Posted (Tina) in All Posts on November-28-2010

The past few weeks have continued to go well with the blenderized diet. We’ve had only a few retching moments that caused something to come up. In the past several months using commercial formula, we were having that challenge multiples times per day, so that part is better. The unfortunate part: weight loss. We dipped below 13 kilos or 28 pounds, but contacted the dietician who told us to double the amount of MCT Procal powder to 3 tablespoons per day. This adds extra fat and calories without adding fluid. We just weighed her on 11/24 and she was 13 kilos. We’re hoping to see that number increase within the next few weeks at our next check-up.

The biggest concern, especially the past couple of weeks, is pass-out spells. We mentioned that we were having some on the last post and that we’d given Bumex. But it seemed like the Bumex wasn’t doing much and the frequency of the spells was continuing to increase. We’ve had several per week, sometimes multiple per day. Since we resolved that fluid overload was not the cause as the Bumex wasn’t doing much (last dose on 11/20), we next thought it might be her SVC. We have not been to the heart cath lab since Sept 2009, so maybe it was related to her heart. We had six pass out spells over the 5 days prior to Thanksgiving. Sure enough, the doctors were concerned enough to bring her in for an urgent echo to check her SVC and pulmonary hypertension on Wed, 11/24, the day before Thanksgiving. When we arrived at the hospital, we were not sure if we would be admitted and have to stay there for Turkey Day. Fortunately, Ella checked out great. The gradient pressure in her SVC was 2, versus 5-7 on the last echo. That is very little pressure and great flow which means her heart stents and blood thinners are working great. There was no evidence of pulmonary hypertension. So we were relieved at these findings plus that we did not need an admission. Although it still leaves a mystery, why the sudden increase in pass out spells?

One other theory is reflux. Apparently, the reflux can cause a lot of pain. So when she clamps down to cry real hard, it can place lots of pressure on her belly and the reflux can initiate, shooting up her esophagus. I’ve never experienced reflux but countless others have described the pain it can bring, almost to the point of not being able to breathe. One other interesting clinical symptom that just came on the past week: bad breath. This can also be another symptom of reflux. Literally, you will brush her teeth, mouth, tongue and right afterwards, her breath still smells. We do think her nissen has loosened since her last nissen surgery in Sept 2008. There are a few ways to check and quantify the reflux: an upper GI (see if they can see the barium dye going into her esophagus) or a ph study which includes a hospital admission and a tube up her nose for 24 hours. We’d prefer to try other, less invasive tests first.

All in all, we’re just hoping the pass out spells go away or are greatly reduced. It is scary to have your kid turn purple/blue, eyes roll back, and lose consciousness for a brief time. It is normal for toddlers to have behavioral breath holding spells and I think if Ella did not have serious underlying medical conditions, I would chalk it up to that solely. With her medical history, her doctors are not convinced that it is just breath holding spells either. And it is strange because we can go months without any and then all of a sudden they will come on with such great frequency. The past has shown us that there is an underlying medical reason.

So, we discussed a few things: we started her new blenderized diet at the end of October, but sure enough, pass out spells have increased. Could she be allergic to something and/or is there an ingredient in the diet that is causing more reflux? Ella already takes Zantac which is used to treat gastroesophageal reflux disease (GERD), a condition in which backward flow of acid from the stomach causes heartburn and injury of the food pipe (esophagus); and conditions where the stomach produces too much acid. But we asked if the dosage could be increased. Sure enough, instead of 30mg per day, she can have up to 40mg per day. We opted to try that out. Also, they mentioned that calcium carbonate might not help as it is short acting, but certainly won’t hurt (Tums). It turns out that Children’s Pepto Bismol tablets have the exact amount per dosage that work for Ella. She can have 400mg, a max of 3 doses per day. So, we just dissolve in a syringe and give to her with 3 of her feeds throughout the day. Once we left the hospital, we bought some and started administering on Wednesday evening along with the increase in Zantac dosage. She has not passed out since Tuesday night and it is Sunday. We’re hopeful that maybe these two slight changes are making enough of a difference. Just to be safe, we were supposed to be headed to the hospital tomorrow morning for an upper GI. This will give us a picture of her tummy and its function with the barium dye placed in it. Now that we have not had any pass out spells with the medication adjustments, we’re opting to wait it out a bit longer.

