Tubes for Christmas

Have you ever had the experience of waiting so long to call someone, you are too embarrassed to even call them, but then it gets worse because you keep waiting, but then you get more embarrassed and then it's this vicious cycle? Happens to me all the time. I really, REALLY have a hard time with the phone....I know. Crazy. Just one more of my issues.

Well, that is almost how I was feeling about my blog. It's been so long since I have blogged, I didn't know how in the world I could ever catch up? I have thought of things to write, but haven't felt like I could write about what happened today without catching up on what happened a month ago. And then I realized, "Hey. This is MY blog. I can write whatever I want!" I have realized that I am never going to "catch up". To say that life has been nuts since we returned from the Shriner's Hospital would be an incredible understatement. I don't rememeber a time when we have been so busy as a family. Hopefully, though, things are going to settle down once this week is over, and we will be able to enjoy a peaceful Christmas.

A girl can dream, right?

In the meantime, I wanted to update everyone on Clara, just a little, mostly because the events of her most recent procedure were hysterically funny.

In the past year, Clara has failed three hearing tests. Each time she fails, she is put on a round of antihistamines, the fluid dries up, and she passes her test again. Well, a few months ago, Clara failed, yet another, hearing test. The tympanogram on each ear was flat, and she had little-to-no hearing in her left ear, and only about 50% in her right ear. Our first clue that something was wrong was the way she YELLED at us every time we asked her something, and we can only assume that was the reason for some "selective hearing" in instances where she was disciplined for disobeying us....just one more in a long line of Parent of the Year moments.

I digress.

The doctor felt that things were too far advanced, and that medication would not help this time. Her second round of tubes would be necessary. She had them when she was 14 months old and they worked wonders! And while the procedure itself is "no big deal", with the remaining possibility of mitochondrial disease in her body, she is at greater risk of malignant hypothermia because of anesthesia, so that was a little of a concern. Even with that, we had a complete peace about it, and really felt it was be no big deal. And it wasn't. She came through like a champ and is doing splendidly.

However, there were some hysterically funny moments at the hospital yesterday that were too good not to share. (With Clara, it's always something.) If your young children have ever had to have a procedure at the hospital, you know that they expect you to be there at 0:dark-thirty in the morning. So we were sitting in her little room, still half-way asleep, while the O.R. nurse went over her health history with us. She was running through all of the questions, and I was answering, mostly without even thinking, and she gets to this question:

"Jewelry....is she wearing any jewelry?"

But before I could even answer, she looks at Clara and says, "Clara, you don't have any nipple rings, do you?"

Ha! I nearly fell out of my chair. At first I was shocked. And then I looked at Clara, and she was completely confused. Then all I could do was laugh like a hyena. Clara doesn't even know what a nipple is...let alone a nipple ring, but at that point...we were awake!

Really....I didn't think I was going to recover, I laughed so hard.

As the nurse was finishing up, she was asking if we had any other questions about the procedure. Since three of my four children had tubes in their ears, I could not think of one thing I could possibly ask.

Clara, however, had a question.

She looked right at me and asked,  "Where did Megan have her tonsils out?"

"Right here", I replied.

"Then who was her doctor?"

"Um....Dr. Habenicht...just like you".

Clara got this really strange look on her face and said, "What?! Wait a minute! Megan told me that her doctor's name was 'Christine' and that she was a movie star!"

Again, all I could do was laugh hysterically. I have always known Megan to be a competitive little thing, but had not considered that she would want the best "hospital" story, too. Little nut.

These are the moments that get us through this crazy life. You have to laugh, because the alternative is not acceptable.

All set, and ready to go!

Headed to the O. R.

This was the view from the hospital window. Very pretty.

Otherwise, things at the Bryant house are okay. We had our first real snow yesterday, but thankfully, it is melting already. We have a really crazy week this week, to be finished off with our Children's Ministry's Christmas Musical at church, but we are looking forward to spending a Christmas at home. Don't get me wrong. I will miss our family terribly. I am, however, looking forward to having a chance to make some family memories and traditions that do not involve running all over creation until we are run ragged, and having some moments of family worship to remember why it is we celebrate Christmas. But more on that later....

I pray your day is blessed!! Much love to you all!

Jeannie

Final Follow-Up from Our Visit With the Shriners

"Grateful" is an understatement for the way I feel about our visit to the Shriners Hospital for Children. Overall, I can't say enough good things about the care Clara received there and the experience we had with all of the doctors who are a part of her care team. The fact that they even work as a team, to me, is nothing short of marvelous. As ready as I was to go home on Friday, Clara was still not ready to leave. I think that is a huge testament to the environment they have created for the children. Even with a lot of the "yuck" she had to experience, the "fun and wonderful" way outweighed the yuck, and she loved it there.

I am in awe of the way God provided for us. I am so thankful for family who helped provide what we needed to be able to go. I am thankful for the prayers, notes, cards, dinners, gifts, and words of encouragement that were given along the way. I am beyond thankful for my friend, Elvie, who got me connected with her endocrinologist who got us connected with The Shriners Hospital. I am thankful for the new friends Clara and I both made at the hospital, and for a group of people who can really relate to our experience with bone disease. I am beyond thankful for the angel God sent our way to help us through the nightmare of TSA at the St. Louis airport. O'Hare was great. St. Louis....not so much. When all was said and done, we had Clara's adaptive stroller, and six, yes six, of those little gray bins full of stuff on that conveyor belt. (Why we had to fold up that contraption of a stroller and put it on the belt is beyond me. At O'Hare they just took it to the side and scanned it. MUCH easier.)

We left St. Louis with lots of great news and information, and a peace that I have not felt in, at least, five-and-a-half years. Peace in knowing we are finally in the right hands. Before we left, they changed the dosage of medication one last time, even since the last time I posted about it. They will be monitoring her via of blood and urine every three months until we return next September. If anything is ever "off", they will contact us immediately and deal with the issue. They didn't tell me this until we were about to leave, but the geneticist said that he was so afraid her kidneys had already started calcifying because of the large dose of medication she was on. Everyone was so relieved when her renal ultrasound came back normal. They are big believers in treating the kids over the whole course of their growing years, as opposed to treating them really quickly, so there is not so much damage later in life. Honestly, and I told the doctors this on the first day, I had been beating myself up because it was so hard to get all six of her doses in everyday. Now I'm glad that I wasn't always able to accomplish all six, and they are, too. They encouraged me and affirmed me in so many areas, because as a Mom, I always question, "Did I make the right decision?", and "Have I done all I can do?". They certainly felt we had seen all the best doctors and done all we could do based on the information we had been given up to that point.

