Monday, April 13, 2009

update and prayer request

Unless something drastic happens or we learn new information, Matt and I have decided to have them scope Jonah's upper airway tomorrow morning (just the upper airway scope done in the clinic, not the OR one that would require sedation).

We read the article, "Tracheolaryngeal Complications of Inherited Epidermolysis Bullosa" by Dr. Fine, and that pretty much made up our minds for us. It was a very scary article based on a lot of NOT encouraging findings, and I end the day on a low after having read the information. It does not give a good outlook for Junctional EB - either kind. Although not every child with JEB suffers from airway complications, a good number do, and Jonah is already showing the signs.

Here are some parts we highlighted:
- In a 1980 publication, a 3-week-old child with an autosomal recessive form of EB, clinically most consistent with JEB, presented with inspiratory stridor and retractions, necessitating tracheostomy.

- Approximately 55% and 47% of all patients with JEB-H (Herlitz) and JEB-nH (non-Herlitz) die by age 6 as based on EB Registry lifetable analyses published in 1999. (47% ??????)

- By excluding all other well-known causes of death in these patients, to include failure to thrive, sepsis, pneumonia, and renal failure, we can estimate that up to approximately 10% and 23% of all JEB-H and JEB-nH patients, respectively, die from some other undefinable cause, which would include airway occlusion.

- The magnitude of these cumulative risks for laryngeal stenosis or stricture becomes clinically even more concerning given the fact that sudden airway occlusion and death have been repeatedly described anecdotally in JEB infants and children.

- Because the earliest sign of laryngeal involvement is a weak or hoarse cry, we strongly recommend visualization of the upper airway in any child with EB who presents these findings.

- Elective tracheostomy is the most prudent approach to prevent the risk of sudden airway occlusion occurring later.

- Indeed, we have had two infants die when tracheostomy was deferred because of apparent clinical resolution or improvement of symptoms.

- ... there is a substantial risk of laryngeal stenosis or stricture in infants with JEB and that airway obstruction does contribute to the overall high risk of early childhood motality as well as morbidity.

- Because it is now possible to prevent the majority of JEB infants and children from dying of other causes, most notably septicemia (infection), we would argue that the possibility of preventing airway obstruction in these patients appears to outweigh any concerns over surgical risks from the performance of elective tracheostomy.

- Initially presenting as hoarseness of cry or voice, this may progress to inspiratory stridor and partial or complete airway occlusion. Given that sudden death by suffocation will occur if complete airway occlusion is not immediately reversed, performance of elective tracheostomy when early symptoms of airway compromise arise may prove to be a life-saving preventative intervention.

So anyway, please pray that it's only a cold with caused all of his symptoms or that it is a result of the acid reflux he's battled. I don't want to focus on the negative or believe the worst, but I am really, really scared.

Please pray for Jonah tomorrow as they scope him. Please pray that he will be strong and that his interior will be tough and can resist damage. Please pray for the results, that it is not serious and not something that will result in a tracheostomy. Please pray for us - that we will have peace and wisdom as we talk to the doctors and make decisions for Jonah. Please pray that this is not what it seems, and that all these symptoms are indicative of something much less serious. Please pray that God will send a legion of angels to watch over Jonah tomorrow as he gets the scope and well, that they'll just hang around and protect him for the rest of his long, long life. :)

Thank you for praying. I'll update as soon as I can.

72 comments:

MelH said...

Patrice,
There are no words to describe how hard I am praying for Jonah. I am praying for that legion of angels tomorrow morning and for the power of our Lord to be in that hospital with you all. Dear momma, I will be thinking and praying for you all during that time. With much love from Arkansas...The Hyso's

On a side note, a few of us here in NW Arkansas are walking in the March for Babies next month and I and my sons will be representing Jonah and Gabe. Your family has touched mine and will always. You have an extended family here in the Ozarks that love Jonah in a fierce way.

courtney said...

Praying for you all.....

Lindsay K said...

I wanted to share this with you, as it helped me in dealing with my daughter's birth defect and all that has come with it. Know that your family is in my prayers, and it will get better.

