Tuesday, December 27, 2011

New baseline

If we are what we eat, then Juliana is a little lamb.

Two weeks ago I pulled dairy from Juliana's diet. She now eats nothing but lamb liver-based formula and she is doing very well on it! The eczema on her face actually increased for the first week or so before fading entirely. I've found that the eczema will return if I am not careful when measuring some of the ingredients. This has led me to believe that Juliana's eczema was a detox reaction.

She's not having any GI issues: no reflux, no diarrhea, no constipation, no diaper rash, no digestive pain. Her respiratory issues are gone. She's gaining weight. She's happy. Perhaps one of the most exciting things of all is that she seems a little less foggy and a little more interactive. Juliana has always been communicative and observant (when not in pain). But she's been only selectively interactive with us. For example, it's only in the past week that she's been smiling at us when we play peek-a-boo. In fact since switching to 100% lamb she has smiled much more overall. She has also just recently begun to mimic. I am hoping and praying that good nutrition will help her cognitive development.

This is our new baseline. I don't know where we will go from here. We are consulting with a GAPS-friendly nutritionist soon and I am hoping that her expertise will shed some light on our next steps.

Friday, December 23, 2011


Quite honestly, it's taken me awhile to feel thankful. After several months of trauma and drama, after many months of questions with no answers, after postpartum depression, after receiving bad news after bad news, after many failed attempts to help Juliana - I was not thankful. In fact a post from Ann Voskamp made me realize that I was on the fast track to bitterness:
“If God really works in everything, why don’t we thank Him for everything? Why do we accept good from His hand — and not bad?
This is hard. Maybe the hardest of all. 
I have held dying babies. Eaten with those who live on the town garbage heap. Wept with women who’ve been violated, with the bankrupt, the heart crushed, the terminal. And this never stops being true: Neglecting to give thanks only deepens the wound of the world. 
To thank God in all is to give God glory in all. Is this not our chief end?
After the realization, the confession, and the move to be intentionally thankful - things are better. 

This weekend I am spending Christmas with my family in a cabin in North Georgia. I am feeling so thankful for so many simple but wonderful things: kids who sleep well, lots of good GAPS food, a fun place to stay, cable TV to watch while I do therapy with Juliana, other people around to play with the kids, the emotional and physical support of my family. While this year has been nothing like I envisioned, I'm finally at a place where I can reflect on the past and look to the future with thanksgiving. 

A GAPS Christmas Feast

I've spent a wonderful Christmas weekend with my family. Check out our menu below. Everything is grain-free and sugar-free.

coconut flour pancakes
grain-free blueberry bars
eggs and bacon

Dinner #1
cheese burgers
cheesy green bean casserole*
(cooked in the dark, on a grill, after we lost power for several hours!)

Dinner #2
lamb roast
candied pecan butternut squash*

Dinner #3
carrot soup
7 layer salad*
cheesy coconut biscuits*

dutch apple pie w/ vanilla cream topping*
chocolate banana brownies*
coconut flour snickerdoodles

grain-free crackers*
beef jerky
spinach artichoke dip*

*These recipes are from A Whole Food Holiday

Wednesday, December 14, 2011

Ditching the canned nutrition (part 2 of ?)

When we switched to a real food formula I told myself that I would only go back to Elecare if Juliana's symptoms were noticeably worse. Even if her symptoms were exactly the same, I would continue to choose the real food formula over the 55% corn syrup solids formula.

Those first few days on raw milk formula were great. No reflux! No diarrhea! She was sleeping well and seemed happy and comfortable. And, an amazing development - she started drinking from a straw!

But then we experienced a brand new problem... constipation. Pasteurized milk can cause constipation but raw milk shouldn't. Apparently Juliana's little system just isn't recovered to a point where it can handle a diet of mostly milk. For some time I have suspected that she has low bile production and this constipation issue has helped confirm that thought. Per the suggestion of a knowledgeable Weston A. Price leader I started using raw milk kefir as the base of the formula. That helped but only a little bit. Fresh carrot juice also helped but not to the point of solving the problem.

I was in limbo for several weeks considering my next step. Go back to Elecare? Not an option. Go to the liver-based formula instead? I had a list of excuses and reasons why I didn't want to do that: she has only one safe meat right now (lamb). The liver formula is so, so much more work to make. I like knowing the exact amount of calories in the milk formula, whereas with the liver formula it feels a little less certain. I liked that she was quickly gaining weight on the milk formula.

On the other hand, constipation wasn't acceptable. So on 12/1 I made my first batch of lamb liver formula. Very quickly Juliana was no longer constipated. Still mourning the loss of the milk/kefir formula, I decided to add it back in. She now gets 50% liver formula and 50% milk/kefir formula. For awhile this seemed to be the perfect ratio of ingredients....

But now I am not as sure. Juliana has a bit of eczema on her cheeks and that really concerns me. It's popped up just in the past week or so. She's pooping just a bit too much. She has a small diaper rash but I don't like the looks of it. A typical mom might not pay much attention to these things, but to me they are red flags. I don't know if these are possible die-off/detox reactions or if they are indeed a food allergy. In either case I suspect the lamb. She was on raw milk formula alone for over three weeks and in that time she did not have any eczema or any diaper rash.

One interesting item of note: she likes her lamb formula very, very much. In fact it's not uncommon for her to finish a whole bottle of it! She doesn't like her kefir formula as much, I think because the kefir is so sour. I think this could be an important clue because the lamb must not bother her stomach if she is so willing to drink it.

