Patients' knowledge of pregnancy-related issues in inflammatory bowel disease and validation of a novel assessment tool ('CCPKnow').

BACKGROUND: Inflammatory bowel diseases (IBD) require complex therapeutic decisions and life choices concerning pregnancy, but little is known about patient's knowledge of IBD and its treatment before and during pregnancy. AIM: To develop a novel tool (Crohn's and Colitis Pregnancy Knowledge Score 'CCPKnow') to assess knowledge of pregnancy-related issues in IBD. The validated tool was then applied to determine knowledge in patients. METHOD: Discriminate ability of 'CCPKnow' was validated in four groups with different levels of IBD knowledge. Reliability and readability were tested by Cronbach-α and Flesch-Kencaid. Construct validity was subsequently assessed against general IBD knowledge (CCKnow) in 145 women with IBD. Associations between patient factors and knowledge were studied. RESULTS: Median CCPKnow scores differed significantly between the validation groups (P < 0.001). CCPKnow displayed excellent internal consistency, reliability (Cronbach-α 0.94), readability (reading age 9 years) and close correlation with CCKnow (Spearman's ρ 0.64; P < 0.001). Of 145 patients, 44.8% had poor, 27.6% adequate, 17.3% good and only 10.3% very good knowledge. Better knowledge was associated with Caucasian ethnicity, higher income, having a partner, having children, Crohn's and Colitis Association membership, longer disease duration and Crohn's disease. CONCLUSIONS: Crohn's and Colitis Pregnancy Knowledge Score, a novel knowledge assessment tool of pregnancy and IBD, demonstrated excellent test characteristics. We found that nearly half of the women with IBD had poor knowledge, identifying a pressing need for better education.

Review article: colorectal carcinoma and inflammatory bowel disease.

The risk of colorectal cancer for any patient with ulcerative colitis is estimated to be 2% after 10 years, 8% after 20 years and 18% after 30 years of disease. The relative risk of colorectal cancer in Crohn's colitis is approximately 5.6 and should raise the same concerns as in ulcerative colitis. Risk factors for colorectal cancer include disease duration, early onset, extensive disease, primary sclerosing cholangitis and a family history of sporadic colorectal cancer. All patients should have a review colonoscopy 8-10 years after diagnosis to establish the extent of the disease. Surveillance should begin 8-10 years after disease onset for pancolitis and 15-20 years after disease onset for left-sided disease. Regular surveillance is recommended, with a screening interval every 3 years in the second decade of disease and annually by the fourth decade. Random biopsies should be taken at regular intervals with attention paid to dysplasia-associated lesions or masses, irregular plaques, villiform elevations, ulcers and strictures. Dysplasia is recognized as a premalignant condition, but the likelihood of progression to cancer is difficult to predict. High-grade dysplasia, confirmed by two expert gastrointestinal pathologists, is a strong indication for colectomy, as is low-grade dysplasia, although the diagnosis of low-grade dysplasia is unreliable. Surveillance programmes indicate that the overall 5-year survival rate is higher in surveyed patients, although patients still present with Dukes C cancers or disseminated malignancy. Surveillance has huge socioeconomic implications. As surveillance is not 100% effective, alternative ways of reducing the cancer risk with chemopreventive agents, such as aminosalicylates, are being considered.

Review article: the data supporting a role for aminosalicylates in the chemoprevention of colorectal cancer in patients with inflammatory bowel disease.

The chemoprevention of colorectal cancer (CRC) with long-term sulfasalazine and 5-aminosalicyclic acid (5-ASA) treatment has been demonstrated through epidemiological and experimental studies, in patients with ulcerative colitis. In a large case-controlled study, there was a trend for long-term nonsteroidal anti-inflammatory consumption to be protective against CRC in patients with inflammatory bowel disease (IBD) (OR, 0.84). Sulfasalazine treatment over a 3-month period was shown to be protective (OR, 0.38), independently of disease activity. A longer-term study of sulfasalazine also revealed a relationship with treatment compliance, where 3% of compliant patients developed CRC compared with 31% of the noncompliers. Treatment with 5-ASA was associated with an increase in apoptosis, a decrease in proliferation of colorectal mucosa and has been shown to lower the rate of spontaneous mutation. In a retrospective, matched case-control study conducted in the UK, 102 patients with ulcerative colitis and CRC were matched with ulcerative colitis patients without CRC. Incidence was correlated to treatment protocols, family history and other risk factors. Mesalazine was the only treatment to be associated with a statistically significant reduction in the risk of developing cancer. Even after adjusting for variables, mesalazine at doses > 1.2 g / day reduced the risk of cancer by 81% (P = 0.006). Frequent visits to the clinician were also associated with a protective effect.

