zach's winter concert

Happy December

Happy December....It's been an exhausting month here..On the brighter side, Casey grew two inches..I can't believe how much he's grown.  I just hemmed all his new school clothes only to unhem them before he could ever wear most of them. With EB kids, you have to celebrate every inch and every pound.  We're still at 35 pounds though..On the other hand, We went through a rough time with Casey for two weeks.  Thanksgiving morning he woke up gagging and coughing...Another blister in his throat.  I can't put into words what it's like to wake up from a sound sleep to watch you child gagging to break a blister that has blocked off his upper airway..and then begin coughing up blood..  If we're LUCKY he coughs up the skin right away..not so lucky scenario is that it hangs in there gagging him until he finally gets it out or it comes up far enough for me to grab it and pull it free. That's right guys..it hangs out of his mouth and I yank it free. Those of you with EB kids can sympathize with me...EB is not for the weak hearted. .....Then over the next few days we listen to him stridor and struggle to breathe as his upper airway begins to swell.  We go back on nebulizers and steroids..This guys is the reason we have not removed his trach.  The episodes continue and they are impossible to manage without it. And why on Thanksgiving??  At church that week a friend said to me..."you know..god's will will never take you where his grace cannot protect you".  I just smiled.  I sat that day and really didn't hear a word of the sermon.  I am very disconnected from accepting the word and I'm not sure I have TRUE faith any longer. I go to church and I just go through the motions..Alot of times I write a grocery list or clean out my purse while the pastor speaks. And it's not the church.  It's a great church and great services.... Some people reading this will understand why I feel the way I do and others won't.  Some will pity me and some will try to 'fix' me.  Your help won't help..It's something I have to work out on my own... Anyway, we managed to have a pretty decent day believe it or not.  Casey is one amazing little boy.  I learn many of the most important life lessons from my son.  He is so beautiful inside and out.  Luckily now he is back in school and feeling well because we have a very busy December agenda and he needs to keep up!!  :)  On our list...See Santa and his reindeers (check), Pick out a tree and decorate (check), go to the two local parades (check) take pictures for christmas (check) make special ornaments with the family and kids (check) ..The only things left on our list is for the kids to shop for each other and their families ..This is the cutest part of christmas for me..when they break out their banks and shop for each other.  Casey took out his piggy yesterday to get money to get his brother a tech deck..and Zachary took out his bank to get Casey a tech deck...I just love my boys.  And this year my family beware...you get what the kids pick out this year ...  :)  I am not going to buy FOR them...And I bet you get the most precious things ...I still have an acorn and one silver earring in a pink ring box my neice gave me years ago...she's 21 and it's still special to me...

Becky is a cutie too.  She is such a girl.  She runs like a girl, smiles like a girl and loves all the girly things...Shoes, dresses and jewelry.  I encourage none of it as I'm a tomboy at heart...it's just like instinct to her..   But what is sooo cool about Becky is that she is dressed in her fancy shoes and bracelets one minute and rolling in dirt the next.  She is everything I've ever wished for in a daughter. 

We also got to go to a great winter concert Zachary was in...He plays trombone in the band.  This year he remembered to wear his suit jacket and didn't fight to take the tie off.  Last year I dropped him off with a suit jacket and tie and when he walked on stage, his jacket was off, tie was not on and his shirt was hanging out. I almost passed out when I saw him. This year he's matured alot and he looked soooooooo handsome.  (ok..maybe it wasn't EXACTLY maturing ..maybe it was the threats of violence I bestowed upon him when I dropped him off about removing the jacket this year?  :)
Well, Happy December all...Take time to enjoy all the magic of the season.

Happy Thanksgiving

Hello all and Happy Thanksgiving.  Can't belive it's that time of year already.  The holidays just creep up on me and I find myself unprepared every time.  For Thanksgiving we will be joining my family for a feast at my sister's house.  My children just love their Aunt Kelly..We always say she is their "mom away from mom"..like John and I could die tomorrow and as long as they had her they wouldn't even miss us...  :)  When we think about all we are thankful for this holiday my sister is tops on my list. I don't know what we would all do without her.  My fellow EB moms know how one family member usually steps up to help in every way possible..to learn all there is to learn... to do whatever you need them to do..who is NEVER absorbed so much in their own life that they don't have time to spend with us. .....For us that is my sister. Let's face it, we are all busy..we all have things that take our time and obligations that keep us busy and prevent us from spending time with the ones we love..but we all find time to do what we REALLY want to do...It's not like we don't HAVE the time..we just choose to use it in other ways.  I miss visiting my friends and family as much as I used to but it's just not so easy anymore.  Casey's needs consume almost my ENTIRE day. To keep him alive, healthy, coordinate his care, keep up on supplies, Dr. appointments and therapies is a full time job and leaving the house most days is just not an option when it takes two hours just to bathe him.  I thank the friends and family that understand that and am sorry for the ones who can't. 

