Chronic intestinal failure happens when the intestines cannot absorb sufficient nutrients, water and electrolytes that the body needs to stay healthy.¹ This can be caused by a range of conditions, such as gastrointestinal or systemic conditions, and certain cancers that affect the gut function and absorption of food.¹ Malabsorption of nutrients results in dehydration, malnutrition, poor growth and can affect other organs. Chronic intestinal failure may be reversible or irreversible depending on the underlying cause, treatment and the ability of the intestines to recover and function. Chronic intestinal failure can affect children and adults.

People with chronic intestinal failure usually require a type of nutrition support called parenteral nutrition (PN) to maintain their health.² PN is an intravenous (IV) feeding (into the bloodstream) of a specialised nutrition solution via a tube/catheter connected to a large central vein near the heart, bypassing the digestive tract.³ PN is commonly administered at home, this is called home parenteral nutrition (HPN).² Many people with chronic intestinal failure require HPN for many years, or even the rest of their lives.⁴

Synonyms: CIF

Universal rare disease classifications provide a common language for recording, reporting and monitoring diseases. Please visit the Rare Disease Classifications page for more information about these internationally recognised classifications.

ORPHA:294422 Chronic intestinal failure

ICD-11: DA96.05 Intestinal failure

The symptoms and severity of chronic intestinal failure vary between individuals. Some common symptoms include malnutrition, vitamin deficiencies, nausea, vomiting, weight loss, diarrhea, dehydration, abdominal pain, constipation, poor growth, intestinal bacterial overgrowth and others.^5,10 There may be other symptoms depending on the underlying cause of intestinal failure.

Chronic intestinal failure can lead to complications in other organs such as the liver, stomach, kidney, colon and bones.¹⁰

People with chronic intestinal failure requiring HPN may be at risk of HPN-related complications such as parenteral nutrition-associated liver disease, gall bladder stones, kidney failure, thrombosis (blood clots), central-line associated bloodstream infection, and loss of central venous access.^4,7

Please speak to your medical team to learn more about the symptoms and complications of chronic intestinal failure.

Rare diseases are often serious and progressive, exhibiting a high degree of symptom complexity, leading to significant disability. Majority of the estimated two million Australians living with a rare disease meet the Australian Government’s definition for disability (in accordance to the Australian Public Service Commission and Australian Bureau of Statistics), and many experience severe and permanent disability impacts. If you or someone you care for is experiencing disability-related impacts from a rare condition, please speak with a health or disability professional for advice. Information about relevant disability support can be found at the RARE Portal’s Disability Support Information page.

Chronic intestinal failure can be caused by a wide range of conditions which affect the gut function and nutrients absorption. This include:^1,6,10

• Other gastrointestinal conditions such as short bowel syndrome (the most common cause of intestinal failure), Crohn’s disease, mesenteric ischemia, chronic intestinal pseudo-obstruction, malformation of the intestine, coeliac, , Hirschprung’s disease and others
• Systemic disease that affects multiple body systems such as scleroderma
• Autoimmune disorders such as autoimmune autonomic ganglionopathy
• Neurological diseases
• Cancer such as intra-abdominal cancer, pelvic cancer
• Radiation leading to inflammation and damage to the intestine (radiation enteritis)
• Surgery complications

Diagnosis of chronic intestinal failure may be made based on physical examination, medical history, blood tests, stool examinations, nutritional assessments, imaging tests such as X-ray, CT scans, and intestinal biopsy.⁸

Please speak to your medical team to learn more about the available diagnostic pathways for chronic intestinal failure.

There is currently no curative treatment for chronic intestinal failure. Treatment is targeted at managing symptoms (symptomatic management) and involves a multidisciplinary care team.

Parenteral nutrition (PN) or home parenteral nutrition (HPN) is the primary treatment for chronic intestinal failure, and involves infusion of a specialised nutrition solution into the bloodstream through a tube/catheter passing into a large central vein.³ The specialised nutrition solution is made up of nutrients the body needs, including carbohydrates, proteins, fats, vitamins, amino acids, fluids and electrolytes and is tailored to each individual. These tubes generally remain in place for long periods of time and are called central venous access device or central venous line. It is important that HPN users, their carers or families are adequately trained and retrained in the latest best practice of HPN to prevent complications.⁴ More information about PN can be found at NSW Agency for Clinical Innovation: Parenteral nutrition.

Other treatments may include:^7,9

• Medications to improve bowel absorption, decrease intestinal loss and reduce the need for IV supplementation
• Diet and nutritional therapy
• Non-transplant surgery to lengthen the intestines to improve bowel function
• Intestinal transplant (this may not be suitable for everyone and may have other risk)

Please speak to your medical team to learn more about the possible treatment or management options for your condition. Treatment will depend on an individual’s specific condition and symptoms. It is also important to stay connected to your medical team so that you can be made aware of any upcoming clinical trial opportunities.

