Posted (jooosh) in All Posts on February-27-2011

Hey there Ella fans. It’s been awhile since I posted. Tina’s been doing the lion’s share of keeping you all updated, but I felt prompted to give an update.

Ella was in a real honeymoon period right before our last post. It felt like we had things dialed pretty good and overall she was doing well. I think it’s always my hope that, after a big surgery, that we’ll get a good long break between any issues. It’s also a pretty typical trend of Ella’s to not let this be. We’ve had some decent stretches over the last 3+ years that were relatively uneventful, but nothing that really gave us a chance to escape into what one would think as “normalcy”.

I share this to say that this stretch only lasted a week, and right after our previous post, Ella starting to show new signs of trouble. All day last Sunday (2/20) she was grunting throughout the day, almost like she was constipated. The weird thing is that she wasn’t. She was also showing signs of discomfort after feeds, which caused her to pass out. There were symptoms of what we thought was reflux as well. Ella would often start coughing after a feed, as if something was in the back of her throat. All these symptoms were a bit disconcerting.

This trend continued to intensify throughout the next day (Monday) which really started to cause concern. Ella passed out a total of 4 times on this day. I was feeling anxious enough to request that we head off to the ER to get some tests done, but Tina didn’t feel she was in a real emergent situation, plus we did have multiple checkup visits already scheduled that week. I trust Tina’s gut in these situations and know that she had a good feel for Ella’s state. We agreed to see how Ella behaved the next day. Tuesday came and unfortunately it was the same as Monday: grunting, coughing, and, once again, passing out 4 times.

The one thing that did give us some solace was that there were moments where Ella was behaving normally. She was consolable, but there was no denying that something was going on inside her and it was causing severe discomfort.

On Wednesday Ella had a followup appointment with a neurologist. She had an MRI of her brain when she was in the hospital in January. This was the appointment to discuss details around the results. I was at work that day but Tina emailed me a list of questions that she was going to ask the doctor. Just consuming the reality of the questions we were going to ask was overwhelming. To give you a taste, here’s the list we came up with:

  • How does Ella’s brain compare to a “normal” brain her age?
  • Is the global cerebral atrophy the same or different than the NICU scan?
  • When the atrophy shows that the brain is smaller than it is supposed to be, does it stay the same or can the rate of growth change? In other words, is she a certain percentage smaller, like 40% smaller and we can expect that to remain constant or can it change? Can it get better or worse?
  • Are there any environmental factors that impact global cerebral atrophy?
  • I know you cannot predict and Ella can always surprise us, but I’m trying to get a realistic perspective of what I can and cannot expect my daughter to accomplish as she gets older. What do other children look like with this diagnosis, as they age? What types of things are they able to do or not do? Can you share some examples of different outcomes you’ve seen.
  • The best plan of action: continue OT, PT and Speech Therapy and try to make the connections for simple tasks?
  • Do we IQ test at school age, like 6-7 years old?
  • Is this the type of diagnosis that goes along with Mental Retardation?
  • Do we ever need another MRI? or only if we see further neurological symptoms? What are symptoms that I should be aware of to indicate that further testing is needed?
  • What are the next steps for Ella (what is her health care plan neurologically)?

I highlighted the the item above because it’s the question that, in my opinion, stood out more than any other. We knew since being in the NICU with Ella that she sustained some brain damage. We also knew that it was pretty bad, but it’s never been clarified how bad. With this latest MRI though, we were hoping to get more insight.

