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8/20/2017

IBD-Rough road: case study in IBD from presentation to management

IBD-Rough road: case study in IBD from presentation to management

Quality of life and a role in decision making must be a priority for patients with inflammatory bowel disease, writes Niamh Hogan

Inflammatory bowel disease can be a debilitating and embarrassing illness. Patients often find it difficult to talk about their condition and symptoms. Due to the severity of the symptoms associated with IBD patients may be absent from school and work for long periods. Unfortunately the symptoms and sometimes the treatments used to manage the condition may cause side-effects such as growth retardation in children and delayed puberty in adolescents. Medical treatments can cause other problems and surgery may also result in long-term complications.

This article will illustrate a patient’s journey through presentation, diagnosis and medical management. The patient described is genuine but details have been changed to protect anonymity.

Sinead’s story Sinead, a 17-year-old Leaving Cert student living at home with her mother and 26-year-old older brother, presented to A&E complaining of a six-month history of anorexia, weight loss, right-sided abdominal pain, diarrhoea associated with urgency and fatigue. She also complained of a perianal pain and had noticed a continuous drainage of muco-purulent fluid from a tiny sinus around her back passage. She described the symptoms as getting progressively worse over the last two months but had felt this was due to exam stress. Her mother stated that Sinead would often get up to go the toilet three to four times at night and was always in the bathroom early in the morning. Sinead said the abdominal pain was very severe with no relieving factors and associated with very loud gurgling noises.

She was seen and examined by the A&E doctor. He found tenderness and guarding on the right side in particular. Sinead thought she could feel a ‘lump’ on her right side and the doctor found a palpable mass on examination. She appeared pale, tired looking and had a low body mass index. She had a pyrexia of 38.4C and was tachycardic. Sinead said she noticed her clothes were ‘baggy-looking’ on her recently. Her mother said her older brother Jeff has Crohn’s disease and had presented in a similar way.

Sinead was then seen by the gastroenterology team and they ordered baseline bloods of a full blood count (FBC), urea and electrolytes (U&E), Albumin, erthrocyte sedimentation rate (ESR) and c-reactive protein (CRP). Stool samples were sent for microbiological testing for infectious diarrhoea including clostridium difficile toxin. They also ordered a small bowel follow through and an urgent colonoscopy to assess both the large and small intestine. In view of her clinical symptoms and family history a provisional diagnosis of inflammatory bowel disease (IBD) was made.

IBD The cause of IBD remains unknown although some theories suggest there may be a genetic predisposition. Other factors thought to influence the cause of IBD include environmental factors, immunological dysfunction or an infective influence.

Ulcerative Colitis The diagnosis of which type of IBD (ulcerative colitis or Crohn’s disease) a patient has may be difficult to determine. Ulcerative colitis (UC) is characterised by diffuse mucosal inflammation limited to the large intestine. The extent of disease is divided into distal colitis which affects the rectum (proctitis) or rectum and sigmoid (rectosigmoiditis). UC may be more extensive than this and extend to the left side of the colon (up as far as the splenic flexure) or further on to the hepatic flexure. Colitis which involves the entire large colon is known as pancolitis. The predominant symptoms of UC are loose bloody diarrhoea and colicky abdominal pain, often associated with tenesmus and urgency. The disease always involves the rectum and is characterised by relapses and remission. The patient with UC will not have any perianal disease as only the mucosa is affected.

8/20/2017

IBD-Rough road: case study in IBD from presentation to management

Crohn’s disease Crohn’s disease may affect any part of the gastrointestinal tract from the mouth to the anus. It is characterised as patchy, transmural inflammation. The mucosa may demonstrate a cobblestone appearance in severe ulceration and the bowel may show areas of inflammation interspersed with areas of healthy bowel described as skip lesions. It may present at any age but usually presents between the age of 15-40 years and affects males or females. It is twice as common in smokers as non-smokers; stopping smoking reduces the risk of relapse, need for immunosuppression and surgery. Clinically patients may present with symptoms of anorexia, malaise, fever, tachycardia, weight loss, abdominal pain and diarrhoea. Diagnosis can be difficult to make as other conditions may also cause these symptoms. Therefore a combination of endoscopic, radiological and blood test are needed to confirm the diagnosis.

The condition is also classified according to site, extent, and pattern of disease. This then influences the medical management, likelihood of surgery and prognosis.

Where the disease presents also affects how the patient will present. In 2004 the British Society of Gastroenterology (BSG) developed guidelines to assist healthcare professionals on the management of the patient with IBD. They stated the general principles to consider are:

The site (ileal, ileocolic, colonic, other)IBD. They stated the general principles to consider are: The pattern (inflammatory, structuring, fistulising) The

The pattern (inflammatory, structuring, fistulising)to consider are: The site (ileal, ileocolic, colonic, other) The activity of the disease before any

The activity of the disease before any decisions on treatment are made.other) The pattern (inflammatory, structuring, fistulising) They believe the patient must be involved in the decision

They believe the patient must be involved in the decision making process.

Investigations Sinead’s blood results were as follows:

FBC

Platelets: 650,

WCC:18.1,

Hb 10.1g/dl

U&E

Normal limits

ESR

65

LFTs

Normal limits

CRP

105

Albumin

28

According to Rampton and Shanahan, in patients presenting with abdominal pain and/or diarrhoea, test results revealing anaemia, raised platelet count and raised ESR may suggest active IBD, but are not diagnostic. 1 Other investigations must be completed before confirming a diagnosis. Also a raised CRP and low serum albumin levels may also suggest active disease, but confirmation needs to be achieved with other investigations first.

