More newbie advice please.....

Hi All

I am new to this site but have been lurking for a while and was after some advice on treatment for UC for my 12 year old daughter if anybody can help?

To cut a long story short she started passing blood in early December 2017 and got progressively worse with stomach pain and bloody loose stools with various visits to the GP/A&E before diagnosis of moderate to severe pan colitis at the end of January this year by a GI specialist. To put things in context although she did suffer she was lucky enough (if I can put it that way!) to suffer more with constipation and less with frequency and urgency. She was admitted to hospital on a Tuesday for IV steroids and by the time we left on the Friday she was completely symptom free and on 40mg of prednisolone with taper and 1g of oral Pentasa twice a day.

Fast forward to the end of March when we reached 10mg of prednisolone there again was a small amount of bright red blood after passing a fully formed stool, we then went back up to 20mg of pred, stabilized and began the taper again. Again we reached 10mg and yet again the bright red blood returned after a fully formed stool. After speaking to the doctor’s assistant she advised to go back up to 20mg of pred and wanted her to begin Imuran at the same time. Due to the harsh nature of this drug I am reluctant and we have agreed to discuss next week when we have a hospital appointment with her actual doctor as she is now on pred for the next four weeks anyway.

Could you possible help me with two questions:

The first is whether you would interpret this blood as being the initial flare not having been dealt with/cleared with steroids or whether you believe this to be the beginning of a second flare?

Secondly even though she has been diagnosed with pan colitis as this is bright red blood accompanied by no other symptoms (no stomach pains, loose stools etc) do you think it is fair to assume that at the beginning at least it is likely to be more rectal (I am in no doubt it would spread if left untreated)?

The reason I ask is that I am reluctant for her to move to Imuran unless it is absolutely necessary and want to discuss with the GI whether suppositories/enemas along with oral pentasa may be worth a try in the first instance? Though I appreciate rectal medication may not be nice for a 12 year old I would absolutely want to try it if there was a chance it would work before moving up!

Any advice/comments with your own experience would be appreciated. Obviously I know she can’t stay on pred too much longer and don’t want her to suffer at all if I can help it and if that means going for Imuran next week of course we will.

Many of us feel 100% normal on prednisone, but it is only a temporary rescue medication that buys us time while our other UC medications are given time to work. The goal is to taper off of pred without issue. However, what you describe (re-occurrence of blood at 10-mgs of pred) is a tale-tale sign of her maintenance medications being insufficient. What needs to happen is go back on a higher dose of pred (you did to 20mgs), adjust her maintenance medications, give that adjustment time to work, and then resume the pred taper again.

"Due to the harsh nature of this drug I am reluctant..." This type of thinking is going to make your daughter's experience with UC a lot more difficult than it otherwise needs to be. The fact is she is a moderate/severe pancolitis case (pancolitis being the largest extent of UC) and has been hospitalized, and those things together place your daughter in the highest-risk category of UC (of relapse, of further hospitalization, and of ultimately needing a surgery) and the biggest reason to go on the stronger medications earlier in order to control her UC fast, and get her UC into a remission (that is an absence of UC symptoms). I don't mean to scare you needlessly, but what I do mean to do is stress that you should not take her UC pancolitis lightly, and under-treat it as a consequence thereof. I'd follow your doctor's advice and go on the imuran, and likely ask about going on a biologic medication at the same time (remicade/humira/simponi).

The goal is to hit a UC flare hard, get the UC patient in a long, lasting remission (where she'll be essentially normal for years), and that will produce the best quality of life and the least cause for suffering. While, you could kick-the-can-down-the-road and try many smaller things that may/may not work and that could result in more lost school, many more months of struggling, and you do worry about impacts on growth-rate and other things of that nature.

Most USA gasteroenteroligists are strong proponents of using the top-down-approach especially with pediatric cases of UC, of using the stronger medications earlier, and that is what I am describing in the above. As they have to live with the consequences of UC the longest and you want their quality of life to be the best possible. Would you rather try and stop a fire when it is still a smoldering ember? Or would you rather wait until the entire house is engulfed in fire before trying your strongest treatments? Thinking is knock it hard, early, and fast, and move on with your life.

There's still the bottom-up approach of using the least amount of medications possible, which some adhere to. Just know there will be a lot more trial-and-error, a longer stay on pred, a gradual escalation of med doses and adding more meds, and it might ultimately end up with using the stronger meds anyways. It took me 2-years to achieve my initial remission with the bottom-up approach, and second, very severe flareup as a consequence thereof.

We all worry about starting new classes of medications. A good read is this by the Crohn's and Colitis Foundation of America, which discusses the risks versus benefits of using the stronger meds on UC patients. Nothing is without risk, but the benefit often far outweighs those risks. It's easy to fear when you don't know the odds, and often reassuring to see that risks are indeed much smaller than we initially believe them to be:

Webcast: programs.rmei.com/CCFA139VL/presentation/player.html
Transcript: http://www.ccfa.org/assets/pdfs/risk-and-benefits-transcript.pdf

Is that all she's taking? As that's certifiably-crazy for a moderate/severe pancolitis case....