The images below show the structural part of a normal brain and the part of Ella’s brain where her injuries occured, likely due to lack of oxygen. But the MRI does not tell you how the brain functions. The neurologist compared it to opening up the hood of a car and looking at the engine and its various parts. Doing that is not going to tell you how the car is going to run, but in can give you some insight. More importantly, they use developmental scales in various areas to test children and see how their brain is developing. Here are some other tidbits we learned:

(this is a normal MRI image, grabbed from Medscape.com as an example)

(this is Ella’s recent MRI, frontal view, reversed right to left)

  • You can see the part of her brain that died off due to a stroke in the upper left side of her brain (seen on the right here).
  • Ella’s brain is about 20-30% smaller than it should be for a child her age due to global cerebral atrophy. The white matter should be tight like cauliflower, pressed all the way to the edges of the skull. See all the extra fluid and how it is open and flowery? That is the global cerebral atrophy that makes her brain smaller.
  • Ella’s head circumference is on the growth chart; this means that her brain has grown as she has aged. The doctor thinks that it will continue to grow, based on the NICU MRI and the recent one. This means that unless we see neurological symptoms that are alarming, we do not need another MRI and her brain is expected to grow and maintain a 20-30% deficiency as she ages.
  • The corpus callosum is the main transverse tract of fibers that connects the two cerebral hemispheres. It is made of more than 200 million nerve fibers. The primary function of the corpus callosum is to integrate motor, sensory, and cognitive activity between the left and right hemispheres. Ella’s corpus callosum was the area most impacted by lack of oxygen. It has thickened since birth, but it is not the thickness that you normally see for a child her age, so she basically doesn’t have the amount of nerve fibers that would be ideal for learning and growing developmentally. This means she will be able to learn certain associations (like she has with walking), but learning simple tasks and associations takes lots of repetition, therapy, etc. and there is no guarantee which types of tasks she will be able to grasp.
  • Since the MRI only shows the structural part of the brain, the neurologist pulled out the Denver Scale (a test for screening cognitive and behavioral problems in preschool children). The scale reflects what percentage of a certain age group is able to perform a certain task. Tasks are grouped into four categories (social contact, fine motor skill, language, and gross motor skill) and include items such as smiles spontaneously (performed by 90% of three-month-olds), knocks two building blocks against each other (90% of 13-month-olds), speaks three words other than “mom” and “dad” (90% of 21-month-olds), or hops on one leg (90% of 5-year-olds). Ella scored between 12-15 months old in gross motor skills and 6-9 months old in the social contact, fine motor skill and language areas.

Tina received assurance from the neurologist that she expects Ella to continue to move forward and make progress, albeit slow, just like she has over the past few years. The doctor also noted that society no longer uses “mental retardation” for classification these days, but instead would use the term “developmentally delayed”. However, the neurologist also made a point to state that the professional community does not use this terminology any longer either because it gives the impression that Ella is simply delayed and that she will inevitably gain these skills, which she very well may not. So, the doctor labeled her as “intellectually and developmentally disabled”.

Knowing that Ella will continue to move forward, but not at a rate allowing her to catch up with other kids, Tina pressed the doc for a more specific answer even though it would probably be tough to swallow. After talking back and forth Tina pinned the doctor down by asking this question: “So when my daughter is 16 years old, will she probably only function at the level of, let’s say a 5 year old?” The doctor nodded and responded, “That is a reasonable assumption. Put it this way, Ella will not be 16 years old and functioning like a 14 year old”.

Ugggghghhh….the reality of this was like a punch in the gut. I knew this punch was coming, but I just didn’t know how bad the punch was going to hurt until now.

OK…so before you go off and start thinking about telling us how doctors don’t know exactly the outcome, and that she could surprise us, and that we never know where she’ll end up, and it’s in God’s hands, and it’ll be ok, and that she’s amazing…well, your right…all that is right…and we know this truth. BUT the reality of the moment is that we have an amazingly sweet precious little girl with a brain injury that will affect HER and US for the rest of our lives. We don’t know what the future holds, but the impact of this revelation provided by these details is deep.

Seeing Ella deal with her physical issues along with this news just really wrecked me. It’s been real heavy for Tina and I to process and I thank God that we can at least cling to each other through it.

On top of this, Ella continued to have her spells after the feeds so I ended up taking Thursday off work to help out. It can be so absolutely exhausting taking care of Ella, and I’m only doing it part time! I can’t believe the Superwoman Wife that I’m married to that does this full time and also maintains any sense of sanity!