So... we're getting there but we're not there. I can't call anything a complete success when she has eczema, even if it is a very small patch. Same deal with the poops (I pay an inordinate amount of attention to poop!)

I am still contemplating my next steps. We are in a decent position right now but we still haven't arrived at a real food baseline. I think that once we get to that point I will be able to push her in the direction of eating all of her food orally. I am eager to get to that point and hopeful that we are close.

Thursday, December 8, 2011

Ditching the canned nutrition (part 1)

My plan was that Juliana would never receive a bottle. I was (am?) a bit of a lactivist. Calvin was weaned just over a year ago; several months after his second birthday. The main reason I weaned at that time was because I was in the second trimester of pregnancy. I enjoy nursing and saw no reason why I wouldn't have the same experience with Juliana. Because I planned to stay home with my kids I didn't see any reason why I would need to pump or mess around with bottles.

The best-laid plans....

I fought and fought to avoid formula. I really think I did everything in my power to avoid it. I spoke with everyone who knew anything about breastfeeding.... from doctors to IBCLCs to La Leche League. (In fact it was an LLL leader who repeatedly encouraged me to try elemental formula, as she had been in a similar predicament with one of her children). If I could go back in time - to the time before I was even pregnant - then I think that possibly I could have avoided Juliana's GI problems. But kids with chromosomal disorders often have GI difficulties, so who is to say? In any case, I've come to peace with it.

Amazingly, the ingredients below were easier on her system than breast milk:

Yuck. It was important to me to get her off of this as soon as possible, but how to do that with a baby that had reactions to something in breast milk?

Enter GAPS. Dr. Natasha Campbell-McBride suggested right off the bat that I give raw milk formula to Juliana. I was too afraid. She had such a terrible reaction to something in breast milk and then to Neocate and now she was doing so well on Elecare. I didn't want to mess with a good thing. But the more I read about raw milk, the more I wanted to give it to her. But instead I just inched along trying out different broths.

A few months ago I learned that Elecare was changing their formula recipe. This made me incredibly nervous. Juliana is so sensitive - what if the changes to the formula didn't agree with her? We were at the end of the line having already failed all other elemental formulas. I waited for my next shipment of formula. When the new stuff I arrived, I decided to try it right away. I still had plenty of old formula in reserve but I wanted to know how she would react to the new recipe.

She did not react well. While it wasn't as bad as anything else we had previously tried, she was having reflux that was bringing her to tears and she was spitting up. These were both things that were completely gone on the old version of the formula. I quickly switched her back to the old formula and began desperately praying and researching.

I began to again wonder if Juliana has a corn intolerance. I read this article about a baby who didn't do well on anything except whole milk. I googled around and found a few similar stories (not many, but a few). I then spoke to a local Weston A. Price chapter leader who told me that she had seen a number of supposedly lactose-intolerant babies do very well on raw milk formula.

That was enough for me. I had to move Juliana to a homemade formula, and I had to do it before she ran out of the old Elecare.

On November 4th I mixed my first batch of raw milk formula, said a prayer, and gave four ounces to Juliana. And then we sat back and waited. Twenty minutes went by and she was still smiling and playing. An hour. Two hours. She took a nap without any problem. She pooped (this is an important detail for babies with GI problems!). She played some more. Everything was good.

I really couldn't believe it. My baby - the one who would scream the instant that breast milk hit her stomach - was totally fine with cow's milk (raw, grass-fed cow's milk!).

That's part one of the story. Part two is how I refined her diet and why I still think we haven't quite arrived yet at a real food baseline.

Tuesday, December 6, 2011

A day in the life

This week is not uncommon:

Monday morning: appointment with ENT. We are there for two and a half hours. I have both kids in tow.
Tuesday afternoon: a trip to DFCS to find out more about the deeming waiver. A failed trip, since again I had two kids with me and about 50 people ahead of me in line.
Wednesday morning: physical therapy
Wednesday afternoon: appointment with neurologist
Wednesday at some point to be determined: craniosacral therapist
Thursday morning: weight check and quick check-in with GI
Friday morning: physical therapy
Friday afternoon: occupational therapy
Friday at some point to be determined: another craniosacral session (maybe)

We also have some other typical things to do throughout the week. I try to go to a playgroup each Thursday. Obviously we have to go grocery shopping. I cook most of our food completely from scratch. I make Juliana's food. I try to do fun things with Calvin. I have to pay bills and I have to figure out insurance paperwork. I do laundry.

And I also like to sleep.

I have been forced to come to the realization that I need more help. Most weeks I can handle the load, but I am spent by the end of the day and the end of the week. Most nights David isn't home until around 8pm (he is working on a second degree). And oh yeah, my husband? Well I have not been out with him in a year.

What I dislike the most though is when Calvin spends too much time at the doctor's office and not enough time doing crafts, reading books or playing make-believe. Not every week is like that but this week has been.

But I've been reluctant to ask for help. For the most part I've asked for only the bare minimum.One reason is because I truly can manage it, I just can't manage it as well as I want. A second reason is probably pride and a severe, life-long case of "I can do it myself."  I may also feel a bit guilty knowing that there are kids out there who are in far worse shape and parents dealing with things far more difficult. The final reason, one that has paralyzed me more than the others, is because I feel strange about asking for help when I only have two kids. However the more I think about it the more I realize that when you have to teach your kid how to eat, have to figure out what in the world your kid can eat, have to learn how to be your kid's therapist and have to take your kid to a million appointments --- you're doing way more than parenting just two children. We've had weeks here or there where we only have one or two appointments, and those weeks are easy.

