Hi Kim and welcome back!  Sorry about your family's troubles    

I have no idea if they're the same thing, but my husband just went through a few weeks of having lumps under his skin.  They did feel like fatty cysts, but they were angioedema.  He'd get one or two and then a couple of days later those would go away, and it would pop up elsewhere.  He had them on the back of his head, on his legs and back.  It was very strange.  Apparently, some people get them and they stay for a while.

I hope the visit to Dr. Castells gives you some answers and a treatment plan!  Be sure to ask how to contact her if you have questions after the visit!

Hi Kim!!

I was hoping your absence was good news in that Brie was doing better.  Im really glad to hear that she has been!  You were in a really tight spot with her 6 months ago!  I'm sorry to hear about your family, however.  It's hard having to juggle it all by yourself!

I wish I knew about those lumps, but I don't.  Sorry!!

Give Brie a hug and my very best to you, my friend!

Lisa

FINALLY!!!!!!!!!!!!!!!!!!!!!!!!!!!!


THIS IS INDEED TREMENDOUS NEWS!!!!!!!!   CAN'T TELL YOU HOW HAPPY IT MAKES ME!!   YIPEEEE!!

Tell Brie that all of us are praying for her, and of course, Kim, you are most definitely in all of our prayers for this trip!!!


Please do let us know, for I, for one, will be quite anxious to hear how it all goes!!!


BIG HUGS TO YOU BOTH!!!!


Lisa

Well it has been a very busy couple of weeks here in Boston.  Brie has been such a trooper through all of this.  We arrived in Boston a week ago Tuesday in the evening.  She thought the plane ride "felt like being up in Heaven."  She had long days at the Boston Childrens Hospital on Wed., Thurs, and Fri.  She was seen by several different Specialists during that time (Allergy/Immunology; Hematology;  Dermatology and GI).  They were all so very thorough and very kind... we loved every one them.  We spent a minimum of 1 3/4 hours with each one of these specialties.  They worked very hard in gathering all history and looking at other possibilities for Brie's issues.  They will be the hospital / doctors to do the Bone Marrow Aspiration and all verbalized great concern with doing this and setting off Brie's Mast Cells so they wanted to leave the final decision for Dr. Castells.

We tried to do some touring of Boston in the evenings.  She was free from appointments on Sat., Sun. and Mon so we got out and about a lot during that time.  On Tuesday we saw Dr. Castells.  This visit was very rushed but pretty thorough and to the point.  To sum it up she feels Brie definitely fits the diagnosis for  Mast Cell Activation Syndrome.  This would fall under one of three (Mastocytosis; Monoclonal Mast Cell Activation Syndrome or Mast Cell Activation Syndrome).  If she has any of these the last one is the one we hope she has as this is the noncancerous form and has a possibility for her to go into remission sometime around puberty.  If it turns out to fall into one of the first 2 then this would require Chemo drugs as they are a form of cancer. Although this disease is very rare it would be even more rare for her to have one of the first two... although Dr Castells says that her symptoms do fit those categories so she called the other docs and informed them that we are going to do the Bone Marrow Biopsy.

On Wed. we spent all day up at the Children's Hospital doing all of the Preop tasks.  Since Brie is a high risk for being put under with anesthesia it was decided that she will have several procedures done at the same time so she will not have to be put under more than once.  They coordinated upper and lower scopes, biopsies of the scalp growths and the Bone Marrow Biopsy to be done by all docs together.  She had a VERY rough evening Wed as she had to be prepped for the upper and lower scopes and she was unable to hold down the drinks that she needed to clean her system out.  The poor thing spent 5 hours trying and 5 hours vomiting! I figured there was no way they were going to get a look at the colon.

She had all of the procedures done Thursday.  They premedicated her with Dr. Castells protocol so that it lessened the chance of activating her mast cells.  She has not done as well as usual since we left Rapid City so I was a little nervous.  She displayed minor symptoms both in pre and post up and then once she was admitted to the floor but they were able to keep it all under control with medications.  It was nothing like her history of reacting in the past (when she was not on medications as we had no idea back then)... so I was very pleased as to how this all went.  The GI doc almost did not do the scopes as Bries cortisone level is very low but he deferred the decision to the Anesthesiologist (she had 2 of these due to her situation) and they assured all involved that they felt confident they could control whatever happened... and a marvelous job they did!!  They are the ones who said she would have to be kept overnight in the hospital to be monitored for any further problems.  She has only had mild symptoms although she has been sleeping almost constantly (which in itself is one of her symptoms). Some back pain from the BMB but this is controled pretty well with over the counter pain meds. We think she will be discharged yet today and we will lay low in the hotel and fly back home Sunday.  Brie is so ready to get home as she "misses her grandma and can't even remember what home looks like."  

Now it is time to sit back and wait patiently as all biopsies and special stains won't be back for 2 to 3 weeks so it may be a month before we have any answers. Dr Castells will then follow Brie every 6 months to 1 year and she will work with Bries doctors back home to help with her medications, ongoing care and the need to pursue answers to her Adrenal Gland issues.

So glad to have this part of things behind us... some answers coming... and a plan for the future can then be made.

                                                                                                                                                                               Kim

Hi Kim,

  So happy to hear that the testing is finished and that Brie made it through very well!  I hope you get good news and that Dr. Castells can help get her symptoms under control.

  You've been amazing and your persistence is paying off.  At least she now has been seen at the best in the country and is getting expert advice.

  Keep us posted!

The credit here belongs to all of you!!! (not me)..... thanks for this forum, for each and every one of you sharing your experiences and advice, your love and support and all of the direction and guidance and persistant "pushing" to get Brie and I where we are at today.... this would have never happened if I would not have stumbled across this forum almost a year ago as I had no idea where to find the resources and specialists (even after over 6 months of searching for it on my own).  We love you all!!!

Kim,

I can't tell you how relieved and thankful I am that you got up to Boston IN TIME!!   Your doctors would NEVER have been able to figure this out without the help of those doctors there for the masto is a definitive part of all of this and without looking at the WHOLE PICTURE they could never figure out the finer details of it all!!  

Please tell Brie that I'm praying for her, and know that I'm here and are the others here to SUPPORT YOU and to STAND WITH YOU on this!  I know that all of us are praying for not just Brie and you but also for her doctors so that they will gain understanding and enlightenment as to how to deal with this and help Brie!!

Keep us updated as to what is happening here, PLEASE!!!   I WANT TO KNOW!!

Hugs and Kisses!!

Lisa

The good news is that Brie's BMB & GI Biopsies are negative for Mast Cells so the Dr.'s feel pretty confident saying she does not have Mastocytosis.  We are left with the diagnosis of MCAS and the Dr.'s will continue to follow her and her symptoms.  

Her colon is still filled with Lymphoid Hyperplasia but they do not know why this is.  It can at times be found in healthy children or Immuno Deficiency but so far Brie does not show this.

We are left with "Ideopathic" Urticaria and Angioedema.  She also is now testing positive for some allergies to dogs, cats, mold, pollen and grasses but this does not explain why she reacts some times to cats and dogs and other times not, or why she reacts when not around these or when she reacts all year round versus seasonally, etc.  

They want us to continue to pursue the GI issues and she will have to have an MRI of the colon and abdomen under anesthesia.  Their biggest concern right now is the Arenal Glands and Cortisol level of 0.6.  Her local Dr.'s are still scrambling to get all of the Boston records they need to to coordinate with our local hospital to do further testing for this and the MRI.  Sounds like Brie will be hopitalized for a couple days next week or the week after to accomplish this. I will update when I know more or have some results.

                                                                 Hugs,  Kim