collagenous sprue Archives

Refractory Celiac Disease: A New Diagnosis

Recently I went through a few diagnostic procedures to see what the heck is going on in my guts since they have been quite tempestuous over the past year. Once again landing me in the hospital a number of times. Has something new developed? I had the usual colonoscopy and endoscopy in addition to a capsule endoscopy where you swallow a vitamin-size camera (a very large vitamin, I might add!). The camera spends the next several hours taking pictures as it moves through your intestinal tract. My nurse told me it takes roughly two pictures per second. That’s a lot of selfies!

The results were similar but different. Thankfully, the microscopic colitis is in remission – my colon looks healthy. The inflammation remains in the small intestine with the pathologist’s report results showing that I have refractory celiac disease – or refractory sprue and not collagenous sprue. The difference in part having to something to do with the thickness of the mucosal lining in the intestines.

What Is Refractory Celiac Disease?

So much has yet to be learned about all diseases of the bowels, but being so rare, even less is known about refractory celiac disease (RCD). You can read about some of the medical details and differences between refractory and collagenous sprue and celiac disease here at The Archives of Pathology and Laboratory Medicine but how I see it is that it’s celiac disease on overdrive.

It is a complicated autoimmune disorder, similar to celiac disease, but does not respond to a minimum of six months on a gluten-free diet. The villi in your intestines deteriorate (mine are apparently blunted) and are unable to absorb nutrients and fluids from food. Hence the malabsorption – aka: diarrhea. With RCD the small intestine does not repair and damage continues despite maintaining a gluten-free diet.

There are two types of RCD – Type 2 the more aggressive and difficult to manage can lead to enteropathy-associated T-Cell lymphoma. That sounds frightening – and it is – it does not have a good prognosis.

Somewhere along the line, I have gathered the information that some researchers consider RCD to be a complication of poorly controlled celiac disease and yet others find that the disorder has its own particular features. (I have recently read so many reports and cannot remember or am unable to find the source of said information so please do your own research too.) Research has also found that the bulk of onset of the disease is in your 50s and the incidence in women is higher than in men. Yay me! Not a criteria I really wanted to fit in to.

What next?

For me, the new diagnosis is a somewhat ‘six of this and half a dozen of the other’. The symptoms are the same, the prognosis is similar and the treatment is the same. The fact remains that I am not able to tolerate or am allergic to the therapies that are known to work. Other than Prednisone as I’ve mentioned in a previous post.

Still, I am not disheartened. Over the years my blood tests have always come back negative for celiac disease. Therefore, this new diagnosis of RCD did come as a bit of a surprise, but it may be a bit of a silver lining. Well, that’s how I choose to look at it anyway.

In searching for information on RCD, I came across a vaccine that has been developed and in clinical trials for the treatment of celiac disease – Nexvax2. The vaccine works for patients that carry the HLA-DQ2 gene which most celiac patients have. Beginning with small and then gradually increasing doses, the vaccine is meant to help the body build up a tolerance to gluten.

In this case, I’m happy to fit the criteria! Along with the aforementioned endoscopies and colonoscopy, I was tested to see if I carry the HLA-DQ2 gene and I do.

So for now, I’ll keep on as I am and excited and hopeful for new therapies in the future.

Food Anxiety: What Can I Eat?

It used to be that I didn’t need to consider what I could or couldn’t eat, aside of course, from those times when I chose to go on a diet. I was hungry, I purchased and/or prepared food, and I ate food. And for the most part, without any adverse effects. Pretty simple. Now though, what I consume is of constant concern, always wondering if what I eat will aggravate my insides.

Hoping to heal my digestive tract by changing my diet, I began to investigate what might work. There is so much information out there, it’s fantastic and at times overwhelming. My food quest had begun!