After a lot of contemplation I've realized that even just a little bit of help could go a long way. I'm not sure yet how this will happen, but I'd really like more quality time with Calvin, some time to exercise, and - if I'm really being optimistic - a little less house cleaning. And of course, a date with my husband would be nice - hopefully sometime this year.

Friday, December 2, 2011

Oral aversion, and how we moved past it.

Huge disclaimer: I'm not an expert on oral aversion or feeding issues. Every situation is unique. But I do want to share our story and how I helped Juliana overcome her oral aversion.

Late one night I wrote a list of things I needed to work on with Juliana. One of the things on that list: get her to burp without vomiting. Juliana had become so sensitive that a burp, a cough, or too much crying could cause her to vomit. We had a lot of damage to undo before she could even begin to reacquire eating skills.

Juliana's oral aversion began when she had great difficulty eating due to digestive pain and possibly some reflux. It peaked when she began vomiting violently. In the aftermath I was left with a baby who would gag to the point of vomiting if something (bottle, pacifier) was put in her mouth. She was overly sensitive and did not associate eating with pleasure but rather with pain.

The first step was to get to the root of the problem, and in retrospect it took awhile to do that. We went from breast milk, to breast milk on a Total Elimination Diet, to Neocate (fail), to Elecare (pass). During all of this we also gave her Zegerid, a proton-pump inhibitor that seems to help most babies with reflux. It didn't really help Juliana - I think that her reflux was minimal to begin with and I later determined that she was also reacting to the drug.

I do believe that a g tube could have been entirely avoidable if we had just figured out the problem sooner. She would have needed an NG tube for awhile but I imagine that she would have been taking full feeds by the time she was a few months old. I hate that this was avoidable but I don't blame anyone for it. Even now, Juliana's digestion is still a bit of an enigma to me.

Our second step was to get rid of the NG tube. The NG tube is such a catch-22: it gives your kid a way to eat but it also increases their oral aversion so that they don't want to eat orally. I was so glad to be done with it.

After g tube placement I gave Juliana a few days off to recover and to become accustomed to the sensation of a tube-free nose and throat. Then we began working in earnest. Her GI issues had been taken care of but the lingering effects of the trauma were still very much in her memory. She would gag, usually out of the blue. The gagging would turn to retching which would turn to vomiting. She has always been very communicative and I found that if I spoke to her in a comforting but somewhat firm voice I was able to convince her not to vomit. When that failed, I would quickly empty her stomach before she had the chance to expel anything (a huge benefit of the g tube, at the time). I figured that dry heaving is less damaging to the psyche than actual vomiting. During this time of extreme sensitivity I gave her very small, frequent meals. I also stayed away from continuous feeds.

Things slowly began to improve over the course of the next month or so. After awhile I noticed that Juliana had a pattern where she was much more likely to gag between the hours of 2AM and 11AM. Why those hours - I have no idea. But I really began to focus my efforts to those time periods in order to ensure a gag-free day. She would usually start to gag at times when I was not holding her, and I would run to her as soon as I heard the tell-tale sounds. I also tried to encourage her to burp or cough without gagging/vomiting.

As we worked past these habitual behaviors I also began to slowly desensitize her mouth. Eventually I was able to put a bottle into her mouth without any trouble. Unfortunately, she lost her suckling reflex after the vomiting spells caused an oral shutdown. Not too long after this I began spoon feeding her broth and I was thrilled that she would allow me to feed her this way.

It seems so simple: remove offending food, heal GI tract, encourage baby to stop vomiting, desensitize mouth, work on oral skills. But it wasn't easy. It really has to be done with great sensitivity - for instance I would always work with her in a quiet place which was not always easy with a rambunctious older brother running around. And there have been many times when I feared that I pushed the envelope too far and that I might undo some of the progress that we made. Fortunately any setbacks we experienced were minor and quickly overcome.

We still have some work to do but we've come a long way. Today Juliana can drink with a straw and most of the time she loves eating solids. I am really excited by her progress!

Thursday, December 1, 2011

More thoughts on receiving a diagnosis

We weren't sure what to expect at our appointment with the geneticist. Neither of us expected to receive a diagnosis so easily. I expected the doctor to say that it could be this or could be that and that we could try to run more specific tests. That seems to be the norm for a lot of people who get a clean microarray result. I kind of expected that we might be looking at a syndrome without a name.

The genetic counselor told us about Kabuki Syndrome and then she left the room to go get the doctor. David said "well, if that's the diagnosis, I'll take it!" We both know that there are far, far scarier things out there.

You might think that it would be sad to receive a diagnosis of a genetic disorder, but it truly wasn't. I had to face the fact that something wasn't right many months ago. It is very difficult to know that your child will have significant challenges doing the things that come naturally to typical children - even simple things like eating, or sitting up, or walking, or talking. When you have a typical child you may wonder when he or she will walk or talk, but you don't ever wonder if he or she will do those things. I suppose it will always sadden me but overall I've come to peace with it. It has certainly helped to read many stories of Kabuki kids who are doing quite well.

There are never any guarantees in life. We all know that yet we seem not to really know it. I've just barely begun to realize how much I take health for granted. I look at Calvin and can't believe how blessed I am to have such a smart, sweet, robust, healthy child. I look at Juliana and I'm grateful for her sweetness too and for the amazing progress that she has made. I think she is a smart and tough little girl.

