My son was diagnosed SJIA at age 12 then Crohn's at age 13

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sjiadad

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Hello,
My son was diagnosed SJIA at age 12 then Crohn's at age 13. He is now 13. We tried Humira then Infliximab (has sever reaction from Infliximab at 3rd dose so we stopped it). Humira helped for 3 months only. Now we are on Rinvoq, MTX and actemra for two weeks now. His Arthritis is still worse and his calprotectin above 1000. He is on a wheel chair for Alost 5 month. Arthritis affect badly his knees, feet and hands. Diarrhea 3 times a day lost a lot of his weight. His growth stopped. we tried PediaSure® Peptide 1.5 Cal. I do not see any improvement sicne he was diagnosed with SJIA just adding Crohn's. Any advice?
 
I am not familiar with the arthritis issues, but I can talk about Crohn's. I believe arthritis could be a result of Crohn's (or the result of gut bacteria/antigens going places they shouldn't, due to the intestinal damage of Crohn's), but I don't know.

This sounds like a severe case of Crohn's. Exclusive Enteral Nutrition (EEN) is one of the first options for bringing down inflammation fast. Pediasure Peptide is one option for that, but he shouldn't have any other food while trying EEN. I can't tell from your post if he's trying EEN.

The 1.5 version probably doesn't have enough water in it, so he'll also have to have some water. We found the 1.0 version to be much more convenient.

If he's having trouble with drinking it, an NG feeding tube is a possibility. He may be able to gain some weight that way, but be careful not to add weight too fast.

It sounds like he's on a pretty high level of immune suppression right now. I can't give any specific advice about those medicines, but be careful with all of that. I'm skeptical of adding too much immune suppression for Crohn's.

If these drugs aren't working, Stelara, Skyrizi, and Entyvio are also options.

In general, keep monitoring symptoms and test results (such as fecal calprotectin, ESR, CRP), and make sure that things are improving. If the situation is not improving, then push to try something else.

Where is the Crohn's located? Sometimes surgery can be a good option, especially if the Crohn's can be localized into a small region.

My son had a very difficult case of Crohn's, and what eventually worked for him was EEN plus surgery plus Stelara. I think all of those were necessary in combination in order to finally get it under control.
 
Last edited:
So sjia is systemic juvenile arthritis which is much harder to treat than normal jia and a whole other level of disease as you know

Some other sjia drugs are anakinra (kineret ) which is a painful daily injection
And ilaris -canakinumab
Ilaris does not hurt when injected and can be given every two weeks

my child has been on both for a different auto inflammatory disease plus jia and crohns

neocate Jr or elecare jr are elemental formulas which are completely broken down -only need a few inches of good intestine to absorb them
They taste horrid but a ng tube can be used.
My kiddo was able to drink neocate jr chocolate at 12 or 13 that can help with weight and treat crohns

my child needed a wheelchair for a few months for arthritis so I understand your frustration.

tagging @crohnsinct on the rinvoq

From our experience the jia /auto inflammatory drugs did not help with crohns much
And if the jia was out of control the crohns would flare as well.

My kiddo has been on ilaris and Stelara and mtx
For over five years
The Stelara is for his crohns /jia
Mtx for jia
And ilaris for the auto inflammatory disease

have you gotten a second opinion at a big place for sjia (such a children’s hospital of Philadelphia) or Boston children’s ?

second opinions are a huge help. When complicated kids are involved
Are they using steriods as a bridge therapy until the other meds kick in ?

Mtx takes up to three months to work
Acetmera and Rinvoq I assume take a few months as well but never used either .
 
Thank you we live in California. We got second opinion from UCLA. With same diagnosis. We tried ilaris then daily Anakinra with no effect.
 
