Finally, a diagnosis!

We met with Owen's Immunologist on July 11. This was our first visit with her and I completely love Dr. B.

Remember the story of Landon I posted here? Well, I was right. Owen has FPIES.

We are so grateful for the diagnosis and are confident Owen's journey is nearing an end. He is almost 2 and most children grow out of this by 3. We leave next week for vacation and I am busy at work preparing to be gone. Until I have more of a chance to fill you in, here is a description of FPIES.

What Does FPIES Stand For?
FPIES is Food Protein-Induced Enterocolitis Syndrome. It is commonly pronounced "F-Pies", as in "apple pies", though some physicians may refer to it as FIES (pronounced "fees", considering food-protein as one word). Enterocolitis is inflammation involving both the small intestine and the colon (large intestine).

What is FPIES?
FPIES is a non-IgE mediated immune reaction in the gastrointestinal system to one or more specific foods, commonly characterized by profuse vomiting and diarrhea. FPIES is presumed to be cell mediated. Poor growth may occur with continual ingestion. Upon removing the problem food(s), all FPIES symptoms subside. (Note: Having FPIES does not preclude one from having other allergies/intolerances with the food.) The most common FPIES triggers are cow's milk (dairy) and soy. However, any food can cause an FPIES reaction, even those not commonly considered allergens, such as rice, oat and barley.

A child with FPIES may experience what appears to be a severe stomach bug, but the "bug" only starts a couple hours after the offending food is given. Many FPIES parents have rushed their children to the ER, limp from extreme, repeated projectile vomiting, only to be told, "It's the stomach flu." However, the next time they feed their children the same solids, the dramatic symptoms return.

What Does IgE vs Cell Mediated Mean?
IgE stands for Immunoglobulin E. It is a type of antibody, formed to protect the body from infection, that functions in allergic reactions. IgE-mediated reactions are considered immediate hypersensitivity immune system reactions, while cell mediated reactions are considered delayed hypersensitivity. Antibodies are not involved in cell mediated reactions. For the purpose of understanding FPIES, you can disregard all you know about IgE-mediated reactions.

When Do FPIES Reactions Occur?
FPIES reactions often show up in the first weeks or months of life, or at an older age for the exclusively breastfed child. Reactions usually occur upon introducing first solid foods, such as infant cereals or formulas, which are typically made with dairy or soy. (Infant formulas are considered solids for FPIES purposes.) While a child may have allergies and intolerances to food proteins they are exposed to through breastmilk, FPIES reactions usually don't occur from breastmilk, regardless of the mother's diet. An FPIES reaction typically takes place when the child has directly ingested the trigger food(s).

What is a Typical FPIES Reaction?
As with all things, each child is different, and the range, severity and duration of symptoms may vary from reaction to reaction. Unlike traditional IgE-mediated allergies, FPIES reactions do not manifest with itching, hives, swelling, coughing or wheezing, etc. Symptoms typically only involve the gastrointestinal system, and other body organs are not involved. FPIES reactions almost always begin with delayed onset vomiting (usually two hours after ingestion, sometimes as late as eight hours after). Symptoms can range from mild (an increase in reflux and several days of runny stools) to life threatening (shock). In severe cases, after repeatedly vomiting, children often begin vomiting bile. Commonly, diarrhea follows and can last up to several days. In the worst reactions (about 20% of the time), the child has such severe vomiting and diarrhea that s/he rapidly becomes seriously dehydrated and may go into shock.

What is Shock and What are the Symptoms?
Shock is a life-threatening condition. Shock may develop as the result of sudden illness, injury, or bleeding. When the body cannot get enough blood to the vital organs, it goes into shock.
Signs of shock include:
Weakness, dizziness, and fainting.
Cool, pale, clammy skin.
Weak, fast pulse.
Shallow, fast breathing.
Low blood pressure.
Extreme thirst, nausea, or vomiting.
Confusion or anxiety.

How Do You Treat an FPIES Reaction?
Always follow your doctor's emergency plan pertaining to your specific situation. Rapid dehydration and shock are medical emergencies. If your child is experiencing symptoms of FPIES or shock, immediately contact your local emergency services (9-1-1). If you are uncertain if your child is in need of emergency services, contact 9-1-1 or your physician for guidance.

The most critical treatment during an FPIES reaction is intravenous (IV) fluids, because of the risk and prevalence of dehydration. Children experiencing more severe symptoms may also need steroids and in-hospital monitoring. Mild reactions may be able to be treated at home with oral electrolyte re-hydration (e.g., Pedialyte®).