So we did not greet this diagnosis with any sort of despair - in fact we felt immense relief.  I felt excitement that I could finally connect with other people who were also familiar with this label. Since Kabuki is very rare I've also felt a renewed appreciation for the internet and the way I've been able to connect with people all over the world.

I won't lie - life is very tough right now. But when I have a free moment to reflect on it (preferably this free moment will come at the end of the day and will also involve wine) I can truly say that we are blessed.

Wednesday, November 23, 2011

Genetic Answers

When the NICU nurse told me that it wasn't normal for Juliana not to be eating, I cried. I didn't know the ramifications of her statement but I knew that this wasn't good news.

When the pediatrician told me that we should seek genetic counseling if we wanted to have another child, I cried some more. She wisely suggested an anti-depressant.

When the neurologist said that it was time to order genetic testing, I cried again, but by this point I was becoming accustomed to the tears. We drew the blood and I promptly tried to forgot about it.

When the initial genetic testing came back with normal results, we were happy but we weren't dancing in the streets - we still knew that something wasn't right. The microarray testing was good in that it eliminated many different syndromes and it helped narrow the focus.

We waited six long months for an appointment with a geneticist. That appointment was today. I am thankful for the long wait because I have needed every bit of that time to come to peace with Juliana's health.

I thought I might cry at today's appointment, but I didn't. It was actually a very good appointment. I was glad to have David with me today - I knew that there would be a lot of information and I wanted him to hear it first-hand.

First we met with a genetic counselor. She reviewed Juliana's health history and she asked us about our family's backgrounds. She asked about research that we had done and if we had any inclinations as to what kind of syndrome that Juliana has. David and I gave up trying to diagnose Juliana a long time ago, there are SO many genetic disorders and we didn't see the point in trying to filter through all of them. There were a few genetic disorders that I was especially afraid of and I held my breath a bit when the genetic counselor told us that they had a theory as to which syndrome we were dealing with.

After awhile the doctor came in. She did a physical examination of Juliana which confirmed suspicions of the suspected syndrome.

And, drum roll please.... it's called Kabuki Syndrome. I'll be surprised if anyone has heard of it as it affects something like 1 in 32,000 people. Juliana will have a blood test to see if she has a mutation on the MLL2 gene, however the test comes back with a positive result only about 50% of the time. Even if the test is not positive, she will still be considered to have Kabuki. The test on the MLL2 is very, very new and there could be another gene that also causes the syndrome. Genetics is a rapidly changing field and I am sure that as the years go by we will know more and more specifics about Juliana's syndrome.

David and I were rather relieved by the diagnosis. No, it isn't "normal;" it isn't typical. She will continue to have many challenges. But it's not a regressive condition. There is not a shortened life expectancy. She will undoubtedly have learning disabilities but there's the chance that they could be mild. There were only two concerns that we took away from the appointment. The first is that there is always the possibility that she could have seizures. Although she has never had a seizure, it is something that could show up later. The second is that there is a possibility for hearing loss.

I did cry again today... but tears of relief. It's good to have a diagnosis. I didn't realize how much it would mean to me to have a syndrome with a name.

Tuesday, November 22, 2011

The Osteopath

Yesterday we saw an osteopath. What's an osteopath, you ask? I had no idea either. I guess you could say that an osteopath is like a holistic MD. They receive the same training as medical doctors but with more emphasis on "hands-on techniques to restore normal functioning". They believe that the body has abilities to heal itself:
In Osteopathy, the structure (anatomy) of the body and the function (physiology) of the body are interdependent. The Osteopathic physician works to remove any "roadblocks" to function and allow the body to show how it can heal itself. Examples of "roadblocks" are blockages in the flow of blood, fluids, nerve messages, tissue, energetic pathways, etc.
This is something that I wanted to try because Juliana undeniably has some sort of "roadblock" particularly when it comes to her low muscle tone and developmental delays. I respect modern medicine (allopathy) very much as it has literally saved Juliana's life on several occasions. However it's not getting us much further than that. As of this date, Juliana does not even have any sort of diagnosis for her health issues. I want to do all that I can to help her and even if we manage to shove a roadblock only a little bit out of the way - that would be a very good thing indeed.

I expected our visit to be some sort of hybrid of a chiropractic adjustment and a consult with an MD. Ultimately it was more like the chiropractor than the MD. I'll be the first to admit that it seemed a little goofy. However I can tell you that in the middle of the session Juliana went from crying to sleeping. She never falls asleep that easily or quickly so I am sure that something was going on. (I was curious if this would happen since Ellie just experienced something similar with a physiologist.) 

We'll go back again in a few weeks. Unlike chiropractors, osteopaths don't want to see you over and over again. You go a few times and then you're supposed to be fixed up and that's that. So the worst case scenario, I've wasted my time and money. But if he was able to move one roadblock just a tiny little bit... then it was worth it. 

One last note... if you want to see an osteopath, ask around for recommendations. Many are more like MDs and less "alternative" (for lack of a better word). Our osteopath came with a recommendation from a Weston A. Price chapter leader.


Meet other families healing through nutrition. Check it out! 

Saturday, November 19, 2011

Physical Therapy

About a month or so I began to suspect that OT wasn't cutting it for Juliana. Either she wasn't getting enough therapy or she wasn't getting the right kind of therapy. The neurologist had told me that I could expect her to begin to sit up around 8 months old. Honestly I think she pulled this age out of a hat while I was in the waiting room, but it's a statement that has stuck to me and I began to get nervous as time went on. Juliana was no where near sitting up. I decided to get a second opinion from a PT.

