Thursday, October 25, 2012

My Meme...


Early this morning the greatest woman I've ever known went home to be with the Lord.  My Meme was my maternal grandmother but she was so much more than just a title.  She was truly one of the greatest influences in my life.  Selfishly my heart is broken today but I am also celebrating her great reward in heaven.  If anyone in my life deserved to hear "Well done my good and faithful servant" it was Meme.

She faithfully loved my Papa for 62 years until his passing two years ago. In today's world that is a HUGE accomplishment.   They married in 1948, the day after her 18th birthday, and never looked back.  She would tell you he drove her bananas at times with his opinions and crazy politics but she loved him nonetheless.  She was a mother of two, grandmother of four and great-grandmother of eleven.  Alzheimer's is a terrible disease that robbed my Meme of so many wonderful memories over the past twenty years.  She slowly forgot the names of my babies, my husband and so many of those she loved dearly.  She even forgot our oldest daughter was her namesake.  It has been so difficult to watch from the sidelines as her mind slowly slipped away but I am thankful she is now whole, healed and in the presence of her Creator.  I know, because of all she taught me, I will see her again.

If she taught me anything it was to love Jesus.  If they cracked a window to the church building my Meme and Papa were there with bells on.  During service, she always sat with Papa to her right and me to her left.  She would wrap her arm around me and hold my hand while I played with her wedding ring.  She talked to me often about what it meant to love God and make choices that were pleasing to Him.  I had no idea at the time how those days would eventually come full circle.  I'm thankful she chose to "train up a child" even when all I wanted to do was sleep in.  To this day each time I sat beside her she would reach for my hand to hold.  This 34 year old wife and Mother of four gladly reached back to hold the frail hand of one who has loved me so well. 

My Mom and I lived with Meme and Papa from the time I was three or four until I was in 5th grade.  Mom worked about an hour away so my Meme was usually the one to get me ready for the 6:10 am bus stop.  I vividly remember the smell of sausage gravy and biscuits as she carried me through the kitchen to the breakfast table.  Most mornings I didn't touch my food opting for an extra 10 minutes of sleep but she always got up at 5 am to cook regardless.  As I got older I would tell her she didn't have to cook for me but she wouldn't hear of it.  "Breakfast IS the most important meal of the day" she would always say.  I had to go school with a full tummy so my "brain would be awake and ready to learn".  She always regretted her lack of formal education and constantly stressed to me the importance.  I never forgot. 

Deep down I think she wanted me to be a singer so she encouraged me to sing at church and play piano. That was a complete and TOTAL pipe dream on her part but it took her a while to cut me some slack on that one. Her confidence in me to do ANYTHING was always genuine. However, if you've heard me sing you would have questioned her judgement on this one. It just goes to show a grandmother's love is much like a mothers. Tone deaf. 

As I reflect I realize many of my memories of Meme come from her kitchen.  She always saved the biscuit dough for me to play with (back when PlayDoh wasn't a household staple).  She made me eat the disgusting broccoli casserole because all good girls eat their vegetables.  She made the BEST chess pies on every semi-important occasion.  One chess pie filling + 2 pie crusts = the skinniest chess pies known to man.  My entire childhood I thought chess pies were suppose to be skinny.  I didn't know it was because she knew how to stretch a dollar.  She taught me to shape a perfect hamburger patty.  Then she regretted the decision when I wrapped one patty of perfection in aluminum foil, placed it in the MICROWAVE for 10 minutes and went back outside to play.  :)  Thankfully she forgave me for almost burning down the house after a few days and a quick batch of her favorite no bake cookies as a peace offering.  Even then I knew my sweet loving Meme couldn't resist a little chocolate and a good hug from her one and only granddaughter.   