CARD15/NOD2 risk alleles in the development of Crohn's disease in the Australian population.

We have previously reported strong evidence for linkage between IBD1 and Crohn's disease (CD) in Australian Crohn's disease families. Three risk alleles for Crohn's disease, (Arg702Trp (C/T), Gly908Arg (G/C) and 980fs981 (-/C), were recently identified in the CARD15/NOD2 gene on chromosome 16, implicating this as the IBD1 locus. Using a novel diagnostic PCR-RFLP, we have examined the frequency of these alleles in 205 multiplex IBD families, 107 sporadic Crohn's disease cases and 409 normal individuals. We demonstrate that the three risk alleles are more frequent in Crohn's disease, than in controls, with allelic frequencies of 0.11, 0.02 and 0.07 respectively. Heterozygosity for individual variants conferred a three-fold increase in risk for Crohn's disease while substantially higher risks were associated with being homozygous or compound heterozygous. Despite a significantly lower population allele frequency for the frameshift mutation than reported by other groups, we see a similar contribution by this allele to the risk of developing Crohn's disease. While the three risk alleles influence susceptibility to Crohn's disease in Australia, we show that these alleles do not fully explain the linkage evidence and suggest that there are very likely additional IBD1 susceptibility alleles yet to be described in Australian CD at the NOD2 locus. We also show a second linkage peak in Australian CD that provides some support for a second disease susceptibility locus on chromosome 16.

Does patient knowledge affect the colorectal cancer risk in ulcerative colitis?

OBJECTIVES: Formal efforts to improve patient education are associated with fewer disease complications in a number of conditions. The possible relationship between knowledge about ulcerative colitis and its cancer risk, and the development of colorectal cancer using a previously developed and validated instrument-the Crohn's and colitis knowledge (CCKNOW) score-were investigated. METHODS: The 24 item CCKNOW questionnaire was mailed to patients known to have developed colorectal cancer as a complication of ulcerative colitis (cases) and to colitics from the Leicestershire inflammatory bowel disease patient database who had not developed cancer (controls). RESULTS: The mean (SD) CCKNOW scores for cases was 8.21 (3.02) and for controls was 8.27 (4.3). These scores did not differ significantly between cases and controls (difference 0.06, 95% confidence interval (CI) -1.7 to 1.5, p=0.9). There were four times as many members of the National Association of Crohn's and Colitis (NACC) in the control group compared with the cancer group and patients who are members of NACC achieve statistically significantly higher scores than non-members (11.6 v 7.8, p=0.05, 95% CI -0.1 to 7.6). However, after adjusting for NACC membership, the CCKNOW score did not appear to be associated with having developed cancer (odds ratio 1.04, 95% CI 0.92 to 1.18, p=0.5). CONCLUSIONS: The CCKNOW scores were comparable in cases and controls. Thus, in a retrospective study, no evidence has been demonstrated of an association between patient knowledge and the risk of developing colorectal cancer in patients with ulcerative colitis. However, knowledge may have been increased in cases as a direct result of having had colorectal cancer as a complication of ulcerative colitis.

Inter-observer variation between general and specialist gastrointestinal pathologists when grading dysplasia in ulcerative colitis.

