Discouraging news - the biopsies are in

So yesterday we jumped right back into our routine of therapy and appointments after her procedure on Monday.  We started the morning with OT at 8am. She is working on some sensory issues in OT and her therapist, Ms. Heather, had a brand new dress up dress and crown for her to wear while she worked! (When it came to taking it off we were anything but smiles!)

After OT and feeding therapy we picked up 827 and Cowpoke and headed off to the eye doctor about 45 minutes away.  Our pediatric ophthalmologist is fabulous and he sees Miss Grace every 3 months.

She was wonderful in the waiting room waiting to see him and then was all smiles when she found out she didn't have to get her eyes dilated and just had to play the seeing games!

 

 

We did find out her vision continues to get worse. She was down to 20/80 with correction. He will see us again in 3 months and we will see what her vision is then and order new glasses at that point. Her eyes are really bad and he said LASIK can stop the vision loss but they don't do it until they stop growing and with girls is aged 16-18. That is a long way off.

 

 

 

 

We took 827 to work afterwards. There is a botanical gardens area right across from the police department. Grace always wants to go see the koi fish. Sadly it was to hot and they were all hiding deep in the pond and under the shade of the bridge. She settled for a picture with the flowers today. 

 



 

When we got home she got a surprise visit from her favorite big sister! She loves her so!


Today was back to OT, PT and feeding and then a fun afternoon coloring sidewalk chalk with Daddy. We had just gotten to the pool for some PT homework when her doctor called.

So now about the biopsies and what you have really been waiting for . . . Her eosinophil numbers are back up. She has to go back on the high levels of steroids. What that tells us is that there is still something or somethings in her diet that her body can not tolerate. The sad thing is that there is so little she can eat we are getting pushed to the lots of formula point that we have worked so hard in the past 18 months to move away from. It is hard to understand why something your body needs to survive (food) that it cannot handle. The doctor wants her on the high steroids again (and she cried when she had to take them tonight) and he will see her when he is here in 3 weeks. He said we need to postpone inpatient for 6-12 weeks and let her body rest and let the inflammation go down. Sadly this probably means she is loosing her beloved sunbutter (a peanut/nut free alternative to peanut butter of ground sunflower seeds), tapioca bread and bananas. It just makes me so sad for her. I pray that very soon a cure will be found for eosinophilic esophagitis and that she will be able to eat. I pray her joint pain from the high eosinophil levels will be gone and she can run and jump and play like other kids without the pain and discomfort. I pray her crooked legs and feet would straighten and the tremor in her hands from the cerebral palsy will be gone. I pray God would restore her vision or at least stop its loss. I know He is able. I know He loves my baby girl more than me and she is on loan to us from Him. I pray her story would impact nations and Christ would be glorified in her life. As always thank you for praying for my precious little girl.

Thankful for a smooth procedure

First off THANK YOU to everyone who prayed for me, 827 and Miss Grace today! We could feel them in a mighty way. It was amazing how God worked every little detail out - from opening up traffic lanes so we could get there on time to having the same anesthesiologist we had at a different hospital last time who has taken a special liking to our Grace. Every detail went smoothly. I was able to hold her as she woke up from anesthesia and they already had her IV and leads off of her. We were in a quiet room, with a really sweet recovery nurse who knew to have a purple popsicle ready before we even told her. It is amazing how attention to detail and quality patient care makes for such a positive experience. Today was a blessing and 827 and I commented several times it was totally God at work.

Here she is waiting in pre-op. She looks like such a big girl.  She knew what was coming and she was not happy about it but she held on to Gator Dog and she was okay.

We walk Miss Grace into the OR and give her a bear hug as they gas her. She doesn't do vercid ("goofy juice") well so we avoid that.  Gator goes all the way into the OR. She got upset so we let her hold him for a minute and get her breathing back under control. 827 held his leash and Gator's head was right by hers as she got the gas. He was not happy that we had to leave her in there. He was pulling on his leash in the waiting room and whining. There were a bunch of older adults in there waiting for procedures in the Heart Cath Lab and they kept looking at him. He wanted his girl back!

 

 

Waking up was so smooth. Our doctor and anesthesiologist were amazing. They really have taken time to know our girl and that means so much to me and 827. We were on the road and heading home within about 3 1/2 hours. Much better than the 8+ hours of her procedure in March. She was still tired. It was dark when we went into the hospital and she wasn't ready for it to be "good morning time."

