquid, it is released into the small intestine.

Small intestine: The small intestine is the longest part of the digestive system. Babies born at term have an average of about 200 to 250 cm of small intestine. As children grow, their small intestine also grows. It reaches about 3000 to 5000 cm in length by adulthood. Millions of tiny finger-like projections called 'villi' extend inwards from the inner lining of the entire length of small intestine. Each villi has even tinier finger-like projections called 'microvilli'. These villi and microvilli significantly increase the surface area available for absorption.

The small intestine has 3 parts: the duodenum, the jejunum and the ileum. It is important to consider the function of each segment.

The duodenum is the first and shortest segment of the small intestine. The liver, gallbladder and pancreas drain juices into the duodenum. Food mixes with these juices to continue the digestion process. Some nutrients, like iron, are absorbed in the duodenum.

The jejunum is the second segment of the small intestine. Most nutrients and many medications are absorbed in the jejunum. The fluid in the jejunum is high in electrolytes, like sodium. This is important to remember for children who have a jejunostomy: they will lose a lot of sodium in their ostomy output.

The ileum is the third segment of the small bowel. Food moves more slowly through the ileum. This gives the small intestine one last chance to absorb nutrients before they go into the large intestine. If undigested nutrients reach the ileum, it sends complicated signals to the stomach, the duodenum and the jejunum to slow down and be better at digesting and absorbing nutrients. Many nutrients, including vitamin B12, are absorbed in the ileum.

Ileocecal valve: The ileocecal valve is a one-way door between the ileum and the large intestine. It helps control the passage of contents from the small intestine into the large intestine. It also helps prevent bacteria in the large intestine from flowing back into the small intestine.

Large intestine (also called colon): The large intestine is the last and slowest part of the digestive system. Babies born at term have an average of about 60cm of large intestine. As children grow, their large intestine also grows. It reaches about 1500 cm in length by adulthood. The main function of the large intestine is to absorb fluid, as well as vitamins. Bacteria in the large intestine can also ferment fibre in the diet to produce short-chain fatty acids. Short-chain fatty acids are the main source of energy for cells in the large intestine. They also stimulate absorption of water and sodium, and stimulate intestinal adaptation.

Images:

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Gastrointestinal motility or peristalsis is the complicated series of wave-like muscle stretching and contractions that moves food, from the esophagus to the anus.

For some children with IF, problems with the nerves or the muscles in the intestine can cause those muscle contractions to be weak or disorganized. This prevents food from moving efficiently though the intestines. This is called "intestinal dysmotility". Intestinal dysmotility can affect one segment of the intestine, many segments of the intestine, or the entire intestine.

Symptoms of intestinal dysmotility can be similar to those caused by a blockage of the intestine, even when there is no physical blockage. These can include abdominal distension (bloating), abdominal pain, nausea, vomiting, constipation, diarrhea and loss of appetite.

What is intestinal adaptation?

When part of the intestine is removed, the remaining intestine can change its structure and function to try and make up for what was lost. This process is called adaptation. If adaptation is successful, the remaining intestine will become better at absorbing the nutrients and fluids needed for growth and good health. For this reason, even children with very short intestines may be able to reduce or stop PN.

What happens during adaptation?

The small intestine more than doubles in length from birth to adulthood. In children who have lost part of their intestine, the intestine continues to grow as the child grows. This helps with the adaptation process and to improve absorption. The growth rate of the intestine generally mimics the growth rate of the child. It is quickest in premature babies and young infants, and gradually slows down over the first few years of life.

Beyond normal growth, a shorter intestine can change in other ways to work better. In everyone, absorption of nutrients and fluids is increased dramatically because of the finger-like projections (villi and microvilli) on the lining of the intestine. During adaptation, there is an increase in the height of the villi and density of the microvilli, which can increase the area for absorption. This can help decrease the need for PN. All of these changes occur slowly over weeks, months and sometimes years.