Does FPIES Require Epinephrine?
Not usually, because epinephrine reverses IgE-mediated symptoms, and FPIES is not IgE-mediated. Based on the patient's history, some doctors might prescribe epinephrine to reverse specific symptoms of shock (e.g., low blood pressure). However, this is only prescribed in specific cases.

What are Some Common FPIES Triggers?
The most common FPIES triggers are traditional first foods, such as dairy and soy. Other common triggers are rice, oat, barley, green beans, peas, sweet potatoes, squash, chicken and turkey. A reaction to one common food does not mean that all of the common foods will be an issue, but patients are often advised to proceed with caution with those foods. Note that while the above foods are the most prevalent, they are not exclusive triggers. Any food has the potential to trigger an FPIES reaction. Even trace amounts can cause a reaction.

How is FPIES Diagnosed?
FPIES is difficult to diagnose, unless the reaction has happened more than once, as it is diagnosed by symptom presentation. Typically, foods that trigger FPIES reactions are negative with standard skin and blood allergy tests (SPT, RAST) because they look for IgE-mediated responses. However, as stated before, FPIES is not IgE-mediated.

Atopy patch testing (APT) is being studied for its effectiveness in diagnosing FPIES, as well as predicting if the problem food is no longer a trigger. Thus, the outcome of APT may determine if the child is a potential candidate for an oral food challenge (OFC). APT involves placing the trigger food in a metal cap, which is left on the skin for 48 hours. The skin is then watched for symptoms in the following days after removal. Please consult your child's doctor to discuss if APT is indicated in your situation.

How Do You Care for a Child With FPIES?
Treatment varies, depending on the patient and his/her specific reactions. Often, infants who have reacted to both dairy and soy formulas will be placed on hypoallergenic or elemental formula. Some children do well breastfeeding. Other children who have fewer triggers may just strictly avoid the offending food(s).

New foods are usually introduced very slowly, one food at a time, for an extended period of time per food. Some doctors recommend trialing a single food for up to three weeks before introducing another.

Because it's a rare, but serious condition, in the event of an emergency, it is vital to get the correct treatment. Some doctors provide their patients with a letter containing a brief description of FPIES and its proper treatment. In the event of a reaction, this letter can be taken to the ER with the child.

Is FPIES A Lifelong Condition?
Typically, no. Many children outgrow FPIES by about age three. Note, however, that the time varies per individual and the offending food, so statistics are a guide, but not an absolute. In one study, 100% of children with FPIES reactions to barley had outgrown and were tolerating barley by age three. However, only 40% of those with FPIES to rice, and 60% to dairy tolerated it by the same age.

How Do I know If My Child Has Outgrown FPIES?
Together with your child's doctor, you should determine if/when it is likely that your child may have outgrown any triggers. Obviously, determining if a child has outgrown a trigger is something that needs to be evaluated on a food-by-food basis. As stated earlier, APT testing may be an option to assess oral challenge readiness. Another factor for you and your doctor to consider is if your child would physically be able to handle a possible failed challenge.

When the time comes to orally challenge an FPIES trigger, most doctors familiar with FPIES will want to schedule an in-office food challenge. Some doctors (especially those not practicing in a hospital clinic setting) may choose to challenge in the hospital, with an IV already in place, in case of emergency. Each doctor may have his or her own protocol, but an FPIES trigger is something you should definitely NOT challenge without discussing thoroughly with your doctor.

Be aware that if a child passes the in-office portion of the challenge, it does not mean this food is automatically guaranteed "safe." If a child's delay in reaction is fairly short, a child may fail an FPIES food challenge while still at the office/hospital. For those with longer reaction times, it may not be until later that day that symptoms manifest. Some may react up to three days later. Delay times may vary by food as well. If a child has FPIES to multiple foods, one food may trigger symptoms within four hours; a different food may not trigger symptoms until six or eight hours after ingestion.

How is FPIES Different From MSPI, MSPIES, MPIES, Etc.?
MPIES (milk-protein induced enterocolitis syndrome) is FPIES to cow's milk only. MSPIES (milk- and soy-protein induced enterocolitis syndrome) is FPIES to milk and soy. Some doctors do create these subdivisions, while others declare that milk and soy are simply the two most common FPIES triggers and give the diagnosis of "FPIES to milk and/or soy."

MSPI is milk and soy protein intolerance. Symptoms are those of allergic colitis and can include colic, vomiting, diarrhea and blood in stools. These reactions are not as severe or immediate as an FPIES reaction.

