Prayers for Janie

I just saw on the WAFB website that Janie Distefano was found dead. Please continue prayers for her and her family and friends. My heart goes out to them all.

Big Eater

Continuing to be mysterious...Lydia downed a whole 25 oz. yesterday...eating 4 oz at almost every feeding!!! I won't try to figure her out...I'll just be glad that she's had such an appetite.

We had great fun yesterday hanging out with the Devillier bunch! Thanks for inviting us...the girls and I had a great time!

For those that don't read the comments posted by others...please read this from Allison Kirkland and please keep "Janie" in your prayers:
Could you please ask all the spiritual people who read your blog to add a special prayer for Janaver (Janie) Distefano who has been missing for one day. SHe is a very close friend of our family and we hope they find her safe! She has been missing since 12 noon in Marksville, LA. She is on the news on channel 9. This has hit too close to home. I have been knowing her all of my life! She is one of the sweetest ladies you could ever know. When I was growing up, she was just a sweet person and cared about my sister and I so much. She has no children. THANKS! - Any prayers would help! Sometimes we don't understand why things happen.. This is definetly a time! Love, Allison Kirkland

Once Again...

I knew that God would lead us to a decision about the feeding tube..I believe he has. Beginning last night, Lydia is starting her decline in appetite again. I have still mostly been able to get her to put the food down, but she is trying to refuse. This time, there is no excuse...no reason to explain her decrease in appetite. I feel solid about the decision to get the button (g-tube). (For now anyway:) All of the practical things involved in caring for the tube will come later...I'll learn all that I need to learn about it, but it will not influence my decision to get it or not. If she needs it, we going to get it. Plans are still the same, though...pharyngogram 4/9...follow-up with Dr Alberty 4/11...then appt with surgeon.

A little reflection:
I've received much praise from family and friends about how I am dealing with all of this...it is very hard for me to accept those kinds of compliments..or any. But, I can kind of relate to what you're thinking. In the past, I remember looking at parents of children with special needs and seeing them as sort of "set apart" from the rest...saintly, even Godly people. Now that I have children of my own with special needs, my thoughts have changed...we're all just regular people. We just lean on God to give us the tools we need to face the challenges that lie before us...and He always comes through...it is easy to see His blessings on a daily basis. My lowest times are probably when I think back to my pregnancy with Lydia and how I just imagined life with her to be so much easier than with Elise...no worries about if and how fast she would develop...just smooth sailing. I now know, of course, that that is not the case. Then I sometimes look at other people with multiple children (like shows on TV: Jon and Kate + 8...and Kids by the Dozen) and I think...how can people have sooo many children and all of them be perfectly healthy? When I start to have these kinds of feelings, I just have to lean on God and remind myself what I know to be true: He chose us for Lydia and Elise and Audrey. He saw something in us that we may not have seen in ourselves. We may not know now His masterful plan, but we have faith that it will be revealed to us...and I pray that we will be all that He has planned for us to be. I am so thankful for your continued prayers for me and our family. I have felt God's loving hand upon us through many of our challenges...I see changes in myself from just maybe a few weeks ago...less overwhelmed less overburdened...just one minute at a time...I attribute a lot of that to your prayers for me. Thank you!

Dr Appt Today

We saw Dr Wood this morning for our surgery follow-up and for Elise's ear infection follow-up. Both went well...ears are fine...incision is fine. Dr Wood immediately said how good it looked as soon as he removed the bandage...good thing...I was a little surprised by it. I'm not sure what I was expecting, but it made me feel bad for Lydia all over again when I saw it. It looks a lot longer and more traumatic than what I expected...I knew it was about 1 1/2 inches, but I'm not a very good estimator...so it looked really long to me. I guess, like Martin said, her leg is so short that 1 1/2 inches is just about her whole thigh...so it is like looking at about 8-10 inches of our thigh. Anyway, I was glad that Dr Wood thought it looked good. He told us to treat it with neosporin for a few days and keep a butterfly bandage on it...Audrey said, "or we could put a heart bandage.." Such innocence! :) We've had another good day today...Lydia taking at least 3 oz at every feeding...spoon feeding pretty well too. I talked to Dr Wood this morning about our possibility of a feeding tube. He said, "Well we knew from the beginning that that would probably be inevitable." I was a little surprised at that...I never knew that it would be inevitable...but it is looking that way now anyway. According to her chart at Dr Wood's office, she gained 4 oz in the past 24 days...considering that Dr Alberty wants her to gain 1/2 oz per day, she is considerably below the mark. He agreed that we don't need to rush into it right now since she is eating well...but, if and when she has another dip, we should go ahead and do it. A part of me just feels like all of her dips have been explainable...reflux, weaning from ACTH, recovering from procedure...but, I guess if every problem that she has causes her appetite to decrease...and we expect multiple problems, it doesn't matter if there is a reason for the lack of appetite...we still need her to eat. I think they are most concerned with these first 2 years or so of her life...to ensure maximum brain growth and development. He said that we can watch for the next 2-3 weeks to see how she does to know for sure that she will continue to eat well. I think that is a good plan.

Have a great weekend!

Girls' Day In

It's been a fun and laid back day...Martin is away on a fishing trip until tomorrow. The girls and I had a good day together. We all went to therapy this morning...Lydia saw Susan (PT)...Elise saw Laura (OT)... and Audrey spent some time with Terry's daughter...it worked out great! Susan saw some good things with Lydia today, though she was on the verge of sleep the whole time. Susan says that Lydia just shuts down...she's not really tired...what a smart little cookie she is! I always find it uplifting to go to therapy with my girls...I was telling Susan today...I find that therapists are so positive, and doctors are so negative. I guess it's just the nature of their jobs, but I usually leave therapy visits feeling proud of what my girls are doing, and I usually leave dr visits feeling a little sad about what they are not doing.

Lydia has continued to be a little piglet today...yeah! You won't believe this....she took 7 oz before bed last night...that's right....I said 7! Today she has taken a minimum of 3 oz at every feeding...she even did a little better with her spoon feeding today. Thank you all for the wisdom and practical advice that you've shared about the feeding tube decision...I believe as you have told me...God will be our guide...He will lead us to the right decision...and lucky for us, God doesn't make mistakes.

I am just feeling so blessed today! We really had some good bonding time today...Audrey is really into singing and what I call- liturgical dancing lately. It is a real treat. She really gets into it. We all, including Lydia, love to watch her sing and perform. Audrey is also helping lately to calm Elise's "terrible two" fits. I know her frustration is heightened by her speech difficulties, but sometimes it's hard to remain patient. Of course, sometimes Audrey also induces some of those fits...that's just how it goes with sisters, I guess.

I feel like I'm rambling...as if I haven't done that before. :) Thank you God for all the blessings in my life and God bless you all who are connected to us through your love and prayers for Lydia and our family.

My little piglet has had a great day today...eating well...good moods...lots of fun!

Here is the Easter Bunny picture that I promised to share:

Latest Feeding News

Lydia had her VCUG yesterday...went fine. Probably a couple of days before results, but the physician's assistant said that she didn't see anything alarming...we pray she is correct.

I talked to Dr Alberty yesterday...told him that we were in much need of guidance regarding the issue of the feeding tube. Gave him the facts: Lydia lost 2 1/2 oz last week...gained 1 ounce the next two days. He agreed that we probably shouldn't continue to explain away Lydia's variability in feeding. He recommended that we go ahead and get the G tube...it would relieve any problems getting her to eat on a regular basis...she would still continue to eat as usual...we would just suplement what she doesn't eat by using the tube. He also explained that it can be permanent, but it doesn't have to be. If she gets a g-tube, then starts to eat well on her own for 6 mos. or so, we can just take it out. It was obvious that it was weighing heavily on me, I think...he said, "Y'all really don't need to be agonizing over this...kids get G-tubes all the time...it's not like we're removing a limb..." I sort of laughed...but, he did acknowledge that there is an emotional issue involved too. He thought it was more of acknowledging that "there is really something wrong with my child if I have to do this." I explained that we've had that understanding for quite some time...it's not that. I just don't want to put her through something (and all of us) that may end up being unnecessary.