The kids have been good.  Zach was chosen to participate in a day of trombone workshops with a trombone player from the metropolitan opera.  It was a great oportunity for him and he really loved it.  So many of our family members came out to watch him in his concert that night and for that he was so proud.  Thank you guys for making it special for him.  School has been going nicely for him this year too.  He loves the teacher.  He is such a smart boy.  I can only remember a handful of times he did not score 100 on a test.  And he got first chair in the fifth grade band..(may I brag that he is only in FOURTH grade)  :)  He really makes me proud.  Becky is growing up fast.  She is a beautiful little angel.  She doesn't give me an OUNCE of greif.  She's a special little girl with a special purpose in our family. 


Casey has been good too.  I almost hate to say it and jinx myself.  The only problem I'm seeing that bothers me right now is that one toe is turning towards the bottom of his foot and the skin is tight and pulling it down.  I've decided to unwrap them in the evening so that he can walk barefoot and sleep unwrapped and wiggle them around a bit in hopes of stretching it a bit.(I just pray his little sister doesn't step on them)...It's just one of the complications of EB..Anyone with any good suggestions, can feel free to email me..I'm not sure what to do about it..if he straightens the toe it pulls the skin and hurts..(Adele...any thoughts, you are my EB expert.....  :) At his last physical he had gained two pounds and grew two inches.  This is thanks to the great blended organic food diet his AWESOME nutritionist opened my eyes to. It has made an incredible difference in the vomiting and his energy level has doubled.  He just looks healthier and feels so much better and wound healing is so much faster with the right nutrition.. And he is loving school.  He's the class pet and I'm not surprised.  He has the personality that can light up a room.  The kids in school love him.  We are due this winter for a bronchoscope..actually overdue.  We keep procrastinating.  I don't like to fly in the winter.  We've had too many bad experiences with the weather..and the prices for airline tickets skyrocket this time of year..We've been looking at an average of 550.00 a ticket.  That's not in the budget right now..Heck, bread and milk isn't even in the budget right now.  Since Casey's been born I have never before been stretched as much physically, emotionally, financially or spiritually.  I have just been drained.  Casey has a sinus infection again..and the associated eye infections that go along with his.   We're back on antibiotics..(the joys of preschool)..before that is was a stomach virus  (the joys of preschool)  and a cold (the joys of preschool) and pink eye (the joys again of preschool)...


Well, as everyone gathers tomorrow, take a few minutes out of your day to reflect upon all you have in your lives to be thankful for. We all have more than we realize.  We are all so hurried and busy every day that we tend to  overlook our blessings  ... Make sure your family and friends   know how much they mean to you and tell your children how much you love them. 

Happy Thanksgiving to all our friends and family and special prayers to all our EB friends..May you have a peaceful holiday weekend.
Hugs,
John, Beth, Zachary, Casey and Becky

Hello..Can't believe it's almost the end of October and Halloween. We love the fall in our house..apple picking, pumpkin picking, trick or treating..thanksgiving...and cool weather for Casey! There are no other seasons that brings us so much fun. It's the time of year that Casey can get out of the house and enjoy the outdoors. We still have a few more outings on our agenda ..hope we can accomplish them all.

It is a great relief to say that everyone here is doing good. My older son got his cast of after what seemed like an eternity. The bone is still not perfectly aligned but it is finally healed. The orthopedist says as he grows it will stretch and fall perfectly back in place. So, for the next few weeks he will have a fracture brace and that's just fine with him because he can take it off to sleep and shower! He is excited because he will be playing in an all trombone concert in November. He received a perfect...yes PERFECT score on his English language Arts state exam and an Above average on his Math state test..add to that he earned a Music Acheivement Award and a perfect mid semester academic report and it makes for one proud mom!!! Now if he could only organise himself we'd be really getting somewhere.

Becky is 14 months old now. She is an absolute doll. She is the perfect baby...REALLY..I swear!
She is just a delight. She loves shoes..she wakes up and we have to immediately put her shoes on and she wears them all day..If you take them off her, she'll follow you around holding them up to you to put on again. Her speech development, as far as I am concerned (nobody else seems to be) is slow. She does say mama, dada, baba, apple, cat and ach (for her brother's name)...other than than she is just quiet. I don't know if it is the chronic ear infections and hearing difficulties she had for 6 months out of her life or the fact that being the third child gives her a disadvantage. See, she rarely has to ask for something.. The boys can both predict what she wants and cater to her. And Casey keeps me so busy I rarely have time to repeat over and over...Say "more"...by the third attempt I just throw her what she wants and go on to something else..I just don't have the time. She is running all over and enjoys EVERYTHING! She is just all smiles.