Where Can I Find More Information?
Fogg MI, Brown-Whitehorn TA, Pawlowski NA, Spergel JM. (2006). Atopy Patch Test for the Diagnosis of Food Protein-Induced Enterocolitis Syndrome. Pediatric Allergy and Immunology 17: 351–355. Retrieved on December 31, 2007 from http://pediatrics.aappublications.org/cgi/content/abstract/120/Supplement_3/S116.

Burks, AW. (2006). Don't Feed Her That! Diagnosing and Managing Pediatric Food Allergy. Pediatric Basics. Gerber Products Company: 115. Retrieved on December 31, 2007 from http://www.gerber.com/content/usa/html/pages/pediatricbasics/articles/115_01-dontfeed.html.

Moore, D. Food Protein-Induced Enterocolitis Syndrome. (2007, April 11). Retrieved on December 31, 2007 from http://allergies.about.com/od/foodallergies/a/fpies.htm.

Sicherer, SH. (2005). Food Protein-Induced Enterocolitis Syndrome: Case Presentations and Management Lessons. Journal of Allergy and Clinical Immunology Vol. 115, 1:149-156. Retrieved on December 31, 2007 from http://www.jacionline.org/article/PIIS0091674904024881/fulltext.

Nowak-Wegrzyn, A., Sampson, HA, Wood, RA, Sicherer, SH. MD, Robert A. Wood, MD and Scott H. Sicherer, MD. (2003). Food Protein-Induced Enterocolitis Syndrome Caused by Solid Food Proteins. Pediatrics. Vol. 111. 4: 829-835. Retrieved on December 31, 2007 from http://pediatrics.aappublications.org/cgi/content/full/111/4/829#T1.

Nocerino, A., Guandalini, S. (2006, April 11). Protein Intolerance. Retrieved on December 31, 2007 from http://www.emedicine.com/ped/topic1908.htm.
WebMD Medical Reference from Healthwise. (2006, May 31). Shock, Topic Overview. Retrieved on December 31, 2007 from http://www.webmd.com/a-to-z-guides/shock-topic-overview.

American Academy of Allergy, Asthma and Immunology. (2007). Tips to Remember: What is an Allergic Reaction? Retrieved on December 31, 2007 from http://www.aaaai.org/patients/publicedmat/tips/whatisallergicreaction.stm.

Sicherer, SH. (2006). Understanding and Managing Your Child's Food Allergies. A Johns Hopkins Press Health Book. 336.

Magic Cream

I meant to post a story about magic cream months ago (November) but never got around to it. This is what it was:

Yesterday Owen had blood drawn, again. I am really seeing that all the moms that go in and out of TCH look out for each other. For example, the last time Owen had blood work a little girl (5 or so) started talking to us. She proudly showed off her ‘magic cream’ and asked why Owen didn’t have any. I looked over to her mom sheepishly and asked “what is magic cream” and she told me there was numbing cream they put on the kids so they wouldn’t feel the needle. Say what? I hadn’t been told about this. So we went to the front desk and asked for the numbing cream. As it turns out, the doctor ordering the work-up had to request it. Dr. K did not … So this time, when we were handed our lab order, we asked for it!!! Poor guy still hated being restrained but he didn’t even flinch when they inserted the needle. Awesome! I love that I was about to make it a little better for him. And what made it even better, I was able to pass the information on to another mom. Her poor son was 12 or so and had been having blood drawn for years and they never heard it either. Come on doctors.

On June 1, Owen got magic cream again…this stuff really is amazing. He didn’t shed ONE tear. In fact, he didn’t fight at all. Sure does make the whole process a lot more bearable. I will be forever grateful to that sweet little girl who passed along this helpful info.

Plan.

On June 1 we went to see Dr. K. Owen wasn’t scheduled for his regular 3 month visit until June 22 but after some interesting moments and weeks, our new pediatrician called to tell him he needed to see Owen sooner.

In recent weeks, we have had numerous high temperatures (104.8 being the highest), his entire body covered in a rash that looked like hives, a patch of eczema that won’t go away, the accidental consumption of wheat and dairy, and a lot of bloody diapers.

I am tempted to believe Dr. K that we now have a plan to figure out what is going on, but I don’t want to get my hopes up.