We had our first solo date night. Boy, that was a treat – it had been WAY too long.

Also, we had a nice, mellow Thanksgiving besides the 4 canines!

On a positive note, Ella is cruising. Not on a ship, but walking around this house like nobody’s business! It’s really fun to see her progression in such a short amount of time. We figured that we would post a couple videos, so you can see for yourself. She’s also starting to pay attention to the dogs more and found a new game to play with Dad. Enjoy!

And finally, I felt prompted to share a portion of a blog that I’ve been following lately. The baby was born with a congenital heart defect and was just released from the NICU. The father wrote these words on their blog the other day and I couldn’t share the same sentiments any better:

With Thanksgiving coming up tomorrow, I’ve been reflecting a lot on the gifts that God has given our family, and I can’t stop thinking about the most unexpected ones. My thoughts lead me back to what I wrote in my post on September 17th while Bowen’s chest was still open from surgery, only days after his cardiac arrest. These words feel as true to me now as they did when I wrote them.

“A friend told me that I’ve seen too much, but I’m realizing that I might not see enough. Everything I’ve watched happen in this hospital, all the pain I’ve felt, is deepening my faith, strengthening my marriage, and molding my character. As I lovingly stared into Bowen’s eyes just before midnight, my face only inches from his chest, I thought, ‘This love is an awesome mess.’ I know I’m not the first person to think or to say something like that. Many great works of art have titles that are reminiscent of those words. I believe it’s because tension is the place where the worst of life and the best of true hope meet to unveil our eyes to God’s artistic work of redemption. What a mighty and creatively loving God we serve. He allows us to know great pain, so that we can know the greater pleasure of trading it in for purpose.”

This Thanksgiving, because of all we’ve been through, we have even more to be grateful for. I’m not suggesting that we’re thankful for pain and loss, but that pain and loss can make us thankful.

Posted (Tina) in All Posts on November-7-2010

We’re so sorry to all the loyal Ella fans that have been patiently waiting for an update. Wow, there is so much to catch up on. Where do I begin?

Ella has been pretty stable.

I noticed in mid-to-late September that Ella was doing her pass out spells on me again. Slowly they started to occur more frequently, then once per day, then multiple times per day. Based on my past experience in working with her pulmonologist, it is typically fluid retention. So on Sept 23rd, we gave her a dose of Bumex. Within a few hours, she had some huge diapers which confirms that was likely the case.

We went to see the GI doctor the next day on Sept 24th. At that point, we were still having trouble with Ella using EleCare. So, he prescribed MCT Procal powder to try and ease Ella’s retching and discomfort with feeds. He wanted me to try all three formulas with the MCT Procal powder to see if it would help; he was suspecting that she might have dumping syndrome. On the other hand, I was skeptical with the dumping syndrome diagnosis, but I just knew that feeding seemed to be very rough these days. At this visit, she weighed 13 kilos (28.6 lbs). I did express frustration that no matter how I seemed to feed Ella (multiple bolus feeds, water diluted in the food or straight water flushes, food pump at night or not, etc, etc, etc), it just seemed like nothing I did was making much difference. He noted that if none of the formulas with MCT Procal were successful, he’d like to hospitalize her for more tests and worse case scenario, change out her G-tube for a GJ tube.

Two and a half weeks later, we went to get Ella’s SVC checked via ECHO and see the pulmonologist. Even during that routine day long visit to the hospital, Ella was retching several times throughout the day. On the lunch break, we left the building so I could get some real food and I fed her. I had to stop 3 times within 10 minutes while driving, just to clear her airway from her retching up food and secretions!

