What Is Paediatric Crohn’s Disease? 

Introduction

Crohn’s disease is a type of inflammatory bowel disease that causes inflammation of the digestive system.¹ Crohn’s disease can occur in any age group, whilst paediatric Crohn’s disease is a specific subtype that occurs in individuals under the age of 18.

20–30% of individuals with Crohn’s disease have symptoms that start before the age of 20, though the average age of diagnosis is 30 years old. It often takes a long time for Crohn’s disease to be diagnosed, which is why it is so important for both healthcare professionals and patients to be aware of the signs and symptoms to assist in early detection and management.

What is crohn's disease?

Crohn’s disease is a type of inflammatory bowel disease that occurs when the lining of the digestive system becomes inflamed. Areas in the gastrointestinal tract, anywhere from the mouth to the rectum, can be affected.² This can lead to a number of different symptoms, including mouth ulcers, stomach aches and diarrhoea. 

This inflammation caused by Crohn’s disease is mainly due to changes in the immune system. Individuals with Crohn’s disease have a high number of neutrophils, a type of immune cell that fights infection, in their gut.¹ These neutrophils attack the healthy bacteria and viruses found in the digestive system, causing an imbalance in the gut.

Crohn’s disease occurs in episodes, where there are periods of active, symptomatic disease, followed by periods where symptoms temporarily resolve. These symptomatic episodes are known as ‘relapses’ or ‘flares’.² The primary aim of managing Crohn’s disease is to decrease the number of relapses that a patient has. 

Causes and risk factors of paediatric crohn’s disease

The exact causes of Crohn’s disease are unknown; however, the following factors are thought to be causes of or contributors to the disease:³

• Genetics and family history
• Smoking
• Diet, including whether or not the child was breastfed
• Lifestyle and environmental factors
• Medication use, including antibiotics 

Lots of research has been done on the link between Crohn’s disease and genetics. Crohn’s disease does not have a specific inheritance pattern, meaning that it is not passed down from our parents. Instead, it is more likely that there are a large number of genes that increase a patient’s risk of developing Crohn’s disease throughout their lifetime.⁴

The development of Crohn’s disease is also related to the gut microbiome. The gut microbiome is the community of microorganisms (including bacteria, viruses and fungi) present in the digestive system. Having a variety of microorganisms in the gut is healthy and aids metabolism and digestion. However, when there is a poor variety of microorganisms, or the species are unbalanced, this can lead to infection or chronic diseases such as inflammatory bowel disease (e.g. Crohn’s). Type 2 diabetes and cardiovascular disease are also linked to an unbalanced gut microbiome. An unbalanced gut microbiome may be caused by antibiotic use and/or a typical ‘Western’ diet, including lots of processed food and minimal fruits and vegetables. Gut health is often better in individuals who are breastfed, consume probiotic foods regularly and have a varied, balanced diet.

What are the symptoms of crohn’s disease?

Symptoms experienced by those with Crohn’s disease can be categorised into digestive and non-digestive symptoms. These tend to occur during relapses or acute flares only, though some children with paediatric Chrone’s disease may have symptoms at all times.

Digestive symptoms experienced by children with Crohn’s disease may include:¹

• Tummy aches
• Diarrhoea
• Rectal bleeding
• Reduced appetite
• Weight loss
• Mouth ulcers
• Skin tags or fissures (breakages) on or around the anus 

Non-digestive symptoms experienced by children with Crohn’s disease may include but are not limited to:¹

• Stunted or slow growth
• Fever
• Lethargy
• Joint pain
• Rashes, for example, erythema nodosum
• Brittle bones
• Eye problems

The most common symptom is abdominal pain, experienced by over 67% of patients at diagnosis. Weight loss, diarrhoea and fever are also very common first symptoms. 

Growth delay is a symptom that is specific to paediatric Crohn’s disease. It is estimated that 15–40% of patients with Crohn’s disease suffer from growth deficiency throughout the course of their disease.³ This occurs due to poor food absorption in the gut and excess nutrient loss through diarrhoea. With reduced appetite being one of the main signs of Crohn’s disease, it is especially hard for parents of young children diagnosed with Crohn’s to ensure their child is getting enough nutrients. Deficiencies in important vitamins and minerals, such as vitamin D, can increase the risk of delayed puberty and, in later life, weak bones. Prompt diagnosis can help patients avoid excess weight loss or growth stunting throughout their childhood and teenage years.

How is crohn’s disease diagnosed?

To diagnose paediatric Crohn’s disease, initially, a medical practitioner will ask about any symptoms and do a physical examination.⁶ This may involve checking your child’s weight, checking for mouth ulcers, an abdominal examination, and in rare cases, checking for anal fissures or skin tags indicative of damage caused by Crohn’s. As this is an intimate examination, a chaperone will be provided. It is also within your or your child’s right to decline an intimate examination; however, these examinations are often not required for diagnosis unless the child expresses discomfort in the area.