The dietitian met with us on the last day, and while they were thrilled at the variety of foods Clara enjoys, they did put some limitations on her diet because of the way some foods interact with the phosphorous she will now be taking four times a day (instead of six....YAH-HOOOOO!). Her medication also contains sodium, so we have to limit that, too, which will benefit everyone. Thankfully we eat very little processed food, so it will be easy for me to control how much salt is being added to things.

The orthopedic doctors felt really good about her legs. She does have some significant weakness on her left side, but with lots of work, that can be helped. They recommended some exercises to do at home, in addition to some sports that might help her. They also fitted her for, and made, some orthotics and told us to get some really good tennis shoes for her. They believe that her alignment (or lack thereof) is what is causing most of her pain. Because her little legs are bowed, she puts a lot of pressure on the outside edge of her foot, which affects her knees, hips, and all the way up her body.

The geneticist feels that her muscle biopsy report does indicate that she has a mitochondrial disease. However, he, and everyone else, are puzzled as to why she looks so good. He has a theory about the way her cells have divided, yada yada, that was actually the best theory I have heard up to this point, but it is lengthy and I don't really want to type it all here. (If you really want to know, you can ask me.) Whatever the theory, I believe we have a great, big God who is still the Great Physician and can use whatever method He wants to heal her little body. I am thankful for answered prayer and that she looks so healthy!

I am extremely thankful that, for the first time in a long time, I had a doctor look me in the eyes and tell me that he expected Clara to live a long, healthy life. It will not be without pain and some trials, but whose is? There is no way to know if she will need hip or knee replacement in the future, but they felt very confident that she probably would not. I do grieve some for her future children, but I have to let that go and grieve when it's time. I am excited to move forward and just enjoy this precious little girl, and the other three precious girls, that God has blessed us with! The doctors have assured me that the treatment they have her on is state of the art, so we will continue to give that to her and do all we can to help her. Beyond that, we have seen God's hand so often in her little life that we know, have absolute faith, that He will continue to hold her.

I think that just about covers it. Hopefully I haven't left out anything. Just ask me if you have questions. I am so thankful for everyone who has taken the time to keep up with Clara via our blog, and there are no words to express my thankfulness for your prayers! We felt them, and they were answered!

Just for fun, there were a few pictures I didn't share from St. Louis. Enjoy. :-)

 Just about to leave for the airport.

 We saw a Delorean on the way to the airport. The license plate cracked me up.

The Arts & Crafts room at the Haven House had hundreds of paper cranes hanging from the ceiling. It made me think of Leslie.

This is the hospital gown Clara and I made together for her Bitty Twin. She was so proud of it!

We were at the Haven House for about 12 hours. In that time, Clara managed to make a friend. (She has a way of doing that.) She made her a card in the Arts & Crafts room, and the next morning, Ms. Lori left this note on the survey she slid under our door. I thought it was so sweet. :-)

One of the incredible aquariums at St. Louis Children's Hospital.

 She travels lightly. :-)

On Wednesday they had Pet Therapy at Shriners. Clara looked forward to that ALL WEEK! On Wednesday morning, the first thing she said was, "Mom! Today is 'Pet Physical Therapy' Day!" :-) I told her that she could bet her hat on it. :-)

 Such a nice dog.

She got to walk this one all over the second floor with another little girl who was in a wheel chair. This dog's name is Faith, which is Clara's middle name. I thought that was sort of neat.

Dr. Gottesman, her geneticist at Shriners.

Balloons and a bear from Papa and Grandma!

Ms. Beatriz, one of her dietitians.

Bingo night!

Ms. Valerie, Clara's nurse...her very own. :-) She was one special lady!

Clara and Shawn had to say, "Good-bye". :-( Such a sweet little guy.

Much  Love,

Jeannie

Day 4....Some Fun Things

I know we all handle stress differently, and for me, humor is usually my go-to coping mechanism. I can remember the day of the wedding of my best friend in high school. We were in the bridal room before it was time to go down the aisle, and things were way too tense. Without even thinking about it, I just started cracking jokes. That tends to be how I handle every tense situation, which is fine when I am around other people like me, but it really just annoys most people, I think.

Regardless, as we have progressed through this week, there are some things I have found hilarious.

For one, this is the paper I had to fill out about our immediate and extended family on Tuesday:

 I guess they know we're Dawgs?

 

 

This was one of the questions on the two page worksheet (Check out # 4):

Notice that I didn't fill in any blanks before I took a picture. You'll never know what it said when I was done. :-)

 

Another interesting thing: Dr. Gottesman, the very serious (and brilliant) geneticist that Clara has been seeing here rides a unicycle. Maybe that doesn't sound that funny, but I thought only clowns rode unicycles. He didn't find that funny. Although, most people in the north don't think things are as funny as I do....maybe that means I need to be a little more serious. It could be bad to think things are too funny, I guess.

 

The hat rack has been the most hilarious...but you know all about that already.

 

Something fun that happened today: The parents of one of my closest high school friends lives here in St. Louis! They came by the hospital today to meet Clara and to take me out to lunch. It was SO GOOD to see them! I had fun catching up on their family, but also, her Dad worked with my Dad at Eastern Airlines as a mechanic, so it was fun to talk "aviation" for a while. (Even though it made me miss my Deddy.) I have always loved them...such sweet people.

 

Mr. and Mrs. Hargreaves (and me...)

 

 

A little something sweet: Clara has become really attached to the little boy acros the hall from us. We have enjoyed getting to know his family this week. She is going to be sad to say "good-bye" to him.

