Best made summer plans...

I am sorry but my pictures went crazy and all ended up at the bottom. I tried to move them into place and they just won't go.. So below are the pictures that went with the text...

We had the best of plans for Annabel to really enjoy here summer for the first time in years.  This meaning she was free of any central lines.

We rescheduled all routine doctors appointments until the end of August and were going to spend most of our days at the beach.  Just spending lazy days, living a much slower pace.

So here is Annabel spending time in her little pool up on the middle deck at the beach house. We fill in the morning so by afternoon the water is just right.

If the beach water is smooth and mostly clear we do go to the edge of the water and let her be rocked by the tiny waves.  Mostly we opt for the clean water on the deck.

As we do have a pool at home she does spend sometime in the pool when we are home.

This is Annabel having lunch with her much loved teacher, Ms. Ann...who we will miss so much due to her retiring.

As you can tell she has eyes only for her Ms. Ann.

So for the update on Annabel's health, she will be having her supra-pubic cath placed on this Monday. The surgery isn't a biggie but always some concern when we have to put her to sleep. If all goes well, we should be returning home that evening

The neurologist (movement disorder specialist) wanted her to have a PH probe to rule out reflux. We have done this several years ago and found nothing of reflux. She just wants to make sure these movement have nothing to do with reflux. So they will sedate her on the 22nd of July and place the probe. We will stay in Houston for the evening and then be there in the morning to have removed.

Our newest issue is that Annabel has begun to not tolerate her feeds very much. We are still giving her most of the feeds but basically forcing her to take them (g-tube). She is uncomfortable and begins letting you know at the beginning of the feeds. Also her stomach is becoming very distended and hard. She is having very few BM's even though her stool is watery. Tonight she just had a KUB and it shows she is so filled with air. All her bowel loops, like they were prior to her surgery this past July. This new upsets me greatly, because if Annabel doesn't eat, her only option is central line and we don't even want to go there...

Enjoying the breeze on the deck!

We had to seen an ENT today after the finding of her sleep study. Back in 2009 she was said to  have severe obstructed and central sleep apnea. She was placed on c-pap which work for a couple months then needed more oxygen and there another sleep study that led to her being on bi-pap. That was a no-go. Her tummy filled with so much more air and there for hindered her feeds. Annabel has been on oxygen since then but the sleep study again was severe and they want her on bi-pap. So at the ENT they said her tonsils were so tiny (like back in 2009) and then xrays her adenoids and they said they are really big and only leaves her a very tiny airway... So she will have her adenoids also out probably August. He also went on to share about reducing her tongue by killing some of the tissue in the back, then decided the back part of her tongue was not that big it was the front of her tongue. We know this falls back when she sleeps and blocks her airway also.  He mentioned about when he scoped her that if he found things

wrong with her airway he would have to perform a tracheotomy...

WHAT the heck????  We agreed he could do the adenoids because

they are obviously an issue but likely not the only issue.

There would have to be the most significant findings that would

convince me that Annabel would be better off dramatically

with a trach.

                                                 

This photo below is the morning Annabel woke

with Hand, Foot and Mouth Disease(Virus). She was awake all night due to be so uncomfortable.

In the morning she was covered on her inside palms and wrist, bottom, top of feet and ankles,

and her lips were so red and puffy. She did end up with the sores in her mouth.  All in all, I don't think it was as bad as it could have been. She

basically lived on Benadryl for the first 3 days. She still has the rash but the intense itching is seeming to slow down.

                               

Prayer request are for her to do well with the multiple times she will be going under for sedation...

Also that her stomach will calm down and that she will be able to receive all her nutrition from her g-button.

Last one is that the supra-pubic catheter procedure will give Annabel relief from the UTI's and that the more invasive surgery, vesicostomy will not need to be performed.

