Well, I guess the first order of business is announcing our newest addition! Kender Allyn Hunt was born at home on July 22, 2008, weighing in at 10 pounds 8 ounces. He's a big chunky monkey, just like his big brothers were, and everybody just loves him. We named him after the Allyn branch of my mother's ancestors, who we recently found out actually come from Vermontville, Michigan, just up the road from where we live now.

Second order of business is the discovery of a genetic mutation in our family. Most of you are aware that Brian has been blind from birth. He was told his whole life that it was not genetic. This was repeated when Brian's old pediatric ophthalmologist examined the triplets in the hospital, he said there was no way they could have gotten it.

Fast forward to Kender. By two months, he was not showing any signs of seeing anything, no age-appropriate tracking, no smiling on seeing people (although he smiled for kisses and cuddles), etc. Then I noticed some white stuff in his eyes; if you looked in at an angle, with the light right, you could see all this white around the inside edges of his eyes. Because this can be a warning sign of retinoblastoma, a dangerous cancer, we were able to get an immediate appointment for him at the Kellogg Eye Center. They ruled out the cancer, but said there was a definite problem, either PHPV or FEVR, and got us an appointment with Dr. Trese for an anesthetized exam the next week.

Dr. Michael Trese is the world's leading specialist in retinal diseases, especially in children. People come from all over the world to see him; I have spoken with patients from Australia, New Zealand, the Carribbean, etc., and often their insurance pays for the trips, that's how good this guy is. He looked at Kender's eyes under anesthesia and diagnosed him with stage IV FEVR. FEVR stands for Familial Exudative Vitreo-Retinopathy, a genetic condition that leads to the full spectrum of eye problems that Brian has. At the first exam, Dr. Trese found that Kender's right eye had a fold that was covering the back of his lens, and he went in surgically and peeled that away. The left eye had a fold, but the lens was clear, so he did lasering to the retina in that eye to prevent exudate, or leakage of blood vessels, which is one of the primary ways this disease progresses. Kender has no discernible fovea and little macula, areas of the eye with high concentrations of cells used for focal vision. He also has poor vascularization of the retina. Dr. Trese says he is certainly legally blind. He has light perception, but whether he has more than that we won't know until he is older. He may have "ambulatory" vision, which is about what Brian has. Finally, they drew blood on him for gene testing.

At follow-up exams, Dr. Trese confirmed a FEVR diagnosis for Brian and found no signs of the disease in either Brian's parents or myself. The four older kids were screened in the office. Brenden had stage I FEVR, but still has perfect vision, so needs to be monitored every few months in the office for progression. Liam has no signs of the disease. Tamara and Caitlin both had signs of retinal changes and exudate, and they were scheduled for anesthetized exams along with Jarod, who is too young to examine in the office. Kender developed a rapidly progressing cataract in his right eye after his first surgery, and he was scheduled for exam/surgery the same day as the other three.

Dr. Trese's exam of Brian also showed that his lens implant had become encapsulated, meaning that scar tissue was growing on and around it. This was causing some of the decrease in vision he had noticed in recent years. Dr. Trese recommend he get a YAG laser procedure done to clear out some of that encapsulation. He had that done about two weeks ago, and is seeing much better now. Not clearer, but it's the difference between looking through a veil and then having that veil taken away, and he's been wandering around the house asking, "How long has THAT spot been there?" :)

Tamara, Caitlin, Jarod, and Kender had surgical exams last Monday. Tamara has stage II FEVR in both eyes, with lattice stresses on the retina which are at high risk for tearing. She had lasering on both eyes. Caitlin also has stage II FEVR with evidence of past exudate and minor folds around the edges of the eyes, and she also had lasering in both eyes. Jarod has stage III/IV FEVR. He has a fold in the left eye which extends into the fovea, and extensive folds and exudate in the right eye. He had lasering, and next month he will need to have a vitrectomy to remove cloudy fluid from his left eye. Kender's cataract had developed to the point of being opaque, so he had a lensectomy in his right eye, along with a vitrectomy, and is still recovering from that. Next month he will be fitted for a contact lens; he can't have a lens implant because of what happened to Brian's implant (Brian would not have received an implant at all if they had known he had FEVR at the time). Also, Kender's genetic test came back positive for the frizzled-4 mutation, an autosomal dominant FEVR mutation, which explains the huge penetration into our family. It's also the rarest mutation causing this extremely rare eye condition. All of the other children are now being tested to see if they have this same gene (most likely).

It looks like we have two blind children, two that very well could go blind at any time, and at least one more with a chance of losing vision. We are so far averaging 2 surgeries per month, at a cost of $26,000 per surgery, with I think 10% not covered by insurance (bills are just starting to roll in). And we currently live in a state with no School for the Blind and below-adequate mobility training resources; the things I see blind people doing with canes makes me afraid to let Kender or Jarod anywhere near the system here! We desperately want to move back to Texas now, preferably before Kender turns 5, but with medical expenses now eating up what little disposable income we have, I don't know if we will succeed. I want to be financially secure before risking Brian losing his great job; I want all bills paid, the car paid, and the house at least paid down significantly. I have taken on two jobs now myself to help bring in more money, and between that and making the 2-hour drive into Detroit every week (sometimes every other day!) I don't know whether I'm coming or going! But we're hanging in, just taking it one day at a time for now.

We named him Kender because he was unplanned. Kender is the name of a race of humanoids in the Dragonlance novels. They are diminutive, about the size of a human child when full grown. They are playful, they love to play practical jokes, they have no sense of private property, and they are extremely inquisitive. They also have a heart of gold. As I said, we gave that name to Kender because he came to us on his own, unexpectedly, and it seemed like a practical joke at the time. Now it seems that his coming may have saved some vision for the rest of the family, by being the catalyst for this diagnosis in everybody, prompting us to be inquisitive and learn more. His coming has truly been a blessing in disguise.