But....during our conversation, we also talked about Lydia's reflux...he asked if we felt that was resolved...I told him that I don't think that her feeding problems are related to reflux anymore.. He said that either the Prevacid is working...or maybe that she has just outgrown the reflux (which tends to happen in most typical kids around Lydia's age). I told him about her gagging and gasping episodes that she does sometimes when she eats but mostly when she takes medicine. I also told him about an email I had gotten from a mother of a child with Rett syndrome & mitochondrial dysfunction...who talked about the coordination of nose breathing being a problem. The problem of nose breathing coordination is that a person can suck and breathe through their nose at the same time with no problem....swallowing and breathing through the nose is a different story...it can not be done simultaneously....it has to be alternated. That presents a problem for some kids with mito issues. I've started to think that this may be Lydia's problem when she gags and gasps. That lead him to think that we need to repeat the pharyngogram (swallow study). We did one when Lydia was about 6-8 weeks old...results were fine, but we haven't done one since. The plan is this: pharyngogram scheduled for April 9th...follow-up with Dr Alberty April 11th with a plan to schedule an appt with a surgeon for the G tube.

In the meantime.....Lydia took 5 oz last night, then slept all night for the first time since her biopsy...she has taken at least 3 oz at every feeding so far today....4 oz at her last feeding. I guess she plans to keep us guessing!! These feeding habits lead me to believe that maybe she doesn't really need a feeding tube, but I've seen her appetite pick up like this before...then decline again. At least we'll have a few weeks to observe her feeding before actually inserting the tube...that may lend some pertinent information...to let us know that we are making the right decision or to let us know that we need to change our minds again. I'll continue to lean on the Lord to guide us to the right decision and to ask Him to be with Lydia through it all.

HAPPY EASTER!

We had a great family weekend together...just as I hoped it would be...much fun with the Comeaux's and the Poche's...and a little alone time for "just us."

The decision about the feeding tube is still up in the air, though Martin and I are leaning towards getting it. I will call to talk to Dr Alberty about it tomorrow. Thank you for your wisdom Missy and Teresa...it really helps. I am trying to hand this decision over to God...and I feel a huge weight lifted from me already. If I just let God lead us to the right decision, I know it will be easy.

Feeding:
Still varying...ate pretty well today. Weight tonight was 13 lbs. 10 oz. (1 oz gain since Friday) That is good news, but we still don't know if it promises continued feeding success.

Fever:
Has subsided in the last couple of days...hooray!!

Celebrating Elise:
She is starting to make words with more than one vowel sound in them...a new accomplishment! Examples: Mommy, baby, Daddy (Before, she would drop the final vowel sound.) Also...she actually made a full sentence yesterday: she wanted something (I can't remember what??) and I asked her to get her daddy to do it for her...she said, "Mommy do it." She is really communicating well. :) Martin made the comment last week that he sees a real difference in her since starting at BR Speech and Hearing...that solidifies that decision even more. :)

Sweet Audrey:
At Grandma's yesterday, they were picking "wishing flowers" and making wishes...she said, "I wish that no one will come to my house except Mommy and Daddy." I think that was her 4-year-old way of saying that she doesn't want us to leave again and that she's glad that we are home. Tonight, we were visiting with Papa, Ms Tonita, and Granny...she was giving lots of hugs and kisses...she said it was because at night in her bed, she fills her heart with all those hugs and kisses to give away. How sweet! Her daddy asked if Jesus helped her to fill her heart, she said, "yes."

How grateful we are for all of the blessings in our lives! Happy, blessed Easter!

Fun day with the girls

It was so much fun catching up with our girls today! We had a lounging morning and afternoon...went to the mall this evening for pictures with the Easter bunny, playtime, and supper. We had a ball! The picture came out so cute...I may try to scan it soon...we were so proud of Elise...she is usually skittish with the Easter Bunny, Santa, etc. She smiled proudly tonight and enjoyed it. They all looked really cute!

Thanks so much to Allison Kirkland for our sweet surprise this afternoon...a cookie bouquet delivered for our family...we all enjoyed! :)

Call from Dr Alberty (GI):
I forgot to mention the call we got from Dr Alberty at the airport yesterday...he called us back because his nurse said that the next available appt was April 7...I didn't want to wait that long before talking to him. Side note: His nurse told me that he was really busy yesterday...scopes all day...2 emergency procedures to tend to...she said not to expect his call until Monday...I was very impressed that he called me back so quickly. Lydia is so lucky to be treated so special by all her doctors...on second thought...they must just all know how special she is! (I'm just a tad biased.)
Anyway...we talked about Dr Schoffner's suggestion for considering a feeding tube and the need to be aware of her daily caloric intake. Dr Alberty seemed to side with Dr Wood on this issue..."the proof is in the pudding" (as Dr Wood would say) As long as she is gaining weight, we know that she is getting what she needs. We knew that at her last appt with Dr Wood, she had gained 4 oz in a week. Dr Alberty said that sufficient weight gain to him is 1/2 oz per day...so, that seemed right on track. I told him that I was still concerned because she hasn't been eating well the last few days...we both agreed that some of that may be contributed to the procedure...the ordeal might make her not want to eat. Today, I have a new attitude...she is still not eating well again...taking less than 3 oz at every feeding, sometimes less than 2 oz. Dr Alberty also said that he wants to know right away about the results of the testing...he said that if she has a significant mitochondrial disorder, it is nothing to play around with. He would recommend inserting a G-tube right away...even if no feeding problems exist at that time...we would be proactive because of what would be to come. So....Martin and I talked tonight...we are ready to do it now. I think it would relieve much of the stress about whether she is getting enough nutrition or not. I will probably call Dr Alberty on Monday to get the ball rolling. Making difficult decisions is the hardest part of being a parent. You know that you are ultimately responsible for making sure that your children are taken care of and that everything is done in their best interest...sometimes it is just hard to know what their best interest is. On one hand, I want to get the feeding tube to be sure that she is getting enough nutrition...on the other hand, I don't want to rush into it if she really doesn't need it. That is why I've been on the fence about it since her last hospital stay. I was convinced then that the feeding problems were going to be temporary...I am no longer convinced of that...though I'm also not convinced that it's not temporary....you see why it's so hard??

Call from the Pediatric Surgery Center:
A nurse from the surgery center where Lydia's procedure was done on Wed called today to see how she was doing. I told her about a fever that Lydia had developed again last night...101.5 She said that if it went away, I was off the hook...but if she still had a fever this afternoon, I should call the surgeon...and then probably our pediatrician. She said that it was a long time after the surgery to be having a fever...she said to talk to the surgeon first, but it was probably a medical issue unrelated to the surgery...she thought maybe even dehydration since she hasn't been eating well. But, she has been wetting diapers. Anyway, her temp went down the next time I checked..so, I didn't make any calls. Again, we'll be watching her closely to be sure that it doesn't rise again. Tonight, it was 99.5

I don't think I ever posted about Lydia's VCUG procedure...it is scheduled for Monday. It is the test that Dr Ramey ordered because of the UTI...babies don't normally have urinary tract infections, so they ordered two tests to be sure that all of her urinary anatomy is in tact. They did an ultrasound in the hospital, and it was normal. For the VCUG on Monday, they will inject a dye into her bladder with a catheter, and take repeated x-rays as she urinates. I'm not sure when those results will be ready....I'll ask on Monday.