Histological dysplasia is the cornerstone of colorectal cancer surveillance in ulcerative colitis (UC). Recently, pathologists have received unfavourable media attention concerning other cancer screening programmes. The aim of this study was to determine whether colonic biopsy specimens should be examined by gastrointestinal pathologists as opposed to generalists, by examining inter-observer variation between the two groups. Fifty-one coded slides showing varying degrees of dysplasia were mailed to seven gastrointestinal and six general histopathologists. Pathologists allocated each biopsy into one of four categories without the benefit of a clinical history or an opportunity to use the 'indefinite' category that is included in the Riddell classification. The responses were analysed using kappa statistics. The overall kappa statistic for gastrointestinal pathologists was 0.30 [95% confidence interval (CI)=0.26-0.34] and for general pathologists 0.28 (95% CI=0.23-0.32). Agreement was best for high-grade dysplasia (kappa of 0.54 and 0.61 for GI and general pathologists, respectively). There was total concordance of the 13 pathologists in only four of the 51 slides (7.8%) (95% CI=0.4-15.2%). It is concluded from these results that gastrointestinal pathologists are no better than generalists when grading dysplasia in UC and that agreement is poor in both groups. There is therefore no evidence that there would be any benefit in having specialist histopathology centres concentrating specifically on the interpretation of all surveillance colonoscopy biopsies from around the UK. It must be made clear to the public that surveillance and screening programmes carry a significant rate of histological error and that perfection cannot be expected or achieved with present methods.

Screening: the legal view.

Screening has become central to the effective prevention of several diseases, but implementation suffers from difficulties with targeting and rates of compliance. Such issues are also complicated by the need to consider legal provisions regarding confidentiality of patients and other human rights issues. Screening has been an inexact science in relation to, e.g., faecal occult blood testing for colorectal cancer, false positive and false negative tests for HIV, and there have been inadequate quality controls in breast cancer screening programmes. The public need to be made aware of what the screening programmes really offer, balanced against the expectations they may have. There needs to be a clearer understanding of the nature of the contractual and other legal rights of patients/consumers as against providers. A positive screening test may carry adverse consequences as well as benefits. It could alert an insurance company to a risk and lead to additional weighting or even outright rejection for life insurance policies. Job prospects may also be affected for employees. The method of informing patients in relation to screening and screening failure has already been considered by the courts. Realistic information about both screening and treatment efficiency needs to be offered to patients so that they can have a real understanding of what can and cannot be achieved by current science. The development of understanding of the human genome makes the need for clearer legislation in this are more urgent.

The risk of colorectal cancer in ulcerative colitis: a meta-analysis.

BACKGROUND AND AIMS: Controversy surrounds the risk of colorectal cancer (CRC) in ulcerative colitis (UC). Many studies have investigated this risk and reported widely varying rates. METHODS: A literature search using Medline with the explosion of references identified 194 studies. Of these, 116 met our inclusion criteria from which the number of patients and cancers detected could be extracted. Overall pooled estimates, with 95% confidence intervals (CI), of cancer prevalence and incidence were obtained using a random effects model on either the log odds or log incidence scale, as appropriate. RESULTS: The overall prevalence of CRC in any UC patient, based on 116 studies, was estimated to be 3.7% (95% CI 3.2-4.2%). Of the 116 studies, 41 reported colitis duration. From these the overall incidence rate was 3/1000 person years duration (pyd), (95% CI 2/1000 to 4/1000). The overall incidence rate for any child was 6/1000 pyd (95% CI 3/1000 to 13/1000). Of the 41 studies, 19 reported results stratified into 10 year intervals of disease duration. For the first 10 years the incidence rate was 2/1000 pyd (95% CI 1/1000 to 2/1000), for the second decade the incidence rate was estimated to be 7/1000 pyd (95% CI 4/1000 to 12/1000), and in the third decade the incidence rate was 12/1000 pyd (95% CI 7/1000 to 19/1000). These incidence rates corresponded to cumulative probabilities of 2% by 10 years, 8% by 20 years, and 18% by 30 years. The worldwide cancer incidence rates varied geographically, being 5/1000 pyd in the USA, 4/1000 pyd in the UK, and 2/1000 pyd in Scandinavia and other countries. Over time the cancer risk has increased since 1955 but this finding was not significant (p=0.8). CONCLUSIONS: Using new meta-analysis techniques we determined the risk of CRC in UC by decade of disease and defined the risk in pancolitics and children. We found a non-significant increase in risk over time and estimated how risk varies with geography.

How gastroenterologists screen for colonic cancer in ulcerative colitis: an analysis of performance.