Adaptation and intestinal rehabilitation

While adaptation happens naturally after surgery, some things encourage adaptation and some may slow it down. Our goal is to maximize adaptation by taking advantage of all possible strategies, and preventing or correcting anything that may stand in the way. The most important approach to promote adaptation is feeding into the digestive tract. Adaptation may be limited by many factors, including overall medical condition, infection, liver disease, inability to feed, malnutrition and others. You will find more information on both the positive and negative factors in other sections of this website.

How do we know if intestine is adapting?

Although there is often much discussion early on about intestinal length, what ultimately matters most is the intestine's ability to do its job to absorb nutrients and fluids. As adaptation occurs, you will see an increasing ability to use the food and fluids provided by mouth or tube for growth, and a decreasing need for PN. Even if absorption is not "perfect" (ie, there may be more frequent stools or looser stools than usual), a child may be able to eat and drink enough to grow and stay healthy with less PN or without PN over time as adaptation progresses. This will be a focus of every clinic visit.

‎The liver is the largest solid organ in the body. It is an essential organ with many functions that are vital to life. Some if its most important functions include:

• Filtering all the blood in the body and removing toxins and bacteria
• Producing bile, which helps with breaking down and absorbing fat
• Storing glucose and releasing it when the body needs energy
• Storing some vitamins and minerals, like vitamins A, D, E, K and B12, and iron and copper

Children with IF who need long-term PN are at risk of developing Intestinal Failure-Associated Liver Disease (IFALD), where the liver is not able to do all of the work it needs to do. Approximately 20% of children on long-term PN will develop IFALD, and approximately 4 to 10% of children will progress to liver failure and will need a liver transplant. However, many new treatments and strategies have been developed in the last 20 years. These have been very helpful in preventing and treating IFALD, even in high-risk babies, and are part of the care we provide to every child with IF.

IFALD is complicated and not well understood. It is more common in children who:

• Were born prematurely
• Had serious infections early in life or have had many serious infections
• Have short bowel
• Are not able to receive or tolerate feeds into the gastrointestinal tract
• Need long-term PN

IFALD is most commonly detected by following the bilirubin level in the blood. Liver enzyme levels may also be elevated when there is inflammation in the liver. We monitor these blood tests routinely, so we can quickly detect and treat any concerns for IFALD.

Prevention and treatment

Feeding: Feeding into the digestive tract helps protect the liver by stimulating gut hormone release and improving bile flow through the liver. Even small amounts help prevent inflammation in the liver, so we will recommend feeding your child into their digestive tract as soon as it is safe to do so.

Parenteral nutrition: Imbalances of PN nutrients can worsen inflammation in the liver. Providing too much or too little of certain nutrients can be harmful. We will closely monitor the composition of your child's PN and make any changes necessary. We will also decrease the amount of time your child is receiving PN as soon as possible and as much as tolerated. This is called "cycling" PN and allows for some hours off during the day. This will give your child more freedom to move around and helps keep their liver healthy.

Prevention of infection: Bloodstream, urinary tract or respiratory infections can worsen inflammation and lead to decreased bile flow through the liver. Children who receive PN are at risk for bloodstream infections because they have a central line in place. We take extra care when handling central lines to decrease the risk for bloodstream infections and we take any suspected infection very seriously.

Central venous catheters, or "central lines", are intravenous catheters that end in one of the body's big vein close to the heart. They are needed to deliver highly concentrated solutions into the bloodstream that can damage smaller veins. PN requires a central line for safe delivery.

There are several different types of central lines; the ones most commonly used children with IF are peripherally inserted central catheters (PICCs), Broviac or Hickman catheters or, less commonly, ports. CHIRP patients use PICCs most of the time while receiving PN.

What is a cuffed PICC?

