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!)