The first step was removing gluten, followed by learning about the Paleo diet, I eliminated all grains too. I could also no longer eat eggs unless of course, I wanted to sprint to the toilet. Not the most pleasant way to get your cardio in. Coffee suddenly had the same and almost immediate effect. I discovered too through my naturopath dairy was no longer a friend to me.

These are foods that I have enjoyed throughout my life and now they are causing me pain and discomfort. Gluten, dairy, and eggs are on the shortlist of what my body has become sensitive to. There are so many other foods also – it is daunting.

So much to give up! It was difficult to switch my thinking from what I can’t have to what I can have. I’m still working on that by the way! There are moments when I crave an old favourite. Yes, there are so many recipes now that are Paleo, vegan, gluten-free and they’re delicious – of this I am thankful – but…

…let’s just say, I’m still adjusting.

Over the last handful of years, this food quest has also led me to try the Specific Carbohydrate Diet, Total Elimination Diet, FODMAP, and Food Combining Diet. These dietary changes bring about relief and remission for some people diagnosed with Crohn’s, ulcerative colitis and celiac disease. Unfortunately not the case for me.

I am in a perpetual flare up regardless of what I eat. Removing dairy, eggs, and gluten has reduced the urgency and number of sprints to the toilet in a given day but it has not healed my digestive tract enough to be able to get through the day without the aid of medications. I found too being completely grain-free made things worse so I have reintroduced rice into my life.

I feel somehow that I have failed, which of course, adds to the anxiety wrapped around food. Sometimes, I’m just afraid to eat. Or perhaps it’s not so much failing but coming to the realization that despite all the information available and my best efforts, I have little if any control over this disease.

I am aware that food is but one of many aspects to any gastrointestinal disorder, so I do let myself off the hook for not being able to fix this most of the time. So little is known still about the causes of inflammatory bowel disease let alone how to cure it.

But they’re working on it! According to Guilia Enders, author of Gut-The Inside Story of Our Body’s Most Underrated Organ in recent decades so much knowledge has been accumulated about our microbiome, the trillions of bacteria that live in our intestines and how they impact the connections between the brain and the gut. These microorganisms can affect how we feel and how we feel can affect our microbiome.

She goes on to explain each of us has an individualized microbiome – a unique fingerprint so to speak, depending on if you were breastfed, were born by cesarean or vaginal birth, have taken antibiotics, ate dirt as a toddler, had pets growing up… hence one diet does not fit all, nor can it cure-all I would think.

So I will let myself off the hook a little more for not being able to put this collagenous sprue into remission through lifestyle and dietary adjustments. That doesn’t mean though that I will give up trying! I have learned much through trying these diets, about what works for me personally and I do feel better in general through the dietary changes I have made.

At this point, I feel I need to chill about food, try to not become quite so anxious about ‘doing it right’ with regards to healing my body through what I consume. It’s a part of the puzzle, but not the entire picture. After all, stress does exacerbate things too.

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Corticosteriods: They Giveth and They Taketh Away

I have a love/hate relationship with this medication. Like so many things in life, Prednisone has its good aspects and then there’s the not so good. I have had to learn to live with them both since, at this point, I cannot tolerate other medications known to be effective with my particular type of inflammatory bowel disease, or the medications simply don’t work.

Let’s begin with the giving part of it and my favourite. It’s keeping me alive! That’s a big one.

I held off trying Prednisone for a number of years after my lymphocytic colitis diagnosis, being concerned about all the side effects. Instead, I began making changes to my diet, hoping to heal with food. Results with this were minimal but more on that another time. Loperamide was my go-to for getting my gut to behave (relatively speaking) during these years. It was not until diarrhea suddenly became acute; uncontrollable and lasted for weeks landing me in the hospital that I agreed to the Prednisone.

Once I started, I wondered why I had held off for so long. The first days, even weeks, were very giving and wonderful. I felt downright euphoric at times and was given a surge in focus and energy like I never have before. Oh, what I felt I could accomplish in those days! The energy boost is great especially on the tail end of being drained from weeks of literally being drained but it can make you at times, manic.