The parts of a cuffed PICC are the following:

1. The PICC is a thin, flexible tube (the catheter) inserted into a vein in the arm, and then travels to a larger vein that drains directly into the heart
2. The Dacron cuff rests under the skin where the catheter exits the body, and helps hold it in place
3. The tip of the catheter lies in a large vein close to the heart and this is where the PN will infuse
4. The clamp that stops fluid flowing in and out of the catheter is called a pinch clamp
5. At the end of the catheter, outside the body, is a positive pressure cap. It makes sure blood does not collect at the catheter tip inside the body.

How is it put in?

• Cuffed PICCs are inserted by an interventional radiologist. Special equipment is used to make sure the tip is in the right place.
• A small needle is inserted into a vein in your child's arm under anaesthetic.
• The catheter is then inserted through the needle access point. There may be some bleeding at the exit site at first, this is normal.
• The cuff lies under the skin, close to the exit site.
• A clear bandage or dressing is place over the PICC after coiling the catheter to make sure it stays in place.
  

What stops the PICC from falling out?

The PICC is held in place by the cuff and the dressing. Your child's dressing should be changed every week or when it is wet, dirty or falling off. The Home IV Therapy nurse will teach you how to change the dressing. You will practice on a model and then change your child's dressing with supervision until you feel comfortable.

This is an example of how a PICC can look once the dressing is on. Your child's dressing may not look exactly like this; there are many different ways to place the dressing.

Other types of central lines

Hickman catheters, Broviac catheters and ports are other types of central lines that can also be used for PN in children with IF. They are similar to PICCs, except they are inserted directly into a larger vein in the chest or neck and exit on the chest instead of the arm. There are instances when these types of catheters are a better choice for your child and the CHIRP team will discuss this with you if needed.

Potential central line problems

Although central lines are absolutely necessary for children who need PN, there are some potential problems that can arise.

Infection: Microorganisms (bacteria/fungi) are always present in the environment. They live on our skin and in our bodies, and are found in the air, soil, water and on clothing. Microorganisms are beneficial, but at times can cause disease or illness. Serious infection is one of the more worrisome risks of PN therapy. If your child experiences any symptoms of infection (fever, chills, swelling, redness or drainage from the catheter exit site), your child needs to be evaluated for infection. Details on what to do and who to call are included in our home PN manual. We also sometimes use special solutions in the catheter (line lock solutions) when it is not being used to help prevent infection from occurring.

Breakage: Central lines can break outside of the body. This is a concern because it can not be used and increases the risk of infection. We will teach you what to do if you suspect that your child's line is broken. We may be able to repair some broken lines. If we are not able to repair the breakage, your child's line will need to be replaced.

Blockage: Central lines / PICCs can become blocked, making it difficult or impossible to push fluid through it. This can happen when there is build-up within the catheter from fibrin, calcium or a blood clot. If this happens, we will treat the catheter with a special solution called tPA that helps break up the blockage. Occasionally, if the catheter can not be unblocked, it will need to be replaced.

Everyone has bacteria in their intestines. The small intestine has a small number of bacteria, the large intestine has a large number of bacteria. They play an important role in supporting good health. They are important for forming poops, absorbing nutrients, making essential vitamins and helping the body fight some diseases.

The body uses different mechanisms to maintain a healthy intestinal flora. These include:

• A complicated series of muscle contractions moves food, waste and bacteria down from the stomach to the rectum, preventing bacteria from accumulating in the intestine.
• Stomach acid and bile kill many bacteria before they can reach the small intestine.
• A valve between the small and large intestines (the ileocecal valve or ICV) prevents bacteria from flowing backward from the large intestine into the small intestine.

In children with intestinal failure, one or more of these mechanisms is disrupted and too many bacteria sometimes grow in the small intestine. Symptoms of bacterial overgrowth can include abdominal distension (bloating), abdominal pain, gassiness, diarrhea, loss of appetite, poor weight gain and nutrient deficiencies.

Diagnosis

Bacterial overgrowth can sometimes be diagnosed using different tests. These tests can be invasive and are often difficult to interpret or to perform in children with IF. We may recommend starting treatment based on symptoms only and without specific testing.

