Friday, December 31, 2010
The Presidents' Daughters
Wednesday, December 22, 2010
I have a Veggie Tales plastic nativity set on my counter that my twins play with every day. Sometimes they leave the figures in very interesting positions.
I know other families who do this and you end up with little people and pretty ponies visiting baby Jesus…how sweet…
Things are a little different at my house. I found baby Jesus propped on the roof of the stable, three wise men lying down in front and everyone else behind the stable, except for one green army guy with a gun lying near the wise men. “Hey, Mark, what happened?”
I knew I was in for a story because he sat down, grabbed a wise man and started, “Well, mom…”
“You know how Herod wants Jesus dead? Well, he tricked these wise man into becoming his minions!
They came here to kill Jesus, but this army guy shot at them. Pshoo! Pshoo!
And he killed one, but the other two wise men got him…arr!!….
So everyone ran for cover screaming ahhhh!!
Except Joseph because he just had a dream about the whole thing and was already behind the stable. He just didn’t have time to warn them or maybe he forgot. Oops!
But Baby Jesus can’t run. And, because he’s really God, of course, and He’s got power. Woosh!! So his manger flew up into the air, waashoom!!
and he killed the other two wise guys with red-hot laser beams!” uh,,uh,,, and they fell down. Amen!, I mean, the end.”
This left me wondering what will happen tomorrow?
Wednesday, December 8, 2010
The Westphal Christmas Letter 2010 by Marcus and Lucas
Mom’s been kinda busy this year so we decided to write the Christmas letter. We turned 5 in February and Mom and Dad abandoned us for three days to fly to Dallas, Texas. Apparently, it was a research trip to the Retina Foundation of the Southwest and the docs got to look at mom’s eyes, but we think it was a vacation. They even sent postcards and went to the Sixth Floor, a museum all about JFK’s assassination. The museum is actually located on the same floor of the bldg. where Oswald fired his rifle. They also saw Phantom of the Opera. We are sure it was a vacation.
We also finished soccer in the spring. It was really fun to knock the other kids down so mom signed us up for swimming lessons instead this fall. We love to splash and move. Tim and Sarah did soccer this fall so we still got to go to their games and run around like maniacs. I think Mom and Dad only lost us twice. If you cry loud and pray to God, someone will help you find your parents. You can also make all the ladies smile if you tap their arm and say, “you’re so beautiful”.
Mom also got to be a pirate at night. She had to wear eye patches because she had cataract surgery. She said it was actually good news because her eye disease isn’t encroaching on her central vision yet, which is what they thought was happening at first. The good news is that now she can drive again. It was tough when we were stuck at home, but we did have some good people help us out with rides. One car even had a TV in it. We prayed for rides and got them. God’s so good to us.
Dad has been busy with his new job, and we “pray for sales” every day. It’s called ICR, and they repair electrical components and robots in all kinds of industries. So if you need a repair, call our Dad. Dad’s also doing school at Cornerstone College for something called a BA in Business Management, and he only has a few classes left. He was able to take a break for his new job and it’s been fun to wrestle him more. If we take turns, he doesn’t get a break, and we never get tired of playing with Dad.
Mom is our teacher. She does school every day with all four of us, but the good news is, that if the weather is nice, we get to take a “field trip.” We like phonics and numbers, and Tim likes science, and Sarah likes typing. We can’t read very much yet, but when we ask Sarah to read to us, she says yes. If we ask Tim, he says, “Ask Sarah.” Tim is nine, and Sarah is seven, and Mom didn’t really want her and Dad’s ages in the letter. Thanks for praying for our family as we keep following God’s path.
Friday, November 19, 2010
Monsters
Do you remember those commercials with the little orange fuzzy guy trying to get someone's attention while the narrator talked about fighting hunger?
Thursday, November 11, 2010
Things overheard from the kids:
“I really want a canyon. That way, I can really blow things up.”
“Luc! You already chopped that leg off!”
“Now pretend I’m really short.”
“Mom, what’s a blaz, er?” “A what?” Daughter points to the sign in women’s clothing—“BLAZERS”.
“I killed you, and now, you’re alive again.”
“Oh no, I’ve found the dead monkey!”
“Owie, owie, owie…I banged my toe….REALLY HARDLY!”
“I always check out the bathroom trash when I go potty.”
