Friday, April 6, 2012


It has been quite the week full of emotional highs and lows. Like every other time we have come to Houston, we have made some incredible and lasting memories...even more so this time than others, which I will write about in a few days. And like every other time, leaving the city limits gives mixed emotions.

We saw GI this morning for a routine appt where we primarily discussed Nate's stimulator. It is quite exciting seeing how interested everyone is in this treatment. We really enjoyed meeting the Houston Medtronic rep when Nate was in the hospital here. He was such a kind man, who told Nate after he turned back on his stimulator that he and his own 4 year old had prayed for him the night before and were going to continue to pray for him. It was such a touching comment from someone we had just met for a few minutes the day before.

He also mentioned that Texas Childrens is starting a peds GI stimulator program very soon and asked if I would mind if the doctor starting that program could contact me sometime in the near future about our experience. Of course I said I would love to help in any way I can. It is pretty exciting that other hospitals are seeing the benefits of this therapy and wanting to start their own programs.

Anyway, the appt that I was anxiously anticipating was today as well: our first visit with the pulmonologist who sees most of the mito kids in the clinic. She was such a great doctor who completely lived up to the wonderful comments I have heard about her. Being well versed in mito and in pulmonary disorders/breathing abnormalities, I have a feeling she will be a huge asset in Nate's care from now on.

We discussed so much information throughout the long and thorough appointment. And even though I had a feeling that I knew what her answer was going to be, it was still a bit disheartening to hear her say that she did not recommend we try to wean or trial down Nate's oxygen use. Due to the way his body is affected by this disease, we run the risk of making things worse by stressing his body more. Not only can we not taper down his O2 supplementation, but we are going to do additional testing in October to determine if he may need additional respiratory support at night in the form of a nasal pillow that gives positive pressure in order to help inflate his lungs better. Only, the nasal pillow is pretty new and has not had a pediatric design created for kids as small as Nate yet. Hopefully his testing will be normal and he won't need this equipment, but at least we know they are working at creating this design for peds in the near future.

It was good to get this pulmonologist's take on what is happening with the little guy's respiratory system, and it turns out that the theories our home doctors have had all along have been correct. It seems that Nate has a couple of things against him in regards to his breathing abnormalities. The first is the exact theory that our very first pulmonologist had...and how he has gotten insurance coverage of the oxygen and supplies for the little man all this time. You see, in order for mitochondria to make ATP (energy molecules), it needs food and oxygen. A big problem in Nate's body is that even when he has normal saturations on room air, his "work of breathing" is significantly increased. His body is trying desperately to get more and more oxygen into his cells by breathing harder and faster. Since he can not do so without exhausting himself, his lactic acid rises in order to provide more energy for the body. This is called anaerobic metabolism, and that term is often referred to when you talk about someone "working out"...except that Nate does not have to work out to experience this type of metabolism. Its the unfortunate effects from deficits in his mitochondria.

But when he is given extra O2, his work of breathing normalizes, he does not retract as much or as deeply, and his lactic acid normalizes. He has more energy for the rest of his organs, and he just feels and looks much better. Oxygen supplementation seems to keep Nate's cellular respiration in a more normal aerobic metabolism. This theory is not new to us, but it is nice to hear it pretty much confirmed by our new pulmonologist in regards to why Nathan responds the way he does. Some mito kids don't need extra oxygen at all, and for some, O2 doesn't seem to help at all...while some only require it at night for optimal effects, and then a few others, like Nate, require it most all of the time in order for their bodies to handle the other stresses from this disease.

The other thing working against the little guy in the respiratory arena is the fact that he is "quite neuromuscular", as his new pulmo put it. Even though he seems to be quite strong in his extremities, his trunk and respiratory muscles are weak, so that also increases his "work of breathing". It is just harder for him to take deep breaths. This is why he may need additional positive pressure help at night at some point in the future.

I asked point blank if it was possible for Nate to be off O2 for enough time during the day so that if he is healthy enough, he may attend school without the oxygen supplementation. She just shook her head back and forth and said that she did not want him attending school without oxygen. It will be too mentally and physically taxing on his body to do so without the added support. She did give permission to keep letting him have time off in small spurts to play outside or go to the park, just like we have always allowed him...he needs to have a little bit of time to run around like a regular kid without being attached. But it is very clear to her that Nathan's body needs extra oxygen to work as well as it can.

As hard as it was to hear her thoughts and recommendations, it was not something that I wasn't expecting. The question right now is how high of a liter flow he actually needs. Previous sleep studies have shown that his heart rate and respiratory rate normalize once we give up to 1L. However, he will still retract fairly often during the day unless he is on 2L, more than likely due to activity level and need for more energy. We will do more testing when we come back in October to determine what liter flow is better for Nate's body. It may even be that we have different flows for day and night.

It was a long day. We left directly after the appt (well, after a chest xray), and headed east toward home. Nate instantly fell asleep once we got in the van, so I had a lot of time to think...and pray...and experience all of the emotions you expect when you are disappointed and a bit sad. I wish it all was different some days. This doesn't change anything we have been doing already, obviously, and Nathan never knew of my desire to see if it was possible for him to discontinue the oxygen therapy, so he thankfully shares none of the disappointment of the conversations during the appt. Nate is incredibly resilient and none of this seems to bother him too much or slow him down...but I just wish he didn't have to be endure it all. I pray he remains resilient and as happy as he continues to be. I know God is with him always.


Kyla on April 7, 2012 at 9:57 AM said...

I'm so sorry you did not get the news you were hoping for on this trip. Disappointment is tough, even when you are mostly prepared for it...there is always that bit of hope that can't be stifled, which is actually a good thing, I think. We never stop wanting the best for our kids, wishing for a bit of an easier road for them.

We saw Dr. J for this first time this week, too. I was very impressed with how thorough she is! It was a very nice change from our last experience with a pulmonologist.

jengame on April 7, 2012 at 12:59 PM said...

I love the way you explain the medical aspects of Nate's needs and the care he is getting. It makes it so much easier to understand.
I also love your positive attitude, and your deep, abiding love for your children. Nathan is strong and resilient, because you are strong and resilient.
You are a blessing, Amber!
Love, Jenny

Anonymous said...

Love you and always thinking of you guys!
Love, Dawn

dlefler on April 22, 2012 at 4:23 PM said...

I wonder if another type of C-Pap mask would work for your little guy? Nolan uses a full-face mask (the FitLife), which is "technically" for kids ages seven and up. He's 32 pounds and 4 years old, so he's a pretty little guy, and the full face mask works well for him. It has a port to hook an oxygen line to - Nolan doesn't use O2, but it might work for Nate... we had a terrible time finding C-Pap masks that would fit Nolan at all, and I think the FitLife is the only one that has the ability to add an oxygen line to the mask. We never found nasal pillows small enough.

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