Prevention and treatment

Antibiotics: The first treatment for bacterial overgrowth is usually low-dose antibiotics. They decrease the number of bacteria in the small intestine and help manage symptoms. Some children do well with one short course of antibiotics; others need to rotate multiple antibiotics over a long time. We will only recommend as much (or as little) antibiotics as your child needs.

Many parents worry that their child will become resistant to antibiotics that could be needed in the future. Antibiotics used for bacterial overgrowth are given in doses much lower than those needed to treat an infection. They act mainly on the bacteria in the small intestine and little antibiotic is absorbed.

Prokinetics: If impaired intestinal motility is contributing to bacterial overgrowth, prokinetics can sometimes help. These are medications that help the intestine contract better.

Surgery: When the small intestine is dilated, it cannot contract well enough to move food, waste and bacteria down to the rectum. If this is contributing to bacterial overgrowth and medications have not helped, we may recommend surgery to decrease the diameter of the small intestine.

Diet: While certain foods can make some symptoms worse, diet cannot prevent or treat bacterial overgrowth in children with IF. We may make recommendations to correct nutrient deficiencies made worse by bacterial overgrowth, if needed.

Probiotics

Probiotics are bacteria that help the body work well and so, are often called "good bacteria". They are naturally part of a healthy intestinal flora and help defend the body from infections caused by "bad bacteria".

Many parents ask if their child can use probiotics in addition to or instead of antibiotics. There is a lot of interest in using probiotics in children with IF. Unfortunately, probiotics are relatively new and we still do not know enough about them, including which type and how much to give. There may be benefits to using them, but there are also potential risks. If you are interested in giving probiotics to your child, discuss this with the CHIRP team.

Our goal is always to decrease or end your child's need for PN, but this is not always possible. Some children continue to need PN over the long-term despite everyone's best efforts. When this is the case, serious complications like worsening liver disease with complications or increasing challenges maintaining a suitable central line can sometimes arise. When IR does not work for a child, intestinal transplant may be the next step.

BC Children's Hospital is not an intestinal transplant centre. When a child may need a transplant, we work closely with one of the intestinal transplant teams in Canada. Transplant assessments are done at one of these transplant centres. They are thorough and can last up to 4 weeks, during which many tests are performed. Once the assessment is complete, the transplant team determines if transplant is the best option.

Children who are eligible for transplant may receive a small intestine, a small intestine and liver or a multivisceral transplant (stomach, small intestine, liver and pancreas) depending on their medical needs.

For children with severe or progressive complications of IF, transplantation can be life-saving. However, it carries its own risks and challenges. These need to be discussed and considered before choosing this option. This discussion would include you (including your child, where appropriate), the consulting transplant team and our team, and would weight the benefits and risks of all available management options.

Parenteral nutrition (PN) is a complex treatment that is almost always done in the hospital. You may hear some people call it TPN, although Total Parenteral Nutrition suggests that it is the only source of nutrition.

Children with IF cannot absorb enough nutrients from a regular diet, either because their bowel is too short or because it does not work very well. They need PN to grow and be healthy. Some children with IF receive most of their nutrition from PN; others use it as a supplement to eating and drinking.

The body needs water, protein, carbohydrates, fat, vitamins and minerals to stay healthy. PN delivers nutrients directly into the bloodstream using an intravenous (IV) line. They do not need to be digested or absorbed, so they do not go into the stomach or bowel. PN allows the body to get proper nutrition and stay healthy when a child cannot get all their nutrition from food.

Many parents ask if their child would feel hungrier if their PN was decreased or stopped. Hunger and fullness are complicated processes and are affected by many factors. These include how empty or full the stomach and intestines are, blood sugar levels and a number of hormones. While PN may affect blood sugar and certain hormone levels, and can therefore affect hunger and fullness, it is not nearly as effective as food at satisfying hunger. Some parents have noticed an increase in their child's appetite when PN is unexpectedly stopped, but the increase in oral intake is usually small and not proportional to the decrease in PN intake. Other parents have noticed a decrease in their child's appetite when PN is increased, but this decrease is generally temporary.

