The Best Birthday Ever!

I got pretty mad at my husband Rickie (he’s decided to go back to his childhood name) when he sent out a notice to all of our friends five years ago announcing that I was turning 60 and that they should send me a nice message! Not only was I worried about bothering them with worrying about writing something to me, but also I just couldn’t stand thinking that I was that old, not to mention that everyone knew i!
Five years later, I’ve mellowed. I really don’t care if people know that I just had the best 65th birthday imaginable! And besides that I can now get senior rates at all the movies and Medicare!  My simple little statin pill just went from being $400 a month (long story) to $10 a month. You can’t beat that! 
It just happened that I spent my birthday with Saydi and her family and Shawni in Essex County, England, the homeland of all of the ancestors on my mother’s father’s side of the family. You can’t beat that either!  But before we get to all that let me tell you about how I started my fun day:
Saydi is pretty much a gourmet cook and made sure that we had an extraordinary breakfast, even though we had to get everybody ready to step out the door at 9 a.m. for church. 
Their Homeschool Room, turned into a sun-filled celebration room complete with banners, English scones with clotted cream and strawberry jam and ham, cheese, onion, mushroom eggs topped with avocado. Mmmmm!





Sitting in front of my plate were envelopes that contained 65 memories of our time together from the great Rickie and all the kids and grandkids.  Another amazing feast that lasted all day! Every time there was a lull in the conversation, out popped more memories. Everyone, including Hazel took their turns to read. There were fun memories, hilarious memories, poignant memories and right down crazy memories!  SO fun!

In addition, the kids presented me with beautiful birthday cards!



We headed out for church where Saydi made announcements for an upcoming activity and Jeff taught his first gospel doctrine lesson (his new calling). I must admit that I was blown away with Jeff brilliance in not only presenting the material but also his mastery of getting the class involved. This guy is incredibly smart!  We already knew that but it was personified in that terrific lesson!
We thought we’d leave early from church for our Ancestor Adventure, but when we went to get in the car, there were 10 cars blocking us from getting out. It was ward conference and there were tons of stake visitors and the next ward just didn’t have anywhere else to park.  That did not deter Saydi for a second. She went to a man in the other ward who somehow fished out everyone whose cars were behind us,  they were moved lickety split and we were off!
Daniel Clark and Elizabeth Gower Clark were our first “right angle” ancestors who joined the Mormon church and then immigrated to the US to join fellow saints in their pilgrimage to Utah.  He was born in 1815 in Colchester Essex, England and she was born in 1819 in a town not far away called Little Baddow and they were married in Colchester. A town which holds special significance to us because that is exactly where Jonah served the last part of his mission.
The Clarks had 11 children. Three died as infants and so they were left with nine children who were persecuted mercilessly for joining the church and not allowed to attend school. Dirt poor and full of the excitement of their new church and a new life, they headed for Liverpool, sailed to America and joined the Overland Company to make the long trip by foot and by covered wagon to join the Saints in Zion.
Daniel who had been a shoemaker did his best to become a cowhand as he nutured his family across the plains. As a night watchman of the cattle by the Platte River in Nebraska, he drank some polluted water and contracted cholera.  Within three weeks he was dead. So sad!  Elizabeth valiantly carried on with the children and settled in Utah. She is buried in Morgan, near where Saren now lives.
Back to England!  Daniel’s father was named William, as was his father and his father. All were born and married in Colchester and many were buried there. The old church where Daniel and his father were married has been demolished but some walls of the really old church where his grandparents and great grandparents were married and/or buried was built by an ancient Roman Wall still stands. We had a fabulous time exploring the haunts of our dearly departed ancestors!
Below is the old Roman Wall and the church nearby where those ancestors feet walked all those years ago:


Here are Hazel and Charlie, standing in at the church door for William Clark and Hannah Hills who were married here.

See the old walls that are part of the original at another door seen below.  Those ancestors would love these girls!

Here’s the back of the church which has been added on in recent times.

Another Grandfather was buried at a place called Lion Walk. There were two churches very near there so we’re not sure which churchyard he was buried at, but we know that we’re close! (note the daisy crown on my head that Hazel made for my birthday). 










This is a newly rebuilt church in the center of town.