Casey has been good. He's back to school (five half days in a three year old program)and loving it..I mean LOVES it. He has a great happy outgoing personality (quite the opposite of what I predicted a child with EB would have). He is the class pet..to the teacher and most of the kids. They just love him and he gets all his therapies in school. Our district pays for preschool five days a week, the bus, the nurse and all his therapies..who could ask for anything more!! Casey has had a bad foot injury and blistered both his knees when he fell chasing his brother through a spooky corn trail and he's on antibiotics now for a sinus infection I let go for way too long. I try to keep him off antibiotics at all costs. I can count the several times in his life he's been on an antibiotic for anything. But sometimes you have to concede to the antibiotic. He is going to be a devil for halloween. He's always been fascinated with devils for some odd reason. He says he wants to be a devil with a cape and boobies...(he thinks the muscles on the chest we saw on a devil stuffed toy are boobies)..and he wants a pitch fork so he can fork the kids in his class..(doesn't sound good!) He has a great sense of humor ..I hope he keeps it. Last night was the first time he said to me "I wish I didn't have bandages"..He said it as I was wrapping him so I'm pretty sure he meant he wish he didn't have bandages because he wouldn't have to sit still so long for me. I told him I wish I didn't have "rotten hair' and my older son said " I wish I was taller"..We all wish we didn't have things about us that we do. EB is just one of the things Casey has about him he will wish he didn't ..Not as if rotten hair compares...but when you are three I don't think the things you say have as deep a meaning as we adults think they do. We are probably going to head back to cincinnati for a bronchoscope this winter. Casey has been donig well. We have added vitamin D and fish oil to his regimen. Not much else new. I have gotten so much email about my thoughts on Bone marrow transplant for Casey. I do have my opinions on it and I'd be lying if I said I didn't struggle with it every day. It's a personal decision and one that has to be made on an indivdual basis. There's alot of soul searching needed to be done to explore your personal thoughts on the process and the outcomes. Right now from my perspective, it's still a clinical trial and not a "cure". I think it has a long way to go. Parents exploring this avenue now are inspirational to me as they are paving the way for my son to one day see a cure. But, placing your child into a clinical trial for anything is risky and I am quite the chicken when it comes to risky. I've never been a risk taker. We pray long and hard for each child going through transplant and we honestely root for them and celebrate when they reach a goal. I feel very blessed that parents have shared their journey with us and allowed us into their lives through the process. The are the bravest of pioneers.

Please pray real hard for the Ringold family. They just lost their daugther Bella. She had dystrophic EB like Casey. This is one that really hit home for me and I really miss this little girl even having never met her. Her family is inspirational. Pray for strength and peace for her family and add some prayers for Baby Elle in ICU and our friend Tripp who is going though some rough EB times right now.. We think of all these children as our own. The EB community is a small one but we all care for each other's child as if they were our own. We pray for each other when times are hard and celebrate each acheivement when things are good.

Hope everyone is enjoying a peaceful start to fall.....
Beth

Hello all..It's been awhile..but not much going on her worth writing about...The boys are BACK TO SCHOOL!! This is the first year that I have actually wished my precious angels away...It's also the first year I've had to face sibling rivalry...casey fights with Zach and becky annoys casey...It's been a summer of being peacemaker and I'm ready for a break...Zach's arm continues to be a problem.. One bone is closer to being in place but it's pushed the other one slightly out now. We go back to the orthopedist on Tuesday with him. we are hoping for an all good and a shorter cast so he can bend his arm.

Becky turned one. It's always a significant milestone for me. She leaves "baby" once she turns one. I want to keep her a baby forever.

Casey's been good. A few toe injuries this week but nothing he can't handle. He's back to preschool five half days this september. I hope he has a fun year.

Happy Labor day!