 

A new skill: Clara is learning how to swallow pills. The tablet form of her meds is more accurate AND more convenient. We were mixing a packet of K-Phos with 3 ouces of water 6 times a day (in theory) to get in all of her medication. Now, we can hand her the tablets 5 times a day. MUCH easier. The best part for her: They gave her mini M&Ms to practice with. :-)

There are fewer updates today on Clara's medical issues. The orthopedic doctors came by to see Clara during their rounds today. The attending presented her case, and the head orthopedic doctor released officially declared that, from a surgical stand-point, she looks good. That's the official word. Not that her legs are perfect, by any stretch, but they are not serious enough right now to warrant surgery and they believe that after staying on her treatment through her growing years, her legs will be nice and straight by the time she is a teenager. Works for me!

Clara was also fit for orthotics today (that the Shriners will pay for...woo-hoo!), and we will probably have them before we leave. The only thing we have to do when we get home is get her a really good pair of tennis shoes. They aren't too crazy about her flip-flops or her Chucks. It was good while it lasted. :-)

I just took a break from typing because the rheumatologist came in to evaluate her. Guess what she said? "Clara needs a really good pair of tennis shoes. No more flip-flops." (sigh) It looks like we'll be shopping when we get back. This whole shoe/orthotics issue has always been a frustration to me . One doctor swears by them whole-heartedly. Another doctor says that it really doesn't make a difference. Some doctors say the orthotics do her more harm than good. But as of this week, I have decided that whatever they say at the Shriners Hospital is what we will do. Case closed. One more stressful decision checked off of my list.

They have also recommended some very specific exercises for her, in addition to some sports that would be really good to build up the specific muscle groups she needs to work on. I can't even think about all of that right now. I need at least two nights in my own bed first. :-)

Tomorrow will be the last time we meet with all of the doctors at one time before next year. After that, we will be discharged and will fly home. There is a very good chance I will not find time to blog, but I promise to update all the final results of everything once we are home.

As I leave here, I can't help but feel such deep thankfulness to the people who make this place possible. I don't have enough good words in my vocabulary to express how wonderful it is here for children like Clara who have been on a medical roller coaster their entire lives. This place gives hope. I have met some children with some of the worst deformities I have ever seen, yet they inspire me with their joyful hearts and positive attitudes. I look at them and think that most of us really don't know what it is to suffer, yet they do it without complaining. They have figured out a way to live their lives as fully as they can. I have also walked the halls here, thanking every Shriner I have seen...I think I have thrown a few of them off guard. I just can't help it. I am so thankful. There is a wall on the way to the cafeteria, that I pass at least 6 times a day, that is full of pictures of past and present Shriners. I pray for those men and their families, thanking the Lord for using them to make the lives of these children better. I pray for their families that support them and the time they spend fund-raising to fund the work that is done here. Obviously, I don't know any of their spiritual beliefs, but I pray that they all know Jesus.

I will never look at the red velvet hats the same again. Anytime I see those hats, if there is anything in my pocket, something will be going into that hat.

Please pray for us tomorrow as we meet with the doctors, that if there are any questions I need to ask, God will bring those to mind. Also, please pray that we will have safe travels. I can't wait to see Jeff and my other three girls tomorrow night! I have missed them!! And for the first time all week, I don't think anyone is going to mess with Clara's sleep...THANK THE LORD! She has been a GRUMPY MONKEY today!

Praying your night is wonderful!

Much Love,
Jeannie

 

Day Three...Information Overload

Hi Everyone!

Today has been a big day with lots of information, and I am eager to share it all. Before I do, I wanted to clarify something from yesterday. When I said something happened that made me angry, it had nothing to do with Clara's medical team. Her doctors and her nurses have been wonderful. Even the urine fiasco didn't really make me angry. I was bummed that we missed an hour of sleep for nothing, and that her testing was messed up, but somehow I was able to pretty much overlook that. It wasn't her main nurse involved, and I know nurses have a lot on them. Granted, the helpers should be a little more thorough, and I'm not even sure the two that messed up her urine were actual nurses, and ultimately, we were not directly affected. The episode to which I was referring was one I don't wish to re-hash. I will just say that it involved some very inconsiderate people and Clara was in no way involved...she was oblivious. Ultimately I just keep praying for a humble heart and a gentle spirit so that none of the other even matters. A good night's sleep probably wouldn't hurt either....

As for today...we met with  Clara's team of specialists around lunchtime. (Yes, Clara's food was messed with, once again.) :-) Overall, they say Clara looks very good and healthy. That is a big praise! Her renal ultrasound came out perfect...no calcium deposits. Woo-hoo!! Her x-rays look good...bones look much better than they did compared to previous x-rays. They are reducing her dosage of her medications just a little, for the simple reason that Dr. Whyte likes to operate on the safe side of things than have to deal with other consequences from too much medication later. Furthermore, they are changing our method. So instead of having to mix a powder with water 6 times per day, she will now take a capsule or tablet 4 1/2 times per day. That just feels much more manageable to me. When a kid is only awake for twelve hours a day, it is hard to get in 6 doeses of medication. This team of doctors will now be in charge of Clara's care, which means, no more trips to the U of M, but one big trip to the Shriners Hospital every year. We will also have to have blood drawn and a urine specimen taken to be mailed to Shriners every three months. The hard part is going to be getting the lab on board. Basically, they don't want the lab to do any testing. They have the lab draw the specimens, spin, freeze, and whatever else needs to be done to them, and then mail them here to this hospital where they will do the testing.

Clara will have hypophosphatemic rickets for the rest of her life. There is a 50/50 chance that her children will get it, too. She will only be dealing with the consequences of it in her own body until about a year after menses, though, and who knows? Maybe the research we have taken part in here at Shriners this week will help her future children if they, too, deal with the same disease. We are still unsure where Clara's came from. They will be drawing blood from me tomorrow to see if I am a carrier, and they said that they may want to test her sisters, as well. There is also one more genetic test (about the only one she hasn't had) to know, once and for all, if hers is a sporadic case, or if it was handed down to her.

