Here you will not only find the latest happenings with DDB, but you will also get to read about little snippets of things I find moving, important, random, or informative/helpful to others. I hope that by giving you a little insight into my life that you will have the opportunity to understand me a bit more. Thanks for stopping by!

Wednesday, October 28, 2009

Devastating News....

Well yesterday, my husban'ds family received some heart breaking news. My niece Lena who is 2 1/2 has been suffering from a multiple seizure disorder since she was 8 1/2 months old. Yesterday she recieved a new diagnosis. Genetic testing showed positive for a gene mutation of the SCN1A gene. Which has given her the diagnosis of Dravet Syndrome. We've been hoping and praying for the last couple of years that she would just grow out of this. But it looks like this is a lifetime thing she will have to deal with. Not a whole lot of information can be found on this disease as it is rare. Most of the time the biological parents are not even carriers of the gene. This gene mutates during the embryonic stage of development. There are only about 426 documented known cases of Dravet Syndrome. I'm sure there are other children out there with such a diagnosis, but because of the lack of awareness they aren't known. I plan on doing whatever I can to help my brother in law and sister in law care for their beautiful little girl. I also plan on doing whatever I can to help them raise funds for Lena's foundation and raise awareness about Dravet Syndrome. Here is some information I found online below: More info can also be found on this blog: http://dangerouslyrare.blogspot.com/

How Does this Condition Evolve?

Primarily the start of the condition includes a clonic seizure that is associated with a fever. The seizure may affect one side of the body or both, sometimes changing. Within a few months another seizure occurs very similarly to the first. These seizures are usually status epilepticus, meaning basically they last too long. In some kids myoclonic seizures then follow.The first seizure the child has occurs in the first year of life and seizure control is very difficult to obtain. Unfortunately at about age 1 development slows considerably and doesn't pick up again until 4. Some regression in development can occur. Meaning that children can lose skills during this time. Most or all children will grow up and have at least some form of mental retardation, varying in degrees.These kids can and usually do have a wide variety of seizure types. The seizures are frequent and often reach status epilepticus. After the first two clonic seizures, fever may or may not be present.Although the syndrome name includes "Myoclonic" it does not necessarily mean that the child will have myoclonic seizures although many children do go on to have them.SpeechOn observation it appears that all children with Dravet syndrome appear to have more of a problem with speech then any other developmental skill.

What are Clonic Seizures?

A continuous punching action that occurs without stiffening of the body.What are Myoclonic Seizures?Myoclonic seizures are quick jerks, usually singular and can be mistaken for a startled reflex. The myoclonics may occur in clusters (meaning a lot of jerks in a group) sometimes lasting hours. The person experiencing myoclonic seizures are fully awake and aware of what is happening. Myoclonic seizures can be as subtle as a chin twitch and as vicious as to knock a person to the floor.

What other Seizure Types can Dravet Syndrome Children Experience?

All seizure types, but here are the main ones:Tonic Clonic: Child goes stiff, becoming unconscious, followed with punching of the arms and legs.Tonic: The body goes stiffAtonic: The body goes limpAbsent Seizures: Staring of for a second or so.Complex Partials: Complex and repetitive movement with little or no awareness of what they are doing. Includes: Chewing, strange movements perhaps with just one or more appendages (arm, leg, head etc.), as an example, some may dress and undress.Simple Partials: Same as Complex Partials only the child is fully aware of what they are doing but are unable to control or stop it.Many children will have a combination of seizure types all in the one go.Diagnosing Dravet SyndromeIs based on clinical observations. This includes the child's seizure history and EEG records, many children will have a normal EEG reading early on, that will then start showing specific abnormalities as the syndrome progresses. In some cases a genetic link has been found. A test can be performed (in research phase) to determine if a child has this gene mutation, called SCN1A mutation. This is not however conclusive as some children although diagnosed with SMEI do not have this mutation.

Seizure Triggers Specific to Dravet Syndrome:

Heat can be a trigger for seizure activity in these childrenThe usual triggers for seizures are also sometimes present including:PhotosensitivityBeing tired, or lack of sleepIllness and often feverOver excitementBeing outside of their comfort levelBest treating drugs for Dravet Syndrome suggest:Valproate (Sodium Valproate, Valproic Acid, Epilim) etc.Benzodiazepines (Clonazepam, Lorazepam)Stiripentol - Great expectations here but not easily availableTopiramate (Topamax)Ethosuximide (Zarontin)

Drugs to stay away from:

Lamotragine (Lamictal)Carbamazepine (Atretol, Depitol, Epitol, Tegretol)Some research also suggests Phenytoin (Dilantin) can also aggravate

