A Whole New World

Yesterday Ellie performed a most typical toddler task, getting her fingers into our sticker bucket. Here is a picture of what resulted:


For those of you who recognize it, yes it is the sheet of millions of teddy bear heads from Lakeshore. This is quite significant. Why? Because not long ago the corn in the sticker adhesive would have sent Ellie into a full blown FPIES reaction within a matter of minutes or hours. For the first time I did not have to panic. She had no reaction (though I did remove them all immediately).

This is a whole new world for us. This is a sign that her body is healing from the inside out, and confirmation that the formula was keeping her from progressing. And let's not forget the advance in fine motor skills she needed in order to pick each of those little bear heads off of the paper and place them on her leg.

In the last few weeks Ellie has colored with crayons, wrote on her body with markers, played with stickers, and even touched plain old playdough. All with no reaction.

She still has limitations (obviously), and we still must be diligent with what she is exposed to (Bandaids still give her a rash), but this is MOST noteworthy of progress and we are VERY pleased to see her little body begin to take care of itself. All from the inside out.

A Quick Soap Revisit

I have been asked a lot about what soap we have found and use for Ellie. At this point I would love to say that I make her funk free soap at home, but really? Who has time for that?

We have actually been the most successful with Ivory Original bar soap. Its not great on her hair, and sometimes we fudge with a quick conditioner rinse using her sister's organic/natural conditioner and it does not seem to cause too much disruption. I have been told you can use the Ivory bars to even make laundry soap but we have not gotten there, yet, either!

For a break down on the Ivory soap story and ingredients here is a link to my previous post:
CLICK HERE!

Another Crash Course: Sugar Intolerance 101

After finally getting a copy of Ellie's dissacharide test, I realized I had no idea what I was looking at. The nurse had suggested they were concerning and very low, and with no current treating GI doctor I figured I had better get my fanny moving on what these new terms meant. What her test results showed, and what a quick google search along with consulting a few fellow FPIES mamas revealed:

Lactase - 4.8 (below 15 is abnormal, but normal starts at 24.5ish)
Sucrase - 11.6 (below 25 is abnormal, but normal starts at 54.4ish)
Glycoamylase - 0 (I have no idea what is abnormal but am pretty sure ZERO is not normal)
Palatenase - 2.8 (below 5 is abnormal, but normal starts at 11.1ish)

Keeping in mind that lab results always give a wide range that can be considered normal, these results were still extremely low. But what in the world were these things? They are enzymes. Digestive enzymes found in the intestine that split double sugars for proper processing by the body. Without these enzymes Ellie was unable to process these double sugars. So what are these double sugars?

Latase. OK got it. Sucrase. Alright. But glucoamylase? And palatenase? uh..... And since she is the lowest in these two, I really needed to know what they are. Here is what I found, perhaps over simplified, using my non-medical layman jargon:

glucoamylase is STARCH, that leads to proper digestion of maltase
palatenase is isomaltulose, that leads to proper digestion of FRUCTOSE

OK. So now that I knew what they were, I had to find out what this meant. What I quickly discovered was that these deficiencies could be genetic or acquired, and came with a whole list of other names for diagnosis. And they all had very similar symptoms to FPIES. And guess what? The medical community doesn't know anything much about those diagnoses either. What they do know is that the majority of intestinal disorders come with enzyme deficiency to some degree (more on this later).

*sigh* Back to the mommy brain to digest what this means (pun intended).

What I now know:
Ellie has sugar intolerance. Her intestines have virtually no digestive enzymes to break down sugars.

What I needed to know:
How do we get them back?
How does this affect what I feed her?

With almost four weeks until our next 'new patient' GI appointment I started my next medical crash course on digestive enzymes.

I Shopped The Black Market

Yes I did. For formula. Before we had a prescription that was approved by the insurance company. Off of craigslist because those with WIC can get it for free and resell it illegally. Yup. That's right. Welcome to our world. And before you judge me, remember that the first round of special formula that Ellie was on cost us $25 a can, and the formula she is currently prescribed and we are trying to keep her off of cost $45 a can.

While Ellie's formula is covered for the moment, many other moms are not as lucky, and their insurance (Kaiser) won't pay for the formula at all. Unless their child is hospitalized and given a feeding tube. I remember these days. Back when we first dealt with the transition and the insurance company told us that if we starved her until she was hospitalized they would pay for the formula, but that was the only way. Nice. I have other posts on here about formula back at the beginning. But another FPIES mama local to me is dealing with an outrageous monthly bill right now. She averages $800 a month. Another local mom has TWO FPIES kids on this formula and is paying out of pocket.