“You have to wake daddies up gently.” Little girl
“Nahh, I wake up my daddy aggressively!” my son.
Thursday, October 14, 2010
Monday, October 4, 2010
"Cultivating a thankful heart in your child." Sounds like a great book title, doesn't it? I am not writing it, I am looking for it. One of my children seems to be having some trouble being grateful for anything in his life these days, and this cultivation is my new goal... Granted, cultivation takes time, and the seeds don't grow right away....
Thursday, September 23, 2010
A Typical Day in the Westphal Household
(Did that say "a typical" or "atypical" ? No matter...)
Friday, August 20, 2010
Saturday, August 7, 2010
Vacation (Have to get away) Bible School
My parents live on at least half an acre of wooded land near a lake in northern Michigan. It makes for a nice vacation for the kids and Mom. We can swim in the lake, ride in the paddleboat, build forts in the woods, and have a great time. In fact, we go every year for one very special week. It is special because my parents’ small country church hosts a huge Vacation Bible School event. Monday through Friday, 7-9 pm with music, games, craft, Bible story, true evangelism, and snack for preschoolers to pre-teens. The kindergarten-1st. grade class my boys were part of boasted 15-20 kids each night.
While I still envy those parents who simply drop off and pick up their children, I am glad I stayed each night. I hung out with the boys’ class and helped them line up, take turns, and deal with disappointment. Story time was the best. The pastor dressed up and had props. One night he was a drill sergeant hollering orders and told us the story of Naaman. One night he was a fisherman who followed Jesus. One night he was a doctor named Luke. It was so good to see those little hands raised when he asked if they wanted to give their hearts to Jesus.
My boys, five years olds each, and full of energy, imagination, and their own ideas, were two of almost 20 in their class. They pulled the horn of a snow plow, watched the lights of a firetruck, and washed hands with a real nurse. The boys made crosses with real construction workers, and quickly turned them upside down so they could be swords. They ran when they were supposed to be still, talked when shushed, pushed others when told to line up, flatly refused to color anything, cried when they didn’t get picked to do an activity and choose not to obey their mom….experienced time outs, discipline from Mom, and talkings to, but not all the time…they also listened to story time, learned how to be a HERO for God, learned Bible verses, asked questions, sat in a circle, played duck, duck, goose, ate snack, listened to others, and one night, both raised their hands to give their hearts to Jesus.
So while part of me still envies those drop off and pick up parents, part of me is eternally grateful for the opportunity to watch my boys grow and learn, especially about my savior, Jesus. Thank you, Dighton Wesleyan Church, for evangelizing children.
Tuesday, July 20, 2010
Visual Sleep AId
I am used to clearing an empty glass from the bedroom each morning. My husband almost always has a large glass of milk before bed. But recently, I found a full glass of milk next to the bed. Ryan is a very hard worker, and I think it caught up with him. He just fell asleep before he could drink it.
Friday, July 16, 2010
I Love Surprises
It finally happened. The realization that my husband and I actually read ONE of the SAME books in our childhood! This wonderment came as we were driving to pick up kids from camping. Books on tape are a wonderful thing on a road trip. We have heard everything from Adventures in Odyssey to The Tale of Despereaux. Today's choice was Beverly Cleary's The Mouse and the Motorcycle.
Tuesday, June 22, 2010
summer 2010
What’s the deal with the Bible stories I never heard about?
I mean, I grew up in church, I memorized John 3:16, I can recite the Old Testament, and yet, this remarkable book of God’s keeps surprising me.
Perhaps it’s the fact that I am not a scholar or these stories aren’t in ALL four gospels or perhaps they contain something controversial that my simple mind is missing.
Still, it’s rather surprising, entertaining and enlightening. Take for example, today in John chapter 12. I read of Jesus raising Lazarus from the dead, Jesus coming back to his house for the Passover, and riding the colt into Jerusalem. Lots of visual pictures from my memories, Sunday school posters, plays, palm leaves, real and construction paper….it’s the next passage that got me.
Greeks wanted to see Jesus, they talked to Philip, Philip talked to Andrew, Andrew talked to Jesus and then….there’s these wonderful words of Christ predicting his own death and a voice answering him from heaven. Jesus says, “Father, glorify your name.” God says, “I have both glorified it, and will glorify it again.” Wow! How could I not even know of this exchange?