The long term affects are also unknown at this point. Up until now, you were almost guaranteed a knee and/or hip replacement in your adult life if you had hypophosphatemic rickets. However, there has been no study done, yet, that follows up with the adults who have been treated the way Clara has. This Shriners hospital is the only facility anywhere that studies XLH the way it is being studied here. The study has been running for almost 30 years, and they are just about to start trying to follow up with the people they treated 30 years ago. They want to know if the outcome is the same if you were treated for the rickets, versus never having had any treatment. I think the only thing stopping them at this point is funding. The only thing they will say for Clara's future is that she will be short, but not a dwarf. Everything else is unknown at this time.

This is Clara, right before going in to meet with the doctors.

It does feel like there is some finality finally coming to our search for help for Clara. This has been 5 1/2 long years of doctors, hospitals, and tests. We don't have all the answers, but I think we almost have as many as we are going to get...I think I will just have to live with that. Everyday, I am feeling a little more peace about the whole situation. The biggest question that still remains is, "What about the mitochondrial disease?" That is a question to which we may never have an answer, at least until more research is done and more genes are sequenced. Right now they have Clara's testing classified as "inconclusive", which I think will line up with the way Dr. Kendall, her geneticist in Atlanta, has felt all along. The geneticist told us this morning that what is there, is a mild case. That is a good thing.

As far as Clara's pain is concerned, they don't have any real answers except, "That's just rickets." They do want us to meet with the rheumatologist here to possibly figure out some ways to help her. The biggest thing that doesn't make sense to me is: If her rickets is getting better, why is her pain level increasing? I asked that question, and they don't know.

At this point, my brain hurts. I feel like I have been holding onto this information for so long, and I am tired. Maybe with this new information and new care team for Clara, I can just let it go for a while. Some of our sweet Michigan friends have been sending the sweetest notes this week, (They timed it so we would get one every day...isn't that precious?) and today, the one Clara received had a place to list all the things that make her happy. One of her answers was, "Having a hospital that takes good care of me." I think one of the reasons she has been such a sport about the 24 hour urine tests and the special diet is because she understands that the doctors here need this information to help her legs not hurt. She has basically said that to me in so many words. This girl just takes my breath away. Such a jewel.

Some of you have asked how Jeff and the girls are doing. I haven't been able to talk to him much, but they seem to be doing really well. When I talked to Jeff this afternoon after the meeting, he sounded as tired as I always feel, but I think he's done a great job with the girls. Their school work is getting done, they're eating (They've had a little help from friends in that department....so thankful), and they all seem pretty happy. If you think about it, please pray for him tomorrow, as he will be taking the girls to co-op by himself. Last week was my first week and I was toast when I got home. I think we will all be glad to get back together. Clara told me today that she is missing her sisters. Two more days!

I would also like to thank everyone for your notes and messages via e-mail and Facebook. I am reading them all....and love them!! I just haven't had the time to respond to them all. Please know I am getting them, though. They have been such an encouragement to me. They help me not feel quite so alone in this great, big place. :-)

There was something really funny I wanted to share with you, but since this post is already so long, I'll wait until tomorrow. I am not sure what is on tap for tomorrow, aside from my fasting blood test in the morning, but Friday will be another very busy day of meeting with doctors and heading home!

 

And just for fun, some pictures of Clara in the RT room...her favorite place!

 

She has lived at the air hockey table!

 

She has thoroughly enjoyed the crafts, as well.

 

 



Thank you for your love and prayers.

Much Love,
Jeannie

Day 2....Urine Good Hands, Baby.

This girl of mine is pretty laid back and easy going, as long as you don’t mess with her food or her sleep.

 This morning, they messed with both.

 This is what her morning was supposed to look like:

5:00 am-Urine Sample

6:00 am-Blood work

7:00 am- Urine sample

8:00 am- Blood Work

9:00 am- Breakfast

 

What her morning actually looked like:

5:00 am- Failed attempt at urine sample and a meltdown because she felt like she did something wrong

5:05 am- (after the nurse left the room)- My angry 6 year-old with her thumb in her mouth said to me, “Mom, I thought they were going to wake me up in the morning!” (They really should install artificial sunshine on the outside of the windows with a switch the nurses can flip before they wake up the kids.)

5:15 am- another failed attempt at a urine sample, Clara is given a cup of water to drink (at 0-dark-thirty….YUCK!)

5:40 am- One more failed attempt

6:00 am- 2^nd nurse comes in; Clara succeeds!..But nurse forgets to put the hat in the toilet that was supposed to catch urine. Ooops!

6:02 am- Clara is now wide awake, dancing around the room,  and ready to play board games. I am ready to fall back into a coma.

7:00 am- Blood work

8:00 am- Another successful attempt at a urine collection, and Clara is about to STARVE TO DEATH...at least she thought so. "Mom, normally I get to eat as soon as I wake up." It's a gift my children have always possessed....being able to eat the second their feet hit the floor, unlike their mother who needs at least three hours and a cup of coffee to wake up before eating.

In light of the before-mentioned state of her stomach, the second blood draw was canceled, as it had to be done before eating, and she couldn't eat for an hour afterwards...after one more urine sample...which would have put her breakfast at 10:00 am. I was a little bummed, because I really wanted them to be able to have as much information as possible, but at the same time, I was thankful for their sensitivity to her situation. She is at the borderline age for needing that second sample, so this year, she gets away with it. :-)

Once Clara had eaten breakfast and the RT room was open, Clara was chomping-at-the-bit to go and play. That was my opportunity for a shower....a much needed shower. I left her in the RT room and an hour later, I was a new woman. However, I was not able to bask in the bliss for long. I took Clara back to her room, and in a round-about way, found out that Clara had been to the potty while in the RT room.

The urine saga continues...

They are collecting her urine every single time she potties this week, 24 hours a day. (Once she goes, we hang the hat up on a rack in the bathroom. Who knew there were racks for that?) It seems that the volunteer in the RT room took her back to her room to potty and handed her over to a nurse...a different nurse than the original one who lost her urine....and that nurse took Clara into her potty and

forgot.

to.
put.
the.
hat.
on.
the.
toilet. (sigh)

Because I am Southern Baptist, and somewhere deep in my bloodline there is a gene that makes me convinced that everything is my fault, my stomach was in knots. Hopefully their research on her is not totally blown...but either way, what was I supposed to do? I was in the shower!

