James had a super first Christmas, although he got a little tired out. Thank you to everyone who sent him gifts. He is actually still in the process of opening them. There were so many we decided to space it out.
We had a great visit to Dr. Saffran, the ENT (ear-nose-throat) doc yesterday. He seemed surprised that the Carter Center would rule out James’s ability to smell from his MRI. It is such a small part of the brain that he thought it would be hard to tell for sure that it was not present. This was just his reaction, nothing conclusive, but it was encouraging that he was at least being optimistic.
The best news was that the doc was able to see James’s left eardrum (his better ear) using his little pokey instrument with the light on it, something that was not possible when we visited six weeks ago. James has had probably 20 tympanograms on that ear, where they bounce sounds off his eardrum under different pressures and check for its response, with almost all the results being abnormally “flat.” This meant that his eardrum had fluid behind it or that it just didn’t move for some reason. This time, on the first try the tympanogram showed a tiny little curve that indicated a response, and the second confirmed it. His left eardrum is starting to move more! Also, Dr. Saffran sounded a tuning fork on both sides, and James turned toward the sound for both ears!
Dr. Saffran was very pleased with James’s improvement, but he still wants to know more about how well James can hear on the left and whether we need to get him a hearing aid. The right (smallest) ear, which probably has an incomplete ear canal with a blind ending, can be helped through surgery in a couple years, but the left needs to hear well now while he is trying to learn language. To know more, we have to put him under sedation and do a CT scan to see his bony ear structures and a threshold auditory brainstem response test (ABR) to define his amount and type of hearing loss. I haven’t gotten a date for this yet.
When I called John with the good news, he remarked that at least we don’t have to worry about telling James something that goes in one ear and out the other.
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Lest the last couple “serious” posts mislead you, there have been many bright moments recently to rejoice over. Pour yourself some eggnog and sing along with us:
On James’s first Christmas, twelve things we celebrate (1):
Lots of hand-me-downs (2)
Nana’s cooking lessons
Free formula and diapers
A back-up Mic-Key button
Free Christmas dinner (3)
“First Christmas” ornaments
Turning toward his name (4)
$500 anonymous gift! (5)
A healing Mic-Key site (6)
A three-dollar hospital bill (7)
Two grabbing hands,
And NO cystic fibrosis (8)!
(1) Well, more than twelve, but we are trying to fit the song here.
(2) Thank you, Jessica and Frank!
(3) Dr. Yang, Dr. Desrosiers, and Dr. Banks’ Pediatric Endocrinology office chose to bless our family this Christmas with a Christmas dinner from Honeybaked Ham and a bunch of gifts for James which we haven’t finished opening yet. So far we’ve seen oodles of diapers and formula! They also asked if there was anything in particular we needed…and as a long shot, I told them we needed another Mic-Key button as a back-up in case his had to come out or was pulled out. (A Mic-Key is the stomach button that snaps open and shut and has a tube that screws in when we need to feed James.) We had to replace his first one because the balloon that holds it in was leaking and the site was getting irritated, and Medicaid would not cover a second back-up in less than 90 days. Our medical supply company said we could buy one for $185, which I figured was beyond what the Endocrine office wanted to do. But what do you know…they had one sent to us in the mail right away.
(4) James never reacted to his name being called until yesterday. I got on his left side and called him when he was looking to the right, and twice he turned and looked at me. I don’t know if he is responding to the name or just my voice, but either way, it’s progress! At the neurologist’s office last week, Dr. Barr hit a tuning fork and found that James definitely heard it when it was touched to his bone, but not through the air. This confirms that he has the sensory-neural equipment needed to hear sounds, just not the conductive ear apparatuses to get the sound out of the air. It will be much easier to fix his ear than it would have been to fix his brain’s ability to hear. Dr. Barr seemed very pleased with how well James was doing. He said the angry breath-holding fits would probably decrease with age.
(5) This came from someone at our church (Orlando Grace).
(6) This makes James much more cuddly again since his tummy doesn’t hurt.
(7) Incredibly, we got the final bill for James’s 135 days in the NICU. Although I know the NICU price CIGNA was charged was over $500,000, which did not count all the doctors’ visits or surgeries, the final cost for us after insurance and Medicaid was $3. We wonder what exactly that covers, but we are afraid to ask in case they decide we should pay more.
(8) Yep, the results came back and he is totally negative for about 90 types of cystic fibrosis. PRAISE GOD!
