We saw his doctor this morning. He still needs oxygen each evening when he goes to sleep. Since the virus he had is completely out of his system now, the doctor is not comfortable with his low saturation levels on his own. He is starting him on an inhaler-type steroid to see if that helps to open his airways and keep his oxygen saturation levels higher. He also lost the 6 pounds he gained last time so we had to raise his formula level back again to try to maintain but not gain. Always a balancing act! We are also changing his sleep aid medicine to see if he can maintain his sleep cycle with nothing stronger than melatonin. The sleep aids he has been taking have negative interactions with tetrabenazine so we want to avoid them if possible.
Philip’s wheelchair will be in next Monday! Yeah! We almost didn’t believe it when we received the call to schedule his fitting. I had to clarify that the chair was actually in and that this wasn’t more measurements. We are looking forward to the support this chair will give to him and it’s also about time for Joyanna to take over his current one.
Joyanna has not been doing very well the last week or so. I can truly tell a difference that would point to regress because of the disease and not just seizure activity. Therefore, we’re not sure changing a medicine right now would help her. She is tiring more quickly when eating so is not eating as much. She is also having more trouble chewing and swallowing. She has lost 2 pounds since the beginning of December so a formula raise is due for her soon, as well. She is also very wobbly and falling often. She has been able to maintain the words that she says most often, even trying to sing from time to time.
Of course, for her we’re still waiting on Brineura…that’s the brand name/trade name for the enzyme replacement. No word on its progress through the FDA approval. I did some reading on it last night and supposedly there is a deadline for the FDA’s decision by April 27. I’m still praying that they will come to a decision sooner than that. In fact, an original deadline agreed upon by BioMarin was January 27. This was extended due to a major amendment on the drug. I don’t understand everything in that red tape world so I don’t read all the specifics but at least we know how to pray. I am including this link if you are interested in watching a short video describing Brineura and its effect on NCL. It was done by BioMarin, the pharmaceutical company producing it. There is also a pdf that is a quick guide to Brineura. They are both well done and very informative.
Click here for video.
Click here for pdf.
We all know how we would like to control the situation. It is frustrating to think the drug is just a little over 900 miles away but it might as well be on the moon. We will keep praying it will be only a little over 9 miles away at OU Childrens and available for Joyanna here in OKC. Whatever happens, we are assured it will be good and right as it has been ordained by a very good and loving God. We must continually give Joyanna and Philip (and Ethan for that matter) into God’s hands to fulfill the purpose He has for them. That will be good and right. Only in that way, will His name be truly glorified. Sometimes it breaks our human hearts to know it might be a harder route than we might choose, but we know in the end we will see His hand in it all.
Trust His Heart
All things work for our good
Though sometimes we can't
See how they could
Struggles that break our hearts in two
Sometimes blind us to the truth
Our Father knows what's best for us
His ways are not our own
So when your pathway grows dim
And you just can't see Him,
Remember you're never alone
God is too wise to be mistaken
God is too good to be unkind
So when you don't understand
When don't see His plan
When you can't trace His hand
Trust His Heart
He sees the master plan
He holds the future in His hand,
So don't live as those who have no hope,
All our hope is found in Him.
We see the present clearly
But He sees the first and last
And like a tapestry He's weaving you and me,
To someday be just like Him
God is too wise to be mistaken
God is too good to be unkind
So when you don't understand
When don't see His plan
When you can't trace His hand
Trust His Heart
He alone is faithful and true
He alone knows what is best for you
God is too wise to be mistaken
God is too good to be unkind
So when you don't understand
When don't see His plan
When you can't trace His hand
Trust His Heart