I have been reminded many times today that as a Christ-follower, I am never released from the Great Commission...the whole world is my mission field. I don't get to take a break just because I don't feel like it. There were a few times today I wanted to lose my cool, to be a real jerk. Thankfully, by the power of Christ in me (because there is not enough power in me to keep me from blowing my top a few times today), I was able to bite my tongue, smile, and be loving to those around me. As a result, I was given the neatest opportunity today to get to know another precious mother here at the hospital. The burden she bears with her daughter seems almost unbearable....proof that God gives grace when grace is needed. She is a believer and is not wavering in her faith, but life is just hard for her right now. I talked to her for a long time in the RT room, and it so happened that we ate lunch in the cafeteria together. I think she really needed to talk.....and I really needed to listen. When lunch was over, we went to the parent lounge and prayed together. It was such a sweet time....a gift from the Lord..that I know I would have missed out on had a been a jerk to the person I wanted to be a jerk to just a few minutes earlier....and I have a new friend, to boot!

Clara also had PT today, which was unexpected, but fun! The therapist here gave me some great ideas for exercises to strengthen some of her weakest muscles, and ways to make it fun. I am always amazed at the creativity of Physical Therapists. I am also amazed at their hearts. I haven't met one yet who doesn't love the children she works with.

Clara and Ms. Donna, the physical therapist.

After lunch today, we took our trip to the St. Louis Children's Hospital. That place is fantastic! It is ranked #5 in the nation, and after just a few minutes there, I knew why. Clara made instant friends with Ms. Gloria, who checked us in, and gained a few more goodies for her loot. I really think I should have brought an extra suitcase for all of the gifts she's been given this week. She went to the hospital with her backpack containing a teddy bear and her toy laptop. She left that hospital with two new stuffed animals, a new pair of gloves, and six new princess stickers. Spoiled. Rotten. The ultrasound went great..took less than ten minutes. We won't know the results until tomorrow.

Leaving for St. Louis Children's

Clara with Ms. Gloria, our new friend at St. Louis Children's.

Tomorrow is a really big day. At some point right after lunch, every single person that has taken part in Clara's team (geneticist, nurses, lab techs, radiologist, physical therapists, etc.) will be meeting together to discuss Clara's case. We were told that they would talk for an hour or an hour-and-a-half and then call us in to ask us both questions. Please pray that my mind will be clear....clearer than it was the other day when I bumbled like an idiot to the geneticist, and that Clara will not be nervous, but will speak up when spoken to. After tomorrow, we might have some more answers, but most of our answers will likely come on Friday before we leave.

I do know a few things for right now.

1) They feel nearly 100% sure she can avoid the surgery on her legs...at least until she is a teenager. Her geneticist thinks her legs will straighten right up and she will never need the surgery. Praise the Lord!!

2)According to her x-rays, she definitely still has hypophosphatemic rickets.

As for the rest, I will post as I know for sure.

Overall, this is still a really productive trip. I am so thankful to be here! I must say, though, that getting a little taste of the outside world to go to the other hospital today made me dread coming back to our little room. Clara couldn't wait to get back, though....she is happy. Therefore, I will find my contentment. :-) For this week, it seems that "Home is where we hang our hat". :-) (You get bonus points if you get that one.)

Thank you again for your love and prayers. Your messages have been so encouraging!

Much Love,

Jeannie

 

Day One, Half-Way Done!

Hi Everyone!

I just want to start by thanking you all for your prayers, encouragement, and concern for Clara. I am amazed at how smoothly everything has gone so far. God has answered prayers in a huge way!

As nervous as I was about making it through the airport yesterday, it went remarkably well. We hit a ton of traffic in Chicago, but thankfully we left our house a little earlier than we thought we needed to, so it worked out fine. When we arrived at the airport, they had a little fella named Justin help me through security and to the gate with Clara. He was able to take us through a special gate at security, and he helped me get everything onto the conveyor belt that goes through the x-ray machine, so security was a dream! No line and I didn't have to juggle everything on my own. Huge blessing. I don't know what they pay him, but I am sure it isn't enough. :-)

We arrived at the gate about 45 minutes early. I had these grand visions of Clara drawing or playing on her toy laptop so I could read. NOT! She was far too excited for that! She watched planes and people and planes and people, and before we knew it, they were calling us to load! The flight was smooth (and fast...as soon as we were up, we were headed down), and I would give anything if I could have gotten pictures of her sweet, little excited face. She had a hundred questions, but it was so fun to see something that has become very normal to me through the eyes of a six year-old little girl who has never flown before, and to experience the wonder all over again!

Waiting to board.

 

On the plane...waiting for take-off!

I have always had a soft spot for old men, and when I saw the Shriner that came to the airport to take us to the Haven House, I just want to scoop him up and take him home to be very own little old man! He was precious! His name was Bill and he has been a Shriner for over 25 years. I asked him to tell me his favorite part of being a Shriner. He said, "This is!". He said that they have a hard time getting some of the younger guys to be a part of the escort system, but he said, "I don't want to tell them how great it is, because that might cut into my time." So sweet. He had his wife, Linda, in the van, so I really enjoyed talking to them and getting to know all about their family and their involvement with the Shriners. More than anything, it was so great to finally be able to say, "thank you" to an actual person.

Clara with Mr. Bill, her very own Shriner. :-)

We arrived at the Haven House (similar to a Ronald McDonald House) around 5:00, ate dinner (that was provided by volunteers), and for the first time in days, we were able to relax. Clara went to the craft room for a while and a large playroom that is there, and I just enjoyed sitting back and watching her. I know every Mom feels this way about her children, but every once in a while, it is so nice to sit back and marvel at how beautiful she is. I think she is truly one of God's most magnificent creations....all four of my girls. Just love 'em!