WELCOME TO HOLLAND
by
Emily Perl Kingsley.

c1987 by Emily Perl Kingsley. All rights reserved

I am often asked to describe the experience of raising a child with a disability - to try to help people who have not shared that unique experience to understand it, to imagine how it would feel. It's like this......

When you're going to have a baby, it's like planning a fabulous vacation trip - to Italy. You buy a bunch of guide books and make your wonderful plans. The Coliseum. The Michelangelo David. The gondolas in Venice. You may learn some handy phrases in Italian. It's all very exciting.

After months of eager anticipation, the day finally arrives. You pack your bags and off you go. Several hours later, the plane lands. The stewardess comes in and says, "Welcome to Holland."

"Holland?!?" you say. "What do you mean Holland?? I signed up for Italy! I'm supposed to be in Italy. All my life I've dreamed of going to Italy."

But there's been a change in the flight plan. They've landed in Holland and there you must stay.

The important thing is that they haven't taken you to a horrible, disgusting, filthy place, full of pestilence, famine and disease. It's just a different place.

So you must go out and buy new guide books. And you must learn a whole new language. And you will meet a whole new group of people you would never have met.

It's just a different place. It's slower-paced than Italy, less flashy than Italy. But after you've been there for a while and you catch your breath, you look around.... and you begin to notice that Holland has windmills....and Holland has tulips. Holland even has Rembrandts.

But everyone you know is busy coming and going from Italy... and they're all bragging about what a wonderful time they had there. And for the rest of your life, you will say "Yes, that's where I was supposed to go. That's what I had planned."

And the pain of that will never, ever, ever, ever go away... because the loss of that dream is a very very significant loss.

But... if you spend your life mourning the fact that you didn't get to Italy, you may never be free to enjoy the very special, the very lovely things ... about Holland.


It's not fair that any parent has to hear the words that there is something wrong with their child, but God has a plan and there is a reason God trusted you with the care of this precious, precious child. It's scary not knowing what the future holds, but you are doing a great job dealing with your little one's situation one day at a time. As parents, we do the best we can, and make the most educated choices we can for our children's health. You are an AMAZING mother, and your family is in my prayers.

Jill said...

Simply praying for that little sweet face with those adorable blue eyes, and strength for you and Matt.

Mike and Molly Spivey said...

Patrice - we are praying very hard for you, Matt and Baby Jonah! We are praying that the Lord protect him and guide the Doctors. We are praying for wisdom for you and the doctors and we are praying for peace for you and Matt. You are the best Momma for the little man - keep fighting for him!

Natalie said...

I have been so touched by your sweet little boy. You have really been trusted by our heavenly father to take care and love this sweet little boy. I wish you all the best and love seeing the pictures of your cute little man. Praying for you...

RLR said...

Patrice, you have once again written one of the most honest, most beautiful prayer requests I've ever read. Praying for all of that and more for your sweet boy....

Watts Family said...

My heart is heavy for you cause I know as parents you are so very worried. We are all still praying for you and we serve an Awesome God who will always be there for you. Jonah has shown you his strength thus far...he is a fighter!! Stay strong Patrice!! We're praying!!!

Many Blessings,
Amanda

elle said...

Sweet sweet Patrice,

Please know how hard my family and I are praying for your precious boy. We hope you can feel all the love and prayers directed towards your every hope, wish, and need. So many people are coming together in prayer over Jonah- he has already performed miracles in his little life! And he will continue to do so. Please keep your eye on the Lord, and continue to look to Him for the strength you, Matt, and Jonah need. With fervent prayer and love, The Auvil Family

jewelrylady said...

Patrice: Our family is praying so hard for your sweet little Jonah and Matt! You're all on my heart all day & into the night.

You, once again amaze me at your honesty in your prayer requests. We so believe in being specific in our needs when we go to our Heavenly Father. Know that you're being showered with prayers....prayers for God's love and peace to surround you and Matt, strength & knowledge for Jonah's medical team and strength and healing for your little man!

Nicole said...

Please, please know that I'm begging God to protect Jonah and for healing for your precious son...

littleleaps said...

Praying for your sweet Jonah and all of the technicians, nurses, and doctors who will be working with him tomorrow. I pray for peace for you tonight and for your fears to be taken far from you. Lord, we beg for your tender mercies to be new in the morning.

