# We have a diagnosis! (And I'm not crazy!)



## DanceMom (Jun 8, 2016)

Doctors have a way of making you feel crazy when you take your child in repeatedly, stating how sick she is, but they can't figure out what's wrong. Well today we got a "real diagnosis" and validation that I'm not crazy.

A has been diagnosed with Intestinal Lymphangiectasia. This is the cause of her Hypogammaglobulinemia. Her biopsy from January discovered this but her GI didn't seem to agree. We met with the Immunologist today and she stated that she always thought A had IL but couldn't prove it until now. Primary IL is extremely rare and the Immunologist is currently looking for a doctor that can treat her. Our GI does not have experience with this condition, so as much as we love him we may have to move on. 

Treatment could include diet changes, bowel resection, medication, or a combo of those things. I'm anxious to get her on the right track! I've been on the verge of an anxiety attack all day.


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## ronroush7 (Jun 8, 2016)

I am glad you finally have a diagnosis.


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## CarolinAlaska (Jun 9, 2016)

I've never heard of this.  I'm glad she (and you) finally have an answer.  I'll pray you find just the right doctor to treat her.


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## CarolinAlaska (Jun 9, 2016)

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2288596/

Maybe you found this, but I found it informative about primary intestinal lymphangectasia.


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## caretothepeople (Jun 9, 2016)

The National Organization for Rare Disorders also has an information page with a list of resources and support organizations: http://rarediseases.org/rare-diseases/primary-intestinal-lymphangiectasia/

In particular the Lymphatic Education network might be worthwhile to check out : http://lymphaticnetwork.org/ask-experts/e/stanley-rockson/P30

Good luck and it's great to hear your daughter is getting the care she needs!


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## Farmwife (Jun 9, 2016)

We knew you weren't crazy.:ghug:
Because if you were crazy 
that meant we were crazy 
and we couldn't have that.

I'm glad for answers for her and peace of mind for you.


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## DanceMom (Jun 26, 2016)

We're still waiting to hear back from the Immunologist about a treatment plan. When I called to check in last week the nurse stated that the doctor was waiting to hear back from a physician she contacted (one I was not familiar with). I'm just ready to get things going.

A's IgE lab came back elevated at 156 (norm is <114). Anyone else have an elevated IgE? I know it can be indicative of several things and from what I've read it is common with PIL.


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## my little penguin (Jun 26, 2016)

Elevated IgE is extremely common in allergic kids 
Particularly environmental allergies 
Ds has had high IgE levels every time they tested 
Let me look for the values
I remeber them being really high
But not hyper IgE syndrome high 
Never a concern 
Just part of allergies


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## my little penguin (Jun 26, 2016)

Last IgE was 417

Normal <88 for his age and the lab

At age 3 number was 305
Normal was < 60 for his age at the time


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## kimmidwife (Jul 4, 2016)

Hooray for answers! Sorry I didn't see this sooner! Will be waiting to hear more as you hear more. Two questions, is she still diagnosed as having an immune disorder or does this take the place of that diagnosis? 
Does she have extremity swelling as that seems to be a big symptom of this diagnosis?


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## DanceMom (Jul 5, 2016)

Her immune deficiency is considered secondary to the PIL. Her face swells, not her extremities. In talking with others that have PIL it seems that facial swelling is more typical in children and swelling of the extremities more typical in adulthood.

We're away at her National competition now (she's dancing really well!) and dealing with GI issues, asthma flare, and now a very infected lymph node. Calling the Immunologist this morning to see what can be done.


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## kimmidwife (Jul 5, 2016)

Keep us posted! Sending a prayer for her to feel good at the competition.


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## DanceMom (Jul 6, 2016)

Ended up at the ER. They decided it was an abscess with cellulitis. 10 days of antibiotics and watching it closely. Poor kid can't catch a break.