Modeling Day #1 of new PJs for Clara and her baby, Katie, from Grandma Jackie. If a girl's got to be at the hospital, she might as well be cute, after all. :-)

We rode a shuttle from the Haven House to the Shriners hospital this morning, and... aside from everyone having to get off the first van and get into a bigger one because all of mine and Clara's stuff wouldn't fit (None of the other people were living at the hospital all week), and hitting terrible traffic and thinking I was close to death a few times when our driver was racing forward and looking behind him...we arrived safe and sound. :-)

This is Clara with Ms. Valerie, her nurse.



All checked into my room!!

This has been a big day. We first met with the dietitian, who is responsible for making sure Clara's diet this week is nutritionally close to what she eats at home. There was, then, a ton of paperwork/releases to be signed, then we spent at least an hour, if not an hour and a half with the geneticist looking after Clara this week. Remarkably, Clara has her own geneticist and nurse this week. There are usually two children per doctor and nurse, with a total of four children in the research center per week, but this week, there are only two children, so we get our very own. :-) It was a very casual conversation. He sat back and crossed hi legs and we just talked....talked about Clara, her history, and a myriad of other things.

This afternoon, Clara went to radiology for a bazillion x-rays and a bone density scan. I am always amazed, as are the staff, at how well she does with these tests. That is a blessing in the midst of this, because if she didn't do the testing well, I think I would be in the loony bin. Tomorrow will be two blood draws and they will collect urine for the next three days to get an accurate picture of what her body is doing.

Beyond that, we wait. Wait for test results. Wait for the team of doctors to collaborate. And then wait some more. I was given a lot of information this morning by the geneticist, but until things are final, I will not share. Some of it was speculation about what "might' be, based on what they can see for now, and some of it was not significant in light of what we already know. What I can say is that she is not nearly the most severe case they have seen, and they all marvel at how healthy she looks. So thankful for that!

There is one concern for which I would like to ask for prayer....I know my prayer warrior friends like specifics. :-) There is a possibility that she has been on a higher dose of her ricket medication than what she needs and as a result, she could have some calcium in her kidneys, which they can do nothing about. We go to another local hospital tomorrow for Clara to have a renal ultrasound tomorrow, and I am praying that her kidneys are still healthy.

Wednesday, we meet with the whole team of doctors/technicians/nurses/staff on Clara's case. They said there would be about fourteen of them, and then Clara and me. Sounds intimidating. That is the day we will meet Dr. Whyte, the one we came to see, for the first time, and I need to be prepared with questions. If you can think of something I need to ask that I may forget to ask, feel free to share. :-) I'll take all the help I can get at this point. :-) On Friday, we will meet with Dr. Whyte once again so he can fill us in on her treatment plan.

Beyond that, they said they will want us to do x-rays and blood work every three months from home, and we will come back here once a year until after puberty.

I will give more information as I know it. I can say, with certainty, that I finally think we are in the right hands. Dealing with XLH is like breathing to the people here...they have been doing it for thirty years. Ms. Valerie, Clara's nurse, has been here for twenty-seven years! I find that amazing. It really does seem to be an amazing place. Maybe we are finally stepping off of our merry-go-round.

I am very hard to reach by phone right now, but I am frequently checking texts, Facebook, and e-mails as I am able. I love getitng messages! Please keep them coming!!

I love you all and greatly appreciate your prayers!

Much Love!
Jeannie

Last Thoughts Before We Go

Suddenly, I want to change my mind...to stay home with my family.

Suddenly, I just want to say, "Never mind! I was just kidding! I don't really want to go!"

But I can't. I know this is what is best for my sweet girl.

Suddenly, for the first time in quite a while, I am angry. Angry that Clara has to suffer. Angry that the spirit that is inside my little girl is held back by the limitations of her physical body. And selfishly, that the only family vacations we seem to have revolve around a hospital visit or doctor's appointment. Would it be too much to ask to have a normal vacation like normal families? You know...like...to go to the beach? Or an amusement park? Or somewhere fun?

But that is not the life we have been given for now, and somehow in the midst of all of this, we have to focus on One. The One who created us....created our beautiful girl....and trust that He knows best. Trust that, somehow, this whole situation will serve to sanctify us...especially Clara.

Tonight, as I am finishing up packing, I have had a few minutes to stop for the first time all week, and that's when these sort of thought start taking over. Realizing how much I am giong to miss Jeff and my other three girls, and how long a week really is when you're trapped inside a hospital with a precious, and very talkative, six year-old. The realization that Clara is going to suffer....some more...albeit for a good cause.

My hope is still that we will get answers and a treatment plan that will work to get her our of pain and get her body growing normally. Ultimately, my hope is in the Lord. I know He never makes mistakes. He has allowed this, and He has also provided everything we need to take care of Clara.

I am especially thankful for our family and friends we love so much who have helped us, prayed for us, and loved us. Thank you from the bottom of my heart. Please continue to pray for us this week...Clara and me at the hospital, and Jeff and the girls at home. Thankfully, Clara is mostly excited. She is excited about flying in an airplane, and after a shopping trip with Grandma Jackie, she is well-equipped with cute pajamas for herself AND her Bitty Twin. She and I also worked together to sew a hospital gown for Katie (her baby), complete with a Shriner's Hospital logo iron-on....and those are the things that were packed first. Ya know...a girl has to have her priorities. :-)

I am hoping to update my blog each day...maybe even with pictures (if I can figure out how to do that on a different computer.). We have some sweet friends at church who have loaned us their laptop, so it should be much easier than having to leave Clara to go to the computer room.

Thanks again for everyone who has played a part in this trip. We love you so very much.

Much Love,
Jeannie

Round and Round...

That title pretty much describes our life right now! We are busy, busy wrapping up the summer (boo-hoo...where did it go?), getting ready for a new school year, and getting ready for our big trip to St. Louis.

There have been moments recently where I have really believed I am losing my mind....or what little is left, at least. I keep forgetting things and have trouble focusing on the task at hand. Friday morning, I woke up with the mission of tracing a pattern for a dress. That's it. However, what actually happened was that the pattern to that dress jumped on the merry-go-round that is my brain right now. I laid the whole thing out on the table and then remembered that I needed tissue paper on which to trace the pattern. I went to the basement to get the tissue paper and on the way back, saw the cucumbers from the garden that I had not yet pickled. So I pulled out all of my canning stuff, and as the pickling mixture was heating up, I noticed the basil on the counter that had been harvested a day earlier and still not been made into pesto to freeze. So I ran back down the basement to get the food processor, and on the way up, the lid slung off of the food processor and busted. So I went into the dining room to get super glue to glue the lid back together, and noticed the pattern still laying on the dining room table, still waiting to be traced onto tissue paper.