Please take the time to read another story here. And though I have encouraged her to put a paypal donation link on her blog, she hasn't (yet?). So if you would like to sponsor Carter's ability to eat with even a $5 donation, or by purchasing one can, let me know and I will make sure she gets it.

Broth, Broth, Baby, To Go...To Go....

This topic keeps coming up. Many have quickly recognized the work that Ellie's diet change is taking when it comes to mealtime, or when it comes to preparing her special diet. But most do not seem to understand the implications of trying to actually get out of the house. Here is my attempt to illustrate and compare:

When breastfed, babies are so easy to pack for. Mama? check. Diapers? check. Good to go. All fitting neatly in a normal - to - small size bag. Risk level: forgetting diapers means a stop at the grocery store.

When using commercial formula things are a bit more complicated, and at one point I had the nerve to complain about it. Bottles? check. Formula? check. Heated water? check. Diapers? check? Bag must be a bit larger, and may need to include a thermos for water. Risk level: forgetting any items requires a stop at the grocery store, or finding a restaurant that can provide hot water or ice.

When using prescription formula the diaper bag must get even larger, and is more inconvenient. Bottles? check. Entire can of formula? check. Heated water? check. Ice to cool down heated water if needed? check. Extra clothes for the child prone to vomit or blow out? check. Extra clothes for mama? check. Diapers? check. And in Ellie's case: Homemade wipes? check. Cloth diapers? check. As you can see the bag required is much larger to accommodate the specific needs of a special child. Risk level: substantial. Not enough formula packed means no food. The fear of getting stranded somewhere too far from home without anything to feed my child spurred me to keep a plastic tub in the back of my car with extra formula, bottles, diapers, clothes, bottled water, and whatever else we were restricted to at the time. That covered everything except for the issue of hot water, because Ellie refused to drink her bottle unless it was warmed properly, regardless of how hungry she got or how loud she yelled.

When on a special diet, or in our case, a diet of bone and meat broth, meat puree, and a vegetable, getting out the door becomes much more complicated. We start by packing the 'regular' stuff: diapers, wipes, extra outfit, throw in an empty bottle or two. Then we have to stop and evaluate. How long will be gone? What will we have access to? How far are we traveling away from home? How long will we be in the car? We have two thermoses we can use, that can sustain us for about 3 hours outside of the house. Longer than that we must pack an ice chest. And try to figure out how we would heat more broth. After considering this we begin warming the appropriate amount of broth, pureed meat and vegetable on the stove top. If we are going to be longer than a quick run to the library we have to make it hot enough to last, which is too hot to drink. We warm it and fill the thermoses, and then fill an additional small thermos full of ice to cool down the broth if she needs to eat before it has cooled enough. (This always makes me a bit nervous because it waters down the nutritional content and calories in her bottle, and is another reason we can not stay out too long)

The bag required at this point is rather large. In fact, our bag usually wont fit in the basket of our McClaren umbrella stroller. I am currently shopping for a large backpack that would be similar to one used hiking. And the risk level? Tremendous. I have already made the mistake of not packing enough broth for her on two separate occasions. She just ate more those mornings than she normally does, and the result was a hungry baby who had nothing to eat until we were able to get home.

The three hour mark is a crucial one. When we are out and she is refusing to eat, that is about how long we can stretch her before having full meltdown. At home she eats one 9 ounce bottle every 1.5 - 2 hours, on average. But due to her sensory issues, she sometimes shuts down when we are out and about and refuses to eat. It may be too much stimulus, she may be too busy, or the moon may just be in a funky position. Who knows. Sometimes we can confine her to a stroller and block out surrounding stimulus, or put her in the carseat, and she will be agreeable and eat, allowing us to stay out longer.

Overall, part of our new normal requires us to be at home much more than before. Not because we have become anti-social hermits, but because it can be damaging to Elianna. Or because I have to make broth. And sometimes because I am too pickin tired to deal with the checklist. So for those of you who have gone out of your way to visit us, sometimes driving quite a distance, thank you. Thank you for allowing my two girls some play time, and for allowing this mommy to enjoy company where I know I have everything I need for Ellie. And for those times I am able to get out of my house, thank you for understanding our limitations, and allowing me to use your stove top (but I will bring my own pot)!

Where, Oh Where, Did Our Good G.I. Go?