Is it just not as incredulous as “This is my son, in whom I am well pleased.”? Harder to explain than Noah’s Ark? Perhaps I just didn’t read the Bible enough as a kid and relied on my Sunday School teachers too much.
Thanks, God, for always surprising me. For more details, see John 12.
Tuesday, June 15, 2010
"Well-liked by All"
Thursday, May 13, 2010
Increase our faith
1. "Marc, you left your dominoes out."
"I forgive you."
Monday, May 10, 2010
Mother's Day 2010
My own mother, bless her heart, was stranded at my home with a horrible cold that swelled up her eyes so much she couldn't drive home, so she and Dad stayed an extra night, and I got to help take care of her.
Wednesday, May 5, 2010
We now have a scale in the bathroom. I have always been opposed to this practice. Remember those commercials with the people chained to their bathroom scales? I have never wanted to be a slave to the scale or obsess over those daily changes in my weight. Hence, I have NEVER had a bathroom scale. Why now?
It’s actually Ryan’s scale. He used to keep it at work, but now that his office is home, the scale lives in the bathroom. I hid it in the closet for awhile, but it kept mysteriously reappearing. I have flatly refused to step on it. Instead of my becoming a slave to the scale (whew!), something else has happened. Something I didn’t, but should’ve, anticipated.
I now get daily reports, sometimes twice a day, on how much my eight year old weighs, how much a game weighs, how much the trash can weighs, how much…you get the idea…
So, we now have a scale in the bathroom.
Friday, April 16, 2010
Soccer begins
Timothy's soccer schedule:
Thursday, April 15, 2010
Elephant Guns
People who seek spiritual contentment do not want to be challenged or moved. What I love about my God is that He does not let me stay as I am; he wants me to grow, and like a parent, He rejoices in my growth, and nothing is better than that Hallelujah!
Tuesday, April 13, 2010
The Continuing Saga of the Pine Cones
We have the largest pine tree in our neighborhood. It’s at least 3 feet in diameter and twice as tall as our house. It makes a huge noise when the wind whips through its branches, and the pine cones rain down everywhere. It’s the pine cones that are the problem.
Mom –“Don’t touch the pine cones. There’s sticky sap on them.” This was not fair of Mama…all those tempting pine cones..Hand sanitizer is the best defense against sap. Just rub a big glob of it on the sap-encrusted foot or hand and voila, sap free…
Mom – “Don’t throw the pine cones at the deer.” There’s a nursing home behind our home and their deer pen is behind our shed.
Mom – “Don’t throw pine cones at the house.”
Mom – “Don’t throw pine cones at the cars.”
Mom –“Don’t throw pine cones at each other.”
Kids –“What can we throw the pine cones at?”
Kids—“Ahhhh! Sap! I need tizer, please!”
Kids—“See how far I can throw this pine cone, isn’t it AMAZING?”
Kids—“Mom, watch me twist this pine cone in half!”
Kids—“Look, I can draw with chalk on the pine cone.”
Kids—“Sorry Mom, I didn’t mean to hit you. You moved at the wrong time.”
Mom—“Let’s pick up ALL the pine cones and throw them away.”
Kids—“bummer.”
Sunday, April 11, 2010
Thankfully, God designed men and women differently. We all know this and live with it every day as husband and wife or in watching our parents as we grow up. Still, for some strange reason, we expect our kids to learn the same way. We seem surprised at their differences sometimes. This subject extends far beyond gender, as each individual is a unique creation of God, but I am always being surprised by my children. So here’s a reminder that boys are different than girls.
Boys SEE Differently
“Males have more rods in their eyes versus cones. Rods help us to see distance and speed. Females have more cones than rods. Cones help us to see color and shape. Because of this difference, boys tend to draw verbs with little color variation in their pictures while girls tend to draw nouns with lots of different colors.
When asked to draw a picture, Sally will draw a house with people and flowers and lots of pretty colors. Steve will draw a tornado which is knocking down a house - and his picture will look like a large black swirl.” August 13, 2008 -- Homeschool-Your-Boys.com - Boys Learn Differently Than Girls From Homeschool-Your-Boys Homeschool-Your-Boys.com - FREE Advice on How to Homeschool ...
Allow me to illustrate in these two great pictures from my 8 year old son and 6 year old daughter. See if you can pick who drew what. J
Friday, April 2, 2010
Another beautiful Michigan summer day in Spring. Unseasonably warm at 77 degrees and sunny. My happy yellow daffodils are trumpeting spring's arrival with all their hearts in the flowerbeds. Today we abandoned our books for the park.