When the intestines are sick or recovering from surgery, feeding into the gastrointestinal tract is not possible. Until they start to recover, children with intestinal failure receive all their nutrition from PN.

Feeding even small amounts into the gastrointestinal tract stimulates intestinal adaptation, so feeds are introduced slowly as soon as it is safe to do so. At first, these are usually given through a feeding tube at a very slow rate and increased gradually. Once your child is tolerating small amounts of tube feeds, we will slowly introduce oral feeds and increase them as tolerated. With time, some children will be able to stop using their feeding tube. Others will continue to need it.

Oral feeds are introduced slowly as soon as it is safe to do so. For most babies, this usually means starting with small volumes by bottle twice per day. Feed volumes and frequency will slowly be increased.

The decision to breastfeed / chestfeed, bottle-feed or do both will be yours, and only yours, to make. We will support whatever feels right for you and your family.

If you are interested in breastfeeding / chestfeeding, discuss this with the CHIRP team. Some babies may be able to tolerate feeding to a full breast / chest; others may do better with feeding to a partially or fully pumped breast / chest. We will discuss a plan that works both for you and your baby.

If you think your child is ready to start solids, discuss this with your dietitian. You will work closely to tailor your child's diet to their needs.

Babies with IF are at risk of feeding difficulties. It is important that your child experiment with different foods and textures as soon as they are ready. This usually happens around 6 months of age, but it can happen earlier or later.

Your child is showing signs that they might be ready to start solid foods if they:

• Can sit on their own for 10 to 30 seconds
• Can hold their head up and have strongly developing trunk control
• Are interested in what you eat
• Open their mouth for the spoon and close their lips around the spoon
• Don't push food out of their mouth with their tongue

Getting Started

• Start slowly; your child is learning a new skill. They will gradually get better over the next 6 to 12 months.
• Choose a time when your child is awake, alert and interested in eating, but not too hungry so they don't become frustrated.
• Provide supportive seating with good support for your child's feet. At first, they might need a high chair with a high back and towel rolls around their trunk. With time, an adjustable high chair or a booster seat may be better.
• Create a mealtime routine and try to do this at every meal. This will teach your child what to expect when coming to the table. Your routine can be very simple; for example: washing your hands, sitting at the table, putting on their bib, eating and then cleaning up.
• Create a calm environment and minimize distractions at mealtimes (ex: turn off the television).
• Don't worry if things get messy! Let your child learn new skills and explore with their hands. Avoid cleaning their face after every bite. This unpleasant experience can create a negative association with mealtimes and with eating. Wait until AFTER the meal is done to clean them up.
• Your child will learn to eat by watching others and by smelling new foods. Include them in family meals, it will teach them the importance of eating together. It will also help them develop important language and social skills.
• You may want to give your child a clean spoon to hold while you feed them with a different spoon. Remember – they will learn by experimenting!

What to offer

Help your child develop healthy eating habits and attitudes. Be a healthy-eating role model and offer a variety of nutritious foods over and over again, even if they refuse them. Many babies need to be exposed to the same food 15 to 20 times before accepting it. Keep in mind that your child will have their own food likes and dislikes. If they continue to refuse certain foods after 15 to 20 exposures, respect that they may simply not like that food.

Feed your child based on what they can do, not on how old they are. Pay attention to them and trust your own judgment. Some babies will move quickly to easy-to-chew grown-up foods, others will take more time with each step. Moving to the next stage before they are ready increases the risk of choking and can lead to negative experiences with food.

Signs that the foods your child is eating may not match their developmental stage include:

• Gagging when eating, although this can be a normal part of the learning process. If you are concerned, talk to us about it.
• Vomiting when eating
• Coughing or choking when eating
• Spitting out food
• Holding food inside their mouth or cheeks
• Refusing to eat
• Swallowing without chewing

How much to offer

Your baby's appetite will vary from meal to meal and from day to day. They may eat a lot on some days and very little on other days. At some meals, they may not eat anything at all. This is normal. Never pressure them to eat or to eat more.