She wasn't fussy or pretentious. She was simply LOVE. She never made you question if you were valued or important. She told you. You never entered or left her presence without a hug and an I love you. It was simply a given. When I was six years old, I lost my half sister in a tragic accident. The last time I saw her I refused to hug her goodbye and tell her I loved her because I was mad. I'm sure it was over something silly but after she died I was so upset about my decision. I confided in my Meme about what had happened and why I was so sad. I remember where we sat, the color of the dress she wore and the way she stroked my hair as I cried. She told me she was sure that A knew I loved her. However, I should ALWAYS tell the people I care about that I love them no matter what... even if I'm mad. That one conversation has stuck with me my entire life. If you are dear to me, you'll know. I have no reservation in telling you.  I am proud to say the greatest example of unconditional love I've ever known taught me that.

Yes, I am sad.  How strange it was to inch up the driveway today knowing neither my Meme or Papa were at home.  The house was quiet.  No one smiled brightly when I walked in the door.  No one asked me how I was at least 25 times or told me to enjoy my children because they will grow up fast.  No one told me the story of the time me and my three cousins took a pill and got in big trouble.  No one chuckled when she spoke of fly swat spankings or endless summers of mischief.  No one reached for my hand today or hugged me with the long lingering hug of a grandmother.

However...

Today my Meme is no longer fragile and suffering.  For that I am overjoyed.  I am indescribably thankful for her life and the gifts of love she leaves behind. She was a great woman who loved God and loved others more than she loved herself. In the end there could be no greater legacy.

Sunday, September 16, 2012

Two months with Little Buddy...

Two months ago today we met our little caboose. He walked straight into my arms, appropriately clad in his Thomas the train shirt, and never looked back.


That one little shirt was and continues to be our reminder of what we knew last October. We were the family for him regardless of any trial or circumstance to come. 
 
 
I wish you could hear the belly laugh that accompanies these photographs. Laughter within the first five minutes.
 
 
 It was a gift we never dreamed. 

 
Many have prayed, supported and celebrated with us over the past two months. I can't begin to call you all by name but we thank you for loving us and our new little blessing. 
 
 
Happy TWO months Little Buddy! You sure know how to shake things up around here but we can't imagine life without the JOY of you!

Friday, September 7, 2012

What is g6pdd?

Glucose-6-phosphate dehydrogenase (g6pd) Deficiency
The following information copied directly from No Hands but Ours  

Overview

G6PD deficiency is an X-linked inherited blood disorder in which the body doesn’t have enough of the enzyme G6PD. This means that if a boy (XY) receives an X chromosome that is deficient, he will have a significant G6PD deficiency. A girl (XX) can receive either one deficient X chromosome, or two. This means that girls can have more varied levels of G6PD deficiency, from non-symptomatic to highly deficient. Even some girls who are carriers (one good X and one bad X) have been found to be symptomatic. Some females are more highly deficient than expected, because they have one deficient X chromosome and a second mutated and/or damaged X chromosome.
  • This is the most common inherited enzyme defect in the world.
  • With the right precautions, a child with G6PD deficiency can lead a healthy and active life.
  • G6PD deficiency is common in Guangdong, Taiwan, Guangxi and other parts of South China. It is found in the Han, Zhuang, Li and Miao ethnic groups, as well as others.
  • In China many of those affected with G6PD deficiency have less than 10% enzyme activity, resulting in a high degree of sensitivity to oxidizing substances.
  • Some of the G6PD variants result in chronic hemolytic anemia (CNSHA).
  • G6PD deficiency is thought to be a defense against Malaria and occurs in the same regions as the Thalassemias. A person can have both Thalassemia, major or trait and G6PD deficiency.
  • Tests for G6pd deficiency in boys are easily administered and reliable.
  • Tests for G6pd deficiency in girls are difficult, expensive and often require genetic analysis. However in China new more reliable, less expensive tests are being developed to detect G6PD deficiency in girls.
  • G6PD deficiency in women has been found to become more acute as they age.
  • Those who are G6PD deficient are likely to have an increased risk of diabetes, hypertension, sepsis and its complications and cataracts.
G6PD is required to neutralize oxidative substances in the body and metabolize carbohydrates properly. Without enough G6PD, red blood cells begin to break down quickly. G6PD is important for the life of all cells, cell growth and development. G6pd deficiency is not curable at this time. The only treatment is avoidance of trigger substances and hospitalization and blood transfusions in cases of extreme hemolysis (breakdown of red blood cells). Extreme haemolytic episodes can result in renal failure and/or death.