The wind blew last year's leaves skittering, paper thin and brown, along paths and over ground just beginning to green. The four children climbed an inviting tree, rejoicing over footholds and handholds and what they could see from the heights. Even Mama looked tiny. Finally, in their red-cheeked exhaustion, they came to stand under the tree. But it had no leaves to shade them. Even its buds were still closed up tight. We walked through the woods and along the creek bed, searching out signs of spring other than the sun warming our heads. We spied racing water spiders and the green glow of algae on still creek waters. Three friendly dogs greeted us while their owner clung to their leashes.
We headed back to 9our waiting water bottles and the drive home with windows down and radio cranked to the sound of an audio story. This time --Mrs. Frisbee and the Rats of Nimh. It was a beautiful day.
"Whatever you do, work at it with your whole heart, as though working for God and not man." Colossians 3:23
Work. Work. Work. Some days it's downright tough to do the dishes one more time or do yet another load of laundry or spend time making one more meal. I call it the deep-sigh day. It means I need an attitude adjustment from my spiritual chiropractor. All these deep sighs and unspoken complaints are simply symptoms of me-ism. Yep, focusing on myself again instear of my creator and sustainer. Me-ism = selfishness = sin= repentance = J.O.Y. = Jesus Others You.
That's right, "you" is last. "But what about ME? It's isn't fair. I've had enough; now I want my share. Can't you see?. . . " Another selfish 80s song floats through my brain looking for a home, but I know another, "It's no about me, Jesus, that you should do things my way...it's all about you."
Isn't true worship the humbling of ourselfves? Admitting we were wrong and walking in grace, grace--like wrapping up in a dryer-warmed blanket after a cold walk outdoors.
In Luke, the Roman centurian discovered something. He viewed Christ's horrible death from beginning until the end. Darkness covered the land, the veil was rent in two, and we all know his statement, "Truly this was the son of God!" yet we miss the verse before it. Our pastor pointed to this little remembered phrase in the book of Luke and it seemed to lodge in my heart. The scripture says, "He PRAISED God." It's hard to imagine PRAISE at such a time, but there it is. He PRAISED God.--That one little verb I need for my attitude adjustment.
My life needs to be that praise, tha tacknowledgement, that daily love song, even my dishes, my laundry, and my dinners.
Love Song
Today my powm will be written
Not in words
But in the work I do
To make this house
Shining and welcoming.
Today my poem will be written
Not with pen
But with my mop and broom
With my iron and parin gknife
A love song, yes--
One written with my life.
By Elizabeth B. Rooney
Friday, March 26, 2010
A funny thing happened at the hotel
A great time was had by all. With seven kids under the age of 9, the pool was a huge attraction. It had a kiddie pool right in it for the little ones and a seperate hot tub for the big ones. :-) Ahh!! (Ryan even got to show off his painted big toe nail that he let Sarah paint bright pink because no one would ever see it.) We swam and ordered pizza and swam some more (yes, we waited 20 minutes-my cousin has lifeguard training). Then we slept all night and swam again in the morning. We said our goodbyes and the kids and I cleaned up, packed up, and headed to the van (Ry was already at work).
As I shut the back door on our luggage, I noticed some scratches accross the back. I followed them. They were all the way around the van, at least twice! Someone had viciously "keyed" our van. I calmly let the hotel know, and my husband, and we called the police to file a report. It was now lunch time, so I pulled out the emergency box of graham crackers for the kids while we waited for the officer. "Do you have any enemies, Ma'am? Perhaps an angry ex?" Perhaps the vandals had you confused with someone else.
We left for home but had to stop to get gas first. When I opened the gas door, a small pile of dead leaves fell out. Something a child would do - no doubt. SOmething clicks in my brain. I ger a flash of everything my boys have destroyed--wallpaper, furniture, toys, walls, closet shelving, towel bars--I bend closer to look at the scratches near the gas door. Yes...there and there again...that looks like the letter "m". Only one boy makes "m"s like that.
I calmly bring Marc to that side of the van. I smile. I control my voixe. "Is that your "m"?" I ask.
He smiles, swelling with pride. After all, mama loves his "m"s. "Yes," he says expectantly turning his eyes toward me.
