Journal

School is about to start! Britani will be going to the 2nd grade and Haley will probably be attending school also! Yes, she is only 2 and I'm not sure how we will tolerate that, but I think she will love it. We had a meeting last week with the school system and they offered all of her therapy services, plus classroom time as much as we want. We meet tomorrow to go over some more things with her teacher, the assistant, nurse, etc. We will then determine if we feel like she will enjoy school and how often she should go. Right now we are thinking about 3 days a week for about 3 hours, but we may change after we see how she like/dislikes it. Should definitely be interesting! We'll have to make sure all of her medical issues are being taken care of, and physically everything is done correctly, ie. carrying her, etc. Ever since Haley has been born, either Dave and I have taken care of her, or our parents (who have been "trained" on everything they needed to do...after much time). So, having someone totally different watch her and we aren't there to help, will definitely be hard, but mainly for us!! We talk about just letting her stay home this year, since she isn't yet 3 and she is learning a lot at home (like she already knows her colors and some letters and numbers), but she will get more socialization with those her age if she is at school. We are hoping that will make her want to do things like they do, like walk, talk, eat, etc. So, we'll see. Pray for her, AND US!!

Other than that, Haley is still doing well. She's still doing about the same things she was, but I think saying more and more each day. Well, I'm going to go for now, we meet at her school tomorrow, so we'll post how that goes soon.

Wow, tomorrow is AUGUST. I am finally sitting here trying to think of what to write and I can barely remember what we have done the last month. So, I guess this will be short.

Haley has been doing really well. As I read through the last entry it seems as if she is still doing the same things, but I think she is getting better at most of the things. Her eating is still pretty good. Some days she won't eat very much, and other days she eats very well. Her speech is improving a lot. Her vocabulary is expanding and most words are getting clearer. She is still scooting, but getting faster than ever. The last few weeks she has also been putting much more weight on her feet when we hold her. Today she was "showing off" for her Mawmaw and PaPaw and she locked her knees while was holding under her arms, then she bent her knees and pushed all the way back up. That is huge progress. Her legs are still pretty tight, but we are trying to stretch her as much as we can. We had some insurance changes this month, and she has only seen her PT once this month, so hopefully once that picks back up she will show even more progress.

We've taken a few short trips the summer. We went to Charlotte to visit family, but Haley caught a little virus while we were down there, so we didn't really do a lot. We've visited Nana and Buddy in Richmond and Haley and Britani had a ball up there. Haley is so fond of Samsun, the dog, so he keeps her occupied up there. We were also able to take Britani out to putt-putt, so that was fun. We also just returned from a 5 day trip to Gatlinburg, TN. That was really nice. Haley enjoyed seeing the mountains and she really loved playing in the river!

Well, one more month until school starts. We aren't sure what will be going on for Haley this fall, as we are waiting to hear on many things. First of all, the study she is on will be finished in 8 weeks. They will then have to decide whether her infusions should go back to every other week, or if she will stay on them weekly. Also, we are waiting to hear from the School Board on what they will offer Haley for the fall in regards to preschool, or just different therapies, like PT and speech. Then we have a choice on what we would like to do...so we will have to make some decisions. We are also looking into the idea of electric wheelchairs for Haley, which is a very hard thing to have to sit and think about. It really puts her disease into perspective and takes Haley from this perfect looking little girl to "the child in the wheelchair". So, that's difficult, but she is getting soooo heavy for us to carry, and it also would give her some independence. So, anyway, we've been looking in to it, but the one that I like is about $12,000. Who'd of thought that little chair would be so much?! Hopefully that would be something insurance would definitely take care of!

Well, I wrote more than I thought. Thanks for keeping in touch. I hope to put more pictures up soon (have I said that before?!). No, really please check back soon. Take care and God Bless.

I can't believe it's already the middle of June! Well, time is going by so fast and the summer will be over before we know it! Anyway, I've been meaning to write for a while now, but I guess we have been busy.

Haley is doing really well. Thankfully, she has not been ill lately. Physically, she is still doing about the same. She can scoot anywhere she wants, but not quite able to get on her hands and knees to crawl yet. Although, she is trying more lately, which is good. She has also started bearing a little weight on her feet, but that just started a day or so ago, so hopefully she will start to do much more. She really just locks her knees, but she is not able to stand straight because of her thigh muscles being very tight.

Eating-wise she is going great. For a while there I thought she would never eat, but the last week or so, she has been doing wonderful. Before, she gagged on anything that was chunky. Now she can tolerate it much better and the other day she ate a good amount (for her) of Alphabet soup. She also LOVES clam chowder.(Mostly the soup part, but she is getting brave with the potatoes.) And she loves Cheese! So, she is doing much better with the eating.

Haley is also beginning to say so many words. Most are hard to understand, but usually Dave and I can figure out what she is talking about. She also learned to count in about a week. The numbers are getting clearer, but she goes: "Un, oo, ee, or, iv, ix, even, eight, nine, en!" IT's so cute.