On the flip side or ‘taketh away’ of all this energy is not being able to sleep. Insomnia is a chronic problem for me even now after my body has adjusted to the drug. The first few days are always the worst. I sometimes slept as little as 15 mins in a night and couldn’t nap during the day either. You are left feeling wired and tired.

The sleep disruption I’m sure adds to another ‘giving’ nature of corticosteroids; mood swings. So much fun, not only for yourself but those around you. Some people can also become quite aggressive. I’m generally easy going but I have noticed I’m a little less patient in traffic. Ok, ok, there was that one time where my hand stayed on the horn a little longer than it should have. Oops, sorry.

Corticosteroids can give in so many other ways too. There are the puffy hands, feet and the ever wonderful, puffy ‘moonface’. You can also be given a puffy stomach that has entirely nothing to do with weight gain and one heckuva a voracious appetite, which has everything to do with weight gain. Oh my, the appetite! It is endless at times it seems, and the foods you crave tend to be on the sweet side. Not a good combination.

It can also give you thinner skin and even cataracts too! I’m already up against both of those given my middle age. This is not helping the situation.

Taking away, Prednisone depletes your calcium and your potassium that leads to osteoporosis. My bone density has decreased and I am one inch shorter than I was before all this began. Not to give steroids all the glory, since bone loss is also a result of the malabsorption caused by collagenous sprue or any other malabsorption disorder.

The strength in your muscles decreases as well. With long term daily use your muscles can waste away. Basically, with the puffy face, feet, hands, tummy and diminished muscles, I will look like the stick figures I used to draw as a child. Wait… I still draw like that!

There was something else it takes away, just can’t quite place it… Ah yes, memory loss.

That’s about it, I guess with regards to the giving and taking nature of corticosteroids for me personally. Although I am sure there is more than I am aware of.

The effects of inflammatory bowel disease have taken quite a toll on my body and I am very much aware of how the drug that is currently helping me stay alive is taking its own toll. It is a stopgap measure and only one aspect of a holistic approach I am taking towards improving my digestive health.

How Did I End Up Here?

Some Health Backstory

It wasn’t always this way. Things began to veer off course as I was approaching my 50^th birthday. Happily trucking along in life when my guts decided to make things messy – quite literally! Hot flashes and mood swings I had been mentally preparing myself for at this stage in life but chronic diarrhea?! No one warned me about this! At first, I thought it might have something to do with the hormonal changes that menopause brings but no. Turns out I had lymphocytic colitis, a not so common inflammatory bowel disease. ‘Colitis light’ I called it since the symptoms were not as devastating as Ulcerative Colitis or Crohn’s and it did not put me at a higher risk of colon cancer. I learned to manage it for the most part through dietary changes and over the counter medications. For some lucky people, lymphocytic or microscopic colitis somehow clears up on its own. I was sure I would be one of the lucky ones.

Skip ahead a couple of years and my guts became considerably more troublesome almost overnight after taking antibiotics. Relentless diarrhea that landed me in the hospital weak, dehydrated and with low electrolytes. Prednisone was prescribed until further tests, including a colonoscopy, could be done to determine what else was going on in there. Nothing conclusive was found.

Searching for other possible solutions, I began to work with a naturopath. Between taking various supplements and eliminating a number of foods which I had a sensitivity to, the diarrhea was under control and I was slowly reducing the prednisone. For three glorious weeks, I felt hopeful and better than I had in months. That feeling was short-lived. I had mussels at dinner one evening (which are not one of my food sensitivities, I might add!) and all progress was gone. Something must have been off with the mussels as I became violently ill that night and my bowels have not been the same since. At that point, not even the prednisone worked. I did whatever I could with anti-diarrheal medication from the doctors and supplements from the naturopath. All helped enough to get me through the day at work but after weeks of this, I was slowly becoming weaker and losing weight steadily.
It is strange but somehow you adapt to this new debilitated self and it becomes normal to you. Some days or moments, you even feel you might be getting better. Funny how the mind plays tricks and how we can rationalize our situation much to the chagrin of loved ones who are much more cognizant of our decline.