Sarah @ Thrifty Decor Chick said...

I know you must be just sick right now, but know that your child is not every child with EB, or even 47 percent of children with EB. He may be completely different from every one of them. And after tomorrow, at least you will know what is going on, so you can address it. Knowledge is power. Praying for you.

Kelsey said...

Oh Patrice, I don't know you but I wish I could give you a big hug and take all of your worries away. I will be praying for you and thinking of Jonah tomorrow morning when he has his scope. I appreciate you taking the time to type the highlights of the article that you read and put it on your blog, it really helps us blog readers understand what is going on with your Jonah. After reading those main points, I would have made the same decision if it were my child. Take care and thinking of you in Washington State!

Anonymous said...

I am praying that your Sunday is coming. I am praying for a miracle for Jonah - sweet, precious, beautiful baby boy that he is. And I am praying for strength and conviction for you and Matt. You can do this. You can.

Love and prayers from Texas

Kristin said...

Praying for you in Dallas. He is soooo pretty, and you are doing amazing things for him! Keep fighting!

Our family page said...

I can only imagine what you must have been thinking when you were reading that article. I know you are both so scared. But... remember that verse you put on here earlier.... but we are not given a spirit of fear...!! I will be praying, praying, praying! So glad He is with you! Rest in His arms! Love, Missy in Mexico

Laura A said...

I'm praying specifically for the legion of angels, and that Jonah's interior will be strong. Am also praying for peace and wisdom for you and Matt. God will be with you!

Love,
Laura in TX

Just Me - MJ said...

Praying & praying & praying for Jonah, for you, for the doctors who will perform the scope tomorrow.

Anonymous said...

I am praying for your beautiful baby boy. Be strong!

Alex(andra) said...

So many prayers... for all of Jonah... for strength and wisdom for you and Matt... and wisdom for the doctors, among other things. Keep trucking.
love and prayers from KS.

Sara said...

Airway issues are known to happen in all forms of EB. Apx 5% those with simplex and dystrophic have airway issues. But the % is higher in all forms of Junctional. Hopefully the scope will go well and you'll have some answers soon.

Sara Jo said...

Praying that all goes well tomorrow...

Anonymous said...

I know by your eloquent postings,that you have seen the Tulips in Holland(a great comment).You are sharing the beauty you see with all of us,so that we may help to weed the garden and toil the soil.God be with you. Steady the hands of the Doctors and nurses. Your camera really takes some handsome pictures!
PRAYERS
2SHOES

Jenny said...

I am so happy that you know what you need to do- that was a big step for you, and you did it! I am praying hard for your peace. I am praying hard for Jonah to be healed. Continue to take this one step at a time and don't give in to fear. Praying in Kansas!

Leslie Baker said...

praying for your little man tomorrow and for your strength...

Susan said...

I will ABSOLUTELY be in prayer for Jonah tomorrow. You both will also remain in my prayers. I gotta tell you....the pictures that you posted yesterday are so adorable. The one of Jonah and Daddy in the chair with Jonah looking out a window (at least that's what it looks like he's doing)...well, that one really tugs at my heart.
I pray so hard that Jonah will be healed. I want so badly for him to be able to play outside, to run around in the grass barefoot, to do all the things that little boys do. I know you want all this and SO much more that I can't even imagine.
I wish there was something more I could do. I'm just one woman, who loves your sweet Jonah...and I feel helpless. I can only imagine how you feel.
My heart is heavy for you tonight as I read this post. I know you must be so discouraged.
All I can do is pray, so that is what I will do.

Much love to you both, and Sweet, sweet Jonah!

Susan in Indiana

The Q family said...

I am praying that you have peace with the decision you have made. I pray for the doctor who performs the scope to have the most getle study hands and for the scope itself to cause no damage. I pray for the coming decisions you are facing, that you have strength and peace to walk through this!
I pray you grasp and hold this promise!!!!
Isaiah 43:2
When you pass through the waters, I will be with you; And through the rivers, they shall not overflow you. When you walk through the fire, you shall not be burned, Nor shall the flame scorch you.

Yarnmomma said...