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## DanceMom (Jul 6, 2016)

Pulmonology nurse just called with allergy results - Class 2 for dust mites and Class 3 for mold. Said she'd call later with the doctor's recommendations. I'm assuming this is the cause of her nagging sore throat. 

On a positive note all her dances placed 2nd or 3rd at her first National competition including her solo! How she dances so beautifully when not well is baffling. We just arrived at her second National competition and we're relaxing before the whirlwind starts tomorrow. There's 58 in her solo age division, so fingers crossed!


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## my little penguin (Jul 6, 2016)

Dust mites keep the house free of carpeting /curtains as much as possible
Keep temperature below 72 deg
Freeze or run through the dryer all stuffed animals

Good luck


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## CarolinAlaska (Jul 6, 2016)

Dust mites and mold?  Ugh.  I am lucky that my kids are just allergic to pollen and foods...


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## kimmidwife (Jul 6, 2016)

I am allergic to dust mites, mold, grass, and a lot of other things. We have no carpet I g in the house which makes a huge difference. We also got those mattress covers that you zip around the entire mattress. It has really helped me. We also change the AC filter every two months. My husband says buy the cheap fitters and change them more frequently. It does exactly the same thing.


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## DanceMom (Jul 6, 2016)

We only have carpet in the bedrooms, curtains only in the living room. All beds have nice mattress covers. I do need to dry her stuffed animals because she has tons of them. We keep our house between 73-75, which in FL can be difficult and expensive.

Doctor wants to try Zyrtec and gave her Prednisone for her cough. Ready to get her feeling better!


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## kimmidwife (Jul 6, 2016)

You may want to get rid of the carpet in the bedroom.


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## my little penguin (Jul 6, 2016)

Ds has been on Zyrtec daily since he was 18 months old
It does work well
There are also allergy shots which can improve things if you max out on meds 
Ds did that at age 3


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## DanceMom (Jul 7, 2016)

Just wanted to update! Several doses of her antibiotic and 2 doses of Prednisone and A feels great. I wish it was safe to take Prednisone long term because she thrives on it. Her infection looks so much better. And more great news....her solo placed 2nd out of 58 kids from several countries! So happy for her! She gets to perform again in the Showdown tomorrow night. Yay for a good day!


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## CarolinAlaska (Jul 7, 2016)

Congratulations to A!  I'm glad she's feeling better.


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## kimmidwife (Jul 8, 2016)

Very very glad to hear she is feeling better. That is amazing news!!!


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## Sascot (Jul 10, 2016)

Great news she is feeling good and doing well at the dancing


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## DanceMom (Jul 19, 2016)

It has been a crazy few weeks of ups and downs. A has felt pretty poorly all month so the Immunologist ordered a few tests. Turns out she has enterovirus which caused an asthma flare and GI symptoms. Nothing much can be done but it seems to be resolving slowly. Now she has what appears to be 2 infected bug bites on her leg. The doctor wants to avoid antibiotics if possible for fear it'll worsen the diarrhea that seems to be improving.

Her Immunologists (we seemed to have picked up another who is more available and very interested in A's case) have done lots of research and are considering long term steroids. We know there are risks, but they improve her quality of life so much. They want her to get over this virus/infections before starting though. Just seems to be one thing after another this summer!


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## ronroush7 (Jul 19, 2016)

DanceMom said:


> It has been a crazy few weeks of ups and downs. A has felt pretty poorly all month so the Immunologist ordered a few tests. Turns out she has enterovirus which caused an asthma flare and GI symptoms. Nothing much can be done but it seems to be resolving slowly. Now she has what appears to be 2 infected bug bites on her leg. The doctor wants to avoid antibiotics if possible for fear it'll worsen the diarrhea that seems to be improving.
> 
> Her Immunologists (we seemed to have picked up another who is more available and very interested in A's case) have done lots of research and are considering long term steroids. We know there are risks, but they improve her quality of life so much. They want her to get over this virus/infections before starting though. Just seems to be one thing after another this summer!