We had a great GI. He diagnosed Ellie with MSPI at 12 weeks old and FPIES shortly after the introduction of food. This, in the FPIES world, is unheard of. Why? Because for most doctors around the country FPIES is unheard of.

Our GI landed in Sacramento after learning from some great FPIES docs in Chicago. He was the new Pediatric GI in his office, and most likely why we got in to see him. He was supportive, listened, and had an amazing amount of knowledge. Sometimes he still gave me the 'you are a crazy mom' look, but that was OK, because he followed it up with 'I have no idea. That makes no sense.' He got us diagnosed. He got us moving forward. And I am convinced he saved Ellie's life. And he left. BUMMER!

About two months ago the news escaped through the local FPIES mama's that he was expecting his first child and moving to Texas to be near family. DARN! I scheduled a follow up for Ellie right away, and pushed for him to do the upper biopsy and dissacharidases test that we had been putting off. After looking at me and saying, "My leaving is not a good reason to do a biopsy," I further discussed with him why I felt it was time to do it and how there were a few remaining questions that needed to be answered for Ellie. He agreed. (I also did not want him to leave and then have one of the other, less knowledgeable doctors, perform these tests or know nothing about them....but I kept that part to myself).

Ellie's procedure was performed about 5 weeks before he was to leave. I could have sworn he said he was leaving April 1. We were not having success moving Ellie past the meat broths and onto vegetables, and she had huge issue with carrot, which made me pause to wonder if the dissacharide (sugar intolerance) test would shed light on this. I then realized it was the last week of March and I had not heard from the GI. Wait a minute! He had emailed a few times with preliminary results, and said he was waiting on the final test to provide a treatment plan.

The last Monday of March I called the GI office to check in, and was told - "He is gone." What!?!? I was MAD. He told me he would check in before leaving! He just left??? Just like that??? Now what was I to do? I asked to speak with his nurse and waited for a call back.

And that was when things changed. It was as if we had been sucked into a new realm. I received a glimpse of what it must be like for FPIES moms who had to fight from the beginning, received no help, and some of who even were accused of Munchhausen by proxy.

The new nurse is who called. To tell me that the results were lost and had to be requested again. And that there were no open appointments to get Ellie in to one of the other GI docs, even though she needed to be seen and had a food fail. Did she need to go to the emergency room? No. OK well the next appointment was not for 4 weeks or more. Who did I want her to see? Dr. D was scheduled out to May, and Dr. M the end of April. Why? Because Ellie is a new patient. I argued. But they want to have enough time for the first appointment. Ridiculous. Call me with the results when they get in. Get me the results of the test.

Another phone call. What are the test results? Can't give them out because no doctor has read them to interpret. Give me the results! Here are the numbers but I can't counsel you on what they mean. They are low - very low. I would believe that she needs to be seen sooner rather than later so you need to see Dr. M. OK. Soon. How soon? The end of April. That doesn't sound like a priority appointment. It's all they have. Another phone call. Has a doctor seen the test at this point? Yes I can read you his notes. Please do. His notes say that he does not believe the results to be consistent with CSID and that the other numbers do not make sense. There was an error with the biopsy.

There was an ERROR with the BIOPSY? He is saying Dr. B made an error with this test and none of the others that were FINE?

No. He is not saying Dr. B made the error. He is saying the lab made the error.

OH...that lab that is one of the only ones in the country to even do this lab?? The EXPERT lab?

Silence.

I would like a copy of the results so I know how to feed my daughter. Have the nutritionist call me. And try to get us in sooner.

And with that I knew it was time for a change. I went from an understanding doctor who never made us wait more than a few days to be seen because of the urgency of Ellie's condition, to an office that could not fit her in. This is not acceptable to me, and I really don't care how short handed they are after losing our GI. I don't care that the new nurse does not know what FPIES is, or that the remaining GI's do not know Elianna's history. I don't care who has won what award for fantastic medical contribution, or how great of a colonoscopy these other GI's can perform. I care that my daughter needs to be seen by a knowledgeable doctor when she is ill, and that does not wait four weeks unless you live in a country other than this one with less than stellar medical care. I am not new to this. What is that expression? This is not my first time at the rodeo? I am not the new FPIES mama looking for an answer and being sent away with 'she has the flu'. I am a Professional FPIES mama. And I come equipped with experience, information, medical studies, and an attitude of advocacy for my child.

Hello Dr. M. Nice to meet you.

More on Probiotics

For those of you are looking for more info on probiotics, here is a blog post from another FPIES mama who is also a Registered Dietetic Technician.

CLICK HERE :)