New Medical Information

This is what is going on with Annabel medically. The UTI's  are continuing with maybe only 24-48 hours after her antibiotic course is complete before it reoccurs. We are now working with Infectious Disease at Texas Children's Hospital. We have been with them for about 3 months now and in the beginning they seemed to have much hope that there were antibiotics out there for Annabel that could fight this bacteria that comes back so quickly. Two weeks ago we went in for an appointment and we knew Annabel was infected again just two days after finishing her recent round of antibiotics. So the doctor wanted a urine culture and some labs. She also placed her on Keflex until her surgery on July 15th and then one month after. This Tuesday I received a call from her doctor who had given us hope concerning her antibiotic use. In my heart I knew the Keflex wasn't getting the infection because Annabel was uncomfortable during the night and wasn't sleeping well.  She began to tell me how she was going to have to change the Keflex and how she was not even sure what would work anymore.
She said that with every antibiotic given the bug just get stronger and that her fears are that soon we won't have anything to fight this bug. We know she is colonized and will have to do everything to keep her bladder drained. The upcoming procedure is the supra-pubic catheter. If this doesn't work, which I have been told there is only like a 10-15% chance, then the surgery I have put off for years will have to be done. This is called the vesicostomy. I have dreaded this procedure because Annabel will have a wet diapers all the time. She doesn't like being wet, never has. Also, from comments from other moms is that she could also wet her care givers.  The last surgery being explored is to remove the bladder completely. There isn't much finding on pediatric concerning this surgery but there is in adults. It will be a very difficult surgery as we have been told. We feel blessed to have this new Infectious Disease doctor who is young and energetic and doesn't mind seeking help from all over the country.

This past Thursday Annabel saw a doctor specializing in movement disorders. Annabel has been having seizure like activity for a while now. They have been on the increase in the last month and we had seen her current neurologist for these. Even though her doctor didn't see them in her office, we had supplied video. So a few weeks ago they ordered her an EEG while we were in her office. She was hooked up for about 2 hours and we believe what we thought to be seizure like activity did occur on at least one occasion. So the EEG was read and was report that Annabel was not having seizures. We were so relieved. I felt that this was due to some sort of pain in her abdomen or maybe her constant UTI's. They also seem to increase after she received her botox treatment for her teeth grinding. After sending videos to the doctor who had administered the botox and were told they didn't believe this to be a reaction to botox. This is how we  came to meet this new movement disorder doctor. So Thursday after showing her the video so wasn't really sure if it could be something called Sandefur's syndrome (basically reflux that has really damage your esophagus ) or that maybe it was truly seizure that weren't picked up on the EEG due to them being deep brain seizures. If anyone know me I won't medicate Annabel just because someone thinks something and I won't leave her on meds unless there is conclusive evidence that the meds are working. About that time Annabel's nurse told the doctor to look at Annabel and she was beginning what we call her seizure like activity and followed up with about 10 more. They doctor called these clusters and changed her thinking that it was Sandefurs syndrome. She is almost sure these are seizures after seeing eye deviations, muscle tone changes, etc.  As much as I was struggling with the fact that these could be seizures it came to my mind that maybe this was a God thing. God knows I would have to have black/white evidence to medicate Annabel with seizure meds.
The doctor began to review her EEG and said after seeing all the abnormal brain activity and the mis-firing that was going on in her brain she would be shocked if these weren't seizures. I told her how we felt sure she had one of these while hooked up a couple weeks earlier, and she told her she has had many patients that are truly having seizure but they can't catch them or that they are just too deep.
So I asked her what she would do if this was her daughter and she said she would begin meds right away. We talked about some of the side-effects and decided to begin on now that was a very small dose and after a week up it. It will still be a small dose. Then another seizure med that we will use when they are coming with so many a day. She is sleeping much of the day and this is what I dreaded but I have been told her body is having to get use to them.

Other than this Annabel has attended two vacation Bible Schools in the past two weeks. One was at our home church another was a Baptist church here in town. She had such a great time and her nurse was able to attend with her. I am so thankful for the new friends Annabel met while attending.

This is Annabel with some friends who came to visit.

She continues on with her speech, OT and her PT here in our home. Annabel is staying very busy and we will be spending some of our summer at the beach. We continue to be thankful for our same sweet nurses. Please pray for Annabel that her surgery will go well and that she can be on the smallest doses of these meds for seizures. We adore her sweet personality and I understand many of these drug can have an effect on the personality.

I am hoping to post very soon about Annabel's Make-A-Wish gift that maybe arriving this week. I think I am figuring out this computer finally.

Annabel waiting for her craft activity at VBS

Playing on her mat prior to therapy

Annabel on beach time....

A toothless Annabel

Isaiah and Rebekah came to visit with Annabel

We now have a plan...