From there it was back to Grandma Elizabeth’s birthplace where she was raised with 9 brothers and sisters. As serendipity would have it Saydi’s best friend from high school (and life in general) lives with her husband Rob and their three adorable girls who have lived in England for five years now happen to live about 8 minutes from Little Baddow so they met us there for a walk through a wonderful 99 acre wood filled with bluebells. Those bluebells only bloom about three weeks a year and we happen to hit just right! They have probably been blooming ever since the Gower family lived there. 
What a gorgeous birthday present!



We had a glorious time wandering through the woods!








What a memorable hour!  After that fun little trek, we went to the two churches in Little Baddow where surely our Grandma’s parents and brothers and sister were married, harried and  buried.



We ended the day with a hilarious Google + talk with some of the fam on the computer. Some were in church, some we couldn’t hear. Some we couldn’t see and some were in church (stake conference) and skyped anyway!  Funny! 
Jeff, while we were traipsing around the countryside was home taking care of Peter and Emmeline and made me a gorgeous flourless chocolate birthday cake. My favorite!  The guys is amazing!  I am a fan forever!

Thanks to the efforts of so many, it’s a birthday I’ll NEVER forget!  

The Good News and Bad News with Bardet-Biedl Syndrome

The real reason for our visit to London was to attend the Bardet-Biedl Syndrome Conference which is held once a year in Northampton, England.  The President of the organization is  Professor Phil Beales who accidently discovered the syndrome in a patient that no one knew how to help. He is a researcher extraordinaire and has since found an amazing group of parents who have been helping collect people from all over the British Isles with this rare syndrome. In 2010 he was the force behind opening clinics in four location in the UK where families effected with BBS, named after the scientist who first discovered the syndrome.

Those who have BBS can go once a year to see physicians and researchers who know exactly what they can do to help. Since it is so rare it’s hard to find a regular doctor anywhere who knows anything about BBS so this is an enormous advantage for those who live in the British Isles.

Professor Beales has really dedicated his life to discovering what is needed to help people affected with BBS and other rare diseases and the genes that cause them. After many years, they found 10 BBS genes and within the last few years they have boosted that number to 17.  Here he is with his darling wife Helen. They have two girls 15 and 19 and live in Greenwich although he travels the world with his work.

 

He had treated almost everyone at the conference at some point in his career and those effected with BBS and their parents and families come from all over the British Isles to hear his updates and feel his concern for their issues. They all think of him as their second father or brother and the festive atmosphere with a hotel full of these people who meet once a year felt like a family reunion.

Here is the darling lady, who has a 30 year old son with BBS who organizes the event which included about 300 people this year (including social workers and scientists). He has found about 200 people who have been effected with BBS in Great Britain, although we’re sure that there are many undiagnosed cases waiting to be found. What a sweetheart she is!

Phil Beales and several other world-renowned specialists in BBS were there to speak to us, review new findings and give us the bad news along with the good. The most common side effects of the syndrome is loss of vision at a wide variety of ages. The photoreceptors in the eye don’t receive enough of the crucial proteins it needs to survive so the light receptors begin to die. The bad news is that retinitis pigmentosa, which is the condition that many people have that causes similar blindness is not exactly the same as those with BBS. Even though they are doing a myriad of test groups on various methods to save vision, vision loss manifests itself a little differently in BBS kids so it requires a little different care to make it work. Figuring all that out is that is the rub!

It was hard to see very large people 18-40 there walking around with white canes and guide dogs…..until you hear them speak.  There was a man there with a guide dog and a master’s degree in music. He plays in a band nightly and says that he wouldn’t change his sight status for anything. He’s learned more from being blind that he ever could have with his sight.

The last speaker was a terrific young woman from Australia who has BBS, is a registered nurse and who lost 11 stones (over150 pounds) through sheer will power the past couple of years. She still has her vision at 34 and that is GREAT news!

The other good news is that those with BBS can lose weigh with lots of knowledge of the way their own bodies work and a huge amount of will power. The bad news is that the pituitary gland which controls appetite usually sends out a substance called leptin. Here’s the definition from Wikepeidia: Leptin (Greek λεπτός (leptos) meaning thin) is a 16 kDa protein hormone that plays a key role in regulating energy intake and energy expenditure, including appetite and metabolism. It’s a hormone that BBS kids have but it’s so weak that it can’t tell the body that it’s full. Therefore, these kids think they are hungry, even when they really don’t need more food. They just mostly always feel hungry.Thus the weight gain.So unfair! 