The ECHO showed that the SVC pressure was a bit higher between 5-6 versus 2-3 on the last ECHO in late June. Nothing too alarming – but not headed in the right direction. The more alarming finding was that she weighed in at 13.7 kilos (30.14 lbs). There is no way that she would gain that much weight in 2.5 weeks. Her pulmonologist agreed that it was likely fluid retention. I explained when I had given the Bumex and that she had come close to passing out again, but had not quite yet. I was going to wait until that happened. Sure enough as I write this post, she passed out twice just the other night on Nov 4th. So the next morning on Nov 5th, we had to dose her with Bumex again.

The pulmonologist was more concerned with the retching, especially because it was not dry retching. We were having secretions and formula come up. This presents a high risk for aspiration and gave him great concern. So he pushed for the GI plan related to feeds. I told him that we were trying EleCare with MCT Procal, then Peptamen Jr with MCT Procal, and Nutren Jr with MCT Procal. So far, the EleCare with MCT Procal was not working. He let me know that he would call the GI team and apply pressure that this was a problem that needed to be worked out sooner versus later.

At this point, I was pretty fried from the constant discomfort and retching after each feed. Most of you probably don’t know what that experience entails, but trust me, it is very tough and heartbreaking to see Ella so miserable. So I decided to get online and do research. There have to be other parents that are experiencing the same issues. After surfing for awhile, the first significant finding was a method to wean children off of tube feeds. This is something that many parents try all the time with our conventional therapy methods, but the reason this was different: it was an approach used in Europe that is very successful and different than our approaches here in the US; most children go from 100% tube feeds to 100% oral feeds within 2-3 weeks. Wow!

As far as Ella is concerned, she still has some medical causes for using tube feeds and we have not even been able to get tube feeds to work successfully, so she is not ready to be weaned off of tube feeds. BUT, I found tremendous hope in these findings for the European approach – when Ella is ready, we are going to try this approach. I agree with the philosophy much more than the approaches I’ve experienced via therapists so far. As a bonus, I not only found this on another blog – but a blog for another CDH girl that was struggling with tube feeds – so now I can connect with her mom when I decide to try it with Ella. On that note, I did reach out to this mom and one of her questions in her email to me was asking if I had tried a blenderized diet. What is a blenderized diet?

I started googling and came across tons of blogs with tube fed kids that use this and report less retching than commercial formulas. Basically, it is taking regular foods and mixing it in a high speed blender to nourish your child. Ella had never had real food for her nutrition and it sounded like lots of work each day, but if it would solve her discomfort and retching, it would be worth it. As a side note, I’m going to post information about blenderized diets as well as weaning of tube feeds in another section of the site for parents that want to read the detail and educate themselves on an approach that might help their kid. For the rest of you, I’ll spare you the details.

I found a few recipes and was prepared to contact her GI doctor and nutritionist to ask if they would let me try this option with Ella. Although Ella had never consumed real food for her nourishment, she had an allergy test this past summer and passed with flying colors. That is usually the biggest concern. When I called the nutritionist at the hospital, she confirmed that many parents used a blenderized diet and noted that the hospital already had a recipe that I could try!@#@?? Excuse me, why had they not offered this option in the past? Here they wanted me to try 3 formulas with MCT Procal and if that didn’t work, they were prepared to hospitalize her and do invasive tests and even mentioned switching to a GJ tube. If I thought about it too much, it really irritated me. So, I just accepted the standard recipe, tried it and knew that I would likely change some of the ingredients if Ella started to show any promise in this approach (example: recipe calls for vegetable oil, but olive oil or flax seed oil is more nutritious). So what happened?