Vesicostomy vs. Supra Pubic Catheter...that was the debate at yesterdays appointment. Our urologist is a great doctor but is sometimes hard to get him to respond concerning Annabel. We have been very frustrated in the past with Annabel's UTI's but when she had the central line they seemed easier to manage with antibiotic IV. She has been infected since December with E-coli and they say she is colonized. I have just begun to understand that she will not get rid of this bacteria ever. They explained that once you are colonized you will always be. UGH!!! So several years ago the urologist wanted to do a vesicostomy. This is a procedure done where a little hole is place in the lower part of abdomen. There urine will leak out constantly so that her bladder is always empty. This also means that Annabel will be constantly wet. I do understand the thinking that she must always be empty but I can deal with the fact that she will constantly be wet. So there were two other procedures that I had researched for information. One was called a mitranoff (sp) and the other was a supra pubic catheter. The Mitranoff is a much more invasive surgery and it would basically give her a hole high up on her belly with a cover on it like her g-button. Then we would still have to insert catheter to drain her bladder during the day. The supra pubic catheter seems to be the easiest option and seems that maybe we can empty her more often without her constantly draining and her staying wet all the time.  This will have a tube coming out of the lower part of the abdomen with a clamp on it. This way we can leave her open to drain while sleep or at home. But when she is at school we can clamp and empty her every hour. Also, she has become very aware when a new nurse come to learn to cath her and she doesn't want anyone there except myself or my daughter. She does let our main nurses do this but someone new is very hard. I am proud of her for knowing that this is a new person and that she knows she can't trust them yet.
Anyway, her urologist strongly suggested the vesicostomy. I knew I would not be able to decide this procedure until I see if this supra pubic works first.
We have tried so many antibiotics that Annabel is becoming resistant to many. We also have to be very careful with her tummy and don't want the antibiotics to tear up her belly so therefore she wouldn't be able to eat in her g-tube. We have also don't prophylactic doses of antibiotic and she continued to have breakthrough infections. We have done antibiotic washes to her bladder which she became resistant to these antibiotics. She has been dilated twice to help her bladder drain, which has not helped.  According to the doctor we have exhausted all possibilities. I am thankful he listened to my request as to the option of the supra pubic catheter. I ultimately believe he is right about the vesicostomy but feel I need to do this other procedure first.
So sorry that was so long. Our last appointment was to be fitted for her mask for the bipap study she will have as soon as she doesn't have this respiratory infection any longer.
Annabel continues to go to school most days. She also continues to have her in home therapy. We have decided that she will not attend summer school but I am signing her up for vacation bible school. We do hope to spend much of our summer at the beach.  

Stressing out....

I think when life becomes so crazy, I just leave. Of course, I mean concerning the blog. Let me begin by saying I have more help than before due to Kourtni who I have blogged about already. I was able to leave this past weekend to go on a knitting retreat with 5 wonderful ladies. Probably to some this would sound boring. But remember in my life, I long for boring! The couple who ran the bed and breakfast were so nice and accommodating. We shared many laughs, ate wonderful food, and slept as much or little as I wanted.

I had recently reported that Annabel was going to need a vesicostomy. I believe this procedure is a good one but I don't like the fact that she will stay wet all the time. I have talked with several moms and they all say the same thing. Very hard to keep your child dry, they have skin breakdowns (we don't experience this as of yet) and also many yeast infections. I have gone back to cathing her every 3 hours and many times during the night. I am using her feeding pump like an IV to keep water dripping during the night to flush her out. I have also gone back to doing the Gentamycin Irrigation twice daily. All this in the hope that we can keep as many infections at bay until I can be comfortable with the surgery needed.

This past Thursday we had a visit with a neurosurgeon that our Physical Medicine doctor wanted us to see. Annabel was never really considered to have scoliosis but she has a very noticeable curve to the left. So much so that her sweet little head goes right off the head rest on her wheelchair. She also had some changes in the upper chest areas. Almost as if the area is being pushed out. Her kyphosis is still there with changes also. So when we saw the doctor on Thursday he said surgery right away. He looked at MRI from 2007 and said she did have a tethered cord. The surgery is not a big surgery but he said could offer her some relief. It could help her little body straighten up and also help with the neurogenic bladder/bowel. In 2007 the MRI or x-rays showed almost no scoliosis but this months x-ray showed a significant increase. Her little ribs are almost closed on the left side. It broke my heart to see this difference in her little chest. All this said that she is probably in much pain and she doesn't even let us know. It is breaking my heart with so much changing. So much that I just don't even want to think about it. I don't like surgery, don't want to put her through it, but then again if it can help... All this to be said that God truly blessed us with a wonderful neurosurgeon. He was so positive and never questioned if Annabel was worthy of this surgery. He was thorough and informative. We are going to try to get another MRI(for updated purposes) but looks like they are scheduling for this in December. He said MRI wasn't necessary since he had one already so we may proceed. He had me sign surgery releases when we were there. He will need cardiac and pulmonary clearance then he will proceed.