See what I mean?

As crazy as I feel, it is all for good reasons. I wouldn't trade schooling my children for anything, and it is such a gift from God that Clara is going to be seen at the Shriner's Hospital. It is just the preparation that is sucking the life out of my brain. :-)

"What does a dress pattern have to do with Shriner's Hospital preparations?", you may ask.

As we get closer to the date (less than two weeks!), I have gotten past all the major stuff (medical paperwork, food log of everything Clara ate for a whole week, medical records from every doctor she has ever seen, plane tickets, accommodations, etc.) and I am now trying to plan for the minor stuff, like activities for her to do on the plane and in the hospital, how many bags we can check, how to get a car seat to St. Louis, how in the world to push her in her chair and drag two carry-on bags behind me through the airport, and "What in the world am I going to do for five days while she in the hospital?" That's where the pattern comes in. I have always dreamed of my two little girls having matching smocked bishop dresses for Easter. Our budget has never allowed for me to buy them in a boutique, so I decided I would make them...and sitting in a hospital room for five days should give me plenty of time. Besides, I just can't see myself doing "nothing" for five entire days. That would NOT be good for my brain....nor would I want to inflict the punishment of my boredom on those who will be sharing the hospital room with us. (My family can attest to this.) So I now have the dresses assembled, pleated, and ready to smock.....and 5 jars of pickles and two ice trays full of frozen pesto. :-)

There have been many questions about what Clara will be doing when she is at the hospital. The main reason for her visit this time is testing. They will have Clara on a diet very similar to what she eats at home, thus the reason for the food log I mentioned before. This allows them to see exactly what she is taking in and how many phosphates and other nutrients are being absorbed into her blood and what is being lost on the way out. They will monitor this by frequent blood draws and urine specimens. The type of rickets she has is not the dietary Vitamin D deficiency type, where simply increasing the intake of Vitamin D fixes things. It is the genetic type that makes her resistant to Vitamin D and phosphates. These are the two nutrients that work with calcium to make your bones strong. Clara's body, however, wastes phosphates, which is what makes her bones weak, her legs bow, and her first few baby teeth not form completely...among other things. Furthermore, she and I both had to stop all vitamins and supplements of any type this past Sunday. They want a clear picture of what is going on in her body, but they may also take some blood samples from me to do some genetic testing. This is primarily a research facility, and they want all the facts!

Additionally, she will likely undergo a bone biopsy (not looking forward to that), and she will have an orthopedic consult while we are there, as well. I feel sure they will be very thorough, but I think we will still be left with a good bit of time on our hands. We'll see. :-) In the end, however, we should have a good picture of what is going on in Clara's little body, in addition to leaving the hospital on an effective treatment plan. She will have blood work done every three months at home once she returns and they will see her back in St. Louis in a year....and every year after that until she is 18 years old. There is no doubt in my mind that we have found the expert in this field and I am so excited to see what he has to say. You never want something to be wrong with your child, but if there is something wrong, there is great peace in knowing God has put you in the right hands to discover exactly what you're dealing with.

I will try to blog every day while we are in St. Louis to keep everyone updated. I will be taking a friend's laptop with me. Even though the  paperwork we got said "no electronic devices", the nurse made it sound like they are flexible with this rule. I already have to leave the room to eat (I am not allowed to eat with Clara because of her very strict diet), so I don't know that I will feel good about leaving her alone again to go to the computer lab if, for some reason, they do not not allow me to use the laptop. I will do the best I can, though. Hopefully it will be a non-issue.

If you want more information about the hospital, here is the link.

Also, in case you were wondering, she can have Mylar balloons, but no latex. If you send anything else (and I am not asking you to....just saying "if"), please keep in mind that we are traveling with one checked bag and two carry-on bags, so we don't have a lot of space. She is also prohibited from having any tobacco products, so please refrain from sending cigarettes until we are home. :-P

So there it is....all the information you ever wanted about our trip to St. Louis (and then some), and a little tour through my crazy, twisted brain. :-) Aren't you glad you don't live there?

If we come to mind, please pray that:

1) Clara stays healthy between now and then. She had to go off of her Mito cocktail and we have found that she is so much healthier and stronger while on it. It's only for a few weeks....hopefully it won't leave her system that fast.

2) Travel goes smoothly...and packing, too, for that matter. I am so afraid I am going to forget something important.

3) Jeff and the other three girls are safe at home and that all goes smoothly. I don't worry about leaving the girls with Jeff-he is a great Daddy. I just know it can be a lot to juggle, especially if you're not used to it.

4) This is Jeff's busiest time of the year at the ministry. He is working very hard to get everything done before we leave. Please pray things will go smoothly there between now and then so he can be home that week with a peaceful mind and will be able to just enjoy the girls.

5) Clara will have "the peace that passes all understanding". She has mentioned "being nervous" in the last few days. We are praying she will just be able to look forward to the good parts...being on an airplane for the first time ever, spending a week with Mommy, etc.

5) Most of all.....we will get answers once and for all, and the right treatment plan for our sweet girl.

I hope your day is great!

Much Love!
Jeannie

Paper Cranes

Hi Friends!

I have quite a few updates I want to post, but tonight, someone else is very heavy on my heart. If you have followed my blog for a while, you may remember my post about the first mitochondrial disease/FOD symposium I ever attended. While there, not only did I meet Clara's current geneticist, whom we love dearly, but there was another girl there with her mom who made quite an impact on me. She was a lovely young lady who was stricken all of a sudden, at the age of nineteen, while working at a camp for disabled children. Her name is Leslie. A few years have passed since I have seen her and her Mom, but I have kept up via Facebook, and she is declining very quickly.