So sorry


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## Sascot (Jul 21, 2016)

Hope the virus and bug bites clear up soon. Glad you have a doc that is interested in researching and trying to help!


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## CarolinAlaska (Jul 21, 2016)

It helps to have docs interested in helping.  I'm sorry it has to be long term steroids that be the thing that helps, but I'm glad something helps.


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## my little penguin (Jul 21, 2016)

Big hugs long term steriods is not fun
Ds was suppose to do that for his sweets syndrome but even at 5 mg a day
Way too many side effects 
Trying different meds now 
But may have to go back to them 

Good luck


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## Maya142 (Jul 21, 2016)

Yikes! I hope they work well for her. I know they used to use long-term steroids a lot for JRA and RA before there were biologics. What dose is she going to be on?

Fingers crossed for no side effects!


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## DanceMom (Jul 21, 2016)

Maya142 said:


> Yikes! I hope they work well for her. I know they used to use long-term steroids a lot for JRA and RA before there were biologics. What dose is she going to be on?
> 
> Fingers crossed for no side effects!


The doctors haven't agreed on a medication or dosage yet. There just aren't a lot of trials done or case studies on what works. And each kid is so different. We may go with Prednisone or a steroid that targets the gut. We may do daily or several short bursts per month. Probably a trial and error kinda thing.


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## kimmidwife (Jul 24, 2016)

That is frustrating! Hope they figure something out soon and come up with a plan!


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## Imerald (Aug 11, 2016)

I'm a little late, but I'm  so glad you have a diagnosis!   Will you still see Dr. F for other reasons at all? Regardless, I hope you love the next person just as much. I know how much that makes a difference. ((Hugs))


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## DanceMom (Aug 11, 2016)

We have an appointment with the Immunologist next week so hopefully we'll have a treatment plan after that. We see Dr. F next month but I'm not sure if he'll agree with the diagnosis or not. So many things still up in the air.

Meanwhile A seems to be getting worse. She wakes up every morning with facial edema. Could be a sinus thing (she has had bloody nasal discharge for a few weeks) or could be a sign of disease progression. She is definitely more fatigued and I worry she'll be miserable when school starts next week.


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## Imerald (Aug 11, 2016)

Is she still on the steroid? Could that be causing the edema at all?


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## DanceMom (Aug 11, 2016)

No steroid. She's at her best when on steroids. She's currently on Flovent, Zyrtec, IVIG, and nasal rinses. We stopped the Flonase in case it was contributing to the bloody noses.


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## my little penguin (Aug 11, 2016)

For Ds we were told to use the Flonase /nasonex etc then use saline nasal gel which keeps things moist 
For Ds at least using the Flonase nasonex etc caused nose bleeds but not using them increased nose bleeds as well 
So doctor had us add the saline gel 

Did you let the immunologist know about the facial edema ???


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## DanceMom (Aug 11, 2016)

my little penguin said:


> For Ds we were told to use the Flonase /nasonex etc then use saline nasal gel which keeps things moist
> For Ds at least using the Flonase nasonex etc caused nose bleeds but not using them increased nose bleeds as well
> So doctor had us add the saline gel
> 
> Did you let the immunologist know about the facial edema ???


We were planning on d/c the Flonase once she'd been on the Zyrtec awhile anyway. It didn't seem to be working for her. The sinus rinses seem to be clearing her out pretty well. Lots of blood. Yuck.

The Immunologist doesn't know about the facial edema yet. I've taken pictures and will show her at the appointment. She's only in clinic Tuesday and Wednesday so we may as well just wait. It's very common in PIL unfortunately.


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## Sascot (Aug 12, 2016)

Sorry to hear that. Hope the immunologist can help next week


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## DanceMom (Aug 17, 2016)

A is going to start Octreotide. Not sure if Immunologist or GI will be prescribing. Don't know any details really and have to wait on insurance approval. We will be testing vitamin levels next week and supplementing if necessary. Also going to look into a therapist for her. I already know these injections may not go well (she's anxious already). Very hopeful now that we have a plan though!