Baby Jonah is a precious child of god.. and he knows the plan for your handsome son and it is for us to believe that with all our heart.

I'm sending prayers from missouri to you and praying for positive results of tomorrows test..

Krista said...

Trust in the Lord with all your heart and lean not on your own understanding. Prov 3:5

Anonymous said...

My girls both have airway abnormalities that were first identified by a hoarse voice and stridorous cry. (One daughter was diagnosed at age 2 with an airway the size of a 2 lb infant). Although they do not have EB, they have had multiple bronchoscopies under anesthesia. I will be thinking of you tomorrow and of Jonah. It is scary.

Please keep in mind that if the procedure is done is the office, make sure they have ability to do an emergency tracheostomy, should he need one. A very real risk of bronch's are sudden airway swelling. I am also a NICU//PEDS nurse and would be happy to answer any more questions.

melanie at cozard dot net

Erin Hingley said...

Patrice, I have never hoped for nothing so much.

I want there to be nothing but positive things that come out of tomorrow.

I want you to feel nothing but God's peace and wisdom.

I want nothing but strength for sweet Jonah.

I want nothing but skill and precision for the doctors.

Most importantly, I want nothing to be found.

I am praying that Jonah's procedure will prove that he is just fine. I am praying ever so hard for your family. Thank you for bringing me in hourly contact with the Lord.

All my love,
Erin in va
youmeandwipee.blogspot.com

Kirsten: said...

Praying for you, Matt and Jonah's peace, strength and wisdom to get through tomorrow.

Lots of Love from Houston, TX

Anonymous said...

Oh well...since you said on january that you speak fluently spanish...here I go, it´s easier for me to tell you this in my language.
Estoy completamente conmovida pr el pequeño Jonah. He rezado y seguiré rezando mucho por él. Por tu fortaleza, por que tu fe se mantenga firme. Pero sobretodo, por que mañana, todo el procedimiento sea un EXITO y Jonah tendrá unaicamente "the flu".
Abrazos
Astrid desde Guatemala

Brooke said...

Many, many, many prayers being said for tomorrow!

Jenny said...

I will keep you in my thoughts and prayers.

Raising Olives said...

I'm praying, I'm praying. I weep as I think of the fear that you must be struggling against. I'm praying for God's peace and comfort for you, as well as for healing for Jonah.

"And the peace of God, which passeth all understanding, shall guard your hearts and your thoughts in Christ Jesus."

Blessings,
Kimberly

debbie said...

May the Lord's Word comfort you all. " Do not fear for I am with you, do not be dismayed, for I am your God. Yes, I will strengthen you, I will help you, I will uphold you with My righteous right hand" Isaiah 41:10 In all this, may you know He will give you strength and He will help you. He loves you all so much. Praying for you in CA. Love, Debbie

Beth said...

Patrice and Matt,
We will continue to lift you up to the Lord in prayer, especially tomorrow morning!
Since the beginning of Jonah's life you have been faced with difficult decision after difficult decision. The Lord continues to give you strength to face these obstacles and to do what is best for your child.
Have faith that tomorrow, and everyday, the Lord is holding Jonah in His hands! May you feel His prescence tomorrow!!
Praying!

Shari said...

Patrice: I am praying daily for Jonah. Many times a day in fact. I am fasting tomorrow on his behalf. You have so much to take in in such a short period of time. I can only imagine your fear. My love to you.

This IS The Fun Part! said...

Dear Patrice and Matt,

Yesterday you mentioned not being too confident in your instincts. While it's only my opinion - it sure seems to me that so far those instincts have served the Williams family quite well.

You are doing your homework. You ask questions of anyone with any experience - you talk to doctors - you read - you dig - you pray - you fight the battles necessary to take care of your entire family!

I would say that you are ALL dealing with this in the most mature and correct way possible.

Sure, in everything you ever do there are going to be doubts - and second guessing yourselves.

You can't let that deter you. Keep on the way you have been and you will all be just fine.

I just know Gabe is peeking in and doing his part, too!

Praying hard,
Grannie

Krystal said...

You're on my heart and mind, certainly. I am now praying and will be praying as I can. Hugs!

brittany said...