Eventually, I went to the hospital again overnight because I was so dehydrated – to get my fluids topped up so to speak. I also began another medication which I anticipated would calm my insides down. I was fine, I am managing this I felt and did not think that I needed to be admitted to hospital while we waited to see if the new meds would work. I was afraid that I would not come out again, of not being able to work anymore. I was afraid of this un-named disease truly not going away.

I was not fine, I tired easily, could barely walk up stairs and was losing weight drastically. A week or so after my last hospital visit my daughter suggested I visit the family doctor to see what options there might be other than going to emergency. He took one look at me and was calling emergency to get me in ASAP. Skip the line sort of thing. I had been coping, adjusting, and unknowingly become quite critical. My blood pressure was very low as was my potassium level and other electrolytes which put me at risk of ‘fatal heart arrhythmia’ as one nurse told me. I was placed on a heart monitor and I was to be put on Total Parenteral Nutrition (TPN). Despite eating, I was not absorbing many nutrients and slowly starving.

After a number of more diagnostic tests, finally, a diagnosis – collagenous sprue. A rare inflammatory bowel disease thought to be linked to Celiac Disease. ‘Collag-a-whaat?!” I thought. My gastroenterologist was thrilled to have a diagnosis at last; all I could think of was that he stop talking so I could go throw up.

So we began to learn about what this rare disease is. It was unnerving to first read one early documented case, a middle-aged female, in the early 1970s succumbed to the illness. A disorder that along with lymphocytic colitis, women in their 50s are more likely than others to be diagnosed with. Yay me! I won both rare prizes.

On the heels of this overwhelming diagnosis, I had a significant intestinal bleed the following night. Nearly losing consciousness, I required an immediate blood transfusion. It all seemed so unreal, this is not really happening to me, is it? I was afraid to go back to sleep, afraid I would not wake again. I am ever thankful to the nurses and doctor that took care of me that night.

I came home after two months in the hospital and the completion of training for Home TPN Program. I remained positive that the biologics therapy I was on would put the collagenous sprue into remission or at least make it manageable. After two infusions, things were not yet turning around. Over the following five weeks I was at home, the nausea and weakness increased and I experienced other medical complications due to months of malabsorption. Blood work showed that my liver and kidneys were not functioning well. It was back to the hospital for me.

Things were not looking good. It felt like my body was giving up. No one was sure that I was going to get through this. I made peace with my Maker really feeling that I would not be here for many more days. After a couple of weeks miraculously, it seemed, I began to feel marginally better each day and was well enough to come home three weeks later.

I was not giving up on the biologics yet since I had heard that it can take three to four infusions before you really begin to notice substantial improvement. I had the third infusion and began to feel even better roughly a week to ten days following. The nausea had completely subsided and I could finally eat more than a tablespoon or two of food. Many fewer trips to the toilet too.

My hopes were dashed again with the fourth infusion whereupon I had a severe allergic reaction. I tried and didn’t respond well either to other Anti-TNF blockers. Thankfully, at this point, I was off the Prednisone and aside from a daily antidiarrheal, I was holding steady. It seemed I was somewhat in remission for nearly three months until days before our wedding last June. This time it was my Father in the hospital. Worrying about him and thinking he might not make it to our wedding shook me up. Stress can certainly exacerbate IBD symptoms. But who knows, maybe I looked up instead of down or turned left instead of right. So, for now, it continues to be a juggle between dealing with this chronic disease, more hospital visits, hoping to find a therapy that works and getting on with all that is Life.

Thrilled to say that the wedding went off without a hitch – Well, Doug and I did get hitched! The day was beautiful and everyone was healthy and celebrating.