While another storm is headed in tomorrow, today was a lovely day for a nice walk with our girls: Teagan & our cats! Yes, we've trained our cats to walk on a leash so they can go out and not get snatched away by a racoon or coyote.
This is a victory for every CHD (Congenital Heart Defect/Disease) child and their parents and for adults living with CHD. Thank you to those moms, like Kristine, who have fought on behalf of their children (like little Cora) to get better recognition of and screening for CHDs in newborns!
Advisory Committee on Heritable Disorders in Newborns and Children
Letter to the Secretary, U.S. Department of Health and Human Services
October 15, 2010
The Honorable Kathleen Sebelius
Secretary of Health and Human Services
200 Independence Avenue, S.W.
Washington, DC 20201
Dear Secretary Sebelius:
The Secretary’s Advisory Committee on Heritable Disorders in Newborns and Children (the Committee) is charged with making systematic evidence-based and peer-reviewed recommendations that include the heritable disorders that have the potential to affect public health significantly, for which all newborns should be screened. Thus far, nine conditions have been sent to the Committee for consideration of an evidence review and for consideration for addition to the Committee’s Recommended Uniform Screening Panel. In May 2010, Severe Combined Immunodeficiency (SCID) was added to the panel. During the May 13-14, 2010 Committee meeting, the Committee voted to not recommend the addition of Hemoglobin H to the Panel. At the Committee’s most recent meeting on September 17, 2010, the Committee reviewed a final draft report of the evidence review for Critical Congenital Cyanotic Heart Disease and voted to add this disorder to the Panel.
Congenital Heart Disease is an overarching term describing a spectrum of clinical outcomes derived from any number of defects that are present in the structure of the heart at birth. Specific defects may involve the interior walls of the heart, valves inside the heart or the arteries and veins that carry blood to the heart or out to the body. These varied congenital defects change the normal flow of blood through the heart, leading to a range of conditions and symptoms. Congenital Heart Disease affects about 7 to 9 of every 1000 live births in the United States and Europe and is the most common cause of death in the first year of life, with defects accounting for 3% of all infant deaths and more than 40% of all deaths due to congenital malformations. Critical Congenital Heart Disease is a group of defects that cause severe and life-threatening symptoms and require intervention within the first days or first year of life.
Current methods for detecting Congenital Heart Disease generally include prenatal ultrasound screening and careful and repeated clinical examinations, both in the nursery and as part of routine well-child care. Critical Congenital Heart Disease is often missed during the routine clinical exam that generally is scheduled prior to a newborn’s discharge and many cases of Critical Congenital Cyanotic Heart Disease are missed by discharge and post-discharge clinical exams. A large epidemiological population-based study showed that 78% of cases with hypoplastic left heart syndrome (HLHS) were discharged from hospital before diagnosis. HLHS is universally fatal without surgical intervention, sometimes within the first days of life, and the vast majority of deaths in this patient population occur within the first months of life. Fetal ultrasound screening programs improve detection of major congenital heart defects; however, prenatal diagnosis alone picks up less than half of all cases.
Newborn screening using pulse oximetry for detecting Critical Congenital Cyanotic Heart Disease was examined by the Committee’s evidence review workgroup. Pulse oximetry is a method to augment current approaches (clinical exam and prenatal ultrasound) for the detection of Critical Congenital Cyanotic Heart Disease. Newborn screening using pulse oximetry is a test that occurs at the bedside (in the nursery or otherwise) similar to newborn screening for congenital hearing impairment. Pulse oximetry is a non-invasive test that estimates the percentage of hemoglobin in blood that is saturated with oxygen. While some types of Critical Congenital Heart Disease may present with hypoxemia, they do so only some of the time and are therefore less likely to be detected by pulse oximetry screening. Neonates with abnormal pulse oximetry screening results need confirmatory testing for the cause of the cyanosis, and immediate intervention. Virtually every hospital, even small ones, frequently uses pulse oximetry as a standard of care in their newborn nurseries.
When developing its recommendations to the Secretary, the Committee considers the nature of the science itself underlying the potential additions of the technology and the heritable conditions to the Committee’s Recommended Uniform Screening Panel as well as the public health implications of implementation. Although there are recognizable evidence gaps (for example, standardization of screening protocol) there are compelling reasons for recommending screening newborns for Critical Congenital Cyanotic Heart Disease.