Some babies with IF want to eat more than their intestines can tolerate. If this is the case, you may need to limit the amount of food your child can eat. We will help you decide how much food to offer.

Help your child develop healthy eating habits and attitudes. Follow Ellyn Satter's Division of Responsibility. Ellyn Satter is a dietitian and family therapist; she developed the Division of Responsibility, which is considered the gold standard for feeding children.

• You are responsible for offering a variety of nutritious foods at regular times each day. As the caregiver, you decide what foods will be offered, when they will be offered and where they will be offered.
• Your baby is responsible for how much to eat from the foods you offered. Some days, your baby will eat a lot. Some days, your baby will eat very little. At some meals, your baby may not eat anything at all. Trust your baby, let them guide how much food to offer. They know when they are still hungry and when they are full. You are teaching them the very important skill of listening to their own body.

Pay close attention to your baby's cues. This will help build a trusting feeding relationship. If your baby is refusing to eat, end the meal – even if they haven't eaten anything – and try again later. Even meals where little or no food has been eaten are important. Playing with food will get your child used to the texture and smell of foods, and get them comfortable with sitting in a high chair.

Your baby is still hungry if they:

• Get excited when they see food
• Lean forward and reach for food
• Smack their lips
• Open their mouth for food
• Get upset when food is taken away

Your baby is full if they:

• Close their mouth
• Cover their mouth with their hands
• Turn their head away
• Push the food away

Let your baby eat at their own pace. Do not force them to finish eating on a schedule, but do keep mealtimes between 20 to 30 minutes unless your baby is still actively eating.

Never pressure your child to eat. This includes:

• Pressuring: "Finish your carrots"
• Coaxing: "Just eat one more bite"
• Bribing: "If you finish your dinner, you can have dessert". Bribing also includes:
• Using food as a reward for good behaviour: "If you behave nicely, I'll give you dessert"
• Using food to regulate emotions: "You fell and hurt yourself, let's get dessert to make it all better"
• Punishing: "If you don't finish your dinner, you can't have dessert"
• Force-feeding: physically putting food into the child's mouth when they are refusing to eat.

Pressuring a child to eat can have negative long-term impacts. They may:

• Develop a strong dislike to the foods they are being pressured to eat
• Develop a strong dislike to all foods and refuse to eat
• Lose the ability to recognize when they feel hungry or full
• Develop a strong preference for "reward" foods and lose interest in "non-reward" foods
• Continue to use foods to regulate emotions later in life

Refer to the handouts "Preventing Choking in Babies and Young Children: For Child Care Providers" and "Choking Rescue for Babies" from HealthLink BC for more information.

Sodium is a mineral that is found in salt. Our body needs it to stay healthy:

• It helps children grow and develop properly
• It helps maintain proper fluid balance
• It helps muscles and nerves work properly
• It helps control blood pressure

Sodium can easily be absorbed from a healthy bowel. Children with intestinal failure may need more sodium than average if part of their intestine was removed or if their intestine doesn't work properly. This is particularly true for children who have an ostomy, especially if they have high outputs, those who have had large intestine removed, and those who have a lot of poops.

Children with IF who don't absorb enough sodium have difficulty gaining weight even when they receive enough nutrition. By looking at urine sodium levels, weight gain, and poops or ostomy outputs, we'll be able to recommend how much sodium to give to make sure that your child gets enough.

Signs and symptoms of low salt intake include:

• Poor growth
• Decreased appetite
• Nausea or vomiting
  
• Irritability
• Fatigue or weakness

Sodium supplement

Never give a sodium supplement if you have not been instructed to do so and always follow your doctor's or dietitian's instructions. Giving too much or too little sodium, or not following the recipe can cause dangerous electrolyte imbalances.