Ronald McDonald House:
We were really pleased with our stay at the RMH in Atlanta. The fee for our stay was $20 per day...for room and board, all meals, and transportation... for all of that, we owed a mere $60 for 3 nights and 4 days!! We gave a little extra because we felt it was worth it. The next time you are looking for a charity to contribute to, I ask you to consider the RMH... it is really a great cause. A lot of the families there had children staying in the hospital down the street...cancer patients, etc. Some kids with brain injury were there...they are transported to and from the hospital for rehab each day. There were so many volunteers coming and going the entire time. It's just a great atmosphere.

We're home!!

Thanks to all who made our homecoming so nice...clean trucks, mowed lawn, clean house, washed clothes, etc, etc...thank you all!! Martin and I have had so much fun with our two big girls tonight...we missed them a lot...and they were both sooo sweet tonight. I was so relieved and excited to find that they were glad to see us...(during Lydia's last hospital stay, Elise reacted with anger when she visited at the hospital after she hadn't seen us in a couple of days...that was hard on my heart.) It's great to be home!!!

Lydia had another big snooze on the plane ride home. I think she likes flying. :) Her fever went up a little again tonight...101.6 We'll continue to keep a close eye on her...the surgeons in Atlanta don't seem to be concerned. I called them tonight to also check on some bleeding from her incision site...not sure if it was normal or not. The dr said that it is probably just from the skin and because she's been moving around. I actually have noticed that she is moving that leg less...it must really be bothering her. :( The dr told me to put additional gauze for pressure on top of the original dressing to help stop the bleeding. He said that if the whole pad becomes saturated with blood, I should take the bandage off and apply pressure for 10-15 minutes before redressing it....I would probably opt to take her to the dr if that happens. I would trust what they do more than myself.

We received our notes from Dr Schoffner about Lydia's testing...I'll try to pass it along...you'll see why the issues are so complex and difficult to understand. I'll try to email it to those whom I have email addresses for....please pass along to others interested in reading. If you have info about how to upload it to this blog...pass that along to me too. I tried a little to figure it out...gave up.

Fever went down

We kept a close eye on Lydia last night, and we were relieved that her fever continued to go down. Last time we checked, it was 99.6 She has been a little cranky last night and today so far...she has the right! We went for her RMR this morning...Resting Metabolic Rate. It is a procedure that tests the amount of calories her body needs to sustain energy at a rest. I was able to hold her, and they put a little canopy (dome) over her body for 18 minutes. She cried the whole time...I think her little belly was hungry because she has not been eating well. She had to fast for 2 hours before the test at 10:00, so her last feeding was at 7:30...she wouldn't take more than 1 ounce. After the RMR, they also did an EKG...only took 5 minutes, and I was able to feed her during the test. She took almost all 3 oz of that bottle. I am hoping to get an appt with Dr Alberty (GI) soon so that we can discuss her decline in eating habits again...I left a message yesterday. Hopefully, it is only a result of all the procedures that she has had done and that her appetite will return to normal again soon. We plan to head to the airport in about an hour...can't wait to see our other two girls! Aunt T called a little while ago...Elise has an ear infection...always something! I look forward to a restful and fun Easter weekend!

Fever

After my last post, I went back into the room...Lydia was crying so I made her a bottle to feed her. When I was holding her, I noticed that she felt warm...her fever got up to 103.5 (even on Tylenol with codeine). We called the surgeon because they told us to call if her fever got above 101. They told us just to wait it out, and call back if it was still high around 8:00 tonight...and to take her to the hospital if it got as high as 104. Thankfully, it has come down since then. We gave her a new dose of Tylenol with codeine at about 5:40...and her temp was 101.4 We'll be watching her closely tonight. She also seems to be in some pain from the incision on her leg...we can tell by the way she moves it and has a little moaning cry. Please keep up your prayers for her tonight...that her fever subsides and that she can feel comfortable. Thanks!

Surgery went well!

Lydia had a muscle biopsy, skin biopsy, spinal tap, blood and urine tests this morning. We arrived at 7:00, and we were pleasantly surprised that the procedure had been moved up to 8:00. We are so proud of our little trooper! The doctor warned that she might be extremely cranky and irritable after the anesthesia....well, not our little Lydia! We had to stay 2 hours after the procedure for them to monitor her temperature, oxygen saturation, blood pressure and heart rate...she was sacked out the entire time! In fact, we tried to get her to eat before we left, and we only got her to take about 1/4 ounce...she was not ready to wake up. She continued to sleep on our way to the pharmacy to fill her Tylenol with codeine...we even ate in a little cafe in the hospital next to the pharmacy...didn't phase her one bit. I told her that I was going to get worried if she slept any longer than that....she was listening...she woke up as soon as we pulled back into the Ronald McDonald House. She ate 4 oz...then played happily...she's probably excited that Mommy's not allowed to do any therapy with her today...the nurse said to allow her to rest...no strenuous exercise...she deserves a break! Now she is back at rest. They told us that the incision on her thigh is about 1 1/2 inches long...so she has a 2 inch bandage over it. We were told to keep the bandage on and dry until we see Dr Wood for a follow-up in 7-10 days...we scheduled an appt with him for next Friday. Lydia also has a bandaid over her spinal tap puncture spot and a bandage over the IV spot on her foot. Dr Schoffner ordered for them to administer the anesthesia in an IV because one of the disorders that he is testing for causes many complications with anesthesia through a gas mask...since we don't know yet if she has this type of problem, they take those precautions. The nurse felt sure that she probably doesn't have that complication because she has had general anesthesia with the gas mask twice before...thank God!! That could have really caused problems for her when she had her MRI and EGD, and the dr's who performed them probably wouldn't have known why...Thank God for small miracles, too!

I was relieved to find out that we will be able to complete her RMR tomorrow morning...I can stop beating myself up about forgetting that. I was also relieved to find out that she only has to fast for 2 hours instead of 12 like the paperwork stated. That is scheduled for 10:00 tomorrow morning. Our plane leaves at 4:30 to come back home.

A few more things from her exam yesterday....(It is so hard to remember everything...it comes back to me in phases):
-Her visual tracking is significantly delayed (which we already sort of knew)...Dr Schoffner said that it is not a matter of interest...we sort of thought that she prefers to look at faces and people instead of rattles and toys...not true according to Dr Schoffner. He said that babies do not differentiate what they do or do not like to look at. They just follow lights and objects as a reflex. She did not track at all for him yesterday....although we have seen her do it a little more than that.
-Weight-bearing: I was under the impression that Lydia did tolerate some weight while she is held up on her feet. Dr Schoffner found that she was locking her knees to hold her weight...when he unlocked her knees, she was not able to bear any weight at all. In fact, he said that if he wasn't holding her so tightly around her chest, she would have slipped right through his fingers.
-I asked if he thought her tone seemed low...most dr's and therapists have said "no"....more like the low end of normal. He said that she has axial hypotonia (sp?) From what I understand, it means that her core muscles have low tone...her limbs are pretty stiff. Maybe some therapists reading this can elaborate or correct me....Like I've said before, it's all pretty confusing to me...I'm just a lowly mommy! :)
-Dr Schoffner questioned Lydia's caloric intake...I know exactly how many ounces she takes in each day, but I do not have an accurate measure of the amount of calories she takes in. He suggested that I always keep track of that, and that it should be an ongoing conversation between me and Lydia's pediatrician and GI doctor. I plan to call the nutritionist that we saw at the Lake to see if she can help me to figure it out...it is tricky because we add powderized oatmeal to her bottles and she doesn't always finish all of the baby food and cereal by spoon. Hopefully, I can get a good account for keeping up with it so that I can open the discussion with her dr's. I am trying to schedule an appt with Dr Alberty for next week. Dr Schoffner suggests that we should be in discussion about a feeding tube (G-tube, which is surgically inserted into the stomach). He says that children with mitochondrial disorders often have intermittent feeding problems, and her caloric intake is crucial at this age...so a feeding tube would eliminate some of the worries that she is getting enough nutrition...we shouldn't be satisfied with her getting the minimum. He said that he was not suggesting that we run out and get a G-tube tomorrow, he just wants us to be in discussion about it with our dr's, so that we could make an informed decision about whether or not it is necessary for Lydia.