For a UC flareup, a minimum of 4,000-mgs oral pentasa per day should be used. A rectal-route mesalamine should be used, ideally 4,000-mgs of a pentasa enema. Even in pancolitis cases, enemas help as the very worst of our inflammation that causes our very worst symptoms (urgent bms, tenesmus, frequent bms, etc) originates from the rectum. Pills are inconsistent and inadequate at treating the rectum. Rectal-route goes straight to the source. She'll heal the fastest and have the best results using both routes simultaneously.

You can ask her gasteroenterolgist doctor about upping the pentasa dosage, and adding a nightly pentasa/salofalk/rowasa retention enema, wait a few weeks (at least 2, maybe 3 weeks) and reassess given what she's taking. After which add the imuran if nothing has improved. Just know imuran is going to take about 3-months to kick in, during which time you are going to use prednisone as a bridge. A biologic (humira/remicade) would kick-in, in about 3-6 weeks time in comparison (much, much faster if she's suffering) and would be something I'd inquire about if she's pretty bad off.

And know that UC medications are usually additive (no single medication is our 100% solution, rather a combination of medications is), so you don't stop one medication and replace it with another med. Rather you take multiple meds simultaneously until a remission is achieved. After you've been in a remission for 2-months then cutting back meds makes a lot of sense (as it takes more meds to achieve a remission rather than sustain it).

Just know your options, discuss with your doctor (and daughter if she's old enough), and come up with the solution you all agree is best. UC pancolitis for patients who've been hospitalized can be difficult, so don't be afraid to treat aggressively if it acts aggressively and does NOT respond to mesalamine alone.

As a FYI, I am on 6MP (a sister medication to imuran/azathioprine) and have not been sick any more than usual. We have posters on those meds who work in medical offices, are kindergarden teachers, and others who interact with lots of people (many prone to being sick) on a daily basis. So, it is possible to be on Imuran and not be constantly sick. Good handwashing protocol helps as most diseases are spread via touching a commonly touched object and then touching our own faces!

There is lots of good advice above, here are a few other tips from my own personal experience.

Pancolitis is serious and I’d echo the others comments to hit it hard fast. Prednisone is a great rescue drug and bridge to long term treatment plan but not a long term option. Tapering can be a little rough with fatigue and joint pains. Those are good questions to ask the doc, to avoid confusing symptoms as you get a little farther down the road with this.

Mesalamine enemas can work wonders, but they only have a limited “range” they can treat.

Imuran - I’ve been in varying doses of this, on and off since diagnosis. It’s been an effective tool in the toolkit for me. I’ve found that if I take it at night, I sleep thru the side effects I deal with (nausea and fatigue). Those side effects really only bother me the first few months anyway. Expect frequent lab work to monitor while on imuran. Frequency varies by GI, and typically starts to space out as results are normal. There is a metabolite test that can be ordered to check levels to determine if therapeutic range. I use basic precautions (e.g. hand washing, avoid contact with really sick people, replace kitchen towels with clean one daily,etc). I don’t get sick more often.

Hang in there! You’re asking the right questions!

I am not a youngster like your daughter, but I was hospitalized with my initial flare and diagnosed with pancolitis. I am coming off of prednisone right now after almost 5 months due to the lower drugs no longer working and needing a bridge for biologics. Prednisone, on my opinion, is worse than something like Imuran. I am/was otherwise healthy, but the prednisone has given me osteoperosis in my spine and has elevated my blood sugar, along with a host of other less damaging side effects. The reason I am sharing this is that I wish the process of starting a Biologic had moved more quickly for me. Rectal meds can help bridge too, with a lot less side effects. I say bridge, but they can continue to help the lower part. In answer to your question about initial flare vs new, if she never successfully came off of prednisone, it is still the initial flare. She may have healed some, but it's hard to say where she will be back at once off of Prednisone.

First, going slower in a taper doesn't prevent you from flaring again. You either flare or do not as you taper off of the pred.

Steroids, like prednisone, are some of the most effective medications we have for UC: fast acting and have the highest response rate out there. However, steroids are only meant for temporary usage due to irreversible side effects from long-term usage. Pred gives us quick relief from the worst of UC symptoms while we add new meds or increase dosage of other medications (like pentasa) and give those meds time to work. Pred is like a crutch that enables you to heal, and you can either walk without a crutch or still need a crutch to get around.

If we're properly dosed with pentasa and other maintenance meds, then a taper off of prednisone is completely uneventful and you kick the pred without seeing blood or any other UC symptoms return.