BACKGROUND: The aim of this study was to assess the colorectal cancer surveillance practices of British gastroenterologists for patients with ulcerative colitis. METHODS: A questionnaire that investigated aspects of surveillance in patients with ulcerative colitis was mailed to all consultant gastroenterologists in the U.K. (n = 413). RESULTS: Three hundred forty-one questionnaires were returned (response rate 83%). Ninety-four percent of consultants practice cancer surveillance in ulcerative colitis, with 35% maintaining a registry of patients in surveillance programs. All gastroenterologists perform surveillance in patients with pancolitis, 24% in those with left-sided colitis and 2% in patients with proctitis. The mean duration of disease before surveillance is commenced is 9.2 years for pancolitis and 12.4 years for left-sided colitis (p < 0.0001). Only 4% of gastroenterologists routinely offer patients with disease of more than 10 years' duration a prophylactic colectomy. Colonoscopies are conducted by an accredited gastroenterologist in 65% of cases and biopsies are reviewed by specialists in gastrointestinal pathology in 45%. When histology reveals low-grade dysplasia only 4% advise colectomy and when high-grade dysplasia is found 53% recommend colectomy. Sixteen percent of gastroenterologists were unaware of the significance of a dysplasia associated lesion or mass. CONCLUSION: The majority of gastroenterologists practice surveillance on a disorganized basis. There is inconsistency in the management of patients with dysplasia and education of gastroenterologists is needed.

Colorectal cancer prevention in ulcerative colitis: a case-control study.

BACKGROUND: The risk of colorectal cancer (CRC) in ulcerative colitis (UC) increases with extent and duration of disease. Identifying other risk factors would allow targeting of sub-groups at greatest risk, enabling more cost-effective surveillance. METHODS: We conducted a case-control study comparing 102 cases of CRC in UC with matched controls. Odds ratios (OR) for cancer risk were estimated by conditional logistic regression. A multivariate model assessed the contribution of individual variables. RESULTS: Regular 5-aminosalicylic acid (5-ASA) therapy reduces cancer risk by 75% (OR 0.25, 95% CI: 0.13-0.48, P < 0.00001). Adjusting for other variables, taking mesalazine regularly reduces risk by 81% (OR 0.19, 95% CI: 0.06-0.61, P=0.006) and visiting a hospital doctor more than twice a year also reduces risk (OR 0.16, 95% CI: 0.04-0.60, P=0.007). Considering variables independently, having a family history of sporadic CRC in any relative increases risk fivefold (OR 5.0, 95% CI: 1.10-22.82, P < 0.04). CONCLUSIONS: CRC risk among UC patients can be reduced by regular therapy with 5-ASA medication. Colonoscopic surveillance may be best targeted on those unable to take 5-ASAs (e.g. due to allergy) and those with a positive family history of CRC.

The Crohn's and Colitis Knowledge Score: a test for measuring patient knowledge in inflammatory bowel disease.

OBJECTIVE: The aim of this study was to develop a valid and reliable questionnaire assessing patient knowledge of inflammatory bowel disease (IBD) and its treatment--the Crohn's and Colitis Knowledge (CCKNOW) Score. METHODS: A total of 30 multiple choice questions were constructed into a draft questionnaire. This was piloted on a random selection of participants with differing IBD knowledge levels; junior doctors, nurses, and ward clerks. Factor analysis eliminated questions with poor discriminant ability. The resulting 24-item questionnaire (CCKNOW score) was retested on the three groups, and a Kruskal-Wallis test determined the questionnaire's ability to discriminate between the groups. Reliability and readability were tested using Cronbach's a and the Flesch Kincaid reading score. The validated CCKNOW was then tested on patients from the Leicestershire IBD database. RESULTS: CCKNOW scores differed significantly across the groups of doctors, nurses, and ward clerks (median 22, 16, and five, respectively) T = 40.35, p < 0.0001. The reliability was very good with a Cronbach's alpha of 0.95 and the readability was also high. The median score on the CCKNOW for IBD patients was 10, with no significant difference between ulcerative colitis and Crohn's disease. Patients who are members of NACC (National Association of Crohn's and Colitis) achieve statistically significantly higher scores than do nonmembers (difference in medians 4, 95% confidence interval 4-6, p < 0.0001). CONCLUSIONS: The CCKNOW score provides a valuable index of overall knowledge. It is self-administered and psychometric tests show it to be valid, reliable, and readable. It may be used in the future as a tool to evaluate patient education programs.

Questionnaires: the use and abuse of social survey methods in medical research.