Thank you again for all of your continued prayers, support, and encouragement! Lydia is doing well!!

Appt with Dr Schoffner

Thanks Tiffanie for posting an update to let everyone know that we made it here safely...and all is well. There is a wireless Internet network here at the Ronald McDonald House, but I can't seem to get a strong signal. I am typing now on a computer that is available for guests. It is nice here...snacks anytime....supper delivered every night...we have a room of our own and we share a bathroom with one other family. It has worked out so far. We got in so late last night... we all got to bed around 12:00, and we all slept until 8:30!?! I couldn't believe it! I guess Lydia likes Atlanta. :)

Through all of the chaos, somehow I missed an appointment we were supposed to have for this morning for an RMR (resting metabolic rate). I feel awful about it...we are trying to reschedule it for Thursday morning...Lydia has to fast for 12 hours prior, so Thurs morning would really be the only time that we could do it. If we can't schedule it, they said we would schedule to do it when we come for our next follow-up visit with Dr Schoffner...we will probably continue to follow-up with him at different intervals of Lydia's life.

Our appt with Dr Schoffner:
We had our consultation with him this morning. He was very nice and patient. We got lots of information...but mostly lots of questions that won't be answered until after the test results come in. Lydia will have a muscle biopsy, spinal tap, urine test, and blood test tomorrow starting at 10:00. We have to be there at 7:00 am to prep.

Some of the info we got today....
Lydia's case does not sound uncommon to Dr Schoffner...he said he has seen a "gazillion" like her...it's funny, since she has been such a mystery to all of her other doctors. But, in order to get a diagnosis, he must perform the tests tomorrow...then he will spend hours in the lab growing cells, testing, etc. We expect results in 8-12 weeks. He will then send a report to us and Dr Superneau and Dr Hollman. We will follow-up with them to discuss it, and then probably follow-up with Dr Schoffner to discuss the results and management of her condition. He confirmed that she does not have a "curable" disorder. There is nothing that can be done to make her brain grow the way it should...to escape some type of mental retardation. All we can do is discuss management of the condition to optimize her quality of life...medications may be possible depending on the diagnosis.

He said that the main symptoms that are alarming are her significant developmental delay and her lack of head growth....the seizure activity also lends more info. He talked about the increase in lactic acid and pyruvic acid...but, he said that more of the marker to him was her increase in alenine (sp?). The collection of all of these things suggests that there is some type of disorder...probably mitochondrial, could also involve Rett syndrome. The tests tomorrow will test for both, along with several other possibilities. He will write some notes today and tomorrow to give to us outlining all of the possibilities from what he has gathered from Lydia's medical record and our consultation. He warned us not to look on the Internet and to consider the source if we do...there is lots of info about mitochondrial disorders, and the field of mito disorders is so diverse that it is likely that we could work ourselves up about something that may not even be applicable to Lydia. He suggested to direct anyone interested in researching to thir website (google for Medical Neurogenetics). He has lots of talks and powerpoints to explain some things. Good news to us: Less than 15% of mito disorders are fatal, and he does not see symptoms in Lydia that would suggest that hers would be degenerative. Dr Hollman had also said that she would predict that since Lydia has continued to develop...that she would continue to progress, just at her own pace...whatever that may be.

He talked some about Elise's condition. He said that mito disorders are genetic, and it would be very rare for two different genes to be abnormal...so Elise and Lydia's conditions probably are related. (Boy, have we gone full circle on that??) The abnormal gene may just affect the two of them differently. He said that after we have a diagnosis for Lydia, we could then probably do just some simple blood tests on Elise to test her for some variation of the same disorder. He suggested that it might also be necessary to test Audrey...to be sure that there wouldn't be issues that could appear in adolesence or later related to the disorder....or that it wouldn't affect their offspring. That brought a tear to my eye...I pray that any children that our children have will not have to endure what Lydia has had to do.

He also said that in 60-70% of cases, he can determine the likelihood that future children of ours would have the same disorder...we don't have plans for future children...but, God ultimately holds that decision, anyway.

I asked for another explanation about what mitochondrial disorders are, since I still have trouble wrapping my brain around it all. He confirmed that every cell in your body has a mitochondrian, but they may not all be affected by the disorder. Typically, the disorder affects one main organ and possibly other cells in the body. So, in Lydia's case, it affects her brain. We shouldn't be concerned about it affecting her heart, lungs, etc. Dr Schoffner agreed that there is lots of info and it is very complex. He told us not to expect to understand it in 1 or 2 visits with him. So far, he's right. I certainly don't understand it all...never have, not yet anyway. So, I've shared what I've gotten so far. I probably can't answer any further questions. I'll try to share some of the info from the notes that he writes for us.

Keep Lydia in your thoughts and prayers tomorrow for her procedure. Love to all!!

They made it there

The plane ride went well. Lydia slept most of the way. They had a little trouble at the airport, only because they are not used to traveling. They made it to the Ronald McDonald House at 11:00pm. Mandy just called to ask me to let everyone know they are doing alright. The doctor was calling Lydia's name as we hung up. Mandy could not get a signal for her internet in the Dr. office. We will update as soon as possible with new information. Thanks for all the continued prayers.

Tiffanie

UTI Negative

I called for the results of the urine culture today and it was negative...Thank God! Still eating well...having a fun day!! We are on track for our trip on Monday. The surgery center called to confirm Lydia's muscle biopsy for Wednesday...she said that there is also a spinal tap scheduled, but Dr Schoffner would decide for sure if he wants to do that after he meets with us on Tuesday. He just schedules time for it in case he feels that it is necessary. We plan to stay at the Ronald McDonald House in Atlanta...it is free and shuttles to and from the clinic and surgery center. We confirmed that they do have Internet access, so I hope to keep everyone updated through the blog while we are there. We appreciate your continued prayers!

Doing well

Good...but very busy...hadn't had a chance to sit down to the computer the last couple of days. Lydia had her hospital follow-up appt with Dr Wood yesterday...he thought she looked good...like she was feeling better since he last saw her. I told him that she was taking in about 16 ounces with a little bit of baby food each day....he thought that was sufficient since she is gaining weight. She gained 4 ounces since her last visit with him last Tuesday. Feeding has changed considerably since yesterday...I already knew that Dr Hollman was good....but she never ceases to amaze me...she said that she expected Lydia's appetite to get better about 2 weeks after weaning the ACTH...Well, today was exactly 2 weeks since her last shot...she took 5 oz before bed last night (and slept all night!!! Hooray!), then she took 4 oz for her first three feedings today! She slacked off a little tonight, but took at least 2 oz for each feeding....so she still totaled about 16 1/2 ounces for the day, but I am very encouraged by her increase in appetite.

PT with Susan today:
Lydia did very well...she is progressing more and more! I am so proud! Susan taught me some practical things that I can do during our day to fit into our routine, since it is difficult with our schedules to fit in "therapy" time. Lydia's optimal therapy times are so minimal...it's hard to always take advantage of them...assuming that no one else needs mommy at that time...or nothing else is going on. It helps to do "therapy" stuff while doing the things that we do already...diaper changes, dressing...