After this Annabel had to have her button changed again. This is one tough little girl. Since Annabel's mini-one is a non-balloon button it has to be changed in the doctors office. Kourtni was with me, thank goodness. This makes me weak at the knees. They do not sedate in any way and she again did the no sound but tear running out of both eyes. The button is really great but we have been unlucky with the last two.

On Friday we had our cardiology appointment with our new cardiologist. She had recently had an echo and a halter monitor placed. In the last 6 months she had little change, which is good. She does have pulmonary hypertension but at this point is considered mild. Her large VSD is still there but it is working in her favor now so no surgery there. Thank you, Jesus! When the doctor called to report on the halter a few weeks ago he said that it was a very strange looking report and that he would have to consult with his colleagues. So at our appointment he listened a very long time to her heart. He said she has very high/highs and very low/lows but that these seemed normal for her. In my heart I know these are not normal but that this is something that is changing month by month. This was our first visit with the Houston doctor and we just need to be established and let them get their baselines. He asked if it was staying low/high like just minutes or more like hours. I said "oh no, just minutes". He said that was ok. Well of course, Sunday night it lasted for over an hour with going down to 30 and then above 220. Way more lows than highs. Then last night she went higher than ever which was 257 and then down to 31 for almost an hour. She was awake and this normally happens during sleep. She was also very restless, not moving but just couldn't relax. Finally, at 2 a.m. I disconnected her from everything. This is what is frustrating that I know something is going on, it is progressing but there is no help. I really did like this new cardiologist but he just doesn't know Annabel yet. Another thought is that Annabel's heart rate does go up when she is in mega pain. The only difference is that she doesn't follow or begin with the lows. One good thing is that Lance Armstrong's heart rate is sometimes in the 20's. He has a great ticker, right???

She also is having some airway issues. She doesn't have a cold or a respiratory but just trying to control her secretions. We have cut back on the meds to see if this will help. Sometimes a higher does doesn't work any longer and we have to cut back to try to thin the mucous.

I am more comfortable with the surgery for the tethered cord than doing the vesicostomy. Praying that this will help her chest/lung function, straighten her back to where she was and reduce back/leg pain, also play a vital role in her neurogenic bladder/bowel.

I am reminded that we have been given much time with Annabel. I considered it good time with such a happy, joyous baby girl. I think these are just bumps in the road and she needs a good tune-up. What I do struggle with is the reality of this terrible disease called Trisomy 18. The fact is that her little body is becoming weaker and her functions are slowing down. Please pray for Annabel to not be in pain since she cannot let us know what she is experiencing. All the specialist that have seen her as of late have commented on how tough she truly is. Well, we know that!

I need to thank my family who is so involved with Annabel. The older kids bring Annabel so much joy. My family also has been wonderful with Kourtni moving into our home and making her a part of our family. She has been great putting up with our crazy home also. I am so thankful for the relief and help that I have been given. Right now, I truly don't think Annabel could be loved on more! I also want to say that we MISS our soldier boy. Thank God he is still stateside, but I wonder what I will do come December when he is so far away.

Vesicostomy....

if you know anything or anybody who has experience with this could you please let me know...That is what sweet Annabel needs. She can't seem to stay well and is staying almost infected the entire time now. She has lost a pound in the last week and not wanting to eat. Nights are long as she is not comfortable. There is also possibly something going on around her surgery site that is causing much distention and she is in pain from this. She doesn't cry out with noise but today she just lay there on her quilt with tears rolling out the sides of her eyes.

Prayers and Thanksgiving...