Things to avoid

Each person, and each G6PD variant (approximately 400) can react differently to identified trigger substances. There are however, accepted lists of substances that may need to be avoided.

These include:
  1. NSAIDS (Asprin, Ibuprophen)
  2. Tylenol
  3. Quinolones
  4. Drugs metabolized through the liver or known to cause blood or liver related problems or hemolysis
  5. Sulfa drugs
  6. Petrochemically derived substances (This is a long list and gets longer every year. Many artificial foods, dyes and vitamins are included in this list.)
  7. Moth Balls and anything containing naphthalene.
  8. Methylene and Toluidine blue
  9. Legumes and their derivatives (for example: soy, peanut, beans, peas, licorice, food thickeners and gums, MSG)
  10. Other substances including blueberries, blue food coloring, tonic water/quinine, red wine, sulfites, mothballs, and petroleum derived substances.
  11. Illness / fever can also trigger G6PD symptoms.
Some G6PD deficient people try to reduce oxidative stress by ingesting antioxidants (w/o blueberries or blue food coloring) and taking folic acid, in addition to avoiding trigger substances.

Symptoms

Symptoms can be found in both those who have been diagnosed (boys and some girls) and those who have not yet been diagnosed (esp. in girls from South China / SE Asia). Symptoms generally occur within three days of exposure to triggering substance. Once the triggering substance is removed or the illness resolved, the symptoms generally improve over a period of weeks. Mild symptoms can be treated at home, more severe symptoms may require hospitalization.
  • paleness (in darker-skinned children paleness is sometimes best seen in the mouth, especially on the lips or tongue)
  • extreme tiredness
  • rapid heartbeat
  • rapid breathing or shortness of breath
  • an enlarged spleen
  • dark, tea-colored urine
  • abdominal / back pain
  • bruising
  • fever
  • weakness
  • dizziness
  • confusion


Classes of G6PD Enzyme Variants:


Class Level of deficiency Enzyme activity Prevalence
I
Severe
Chronic nonspherocytic hemolytic anemia in the presence of normal erythrocyte function
Uncommon; occurs across populations
II
Severe
Less than 10 percent of normal
Varies; more common in Asian and Mediterranean populations
III
Moderate
10 to 60 percent of normal
10 percent of black males in the United States
IV
Mild to none
60 to 150 percent of normal
Rare
V
None
Greater than 150 percent of normal
Rare


Adapted from AAFP.org

Resources
• www.g6pddeficiency.org
• www.rialto.com
• www.g6pd.org
• www.ghr.nlm.nih.gov
• www.nlm.nih.gov/medlineplus/ency/article/000528.htm
• memo.cgu.edu.tw/cgmj/2809/280902.pdf
• www.hkcpath.org/docs/Topical%20Update/Topical%20Update%20V2I1.pdf
• www.aafp.org/afp/2005/1001/p1277.html
• www.bioinf.org.uk/g6pd/
• Hirono A, Fujii H, Miwa S. Identification of two novel deletion mutations in glucose-6-phosphate dehydrogenase gene causing hemolytic anemia. Blood 1995;85:1118-21.
• Mason PJ, Sonati MF, MacDonald D, et al. New glucose-6-phosphate dehydrogenase mutations associated with chronic anemia. Blood 1995;85:1377-80.

Friday, August 17, 2012

I'm sorry? Can you repeat that? g- what?