A story for Teresa:
When Teresa (Lydia's OT) came yesterday, she let Audrey help her with Lydia's therapy...while I was meeting with our Family Service Coordinator for Early Steps. Teresa and Audrey worked with Lydia and then discussed the notes to record on her log. That afternoon, Audrey was writing on her doodle board, and she was pretending to write notes from therapy...as she wrote, she said, "we rubbed on her pretend teeth..." Then this evening, Grandma kept the girls while I got a hair cut. Grandma walked in on Audrey playing with a baby doll...she had on a white play glove (little girls' Easter gloves) and she was rubbing on the doll's mouth, cheeks, and stomach. She told Grandma that she was Ms. Teresa. :) All of Elise's therapists that have gotten to know Audrey pretty well have said that she is destined to be some type of therapist when she grows up. We'll see...she's getting pretty good practice now.

Elise is just loving life at her new "school" (BR Speech and Hearing). She is so happy to go there every time. And I see her trying to talk so much more. Today she was watching Barney...at the end of every show, they sing the "I love you" song....I probably don't have to type the words for most of the moms reading this, but, in the song they say, "with a great big hug and a kiss from me to you...won't you say you love me too." We always hug and kiss when they sing it....well, I was in Lydia's closet picking something up...she came and found me and said, "gug" which is Eliseeze for "hug." It was so sweet! She was coming to find me for her hug.
I also notice that she is answering "what" questions so much better. When I picked her up from school today, I asked if they played outside...and, she said, "yes." Then, I asked "What did you do outside?" She said, "Slide." It was very interesting, but she paused first and was clearly trying to remember what she did outside. I am so proud of her.

A note about my cell phone...
My cell phone is not very good at alarming me of voice mail messages...it sometimes takes up to 2 weeks to signal that I have a new message...so, if you need to get in touch with me...please leave a message on my home phone. This is probably a problem I need to look into, but it is not at the top of my list of priorities. :)

I'll call Dr Wood's office tomorrow to be sure that the urine culture came back negative. I'll talk with the nurse case manager from Atlanta to confirm appt's and that Lydia is free of infection, fever, etc. We expect to fly out Monday evening at 5:40. Please keep us in your thoughts and prayers for our trip. Special thanks to Dottie for arranging our flights and sharing her flyer miles with us. Special thanks to Tiffanie for volunteering to be our "travel agent" and "babysitter coordinator" for the trip...two big headaches lifted from my brain. Thanks so much to everyone who is lending a hand to help us these days...we are so very blessed!!

Great News!!

Dr Superneau's office called this morning....Elise's remaining MPS test was normal too!!! Fantastic!! She is doing so well....except for her adjustments to all the chaos at home...hopefully we can keep things as normal as possible for her to keep the tantrums to a minimum. :) Her teacher at BR Speech and Hearing today said that she has been doing very well...lots of talking! She said that she sometimes has trouble understanding her...that is to be expected. But, she was not doing near as much talking in her MDO class...I am soooo glad she feels more comfortable there. It is great for her! One thing is for sure....she knows exactly what she's saying every time she talks...she repeats the same unintelligible sounds over and over. The good thing is that she is getting a little less frustrated with it (at least sometimes....when she's not already in a sour mood)...she is starting to laugh at us sometimes as we try to figure out what she's saying...I'll ask her to repeat it over and over again, then I guess what I think she's saying..everytime she says, "No." Yesterday, I sent her to Audrey and then to her daddy to see if they could figure it out...she just kept repeating the same thing each time and smirking at us all because we didn't know what she was saying...it became sort of a game...not very good speech therapy, but it was funny. :) I'm not sure that we ever figured it out...

Appt with Dr Lutfallah (endocrinologist) today...
We will continue Lydia on the half dose of Levoxyl and check her levels again in April...we will see the dr again in May. She tried to explain to me again the details of Lydia's thyroid issues...I understand it a little better now....though I don't know if I can explain all of it....? Lydia does NOT have hypothyroidism because her T4 level (from the thyroid gland) has never been elevated...her TSH (from the brain...tells the thyroid gland how much thyroid hormone to produce) has been elevated, which suggests that her thyroid gland is not producing the right amount of thyroid hormone, and her brain is working too hard to keep it level. It is called non-specific TSH elevation...Dr Lutfallah expects that Lydia's thyroid issues will resolve and that she will NOT develop Hypothyroidism...she said it is fairly common....they see it a lot in babies, but they usually stop taking the medication by age 3. The reason they continue it until then is because it is so critical to brain growth and development. After age 3, they will stop the meds and test levels to be sure that she doesn't still need it. She told me the normal ranges for the levels and Lydia's levels each time they were checked...she has been right on the border each time, so her levels haven't really been very detrimental.

We will see Dr Wood tomorrow for our follow-up to the hospital stay, and we will re-check the urine culture (catheter) to be sure that the UTI has been resolved. It will take 48 hours for results, so we expect to get results by Friday when the case manager from Atlanta will call us to be sure that Lydia is free from infection, fever, sickness, etc. Thank goodness my grandmother is staying with us a couple of days...she'll stay with Elise at LSU while Audrey and I take Lydia to the dr...then we'll pick them both up to come home for an Early Steps meeting for Elise and Lydia. Maw-maw Grace is also great at doing laundry....lucky for us!! :)

Lydia's eating is still about the same...much better than last week...still not as good as I hope for it to get.

Holding on...by a thread

I'm having a little trouble holding it all together today...lots of stress, lots of exhaustion, and lots still left to do. It's my brain that stays the most exhausted. I am feeling guilty for my big girls again...so many daily changes and things for them to deal with...especially their moody mommy. I need God to hold my hand today...to help me to take one minute at a time....one day at a time is not calming enough anymore. God is constantly reminding me that life is a journey...I can't just pray about something and then expect it to be solved. We can't get to a destination and then expect smooth sailing for the rest of our time. He intends for us to constantly lean on Him...He doesn't want us to do it alone. Every new revelation should lead to the next.

How about that? I think I feel a little better now...Thank you God for showing me wisdom.

Please pray for all the girls in my house...that we all get the love we need...and for the only man in our house...for having to deal with all these women! :)

Life is Good

Oh....life is good...it is so good to be home! I didn't realize how much I'd missed my girls and my husband and the craziness! And it is crazy...though we've had some fun family times these past two days...last night was movie night at the Poche's house and today we had a neighborhood Easter egg hunt. It was a beautiful day and lots of fun. My sweet husband has been on a cleaning rampage this weekend....lucky for me!!

Lydia has been eating fairly well today....just the bare minimum, but within accepted range, nonetheless. I hope to see her appetite pick up more and more this week. She is not refusing it by shifting her head side to side anymore, like before. She just stops sucking (or falls asleep) near the end of a bottle sometimes. Her spoon-feeding has gotten a lot better over the past few days. She seems to be enjoying her fruits and veggies. :)

I hear more and more often of new people following our story by reading the blog and joining in prayer. I am inspired, encouraged and strengthened by this more and more. I usually hear about someone new at just the right time...when I need a little pick-me-up...just God following through on His promises to remind me that He is there and sending people to carry me. Thank you!

A little reflection....
I received this story in an email today...it speaks volumes:

WHEN YOUR HUT'S ON FIRE
The only survivor of a shipwreck was washed up on a small, uninhabited island. He prayed feverishly for God to rescue him. Everyday he scanned the horizon for help, but none seemed forthcoming. Exhausted, he eventually managed to build a little hut out of driftwood to protect him from the elements, and to store his few possessions. One day, after scavenging for food, he arrived home to find his little hut in flames, with smoke rolling up to the sky. He felt the worst had happened, and everything was lost. He was stunned with disbelief, grief and anger! He cried out, "God! How could you do this to me?" Early the next day, he was awakened by the sound of a ship approaching the island! It had come to rescue him! "How did you know I was here?" asked the weary man of his rescuers. "We saw your smoke signal," they replied. The Moral of This Story:It's easy to get discouraged when things are going bad, but we shouldn't lose heart, because God is at work in our lives...even in the midst of our pain and suffering. Remember that the next time your little hut seems to be burning to the ground. It just may be a smoke signal that summons the Grace of God.