Blood tests are used to test for markers of inflammation as well as vitamin deficiencies.⁷ A stool test will also likely be ordered to check for any active infections as well as for a protein called calprotectin, which may suggest the presence of inflammatory bowel disease if levels are above a certain threshold.

If blood and stool test results indicate a need for further investigations, the gold standard test for Crohn’s disease is a colonoscopy and/or endoscopy. A colonoscopy is a camera test in which a tube is passed through the anus and rectum into the bowel. An endoscopy is a camera test in which a tube is passed through the mouth, down the oesophagus and into the digestive tract. These are normally done under sedation. During the colonoscopy or endoscopy, a small sample will be taken from the bowel wall and examined under a microscope. This is called a biopsy. This helps differentiate Crohn’s disease from other forms of inflammatory bowel disease, such as ulcerative colitis. 

Other imaging techniques may be required if the patient has severe and sudden symptoms that could indicate a complication of Crohn’s disease has occurred, such as a bowel obstruction. This includes scans such as chest and abdominal X-rays, abdominal CT scans and abdominal/pelvic MRI scans.⁶

Treatment for crohn’s disease

There are two aspects to managing Crohn’s disease: treatment of acute flares and maintaining remission.

Treatment of acute flares

Acute flares of Crohn’s disease must be managed before patients are started on any long-term medications. Firstly, healthcare professionals must establish how at risk the patient is of complications. Normally, the patient will be started on corticosteroids, for example, budesonide or prednisolone.⁷ This medication is taken for a short period until symptoms have resolved. A specialist must be involved if initiating steroid therapy in children.

If the child is at high risk of complications or has severe growth delay, drugs known as TNF inhibitors may be started. This can be for a short period or long term, either alone or in combination with other medication.

If a patient has nutritional deficiencies or has experienced weight loss or stunted growth, extra feeding may be required. This is often done if the patient is very unwell and is admitted to the hospital during the symptom flare. Feeding can be done through a tube up the nose, called an NG tube, or inserted through the skin. 

Maintaining remission

Long-term treatment for Crohn’s disease is often referred to by healthcare professionals as maintaining remission. Maintenance of remission is most commonly done using immunosuppressant medications, such as azathioprine or mercaptopurine, which reduce the activity of cells in the immune system.8 This is continued long-term at a dose specific to each patient to help prevent flares. It does, however, have side effects, including the fact that those on immunosuppressants are more at risk of other illnesses.

It should be noted that there is a wide variety of medications that patients may be given to help manage their Crohn’s disease, and different medications work better for some patients than others. Extra treatments can be given for any symptoms that occur outside the digestive system, for example, joint pain. Abdominal pain or diarrhoea that persists despite maintenance therapy can be treated with antispasmodic and anti-diarrheal medications, respectively.⁷

Rarely, if a patient’s disease is severe, surgery may be discussed as an option for management. These operations are known as bowel resections.⁷ This depends on which areas of the digestive system are affected and whether any of them can be removed. This sometimes results in a patient being left with a temporary or permanent stoma bag. 

Managing paediatric crohn's disease

Extra considerations are made when children are diagnosed with Crohn’s disease to make sure that they are able to have a healthy, undisrupted childhood and adolescence.

Links have been found between depression in young people and increased disease activity, increased relapse rate and poorer treatment adherence. For this reason, it is imperative that young people diagnosed with Crohn’s disease are given extra emotional and psychological support.⁸

In young people with Crohn’s disease, it is especially important to monitor their growth throughout their childhood and adolescence. This can be done through the use of centile charts. Studies have shown that the best way to ensure constant growth in children with Crohn’s is to maintain remission from disease symptoms while eating a balanced diet containing lots of fibre and enough energy, vitamins and minerals.³

School life for children with Crohn’s presents challenges that other children may not be facing. Children with Crohn’s may miss school for appointments and hospital visits and risk falling behind in class. Despite this, a Swedish study found that at the end of compulsory education, the results of children with inflammatory bowel disease were not significantly lower than those children without chronic diseases.¹¹ This is positive, although it does not mean that children at school do not have to cope with fatigue, difficulty concentrating, comparison of themselves with other students and sometimes an inability to join in, for example, with certain sports.

Summary

In summary, paediatric Crohn’s disease affects more than just a child’s physical health. Psychological and social support is extremely important for those coming to terms with their diagnosis and can directly contribute to a lower risk of relapses. Furthermore, with prompt diagnosis and the right treatment, patients are more than capable of achieving a normal, healthy childhood and adolescence.