Our Journey, Our Journey (chronological) | Comments (4)
Categories: Meditations on Scripture, Our Journey, Our Journey (chronological).
based on Psalm 140
Rescue me, O Lord, from my broken body;
protect me from pain and distress
which lies in my tummy and blood sugar every day.
The needles are as sharp as a bee sting;
the medicine is bitter to taste.
Save me, O Lord, from the prophecies of my diagnosis;
protect me from developmental delays that would keep me a baby forever.
The doctors have read my mind’s picture
and they foresee wheelchairs, diapers, and an untrained tongue.
O Lord, I say to You, You are my perfect Doctor;
Hear, gentle Healer, my cry for mercy.
O great Therapist, my strong Deliverer,
who protected my life in my mother’s womb,
Do not fulfill the predictions of the researchers;
do not abandon me to the statistics or the likelihoods;
but humble us all with Your glorious work in me.
Let my brain grow to its proper size,
my lips form words and sentences.
Let my muscles grow strong and coordinated,
my ears understand my mother tongue.
Let my sugar play a happy melody in my veins,
my hormones in harmony as I need them.
I know that the Lord will look out for me especially
and hear my prayers poured out in great need.
Surely the righteous will praise Your name
when they see me before You, healed, today or in the hereafter.
Meditations on Scripture, Our Journey, Our Journey (chronological) | Comments (7)
Categories: Meditations on Scripture, Our Journey, Our Journey (chronological).
I think I know a little of how Mary must have felt, having a famous baby that attracted adoration from multitudes and gifts from complete strangers. James has received presents from people he has never met, people his parents have never met, even anonymous gifts left on the doorstep (thanks for the onesie and the fabulous diaper bag, whoever you are). People visit from down the street and out of town and out of state and want to meet him and bring him something. Others give their gifts in advice, prayer, and encouragement. We still don’t know how some of the people who email James and leave comments discovered his “star,” but he has certainly has amassed a large fan club.
Although the wise men’s gifts had the luxury factor, they certainly weren’t your typical cute baby gifts. They were practical provisions for the Holy Child’s future–from gold needed for the family’s sudden sojourn in Egypt to myrrh for anointing His body for burial. I wonder how much of His path Mary was able to divine from their offerings. I’m not convinced she knew the ending at the beginning of His life. Scripture says “she treasured up these things and pondered them in her heart,” and I wonder if she ever worried like I do about her baby’s future. Jesus had a lot foretold about Him that she might have known about. James’s recent MRI reading predicts his future, but we don’t know how much of it will come true.
Sometimes I take the news from the MRI in stride, and other times it weighs on me. The first night I had to shut myself in the bedroom and cry. The disappointment is great, and the fears are sometimes uncontrollable. I find myself wanting him to stay a baby forever so no one will notice that he is tiny, can’t walk, talk, or be potty trained. This is sad because it keeps me from enjoying his growth. Obviously it is the wrong reaction, and shows my fears of what the world will think of him. I want to protect him.
Other times the future seems distant as I deal with the present day-to-day of doctor’s appointments, feedings, therapy, and sugar checks. I get excited because he reached out and grabbed his rattle for the first time, or touched Nana’s face, or I get absorbed with how to fix his digestive problems with gas and constipation. He smiles, or better yet, laughs his super-cute laugh (in which he lifts his chin and smiles extra big and says “Hmm!”).
I forget about his looming developmental problems until someone in a doctor’s office casually asks if his small head is connected to his diagnosis. I forgot he had a small head…I forgot he is too small, too uncoordinated, too immunologically fragile, too skinny, too behind in so many ways. He starts to feel normal until someone points out a deficiency. Then, suddenly, it all rushes back and the progress I’ve been encouraging myself with seems too small to matter, like a naive but ultimately empty hope. I have to fight these feelings of defeat and despair with faith in a God that is not limited by medical diagnoses.
I keep reminding myself that James is a blessing, but he is under the shadow of a curse. His medical problems, imbedded in his body since conception, are the result of human disobedience and pride, which brought evil into the world long ago. The Christ child came to free us from this curse and make it possible for us to escape sickness, disease, and pain in an eternal life with Him. Someday, I hope James will be free of his limitations, able to smell, hear, and think normally, possessing muscle coordination and a functioning pancreas.
For now, like Mary, I watch and wait in faith that however well he progresses, my baby will touch many people’s hearts and bring glory to God. The gifts of James’s “magi” are not only practical provision for his needs, but also reminders of God’s faithfulness when the future seems uncertain.
Meditations on Scripture, Our Journey, Our Journey (chronological) | Comments (3)
“Therefore the LORD waits to be gracious to you,
and therefore he exalts himself to show mercy to you.