Leslie's family has been working very hard to help her accomplish everything on her bucket list. She was granted a wish last week to meet Reba Mcentire, but the trip was very hard on her and she will no longer be able to travel. However, there were many items on her list that require no travel at all. One of those items is that Leslie wants to fold 1000 paper cranes. My girls and I have folded a few in the last few days and will be mailing them soon. I thought if you had a few minutes and a square piece of paper, you may want to help, too! i will admit that ours are not extrememy lovely (it was a little tricky for me and beyond tricky for my two littles), but now that I have done a few it is getting easier, and it's kind of fun, too!! Here is the website if you're interested:

1000 Paper Cranes for Leslie

I hope you are all doing well! I'll be back soon. :-)

Much Love,
Jeannie

Alyssa and the Mama Bear

I am so thankful for everyone who has asked for an update on Alyssa. The good news is, she is is doing GREAT!

Now she is, anyway.

In case you don't know, Life Action Ministries is just that : ministries....multiple. One of the largest parts of the ministry is the Life Action Retreat and Conference Center, where, for eight weeks every summer (among many, many other activities during the year), the Camp is hopping with 25-30 families every week learning what God has to say about having a godly family, putting your spouse first, loving that spouse completely by first, keeping God the Lord of your life, raising children who love the Lord, and certainly, having a TON OF FUN!!! We went in 2004 and I can honestly say that it was THE BEST week of our lives!

With all this wonderful-ness, they are able to keep their prices VERY reasonable because the camp is staffed by Life Action Staff families, who, like us, live on the support of ministry partners, and dozens of teenagers who volunteer their summer to work in various capacities. So far, Alyssa has been in the kitchen for her first two weeks, and this week, she is working in the Snack Shop, scooping ice cream and selling Cokes, T-Shirts and various other Camp souvenirs.

I am so glad things are good for her now, but her first week at camp was, um....rocky, to say the least. Going to a week-long camp with your church group, ie., friends you've known forever, is totally different than being dropped off in a place, albeit familiar, in a cabin with 19 other girls you have never met. I will refrain from sharing too many details, so as not to embarrass my very-easy-to-embarrass 14 year-old, but let's just say that there were a few moments I thought we were going to have to go and get her. Actually, if I'm being honest, everything inside of this Mama Bear wanted to go get her....to rescue her....but I knew that was my heart speaking, and the heart is, after all, deceitful above all things (Jeremiah 17:9). So I switched from "heart-mode" to "brain-mode" and realized a few things:

       1. She made a committment and she needs to follow through.
       2. I knew it would not be hard forever, and once she got through the "hard", she would would reap benefits that would last her a life time.
       3.  She could NOT be in a better place. She is with people I trust COMPLETELY for her physical,     mental, and spiritual well-being. Not to mention, she is sitting throught through two services a day, taught by some of the best Christian speakers on the face of the earth, in addition to break-out sessions for the CREW.
       4. As much as I wanted her home and to end her suffering, I knew it would end up being a bit of a disaster. Alyssa cannot stand to sit around and "do nothing". While there are certainly plenty of things to be done around the house, I am not much a of a "cruise director", and with our current budget, we are not going very many places beyond the "Y", the library and the park (all less than a mile away) right now. The combination of a bored teenager + three other children in the house is not a good mix. It actually equals bored-teeneager-tormenting-three-other-children-and-her-mother.

SO.....with all of that "head knowledge", I was able to hold myself back, and I am so glad I did. She came home the first Saturday for her sisters' birthday party and we had to take her back that Sunday night, and that was almost as traumatic, if not more-so, than the first drop off. However, we went to visit her play at the beach at the Life Action Camp with the other three girls the following Tuesday, and things took a dramatic turn. I had just finished telling one of my other Life Action friends, who is camp staff and has multiplle children working this summer at the Camp, how hard it had been for Alyssa and how I was looking forward to having her home for a week. In the amount of time it took for me to complete that sentence, take a breath, and turn to notice that Alyssa had just plopped down beside me on the sand, Alyssa immediately says,

"So, Mom. I have a question. What would you think about me asking Mr. A.J. if I can work next week, too?"

"Um.....excuse me? What did you say? Did you just say, 'work another week??'"

Keep in mind that her original schedule was to work for two weeks, come home for a week, and then work another two weeks. This would mean FIVE STRAIGHT WEEKS IN A ROW! Not to mention, she will be away for another week during the week immediately following her last week at the Camp to go on a church mission trip.

My mind was racing a mile-a-minute, and the battle between "level-headed Mom with all the right answers", and "I MISS MY GIRL AND WANT HER HOME Mama" was raging furiously in my head. Meantime, my friend that I was just talking to was laughing hysterically, and I was sitting there looking like a complete liar! (You know that is why we have children, right? To keep us humble.)

My first (selfish) response was, "ABSOLUTELY NOT!". I wanted to look right at Alyssa and say, "But WAIT!! You're miserable, remember? And you desperately want your Mama....remember?". However, I referred back to my brain logic (see previous bullet points), and after Jeff and I talked it over and prayed about it that night, we let her do it. I am not so naive to think there was not other motivation, like 'her best friend was coming to Camp to work that week', but either way, she is there.....and she is HAPPY!! As a result, so am I. :-) She is making a lot of new friends and she is serving others, something she loves to do!

The girls and I had to get Alyssa yesterday to take her to the dentist (she had a tooth that kept breaking off in pieces), and since she was in the chair all of six minutes (doesn't take long to pull a baby tooth that has a permanent tooth underneath it that has already grown in half-way....doesn't this seem like the kind of thing a responsible teenager would mention to her mother?), we had plenty of time to go back to the Camp and hang out a while. I can't tell you how wonderful it was to have all four of my girls together again. I took a deep breath for the first time in a while.

 Together!

 Do you think these two were happy to see each other?

 Megan loves to climb this thing....a thousand times!

 Doesn't she look great? 

(Still hates having her picture taken.)

 Sweet Sisters!

 There's something I really love about this one. 

They were sitting on the dock waiting for fish to "give their feet kisses".

These last two were taken as we were about to leave. They had to run in and go potty "one more time", and as they were walking out of the Lodge, they struck these poses for me. :-)

Blessings to you and yours!

Jeannie