Ella had a GI appointment scheduled for Friday morning but after consulting with the docs earlier that week, they requested that we see the surgery team as well. The GI docs were concerned about the possibility of issues related to the procedure in January which is what prompted them to have us consult with surgery. In addition, they had Ella take a chest xray prior to Friday, just to make sure her diaphragm was still intact.

While driving to work on Friday morning, I talked to Tina as she drove Ella to the hospital. Our conversation was thick with emotion around our daughter’s current situation. I was less than a mile from work when I was consumed by my wife’s mood and my own despair. My current state of mind convinced me to call off going to work that day and instead head to the hospital to be there with my family.

Upon arriving I hugged on my little girl while the GI doc talked through her symptoms and some possible ideas hoping figure out the cause of her issues. Her chest xray looked good – diaphragm still in place. There was no clear direction on what Ella was dealing with, and, as usual, she kinda stumped the doc on what path we should take. On top of that, we had already tried a variety of things to help Ella out and the majority of his solutions covered those same actions. Reviewing Ella’s condition with the surgeon also yielded the same results.

They did agree that Ella should come back in for an upper GI and small bowel series with contrast to determine if there are any obvious reasons for her issues. Tina will be taking Ella in tomorrow (Monday 2/28) for this test that will literally take all day.

Even through all this we decided to take a break on Saturday and escaped in the RV with some friends. We went up into the Evergreen area and had lunch at BeauJo’s. It was nice to get away and Ella did pretty good with minimal episodes.

As of today, it seems that Ella’s symptoms have subsided a bit (Thank God!), although she did pass out once this evening. We’ll see how the test goes on Monday. Hoping they don’t find anything that would require another surgery.

A couple items to share on the lighter side:

  • Ella walked over to the cat and pet her on the back for the first time. It was so awesome to see Ella do this on her own and with such intent.
  • While Tina was sitting on the couch, Ella decided to pull herself up onto Tina’s lap with minimal assistance. Once again…very cool.
  • Ella is now able to climb onto her bounce and spin zebra on her own!

It is the moments like these that are so incredibly cherished.

Heavenly Father: Thank you for your sovereignty and for being the creator of the universe! We know you have a plan for Ella Renae, and it is not to harm her, but your plans are to give us hope and a future. Lord, instill in us that hope so that we may trust in you daily. We praise you Lord for our sweet child. Bless her and heal her Lord. We pray that your Kingdom will be greatly impacted by her life. We pray these things in Jesus name…Amen

I’ll leave you all with something that God put on my heart a few weeks ago during my own personal spiritual wrestling: The verse isn’t “Be still and FEEL that I am God”, it is “Be still and KNOW that I am God”.

Posted (Tina) in All Posts on February-19-2011
WARNING: Graphic surgery photos below

It’s been a month since we’ve been home from the hospital and it has been busy!

Ella had a rough few weeks at home getting her body adjusted back to everything. The first week, we continued the Compleat Pediatric diet regimen as we had tried at the hospital post-surgery. In hind sight, this food did not work great the few days prior to surgery so I’m not sure why we thought it would be that different after surgery. As expected, the Compleat Pediatric diet caused all kinds of issues: tummy distention, gas, etc. After a feed immediately and for several hours, she looked like she was 9 months pregnant with twins! And we would vent or decompress between feeds and we were not getting a bunch out of her g-tube. This meant that the food was draining from her tummy fine, but I think it was making her GI tract very unhappy, thus the distention. She was not going #2 on her own and when we would use stimulants such as Senna or suppositories, we would get watery diarrhea. We were doing lots and lots of laundry. It was a frustrating time knowing that Ella just felt miserable.

After the first horrendous week, Josh suggested we switch back to the homemade blenderized diet. Her tummy did not seem quite as distended, but she was still uncomfortable and fussy after feeds. I tried smaller amounts, several times per day and it did not seem to be making much difference in her comfort. Simultaneously, we switched back to using Miralax, because our thought was to see if we could get her back to what was working the best prior to surgery. At the same time, we decided that it was time to start therapies again so we called all of her therapists, so we could get back on their calendars.