 

 

 

Her biopsy pictures looked better than they did in March but not as good as they did in May. With eosinophillic esophagitis it is a biopsy based diagnosis so we have to wait and see what her eosinophil levels are. Those results should be back the end of the week. The plan will probably be to maintain the previous levels of steroids (so no weaning off like we had started) and keep her diet limited. Our doctor thinks going inpatient to do the feeding program to increase volume is the best plan so we will meet with them about that in a few weeks.

Thank you for praying for our girl! God has great plans for our warrior princess.

What a week

We started off this week with wonderful news in the research of eosinophilic disorders (see previous blog post). It is not a cure but it is a step forward in understanding the in's and out's of this horrible disease. One of the current methods of treatment is high does steroids to reduce swelling and inflammation and allow the patient to eat without pain or allergic reaction. 

Since Miss Grace was diagnosed with EoE in March she has been on a lot of steroids. We got her reflux under control thanks to a medicine change, but she was still on a lot of steroids. She was eating better in feeding therapy and we were please with everything but the fact that she couldn't rest and calm. Steroids make me crazy jittery just for 7-10 days much less taking multiple steroids long term. Cincinnati Children's Hospital and the endocrinology department is doing a study on children with EoE who have been treated with budesonide slurry for at least six months to see what effects long-term corticosteroid use has on the adrenal glands. There are still so many unknowns about long term steroid use in children and with everything else Grace has going on I do worry about that.  After her scope was good in June I pushed to wean off steroids and work on controlling disease with diet. First we eliminated one steroid at a time and then cut the dose of the budesonide slurry in half.  When we did that a lot of her symptoms reemerged.  I tried to ignore them because let's be honest, she is a much more pleasant child without all the steroids.  But she started not to eat well again.  Last week her feeding therapist commented about it and I tried to write it off as a bad session - after all we all have off days. But it has continued and more symptoms are reappearing; she is not sleeping well, complaining of joint paint, unable to swallow bites after 60-90 seconds of chewing and more. Then on Monday her OT and PT both commented that she has developed hand tremors when doing fine motor activities (writing, threading beads, stacking blocks). Was this a result of steroid weaning or because of her CP or something entirely new?  Feeding is always our last therapy of the day and after reviewing our volumes intake logs and having another difficult session our ST/feeding therapist said we needed to contact the doctor. We had an appointment already scheduled for today so she went ahead and called with her concerns about Grace. I knew in my Mama heart that it wasn't going to be good news. I have felt like a short order cook jumping through hoops and fixing anything that she even asked for, but she isn't eating.  She says she is hungry, you fix her food and she doesn't eat.  She has recovered her weight loss from this spring but overall she has only gained 1 ounce since January - that is 7 months.

So we woke up this morning and I posted on facebook that I could use some prayers as I was really anxious myself.  Our appointment was not until 12:30pm so when I got an email about a Home Depot project build at our local library we decided to head that way for some free fun!

Loving on Cowpoke - she thinks he hung the moon

All the hammering of the Home Depot projects got to be a little much for my sensory girl. She went to do a puzzle with Daddy. He ended up doing the puzzle and she snuggled Gator Dog.

Do you want to build a project?

 

 

Afterwards we headed home for an early lunch and then headed to north Dallas. Our GI came in and talked with us. He had already talked to our feeding therapist earlier in the week. He asked us a lot of questions and told us that it is suspicious and looks like she is flaring (having an allergic reaction) to something still in her diet. She is off of all dairy, soy, wheat, eggs, peanuts, tree nuts, fish, shellfish, beef and coconut.  Her diet is so restricted already we don't know what else could be triggering her. She has had no new foods introduced in her diet. He said he needed to look inside of her ASAP and see what is going on. So on Monday at 7:30am she will be scoped and biopsied again to see what is going on.  If her eosinophil levels are back up and her disease is progressing then we will go back on all of the steroids and we will wait 90 more days to let her body heal before going inpatient.  If her eosinophil levels are low we will know that this is behavioral (she is afraid to eat as she associates eating table foods with pain) and we will go back inpatient in September for 4-6 weeks in the feeding program at Baylor Dallas so we can up her food intake volume and wean back down off of her medical formula (the only food she associates as being safe). 