If we are NOT properly dosed with appropriate maintenance meds then the taper off of prednisone results in a return to a flare-state. There's a bit of trial-and-error in treating us all, sometimes the 1st, 2nd, or even 3rd maintenance meds and dosages isn't enough. Generally, 4.0grams of oral pentasa, and 4.0grams of enema are recommended daily for an adult. Pediatric dosages would be similar, I'd defer to your doctor on that. I'd ask is she on the maximum pentasa oral and rectal for her age and weight. That'll give you the quickest and best possible results .

Steroid-dependent is a bit of a misnomer, nobody's addicted like with opiods or nicotine. It means what you know it to mean. To sustain her quality of life it is necessary to be on steroids, there's no addiction. I've been steroid-dependent a couple times and always found my way off of pred by adding more meds or adjusting dosages.

I think she's undertreated. With moderate/severe, it really needs to be treated on the heavy side. I don't think imuran is a bad suggestion, but as the others mentioned, I would first ask about whether she can increase her mesalamine, both orally and rectally. I also know that pentasa isn't usually the best mesalamine for UC.

Can she try Asacol or Lialda? They release lower.
Apriso® - Eudragit-L (pH6) coating of 5-ASA-polymer coated beads, small bowel and colonic release
Asacol® - Eudragit-S coating that releases at pH7, ileocolonic release - replaced with delzicol.
Asacol-HD® - Eudragit-S and Eudragit-L coating, ileocolonic release
Canasa® suppositories – topical rectal release
Colazal® - diazo bond to inert polymer, colonic release
Delzicol®
Lialda® - Eudragit-S coating of metallomatrix 5-ASA, ileocolonic release
Olsalazine (Dipentum®) - diazo bond of two 5-ASAs, colonic release
Pentasa® - ethylcellulose/5-ASA beads, small bowel and colonic release
Rowasa®, sfRowasa®, or Melamine Rectal Suspension (generic) enemas – topical rectosigmoid releases (sfRowasa is sulfate free for those with sulfa allergies)
Sulfasalazine (generic) – diazo bond with sulfapyradine, colonic release

I know I wouldn't want to have her take drugs if I were in your shoes, so I get it, and I wonder about adding a steroid enema, or possibly trying to use something like uceris to get her off the systemic steroids. The problem is that you will still need a good treatment plan to keep her stable once she's off. Too often we try to go with the minimum and we end up back at square one.

Has she had stool tests?
What did the pathology show?
How was she diagnosed?

Thank you both for your replies, everything is so complicated with this disease! We had a stool sample taken last week but it will take two weeks for the results, but what would you expect that to show while she is on steroids? Would it show anything? As I mention she is completely symptom free right now so would it pick up inflammation? Her bloods and are fine, she is low on iron but I would expect that and I know it takes forever to go back up, and at the risk of TMI she missed her periods between Feb and April which I assume was just because she was ill - they have come back so I feel like her body is starting to repair. I do feel like she should have more as although she’s 12 she is for all intents and purposes she is very much adult sized! I have me mentioned it to my IBD nurse but I feel like I am annoying them and they have only mention Azathioprine as an alternative, I feel like she may be ok On Mesalazine as her symptoms were only really bad after about 3 months of not knowing what constipation and tummy aches were! She has pancolitis but the scope only went just into the transverse (?) due to poor bowel prep. Biopsies were taken but they weren’t back when we discharged so assume they were as expected as I haven’t been told they weren’t?!

Two weeks for a stool test is a long time. Considering her symptoms are not worrisome at the moment, it's quite possible the stool test will show nothing of significance.

Do you know the extent of her symptoms based on her telling you? I know when I was suffering with UC, I would often minimize my symptoms when talking to other people, even doctors. I do not know why I did this, but i was always quick to say "things aren't too bad". Please be aware that people do this. I guess I don't like to be fussed over, and I didn't like accepting that I was different, and the easiest, quickest thing was to say OK and get out of there!!

Stool tests can show inflammation, or they can find pathogens. With a pathogen you would need an antibiotic to improve and she's improved so much with steroids... You never know though. Pathogens can make symptoms similar to UC and they can make it seem like your UC is not responding. Her's is, so it's not likely.

Do not feel like you're annoying. It's their job to respond to you, and it's normal that you're worried and you want her to get better. I would ask about the missed periods, but it's probably to do with the prednisone.

Biopsies can take a few weeks, but they will give you a more detailed account of what's going on. It will tell you what's inflamed, how severe, and whether it looks consistent with IBD. Right now, the diagnosis is going based on the GI visually looking at the surface - which isn't the most accurate. Might not be a bad idea to get an upper at some point so she can see beyond the transverse colon since she's dealt with constipation in addition to bleeding. Did the doctor who did the scope tell you where it was inflamed? They should have been able to tell you what it looked like in the rectum vs left side based on what they saw...

I used to always hope for a pathogen for myself personally. Then maybe an antibiotics will wipe it out forever. I will hope for this for your daughter.