The retching was significantly less, but not eliminated completely. When we saw her pediatrician on Oct 27th and gave him an update, I mentioned how the morning feeds seemed to be worse. He had a great idea: give Ella more soluble foods in the morning (banana, milk, oatmeal) and use the other ingredients later in the day. He thinks that her system is slower and doesn’t function as optimally as it should. Giving her proteins and other more challenging foods after no food overnight might be causing the challenge with the morning feed. Sure enough, he was right. His other two suggestions: use honey instead of karo syrup/agave nectar due to how the stomach breaks down those foods. Use the MCT Procal powder because it is a good way to add a high amount of fats and calories with just a tablespoon of powder.

-We’re not feeding with the food pump at night.
-Ella is being fed 6x per day; 1000ml of blenderized diet and 300ml of free water flushes.
-I make two different blenderized foods: breakfast and lunch/dinner meals.
-Real food caused constipation for Ella, so we add Miralax to the blended foods.
-We’re using milk, grain and fruits for breakfast.
-We’re using protein powder, baby food meats, baby food veggies, flax seed oil, honey.
-We’ve added apple cider vinegar since that is known to help GERD.
-We’re adding MCT Procal to help give a high amount of fats and calories to the food.

Even as I type this, we’re still tweaking things to try and come up with the optimal schedule/ingredients. But I have to say, this is the most pain-free eating experience that Ella has had so far. She has always detested feeds and would whine when she saw the syringe. Now, she is often times smiling and active after feeds. Praise God!

She has maintained at 13.7 kilos and is scheduled for a follow-up ECHO and pulmonology visit in the next few months. We’ll see what happens, especially if we have to keep using Bumex!

I feel like I almost have a different kid. She went from a baby to a little girl by the end of October.

First, she was starting to stand on her own, and continued to crawl around and climb the stairs at the beginning of October. She learned how to climb back down the stairs too. Then all of a sudden by the last week of October….drumroll please…..Ella started to take her own steps and walk! Currently, she still requires an object to pull to a stand, but then will let go, take a few steps and fall down! It happens so fast that we have not had a chance to catch it on video. But as she strolls toward the holidays and continues to get stronger each day, we’ll be sure to capture video and share with all of you.

It just seemed to happen like a light switch – much faster than we all expected. To give you an idea: her physical therapist saw her in mid-October, skipped one week of therapy and came back 2 weeks later. She had all these exercises printed out for me to work on with Ella. She came to see her and Ella took a few steps. Her therapist was flabbergasted and proud that all those exercises she printed out were no good, all at the same time! She was in shock and agreed that Ella has taken us all by surprise!

Also, Ella decided that she does not want her binky or frog. I thought that would be a long time habit, hard for her to break. Literally just one day, she decided that she wanted nothing to do with it. Leading up to it, I had taken the binky away during the day because she would constantly chew on them, breaking down the material into small chunks that would come off. As a choking hazard, I decided to only give her a binky when she went to sleep. After a few weeks of that trend, she just decided that she didn’t want the frog or binky at all.

Along with that change, Ella started waking up at nights with typical toddler behavior. She wanted to be held, rocked and put back to sleep. Yet half of the time when you would lay her back down, she would wake up and scream again. In the past, you could hand her the frog and binky to self-soothe. Not anymore. So after a week or so of this behavior and many sleepless nights, we decided to let her cry it out. I think that is hard for any parent, but especially hard when the pulse oximeter is beeping loudly along with the crying over the monitor. But we decided that if the pulse oximeter was going off too much, we would go into her room and turn up the oxygen, but that Ella needed to figure out a way to soothe herself. The first night, she cried and screamed for an hour straight. It has gotten easier, but is still a challenge. I think if she could walk and talk, she would ask me to sleep with her. This is why it seems to be a typical toddler thing.