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## ronroush7 (Aug 17, 2016)

DanceMom said:


> A is going to start Octreotide. Not sure if Immunologist or GI will be prescribing. Don't know any details really and have to wait on insurance approval. We will be testing vitamin levels next week and supplementing if necessary. Also going to look into a therapist for her. I already know these injections may not go well (she's anxious already). Very hopeful now that we have a plan though!


Congratulations


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## Maya142 (Aug 17, 2016)

What is the Octreotide for?

I hope the injections go well. Has she tried Buzzy? That might help a little.


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## DanceMom (Aug 17, 2016)

Maya142 said:


> What is the Octreotide for?
> 
> I hope the injections go well. Has she tried Buzzy? That might help a little.


I'm not 100% sure how the medication works, but it is supposed to reduce the amount of lymph produced/leaked and hopefully resolve the diarrhea.


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## Maya142 (Aug 17, 2016)

Oh I see, that makes sense. Just had never heard of it and I was curious. I hope it works well for A! She deserves a very long break from all of this!

We have had great success with seeing a psychologist. For us, finding one that worked with kids with chronic illnesses is what really made the difference.


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## Sascot (Aug 17, 2016)

Hope the med works great. Fingers crossed she copes okay with the injections


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## kimmidwife (Aug 17, 2016)

Fingers triple cross that this med helps her! she really deserves a break!!! Will be waiting to hear some updates on how she is doing.


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## DustyKat (Aug 18, 2016)

So much catching up to do on my part, DanceMom! I am so happy to hear that a diagnosis has finally been found and am hoping with all my heart that this med provides lasting relief for your lass, bless her. :heart: 

Good luck!!! :goodluck:


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## CarolinAlaska (Aug 19, 2016)

I hope this makes a huge lasting improvement for her.


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## DanceMom (Aug 29, 2016)

Anyone use the Boston Children's online second opinion program? I'm exploring all of our options because I'm sick of standing by while my girl declines and doctors argue about what to do.


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## Maya142 (Aug 29, 2016)

We have been to BCH but haven't used the online program. We've only been to rheumatology there, not GI. It was great!


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## Farmwife (Aug 29, 2016)

FranG is a new mom with an update.  That's where her son goes. 
Hopefully this will tag her.

Hugs to you both


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## Maya142 (Aug 29, 2016)

I'll tag crohnsinct - she has been there for GI.


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## DanceMom (Sep 11, 2016)

We've decided to see a GI in Atlanta that treats other children with IL. We just weren't comfortable starting daily painful shots without his recommendation. So we head up next month and hopefully we'll develop a plan.

A is doing okay. She continues to have diarrhea and fatigue, but that's become her new normal and she handles it well. She's training for a 5k she wants to run in a few weeks. The sicker she becomes the harder she fights. Her WBC are down to 3.3 (4.5-13.5) ....and that was a week post infusion when they should be at their highest. Her disease is progressing.


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## my little penguin (Sep 11, 2016)

Just an FYI running -(training ) will increase diarrhea in most 
Since runners trots is common in non Ibd folks 
Are the docs ok with her WBC count being that low and waiting a month for a consult and basically longer without meds


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## DanceMom (Sep 11, 2016)

my little penguin said:


> Just an FYI running -(training ) will increase diarrhea in most
> Since runners trots is common in non Ibd folks
> Are the docs ok with her WBC count being that low and waiting a month for a consult and basically longer without meds


Somehow she pushes through physical activity (dance, running, PE) but does tend to have accidents. That's not new for her. She's happier when she's active so her doctors don't discourage it.

Our GI here ordered the labs but didn't seem bothered by the results as he wrote me a message and encouraged me to cancel our appointment scheduled for next week. He wanted us to see another GI in the practice but couldn't get us in until the end of October. We decided Atlanta was a better option. I spoke to the Immunology nurse about the low WBC and she said it isn't considered critical for her diagnoses. Hopefully they'll retest at her infusion (a little over a week away).