At the 2 week timeframe, we went to visit the surgeon as a follow-up. Her scar has been healing well. He also gave us photos from the operating room. It was amazing to see just how large the opening was in her diaphragm.

The same day, we also went to the GI doctor to discuss our feeding and GI challenges. When Ella was weighed: she came back at 12.3 kilos (27.06 pounds) which is the low point she was at right after surgery. In other words, she had lost more weight since being home from the hospital. So, we came up with a plan to try and see if we could make some improvement:

  1. Continue increasing Miralax to a point where Ella is going several times per day, with the right consistency that is not causing her to strain in any way
  2. To help with weight gain, add one more packet of MCT Procal to her food (3 packets daily) along with 12 scoops per day of DuoCal, another calorie enhancing powder
  3. Substitue items in the blenderized diet to reduce the volume we need to give Ella to 1000ml per day, so we can get the biggest impact (e.g. instead of 8 oz of fruit juice, substitute a jar of baby fruit)
  4. Call in an order to Apria for a new food pump that can be worn in a backpack – try using food pump to see if slower drip will make her more comfortable

So, I found a backpack in the basement that was given to us by a children’s foundation on one of our various hospital stays and put just a tiny bit of weight in it. I wanted to see if Ella would tolerate wearing the backpack. We switched out the food pump with Apria and started to try the drip feeds. Once again, Ella proved to do worse with drip feeds. I’m not really sure why, but she seemed even more miserable. One again, we determined that Ella does not follow the rules. She tends to do better with a larger volume given all at once and longer breaks in-between feeds. The only part that was great: seeing my little girl walk around with a backpack on – she looked so cute. It seemed like she was going to run and stand outside waiting for the school bus.

Oh, another thing we tried and it didn’t work great, but we just had to try: we found ourselves out in the RV and forgot her stroller. So when we went into a restaurant, we carried her O2 tank and accepted the offer of a high chair – that was a first. Did I mention that putting the O2 tank on the back side of the booth and running your over-sized touché into it on more than one occasion does not feel good? Once again, it only lasted moments (I ended up having to hold her) but she looked like such a big girl!

One positive outcome right away since surgery: she is passing out way less frequently. The only times now seem to be when she is in some type of really bad pain, like gas. I only know because at the height of the pass out spell, she relieves a bunch of gas. And I think all of us know what painful gas can feel like – I guess she just doesn’t have the reserve in that moment to take another breath. But the episodes are much less frequent. Also since the cath lab procedure and being home, we’ve been able to reduce her oxygen requirement to 3/4 liter versus 1 liter. In the past, we’ve had these reduced oxygen periods after a cath lab procedure – everything is nice and wide open. This time, we’d like to think that she is getting stronger, but only time will tell.

Ella is getting more used to playing on the iPad, specifically with an app called “ABC Wildlife”. When I turn it on for her, I can see her getting more interested and engaged. She’s even started to try and tap the screen herself. My favorite thing: the iPad will be turned off, she will see it, walk over and start tapping on the dark screen. No words necessary – I think I know what that means!!!

Another big project this past month: changing insurance. Our COBRA coverage ended on 1/31/2011, so we finally made the switch to a new insurance plan. Plus Ella was approved for Medicaid last fall, but we had already reached all the out-of-pocket maximums for the year, so we really did not end up using Medicaid for doctor visits or medicines. With the new year, it meant getting everything lined up with Medicaid too. Many of Ella’s medications require prior authorization from the primary insurance as well as Medicaid, in order for them to be covered. We even had to go through and switch her most expensive medicine: sildenafil. Even though it is thousands of dollars more, the primary insurance as well as Medicaid would not cover this medicine for pulmonary hypertension. Why? Because it is the same main ingredient found in Viagra. So, we had to change her prescription to Revatio. This is the brand name of the drug whereas sildenafil is the generic name for the drug. The difference in cost is thousands. Silly that they will not see her medical case and simply make an exception for sildenafil because it would cost the insurance company and Medicaid way less money. Another frustration: Medicaid is supposed to cover the costs that our primary insurance will not. But the system is tricky – if the pharmacy makes a medicine starting from a powder versus crushing tablets, can be the difference of whether or not Medicaid pays the difference. So this month, we were able to resolve all but two of the compounds. Hopefully, next month we’ll be able to figure out all of them so that we do not have to pay any put-of-pocket costs for meds (which previously has been hundreds of dollars).