Our evaluation was two weeks ago and as expected, it was pretty depressing. Juliana scored at a 4 month level in two areas and only a 1 month level in a third area. The 1 month score was harshly low but had to be scored that way due to lingering torticollis. But I liked the PT, I liked the facility, and I liked that I was already learning some new ways to help Juliana.

We had out first real PT appointment yesterday and it went really well. Juliana was in a great mood and she warmed up to the therapist immediately. Baby J cooperated for the whole session - I am crossing my fingers that she will always do so well in a fun new environment vs. our boring living room where we usually meet with our OT.

I learned some great new things that will hopefully be easy to implement into our daily life: joint compressions to wake up her sleepy joints, arm strengthening exercise so that someday she will be able to prop sit, and how to get her to do a baby sit-up. These are all things that are new to me and I'm a disappointed that the OT (who surely must know this stuff) did not show them to me earlier. I am not sure if we have a dud OT.

So I feel like we have lost a bit of time but we're now on the right track. We're getting PT at least one time a week or two times a week if it works for everyone's schedule. I am trying to squeeze in a lot of appointments before the end of the year while I still have awesome insurance.

Saturday, November 5, 2011

The week in review

We had a very busy week - six appointments! Because we automatically have three appointments each week (1 OT, 2 craniosacral) it usually happens that at least once a month we will have a crazy week like this. But it was a great week with positive feedback from everyone we saw.

On Monday we saw the feeding therapist. She was really impressed with how well Juliana was eating her baby food (pumpkin). In a nutshell she said that I am doing everything right and agrees that J has come a long way. Hydration/liquid calories are the challenge at this point because J drinks very little. Juliana went over a month where she didn't have anything in her mouth and as a result she forgot/lost her suckling reflex.

Also on Monday we saw the OT. Juliana cried through most of this appointment so we didn't get to work on very much.

On Tuesday we saw the opthamologist. He was pleased with the way her vision has progressed. Her skills are on the rudimentary side but she is improving. I like this doctor very, very much - if you need a ped opthamologist in Atlanta I will be happy to pass along his info.

On Wednesday we saw our craniosacral therapist. He thought that her tone had improved. I don't really know what to think about that, her tone is a weird thing. She has low tone but at the same time she is strong. At any rate, I'll take the positive feedback.

On Thursday we saw the GI. Our GI is another doctor that I like very much. He seems to really care about Juliana and he will spend half an hour with me just to answer my questions. Both those things are a huge, huge deal. He thought that the site of her button looked incredible - although she has very sensitive skin, she has not had any trouble with the area around the button. A lot of people have trouble with granulation tissue but it looks like we won't.

He noticed that she was slipping in the weight charts (which is true and something I've been a bit concerned about) and he wanted to increase the calorie concentration of her formula. However that might not be necessary after all, as we may be having success with a homemade formula... more on that later.

I was really pleased with the visit. He was happy that she was doing well without reflux medications (I think that is pretty huge in the world of tubie kids). He was happy that she liked solid foods and he made a comment that this will be our "saving grace." I told him that I thought that she still reacted to Elecare and he agreed that it sounded like she did. I love it when doctors agree with me!

All in all, Juliana is doing well right now. I know that we're making progress because the things that I worry about are beginning to shift. Initially my number one concern was her gut and her digestive health - after all, how much progress could I expect if she was always in pain? Now I think/hope/pray we're beginning to see improvement there and my concerns are shifting to her spine and her developmental delays. Shifting concerns are a good thing, I think.

I'll close with pictures of my two little munchkins.

Friday, October 28, 2011

The Best Advice

When I told my spiritual father that I was worried about Juliana's future, he gave me some advice that has drastically impacted my outlook on life. He advised that instead of thinking and worrying about what may happen in one, five, ten years, instead I should keep my time perspective focused either on the present moment or on eternity. The eternal perspective is preferable, he told me, but it is hard to stay in the eternal mindset all of the time.

For the most part I've become pretty good at implementing this advice and it really has been liberating. I wish I could say that I no longer worry - of course, I do. But I am certain that I don't worry as much as I would otherwise. When I find myself thinking too often about what lies ahead, I shift my focus to eternity. After recharging there for a bit I can come back to the present moment.

Wednesday, October 26, 2011

Why Juliana doesn't eat (much)

I am not sure that I have ever adequately explained Juliana's feeding issues to anyone other than David. It's a complex issue and there are still more questions than answers. I want to try to explain what's going on because I think that some people are afraid to ask... and other people ask very frequently.

I have my theory and different doctors have their theories. I'll explain each.

My theory - my theory is that she did not eat much in the NICU because she was too sleepy and too tachypnic. My evidence for the sleepiness - I would visit her for eight hours a day and she would literally be awake for a total of 10-15 minutes during that time. Her respiratory rate during that time would sometimes be as fast as 100 breaths per minute. You try eating while breathing that fast and let me know how it goes! I blame both the sleepiness and the respiratory rate on her heart defect.

Days after we came home, she began to exhibit extreme signs of GI pain. There were times I really wanted to pack her up and take her to the hospital but I knew that there was nothing that they could do for her there. (We never would have been admitted anyway, she had a bad habit of calming down when she saw the bright lights in doctors' offices.) The GI pain was a reaction to something in my diet (I still don't know what). It caused reflux and near-constant pain. She would eat a little, but just enough to take the edge off. She typically ate about 20ml per feeding - that's just 2/3 of an ounce.