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## Maya142 (Sep 11, 2016)

Hope you get a good treatment plan from the appt. Fingers crossed.

Way to go A! She is one tough kid. I hope they can get her feeling better SOON.


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## kimmidwife (Sep 11, 2016)

Hope it goes well with this new doctor. She really is an amazing kid!


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## DanceMom (Sep 12, 2016)

Lab results are slowly filtering in....

TTG IgA is 4 (weak positive, normal is less than 4). Her biopsies have always been negative for Celiac. Think this means anything? As far as I know this is the first she's had this lab so nothing to compare it to.


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## my little penguin (Sep 12, 2016)

Biopsies are the gold standard 
When was her last upper scope done 
And was she eating large amounts gluten at the time 
My DH has one blood test positive ( not that test though ) but is not celiac 
Or gluten intolerant 
Ds is gluten intolerant but has negative tests and biopsies


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## DanceMom (Sep 12, 2016)

Upper scope done in January. At that time she was on a regular diet so she had gluten daily.


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## Mehita (Sep 12, 2016)

What about other intolerances? Fructose, or some other less common one? Have you done a food diary in awhile?


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## my little penguin (Sep 12, 2016)

Is she igA deficient that can affect some blood work ?


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## DanceMom (Sep 12, 2016)

No other intolerances. We do have her on a low fat diet for the last few weeks (recommended for patients with IL). 

Her IgA has always been normal. I believe her medication contains small amounts of IgA, so maybe this is a false positive because she actually carries other people's antibodies.

She's been complaining of a stomach ache and left sided rib pain since yesterday's run. I initially thought a side stitch but it hasn't gone away. Spleen maybe? Hers was enlarged before but discovered incidentally and didn't cause any symptoms. Now she has me worried.


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## kimmidwife (Sep 12, 2016)

How is she doing?


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## DanceMom (Sep 12, 2016)

After getting home this evening I looked at her stomach and there's a large (relative to her tiny frame) lump right below her left rib. She's in quite a bit of pain but no fever. On call Immunologist said to have her seen first thing in the morning, sooner if she develops a fever or can't sleep due to pain. Assuming it's her spleen...


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## DanceMom (Sep 13, 2016)

Took her to the ER this morning. They did an ultrasound and xray and couldn't find a reason for the swelling. They diagnosed it as a superficial skin lesion, insisting she'd been scratched by a cat. We don't even have a cat. Her pain was a 6 yesterday, a 9 now. I'm super frustrated. I don't understand why she'd have this large swollen and painful lump because it certainly wasn't from a cat!


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## kimmidwife (Sep 13, 2016)

What does her doctor say?


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## my little penguin (Sep 13, 2016)

Did they do blood work to look for an infection??
What did her immunologist say ??


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## DanceMom (Sep 13, 2016)

kimmidwife said:


> What does her doctor say?


She's not in clinic today. I didn't even bother to call and leave a message with such a ridiculous update. 

She's more swollen now than she was this morning....


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## DanceMom (Sep 13, 2016)

my little penguin said:


> Did they do blood work to look for an infection??
> What did her immunologist say ??


They ran a CBC but only because I demanded one. WBC low, RBC DISTR low, MCHC high.


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## my little penguin (Sep 13, 2016)

If she is worse I would call the immunologist - the er is just to get stable and with complicated medical kids most of the time doesn't see the whole picture
Is her immunologist on staff at the hospital you went to ?


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## DanceMom (Sep 13, 2016)

my little penguin said:


> If she is worse I would call the immunologist - the er is just to get stable and with complicated medical kids most of the time doesn't see the whole picture
> Is her immunologist on staff at the hospital you went to ?