Clinical care for rare diseases often involves a multidisciplinary team of medical, care and support professionals. Please note that the information provided here is as a guide and that RVA does not necessarily monitor or endorse specific clinics or health experts.

Healthcare professionals involved in the care of people with chronic intestinal failure may include general practitioners (GP), paediatricians, gastroenterologists, dietitians, nutritionists, cardiologists and infusion nurses. The need for different healthcare professionals may change over a person’s lifetime and extend beyond those listed here.

We are not aware of any Australian guidelines for chronic intestinal failure. The following guidance are around treatments related to chronic intestinal failure:

• A framework to support quality of care for patients with chronic intestinal failure requiring home parenteral nutrition developed by The Australasian Society for Parenteral and Enteral Nutrition (AuSPEN) including a multidisciplinary working group of 15 clinicians; published in 2019.
• AuSPEN clinical practice guideline for home parenteral nutrition patients in Australia and New Zealand includes recommendations on patient selection, patient training, formulation and monitoring regimens, and preventing and managing complications; published in 2008.
• NSW Agency for Clinical Innovation: Central venous access devices developed by Intensive Care NSW for clinicians managing patients with a central venous access device.
• The Transplantation Society of Australia and New Zealand: Clinical Care Guidelines on Intestinal Transplantation includes information on intestinal transplant for intestinal failure.

The following guidance is available from international experts outside Australia; however, there may be information that is not relevant or applicable to the Australian context, and may not be up to date:

• ESPEN practical guideline: Clinical nutrition in chronic intestinal failure developed by the European Society for Clinical Nutrition and Metabolism (ESPEN) for health care providers involved in the management of patients with chronic intestinal failure; published in 2021.
• ESPEN guidelines on chronic intestinal failure in adults include recommendations for safe and effective management of adult patients with CIF; published in 2016.

Individuals living with rare diseases may have complex medical issues and disabilities, which are not always visible. It is often useful to refer to their medical history as well as personal information such as a medical card, doctor’s letter, or if available, a rare disease passport, for relevant information.

It may be important to consider the following when managing individuals living with chronic intestinal failure at emergency departments/:

HPN users may be at risk of central line infections, air embolus (air entering bloodstream through the central line), unconsciousness and septic shock, which can be life-threatening. Appropriate training on accessing and managing central venous access devices are important to prevent complications. NSW Agency for Clinical Innovation: Central venous access devices includes recommendations for clinicians managing patients with a central venous access device.

There are specific considerations around participating in rare disease research, including clinical trials. It is important to be mindful of issues such as data privacy, research ethics, consent and differences in research regulations between Australia and other countries.

If you are interested in finding clinical trials for your condition, please visit the following websites; however, there may not be any clinical trials available:

• Australian Clinical Trials
• ClinicalTrials.gov

It is best to discuss your interest in any clinical trials with your medical team to determine suitability and eligibility.

Please note that RVA does not necessarily monitor or endorse each group/organisation’s operational governance and activities.

Australian Organisation:

Parenteral Nutrition Down Under (PNDU)

Website: https://pndu.org/

PNDU’s mission is to support, research and inform consumers, carers and providers of parenteral nutrition for intestinal failure.

Please note that RVA does not monitor or endorse each group/organisation’s operational governance and activities. When engaging with a group, please consider the information on the RARE Portal’s Finding Helpful Peer and Community Supports page.

Chronic intestinal failure varies between individuals, and each person’s experience is unique.

Parenteral Nutrition Down Under: Videos has personal stories of people living with chronic intestinal failure and HPN.

Personal stories shared with RVA: Ebony’s story, Jodie’s story

If you would like to share your personal story with RVA, please visit the Rare Voices Australia: Share Your Story page. RVA will consider your story for publishing on our website and inclusion on the RARE Portal.

For information on available government and social services that provide support for individuals with a rare disease, please visit the National and State Services pages.

People living with a rare disease, including families and carers, often face unique challenges such as diagnostic delays, misdiagnoses, limited treatment options, and limited access to rare disease specialists and support. These challenges may impact people’s emotional wellbeing and quality of life. Many people find it helpful to seek mental health and wellbeing support to cope with ongoing stress and uncertainty. Connecting with people who have shared experiences through a support group may also be helpful. Information about relevant mental health and wellbeing support can be found at:

• Mental Health and Wellbeing Support for Australians Living with a Rare Disease
• The National and State Services pages underneath the ‘Mental Health’ sections listed

Further information on chronic intestinal failure and PN can be found at:

• Parenteral Nutrition Down Under: Useful Documents & Resources
• NSW Agency for Clinical Innovation: Information guide for patients and carers on parenteral nutrition

NSW Government eviQ Education: Central Venous Access Devices

The Australasian Society for Parenteral and Enteral Nutrition (AuSPEN): Resources

Orphanet: Chronic intestinal failure

This page has been co-developed by Rare Voices Australia (RVA)’s RARE Portal team in consultation with Parenteral Nutrition Down Under.

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