Malignancy and Mortality in Pediatric Patients with Inflammatory Bowel Disease: A Multinational Study from the Porto Pediatric IBD Group

[kiny]

http://journals.lww.com/ibdjournal/Abstract/2014/02000/Malignancy_and_Mortality_in_Pediatric_Patients.11.aspx

Malignancy and Mortality in Pediatric Patients with Inflammatory Bowel Disease: A Multinational Study from the Porto Pediatric IBD Group

Feb 2014

Background:

The combination of the severity of pediatric-onset inflammatory bowel disease (IBD) phenotypes and the need for intense medical treatment may increase the risk of malignancy and mortality, but evidence regarding the extent of the problem is scarce. Therefore, the Porto Pediatric IBD working group of ESPGHAN conducted a multinational-based survey of cancer and mortality in pediatric IBD.

Methods:

A survey among pediatric gastroenterologists of 20 European countries and Israel on cancer and/or mortality in the pediatric patient population with IBD was undertaken. One representative from each country repeatedly contacted all pediatric gastroenterologists from each country for reporting retrospectively cancer and/or mortality of pediatric patients with IBD after IBD onset, during 2006–2011.

Results:

We identified 18 cases of cancers and/or 31 deaths in 44 children (26 males) who were diagnosed with IBD (ulcerative colitis, n = 21) at a median age of 10.0 years (inter quartile range, 3.0–14.0). Causes of mortality were infectious (n = 14), cancer (n = 5), uncontrolled disease activity of IBD (n = 4), procedure-related (n = 3), other non-IBD related diseases (n = 3), and unknown (n = 2). The most common malignancies were hematopoietic tumors (n = 11), of which 3 were hepatosplenic T-cell lymphoma and 3 Ebstein–Barr virus–associated lymphomas.

Conclusions:

Cancer and mortality in pediatric IBD are rare, but cumulative rates are not insignificant. Mortality is primarily related to infections, particularly in patients with 2 or more immunosuppressive agents, followed by cancer and uncontrolled disease. At least 6 lymphomas were likely treatment-associated by virtue of their phenotype.

 

[kiny]

I never agreed with those GI who invented the top-down approach without any studies backing them up that this was a good idea. The significant amount of children dying from the medicaiton instead of the disease should force those people to reconsider their opinion.

 

[HelenMelb]

I agree with you. There is little real evidence that 'top down' improves long term outcomes-essentially it is only a theory at this stage. We have seen some excellent doctors who mean well, but seem oblivious to the very significant risks of their recommended treatments. I have no doubt that some of these drugs have provided some great outcomes for some people, but it is a decision that needs to be made with a cool head after ALL the relevant facts are considered. Kiny, I really appreciate your very informative posts.

 

[my little penguin]

The problem is most kids with Ibd have a more severe disease course than adults in general and have the disease for much longer . Therefore regardless of top down or step up most kids end up needing to get on the stronger drugs to keep the disease at bay since under treated crohn's can be just as deadly . It's just what is for now .

Add in that most Ibd drugs were not designed for kids to begin with .
Simply put there are not enough good Ibd drugs let alone ones that should be taken or have been proven in children.

I have not met a parent yet of an Ibd kid who takes any of these medication choices lightly without doing a lot of research and soul searching to come up with the Best plan for their kid.
There are just no good answers .

 

[HelenMelb]

I agree with you MLP-there are no easy answers. My niece was 12 when diagnosed and every decision has been a difficult one.

 

[copeland]

Conclusions:

Cancer and mortality in pediatric IBD are rare, but cumulative rates are not insignificant. Mortality is primarily related to infections, particularly in patients with 2 or more immunosuppressive agents, followed by cancer and uncontrolled disease. At least 6 lymphomas were likely treatment-associated by virtue of their phenotype.

This is significant and kind of scary for me. I had active disease at 16, and was diagnosed at 17. Top-down pretty much defines the way I was treated (straight to Remicade).