Most of you don't know the details of something I alluded to after our orphanage visit.  Little Buddy's birth note listed the phrase "Diagnosis g6pd".  I asked our guide what that meant and he said he thought it was his airway condition.  Considering I know nothing about Chinese medicine and the fact that it ended in PD (in my nurse brain pd = Pulmonary Disorder) I accepted what he said as truth.  Unfortunately, that night I consulted Dr. Google and discovered he was wrong.  g6pdd is an acronym for a genetic disorder called Glucose-6-phosphate dehydrogenase deficiencyIt is a genetic enzyme deficiency which can cause hemolytic anemic (a blood disorder).  At the time we had no idea if he truly had this condition but if so it might explain a lot.   

Fast forward...

Friday, August 3, 2012... 

We had hope the birth note listing g6pdd wasn't accurate.  The test had to be sent to a specialized lab so the results came back this afternoon.  Unfortunately, he does, indeed, have g6pd deficiency.  According to Dr. B his level is very low so off to hematology we go.  It was already on our list of consults because of other abnormal lab work but now it moves quickly up the list.

Obviously the Lord knew all of this but now we have to try to sort it all out.  In the big picture it could be worse but it would have been nice to have this information beforehand.  We gave him medication he should not have taken (which may or may not have caused his seizures).  We gave him food and milk he can't eat or drink.  WHY DID THE ORPHANAGE KEEP THIS FROM US????  They knew the information but chose to withhold the information from us.   

It is not very common here in the US but is the most common inherited enzyme deficiency worldwide.  With lots of research I am finding the main change will be the way we eat.  Removing MANY foods from our diet.  Limiting certain vitamins and minerals because he can't process them.  A LONG list of medications he can never ingest.  All of these things can be triggers that send him into a hemolytic crisis.  We do not feel it is fair to make him eat completely different than we do nor am I going to be his short order cook.  Thus the reason this will need to be a change for our entire family.  Quite frankly, we are OVERWHELMED but we will figure it out one day at a time.

The Chief and I?  We're just sad... angry... humbled... frustrated... overwhelmed... yet thankful.  I honestly don't know where to begin.  I know once we figure it out it won't be so overwhelming but right now that's where we are.  Yes, he will live a long life as long as we are diligent about avoiding/removing the triggers from his body.  He will require diligent eyes to keep him safe.  He may require hospitalizations and blood transfusions along the way.  In the big picture this feels like big stuff but eventually it will be small potatoes.  We know that.   Yes, we are overwhelmed but we are still thankful we said yes.  We are even more thankful we said yes on those very scary days in China.  Now more than ever I know he NEEDED to get out of there and receive proper care.  Now he has a Mommy and Daddy who will fight for him instead of ignore what he needs.  He didn't have that three weeks ago.  I guess we'll just become his very granola Mommy and Daddy who are nazi-ish about everything he puts in his mouth.  I guess it could be worst, right?

To those of you who found out this information via Facebook... I am SO sorry!  We would have much rather you heard it from us but there's nothing we can do about it now.  There are always multiple sides to every story.  One you tell your inner circle while you process an enormous life change and one you present to the world after you figure it all out.  Then there's this thing called Facebook that can, unbeknownst to you, tell the world before you are ready.  As many of my friends in real life already know, I posted in what I thought was a CLOSED group about something I wasn't ready to tell the world.  However, I was actually posting in an OPEN group.  8/  So, FB decided it was OK to share my posts in the newsfeeds of 480+ of my "closest" FB friends.  Lovely...

Fast forward until today...

Friday, August 17, 2012... 

Today we saw the hematologist.  We are now awaiting insurance approval before we can determine Little Buddy's g6pdd variant.  Once we know the variant we can learn the characteristics of how it will most likely effect him and to what severity.  I honestly don't feel we know much more than we did yesterday.  I asked many questions.  Some she knew the answers to and some she did not.  We have discovered so much discrepancy between physicians about this disorder we don't know what to believe.  Over the next few weeks we will be searching for more information.  Pray for discernment as we make changes for our Little Buddy.  All the kids are taking it in stride...thankfully!  Now Momma and Daddy need to adjust to the new normal...