I've always felt strongly that pain, suffering, grief, etc. are inevitable in life. I have never felt like God was punishing me during any pain I've endured. I've always felt that we must know pain in order to truly know happiness. How would we ever know how good life was if we never knew how bad times could be? It makes such sense to me. Before Martin and I had our three beautiful girls, our first pregnancy was a miscarriage. Very soon after that happened, I counted it as one of the blessings in my life. I felt so much love and support from my family and friends around me at that time...I saw something new in my husband that made me love him even more...and it all happened in a way that only God could have planned: Martin and I knew that we wanted to get pregnant, and I was leaving for the weekend for a bachelorette party for a friend of mine...I knew I'd probably have some drinks, so I decided to take a pregnancy test before I left, just in case. Well, it surprised us as positive. I made an appt with the OB and we had ultrasounds three weeks in a row because the doctor wasn't sure if it was a good pregnancy or not...it could have just been too early in the pregnancy to see a heartbeat. By the third ultrasound, we found out that the fertilized egg had actually disintegrated in my uterus. I had to have a D&C to rid my uterus of the debris. The doctor told us that if I hadn't had the procedure in time, we may have had trouble getting pregnant again. I had never felt pregnant...had not missed a cycle yet...I would have missed the entire thing, if it wouldn't have been for that bachelorette party I was supposed to go to. I felt so good about it and was so thankful that God planned it to happen the way it did...we may never have had our three beautiful daughters. Sometimes it is hard to recognize the gifts that come with difficult times. I pray that God opens our eyes to those gifts when we have trouble seeing them ourselves. I already am so thankful for the experiences I've had through Lydia's difficulties. I have been granted so much love and support from so many people...I am overwhelmed with gratitude. Yes, some times are really hard...but we know that God has a plan for every minute of every day. We must be thankful for the difficult times because without them, there would be no happy times....how would we know what happy was?

GOING HOME!

Dr Guidroz just left and gave orders to discharge. I can hardly believe it. I guess Lydia just needed the threat of that feeding tube to get her in gear. She has continued to take at least 3 oz at every feeding since last night. We will wait for Martin and the girls to come to get us, then we're free to go home! We'll follow up with Dr Wood next Wed and do a urine culture (which takes 48 hours) to be sure that the infection is gone. The doctor's office in Atlanta will be calling us on Friday to be sure that Lydia is free of infection, fever, no antibiotics...we will have to reschedule the appt if any of that exists. As of now, we are on go for the 18th. And we are going home...without a feeding tube...hooray!

Thank you all for your prayers, your care and concern, your love, and your support....in thoughts and deeds...thank you, thank you, thank you!!! Please continue all of your prayers for us!

Correction...

Since Lydia took 3 oz after Dr Alberty left today, and then...drumroll, please: She took a full 4 oz at her next feeding...Dr Momma and Dr Daddy decided that we should hold off on the feeding tube to give Lydia a chance to see if she might catch up on her own. The nurse called Dr Alberty to confirm, and he agreed. Please root for her to continue successful feedings!!

Feeding Tube

Dr Alberty came by this afternoon. He was not impressed with her 4 oz of intake for the day so far (at about 4:00)...he also witnessed Lydia refusing to take her bottle. We decided it would probably be best to start with the feeding tube tonight. We will offer 3 oz every 3 hours...whatever she does not finish by bottle, the nurses will administer by feeding tube. I don't really have any info about when we might be sent home...it really depends on how Lydia responds in the next couple of days. The nurses actually came in to put in the feeding tube a little while ago, but Lydia had then just finished about 3 oz of formula and about a tsp of oatmeal...so I asked if they could come back later when it was time for her to eat again...they also thought it would be better to wait because she will probably gag when it is inserted, and she might spit up. It will probably be done around 6:30 or so.

We went for Lydia's ultrasound of the kidneys early this afternoon...she was a little trooper. She just laid there and smiled...turning to look at the monitor every once in a while. The tech asked if she was always that sweet...Of course I said she was....I'm just a tad biased. When we were done, she said...it doesn't get any better than that...Lydia made her job very easy. :) We don't have any results from that yet....the tech said that she didn't see anything abnormal, but she is not responsible for reading the results.

The Morning Report

Lydia had a relatively good night last night...woke up for one feeding....2 1/2 oz. I was starting to get hopeful about an increase in her intake, but she's had two feedings so far this morning...both just one ounce each.

Dr. Ramey came in this morning...he was also hopeful that her feeding will turn around before Saturday, so that we don't have to do the feeding tube...but, we'll do what's best for her. He talked to me about the fact that babies don't normally get urinary tract infections. So, that makes them wonder if there may be some type of abnormality with her urinary tract. It is possible that some type of bacteria just got in there to cause the infection...especially with little girls, since their outside is closer to the inside for girls than boys. But, it is worth looking into to make sure that her kidneys, urether, and all the other parts are formed properly...especially considering Lydia's medical history. There are two procedures that they do to determine this: (1)an ultrasound of the kidneys, which is obviously not invasive at all...they will do that while she is here in the hospital....he put the orders in this morning. This is to be sure that she has 2 kidneys, both are the right size and shape and have no other abnormalities. (2) The other test they do (didn't catch the long name he gave for it) is to shoot a dye into her bladder with a catheder...then take multiple x-rays of all parts of the urinary tract to be sure that all of that is normal. He said that they want to wait to do that for a couple of weeks....after the urinary tract infection is definitely gone.

I called Dr Superneau to find out about Elise's test results, but he did not have the report yet. He was going to try to track it down and call me back today....scratch that...they just called. One test came back normal. Hip, hip, hooray!! One test is still pending...they'll call back in the next couple of days.

I also called Atlanta this morning...we have an appt for March 18 and 19: Consultation- on the 18th; Muscle biopsy - on the 19th. Martin, Lydia and I plan to fly out on the 17th, and fly back on the 20th.

Yippee!

I couldn't wait to share this news... The nurse came in and cut off the IV fluids this evening. A couple of hours later, Lydia ate some squash and rice cereal pretty well...then after her massage, she ate almost 4 oz!!! Let's pray that this continues!!

Then, I had put on one of the Baby Einstein videos that the Childlife representative here at the hospital gave us...(not the same video that we have at home)...she didn't pay much attention to it. After she finished her near-4 oz feeding, I put on the one that we have at home. As soon as she heard it start, she looked over at the TV and smiled and cooed. Her daddy says she has a favorite show already. :)

Very encouraging news!

"The Rest of the Story"

As I was typing this the first time, I got a call back from Dr Guidroz, Dr Alberty came, and Lydia decided it was time to eat....yeah! She actually cried from hunger! She ate about 50 ml...less than 2 oz. Then we played together for a while...so much fun!! But, when mom started to demand a little too much, she shut down....she said, "Come on, Mom...I'm in the hospital for God's sake...with an IV and multiple blood sticks...I can take a rest from therapy for a little while." Okay, okay! :) Now she is playing with a toy that the ChildLife representative brought us.

One more thing from Dr Hollman....the reason that Lydia probably didn't run fever with the urinary tract infection to alarm us of it earlier is because ACTH also prevents the body from running fever...we already knew it was powerful stuff...golly gee!