We're praying, Patrice. We're really praying.

amathyst-eyes said...

I'm thinking about you both and Jonah tonight, and you'll all be in my thoughts tomorrow.

Marine Momma said...

Oh Patrice...I can't imagine the worry and fear you must have right now. I'm sorry that Jonah is back in the hospital. I will be PRAYING that God guides those doctors and that everything goes very well and that Jonah just has a cold! He has proven to be SO strong so far! And you and Matt are just amazing. No wonder he picked you guys to be his mommy and daddy! :) Praying for you here in Japan!

singing mama said...

Patrice and Matt,
praying for you and your precious little Jonah (Jonut)

Luv singingmama

Claire said...

I am praying!

Cxx

Anonymous said...

Hello I have been reading your blog for a while now but have never commented. It breaks my heart to think of such a little baby suffering and to think he does not know why. I am glad to hear that he has been home and he is doing better. I will pray about the scope thing. From what I have read, Jonah seems like a little trooper. Hang in there. And remember, God will not give you more than you can handle. He promised that in the Bible.

Brandi Jordan said...

I'm praying today for your precious baby - the three of you have touched my heart in so many ways and opened my eyes to the kind of faith in God that we should all have. Jonah is a beautiful boy and a true blessing. Thank you for sharing with us and for being so honest. Much love to you and this sweet little soul.

Sus said...

Praying for strength, guidance, wisdom and love for you. Praying Jesus to guide the hands and minds of the physicians to seek answers without creating more harm for Jonah and that Jonah will not experience any discomfort. He will provide.

47%....but don't forge t the 53% on the other side of that data. :)

Martin Family said...

Not sure what time the procedure is being done, but I am praying now. I believe you made a very smart choice. I can only imagine how difficult each decision is...So I pray for God to grant you wisdom to make the right decisions for Jonah. And as for the article, it sounds as though it had some important information. But numbers are just that...numbers. They aren't Jonah. And I continue to pray for Jonah and the long, long, life we all pray he has. Sending a "e-hug".

Anonymous said...

All I want to say is that Jonah is one of the most beautiful babies I have ever seen!

jess said...

Praying so hard.

Above Rubies said...

Praying ever so hard...

Aimee Denny

Baby Bird said...

PRAYING RIGHT NOW AND THROUGHOUT THE MORNING! Remember Psalm 91...especially verse 11 which says "For he will command his angels concerning you to guard you in all your ways" - trusting in this promise today and for the rest of Jonah's LONG, LONG life!
Love, Aimee Pence

Hope said...

You don't ever have to explain to me how terrifying a stridor and hoarse cry are. Is Jonah head bobbing or having blue spells? My daughter has been very close to getting a trach put in on a few occasions. Ava was sedated for all her flexable bronchoscopies and taken to an OR. I don't know why, but her pulmonologist does them in the OR. She gets her first rigid (among other things) in May at Cincinnati CMC.

I'm praying for sweet Jonah, you and Matt. I'll be checking back to see what they find! ((Hugs))

I think you made the right decision. You know Jonah better than anyone. Always follow your insticts.

Toni :O) said...

Continuing to lift you up in prayer from Michigan and hope that your worst fears will not be confirmed. He's so beautiful...I just LOVE those huge blue eyes of his. My daughter has the same duckie (Puddles) as Jonah...too cute! Hang in there...you two are both doing an amazing job in the role called parenthood.

Jane said...

My thoughts and prayers are with you, Jonah and Matt. Patrice that was written 10 years ago and I am sure a lot more has been learned about EB babies. Also I am sure every case is different. Jonah has been a fighter so far and I pray he continues. Just put all of this in God's hands, He is the main physician and is in charge. I will be praying extra hard that all turns out well today. Keep the faith and May God Bless and keep you all.

Anonymous said...