Thursday, August 16, 2012

One Month: August Update...

As of today we've been a family of six for one month.  Things are going really well overall but so much has transpired the Chief and I are still spinning out of orbit at times.  Since I am the one who documents the life and times of our family I need to write it all down.  This is going to be a REALLY long update.  Feel free to skip to the pictures if you get bored...

July 26,  2012...
Home Sweet Home!

July 30, 2012...
We arrived home on Thursday evening and saw Dr. B, our new pediatrician, first thing Monday morning.  The short version of our lengthy appointment was Little Buddy needed to see a Pediatric Neurologist ASAP.  We knew that was a given after the events of July 17th.  Thanks to my friend K he already had an appointment for Wednesday with Dr. S (the doctor Dr. B would have sent him to anyway).  Sometimes it is great to have connections.  :)  THANK YOU LORD and K! 

 August 1, 2012...
We saw Dr. S on Wednesday morning.  Liked him a lot.  He first started with the recommendation of medication immediately.  Of course I cringed and talked him out of it.  :)  I would like to have a little more proof of a true neurological issue before we put him on meds.  He agreed, a little reluctantly, but my PCCU nurse shined through and we came to a reasonable agreement.  The current wait for an EEG is 4-5 weeks which is the reason he wanted to start meds quickly.  The nurse came back and said, "Would you believe they had a cancellation for Monday at 1pm?"  My reply was "WHY YES I CAN!  It's for Little Buddy!"

We left Dr. S office and went back to Dr. B's office for lots of lab work.  The first set of labs came back that day.  The short version is his hematocrit and hemoglobin were a little on the lower side for his age but nothing too alarming.  His platelet count remains very high at nearly 800,000.  His lead level was normal. 

August 3, 2012...
To Be Continued...

August 6, 2012...
Monday at 1 pm we ventured to our local Children's Hospital for the EEG.  Little Buddy was a little unsure at first but overall he did REALLY well.  We should have the results by Thursday. 

August 9, 2012...
EEG results:  NORMAL!!!!  

August 16, 2012...
After the first night home Little Buddy has been sleeping pretty well thanks to a little help from our friends Mr. Benadryl and Miss Melatonin the first two weeks.  He is still waking up in the night off and on with what we call "the China cry".  It breaks my heart every time but I know it will get better with time.  The boys switched back within a few days.  It took me about three weeks to switch back completely which contributed to the insanity around here until we were all on the same time zone.  I thought I was going a little nutso.  Thank goodness the Ambien finally worked.  Outside the medical stuff this has been our biggest challenge so I'm glad it's behind us.  Jetlag STINKS! Overall everyone seems to be settling in well.  Mei Mei is a little jealous but nothing out of the ordinary for welcoming a new younger sibling.

Thursday, July 26, 2012

Some Little Boys Come by Plane...

Throughout each China adventure the one thought that never ventured far from my mind was THIS one.  The moment when we would step off the last plane and our family would be together again.  Leaving behind two children each time for the sake of a little one who needs you, just as much if not more, is incredibly bittersweet.  THIS moment?  It's indescribable joy from the second those wheels safely touch the ground.









 
 

The sign on your left says "Welcome Home!  I hope you like trains!"  ;)

 





 
 
















This is the moment our sweet Mei Mei said, "Hi Jian Jian.  Me ____ Yang Su".  Then she hugged him...  {boo to the hoo}






Meeting grandparents...



and special friends...













So what are we waiting on?  Where is this place we call HOME??




 

Excuse me... What is this apparatus and why have you strapped me in?
 
 

The Beginning of life as a Family of SIX!

{photos captured by our dear friend Sara who has been photographing our family since Mei Mei joined our crew.  Since I blog anonymously I can't link her here.  If you are local and need a FANTASTIC photographer with an AMAZING HEART email me.  I am happy to share her.}