"And what did you use to make your m?"
"Oh, a sharp shiny rock."
"Did you go all around the van?"
"Yes, " he's still smiling a little but he's wavering. Perhaps my fake pleasant tone is fading, "I wanted to make it beautiful for you, Mama."
I hugged him. I was glad his heart was in the fight place, but we need to work on modes of expression here. So now you know, the rest of the story.
:
Sunday, March 21, 2010
Weekly (sort of) Wrap Up
- Science week this week. Tim and Sarah worked on reports on Body Systems. Tim is tackling the Skeletal System and Sarah is doing the muscles (She was quite delighted to learn the heart is a muscle, as her last report was on the heart.) Tim loves games and we found one about bones. ABCya! Skeletal System Game: Learn the Bones! We use the Power Point program and then have the kids present and grade one another on presentation, including points for eye contact, smiling, and knowing their slides well. Mama grades them as well. This week, they also did some of their own typing! (Thanks, Heather, for BBC - Schools - Dance Mat Typing - Home
The weather won on Wednesday. It was so beautiful, sunny and warm for Michigan in March. The boys had story time at the library while Tim and Sarah worked on their writing and daily grams, but that was it for school today. Sarah had dance at 1:30, and while I usually stay at the studio and do school with the boys, we left and went to the park. We played pirates at the playground, threw rocks in the water, watched a swimming duck leaving trails on the pond, and tried to catch a couple seagulls. Tim even dug holes in the sand. After dance, we ran errands, and headed to another park for one more hour of the glorious feeling of sunshine warming up your clothes and hair, and made it home just in time to leave for church.
- We are also entering Reading Rainbow’s Young Author’s contest this year (k-3rd. grade). Reading Rainbow Young Writers & Illustrators Contest Sarah created a great story called “bunnies and raspberries” contrasted to Marc’s “The Scary Pirates”, which is also great in its own right.
- I love it when God winks at me. One of my goals this year is for Tim and Sarah to learn their books of the Bible and that’s also the current focus of Children’s Church. We read the list of books every day and found a song on youtube. YouTube - Bible Books
We listened to Stuart Little by E.B. White (author of Charlotte’s Web) and enjoyed it but found ourselves disappointed in the ending, which didn’t feel like much of an ending at all as Stuart had not yet found his bird friend, Margalow.
Tim is also reading Tom Swift and the Aerial Warship (our 2nd. Tom Swift book) and Sarah has started Rebecca of Sunnybrook Farm.
The week ended with Sarah and I attending the ballet, “The Squire and the Scroll”. It’s based on the book of the same name by Jennie Bishop. Wonderful book about the importance of living by God’s word. Check it out at your library. It has knights, a dragon, a princess, and a quest. Wonderful to see live performance that gives God the Glory.
Thursday, March 18, 2010
By Elizabeth K. Rooney
If I could wrap
a field of butterflies
I'd package them
for you.
I'd put in all the colors
and a lot of sunshine, too.
I'd gather up a summer breeze
and all the fluttering
and wrap them with brown paper
and tie them up with string.
I've only words for wrapping
and only rhymes for string.
With these, beloved of my heart,
I give you everything.
Saturday, March 13, 2010
Gentle leading
“It’s great that you know the word, ‘barricade’”, managed Crazy Mom as she chalked up another chapter in her endeavor to always find the positive. On Thursday, the little girl cried during Science and the boy flatly refused to do his daily gram and sat on his bed for a LONG time.
Monday, March 8, 2010
Saturday, March 6, 2010
Texas Highlights
1. The bad news is that I am a “severely affected” female carrier of XLRP. The part of my eyes where my vision loss is most severe are the best parts for driving. Genetic counselor seemed happy I had given up night driving and also strongly suggested that I only drive in ideal conditions in the middle of the day. (Only 7 short years and the Tim-taxi can drive). It was also very cool to see a 3d image of my laser-scanned retina on the computer. Technology is so amazing. The good news is that my three cousins are “mild, no symptoms, and no symptoms.” Retina Foundation of the Southwest - Dallas, Texas
2. More good news is that on the research front, great strides are being made, and the future possibility of gene replacement therapy as treatment is very exciting. The gene that carries XLRP has also been isolated and that holds the promise of genetic testing for families sometime in the future.