No effect on his crohns or no effect on his sjia ?
Ilaris and kineret never touched my kiddos jia or crohns
Just was effective for his sweets syndrome

have you tried Stanford childrens ???
Definitely try the elemental formula
Hard to drink but should let him get nutrition and stop crohns inflammation when used as een
 
I'm so sorry you are dealing with such a challenging diagnosis. My daughter was diagnosed with JIA a few days after turning 12 and Crohn's at 16. She has Enthesitis Related Arthritis, which has now progressed to Ankylosing Spondylitis which has been quite severe. It has been a struggle to find a drug combination that works for both diseases and we eventually went to two biologics - an IL-17 inhibitor for the arthritis and an anti-TNF (like Humira, Cimzia or Remicade) for the Crohn's.

SJIA is challenging by itself to manage and as I'm sure you know, can be life-threatening, which is why your doctors have him on multiple immunosuppressants.

I know it's extremely hard when your child is in a wheelchair - my daughter has such severe arthritis that she had 2 joints replaced a few years ago. She has arthritis in most joints - her fingers, wrists, elbows, shoulders, hips, sacroiliac joints, lumbar spine, ankles, knees, jaw etc. We have tried 9 biologics and while she has responded well to some for 2 years or so, she always loses response around the 2 year mark, so we're still looking for a medication to control her arthritis. Her Crohn's is controlled by Remicade.

She did try Rinvoq + an anti-TNF, which took about two months to work but worked very well for her. However, she got a lot of infections on that particular combination of biologics and her rheumatologist and GI say that now that more is known about JAK inhibitors, that they're not safe to combine with anti-TNFs. But they can be combined with more selective (and less immunosuppressive biologics) like Actemra.

Rinvoq and Actemra together may work for your child. I know it's hard to wait, but give it some time. It can take a few months for these meds to really kick in.

You can use formula in two ways - either as supplemental enteral nutrition (in addition to food) to gain weight and to help with growth or to induce remission for the Crohn's - in that case, typically kids have formula only or 90% formula and 10% food for 6-8 weeks to induce remission. My daughter has done both and found that using a feeding tube was actually a lot easier than trying to drink the formula.

Another option, of course, would be steroids, which will help both the Crohn's and SJIA while you're waiting for Rinvoq and Actemra to kick in but are not great for growth.
 
Thank you so much Maya142 we tried prednisone at starting of his symptoms for 6 month. with no effect at all. We know 16 months since his first diagnosed with SJIA. 'He just grow an inch or two. I can see his growth stopped. I'll talk to his GI about " typically kids have formula only or 90% formula and 10% food for 6-8 weeks "
'

.
 
My daughters are seen at Stanford. They have a new director there and he is wonderful. Very well known in the field and very on top of the latest research.

Where is his disease located? What was his calpro prior to starting Rinvoq? Is he still on loading dose of Rinvoq? What does is he on? Unlike most of the other IBD meds, Rinvoq works very quickly. Our docs told us we would know within two weeks whether or not it was working but there are still things you could do. Mtx takes about 12 weeks to get to a therapeutic level so it will take a while for it to kick in. EEN is an excellent option to help arrest the inflammation while waiting for the meds to kick in.

What dose and frequency of Humira was he on? Did he build antibodies? Did they try to add anything to the Humira to try to help it out?

Have you consulted with a registered dietician at the GI's office? Even if your son is not willing to do EEN, some form of supplemental nutrition will help him put some weight on. Weight loss will lead to inflammations it is really important to help him try to keep as much on a possible.
 
Arthritis located at his ankles, toes, knees and his calpro prior to Rinvoq was 800. His crohn's in large intestine He is on Rinvoq(30MG) for two weeks, I've not seen improvement yet. He is already on MTX. He was on Humira Bi-weekly then weekly but stopped working after 4 month. Got allergy from infliximab. It seems you suggest EEN diet what's your experience?
 
What's the web address for Stanford? Does it require insurance? Could I pay out of bucket if does not accept my insurance? Thank you!
 
Stanford accepts a lot of different insurances and yes they will take out of pocket payments.

https://www.stanfordchildrens.org/en/service/inflammatory-bowel-disease
Rinvoq has a good success rate in the colon. The loading dose is 45mg for 8 weeks. Your son seems to be severe and refractory and I would think he would need the full loading dose. Although he is a pediatric patient and Rinvoq dosing is based on studies in adults. That said, we know that children generally have very aggressive disease and often need much higher doses than adults. All this to say, I would ask the GI why they opted not to do the full loading dose…he is basically on the maintenance dose.