But the last straw was when we tried Neocate. She felt immensely better on Neocate - she stopped crying in her sleep, she stopped having diarrhea, she stopped screaming when food hit her stomach. But she started vomiting violently (these episodes were very scary to all of us. She would stop breathing and she would flail violently). The vomit increased her oral aversion and she just shut down orally - she would gag on anything put into her mouth. For the next month or so (while we waited for the g tube) she did not eat anything by mouth.

The pediatrician's theory - the initial theory was that J didn't eat due to low tone in her mouth. I don't think that's the issue. I have seen J eat very well in moments when she was without pain (this only happened a handful of times, pre-Neocate).

The neurologist's theory - she hasn't really come out and said it but I am pretty sure that the neurologist believes that J doesn't eat because of a suspected chromosomal disorder. Kids with genetic issues very often have feeding problems, although it seems that they eventually do acquire the skills needed to eat. I agree that this is a possibility. Time (and an upcoming visit with Genetics) may tell.

The GI's theory - he doesn't have one. I asked him to theorize with me and his response was "I have no idea." We initially thought that silent reflux was the culprit but he agree with me that her reflux is very minimal and not the issue. When it comes to J's feeding issues he puts a lot of stock in the opinions of the cardiologist, the neurologist, and the feeding therapist.

The cardiologist's theory - he agrees that her heart could be partially to blame. The reactions just sealed the deal.

The feeding therapist's theory - she agrees with my theory that a kiddo who is in GI distress is not going to eat.

I do want to be absolutely clear that her NICU stay in and of itself is NOT to blame for her issues. Babies come home from the NICU without feeding tubes all the time. She was ventilated for less than 24 hours so I believe the vent did not really have an impact on her oral aversion.

Hopefully this illustrates the complexity of J's feeding issues. We've seen progress but it is s-l-o-w. Sometimes people ask when she will have her feeding tube removed. We don't know the answer to this question. As of today she takes approximately 7% of her calories by mouth. That's a huge achievement because a few months ago she took nothing by mouth! She still has some oral defensiveness and she lacks some skills. These are things that don't change overnight. She has the feeding tube indefinitely - maybe forever, but hopefully not. Only God knows if and when she will ever be able to sustain herself on only oral feeds.

Tuesday, October 25, 2011

MRI results

I waited anxiously by the phone all day Wednesday. The neurologist called late in the day and of course I missed the call. But she left me a message and it's always good news when results are left on your voicemail: the brain MRI was normal. She also had the results from the MRI of the spine but she wanted to discuss those with me. This was not a surprise because based on the spinal ultrasound we knew that the MRI would be abnormal.

It did surprise me a bit that her brain MRI came back normal. I had three people wanting the results of the brain MRI: the opthamologist, the orthopedist, and of course the neurologist. *Something* causes her hypotonia and that something could easily be in the brain. But apparently, it's not. The neurologist encouraged me to take this as "good news" and I do, but I am still apprehensive. The brain MRI does not tell us how well her brain works. It does not tell us why she has hypotonia. It does not tell us what else could be lurking around the corner. Genetics may give us more answers to these questions.

On Friday I was able to talk to the neurosurgeon's nurse and get the full results of the spinal MRI. She has a syrinx and some fatty tissue at the base of the spine. However he doesn't want to do anything about it - he doesn't even want to see her for a follow up appointment. They are only interested in seeing her if she develops scoliosis or if she begins to have trouble moving her legs or having bowel movements. They want us to follow up with the orthopedist which we were already doing.

So overall, the MRI results were as good as they could be. I am thankful for that bit of peace but still very concerned about all the other health issues. 

Monday, October 17, 2011

MRI Today

Today Juliana had her PEG switched to a Mic-Key and she had two MRIs (one of her spine and one of her brain).

The spinal MRI will tell us if she has ventriculus terminalis (a normal variant) or syringomyelia (more concerning, would require more follow-up and I'm not sure what else). It will also give us some indication as to what is going on with the curvature in her back. And I guess it will be (hopefully) another confirmation that her sacral dimple doesn't go all the way down to her spinal cord. 

The brain MRI may tell us why she has hypotonia. It may also tell us why her eyes aren't quite in sync. It may also give some clue about the spinal issues (or maybe that's vice versa?).

I left the hospital feeling optimistic, but not about the MRI results. I expect abnormalities on both of those. But I am optimistic that I will continue to do the best I can do for my little girl. I am optimistic because her GI health is fairly stable at the moment. I am optimistic because she is not in pain and because she is learning new things, even if it is at a snail's pace most of the time. 

It can be hard to retain optimism sometimes. Obviously, there is a lot of sadness when you have a child who is ill or disabled or who has special needs. No one wants those things for their child; no one wants to see their baby work so hard to do things that everyone else takes for granted. 

While waiting in the hospital this morning I read an op-ed piece that appeared in today's NY Times, Notes from a Dragon Mom. It's heartbreaking but you need to read it. It certainly gave me a little perspective: I have a very sweet daughter. She does have many health issues and many uphill battles to fight. I don't' know what her future holds and quite frankly I don't think about it. We have an excellent support system, we have good doctors and therapists, everybody is happy and we don't want for any basic needs.

Really, how much more can I ask for?