Her clinic is located in the hospital but is not part of the hospital. I asked them to call and consult with her (Nemours always did) but they kept saying it was a skin issue and wasn't necessary. We've had substandard care before, but this topped it all.


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## my little penguin (Sep 13, 2016)

If they didn't respect your wishes definitely call the immunologist 
They need to know what it looks like now
And what the ER did


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## DanceMom (Sep 14, 2016)

Quick question, would a chest x-ray show the entire small bowel? Any of it?


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## my little penguin (Sep 14, 2016)

Typically no
They would do an abdominal X-ray ( which only shows very bottom of lungs )
If I remember correctly 

http://www.nwhealth.edu/resource/radca/abdoch5.html


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## DanceMom (Sep 14, 2016)

I'm just wondering why they did a chest x-ray and an ultrasound of rib area then....

If she had a small bowel obstruction would that cause swelling in multiple areas of her stomach? Everything I Google sounds like a possible diagnosis, but her BM today was pure liquid and she screamed and felt like she was dying during it. Pain returned to a 6 after BM. Waiting to hear back from Immunologist who was also not impressed with ER care.


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## kimmidwife (Sep 14, 2016)

Thinking of you guys. How is your daughter doing?
What did the immunologist say?


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## DanceMom (Sep 15, 2016)

kimmidwife said:


> Thinking of you guys. How is your daughter doing?
> What did the immunologist say?


We have an appointment this morning. It looks like the swelling has gone down some. She says pain level is the same. This will probably be another of those "I have no idea what's wrong with her" things.


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## kimmidwife (Sep 15, 2016)

Keep us posted. I hope they have an answer for you. When is her appt with the other doctor in Georgia?


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## DanceMom (Sep 15, 2016)

Immunologist said it's erythema nodosum on her ribs....very rare to have it there and extremely painful. Hers are not red, but the doctor said they sometimes aren't when they're on the ribs or spine. She has one just under her right rib and clusters on her left. She was surprised A was calm because she said each breath had to be excruciating. My poor girl. She prescribed Prednisone.

Apparently the Nemours team (GIs and Vascular Surgeons) recommended surgery to remove the portion of her bowel affected by the IL. We're still going to Atlanta for a second opinion. Is this called a resection or something else? I never imagined surgery in her future so I've never researched this until now.


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## my little penguin (Sep 15, 2016)

Depending on how much they take out
Small amount resection as long as they plan to reconnect the two parts but won't it reoccur 
?


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## DanceMom (Sep 15, 2016)

It could definitely reoccur. It could also be other places in her bowel that haven't been identified yet. But apparently surgery is often used when the disease is limited to one or two locations.


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## Maya142 (Sep 15, 2016)

Poor A!! That sounds awful! I hope she feels better with the steroids quickly.

A second opinion makes a lot of sense before surgery.


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## kimmidwife (Sep 17, 2016)

I am surprised they are think g surgery so quickly. Glad you are going for another opinion. That is crazy that it is erythema nodosum. I never knew it could occur other places besides the arms and legs. I hope the steroids clear it right up. Glad you got an answer. 
Keep us posted on the second opinion.


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## Sascot (Sep 18, 2016)

Sorry to hear things have been so bad. Really hope the pred helps reduce the pain. What a lot to go through


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## DanceMom (Sep 18, 2016)

kimmidwife said:


> I am surprised they are think g surgery so quickly. Glad you are going for another opinion. That is crazy that it is erythema nodosum. I never knew it could occur other places besides the arms and legs. I hope the steroids clear it right up. Glad you got an answer.
> Keep us posted on the second opinion.


She's had EN on her spine before but they were smaller and not nearly as painful. The EN has now spread to her legs. Her chest x-ray was not exactly  "normal" as I was told. It indicates thickening of the paraspinal stripe of the upper abdomen bilaterally and suggests doing an abdominal ultrasound (they only did an ultrasound of her rib). I'm not really sure what that means, but she's had a lot of chest x-rays and that finding is new.