Other highlights:
1. Who shot JFK? Lone gunman or conspiracy? We went to “The 6th. floor”. It’s the 6th floor of the book depository building where Oswald shot the president. You can look down onto Dealey Plaza and parking is behind “the grassy knoll”. The 6th. floor is now a museum with a really cool audio tour. For you history buffs visiting Dallas, this one’s for you.
2. The Phantom of the Opera. Yes, I’ve finally seen “the Phantom”. Fabulous music. I love live theater.
Thursday, March 4, 2010
Things Kids Say
"Mom! You are making the kitchen smell all dinnery! yum...."
"Why does my body tell me to stop running and get all tired when I just want to go?"
"Why do we need night time?"
"I wish we had battlestations, Mom!"
"Why don't we have any armor?"
During Bible, we are learning Luke 1:37 "Nothing is impossible with God" and Mark wants to know, "why don't we have a Mark verse? There's a Mark in the Bible you know!"
Lucism:
While waiting for dance class, there is a room full of moms just for Lucas to entertain. He overheard one mom mention her husband, Tim. He went right over and joined the conversation, putting his hand on her shoulder, "That's my brother's name."
"That's my husband's name," she replied. Lucas immediately turned to the other mom in the conversation and asked, "What's your husband's name?" "My husband's name is Mark, " she answered. "That's my other brother's name!" he exclaimed, smiling widely. He immediately turned to the woman seated in the next chair. "What's your husband's name?" he inquired. "His name is Vince," she answered. "Oh," he intoned, obviously disappointed. Not to be deterred, he moved to the next woman and repeated his question. As he made his way around the crowded room, the original two ladies turned to me, "what is his name?" "It's Lucas," I answered. "I don't think he's going to find one," she said.
"Could you please die over there? This is MY space."
"Mom, why don't you call me "your majesty" today?"
Here's a couple Sarahisms too.
"Do not argue or complain about anything. PHILISTINES 2:14"
"Look, Mom, I can melt snow with my bare hands!"
Do you find yourself joining in with the fantasies sometimes?
Momisms:
"Even the King has to wash his hands."
"All knights will remain seated while eating lunch."
"Your mission, should you choose to accept it, will be to retrieve the mail today."
"If this room is not clean, a disaster beyond your imagination will occur!" (Oops, I saw the Phantom in Dallas--guess that was my own fantasy imposing upon reality).
Wednesday, February 24, 2010
God is winking at me again
I laughed, “The world is a classroom.”
“That’s great,” she replied, “keep at it. Some parents don’t teach their kids anything,” and off she went.
It was just a moment, a little wisp of encouragement, and it made me walk a little taller as we headed indoors. Thank you, Lord, for your encouragement.
Saturday, February 20, 2010
I didn’t get as much school accomplished as I intended. (I seem to write this every week—perhaps I need to review my goals so I am actually achieving them.) Coughing kids this week didn’t help things either.
Highlight: Tim and Sarah read chapter books all by themselves! We have some Magic Tree House books, which despite the “magic” have great history lessons and accompanying study guides. Random House Kids Magic Tree House
(Our local bookstore also did a class for homeschoolers based on this series a couple years ago..I keep hoping they’ll bring it back). Anyway, we have about four of these books on the bookshelves, and the kids came down and told me they each were reading one. That’s great, kids. I tucked them into bed so I could watch the Olympics. At 10:00, Tim came downstairs with his book. “Mom, guess what! I finished the WHOLE book!” He was so excited and I was so surprised and proud, that I had to pretend it was only 8:30. We’ve also listened to these on audio from the library.
as our base curriculum and add to it as necessary. It’s nice to have a baseline. They use McGuffey readers http://www.mcguffeyreaders.com/important.htm
and next week, Sarah will finish the 2nd. grade reader and Tim will finish the 3rd. grade reader. I am very proud of them both. I love to hear them read their lessons out loud. Some days I am just amazed that they can read. I now have Tim asking if he can read over my shoulder. “Mom, can I please read what you are typing?”
For Science (Tuesdays and Thursdays), we put our reports into PowerPoint. That way, they can present to Dad.
For my kindergartners, we are tracing letters 3 days a week, doing phonics, and lots of counting. I also need to incorporate daily games for these guys too. I find it best to do school with them in the early morning, starting with a book and related lesson of some sort. I really enjoyed Before Five in a RowFive in A Row Literature-Based Studies with Tim and Sarah and I am just finishing it with Marc and Luc. I love anything that’s literature based.