I have two daughter's with Crohn's. One has primarily small bowel disease. She has done EEN 3 separate times when it seemed that Remicade was not working anymore. Each time, EEN was able to get her back into remission and bought us more time with Remicade. My older daughter had primarily colonic disease. She has also used EEN about 3 times. It worked well the first two times. The 3rd time not so much. The first time she used it, she did so because Remicade was just not getting her to remission. It was helping a bit but was not getting her to remission. We added EEN to help get her to remission and then handed the drug a healed bowel and the drug worked like a charm. Sometimes it is just super hard for a drug to induce remission and it needs a little help. The mtx could potentially help but it takes up to 12 weeks to work. We have used it and unfortunately for my girls it didn't really work well. I also think that you are fighting a losing battle by not giving the full loading dose but your GI could have a very good reason for this. Maybe immune suppression given the other drugs he is on? But honestly, the risks of uncontrolled disease are just as real as immune suppression and the 45 mg dose is only for 8 weeks.

As for the calpro, there isn't much difference between 800 and 1000. Calpro has a high degree of day to day variability, especially at the higher numbers so I wouldn't be scared that the number issuing up but you are right to be concerned they are staying at the same level. This said, two weeks is really a little too soon to expect cal pro to drop. We usually will see a drop a month in.
 
My guess is that they didn’t give him the 45 mg loading dose because of the other meds he is on. In rheumatology, no loading dose is given and the dose is 15 mg, which is why it takes much longer to work than in IBD patients.

Actemra is an IL-6 inhibitor that is notorious for causing low neutrophils, so my guess is that because he’s on a second biologic that can also cause WBC counts to drop, that they chose to use a lower loading dose of Rinvoq. I know of several children whose absolute neutrophil count (ANC) dropped so much on Actemra that they had to be pulled out of school to prevent them from getting serious infections until their counts went up. It could also be because he’s pediatric.

But it is worth asking about the higher loading dose if 30 mg is not working.

What is his Actemra schedule - is he getting it every 2 weeks or every 4 weeks? Is it IV or injections?

Have they considered joint injections (with steroids) into his most problematic joints, to help control the flare and pain while his meds kick in? That would help you avoid the side effects of systemic steroids but would also help his joints. That’s something you could combine with EEN - the EEN would control the Crohn’s and joint injections could control the arthritis as a bridge till his meds kick in.

At his age, many kids can do joint injections without sedation. Both my daughters had joint injections when they were diagnosed. My younger daughter in her knee when she was 12 and my older daughter in both knees and ankles at 14. Both managed them well without sedation - they’re very quick. And both girls were feeling so much better the next day! They both also had fluid drained from their joints. But they can certainly do them with sedation if he prefers that.

Considering how complex your son’s case is, it might be worth traveling to Cincinnati Children’s which has the best SJIA program or to CHOP which also sees a lot of SJIA kids. They may even be able to do a remote second opinion. It’s unusual not to respond to steroids and IL-1 inhibitors so perhaps there is genetic testing that is needed to help explain what’s going on.
 
Hello,
Does any one had experience with Stanford second opinion? Does it change diagnoses or treatment? I appreciate your reply? Thank you!
 
Given your child did not respond to steriods or iL-1 inhibitors
Definitely recommend a second opinion at chop immune dysregulation team
They pull in 3 to 4 depts all at once
You stay in one room and each specialist comes in to see your child one after another
The previous specialist speaks to the next specialist so you don’t have to repeat
They help figure out very complex cases
They will run complete whole genome sequences
Genetics visits
Immunology
Rheumatology
And Gi more than likely on your child’s case
Lots of blood work on the immune system as well

https://www.chop.edu/centers-programs/immune-dysregulation-program
it is really a one of a kind program
My kiddo participated in it
 
I agree - it’s quite unusual not to respond to steroids with SJIA and IL-1 inhibitors, so the CHOP immune dysregulation program is an excellent place to go to figure out what is going on. It’s very hard to get insurance to approve whole exome or genome sequencing otherwise. I know it’s quite a long trip for you but it’s one that is worth looking into. They may be able to identify a genetic mutation and figure out treatment that way.
 