I think the point of surgery would be a quick fix (as opposed to medication that doesn't work for a lot of patients). If the diseased bowel were removed she would be in remission and shouldn't need IVIG anymore. It's a tough decision to make!


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## Farmwife (Sep 18, 2016)

Hugs to you and A.
Grace had EN on her spine and arms before but mainly hits her shins and ankles. 
The ones on her ankles hurt the worst.  I can't imagine the ribs.


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## kimmidwife (Sep 18, 2016)

Dancemom,
I thought the IVIG was to boost her immune system? So how would surgical removal of the diseased bowel allow her to not need IVIG anymore?
I understand of course that surgery is sometimes needed of course but have they tried other treatments for the bowel besides IVIG?
Can she even safely take something like remicade with her immune system issues?


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## DanceMom (Sep 18, 2016)

The Intestinal Lymphangiectasia in her duodenum causes dilated lymph vessels. Immune proteins like WBC and IgG are then lost through the bowel. To put it simply she poops out her immune system. Octreotide is a medication that could potentially shrink the lymph vessels and reduce lymph production but that would require daily painful shots (think Humira) forever. And Octreotide doesn't work for a lot of patients. The IVIG she gets monthly replaces her IgG and gives her immune system a little boost, but as her disease is progressing it just isn't enough. Remicade probably isn't effective in IL because IL doesn't necessarily mean inflammation. It's complicated...it is a lymph disorder of the GI system. The surgery recommendation came from a board of vascular surgeons and GIs. Her current symptoms are diarrhea, severe erythema nodosum, protein losing enteropathy, muscle atrophy, fatigue, vitamin deficiencies, and weight loss. We have to do something, I just don't know what yet. The prednisone is helping some, but it's a fairly low dose and short course (and not a very good long term solution).


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## CarolinAlaska (Sep 18, 2016)

I'm so sorry about all the trouble she is having!  Praying you all can find the right solutions.


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## Sascot (Sep 19, 2016)

Very stressful trying to find something that works. Thinking of you


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## DanceMom (Sep 21, 2016)

A woke up with about 10 EN on her legs. The ones on her knees really hurt. Rib lumps still there as well. I'll have to call and ask for more prednisone. She still plans on going to gym tonight and running the 5k on Friday.....


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## Farmwife (Sep 21, 2016)

We use compression stocking on Grace's when she had more than 5 EN on her legs and knees. 
Has A tried that?


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## DanceMom (Sep 21, 2016)

Farmwife said:


> We use compression stocking on Grace's when she had more than 5 EN on her legs and knees.
> Has A tried that?


Did that help? They're very sensitive to touch so it seems like that'd be excruciating. The doctor prescribed another prednisone pack.


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## kimmidwife (Sep 21, 2016)

I am so sorry to hear. I hope the prednisone helps quickly!


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## DanceMom (Sep 23, 2016)

She has over 20 EN now...it's crazy. She even has them on her upper arms (usually only gets them on lower arms). Her rib area is more swollen and her eyes are almost swollen shut. Our trip to Emory won't come soon enough!


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## Farmwife (Sep 24, 2016)

DanceMom said:


> Did that help? They're very sensitive to touch so it seems like that'd be excruciating. The doctor prescribed another prednisone pack.


I agree, you'd think it would hurt but she says it helps. 
Does she have some tight tights?  Maybe a size to small? 
I did this for grace and just cut the feet out.
Ace bandages were to uncomfortable for her but  a composition  garments seem to be fine.


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## Farmwife (Sep 24, 2016)

Had the EN been biopsy?


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## DanceMom (Sep 24, 2016)

Farmwife said:


> Had the EN been biopsy?


This flare has not been biopsied, but we've had 3 biopsies in the past. Were Grace's biopsied? I mentioned wearing tights and her expression let me know she wasn't having that.

A ran her 5k and did well, but she's paying for it now. Around 30 EN now, and her rib pain is much worse (like it was in the beginning). I hate to see her like this.