 

 

Waiting at the GI's office



So for now we try and ride through the weekend and wait it out for Monday.  As always the biopsy results take 5-7 days to return but with severe EoE he will be able to see immediately if there is furrowing, inflammation and irritation. We are going to watch the hand tremors and keep the neurologist advised. We go back and see him in 6 weeks and by that point we should have regained control of the EoE. So pray for a calm weekend and pray for a smooth procedure on Monday.  She does great with the procedure but she is a bear with anesthesia. We don't do vercid anymore, we carry her to the OR and hold her down while they gas her. That is hard on her and on my and 827. Please pray for a smooth process. No matter the results we will continue to fight and advocate for Miss Grace. I keep reminding myself God loves her more than we do and she is just on loan from him.  She is an overcomer and has survived more in 4 years than many of us face in a lifetime. Thank you for praying for my girl.

Study Finds Cause of Mysterious Food Allergy, Suggests New Treatment Strategy

A few days ago we received the most promising news from head researcher Dr Rothenberg. This is not a cure or even a treatment yet, but this is well on our way to something. Best news in the history of researching Eosinophilic disorders!

Study Finds Cause of Mysterious Food Allergy, Suggests New Treatment Strategy

Sunday, July 13, 2014
New research in Nature Genetics identifies a novel genetic and molecular pathway in the esophagus that causes eosinophilic esophagitis (EoE), opening up potential new therapeutic strategies for an enigmatic and hard-to-treat food allergy.


EoE is a chronic inflammatory disorder of the esophagus. The condition is triggered by allergic hypersensitivity to certain foods and an over-accumulation in the esophagus of white blood cells called eosinophils (part of the body’s immune system). EoE can cause a variety of gastrointestinal complaints including reflux-like symptoms, vomiting, difficulty swallowing, tissue scarring, fibrosis, the formation of strictures and other medical complications.


Reporting their results online, the multi-institutional team of researchers was led by scientists at Cincinnati Children’s Hospital Medical Center. The authors identified a molecular pathway specific to epithelial tissue in the esophagus involving a gene called CAPN14, which they found becomes dramatically up-regulated in the disease process.


Epithelial cells help form the membrane of the esophagus. The scientists report that when these cells were exposed to a well-known molecular activator of EoE – an immune hormone called Interleukin 13 (IL-13) – it caused dramatic up-regulation of CAPN14. The researchers said this happened in what they described as an epigenetic hotspot for EoE on the cells’ chromosomes.


CAPN14 encodes an enzyme in the esophagus that is part of the disease process called calpain14, according to Marc E. Rothenberg, MD, senior investigator on the study and director of the Center for Eosinophilic Disorders at Cincinnati Children’s. Because calpain14 can be targeted and inhibited by drugs, the study opens up new therapeutic strategies for researchers.


“In a nutshell, we have used cutting edge genomic analysis of patient DNA as well as gene and protein analysis to explain why people develop EoE,” Rothenberg explained. “This is a major breakthrough for this condition and gives us a new way to develop therapeutic strategies by modifying the expression of calpain14 and its activity. Our results are immediately applicable to EoE and have broad implications for understanding eosinophilic disorders as well as allergies in general.”


The study follows years of research into EoE by Rothenberg’s laboratory, including the development of novel modeling systems for the disease, and extensive multi-institutional collaboration through the National Institutes of Health’s Consortium of Food Allergy Researchers. Other key collaborators on the current study include first author Leah Kottyan, PhD, a researcher at the Center for Autoimmune Genomic Etiology at Cincinnati Children’s, and co-senior investigator John Harley, MD, PhD, director of the Center of Autoimmune Genomic Etiology.


Rothenberg’s lab years ago identified IL-13 as a key molecular contributor to the allergic reaction process in EoE. His team has since identified a number of related genes and molecular pathways linked to the disease, and they have tested drugs that inhibit IL-13 in an attempt to manage EoE severity.


“The current study links allergic responses mediated through IL-13 with an esophageal specific pathway, and answers a long-standing question in the allergy field of why people develop tissue specific disease manifestations,” Rothenberg explained. “We have uncovered that this can be explained by the interplay of genetic susceptibility elements in allergic sensitization pathways with the newly discovered esophageal specific pathway. Thus, two steps are necessary, one dictated by allergy and one dictated by calpain14 in the esophagus.”


The researchers used computer bioinformatics to conduct a genome-wide association study that analyzed 2.5 million genetic variants in thousands of individuals with and without EoE. This allowed the authors to identify the genetic susceptibility within the CAPN14 gene. The investigators were surprised to learn that CAPN14 was specifically expressed in the esophagus, compared with 130 other tissues in the body they analyzed.