Since Ella is not chewing the binky throughout the day, she decided that she wants to chew her hands. So the skin breakdown on her hands and face around her lips and cheeks is really bad. That behavior along with the dry, winter air is causing her poor hands and face to be so chapped and red. Ouch! We keep using Desonide and Burt’s Bees Wax (we are limited in our choice of products because of the oxygen and because Ella is likely eating whatever we put on her hands and face). We just keep applying and applying…

To get my attention, Ella has been grabbing for my face to turn it to look at her. The only problem: sometimes her hands catch my eyes or earrings. Or she thinks that the harder she uses her hand, the better the response. Well needless to say, it hurts a lot of the time. Yikes, this one is tough to get her to understand.

Not much happening on the verbal/communication front these days – most of the developmental strides are with starting to walk and the “big girl” moves like ditching the binky. Oh, and also just the wonderful, 3yr old tantrums that are fairly constant when she does not get her way. It seems to be on a regular basis each day, and the only thing that makes it probably a bit more challenging: Ella doesn’t know how to communicate. So sometimes it is hard to give her what she wants or to negotiate with her, when you don’t know what she wants and are unsure of what she understands. Hopefully that will improve as the years pass…for now, it just makes those 3 year old tantrums even more fun!

Other Family Updates
Yes, we are cuckoo. We added another member to our family of the canine variety. We decided that it would be good for Lola to have a sister to play with each day. We went to the Denver Dumb Friends League and rescued a very cute German Shorthair Pointer named Lucy. Since she already responded to her name and had such a cute name (Ella’s cousin is named Lucy), we decided to keep the name. So now we have Lola & Lucy. From the start, these two dogs bonded and are the best of friends. It is really cute and disgusting to watch them play, groom and sleep together. Lola is happy to have a buddy to keep her company all the time.

Grandma West and Richard came out for a visit over Halloween. Josh had earned some time off from work for all the late nights he’s been putting in, so that worked out well. We had a great time as a family going around to various landmarks in the Denver area and surrounding mountains. We ate at the Denver Chop House and visited Downtown Denver, the Denver Zoo, Evergreen, Blackhawk/Central City, Garden of the Gods. And of course, we used the RV to transport everyone comfortably. Grandma had a great time helping us get in the ghoul spirit and doing various crafts as well as dressing up Ella in her tutu and passing out candy to the trick-or-treaters. And of course, we had to play the Wii.

Finally, a highlight of October: we found someone to provide respite care! Respite care is basically babysitting for special-needs kids (one of our benefits from Medicaid). We’ve had Medicaid since September, but have not had anyone to provide respite care. In this case, respite care can be provided by anyone that we trust to take care of Ella (family member, friend, etc.). One of Ella’s therapists from the school district found out that we did not have anyone and offered to distribute our need to all the teachers and para educators in the school district. Sure enough, a young grad that takes care of moderate to severe needs kids at the local high school offered to babysit Ella. We’re so excited. Definitely a blessing to our family. We’re looking forward to date nights again — we haven’t had one since Aug 2009.

Another blessing this past month: another mom of a CDH kid emailed us after happening upon Ella’s blog. She thanked us for Ella’s story and simply shared that her daughter was born at Shands, her healthy was sketchy at times, she was developmentally delayed, but she has exceeded all of their expectations: she is now 15 and a freshman in high school. Getting that email was so cool. Sometimes when you are in the midst of the day to day trials, you forget the bigger picture. I know we’ve provided hope to parents that are just getting the diagnosis of CDH. But to hear from another mom of a 15 year old CDH survivor just provides hope for our journey and Ella’s future days.

Josh had this upcoming Monday off, so we decided to get in the RV and head to the mountains for a change of scenery, last minute. Although it was warmer in Denver and we probably should have stayed there to enjoy it while it lasted, we opted to go camping for the weekend. That is where we are writing this post. Looking out at the gorgeous mountain scenery and enjoying family time.

It was kind of funny – Josh started to feel a bit of altitude sickness when we first arrived due to lack of oxygen. Then he realized that he could create his own trip to the oxygen bar! Thanks to Ella for letting Dad use some of your oxygen. After about 30 minutes, the headache and nausea subsided.

Looking forward to this holiday season…we will try to update with any new developments sooner versus later!