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## my little penguin (Sep 24, 2016)

Honestly if it's like ds sweets syndrome(SS)
Until you get the trigger under control 
It's so hard on them 
Ds was miserable with sore lesions/flu like symptoms for close to a year 
Finally after increasing mtx and humira things seem to be calming down
I really hope they can come up with a med fix for her quickly 

Also understand not wanting to biopsy again
Between scars and trauma 
Did the other biopsies confirm EN ?

Never easy when meds are limited btdt with SS


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## kimmidwife (Sep 24, 2016)

Your poor baby! When do you go to Emory? Can you call Dan see if they can get her in sooner? This seems really urgent. They can't leave her suffering like this!


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## DanceMom (Sep 24, 2016)

First biopsy confirmed EN with original Pathologist . Nemours Pathologist thought it could be vasculitis. Second biopsy came back as either EN or vasculitis. Third biopsy came back as EN or hives (definitely isn't hives). Every doctor that has seen it said it definitely looks like EN. Usually it doesn't bother her much but this flare is extremely painful for some reason.

We go to Emory on the 13th. Hopefully if we can get her GI condition under control all else will return to normal.


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## Maya142 (Sep 24, 2016)

Sending hugs! Poor kiddo, that sounds painful. Can they give her something for pain, while you are waiting?

Really hope the second opinion helps.


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## Farmwife (Sep 24, 2016)

DanceMom said:


> This flare has not been biopsied, but we've had 3 biopsies in the past. Were Grace's biopsied? I mentioned wearing tights and her expression let me know she wasn't having that.
> 
> A ran her 5k and did well, but she's paying for it now. Around 30 EN now, and her rib pain is much worse (like it was in the beginning). I hate to see her like this.


No,  never biopsied. 
The rheumatologist,  dermatologist and gp all agreed it was en. 
If grace was like A's,  I would get it done.


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## my little penguin (Sep 24, 2016)

Farmwife 
Has Grace ever had a skin biopsy 
We were told most of the time in auto immune kids the biopsies only show non specific inflammation which is hard to use to Dx anything 
The biopsies are deep and takes weeks to heal to just get the stitches out and months to heal afterwards -not to mention scarring 
Ds took six months to finally heal and just be a scar 
We got lucky and got a Dx off the first biopsy 
The Rheumo explained typically that is not the case 
If it changes meds or adds scary meds in ds case then it's important to try to know
Or if it's a rare Dx as in ds Sweets syndrome - they need to be sure
But if it's not going to change meds with a confirmed biopsy Dx not sure I would do it again


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## Farmwife (Sep 25, 2016)

No mlp, never biopsied.
 Grace's GP is the one who told us that he would wait. 
The only time he went against what the others were saying. 
I'm glad I listened but if she was like A's I would want to make sure.


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## DanceMom (Sep 25, 2016)

A's first two biopsies were punch biopsies. The dermatologist did the first. Immunologist did the second. Pain was minor and she was tumbling two days later, stitches and all. I was so angry that the second was inconclusive and it took me months to agree to the excisional biopsy. I only agreed because I was desperate to help her. She did it without sedation, had pain for about two weeks, but in the end it didn't make much of a difference. I was then told that skin biopsies are very difficult to read and 5 different pathologists could have 5 different diagnoses from the same biopsy. Not very encouraging. A doesn't care about the scars but I'm angry she went through it.


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## DanceMom (Oct 3, 2016)

It's been a rough week. A's ribs are still the same - swollen and sore. She complained of a sore throat so I took her to the pediatrician only to be told it was a virus (because she didn't have a fever). She then developed a painful inflamed lymph node in her armpit. Throat culture came back today positive for Strep. That only took 5 miserable days. Atlanta next week!


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## kimmidwife (Oct 4, 2016)

Thinking of you guys and hoping you get some answers in Atlanta!!!!


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