14 year with some lingering issues and unanswered questions

[crohnsteeny]

Dear Friends

Daughter 14 year old diagnosed with Crohn's based on below

Symptoms before diagnosis

== RLQ and RUQ stomach pain, nausea/vomitting/severe diarrhea/history of anemia, iron def, vitain D def, severe joint pains, migraines, fatigue, , canker sores, blurry vision, irregular periods, pain during menstruation, involuntary weight loss from 110 lbs to 90lbs, weak muscles and so on

== EGD and Colonoscopy showing non-specified inflammation

== Inflammation and thickening (Small Bowel) found during MRE
== Severe inflammation, edeme, several distant Ulcers (Crohns ulcers), redness/etc found in Capsule studies. Diagnosed with Moderate to Severe Distal Small Bowel Crohn's
== Normal CRP/ESR througout (except during Pancreatitis)

== IBD Panel showing both ASCA Igg and Iga positive and elevated levels (

== FCP borderline above 100 and less than 150 indicating possible distal small bowel Crohn's

== IBD expanded panel showing 3 antibodies showing elevated levels (Suggestive of Crohn's disease with high risk of aggressive disease behavior (development of strictures or fistulae).")

== Tests from imunologist

== Elevated IGG2 subclass
== Highly elevated IL-4, IL-5, IL-6

Currently, getting Remicade 10mg/kg every 4 weeks, been on Prednisone 30mg (down from 40) for 3 months (she gets symptoms of joint pain, diarrhea and abdomen pain when trying to taper from 30mg to 25mg)

Prednisone and Remicade has put her in remission (More of steroid induced we feel), since she felt better 70% only just with Remicade alone. and she is pretty much IBD symptom free currently (except for Migraine and backpain due to accident). GI wants to add MTX to try taper Pred

Also, gallbladder removed due to severe RUQ pain and abnormal HIDA scan

Her current minor issues
== High Hemoblogin, High Heamoticrit, Low TSH, Cold feet and legs , high resting heart rate and high Ferritin


Questions would appreciate some feedback

= For her current minor issues, (low tsh, high ferritin, high Hgb and Heamoticrit and cold feet and legs), can these be due to Prednisone ? Any other possible blood disorders requiring heam or endocrin visits ? We will be meeting our GI next week to discuss these.
= We still not convinced if it's Crohn's alone, since she takes birth control for her possible endometreosis. She has been told IBD related Arthritis, since negative ANA. Even though it is all under control with her Pred and Remicade, we were wondering since ANA is negative to look for any other auto immune conditions, since one follows other ?
= ANyone has issues tapering Prednisone ? (Joint pain, diarrha and abdomen pain comes back slowly, not as furious during flare though). We are concerned she has become steroid dependant, but she is pretty much symptom free now.
= Anyone had IGG or Cytokine profiles from Immunologist ? Immunolgist and GI thinks IVIG infusion plus Remicade should help her get rid of steroid dependance ? THoughts ?

 

[my little penguin]

Random thoughts
My kiddo was dx with crohns at 7 but is 18 now

Ivig can cause aseptic meningitis in kids prone to migraine
My kiddo did ivig three times
ER after each due to aseptic meningitis
Resolved once iivig was stopped
Even tried pre treating with dexamethasone/slow drips and Benadryl /imitrex to stop
But he is prone to migraines (used to get them a ton until he got all his issues under control )
Hasn’t had a migraine in years now that everything is treated right

Ibd related arthritis-has two types
Of juvenile spondyloarthritis (ERA )
Both need regular visits with a rheumatologist
One resolves all inflammation in the joints the same time as all Gi issues resolve
Second one -joints flare independently of the Gi tract - my kiddo has juvenile spondyloarthritis that flares independently of his Crohn’s disease

his arthritis is much worse to treat than his crohns at this point .

he currently takes Stelara/methotrexate plus Celebrex for arthritis/crohns

unfortunately you can have more than two things going on

my kiddo also has Sweets syndrome (acute febrile neutrophilic dermatosis) which he takes a second biologic for

plus some other minor random stuff

until we figured out sweets he was miserable and we thought crohns was causing issues

depending where you are at
There is places like childrens hospital of Philadelphia- they have a immune dysregulation team - DIRT
They see kids who don’t quite fit in the mold and try to figure them out
We took DS they agree with his three dx and to keep his drugs the same

some time whole exome sequencing can help as well - nih has a program which looks through for issues
other larger university pediatric centers have them as well
There are auto inflammatory conditions which mimic crohns and other stuff added

sometimes kids are a mystery

as far as getting off steriods
Have you tried adding mtx and een (formula only ) ?
some kids need both to wean
Endo is also helpful in weaning
Really slowly
1-2 mg at a time for a week or two

my kiddo used to get sick weaning off
Beside the fact we had not identified all the issues - we just didn’t know
He was also super sensitive to steriods
Had adrenal insufficiency after getting off them for a year

endo Recommended very slow wean
Took forever but so much easier on his system

 

[crohnsteeny]

@my little penguin Thanks for your response. Really appreciate. Sorry you had to go through so much with your kids. Yes, this is very complicated. We are in the same boat, we have not identified all issues yet as well. GI and Rhuem and Immunlogist think treatment of Crohn's should help her with Joint pains and other symptoms as well. We are currently worried about her cold feet, hands and high Ferritin.