Visit with Dr Alberty:
He listed the possibilites of things that could be the underlying cause(s) of her decline in eating...some short-term, some long-term. The ways to address it would depend on whether the cause is long term or short term. Short term possibilities: ACTH weaning, congestion, UTI, possibly reflux (though I doubt it right now)....long term possibility: an effect of the mitochondrial disorder (if in fact, there is one). Of course, the evaluation in Atlanta would help us to determine this more...but, that information is not available right now. So, the plan for now is to treat it as if it is short-term...these are his orders: Hep-lock the IV in hopes that the lack of fluid and glucose will cause her to be more hungry, and so eat more. We will see how she does for the next couple of days. If her appetite does not pick up by Saturday, he will order an NG tube (feeding tube) for the next 2-3 weeks. We will continue to feed her with a bottle or baby food at each feeding and then supplement with formula in the feeding tube to equal the typical amount for a feeding for a baby of her age and weight. If we see that in 2-3 weeks, after effects of ACTH, UTI, and congestion should be resolved, that she is still not eating adequately on her own, he will consider surgically inserting a Gastrostomy feeding tube (G-button) which is placed in the stomach. It is the more long-term option. If within those weeks, we have an evaluation from Dr Schoffner...that may shed more light and determine the next action too. Dr Alberty predicts that we'll be staying here through the weekend...then go home with or without a feeding tube depending upon what Lydia does in the next couple of days.

A visit from a local comedian:
I had a delightful visit from a coach at Martin's school yesterday, Tommy Douglas....quite a character. He was only here for about 10 minutes, but he kept me laughing for the rest of the night. Thanks Ace! He also brought quite a spread of stuffed animals for all three of our girls...Ms Hilda commented that it looked like Santa Claus had paid us a visit. Such a sweet gesture....it melted the hearts of all the women who have entered our room. Big sisters Audrey and Elise will be here this afternoon, and I know they will be thrilled to see their surprises!

To everyone who called me today....please understand that I am so grateful for you! Please don't get the wrong impression since I declined to tell you all of this over the phone....Jennie, Ms Hilda, Dad, Bridget....and everyone else. It gets hard on the brain to repeat so much info too many times. There's been so much today, that I'm bound to have forgotten something....I'll update again if I remember anything.

Please continue all prayers....we love you!!

Lots of info

Sorry to be so late getting to the computer...I thank you all for your care and concern for our little sweetheart! It has been a long day so far....I am trying to collect my thoughts right now to share all of the info with you. It was a rough night last night...Lydia and I were up from 1:30-4:45...during that time, she took about 2 oz total. Then, when she finally got to sleep, the lab tech came in at 5:30 to take her blood. Thankfully, she fell back asleep relatively quickly after that. The next few hours were flooded with dr visits, nurses, friends, and phone calls. At times, I was answering two phones at once...my cell phone and the room phone, while trying to talk to the nurse and visitors at the same time....busy, busy, busy! :) We are so lucky to have such loving support! Amidst all of that, we were both trying to fill our bellies....Lydia took a full 2 oz at 9:30 this morning...that was better, but still only half of what her typical intake is for a feeding. This afternoon, she took about a teaspoon of oatmeal by spoon and about an ounce of formula. I have found her to do a little better with spoon feeding than bottle feeding...last night she enjoyed some sweet potatoes and rice cereal. We will definitely continue that.

Dr. Ramey came by on rounds this morning. He thought that Lydia looked good...suggested that we might be able to go home in the next day or so...I am doubtful of that...I don't think she could hydrate herself on her own at this point, not to mention that she would be lacking the nutrients that she needs. When Dr Ramey got back to the office, he spoke with Dr Guidroz. (Dr Wood is off today, so that is why his partners are seeing us.) Dr Guidroz called me....she feels like I do...we aren't ready to talk about going home yet. She told me about Lydia's blood test...protein level was normal, but her albumin (sp?) level was at the lowest end of normal (normal range is 14-40; Lydia's was 14.) The albumin level tests the amount of nutrients in her blood. This is crucial because what happens is that if she doesn't have enough nutrients, her protein level will drop....and if she doesn't have enough protein, her body will start to pull it from her muscles, which will then weaken her muscles. We would then have to work to build muscle mass again...which would mean that any development that she's accomplished through PT would have to start all over again. We obviously do not want this. I hope I am explaining this correctly...I feel like such a novice when I try to ingest all of this info...our nurse (who is more of an angel than a nurse) sat with me at length this morning trying to explain all of the options and their ramifications. She offered several hugs, encouraged me as a mother, and even offered to come sit with Lydia after her shift is over tonight so that Martin and I could take the girls downstairs for supper and a little special time with them. God has taught me so much through this entire situation...there are so many good and loving people in the world. We are so grateful and so blessed! So...to finish the medical info....Dr Guidroz wants to consult with Dr Alberty...she is calling him now to see what time he might be able to come see us. They will explore all options of getting Lydia the nutrients that she needs...possible feeding tube, button (different kind of feeding tube). I also have a list of questions for him, one of which is whether or not we could try an appetite stimulant...because the problem is obviously that she does not have an appetite...which brings me to my conversation with Dr Hollman today: Yes, she does expect Lydia's feeding habits to be so drastically affected by the weaning of ACTH. The difference for Lydia is this: most babies gain a large amount of weight and fluid while taking ACTH...to the point of being very puffy and overweight. Then, the lack of appetite after weaning just helps them lose the weight that they gainec. Lydia did not gain that much weight, and so, losing weight is not good for her now.

To be continued....Lydia needs to eat....

Call from Dr Guidroz

It seems we may be a little more concerned than originally expected. When I told Dr Guidroz about the amount of food Lydia has taken in so far today, she agreed that it was "horrible." She talked about cutting back IV fluids even more tomorrow to see if it will make her want to eat more. She didn't want to do it at night because she wouldn't be checking on her during the night. I pointed out to her that Lydia was even not eating well at home, without any fluids, so I'm not convinced that that will help at all. She agreed, but thought it might be worth a try...if the UTI starts to get better...the urine culture is showing a typical UTI that is very common- Rocephin should knock it out. She also wants to do some blood tests tomorrow. She said that these IV fluids will keep Lydia hydrated, but we can't let her go much longer without getting any nutrients. The blood tests will show how much protein is in her body...which will let us know how much longer we can go...I think that's what she said...? I told her that I want to talk to Dr Hollman again because I doubt that she expected her eating habits to change so drastically...dr Guidroz said for me to call Dr Hollman in the morning to find out...so that we'll know what to expect. Then, she also wants to consult with a GI dr...we may need to consider an alternative form of feeding if she doesn't pick up soon...feeding tube, etc. We'll either consult with Dr Alberty or with Dr Khoshoo over the phone. The blood tests should be first thing in the morning...I'll try to update again when I have more info. Thank you all so much for your continued prayers and support!!

Still Not Eating Well

Today's feedings have actually been the worst of all. She hasn't even taken a full ounce since 5:00 this morning...today's total so far (as of 4:00 pm): less than 2 1/2 ounces. I am going to try to call Dr Hollman tomorrow to talk about it...she was not in the office today. I wonder if she expected her appetite to drop this drastically. Needless to say, I suspect we'll be here for tonight for sure. One day at a time from here on out. I am soooo thankful that we went to see Dr Wood yesterday afternoon...she has been so cheerful and pleasant that it would have been hard to tell that she was dehydrated or had an infection. She is still VERY much a delight! She has been so playful and fun...except when we try to feed her. Aunt Linda was here today and said Lydia acted like we were trying to give her the worst-tasting thing in the whole world. After Aunt Linda played with her a while (to let me go home and take a bath and pack more things....thank you!), she told Lydia, "I think your sisters each slipped you a dollar to play sick, so that they could spend some time at Grandma's house." Aside from her congestion (which is the least of her concerns) you would never be able to tell that she is sick.

When I walked in my house today, Betty Lou was there (thank you for your help!!) and a porcelain plaque was propped on my kitchen counter. It said, "Believe in Miracles." It immediately tugged at my heart strings, of course. Then when Betty told me the story about it, it meant even more...she stopped at the store on the way to our house to pick up some laundry detergent to wash some clothes (THANK YOU!) and she was determined not to buy anything else, so tried not to look at anything else in the store....then she just saw this plaque staring at her...she said she had to buy it. Yes, God, I'm listening....I do believe in miracles, and I believe that you have a miracle in store for Lydia. I will try to just wait patiently, trusting in You, to find out what that miracle might be.