For the LORD is a God of justice;
blessed are all those who wait for him.” Isaiah 30:18
This post will be a throwback to the old days of medically specific verbiage and sad news, so brace yourself. Here, I’ll throw in a picture to get you rolling.
Today Nancy Clegg from the Carter Center for Brain Research in Holoprosencephaly called. She had the official reading of the MRI of James’s brain. It listed which structures he had, which he didn’t, and how much his various components were fused together. She also explained what was typical for other patients with HPE.
They confirmed that he has semilobar HPE, which is the medium level between lobar and alobar. Actually, they said “Severe SL,” which I assume stands for severe semilobar.
First, she said James’s brain had a lot of basal ganglia fusion, which affects the area of motor skills. Anything requiring muscle coordination will be hard for him, including swallowing, sitting, reaching, grasping, walking, and talking. She said even pooping will be hard, because that requires muscle coordination, too. She said he probably will never be completely potty trained.
This is not to say that he can’t reach any milestones, but it will probably take years. She said one kid they were following took 3 years to learn to sit correctly. Of 43 semilobar patients in their study, only 2 can walk with or without assistance and only 2 have normal upper extremity function. One third of them can sit independently by 18 mos., one half can reach for objects after 1 year, and two thirds can hold objects after 1 year. Eight of 35 patients could speak words after 1 year old, but most of those were less fused than James. So this is going to be a major, major challenge. Nancy said he needs aggressive therapy, and lots of it.
She said his hypothalamus and pituitary looked good, which is a praise about the hypothalamus because we had been worried about it (thanks for praying, Chantel). The pituitary may look good, but it still doesn’t work, so that’s a mystery. When Dr. Barr saw the MRI months ago, he said the pituitary stalk was so thin it might keep the hormones from getting out, but it would be too tricky to try and fix it since it’s right next to the optical nerve. Still, adjusting for the pituitary is at least possible, while adjusting for the hypothalamus would not be.
His optic chiasm is intact but “dysgentic,” or oddly-shaped. We asked the eye doc about it today, and he said at this point James’s vision seems fine, but we should keep coming in for check-ups.
He needs his eyes because his MRI shows he is lacking his olfactory equipment; in other words, James can’t smell anything. Nancy Clegg said this would affect his eating as well, because it would make foods taste less, like when you have a stuffy nose. She said he would probably be more picky about food based on its texture rather than its taste. HPE kids who can’t smell usually fall into two camps: those who prefer firmer foods like mashed potatoes, and those who prefer slimy foods like peaches. (The fact that she doesn’t mention anything like steak or hamburgers also tells me something about what James won’t be eating.) Although this is bad news, James is already ahead of the game because he eats by mouth regularly. Two thirds of HPE kids need g-tubes to eat.
Because of the brain fusion, James is going to have a hard time processing information and interpreting what he sees. Things like cause and effect will be hard for him to grasp. He may get some mental work-arounds with practice and therapy, which is something to pray for. She said a lot of HPE kids are misdiagnosed as severely retarded because they are unable to show what they understand, but they often understand quite a lot. He may be frustrated and fussy a lot because he can’t express himself. There are some toys and communication devices that we can get for him to help him tell us what he wants when he gets older.
Being an English teacher, I asked if he would be able to read. She said probably not. HPE kids still like to be read to, she said, so keep doing that–they like images, pictures, and pop-up books. He may learn to recognize a few words. But he will not be able to pick up a book and read it by himself.
She also confirmed that he doesn’t show any cortical malformation, which causes seizures that are difficult to control in half the HPE patients. His little “hold his breath/turn blue/scream/faint” fits were explained as muscular dystonia, which she said is common in the back and arms of HPE kids. She said if he stiffens up, makes fists, and arches his back when he is aggravated or upset (if you have known James very long you have seen him do this!), it is involuntary muscular stiffening, not to be confused with spasticity, which is constant muscle tenseness. HPE kids often have spasticity in their legs, but James does not. Maybe that will help him beat the walking odds.
Beat the odds. Who am I kidding? In the day-to-day of doctor’s appointments and feedings and sugar checks, it is easy to forget how difficult a road we have ahead, but today I was reminded, and it was tough. Sorry, John, that you have to read this far away from home. May God grant us wisdom to know what to work on and patience with James every day. Help us to love him first and foremost as Your beautiful creation, and not based on his performance or lack of it. Help him not to become bitter about his limitations, but may he be always full of joy and peace, like he was this morning.
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