sjiadad said:
Hello,
My son was diagnosed SJIA at age 12 then Crohn's at age 13. He is now 13. We tried Humira then Infliximab (has sever reaction from Infliximab at 3rd dose so we stopped it). Humira helped for 3 months only. Now we are on Rinvoq, MTX and actemra for two weeks now. His Arthritis is still worse and his calprotectin above 1000. He is on a wheel chair for Alost 5 month. Arthritis affect badly his knees, feet and hands. Diarrhea 3 times a day lost a lot of his weight. His growth stopped. we tried PediaSure® Peptide 1.5 Cal. I do not see any improvement sicne he was diagnosed with SJIA just adding Crohn's. Any advice?
Click to expand...

Hi, my husband was diagnosed with Crohn's more than 15 years ago and is seeing great improvements with a complete change of diet. We've started the GAPS diet, which is a medical diet, it's difficult at first, but it's free, no drugs and no side effects, and it offers healing, not just coping with the symptoms of Crohn's. You'll find plenty of resources online but don't hesitate to ask me any question. Thanks
 
@Cliffandmorgane
Unfortunately diet will not fix systemic juvenile arthritis which is what the poster is also dealing with
That is a very serious disease that affects multiple organs and the joints
It is not a Gi disease
 
my little penguin said:
@Cliffandmorgane
Unfortunately diet will not fix systemic juvenile arthritis which is what the poster is also dealing with
That is a very serious disease that affects multiple organs and the joints
It is not a Gi disease
Click to expand...

I'm so sorry your son is going through such terrible medical problems. I have a daughter myself and I can't imagine what it must be like for you. If you have the time, do a bit of research on the GAPS diet cause it can also cure arthritis. I know it sounds a bit odd, but healing the gut can heal a lot of auto-immune diseases. Of course it doesn't work for everyone, and many people need medication because of the severity of their diseases, and thank God there are medications available, but a lot of research is being done on gut health and all the ways in which it can affect the whole body.
 
@sjiadad - I am so sorry that your son is going through this terrible ordeal. I hope that your son's doctors find a treatment soon which will work.
 
My daughter actually did the GAPS diet - my older one. It was a real disaster. She lost weight and her joints got even more inflamed. So I would definitely talk to your pediatric rheumatologist and GI before attempting it. We chose to try it before biologics and in hindsight, it was a huge mistake. Once we got her on the right combination of medications she did really well.

@Cliffandmorgane SJIA is an auto inflammatory disease - not autoimmune - and can be fatal, so it extremely important to get it under control and follow the doctor’s instructions. It can affect the heart and lungs and cause Macrophage Activation Syndrome. It is a very serious disease and can have devastating consequences.
 
Thank you so much @Maya142 since you tried Rinvoq. We have been on it for a month with no success sigh at all and. We notice residue in his tools OF Rinvoq. How is your experience did you notice success or residue after a month? should we wait another month? Thank you I appreciate it.
 
We notice residue in his tools OF Rinvoq. How is your experience did you notice success or residue after a month?
Click to expand...
What do you mean, "residue"? Do you mean residue of Rinvoq - like part of the tablet?

When you say no success, do you mean for the Crohn's or the SJIA or both? Is Actemra helping at all?
 
@Maya142 Yes part or whole of Rinvoq tablet. I mean both Crohn's with daily 3-4 diarrhea and arthritis and swelling in his knees, wrists, fingers and ankles.
 
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That can happen with extended release tablets but I’d recommend talking to your pharmacist about it. My daughter does not remember if she saw Rinvoq in her stool.
I would talk to your doctors about the fact that you have not seen any response to these meds. I’m afraid I don’t know enough about systemic JIA to give you advice. My daughter has a different type of JIA that’s treated entirely differently.
It may be worth getting another opinion.
 
@sjiadad - I hope your son is feeling better. It has been a while since your last update and I wanted to check with you how is it going.
 

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