For tapering off Prednisone, we are waiting for insurance approval for Metothrexate., which we should get (Approval) tomorrow. Hopefully that should help taper. We live in Colorado and we go to Rocky Mountain Hospital for Children. Daughter started at 40mg Pred on Oct 7th and now still on 30mg. Hopefully adding MTx next week should help
Immunologist/GI want to try IVIG. Looks like you had a bad experience with IVIG. Besides causing aseptic meningitis, did IVIG help your kid with other symptoms ?

 

[my little penguin]

Ivig did nothing

 

[GirlwiththeCurl]

I don't know tons about this, but with high hb, hc, and ferritin I'd be wondering about hemochromatosis, which is an iron storage disorder.

 

[Maya142]

Hi - it looks like your daughter has had a lot going on. My daughter also has Crohn's and inflammatory arthritis. IBD related arthritis can be divided into two categories. Sometimes it flares when the IBD flares and treating the IBD then also controls the arthritis. But sometimes, it flares independently of the IBD and is a distinct disease. My daughter's arthritis is like this - just getting her Crohn's under control does not get her arthritis under control. In her case, her IBD responded very well to Remicade, but it wasn't enough for her arthritis.
Typically, if small joints like fingers and toes are involved or if the spine or sacroiliac joints are involved, then the arthritis tends to flare independently of the IBD. This type of arthritis is a subtype of juvenile idiopathic arthritis (JIA) called Enthesitis Related Arthritis or ERA. It is also sometimes called juvenile spondyloarthritis, though that's an older name.

Adding something like Methotrexate makes a lot of sense for a kiddo with arthritis. It works well for the joints and might give Remicade a "boost" and help with her gut too.

Does your daughter see a pediatric rheumatologist regularly? She should, if she has any kind of arthritis.

Cold hands and feet - do her hands change color when they're cold? Do you ever see blue or white fingers? Raynaud's Phenomenon or Raynaud's Syndrome is autoimmune and can occur with JIA. My daughter's fingers become blue when they're cold. She has to be careful to warm them up if they do go blue and to always wear thick gloves in the winter. She also always wears thick socks. Once she warms them up, they turn red. That color change is characteristic of Raynaud's.

For her current minor issues, (low tsh, high ferritin, high Hgb and Heamoticrit and cold feet and legs), can these be due to Prednisone ? Any other possible blood disorders requiring heam or endocrin visits ? We will be meeting our GI next week to discuss these.

You can certainly see endocrinology and should if she has a low TSH. They can also help you taper more slowly so you're successful - our endocrinologist always complains that GIs and rheumatologists taper much too fast!

Generally high Ferritin is typically nothing to worry about (well, unless it's REALLY high) - it is an acute phase reactant, which means it goes up when there is inflammation. My daughter had a high Ferritin for years while we were trying to get her arthritis under control. But since your daughter also has a high hemoglobin, then it may be worth seeing a hematologist.

ANyone has issues tapering Prednisone ? (Joint pain, diarrha and abdomen pain comes back slowly, not as furious during flare though). We are concerned she has become steroid dependant, but she is pretty much symptom free now.

Some kids have a harder time than others getting off steroids. MTX should help. Some kids do Exclusive Enteral Nutrition - mostly formula, for 6 weeks or so, to induce remission. It's safer and more effective than steroids, but of course, it is harder to do. But it's an option. I'll tag @crohnsinct because her daughter did it to get off steroids.

We have had some testing done by an immunologist, but they did not suggest IVIG. It's not normally suggested for JIA or IBD. I would honestly get a second opinion before trying that.

 

[crohnsinct]

Yep! My daughter didn't have the arthritis but did have considerable joint pain that went away as soon as her IBD was controlled.

We were in the same predicament. 4 months after dx and we couldn't taper steroids. They told us she was steroid dependent. They wanted us to add methotrexate but thanks to the parents here they suggested EEN. EEN is just as effective at reducing inflammation as steroids and actually even better because it can result in mucosal healing and steroids don't do that. O opted for EEN rather than another med. 8 weeks of EEN and she was great. Felt great and had great labs. Once the EEN knocked down her inflammation, Remicade was able to maintain her. Sometimes these meds just can't tackle the initial inflammation but once handed a healed bowel they are great at maintaining it.