The good news is that they are working on a way to create a way to send stronger doses of leptin messages to the gland; but the bad news is that it will be a while before they expect they can figure that out!

We learned more than we can possibly put in one blog post and probably more that you want to know but here are a few pictures of the wonderful parents and kids who are dealing with this syndrome which manifests itself differently in every single case. Many have heart and kidney problems and other don’t. Some have learning difficulties but many have extraordinary gifts that others don’t have. They are finding that many BBS kids have remarkable memories. Some lose their vision early and others’ eyesight lasts into their 40’s.

Since our granddaughter Lucy is already showing signs of night-blindness at age 5, we are thinking this is going to be an issue with her. Weight is also a problem which she will have to fight all of her life. As the geneticists showed us the delicate balance of proteins and cilia which is still greatly unknown, we felt both encouraged at their discoveries but more and more anxious about what they can do with the results they find in order to make things better for families who deal with BBS. 

The room at the Hilton Hotel in Northampton was filled with people who are trying to work out how to help their family and patients with BBS.

This dad below has three boys and then a darling little two year old daughter just diagnosed with BBS. He is from Austria but living in Zurich  He was jet lagged along with us and just happened to sit right by us in the audience of 300 when he arrived. Serendipity!  He had just returned from Dallas, TX the night before, flown home to Zurich and had gotten up on Saturday morning to fly to England to join us.  What a great guy he was!  One of the real perks of this “job” of keeping up with BBS is meeting other parents who have the same issues to deal with!

These parents have two BBS daughters with entirely different symptoms even though the same gene is affected. They are incredible people who kind of “run the show” at the conferences. They do everything from child care (many of the kids come and are taken care of by saintly care-givers to fun places while the parents learn about their syndrome), to this dad being the treasurer of the organization!

There were so many families dealing with similar issues, only different! This cute boy is blind and has the cutest smile ever!

This young man has managed his weight so well. We keep wondering what makes the difference in size. Body type, genetics, the amount of leptin that ‘s getting through and food portions are all factors. One cute mom from Ireland who has five children said she has, “one wee boy of eight with BBS and actually slender and a wee girl of six with BBS who is not so “wee”.  She says they eat exactly the same things. Such a mystery!

Here is a dad with his 10 year old whom he obviously adores!

This is a darling little three year old with BBS. Her mother died 20 months ago of epilepsy (which she also deals with) and her dad is nowhere to be found. She is being cared for by a darling grandmother in a wheelchair and a caring uncle.

This adoring mother has done so much to help her cute son deal with the syndrome.

And here are a couple of teenagers who have been able to control their weight very well. Darling girls with their support team!

This couple rely on each other for help and support!

So the good news is that we have so many new friends who are all dealing with similar issues although they have a wide degree of variation. The other great news is that we have people like Phil Beales and many doctors and researchers who are gathering information to help. The bad news is that research takes such long time to test before it can become a real help.

The best news of all is that we were told that possible solutions to the numberless problems that these BBS kids face are being worked on and possibilities are jumping in leaps and bounds!

After the meetings we spoke to Phil and Helen briefly about how excited we were about the conference and they actually offered us a ride home (to Saydi’s house). Even though I know it was a little out of their way, we jumped at the chance to spend a precious extra 90 minutes with them getting to know them better and asking some unanswered questions from those swimming in our heads from the conference. What luck! Or maybe purely love from a kind Heavenly Father who knows we need help!

One thing we’re sure of:  Lucy is on the good end of the spectrum of kids affected with BBS. She is absolutely adorable with remarkable language skills and incredible art abilities. It seems that even though some genes are effected adversely, others give them extraordinary abilities. (As a group many seem to have wonderful memories). Lucy reads a little book every night before bed and is so excited to go to a mainstream kindergarten next year. Her parents are doing a stellar job of keeping her weight under control although they are aware of every bite that goes into her mouth. She does hold things that she’s working on very close to her eyes and her glasses seem to help her see better. Although she doesn’t wear them much at home she always has them on at school.

Remarkable teachers (she’s been in pre-school for three years with help for her special needs) and care givers (the state has provided speech therapists and physical therapists as soon as she was diagnosed) have brought tears to our eyes as they have helped her to improve her skills tirelessly!  We adore this little girl and are so grateful to all those who are helping her with special needs along the way!