Friday morning is “Library Day” each week, and all four of my kids fill up all four of the kids computers at the library.
The purpose of this project is to detect genetic factors that influence the clinical consequences of mutations in genes causing X-linked retinitis pigmentosa (RP). Mutations in X-linked RP genes can lead to strikingly different clinical outcomes, even among individuals with the same mutation in the same family. While clinical variation is often seen among males, the most striking differences are seen among females. Phenotypes in “carrier” females can range from no detectible symptoms, to early onset, rapid progression of disease. Finding the factors that account for these differences will improve our understanding of the disease process. Of more importance, though, identifying these factors may suggest ways to slow or arrest the loss of vision in affected individuals. That is, if we can understand what makes individuals with X-linked mutations either asymptomatic or mildly affected, we may be able to use this information to treat patients with more severe symptoms.
The major focus of this project is the X-linked RP gene RPGR (retinitis pigmentosa GTPase regulator) and the clinical consequence of interest is the disease severity in female carriers of RPGR mutations. Mutations in RPGR account for 80% of cases of X-linked RP and affect more than 10,000 Americans. Further, studies suggest that approximately 30% of isolated RP is caused by mutations in RPGR, making mutations in RPGR the most common cause of RP. Males with a mutation in an X-linked gene such as RPGR are almost always affected, because males have only one X-chromosome, but female carriers of an X-linked mutation are often unaffected since females have two X-chromosomes and the second chromosome may provide protection from the mutation. However, many females who carry an RPGR mutation have clinical symptoms of RP, ranging from undetectable or very mild in some cases to severe loss of vision in others.
The project has several key aims. The first aim is to identify, enroll and characterize the families and patients with RPGR mutations. This will be done by Drs. David Birch, Dennis Hoffman, and Dianna Wheaton at the Retina Foundation of the Southwest in Dallas. The goal is to enroll as many individuals with mutations in each family as possible, to evaluate each individual with a panel of tests including advanced retinal imaging, to determine the range of clinical expression associated with each RPGR mutation, and to collect blood and DNA samples for genetic testing. Thus, one outcome of the project will be a comprehensive genotype-phenotype characterization of RPGR mutations.
The second aim is to test the hypothesis that genetic modifying factors may play a role in altering disease severity. We plan to approach this in two ways. First, we plan to investigate the role of two genes that interact with RPGR to determine if slight genetic variations within these genes can affect the behavior of RPGR. Secondly, we plan to test a large class of random genetic variants by microarry analysis in a genome-wide association study.
Finally, the third aim is to extend these studies to clinical variation in males with RPGR mutations.
The detection of factors modifying inherited diseases is a relatively new field of investigation. Completion of the project should substantially expand our understanding of the clinical consequences of mutations causing X-linked RP, and may identify new targets for treatment and prevention of retinal disease.
Tuesday, February 16, 2010
Things Kids Say
“When I grow up, I’m going to marry you, Mom.” –Marc
“Little Bo Beep SAT on her sheep,” says Sarah, giggling.
“And she always knew where to find them,” added Tim.
“Can I have another five birthday?” Lucas inquires.
“No, but you can have a six birthday next year,” answers Mama.
“Can I have another five birthday tomorrow?”
“Jamey, do you want to go outside with me? Deal or No Deal?” asks Marc.
“No Deal,” answers Jamey. (Jamey is our little friend who visits a couple days a week).
Sarah is finding all the states on her map that begin with N and saying them out loud,
“North Dakota, New Mexico….Nevada….New Hamster.”
Postcards from the Past
Thursday, February 11, 2010
Five Things (that I love to wear in winter) and a shrug
What’s my shrug? Oh, my shrug is wonderful. My shrug is what I wear around the house when I’m cold (basically, all winter). My shrug is burgundy, white and black. It’s plaid, and flannel, and a little ripped. It’s quilted and it’s missing at least two buttons. It’s actually a shirt of Ryan’s (the man who is rarely cold and has no practical use for a quilted flannel shirt). So it’s mine. It’s been mine for about 14 years now. I LOVE my shrug. Some mornings I heat it up in the dryer before wearing it. The shrug is not allowed out in public, for obvious reasons, but at home, I wear the shrug, proudly, happily, and cozy warm, ahhh….