The Committee therefore recommends the addition of Critical Congenital Cyanotic Heart Disease to the Committee’s Recommended Uniform Screening Panel with the understanding that the following activities will also take place in a timely manner:
1. The National Institutes of Health shall fund research activities to determine the relationships among the screening technology, diagnostic processes, care provided, and the health outcomes of affected newborns with Critical Congenital Cyanotic Heart Disease as a result of prospective newborn screening;
2. The Centers for Disease Control and Prevention shall fund surveillance activities to monitor the Critical Congenital Cyanotic Heart Disease link to infant mortality and other health outcomes;
3. The Health Resources and Services Administration shall guide the development of screening standards and infrastructure needed for the implementation of a public health approach to point of service screening for Critical Congenital Cyanotic Heart Disease; and
4. The Health Resources and Services Administration shall fund the development of, in collaboration with public health and health care professional organizations and families, appropriate education and training materials for families and public health and health care professionals relevant to the screening and treatment of Critical Congenital Cyanotic Heart Disease.
The Committee fully recognizes that the various Agencies within HHS determine and carry out their missions within their goals and the budgets which they have available.
R. Rodney Howell, M.D.
Source: http://www.hrsa.gov/heritabledisorderscommittee/correspondence/October15th2010letter.htm The Secretary's Advisory Committee on Heritable Disorders in Newborns and Children (SACHDNC) was chartered in February 2003 to advise the Secretary regarding the most appropriate application of universal newborn screening tests, technologies, policies, guidelines and standards for effectively reducing morbidity and mortality in newborns and children having, or at risk for, heritable disorders. SACHDNC assists the Secretary, U.S. Department of Health and Human Services, specifically by providing: -advice and recommendations concerning the grants and projects authorized under the Heritable Disorders Program; -technical information to develop policies and priorities for this program that will enhance the ability of the State and local health agencies to provide for newborn and child screening, counseling and health care services for newborns and children having or at risk for heritable disorders; and -recommendations, advice or information that may be necessary to enhance, expand or improve the ability of the Secretary to reduce the mortality or morbidity in newborns and children from heritable disorders.
Please visit this site to learn more about what tests should be done on your newborn prior to you checking out of the hospital. You can also learn more about CHDs as well read other helpful facts for any expecting parents at Cora's Story.
My father, when cooking, always used to say, "All you need is a good sharp knife.." in order to prepare a great meal.
I always thought that was a little simplistic...I mean you need good ingrediants, a good plan, good pots & pans, etc, etc, etc. But, I've got to say, that while good ingrediants and a fairly decent plan are essentials, the pots and pans quality aren't anywhere near as important as having a good sharp knife in order to prepare food. Honestly, you don't even need measuring cups or spoons...but a good sharp knife: can't do without!
At some point, probably right as I graduated from high school, my parents aquired a really great set of knives, from my grandfather who sold Cutco knives, and my father bequeathed me his butcher knife...it was old, but well-kept, well-sharpened.
I loved that knife! I protected that knife over the years: warned my roommates about its care, to not place it in a dishwasher, to not use it on non-food items. Throughout college and the few years following, I cooked a lot of meals~ranging in size from just myself to over 100 or more people. Always near to hand was that knife. When I would go home to visit my folks, I'd take the knife to make sure my dad properly sharpened it.
(Now I have to admit, that my other knives during this time sucked: they were cheapos from places like the 99 Cent Store~when they broke, I'd just replace them...ironically, doing so adds up, cost-wise.)
Then, about 12 years after I'd recieved this knife, I decided to go to culinary school. I won't name names, as this is a rather well-known culinary school, but it wasn't the CIA (no, not that CIA~The Culinary Institute of America). So, saddly, after paying my first $1000 or so in tuition, I discovered I hated this school. I was bored there... unlike the CIA, they didn't test you before you were accepted. Meaning, anyone could decide that they wanted to be a chef, give them money and show up for classes. They started at a very elementary level... You know: "This is a knife...a paring knife...it is used for..." Or, "These are herbs...fresh herbs...blah, blah, blah." They started with stocks. I was bored stiff. And, since I'd already had 2 light doses of food poisoning in 2 weeks time, and was unemployed and having trouble coming up with the hefty tuition payments, I dropped out. (The only time I ever dropped out of something...I'd never even dropped a class in college!)
I wasn't too upset about it, as I wasn't that impressed with the school. However, the really nice thing about having entered the school and then dropping out is all the gear I had purchased with my first tuition payments. All of which was not returnable. My kit included various spoons, ladels, baking items, measuring spoons & cups, and (cue heavenly "aaaahhhh aaaahhhh" music here) an incredible set of German-made chef's knives.
These knives rock! They were, to quote, Mercer--a company name highly regarded in the culinary trade--is synonymous with quality, professional cutlery. A leader in the commercial market for more than 30 years, and the predominant supplier to more than 90% of culinary academies in North America, Mercer brings you the finest tools used by the industry. Their benefits are:
Superior High Carbon, No-Stain German Steel
One-Piece, Fully Forged Construction
Triple Riveted, Ergonomically Designed POM Handle
Razor-Sharp, Taper Ground Edge, that Allows For Easy Resharpening
Full Tang Provides Superior Balance
Sure, Confident Grip for Comfort, Efficiency, and Safety
Limited Lifetime Warranty
Not only did I get a set of 10 knives, but I got this cool carrying case and even a wheeled suitcase to haul all this stuff around it from my home to my little "catering" jobs. Loved it!