Probably her biggest asset and the very best news of all for her is her amazing family! Supportive parents and siblings have given her a jumpstart on everything from her remarkable abilities to win the memory game to her mastery of  language to jumping on the trampoline, swimming and dancing! Extended family are pouring out love as well. What a lucky little girl! 

Luscious Day in London!

Things went like clockwork! (Well other than dropping the sleeping pillow that Shawni had given me for the trip three times and leaving my roller bag at W.H. Smith for a short time (we were pretty tired). Other than that, we arrived at Gatwick Airport, caught the Gatwick Express to London Victoria (so many memories here) and met our Londoner, Saydi at “Left Luggage” even though she never got my text message to meet us there.

We were giddy to be together and she was delighted to be without her kids. Bless Jeff!  He stayed home from work to take care of the them so we could go like crazy in London without stopping every few minutes for food and the Toilets.

From Victoria we walked straight to Buckingham Palace where we had fun taking pictures:

 

 

Passing British flags all the way down the avenue we saw gorgeous flower gardens that reminded us that it was spring even though it was still pretty cold.

 

A stroll through the adjacent St. James Park was awe inspiring!

 

 

See the London Eye in the background?  That’s where we were headed….

Until we saw this…..

Wonderful Westiminster Abbey was just around the corner!  They wouldn’t let us take pictures inside but none of us had been there in years and with the help of hand held recorders we reveled in what we learned/remembered as we tried to get all that royalty stuff back in our minds. Unbelievably complicated!

We stood on the very ground where kings and queens had been coroneted for thousands of years. These walls have seen myriads of Royal weddings and added to the somber nature of funerals (including Princess Diana’s) down through the ages. Poet’s corner is always a favorite spot. So many incredible people have added richness to our lives through their intellect and passion for music and poetry!

 

 

When we were here 35 years ago this building was all black. A result of a thousand years of dirt and grime from the elements.Today it is glorious to see it in it’s splendor (although the black was pretty awesome too)!

 

Across the street of course were the Houses of Parliament and our old friend Big Ben. Magnificent structures!

And of course our old friend Winston Churchill is still standing there after all these years. Saydi thought she would imitate his expression.  Good job Saydi!

On to the London Eye where we ran into Saydi’s friends from Boston who were spending the night at her house while we were at the conference and were site-seeing in London for the day. Who would have thought that of the millions of people milling around London that day, we would find them! Such a darling family!

 

They had just gotten off of the London Eyre ad we were about to enter. What a amazing sight on earth!

See "The Gherkin” there in the background right below?

And of course St. Paul’s Cathedral…..

And the new building they are scurrying finish by the time the London Olympics arrives in 131 days called “The Shard”:

I saw lots more than I did last time we “made the rounds” including Waterloo station (above) and the Pods in front and in back of us (below) as we slowly rotated around the London.

What an amazing engineering feat that London Eye is!  We walked over the bridge that we had just seen from the heights and right into Trafalgar Square which was blocked off because of a celebration the next day and probably because of the London Marathon too. It was being run the next day by 37,000 people!  London was abuzz! 

We stopped for a lovely little lunch at Prez and then we were on to a quick run through the National Gallery, which is my favorite gallery in all the world. Again, no pictures but we relished seeing the works of geniuses!

As you can see by the clouds, it had been threatening rain, which was in the forecast for the afternoon so we jumped on a bus and drove through Regent Street, the area where Saydi and the fam had stayed for three weeks when they first came to London.

We made a quick stop at Piccadilly Circus.

I must admit that we loved the sights from that second story of the bus, but even better was being able to sit down!  And it did rain!  We got a nice dowsing while shopping for souvenirs for Shawni’s kids and friends but it was amazing that it rained so little while we were out and about!

By then it was time to ride on another double-decker bus through Kennsington, Hyde Park Corner and back to Victoria Station where we picked up our luggage, bid Saydi a fond farewell (until the next night) and headed for Euston station where we caught the train that took us to Northampton for the BBS conference.

It was a luscious day in London!  Collages compliments of Shawni:

 

 

 

 

 

There’s no city that we original Eyrealmers love more than London so this was a day we’ll never forget, thanks to Saydi and her guidance through a day of frolicking and fantastic memories!