Rothenberg said the findings open a new way to consider therapeutic options because calpain14 is an enzyme that can be inhibited by drugs, which means it may be possible to modify the expression and activity of calpain14. Some chemical compounds already exist that block the activity of calpains, although the researchers do not yet know the exact function of calpain14, as very little has been published about it.


Funding support for the study came in part from the National Institute of Allergy and Infectious Disease, the National Institute of Diabetes and Digestive and Kidney Diseases, the National Heart, Lung and Blood Institute, the National Human Genome Research Institute, the National Center for Research Resources (grant numbers U19 AI066738, U01 HG006828, U01 HG006828-S1, U01 HG006828-S2, U01 AI066560, R37 AI024717, P01AI083194, T32 HL7752-19, K23 AI099083, P01 AR049084, TR001082, UL1 TR-000067, UL1 TR-000039, UL1 TR-00083, UL1 TR-000424), the Department of Veterans Affairs (IMMA 9) and the Department of Defense (PR094002).


Additional support came from the Campaign Urging Research for Eosinophilic Diseases (CURED), the Buckeye Foundation, the Food Allergy Research Education (FARE) foundation and the Foundation of the American College of Allergy, Asthma, and Immunology.


Other NIH Consortium of Food Allergy Researchers institution collaborating on the study included: the Icahn School of Medicine at Mount Sinai, New York; Johns Hopkins University School of Medicine, Baltimore; University of Arkansas for Medical Sciences and Arkansas Children’s Hospital, Little Rock, Ark.; the University of North Carolina, Chapel Hill, N.C. and National Jewish Health in Denver, Col.

About Cincinnati Children’s

Cincinnati Children’s Hospital Medical Center ranks third in the nation among all Honor Roll hospitals in U.S.News & World Report’s 2014 Best Children’s Hospitals. It is also ranked in the top 10 for all 10 pediatric specialties. Cincinnati Children’s, a non-profit organization, is one of the top three recipients of pediatric research grants from the National Institutes of Health, and a research and teaching affiliate of the University of Cincinnati College of Medicine. The medical center is internationally recognized for improving child health and transforming delivery of care through fully integrated, globally recognized research, education and innovation. Additional information can be found at www.cincinnatichildrens.org. Connect on the Cincinnati Children’s blog, via Facebook and on Twitter.

Contact Information

Jim Feuer
513-636-4656
Jim.Feuer@cchmc.org

Road trip and Beach Vacation to Santa Rosa Beach, Florida 2014

Our last vacation in late summer 2012 was rough. Miss Grace didn't travel well, she did not sleep well in hotels, she wasn't pleasant to be around, she refused to participate in activities which meant one parent had to stay back with her and of course she didn't eat well.  To say I was concerned about a repeat of that vacation would be putting it mildly. Thankfully that did not happen!

We left our early on the morning of June 27th. 4AM early. Here is a photo diary of our trip . . .

 

Welcome to Louisiana! Time to wake up the kids and change out of pajamas!

 

 

We borrowed DVD players from friends and it made the trip much more enjoyable. Miss Grace watched FROZEN several times.

 

Welcome to Mississippi!

 

We hiked up a hill to look out over the mighty Mississippi River! Cowpoke wanted to be like Huck Finn and try to raft it!

 

Even Gator enjoyed the hour or so we spent exploring at the state line. They even had a dog walking trail which was super nice.

Time for an afternoon nap.

 



 

Welcome to Florida Visitors Station provided free samples of fresh squeezed orange and grapefruit juice

 

 

Look! It's a Blue Angels plane! They are based out of Pensacola Naval Air Station. Our cousin is at the Naval Academy so we took lots of pictures for her!

 

Even Gator was glad to be in Florida! Not much longer until our destination!

 

When I lived in Gulf Breeze, FL, I loved to get fresh seafood straight off the boats at Joe Patti's. I love to take my family there! We got awesome fresh Amberjack, Blue Crabs and Shrimp. The kids were so excited and the sales girl let them look, touch and ask a zillion questions!

 

When we got back on I-10 Cowpoke spotted this boat! We died laughing and had to get a picture for our Gator. Sorry about the reflection.

 

 

We went out to walk the beach our first night and of course had to play in the surf

 

Exhausted after a very full day!