I just talked with Brianna's mom. They had a scare with her last night. Please keep her in your prayers as they try to discover what is causing her spells. Brenda will be making her own post soon. It is such a reminder that with Trisomy 18 one minute they are fine and the next you can have the scare of your life. To visit Brianna's blog just click http//www.briannagiveshope.blogspot.com/

Also, in thanksgiving for Alyssa who has turned one year old today. To get thru this first year is huge. It never means our babies are safe, but it just helps defy those odds that 90% to 95% do not survive their first year with Trisomy 18. Please pray for Alyssa's family as they continue to cope with this diagnosis and the trial they will continue to face. To see Alyssa click here http://www.wndrfllymde.blogspot.com/

The following pictures are in honor of Alyssa's birthday. They ask that we wear T-shirt with money donated to two organizations and a sweet bracelet for Annabel and one for myself.

A PURPLE "A" FOR ALYSSA

T-shirts sold for Alyssa

Sweet bracelet that was also sold for Alyssa

It has been so nice to be back home. Last night all the kids were home and we made homemade pizza and most everyone helped. We all talked about our week at the beach and how excited we are to have my brothers family coming. He has two young children that we will get to spend the entire week with. My parents will also be coming for the week. Tara will have to work and will come up on the weekend. I am hoping my sister will take some time off during the week and come up for more than just the weekend. My nephew and his girlfriend will not be able to attend as the same for my brothers oldest daughter. We would love everyone to be there but we understand sometimes things come up.

We traveled to Houston today for Annabel's follow up appt. with her urologist. With the onset of the UTI last week and the IV antibiotics it was my hope that the decrease in her output into her diaper was due to the infection (pseudomonas). My hope was that when the antibiotics took care of the infection that she would have all those huge wet diapers back. Since we have been home the last couple days this is not happening. My homework from the appt. today is to catch her as soon as she wets her diaper and then cath her immediately to check her residuals. (With Annabel this is not that hard since she strains and then her diapers becomes so hot with the urine). So according to the doctor he wants less than 30 mls. for residuals. The last two diapers this afternoon upon returning have been 50 and 51. Each cath has been 120 and 102. With the infection gone(culture came back negative upon finishing the antibiotic) she still is not emptying her bladder. He wants me to check this 6-10 times and see if this is consistant. He did speak of the vesicostomy today and reminded me we knew the dialation was temporary. Actually, I knew that but thought we would get the 3-6 months out of it. But I don't want to forget how well she did during her surgery. God was totally incharge and He carried her along the way. My twins traveled to Houston with me today for her appt. On the way home as we were talking about what the doctor said and what we are going to do now to see what direction we will be going in with her. One of the girls asked the question "do you think what we are doing to care for Annabel is fair?" Also she said "do you think that we are making her have more pain?" It gave us a great chance to talk about how when I make these decisions that I am trying to make her comfortable for the time she is with us. If we weren't doing these things like, bladder/kidney, bowel, feeding tube placement that she would be very uncomfortable. It lead us to talk about bigger issues that could one day surface like her heart, kidneys or the possibility of Wims tumors(cancer) and we talked about doing things that would hurt her and possibly not change the outcome. Well it was deep but I was glad they were able to share what they were feeling. I think we all feel this and may not let ourselves share with each other. I will continue to whine about this thing I dislike so much, the monster called TRISOMY 18!

Tyler with Annabel

Tara with Annabel

Anyone ever had....????

any of the following procedures??? Annabel has continued to have too many infections with the clean intermittent catherization (CIC). Her last illness in the hospital is believed to be due to the constant use of antibiotics. It was a bacteria call c-diff and due to the severe dehydration she became terribly sick. Her pediatrician does everything possible to keep her off antibiotics but sometimes nothing else can be done short of another type of procedure. Two facts that are known is that Annabel has a neurogenic bladder and that her bladder is very large (due to the amounts of urine she retains).

Her pediatric urologist is proposing that she have a urethral dilation. This supposedly will allow her bladder to constantly drain the urine out into her diaper so she will always be wet. This procedure doesn't require an incision. But she would have to be put to sleep. The downside of course is that she will always wear diapers, smell of strong urine, and have a wet diaper. I have read on two accounts that this is an outdated procedure and that its success rate is not that high. This is the only procedure he will consider and when I question him about the other two procedures he just says he is only going to do the dilation. He says if it isn't successful the first time we can always repeat it. HUH???