We present a working review of survey methods based on market research technology. The structure of questionnaires, their distribution and analysis, are considered, together with techniques for increasing response rates.

Social implications of childhood Crohn's disease.

BACKGROUND: Children with Crohn's disease may have an impaired quality of life. METHODS: A validated questionnaire was mailed to the parents of all child members of Crohn's in Childhood Research Association (CICRA) (age range, 5-17 years). It investigated aspects of the quality of life of children with Crohn's disease. Sixty-four questionnaires were returned together with another 35 from those outside the defined age range. Patients in the latter group were not included in the analysis. RESULTS: Of the children investigated, 60% had significant absences from school, with a mean of 3+/-2.8 months' absence in the previous year. Most (53%) thought that their teachers knew nothing about Crohn's disease, and 50% thought that teachers were unsympathetic. Of the 24 who had taken examinations 80% thought they had underachieved because of ill health. Sixty-seven percent of children were unable to participate in sports on a regular basis, 60% felt unable to leave the house, and 50% were unable to play with their friends. Information about Crohn's disease had predominantly come from doctors (89%), information booklets (83%), and nursing staff (42%). The commonest questions asked by the children were: what is Crohn's disease (50%), why me (64%), will it affect my schooling (52%), and will it affect my chances of getting a job (67%). CONCLUSION: Children with Crohn's disease have a significantly impaired quality of life. They fear everyday childhood activities and fear for their future. These children need sympathetic management, and effort should be concentrated on improving their day-to-day existence to enable them to lead as normal a life as possible.

Achalasia: a critical review of epidemiological studies.

Achalasia is one of the earliest recognized gastroenterological conditions. However, several centuries after it was first described, it remains also among the least understood. One of the main reasons for this is the relative rarity of the disease, which has resulted in limited opportunities to conduct investigative research. Few epidemiological studies have been conducted to date, and their data suggest a worldwide incidence estimated at between 0.03-1.1/10(5)/yr. This review of the literature on the epidemiology of achalasia lends support to the idea that pooling of resources and collaboration at an international level is required, if any significant progress in the cause, treatment, and prevention of the disease is to be made.

The role of complementary medicine in European and Asian patients with inflammatory bowel disease.

Three hundred and eighty-two patients with known inflammatory bowel disease (IBD) (190 European and 192 Asians) and 190 with coeliac disease were sent a previously validated questionnaire to investigate patients' use of alternative medicine and their views on its effectiveness. Details sought included whether they have ever consulted an alternative practitioner, whether they had followed a course of treatment and its clinical effects. Information about where patients had heard about such alternative practitioners and whether they were told to discontinue their current allopathic medication was sought. Results were analysed after three consecutive mailings, including one in Gujurati to Asian patients. A randomly selected group was re-interviewed four months later. To validate the study alternative medicine practitioners were also interviewed to investigate what percentage of their attendees have IBD and how many of those clients were Asians. One hundred and fifty-eight questionnaires were returned from European patients with IBD (response rate = 83%), 145 from patients with coeliac disease (response rate = 76%) but only 81 Asian patients with IBD (response rate = 42%). Forty-seven European and Asian patients with inflammatory bowel disease sought advice or treatment from an alternative practitioner, compared with only 11 with coeliac disease (chi(2) = 11.64, df = 12, P < 0.003). There was no significant difference in consultation rates between Asian and European patients with IBD (Yates corrected chi(2) = 0.78, ns). The most common practitioners consulted by all groups were homeopaths (n = 23) and herbalists (n = 27) but 20 patients consulted more than one practitioner at a time. Patients with coeliac disease and European patients with IBD had consulted osteopaths (n = 6) and reflexologists (n = 7). Ten patients with IBD had also attended a spiritualist and five Asian patients a hakim. Common sources of information about alternative remedies included friends and relatives (n = 13), the media (n = 11), word of mouth (n = 11) and family practitioners (n = 6). Most patients were advised to continue their current medications, although two had been told to stop and 10 advised to reduce the dose of their allopathic medications. Twenty alternative medicine practitioners stated that overall between 2-5% of their attendees have IBD with 10% of those clients being Asian. Asians preferred to consult Asian practitioners rather than European practitioners. There was no clear consensus as to whether complementary therapies were felt beneficial, although many patients with IBD believed them to be helpful.