Of course mine were cooler as they had the culinary school's logo on them.
Point being I freakin' love these knives!!!!!!!!
Ok~stick with me here, I have a point to this whole story.
My brother-in-law's 18 year old nephew found a new job this past week. Now, I love this kid. My sister has been a part of his life since he was since he was about six years old. Which means, we too have been a part of his life for years. Which means we want to support him in his endeavors. Which means that I drove 20 minutes to take him to job training last Friday. And then of course agreed to let him come and give a "practice demonstration" of his product. Oh, did I mention that TJ is the newest Cutco Knive representative?
That's right: Knives!
We had a game plan, mom & I: support TJ by listen to his schpiel and~to support him~buy ONE, 1, UNO, UN Knife from the kid.
I even prepped for our demonstration, making sure that my knives were in super sharp condition. I felt a little sorry for the kid even: here I was with a great set of knives, with a sales background, and virtually no money. But, he was getting his practice in, he's currently getting paid a superior hourly wage whether he sells anything or not, and I would be a good~though gentle~critic.
So...uh....not sure where our plan went but I think "out the window" is a good term. This kids is a freakin' natural salesman!!!!!! He is unreal!!! His demontstration was superb. There were some minor things I pointed out later, some tips I offered later. He knew his product & the company. He knew his knives. He knew how to sell.
The worst part is, his knives were (slightly) better than my current ones! (*Gasp!!!*) I could feel the difference when I cut through rope with mine (3-5 saw motions) versus his (1-3). And also when I cleanly cut through a piece of leather with my knive (as TJ held the leather while I cut) versus when I held his knife and he smoothly slid the leather across the blade with nary a tug to render it in two.
And they were so pretty...sure they have the basic ("classic") black, but they also have these really pretty "pearl" colored handles.
See how pretty they are?
Thes are the table knives...so pretty. And see this nifty 5-piece set:
It has all the sharp knives one can ever want...only in pretty pearl.
And my mom likes that they are American made.
And then there's the warranty...unlike my knives guarantee that dies when I do, Cutco knives can be handed down through the generations, and the guarantee still applies! And, just in case you don't believe my local Cutco representative, who is after all only 18 years old, I checked online:
So what about our plan to buy just one knife from TJ....out the window. 'Cause see those knives pictured above? Yeah, we got them all. That's right, we got the 5-piece space saver that comes with a 4" Paring Knife, a 7-5/8" Petite Chef, a 6-3/4" Petite Carver, a Trimmer, a 2-3/4" Paring Knife, and a Space Saver Block (5-Pc.) to store them all in. Plus not 4, but 6 table knives. (Actually we purchased 2 table knives and my local rep threw in 4 more for free!!! Awesome!) And all with those nice pearl handles.
And do you know why we got so many knives? Why, not because we are total suckers, but because we are extremely supportive family members. Because we were given a deal. Because we get 5 months to pay off our hefty bill. Because I've never been married and my grandfather only gave out the knives as wedding gifts. And, oh, ok, because we just can't say no! We are, in fact, suckers!
Am I going to get rid of my nice German-made knives? Not on your life! I still love them. Hell, I still have the wood-handled butcher knife my dad gave me 21 years ago!
Yeah, so I guess you could say that all you ever need is a kitchen-full of sharp knives.
I mean honestly: who could say NO to this face???
(And yes, that's Teagan with him when she was about 4 months old!)
Yesterday was Teagan's first visit to the pumpkin patch. Needless to say, she loved it! She just loves new adventures! Nothing fazed her. She jumped into the wagon like she rides in one all the time, she wandered around looking at all the pumpkins and vines and squash~the whole time carrying one little pumpkin she had found that caught her attention. It was such a fantastic time! Plus it was Gramma Cindy's birthday, so we had lunch after at the cutest little restaurant where Teag ordered her own lunch: "Avacado please!"
And then she zonked out in the car ride home and her Auntie M staggered upstairs after being dropped off at home and slept for 3 hours too. No wonder we get along so well!
Notice she is already carrying 'her' pumpkin as she explores the larger pumpkins.
Teagan wanted to help pull the wagon, but found that pushing was an easier way to help.
I'm tired! Somebody help me into the wagon, please!
"Six!" Teagan knows her numbers!
Holy crap! These were at least 2 feet long!!!
This pumpkin/gourd was shaped like a flower;
Teagan loved it and kepts "smelling" the pumpkin flower!