As I have mentioned before, Owen was on Nutramigen AA (no dairy) from January 2010 through January 2011 but we took him off when things seemed to be getting better and put him on Nutramigen (does contain some dairy but it is broken down to its most elemental form). On June 1 we took four more vials of his blood. Lots of tests are being run but the one I am most immediately concerned with, is a test to determine if we need to put him back on Nutramigen AA. I should hear the results tomorrow. If we need to take a step back, I will do so in a heartbeat. Whatever is best for him, we will do. But, I would be very sad if this were the case. It is very expensive and we are fortunate to be able to afford this. It isn’t the price that makes me sad. It is the fear that he will have to drink this for the rest of his life. We shall see…

On July 11 we are meeting with an immunologist at Texas Children’s. If the immunologist cannot answer what plagues Owen, Dr. K promises to schedule Owen for an upper GI biopsy, colonoscopy, and liver biopsy. I am praying that we have answers by August 13 (the year anniversary from when we starting seeing Dr. K).

A good visit.

Yesterday was our appointment with Dr. K. It was the most positive/productive of all our visits. Unfortunately, Owen has lost two pounds since Jan. 10. And one of those has been in the last week. I think this got his attention from the get-go. Owen is supposed to be gaining weight, not losing it! Dr. K immediately jumped in with questions trying to ascertain if O had had a stomach bug. Nope. He had had congestion pretty much since January so I had taken him to the Ped. last week. They wanted to put him on meds but were hesitant so they told us to give him Zyrtec. Dr. K interrupted me and said he doesn’t want O taking antibiotics. Hello? Why are you just now telling me this??? Anyway, that is a battle I wasn’t willing to start so I acted surprised and allowed him to move on. Dr. K seemed okay with the Zyrtec until I explained to him what happened last Friday.

Last Thursday night we gave Owen Zyrtec as directed by his pediatrician. I don’t recall it being a particularly difficult night. He went to school and everything seemed fine. But when I picked him up, he just didn’t look right. His face was slightly swollen, he had bright red eczema patches on both cheeks and he was not responding to anyone. His eyes were glazed over … he was not acting like O.

It scared the crap out of me. But, it wasn’t “new”. We had seen this behavior before. Back in August at his 10 month check-up, I bawled in his pediatrician’s office because I was convinced my son was autistic. The pediatrician was concerned with some of his behaviors but he wasn’t willing to diagnose him and basically told me there was nothing I could do. Fast-forward 7 months and I no longer think he is autistic. I do, however, think some foods cause him to act that way. And of all his reactions, this is the one that scares me the most.

Once I described this reaction to Dr. K, he said next time it happened, I needed to take Owen to the ER immediately. The doc agreed that it could have been the Zyrtec or he could have gotten into something at school, either way it was bad, a sign of something much worse and I needed to have him checked immediately should it happen again.

I shared the article I mentioned in my last post with Dr. K and he agreed to do a colonoscopy to see what is going on in there. But, he is more concerned with his liver right now so that is where we will focus in the immediate future. We had another round of blood work (that is not getting any easier) and are awaiting the results. A liver biopsy may be scheduled or another upper GI endoscopy. Dr. K wants to go back and look at O’s liver’s reaction to certain food proteins.

We were told to take him off Coconut milk and to get him back up to 40 ounces of Nutramigen (this means more night feedings because we won’t want to take away his solids during the day). We were also told to start giving him DuoCal, a powdered nutritional supplement to increase his caloric in-take.

We have a meeting with his school on Monday to make sure everyone is doing everything they can to give Owen a safe environment.

And, we discussed vaccines again. While at the pediatrician’s office last week, they pushed for me to vaccinate again and pushed that I get the approval from Dr. K. Nope. Dr. K won’t give it. He says the only way he would allow Owen to be vaccinated is if we met with an Immunologist. While I am totally okay having Owen unvaccinated for now, it is a concern in the back of my head. Am I hurting him by allowing him to remain un-vaccinated? But, I am scared to death to inject him with that stuff. It will be so nice to have someone look at O and say what is safe and what is unsafe for him. I didn’t even know this was available. And frankly, I think it should be available to EVERYONE! Sadly, it is a fact that vaccines do cause harm to some people – why else would they have a vaccine fund to help those injured? Most parents just don’t know if their child will be okay being vaccinated until it is too late. But they are pushed on us for the good of society. A sacrifice for the greater good they say. Hum? Anyway, I am so grateful that Dr. K is making this available to us. So grateful!!!!!!!!!!!

As soon as I know more, I will post more. Have a great night!

More doctors...