There are two other options I would like to discuss but he just shuts me down. One is where an incision would be place in or near her belly button. I believe this is called a Mitrofanoff. It would have a button similar to her G-button used for feeding. We then would cath her through this button to remove her urine. It would surely provide her more privacy and people other than her family would not have to be involved in her private area. I realize the downside to this is the incision and of course she would be put to sleep. I have heard there is a high rate of success with this procedure.

The third option is a small incision in her lower abdomen area where it would allow the bladder to constantly drain into her diaper also as the dilation procedure. I believe this is called a vesicostomy. So the downside is the incision, wet diaper, smelly urine and always wear diapers.

I have been saying I would make a post about her necessary surgery that would be coming up. While in the hospital for the c-diff, bi-pap it was also found that she has a very large, loopy and lazy large bowel. It is becoming a dumping ground and leaves her full of air. This problem didn't just appear overnight but has become much larger than her last scan a while back. They say it must be removed. It is a major surgery according to her doctor. He says on some it may be possible to do it laporscopic but not what he will have to do with Annabel. I really like this surgeon as he talks with me about if I do or if I don't do something. I trust him if he says it must come out. Also he is willing to work with Annabel's urologist to have both procedures done while asleep and she will be open already. She will obviously suffer more pain from the bowel surgery and have a longer hospital stay. So the reason for not doing the later two procedures due to her having to have an incision become a mute point. She will also only have to be put to sleep once and not be administered pain medication at another time.

In the past we have had differences with our urologist. I have stated he is a great surgeon and that is important. But the fact is he doesn't see much use in Annabel's life (this has been said concerning her T18 diagnosis). I have tried to make an appointment with another urologist there at Texas Children's but it has always been 9or more months away. Yes, I should have just made it and waited. She really needs a urologist that is affiliated with a large Children's hospital with all of Annabel's other issues. A year ago when Annabel was in the hospital here locally and we needed answers I called him and he just said he didn't know what else to do. I ask him to please just tell me if he wanted us to seek another doctor (I don't like to dr. hop) and he said he would try one last thing. It did work for a while, but of course this was something I had been asking him to try for 8 months prior and he had refused.

This is where I see God's hand in all of this. I have been struggling with what to do for the last couple weeks. I don't know if I have ever been so emotionally drained as I have been feeling lately. I truly have been feeling I just don't know what to do for Annabel. So yesterday I looked up this other urologist who before was going to take forever to see. I just felt I needed someone to tell me that what her urologist is proposing is the best for her, or possibly one of these other options was better. At least to help me understand all of the options and how they would affect her in the future. I made the call and they told me we could have an appointment on May 20th. I truly couldn't believe it!!! I wanted to cry and I couldn't thank her enough, but of course, didn't want her to think I am emotion wreck. Her bowel surgery can't happen until at least June 9th. So I will be able to see this new urologist prior to the surgery and hopefully be at peace with whatever the decision maybe. The urgency to the urinary decision is that she can't really be on antibiotic to the degree she has been requiring to stay free of infection.

DISCLAIMER: Please know that I do not have medical knowledge past what I have heard, been told or read. I can totally misunderstand all or some of this. So please if the facts are wrong, I don't mind being set straight.

Whew, so sorry for the long post! With all this said if anyone has experience with any of these procedures or just information to share with me I would appreciate it very much. If you are not comfortable with making a post/comment on the blog then my email is suzyque92@hotmail.com

This post is not being made to say anything critical about any doctor. They all have their opinions and we have the right to go elsewhere. It is just so hard when you have a child with so many issues to begin over again. We have done this before, but it is best to not do it unless we have to. There is not a doubt that all our faithful friends will be praying that with the help of these doctors the right decisions will be made. When we have a definite date I will let you know so we can all be stormin' Heaven's doors with prayers.

And here I go again begging for prayers(I know I don't have to beg). Annabel has a new little cousin that is two weeks old. She was born and weighed only 1 pound and 8 ounces, 12 inches long. Can't even wrap my brain around how tiny this is! Obviously, there are lots of issue she will be facing but at this time she is doing as well as can be expected. I am trying to figure out how to link her on this blog but her site is caringbridge.org. Her name is isabellaryan and it is typed all together. They are a precious young family with a cute little boy also. Please lift them up in prayer that God will give them wisdom and strength to carry them through this time.