Hello..It's been awhile since we've posted...I think it's just cause we have a sort of boring life...I think about updating and realize I have nothing interesting to write about..Things here have been good..Becky is set for surgery on the 19th of August..she has had ear infections one after another since December...so off for tubes we will go.. I hope we are making the right choice. Her hearing is not what it should be and her speech is not progressing...It's all scary to me....After casey you would think that nothing would scare us...but it does.. we pray as parents we are making the right choice..part of me says if we leave it alone, she'll outgrow it. The doctors say "NO". Anyway she is walking all around the house. It's so cute and in another month she'll be one year old. Where does the time go? she is truly our angel with such an important purpose in our lives that she can't even begin to imagine. she is our distraction from EB. She and casey have the greatest relationship. A few weeks ago she did hit Casey in the eye with a toy when she sat up suddenly as he was kissing her goodnight. That lead to our first corneal abrasion. Casey will tell you " beckaboo took my eye out!" Luckily with some antibiotic and lubricating drops it was healed in two days leaving it alone. We were very lucky with that one that it healed so fast. That was painful for him...In June we got to go to EB clinic in cincinnati. We hadn't been there since he was one so it was nice to catch up with everyone there. All went good...His echocardiogram and EKG was normal. Normal Dexascan (bone density).. His vitamin D on blood work came back on the low end so we are supplementing with some extra vitamin D to prevent the borderline osteopenia he has. With exercise, good nutrition and some extra vitamin D it will stop the progression...We have increased his g-tube feeds from 4 ounces to 8 ounces a feed by going up 10 mls a day. It worked like a charm...turns out we shrunk his stomach giving him such small feed while he was sick most of the winter.. we also had alot of vomiting and poor weight gain with the pediasure so we switched him to a blended food diet. the vomiting has stopped, his energy level has tripled and his wounds are healing so much faster. This is the single best thing we have ever done for him. He's gained four pounds...Yes, it's a HUGE pain to mix and figure out the nutritional requirements but it is soooo worth it. His bronchoscope of his lower airway in the office was clear...the upper airway will be done in the OR in the fall...He has some heat rashy patches on his back and neck for which we are using a new cream...we thought it was a fungal infection but cultures came back negative....other than that, his PT and OT evals were good..he has no issues with his hands or hand function...we added a new pain med to his regimen for that extreme pain he gets with some injuries. We got to meet lots of EB families while we were there and it was such good therapy to talk with moms who could understand what I go through..The kids we met were incredible...I admire every one of them...they are all my heros.
Please send some extra prayers to Daylon and Bella going through transplants right now in hopes of a cure.. they are both going through rough times and could use some extra prayer.

Casey will go back to preschool in september five half days ...and his nurse will travel with him to and from and remain with him in class. He loves school and can't wait for september. We went to meet his new teacher yesterday and I think he's going to have a great year. what a great and supportive school.....John is still working at the hospital but looking to get out ...he wants a slower paced job closer to home...I still work one day a week at the doctor's office ..It's boring work but I get out of the house and the extra money is soooooo needed...My older son continues to play the trombone..It's his happiness in life. Nine has proven to be a rough age.. I find him trying to express his independance lately and fit in with his peers a bit more....He is still such a good boy who got awards in band and a perfect report card..he makes me so proud.

Well that's it for now...I am going to try to post some pictures..I don't know how yet...but I'm trying to figure it out....
beth

Hello all.....Just a quick update..I don't know how people get on every day to update..I'm been so bad at it..Life with the three kids just keeps me sooo busy..I can't find the time...We've been falling behind on our yearly visits with Casey...so we decided last month to catch up...He had his three year old well visit..he is up to 31 pounds...what a struggle it is to keep up his weight....It's worth every moment of it though as I feel nutrition is the key to EB....We had blood drawn..all is normal with the exception of a few markers for inflammation...that's to be expected with EB. His iron had tripled from what it was last year...so all is well there..his bronchoscope was clear...esophagram, EKG and echocardiogram all good..dexascan showed mild osteopenia..No intervention required there they say...his calcium and vitamin D levels were all normal..so they say no intervention is necessary...Although deep inside I feel we should supplement with some calcium and vitamin D. I've got to look into it for doses. Anyone who has any suggestions or are using supplemental calcium or D email me ...He's been at a rotten state of breakdown the last few weeks...I think it's a combination of being wrapped like a mummy in near 90 degree heat...we just turned our air on...a new evening nurse who just can't remember not to pick him up under his arms leaving huge blisters on his underarms...moving onto underwear from his padded diapers and out of the crib into a real bed...we've had a few head scrapes from the siderails until he got used to it..finally this week he is looking good like his old self again.

Becky is still getting ear infections and we are debating tubes....we are just trying to determine now if the fluid clears up between infections or not...it's not the number of infections you get I found but rather if the fluid remains ..I've pretty much decided that if it is not affecting her speech or hearing and the fluid dries up...I'm going to wait it out till cold season and teething is over and see how it goes..her visit with the ENT showed no fluid after the last one and her hearing was perfect..casey's speech therapist evaluated her and says she is right on target...so, we'll see how it goes.