If your baby is breastfeeding or bottle-feeding, you can give extra sodium using the following homemade salt solution recipe.

Recipe for salt solution

(4 milliequivalents of sodium per millilitre)

• Mix 2 level tablespoons (30mL) of salt with 2/3 cup (160mL) of cooled, previously boiled water. Store any salt solution that has not been used in the refrigerator.
• You can prepare larger portions of this recipe and store it in the refrigerator in a covered container for up to one week. Shake before using.

This salt solution is very salty and some children may not like it. If your child refuses it, you can try:

• Mixing it with breast milk or infant formula
• Using a pacifier designed for giving medications
• Using a syringe placed on the side of their mouth and giving it slowly
• Using a syringe placed on the side of their mouth and giving it quickly
• Giving smaller doses more often throughout the day

If your child continues to refuse this salt solution, contact your dietitian for further support.

High sodium diet

It's important that your child follow a healthy diet. Unless you've been advised otherwise, your child should enjoy a wide variety of healthy foods as recommended by Canada's Food Guide.

As your child grows older and eats more table foods, your dietitian may recommend that you add extra salt to their meals in addition to or instead of the salt solution. To do so, you may:

• Use salt liberally when cooking and at the table.
• Offer foods that have a high sodium content (PDF). These should not be staple foods in your child's diet, but rather they should be part of a healthy diet that also includes fresh and wholesome foods.

All of your home PN solutions and supplies will be provided by Calea, our supply company. Their cost is entirely covered by the Provincial Health Services Authority.

Calea will deliver everything you need for your child's PN right to your door. This includes PN solutions, additives, line locks, flushes, an infusion pump, tubing, a refrigerator, and anything else you might need for your child's PN, as long as they can be provided by Calea and have been approved by the CHIRP team or the Home IV Therapy nurses.

After discharge, you will be responsible for ordering supplies from Calea every two weeks. It's important that you call them on or before your scheduled order dates to confirm which supplies are needed. If you don't place your order on time, there's a risk that you might not get your child's PN solutions and supplies on time. This also affects the production schedule in Calgary, which might affect the production and delivery of other children's PN.

To place an order, contact Calea

Phone: (604) 294-1500, extension 5

Email: CSRVancouverGroup@calea.ca

At Home Program / Product Distribution Centre

If your child has a feeding tube, their supplies will be provided by Product Distribution Centre. Their cost is covered by the At Home Program (PDF), as long as they can be provided by Product Distribution Centre, have been requested by a qualified healthcare professional, and have been approved by the At Home Program.

Product Distribution Centre will deliver your child's supplies right to your door. This may include certain formulas, certain additives, bags and tubing, extension sets, a feeding pump and backpack, feeding tube and syringes.

Only qualified healthcare professionals can submit requests for supplies. You will only be able to order pre-approved supplies. The At Home Program will only approve a specific amount of each supplies and there is an annual funding cap per child for medical benefits. Additional items may be approved with clear justification from a healthcare professional.

After discharge, you will be responsible for ordering supplies from Product Distribution Centre every 1 to 3 months; you can order up to 3 months at a time. If you order more than once in one month, you will be charged a delivery fee. The delivery fee is waived if you are ordering a NEW item that was approved after your first order was placed.

To place an order, contact Product Distribution Centre

Phone: (604) 927-2910

Toll-Free: 1-877-927-2234 

Email: PDC.CallCentre@gov.bc.ca

If we are able to stop your child's PN, your At Home Program funding may stop. Our social worker may be able to find other sources of funding.

Having a child on HPN can be very stressful. It's important to use the supports available to you (family, friends, home care nurses, medical team). If you or any member of your family is struggling with the transition or the impact of living with a child on HPN, contact your social worker.

The social worker can be reached by email or by phone at (604) 875-2345, local 6435 on Monday, Wednesday and Thursday between 9:00 to 17:00.

Dressing changes

Hand hygiene

Flushing the line

Hooking up to parenteral nutrition

Unhooking from parenteral nutrition