Thank you Jory for your words of encouragement today...we don't get mushy very often...it really means a lot when you speak to me from the heart. Thank you for your support...and thank you all for everyone's support...I hear of more and more people who are tuned in to our blog every day...I am so thankful...please continue your prayers...there could never be too many.

Another day

Dr Guidroz came in this morning to check Lydia, and she said not to be surprised if we end up staying at the hospital again tonight...since Lydia still is not eating well. She woke up hungry at 5:00 this morning and aggressively went for the bottle, but after 1 oz., I couldn't get her to take any more. At 7:30, I tried to feed her again....again, she seemed hungry....but, she only took 8mL (1 ounce is 30 mL). She is resting well now...it took her a while to get comfortable after that...plenty of dr's and nurses coming in and out to check on her too...that kept her restless for a while. Dr Guidroz cut back Lydia's IV fluids thinking that she'd be more thirsty with less fluid in her system. Hopefully, that will help. I don't think they plan to send her home unless she is back to her normal feeding schedule, or else she won't be able to maintain hydration on her own. Dr Guidroz said that she would be in touch today to find out how Lydia is doing and to make a final decision about staying or going. She was also going to check on the urine culture at the lab...but, it should take 48 hours for results. The culture will tell them which bacteria is causing the urinary tract infection, which will then tell them which antibiotic to send us home with. For now, she is still getting Rocephin in her IV to fight the infection. Along with all of this, she is still congested...not near as bad as she was, so hopefully that is getting better. I'll update with more news as we get it.

In the Hospital

Yes...we are in the hospital tonight. This has been quite a day...let me start from the beginning. I called to schedule an appt with Dr Wood for this afternoon because Lydia still was not eating well. We saw Dr Hollman at 10:30...that visit was difficult. We talked a lot about hypotheticals for Lydia...about some of the patients she's seen with mitochondrial disorder, etc. She again emphasized that it is rare and very difficult to diagnose. She has only 3 patients with it right now...all ranging in age and severity. One little girl is walking and talking, but has lots of neurological problems...seizures, tremors, jerks. She said that one little girl is doing a lot better than she expected...(this is what really got to me)...she said, "She made it to age 5, and I didn't think that she would live that long." She said that she is not doing well, but her family loves her and is thrilled just that she is alive. She told me that some people live with mitochondrial disorders and some people don't. I'm having a really hard time with that...I pray for God to take those worries away from me...I'm not doing such a good job of handing them over myself. We also talked about the possibility that it could still be Rett syndrome...but she said that Lydia's condition did not present as the typical clinical presentation for Rett. Rett babies usually develop normally for the first six months...roll over, hold head up, sit...then they stop progressing, and their head circumference starts to drop (which Lydia's has...her head size is now below the 2nd percentile...off the chart) This is different than Lydia...which is not to say that it is definitely not that....and there's still the chance that the cause may just be that something didn't happen as it should in utero. The order of testing for us is: mitochondrial disorder (with drs in Atlanta)...if that is negative, blood test for Rett...if that is negative, we'll assume it was just a mishap in utero.

Elise's pyruvic acid came back normal, as well as the lactic acid...great. That rules out mitochondrial disease for her. We will find out about test results for MPS at the end of this week. Dr Hollman doubts that Elise has MPS...that is encouraging...but, we'll find out for sure soon.

I ask for your prayers for Dr Hollman today too. She told me during our visit that her dad was dying today. She said she decided to come to work because she needed to get her mind off of it. Please remember her in your prayers.

Then....we went to see Dr Wood at 2:00. I was able to show him a little discharge from Lydia's private area that I had mentioned to him last week. He decided to order a urine test...a catheter. He also ordered a chem 7 blood test to determine if Lydia was dehydrated. Urine test: Urinary tract infection...he listed all that the test showed...protein in urine, blood in urine, too many white cells to count, and on and on. Blood test: she was dehydrated...no surprise, she has not been eating. Her CO2 level was 17, and he said that if it were 18 he would have sent us home, so it was only borderline. Dr Hollman actually shed some light on Lydia's refusal to eat lately...I overlooked the obvious....coming off of the ACTH. Dr Hollman said that they see that a lot in babies when they stop the shots...she suspects that her eating will return to normal within about 2 weeks. Dr Wood thinks that the UTI may also be affecting her desire to eat. So the plan for this hospital stay: she is getting IV fluids to hydrate her and Rocephin to knock out the UTI. He said that he is willing to send her home tomorrow as long as she is eating better...so that will be the test. Special thanks to Brandi and Kristi for taking special care of us!! They came in to do Lydia's IV. And thanks to all who helped us today....it turned out to be a long day for everyone...Bridget, Aunt Leigh, Aunt Becky, mom...

Atlanta called today...they recieved the packet that we mailed yesterday...now they are waiting to get the medical records from Lydia's doctors so that Dr Schoffner can review her case. Then, the receptionist will call me to set up the appt.

I have a Tablet PC here at the hospital, so I will try to keep everyone updated. Right now, Lydia is snoozing peacefully! She is so beautiful!!!

Not eating any better

I was hopeful yesterday morning because she took a 4 oz. bottle. Then, for the rest of the day, she again would not take more than 1-2 oz. at a time. Teresa even came yesterday afternoon for OT and tried to feed her...she was just as confused as I am. We have no idea why she doesn't want to eat. It doesn't appear to be pain...and she sucks her pacifier just fine...she just doesn't want the bottle. She ate a little rice cereal and sweet potatoes by spoon yesterday evening...but not enough to sustain her. Then she went to bed around 11:00...totally skipping her last feeding. I gave up after about 30 minutes of trying to get her to take it. Then she woke up at 1:30...I thought, "Great...now she's probably hungry enough to eat." No such luck...we were up until 4:30...I made two different bottles because the first one went bad before she took any of it. She did not get a drop of either one. She finally just fell back asleep. We have an appt with Dr Hollman at 10:30..then I made an appt with Dr Wood for 2:00. He told me to bring her in if she didn't eat any better yesterday. Hopefully we'll figure something out.

PLEASE Pray...

...for help with feeding difficulties. It has gotten even worse for Lydia today. I am really confused by it, though her doctors and I should probably be used to being confused by her. She doesn't appear to be in much pain from eating...she is just refusing it...she turns her head from side to side away from the bottle when I try to feed it to her. If I try to hold her head still to eat, that's when she starts to cry. If I actually get it into her mouth, she usually starts to gag. She seems to be pretty content, otherwise...that's what really perplexes me. I know that her congestion is still miserably bad, so it could possibly have gone to ears to cause an infection...or maybe her throat is bothering her...but, I figure that if it were any of those things, she would be crying from hunger because her stomach is just about empty. She usually takes in about 24 oz in a day. Today, she has taken 6 oz. total. I can't get her to eat more than an ounce and a half at a time. I've even resorted to putting formula in her mouth with a medicine dropper and then giving her the pacifier to suck it down...no luck with that either. I weighed her this morning, and she has lost 4 oz. I'm really starting to get worried about this. The most frustrating part is that I will call the doctor in the morning, but I am almost positive that they will not know what to tell me...Lydia is just so confusing. Hopefully, if we get a diagnosis soon, that will help to answer some questions...but that doesn't help with the here and now. I will also call the endocrinologist in the morning to find out what her thyroid levels were last week...maybe that will shed a little light...we'll see. Also, Elise has her urine test tomorrow morning for the MPS disorder...keep her in your thoughts this week too please. Then she has speech therapy at LSU....Lydia has OT at home at 1:00...then Audrey has an ENT check-up at 3:45....busy day!! I will try to remember to b..r..e..a..t...h..e and trust in the Lord...I know He will be there to carry us all.