Zach is doing great..he is the most awesome kid in the universe.. his all state band performance earned him a gold ribbon..the best you can get and did I mention he was the only third grader ever to be allowed to participate..he is a great trombone player and he still gets straight 90s and 100s in his acedemics..He is sooo cool .

Well, we are off to cincinnati children's hospital for follow ups with the ENT and Surgeons there..then we'll go to dental and opthamology and a visit at the EB clinic....It's going to be a busy two days..we leave the 16th. What I learned from planning this trip is that if you fly for medical reasons you can get a free flight if you know the right person to contact. we got three VIP seats for free from Delta..Taxes and luggage included. Always remember to ask if you guys are ever traveling..I know continental does it too and not many people know about it.

well, that's about all. Tuesday I am going to a play my zach is in at school about butterflies..he is the grasshopper...I couldn't be prouder... :) Then casey and zach both have field day that week..casey's luckily is preschool and indoors..I am going to try to figure out how to post some pictures of their birthdays, etc one day....That's about it from our house...Hope everyone is enjoying a peaceful time....
beth

well, hello everyone..I have not posted since January...boy time sure flies...casey turned three this week. My Zachary 9. I don't know where the time goes..casey had strep for this birthday. The poor kid can't catch a break! The day before he had an esophagram..No strictures...well, at least we THINK there are no strictures...and if there IS, they have to be insignificant....He cooperated nicely for the first half of the test, but the second half..lying on your stomach part..he decided he was done and we couldn't go any further..he got upset and vomited barium all over his daddy. so, we think it's good..the films will be put on disc by radiology and sent to his surgeon in cincinnati to review. We have an echocardiogram scheduled this week (routine yearly) and a baseline dexascan (bone density) to get in before the end of the month. Then I believe with the exception of a dermatology and GI visit and a trip to cincinnati, we are done for the year! (EB keeps you running). Casey is now potty trained..that is during the day at least...he still wakes up wet in the morning...but we've come a long way..in return his reward was a new "big boy bed"..My baby no longer wears diapers or sleeps in a crib..I feel he's all grown up! :)
Zachary's band played at the state music festival and scored a gold rating...the best you can get..He loves his trombone and he is soo good at it. he is the youngest one to play in the band.. I am very proud of him and he aces school...all top grades..
Becky is 9 months old now. She is crawling and standing up and destroying my house and my patience..At 42, I am just too old to chase an infant. She is the most patient and content child I have ever encountered. She is happy every moment of every day. She is just beautiful..
And yesterday I turned 42..... Casey got a pool for his birthday..he loves the little pool...unwrapping and doing bandage changes afterwards is rough.. I am going to try to post some pictures later this week..as soon as I figure out how...

Hello..Just wanted to let everyone know that Casey's bronchoscope showed a clear airway again. So, once again, we are forced to revisit the same old question. Do we remove the trach or do we leave it in?? My maternal instincts to protect him say ..NO...My desire to move him forward and the realization that his trouble may have been a one time thing makes me doubt my decision. He has had a clear bronchoscope 6 times in 18 months..basically since the first episode...so, the surgeons want it out...and by the way..they are the two most fantastic men I have ever met..especially in medicine..and probably the most experienced with EB in the world..I trust them, but HEY..it's not THEIR kid if they are WRONG!! So, we will revisit it in 6 months when we are able to save enough money to fly back to Ohio... His esophagus is clear of strictures too, which is good news as well. With EB you have to take the good when you get it..sometimes it throws you a bone..and other times it just punches you in the face...The unpredictability of this disease is what scares me the most. But for now, I will enjoy the easy time ..knowing at any moment it could change. Zach is still enjoying his trombone and is now helping the younger kids in the band..He is the first third grader allowed to play with the fifth grade band. Becky is 5 months old now. She is teething like a monster. She is an angel. The kind of baby that makes you want to have 12 more. Luckily I have Casey ( who although a blessing, makes you never want to have another one!) to even out my thoughts.. Hey, I like this blogging thing..It's like therapy without the $300.00 an hour bill! Thanks everyone for your support of our family....

Well hello all. I have been trying to put this blog together for almost a year. I still have to add some links and information and fix it up a bit but for now, it's up and running! It is so hard to email everyone who asks about our son individually so at your requests, here you go..this blog's for you!! Please log onto our page and follow our journey! I have vowed to share the good, the bad and the crazy! I'll begin posting later in the week, but I wanted to let you all know I finally did it! :)