The following morning Sinead had a colonoscopy. In order to visualise the ileum a colonoscopy with a terminal ileoscopy was performed. This is central to the macroscopic and microscopic diagnosis of Crohn’s disease.

There was evidence of patchy erythema and a small fistula in the rectum. The remainder of the large colon was normal. There was also evidence of ulceration in the terminal ileum. Several biopsies were taken.

8/20/2017

IBD-Rough road: case study in IBD from presentation to management

Medical management Medical management of Crohn’s disease is aimed at relieving symptoms, reducing inflammation and minimising the risk of complications. Current therapeutic guidelines advise a top-up approach. This involves

starting with aminosalicylate (known as 5-ASA’s) drugs such as mesalazine. These are of great value in UC but

are not as effective in the maintenance of remission in Crohn’s disease. In combination with the 5-ASA’s a slowly reducing course of high-dose oral steroids are also given in an acute relapse.

In patients who require more than two courses of steroids in a 12-month period it is generally advisable that

they start an immunomodulatory drug such as azathioprine or mercaptopurine. These drugs are steroid-sparing agents and are largely used for modifying the pattern of the course of the disease.

Antibiotics such as metronidazole and ciprofloxacin may be beneficial for the treatment of fistulising Crohn’s disease.

The biologic drugs such as infliximab and adalimumab were launched in 1998 in the US and in Europe in 1999. These drugs, also known as anti-TNF alpha agents, act by binding to TNF alpha, reducing the need for corticosteroids and maintaining fistula healing is achieved in 70% of patients.

Infliximab is given in the hospital setting by an infusion. The patient initially receives infusions at week zero, two and six and will then go on a maintenance regime every eight weeks. The dose of the drug is 5mg/kg.

Adalimumab 40mgs is a subcutaneous injection that the patient self injects at home every two weeks. They also receive a loading dose on induction of160mg (four injections) or 80mg (two injections) at week zero, 80mg (two injections) at week two and then 40mg (one injection) fortnightly thereafter.

Before starting anti-TNF treatments the patient must be screened for latent TB as these drugs may reactivate latent TB. They should also be screened for the following:

Hepatitis screenlatent TB. They should also be screened for the following: Varicella antibodies History of multiple sclerosis,

Varicella antibodiesshould also be screened for the following: Hepatitis screen History of multiple sclerosis, malignancy, congested cardiac

History of multiple sclerosis, malignancy, congested cardiac failurefor the following: Hepatitis screen Varicella antibodies Abscess and infection It is not recommended for use

Abscess and infectionof multiple sclerosis, malignancy, congested cardiac failure It is not recommended for use in patients with

It is not recommended for use in patients with intestinal strictures.malignancy, congested cardiac failure Abscess and infection A Cochrane review was conducted in 2008 to review

A Cochrane review was conducted in 2008 to review the evidence for the effectiveness of TNF-alpha blocking

agents in the maintenance of remission in Crohn’s disease. 2

The review found that infliximab 5mg/ kg is effective for the maintenance of remission and fistula healing in patients who have responded to the initial induction therapy.

In

Sinead’s case she was treated with IV hydrocortisone 100mg six-hourly for five days and given five days of

IV

antibiotics, metronidazole 500mg TDS and ciprofloxacin 500mg BD. She had begun to improve but the

fistula did not show any signs of healing and while the inflammatory mass on the right side had abated the abdominal pain and diarrhoea remained. A decision was made to give Sinead ‘top-down’ therapy. The top down approach of introducing a biologic agent such as infliximab in combination with immunomodulatory drugs early in the medical management of the patient slows down disease progression and alters its natural

history. 3

Sinead was given her first infusion in hospital and attended the infusion centre for her second and third treatment. Six weeks after discharge from hospital Sinead was seen in the outpatients department. Her blood results were as follows:

FBC

Platelets: 405,

WCC:12.1

Hb 11.1g/dl

8/20/2017

U&E

Normal limits

ESR

5

LFTs

Normal limits

CRP

>3

Albumin

32

IBD-Rough road: case study in IBD from presentation to management

She described her bowels as opening twice a day, semi-solid stool consistency, with no visible blood in the stool, with no urgency or nocturnal symptoms. She had gained 6kg and was feeling much better. The perianal fistula had closed and she had no further episodes of abdominal pain. Sinead had managed to complete her mock leaving cert exams and was flare-free during that time. Her energy levels had improved as did her appetite. The consultant gastroenterologist decided to keep Sinead on maintenance Infliximab to prevent disease recurrence.

Treatments are aimed at optimising medical management and minimising the risk of complications. Up to 80% of patients will require surgery at some stage during the life time of their disease. Newer medical therapies such as Infliximab may result in rapid mucosal healing and is associated with fewer hospitalisations and surgical interventions. The patient must be involved in the decision making process from the outset. They must have rapid access to specialist support in the event of a flare. Their quality of life must be the number one priority for all involved in their care.

Niamh Hogan is a clinical nurse specialist in gastroenterology in the MWRH, Nenagh

References

1. Rampton DS, Shanahan F. Fast Facts: Inflammatory Bowel Disease 2nd Ed Health Press. 2006

2. Behm BW, Bickston SJ. Cochrane database systemic review Jan 23:(1) CD006893. 2008

3. Vermeire S, va Assche G, Rutgeerts P. Review Article: Altering the history of Crohn’s disease- evidence for and against current therapies. Ailmentary Pharmacology & Therapeutics Jan. 2007: 1;25(1) 3-12

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