Patrice..I know I have said this before, but I think you are making a very wise choice scoping your son. Once you know what is going on in there you will get some answers..and can start making decisions that could save your son's life..Once you get your information, you can go on to make your choices..If it's nothing, you can be at peace..if it's trouble, you may be able to help before its too late..You will be able to make your choice on whether or not you wish to intervene..Although my son was always scoped in the OR...(I didn't want to have to repeat it, or have him move and If I was doing it, I wanted the entire picture.he was older though...and children can get issues beyond the view of the scope in the office.but no matter what you choose you are off to a great start...I again think you are making a very very wise choice..My son was always scoped without complication and I am sure your son will be fine with the procedure itself..ENTs can be very talented people and he is aware of the EB so he will proceed with caution. Your son does show clinical signs that he is having airway difficulties..maybe they will turn out to be minor but you are on the right path with the scope..EB can be a very sneaky disease..you think you have it figured out and it changes..stay one step ahead with knowledge..And don't take on that negative attitude..It's easy to be devestated..I lived it too..but for your son's sake, you need to be positive and hopeful even when it seems like it's all going wrong...get sad..get mad...take care of your own emotions but do not stay in any one of them for too long..move forward...EB moms always jump in to offer advice because we know how desperate, confused and alone we all felt when our children were infants..we have all been where you are..struggling to accept EB..Follow your heart, never give up hope, and remember he was sent to YOU for a reason..Always believe he will beat the odds..Keep us posted..and email anytime..johnbeth91@aol.com
beth

Anonymous said...

I can only praise the Lord for what I know He is going to do for Jonah today.

Stephanie said...

Jesus, hold this child! Be near to his mom and dad! Rescue!!!
Do it quickly. May they see Your power and Your ability to sustain.
May He be surrounded by multitudes your heavenly hosts. Fulfill Your purposes for this sweet family.

Patty said...

Prayers are with you, Jonah, and the medical staff. God is watching over him.

Aileen said...

I've been following your story for awhile now, and have been praying for you all. I figured the best way to "help" you, aside from praying, was to let you know that we HAVE been praying for you! I'm hoping it will be an encouragement to know that there are people from all different parts of the world praying for your little guy and for you and Matt. So, know that I will be praying today for Jonah, for safety and good results, and for you and Matt, that you have peace and for strength.

From Canada, in Christ,
Aileen.

Anonymous said...

God did send you wisdome, and I know Jonah has a very special angel all his own to wathc over Him, and that angel has seen the face of God!!! God's Word says so!!! I will be praying for you all today.
Much love and many prayers,
daisy (alabama)

Ronda said...

Many, many prayers on the way for today's procedure. Will be thinking of Jonah and you all today.

Carla said...

I am praying always. God has truly placed you all on my heart as a prayer warrior for you. I will be with a group of ladies this morning and will ask them to pray, too. God bless you.

Love,
Carla in Maryland

CastingCares said...

Praying for those angels right now. I'm also praying for them to surround you and Matt.

jandkland said...

I'll echo another commenter and affirm that knowledge is power. I pray right now that Jonah is doing well during his procedure and that, when it's over, you know what step to take to care for him.

--Kelley in GA

jen lowe said...

patrice,

i was asked to pray for your family by a friend of a friend who knows someone in your family. a few days later i stumbled on your blog and realized this Jonah was the baby i had been praying for. He has completely stolen my heart. i think about him all the time and pray for his healing...and i also pray for you and your dh as you navigate your way through all of this. you have inspired me with the love and devotion you show your son. i will keep jonah in my prayers today that he does well with the procedure and that there is no damage found or done. it really sounds like you are making the best decision to do this. thank you for sharing your sweet baby boy with us...he has certainly changed me forever. ((hug))
jen
k-ville NC

Kristen said...

I'm sorry if I say what has already been said. I couldn't make it past the first comment without starting to cry my eyes out. I'm kind of a sap like that.

I pray that as you all make so many choices that the right ones will be defined with peace, even in the midst of trepidation and being scared.

We are still praying for Jonah and will not stop. Thank you so much for posting the specifics that need to be lifted up. But even if there are times that you feel you can't post, or you are weary, be assured that your brothers and sisters in Christ are still lifting you all up.

Love and Blessings to you all!

Julie said...

Praying so many prayrers...

Amber Schmidt said...

Unending prayers for Jonah, his little body, your peace of mind, wisdom for the medical staff and for our Heavenly Father's healing hand to be in your lives so strong that you will be able to look back and say ... a miracle has happened here!