The first few days she was very leary and kept right by an adult and away from the surf almost the entire time

 

 

But she slowly got braver and closer to the water thanks to a lot of coaxing and encouragement from Uncle Matt and Aunt Ali

 

She LOVED the pool at "the fun house" as she called it. We had a fantastic 4/5 beach rental with a private pool just a short 5 minute walk to the beach. It was amazing! We have stayed at beach rentals before but nothing like this house. It was fantastic! If you want more info message me. It was worth every penny!

ou

 

We spent Tuesday on a girls shopping day in Seaside. It was to crowded for Miss Grace and she did not like all the people but she used her words to tell us and she didn't have a meltdown.

 

We even found a snowcone stand. It didn't have a safe FROZEN (blue coconut & wedding cake) snowcone like Ozzy's, but we settled on a clear coconut and it sufficed for a cold treat on a hot day.

 

She finally put her feet it on day 4!

 

Gator was keeping a watchful eye over his girl!

 

 

She even ASKED Aunt Ali to help her boogie board at the very edge of the surf

 

And I got conned into this mermaid doll by a local shop keeper. This doll floated out to see within about 18 hours of us owning her . . . . oh the memories!

 

Loving the beach!

Dancing with her Daddy before our family photo shoot

 



 

Gator enjoyed relaxing poolside as his girl swam

 

Evening walks on the beach were a hit with Gator

 

During the day he stayed under the canopy and kept his eye on Miss Grace

 

When she needed some snuggles she would come get them

 

Grace enjoyed making "strawberry sand cakes" and "sand tcookies"

 

 

Thankfully the local Publix Grocery Store had Sunbutter and Tapioca Bread! We ran out! We even got Chocolate Sunbutter bars but doesn't like them.

 

Playing with Pop Its on July 4th!

 

Ready for fireworks on the beach!

 

The fireworks were gorgeous! Grace and I agreed our favorite were all the Chinese Lanterns like they had in the movie Tangled. They were gorgeous and you could see them for a long, long time after  they were released. We must get some before we go next year!

 

All in all it was a FANTASTIC trip that we all very much needed. We enjoyed our break from swimteam, work and all our therapies and doctors appointments. While we did still see some issues with Miss Grace, she adapted much better than expected and all the adults in our group commented on how well she did. We are blessed! Now it is back to the grind . . . .

 

A food milestone!

It is no secret that Miss Grace has some severe feeding issues.  She loved peanut butter, but she lost peanut butter after her March scope and she has not had many foods she can eat on the go without cooking.  We tried sunbutter and tapioca bread right after she lost peanut butter and she refused. We mixed it with honey and then with brown sugar and she refused to eat it. Tapioca bread is dense like sourdough bread and she wasn't fond of that texture either. I have been very concerned about feeding her on the road and while we were going to be gone on vacation. At feeding therapy on Thursday, June 26th we tried again and she loved it! She ASKED for a sunbutter and tapioca bread sandwich with brown sugar and cinnamon. Her wish is my command! Here she is eating her sandwich. This is HUGE for us!

VBX 2014

Miss Grace was so excited to attend VBX at our church! VBX was held for 4 nights from 6-8:30pm.  The kids attended a worship rally, snack time, arts & crafts, Bible lesson, recreation and closing show (everything from a professional jump roper, a juggler and Christian Wrestling Federation show) each night. It was a jam packed week full of fun and excitement! There were 4 classes of 4 year olds and we had 38 kids in our class plus Gator! Our church has done an awesome job in recognizing the fact that some kiddos are just wired differently. We have a special needs minister and she worked hard to make sure VBX was enjoyable for ALL children. She had a picture schedule of activities, a designated felt square to sit on during large group activities and noise canceling headphones for when everyone got really pumped up. We also had an ACCESS room where our kiddos could go and have some down time and regroup when and if needed. It was a HUGE blessing and it made VBX successful for Miss Grace. I love my church for many, many reasons and this is just one of the many reasons!  All in all a great time was had by everyone and an afternoon nap was guaranteed the next day too!

Grace was exited to spend time with her precious friend Eliza! They have been friends since Grace was 2 weeks old and Eliza was 7 weeks old!

 

Rocking the pink headphones! Thanks Mrs. Mellisa for having these for us to use!

 

 

Gator was listening to Pastor Wayne talk all about how our mission offering was going to be used to build a road in Guatemala!

 

VBX makes her so tired!

She took a nap EVERYDAY during VBX week!