Wednesday, October 21, 2009

Three Powerful Words

So yesterday I'm sitting in traffic, driving home from my daily 9 to 5 grind. I'm in the right lane. A landrover is next to me. There are two young girls probably no more than 12 with the windows down. One in the front seat and the other in the back. The one in the front seat screams out to me "Jesus Loves You!" At first I'm thinking this is a little strange. But then this smile comes across my face. What powerful words to be screaming out to cars. I saw her scream the same words out to the car in front of me. They also had their window down. And their reaction as well was a positive one. I watched these girls continue their little movement. I continued driving when this feeling just peacefully resonated within me. I guess I NEEDED to hear those three words. I've been struggling lately with negative emotions and things going on. And this was the positive sign I needed to guide me in another direction. SOMEone knew I needed to hear those words. It always seems so much easier just to dwell on the negativity in your life. I consider myself a professional and tend to have my own pity party on a regular basis. But I am in my early 30's and have the tendancy to look forward in time dreading the idea of aging. How nice it would be to stay young forever... But I am now worried that life will slip me by, and I'm spending all these years saying "I wish, I want, If only, etc." Before I know it... I will be 60 and have nothing to look back upon and feel successful about. I may not have the job I always dreamed of. I may not have that child in my arms yet. I may not have that big home that I want to entertain in.... BUT.... I DO have a job that pays my bills and supports me. I DO have an amazing husband who loves me dearly. I DO have a cozy little home that supplies a roof over my head. And all of the above can and will be changed and are within reach. So rather than dwelling on what I don't have, I need to focus on what I do have, and be grateful for those things in my life. Things will change, things WILL get better. I can't live this life I've got now with regrets because I don't know how long I've even got it for.

So to those girls in the land rover: THANK YOU!!! Thank you for those 3 powerful words that really made an impact for me last night. God Bless.

Tuesday, October 20, 2009

Haunting Finds

It's been quite some time since I've had the opportunity to search through fellow etsy creators and see what they are selling. Since we are so close to Halloween, I thought I'd share some haunting finds that I like! Enjoy!

Boo Halloween Premade Scrap book pages by: my2scrappychicks

Price: $19.99 Can be found here: http://www.etsy.com/view_listing.php?listing_id=32978749&ref=sr_list_12&&ga_search_query=Halloween&ga_search_type=handmade&ga_page=3&order=date_desc&includes[]=tags&includes[]=title

20 Halloween Coasters Created by: vermilionstarpress

Price: $7.50

Link: http://www.etsy.com/view_listing.php?listing_id=32890442

LOVE this!

Halloween Gourd Jack-O-Lantern Candy Dish

Created by: PoppyGoRound

Price: $19.00

Link: http://www.etsy.com/view_listing.php?listing_id=32655325

Three Little Witches
Created by: reniebritenbucher
Price: $16.00
Link: http://www.etsy.com/view_listing.php?listing_id=20223184

Wednesday, October 7, 2009

My First 1000 Markets Sale!!

So this morning I woke up to an email on my blackberry that I have never seen before. A sales order from my 1000 Markets Shop!! I have truly neglected that shop since I opened it in May because my main focus has been my etsy shop. I really haven't taken the time to market that shop. I guess because I didn't want to have an item listed in my etsy shop and my 1km shop at the same time out of fear that two people would some how want the same clip at the same time, and here I only have one available. I only want to list items in my 1km shop that I can duplicate and have enough ribbon to do so. Tonight I will be working on getting some new holiday/winter items listed. I have enough ribbon to duplicate, so I plan on listing them in both shops. Then I just have to get out there and do some networking for both. Getting that sale today from Hawaii definitely gave me a little bit of hope because I was about to cancel that shop. So here's to a renewed beginning for my 1km shop!

Tuesday, October 6, 2009

Wow It's been a while!

It has been quite some time since my last posting. Here's a little update as to what's happening in my life and with DDB.

DDB finally completed a HUGE school order that had been hanging over my head for a few weeks. I had to make 25 streamer pony tail ribbons and 25 korker pony tail ribbons. The streamers were a piece of cake and were done within a day or two. The korkers were much more time consuming than I had expected them to be. And I ran out of ribbon at one point and had to order more. Needless to say, the last 19 that I needed to finish after deliver of the first batch took me quite some time. I'm so happy though that this order is finally complete and shipped! I took the weekend off from bows, and last night started back up again rebuilding my etsy stock. I only have 24 items listed, so I've got alot of work ahead of me. I made 6 new items last night that I plan to list tonight. I will continue creating tonight in hopes to build that stock back up quickly. My focus last night was getting holiday/winter items in my shop nice and early. I want to get my inventory rebuilt and keep up some networking in hopes to get sales up. We had a nice jump in sales (more than doubled) in the beginning of September. If I could keep that pace up, then I would definitely start to see the fruits of my labor. Profit/loss wise, I am just shy of breaking even, but it's difficult because I am usually in need of more supplies for something somewhere.

On the personal front... My husband and I are booked every Saturday and Sunday through all of October and even into the first three weeks of November. It's that time of year where you start to think about how a little extra money would be nice. Granted, I think of that on a daily basis all year round. But for some reason when fall hits, I am usually focused on trying to find another way to earn some extra cash if possible, or get a second job. With DDB still fairly new, it's like having a second full time job. I come home and sit down to work on bows. I just need to stay positive and know that all of this hard work WILL pay off someday. Hopefully sooner rather than later. We have the tendancy to go to open houses once in a while. We aren't actively looking to buy a home at the moment, but we like to check out homes and see what they are listed for in order to stay knowledgeable about what's going on in the market. We do eventually want to get into our bigger/permanent home. Well let me tell you..... We walked into a home on Sunday that would be within our price range that I absolutely fell in love with. I walked out of that home with my chin practically quivering and tears almost in my eyes because I could absolutely envision us spending the rest of our lives there. I could picture kids running around and just how comfy and cozy we would make it. We could totally entertain there too. But we are unfortunately not in a position right now to put our house on the market and upgrade. We live in this small lake home. And there are days when I don't know where our money actually went. So it almost feels like we may not ever be able to afford a bigger home some days. And that is just depressing. I love our little home, but we are out growing it as it is. Oh well. There's a reason for everything right....