Wednesday, October 12, 2011

Her Heart

Of all the issues that Juliana deals with, her heart defect is one that I haven't really worried about too much. She has a ventricular septal defect (VSD). It's considered to be on the small side at 2-3mm. There may also be a couple of other very, very tiny holes that are too small to measure. These holes are at the bottom of her heart in the muscular region which is apparently a good place to be. In the past her cardiologist has told me that heart surgery probably won't be needed because the holes are so small. Even if they do not close, her heart will grow bigger and the size will become increasingly insignificant.

Today we had a check-up to see if there had been any change in the last three or four months. Unfortunately, the VSD is still there. Everything else looked good, except that this time the doctor mentioned that Juliana has red blood flowing to her lungs. Red blood should be flowing back to the heart and blue blood should be flowing to the lungs for oxygenation. Apparently this can be considered par for the course with VSDs. This is one explanation that I found:
Blood is crossing through the VSD and going back to the lungs instead of out to the body.  This will cause the heart and lungs to be less efficient at circulating blood.  Since the heart has to work harder to get the correct amount of blood out to the body, there can be concerns with enlarged hearts or changes in growth since the heart is taking up more calories.  
And another:
When there is a lot of blood going back to the lungs it causes over circulation of the lungs and then overload of the left ventricle, as there is more blood returning from the lungs into the left atrium, into the left ventricle. This is what eventually causes heart failure - the heart can't keep up with the volume. It can also cause scarring of the blood vessels in the lungs which can lead to problems later on. That said - that's not common, because if there's that much over-circulation, surgery is normally performed to correct it.
This all sounds scary but the doctor is "cautiously optimistic" that everything with her heart will be ok. So far this seems to be the nature of Juliana's birth defects - they are there, they are obvious, they are concerning, but they can be dealt with. We will continue to see cardiology regularly (every few months) and the doctor will keep an eye on this in case it does not improve or gets worse. He also mentioned the possibility of medication but said that he is not the type who is quick to prescribe (good policy in my opinion).

It's hard to say what sort of impact the VSD may have had on Juliana. I do know that for the first two or three months of life she had a high respiratory rate - she was called "comfortably tachypnic." The VSD caused or contributed to this. I also believe that it would be quite difficult to eat if you were breathing very quickly. Add in reflux and digestive pain, and forget it - eating wasn't happening. Heart babies also tend to lack energy. While J is very energetic these days, in her first few months she slept All. The. Time. It was very concerning to me because it *was* excessive and far beyond normal newborn sleep.

While today's visit did not bring hugely bad news it was still very disappointing to me. I was hoping that the hole had decreased in size or that the doctor would want to see us less frequently. I hate to hear that there is a possible concern even if he is "cautiously optimistic" about it.

Friday, October 7, 2011

Progress Report - 6 months old (adjusted age)

Very, very suddenly I feel like we are making some real progress! I am not sure where credit is due -  the alternative therapies we're doing? the occupational therapy? the daily therapies I do with her? just the mere passage of time? Whatever it is, I am feeling so very thankful to see a ray of sunshine. God's grace indeed.

We had a good week. To break it down:

First - We had two doctor's visits this past week, a visit to a pediatric orthopedic specialist and her third or fourth visit to the neurologist. For the first time ever, these were pleasant appointments. Usually appointments are neutral at best (when they are a rehash of known problems) and attempts not to cry at worst (when I'm told new problems).

The orthopedic specialist was very encouraging and told me that her spine looked good and that her tone was "fantastic" considering that she has low tone. He was enthusiastic about therapy and said to keep doing what we're doing. He is a bit concerned about the curvature in her lower back though and he will look at the results of her upcoming brain/spine MRI. We'll check back with him early next year around her birthday.

The neurologist was also very encouraging. She said that it was great that J was progressing so well (we were watching for any regression which would be a huge red flag) and that her tone seemed better (i.e. she is beginning to compensate for it - hypotonia is not something that goes away). We'll see her again in December, after we meet with the geneticist. If there is nothing huge discovered on the MRI or with genetics then I imagine that we will be released from neurology at that time.

Second - I have noticed in just the past week or so that Juliana feels stronger and sturdier. Sometimes she can sit on my lap with minimal support. Just today I laid her on the floor and when I looked over a minute later, she was on her stomach and lifting her chest off of the floor. She's been rolling for some time but she hasn't been very good at it - she gets stuck easily and never before has she been able to lift herself up like that.

Third - We're making good progress with feeding therapy. She is learning how to drink through a straw which is both cute and messy. Today she drank some water and then ate about an ounce of chicken broth "jello." (Yummmm!)

There are still a few things that we are really wrestling with. Always on my mind is what she gets for nutrition. I have a love/hate relationship with her formula (Elecare). I hate that she is on canned nutrition, yet I am so thankful for it because it is what stopped her pain. We've been trying different foods and usually have seen success, yet we're not at a point where I can feed her a diet of entirely real food. I think I would be at peace with the slow progression of things IF she ate orally. However, she does not, and this really presents a problem as she does not seem interested in drinking the formula. I think that she will be tube-fed until she can be free of the formula.

I would appreciate prayer for her upcoming MRIs (10/17) when she'll be under general anesthesia for two hours. At that time she will also have her PEG feeding tube replaced with a Mic-Key button. (Think of it this way: instead of a surgically implanted straw going into her stomach, she will now have a little beach ball valve in her stomach). I would appreciate prayers that this would go smoothly and that the new g tube wouldn't cause any additional reflux or pain.