We had 3 visitors since our last post:
A quick drop-by from a CA friend that we have known for years

Grandma West for 9 days

Cousin Pete for the weekend

Grandma West did start her huge project of hand painting Ella’s table and chairs, but only got 1/3 way through, so we don’t have any unveiling yet. Here is an unfinished chair – I think you get the idea – it’s going to be very fancy and princess-like.

It was definitely snowy and cold while Grandma West was visiting!

It has felt like a baking extravaganza this past month. When Grandma West was here, we made a couple batches of cookies and a carrot cake for my Bunco group. Then for Valentine’s Day dinner when Cousin Pete was in-town, I made a layered red velvet cake (that was a first) and chocolate covered strawberries.

Finally, Josh’s work had a company meeting and asked everyone to bring something homemade. I opted to make another carrot cake and when I had leftover frosting, my creative juices got flowing and I put their logo on the cake. I must admit, it was fun!

We went to the pediatrician this week, just for a follow-up since our big procedure. I let Ella out of the stroller to walk around and strut her stuff. The doctor was so pleased with how she looked and her increased exploration and interactive skills. The last time he saw her was late October when we were first trying the blenderized diet – she was still retching a lot, uncomfortable, feeding/pooping challenges, she was not walking, etc. He was really happy with her progress. Also, the scale looked favorable. According to his equipment, Ella has gained 2 pounds. We’ll see if that is the real weight gain when we see the GI doctor late this upcoming week. The reality is: I’m hopeful that we have weight gain that is not fluid-related.

Josh had a chance to go snow boarding with friends from work! I was jealous…

One bit of heavy news: we also learned that Grandma Eusey has endometrial cancer and needs surgery in mid-March. We’re hoping and praying that is all she needs to eliminate the cancer completely. Please pray for Ella’s grandma!

For those of you that were praying for Elijah, I wanted to report that he was sent home from the hospital on February 7th, so just under two weeks ago. Pictures are worth a thousand words, so I think all I need to do is post this picture:

Tonight, we get our first respite session since December, before the holidays. No offense against the Bun, but it will be nice to take a break this evening and let someone else play with her!

And then when you have the rough days where just nothing seems to be going right and Ella is fussy and the dogs are crazy, you can get surprised by a message received from someone you don’t know:

Hi hope and pray your baby Ella is doing well.We have been following your blog on and off for the last 4 mnths.Our little girl Faith was diagnosed with CDH at 20 weeks. Today i felt compelled to reach out to your family. Maybe because our baby has 11 more days before she comes into this world.My wife is due March 1st for a C section.Today i just felt a connection,your little girl gives us hope for the our baby Faith. I have learned so much from your blog on what to expect.Thank you for your time in writing your blog. We will continue to follow your babys progress,we hope and pray all goes well. Thank you for all the Bible Readings in your blog they help to get through the difficult times and will help in the days to come. Thank You. May God Be with your family.

All the best to Faith and her parents as they enter the next chapter of their journey with CDH.

It’s these little nuggets that mean so much and remind you that this journey goes much deeper than just our day-to-day experiences, but that God has a bigger plan and purpose for Ella Renae. He’s guided her life since the beginning and continues to through each day.

Psalm 33:21 (NLT)
In him our hearts rejoice,
for we trust in his holy name.

Three years ago