More Doctor visits…

My Aunt forwarded me this story. I want to start off by saying I in no way think Owen has it this bad and I am forever grateful for that reality. However, I do think he has a more mild version. And I am frustrated to no end for this mom, me and so many others out there that are not getting the help we need. I have heard of FPIES before but I dismissed it…until I read Landon’s story. Now, I am not so sure. Owen has exhibited every symptom Landon has. The only difference is that Owen is thriving. That wasn’t always the case. He was born underweight and remained that way until late January of 2010. I have said this before and I will say it again, Owen is thriving only because of the Nutramigen AA (elemental formula). A friend recommended this to me after I cried to her about Owen (note our pediatrician didn’t recommend it) and within a day of putting him on it, he was a different baby. Just because someone was kind enough to point me in this direction and just because we had the financial ability to pay $700 per month to feed our baby, it does not mean that he doesn’t need a diagnosis. He is still sick. If it weren’t for this formula, I am confident Owen would have been classified as failure to thrive. After starting the Nutramigen AA he jumped to the 90th percentile in a few weeks!

So we are going in for a follow up with Dr. K on Wednesday. I am scared. I plan to walk in armed with this article. I am praying he listens.

Then next week, I am interviewing a new pediatrician. We like our current one okay but I think if we found another who was a better champion for Owen’s cause, life would be a little easier (or at least it would be a little easier to get a new GI doc or to get the one we have to listen to us).

A bad day.

I am an attorney. Not sure if I have mentioned that. Anyway, my job is pretty demanding. It is a great job, and I love it, but the demands at work sometimes can be too much – especially having a child whose needs are different and slightly more than our other child’s. Lately, I have had my head in statutes, codes and case law preparing for a CLE presentation I had to give today. I am a transactional attorney. I chose to be a transactional attorney because I do not, absolutely do not, like getting up and talking in front of people. But this was a necessity so I did it. And at 4 yesterday, while in the middle of putting final touches on my paper and trying my hardest to fight the swarm of butterflies that were beginning to build up in my stomach, I got a phone call from Owen’s school. Apparently, he had had a bad day. Owen’s teacher is the sweetest girl. She is full of joy and faith and I am so grateful he is with her; but, I don’t think she understands his GI issues. She proceeds to tell me that he was crying out in pain all day, that he didn’t want to be held, that he was hunched over like his tummy hurt and that he refused to nap. This broke my heart. One, I wasn’t there to hug him, whisper to him, try and distract him (all things I do in the middle of the night) and two, he has rarely had issues during the day. In fact, I don’t think he has had issues during the day since he went on Nutramigen AA back in January of 2010. Does this mean it is getting worse? But the worst part, Owen’s teacher said she thinks it was just a normal tummy ache like all kids get. I wish it were that. I really, really do. But I am his mom. I have another child who is ‘normal’ and gets ‘normal tummy aches’ and what he experiences is anything but normal. I am choosing to believe that she was just trying to make me feel better and I am choosing to ignore the lies that want to creep in that say I am imagining it. I am choosing to believe that she really does get it and that she really does watch him like a hawk during the day to make sure he doesn’t get into something he shouldn’t. I am choosing to believe it is just the shoe-string potatoes that I let him have the night before.

I am not imagining this.

So back to my job and that presentation that was looming…it went great. But Owen did have a rough night last night full of more screaming and more cramping. I gave my presentation on little sleep and now, I am so happy yesterday and today are behind us. I look forward to putting the kids down and drinking wine. I just wish Chad would be home tonight to help celebrate and relax with me. Darn HLSR 

New Stuff.

O wow, it has been over a month! Again, so much has happened, I don’t know where to begin. How about Coconut Milk?!? Yes, Owen is drinking this fabulous stuff. We do half coconut milk, half Nutramigen. Man o man is it tasty (a little thick for my liking but yummy nonetheless). His diet now consists of a white flakey fish, boars head deli turkey (gluten free), cauliflower, mum mums, an occasional GF chicken nugget, and potatoes. We are having second thoughts though about the potatoes. They had become a staple but we have seen too much of old Owen so we are thinking it was/is the potatoes. He is currently suffering from a stomach bug - lots of vomiting and other stuff but no crying out in pain. I plan on making ground turkey for him once he passes this bugger thing.

We have fun news to announce…we bought a house! We are so excited and ready to get in there and make it our home. We were planning on waiting and saving until we could buy our dream home but the more we thought about it, the more the price tag on my “dream” home made us sick…and then this little gem popped up. It is going to take lots of love and attention but we will get there eventually. The best part about the house…it is super close to both sets of grandparents and just a miles’ walk from the world’s two best babysitters. We are a little worried that we won’t be so close to Texas Children’s anymore but they are building a West campus. Soon, it will be just like we never left.

We have a follow up with the GI doctor in March. Chad met with his neurologist last week (work kept me from going) and life seems to be settling down a bit. Last week and the week before were really, really hard with O and his poor tummy. He was waking more and crying out in pain more. Again, we have made some adjustments to his diet and hopefully, that will settle down too.