At this point in time I am not too worried about the actual results of the MRIs. I know that they will tell us something about her condition, and I'm hoping it's more on the benign side of things. She's been doing pretty well lately and life has been fairly sublime (apart from constant therapy appointments, worry over allergies, worry over anesthesia - you get the picture!)

Wednesday, September 28, 2011

Therapizing Baby J

I'll be honest: therapy is a drag. I often have an uncooperative patient and a long list of objectives. It can be difficult to get the timing just right: Juliana needs to be in a good mood and Calvin needs to be occupied. It's a rare day that we accomplish everything that we should but this seems to be the norm for kids in therapy.

Juliana sees an OT once a week and a speech/feeding therapist every other week. I work with her every day practicing the various things that therapists have shown me. Some of the things that I try to do each day:
  • Tummy time - we do this three different ways: flat on the floor, elevated on top of a towel, and on the exercise ball. While she's in this position I have to pull out all the tricks to get her to look from side to side.
  • Rolling - she's been able to roll for quite some time, but she has a strong preference for her right side. We're trying to get her to roll to the left. She's not a fan.
  • Eye patching - I thought that this would be very easy but it's not. She doesn't like wearing the patch and if I don't keep her distracted she'll eventually pull it off. 
  • Feeding therapy - I feed her with a spoon several times a day. Sometimes we try the bottle too. While her oral aversion is very much improved she is still very sensitive and I am really careful when working with her. She has some low tone in her mouth and her eating skills are rudimentary (largely from lack of experience, in my opinion). Though we've seen a lot of progress in the past few months this type of therapy is very slow-going. Feeding disorders are complex. It's sometimes difficult for people to grasp why she won't eat - more on that later.
The above list is just some of what we do daily. Therapists are constantly adding to the list. It may not seem like much.... but trust me, it is. (Add in the fact that David is essentially not home from Monday - Thursday....)

One of the biggest downsides to needing so much therapy is that you start to see your child as a patient and not as a little person. I have to remind myself to play with her in a non-structured way. We spent this past weekend at the beach and I gave myself a break from therapy while we were there.  I did this because *I* needed a break but I didn't realize how beneficial it would be for all of us to just spend the weekend as a "normal" family. It was the first time in almost seven months that I didn't think too much about Juliana's various issues and therapies. Now I feel recharged and ready to tackle our long list of objectives. I am realizing that I need to take a break more often. Juliana was very happy and smiley while we were there - maybe she needed a break, too.

Monday, September 26, 2011

How GAPS found me

I have never in my life prayed so fervently as I did the second month of Juliana's life. If your baby is constantly in pain, if she screams when you feed her, if she cries in her sleep because she hurts so much, if the doctors don't have any answers for you - you WILL be driven to your knees in prayer. 

I scoured the internet for information that might help Juliana. I spent hours and hours each day reading and I really didn't find anything that helped me much. I was without a diagnosis and without much of a prognosis. Juliana had an NG tube at the time (she's had a tube since she was 2 days old due to a NICU stay) and I knew that she would never eat if we couldn't stop the pain.

Enter the POPSICLE forum, one of several forums where I was discussing GI issues. I wrote a post about getting a g tube and I made an offhand mention of Juliana's intolerance to my milk. Not too long after that I received a message from a woman who asked me a little bit more about Juliana. I answered her questions and she told me that my story sounded familiar and she threw out a diagnosis that I hadn't considered: protein intolerance

Nichole doesn't push GAPS onto anyone despite her amazing story. During our initial conversation she casually threw out the term "GAPS" and she gave me a link to her blog. I quickly read everything that she had written and I was blown away by her daughter's story. I began to read everything that I could find about protein intolerance/FPIES and about GAPS. It became clear to me very quickly: I could take a wait-and-see approach and hope that J might someday "outgrow" her food intolerance or I could take steps to heal her NOW. The choice was easy: GAPS was the solution.

I've never before had such a clear-cut answer to prayer. Several hundred people were praying for Juliana and her path to healing was given to me by a stranger on the internet, completely out of the blue. Nichole read my thread about G tubes even though feeding tubes are not a part of her life. Providential? Absolutely. (And if you know me, then you know that I do not make light of such claims).

I sometimes wonder if I would have eventually stumbled onto GAPS on my own. Perhaps I would have, though it's not something that I've ever seen mentioned in the mainstream - not on the message boards and websites that I frequent and *certainly* not in the medical community. I want to help change that, particularly for the many babies and kids out there who need GAPS. 

Sunday, September 25, 2011

Where we are Today

Where to begin the story of our long and arduous journey? At a later point I will try to address Juliana's NICU stay, the second month of her life where she was constantly in pain, the many specialist visits, the g tube, the process of searching for a diagnosis. For now I want to jump right in and record where we are in the healing process.

Calvin and I have been on full GAPS for about 2 months now.  Neither of us seemed to have any major digestive issues pre-GAPS yet I've seen various improvements that are significant enough to keep me motivated. More on this later.

Juliana has been on a diet of 2/3 formula (EleCare - the ONLY formula that she could tolerate and the ONLY food that did not cause pain) and 1/3 bone broth + coconut oil for about a month. She now has several safe foods: lamb, fish (red snapper), chicken, beef, coconut oil and fermented cod liver oil. I think it's pretty amazing considering that she reacted so violently to my breast milk, even when I was on an elimination diet.

In the next month or two I hope to move Juliana to a diet of homemade formula - something like the Weston A. Price liver-based formula. While EleCare was a godsend, I often wonder if it is inhibiting her healing. Next up: I plan to trial olive oil and hope to add it to her safe food list.