Today started the first day of Vacation Bible School at our church and I took Haley so she could be around kids her age. She had a good time, but she didn't really hang out with the other kids. She watched everyone sing, and on the way home she began doing the gestures to the songs. Some of VBS was outside and it was hot and stuffy, so we had to come back in and find stuff to do (find Britani to make Haley happy). We'll miss it tomorrow because we go to Duke, but hopefully we can go the rest of the week.

Haley also saw her first Movie theatre movie this past week (Kung Fu Panda). Britani and Laura were going to go with some family members and I decided to take Haley after I read the reviews of how funny it was. I thought Haley would laugh when other kids laugh like she does most of the time. Needless to say, it wasn't very funny and she got tired. She did stay in there about an hour, then we went to the lobby and she didn't want to go back in after that. So, we won't do that again for a while. (I did find out she loves popcorn-the soft part.)

One more "first" for Haley last week was that she swam in the lake! I was a little uncertain because of her G-tube and the lake not being the cleanest, but I taped it up so good that it stayed dry and she loved it! She stayed in a little float that looks like a boat, but she kicked her legs the whole time and didn't want to get out. It sure helped cool things off too!

We are so blessed that Haley is doing so well. However, we did hear some devastating news a few weeks ago. A 3 month old baby passed away from Pompe Disease. The family contacted us afterwards and told us that their family got lots of hope from Haley's story when their son was first diagnosed. Sadly, he only received one treatment and passed away from complications of Pompe Disease. The family then requested that in lieu of flowers, monetary contributions be sent to Haley. We were so touched by the generosity of their family and friends, however very saddened by the way this family came into our lives. They are obviously wonderful people and we have prayed that they find the strength to get through this extremely difficult time. Please include them in your prayers.

I shall end here. Thanks for all the prayers and the time spent checking in on Haley.

Just a quick update while I have a minute to write. Things are going well. Since I last wrote, Haley did have a short illness with fever and vomiting that delayed her treatment for a few days. But she did get over it fairly quickly. She also had a ECHO of her heart last week and that went well. The cardiologist said it looked normal (improving some from the last one) and she was very pleased. Haley was such a good girl while they were doing the ECHO, which is a big difference from last time they tried. We also met with some people from the school system this past week to talk about what services she may receive when she turns 3. Right now, Haley is able to get speech therapy at home through Early Intervention, but once she is 3 they refer her to the school system to get those services (and others like physical therapy and occupational therapy, and sometimes even pre-preschool classes). So, we'll see what that is all about in a few months because someone has to come do some testing on her to make sure she qualifies. She loved being at Britani's school though!

Haley is also picking up on so many words! We just need to work more on pronunciation so other people understand what she is saying!

Yeah! For those who don't know, Haley has not been able to eat for a year now. Everything she ate/drank was going into her lungs. They repeated her swallow study today and she safely swallowed all consistencies!

She was such a good girl and didn't "freak out" at all during the entire procedure. She didn't really like the food, but we were able to bribe her with ice. She loves ice and she would get a piece after she tasted the foods they wanted. She even ate a small piece of a graham cracker and did really well! We were so happy that she was such a good girl and also obviously so much stronger that she can protect her airway. From here, begins the process of teaching her how to eat again, since lately all she has been able to do is lick things and not really taste them. She is also not used to the taste of many things and that will take some time. But definitely a step in the right direction!!! Thanks for all the prayers!!!

Haley still continues to scoot around wherever she can and she is getting quite fast. She is getting more brave and will try to get from sitting to her hands and knees, but she mostly falls face forward (but slowly). Then she will ask for help to sit back up. On that note, she is saying many words now. I believe when I last wrote, a month ago, she had just said "Dada" which made it about 2 words she could say. Now I would say that she can speak about 15-20 words, although some are hard to understand (but we can usually tell what she means). She says things like: help, out, up, hot (when she sees a candle or flames), bye bye, bath, mess, home, house (for when Dave's at the firehouse), and will repeat words like: lemon, umbrella, in a minute, etc. There are many others, but my brain is too tired to remember them all!! I basically got in this morning from work, spent most of the day at Duke, and we head off to Duke again tomorrow for Haley's treatment. We also start watching our niece, Trista, on Wednesday for a few days a week. So, that should definitely be interesting and fun...Haley will be so happy to have a playmate!!

Well, I need to run, thanks for checking in. Hope to write again soon. Take care.

Hello everyone. I think 2008 is going by faster than 2007 did! I can't believe it is already March! We have been staying busy as usual. Same things as normal, hospital, PT, school, work, etc. The weeks really go by faster since Haley's infusions have been every Tuesday. We also try to fit anything else we can into that same day, so it makes for a long, long day. Lately, we have been doing speech and physical therapy either during the infusion,or right after. We don't get home until 6pm or so, but it saves us from having to drive another day in the week.

Haley has been doing pretty good. Luckily, we have stayed away from most of the "flu bugs" that have been going around. Haley had a really good January and February, I don't think she got sick at all. Britani had a stomach bug last week and I think Haley got the same one a few days ago, but it didn't last long. Dave and I BOTH had the bug last night, so it was pretty miserable around here. We are getting better today and I hope that's all that we see around here.

Physically, Haley has been doing well. She is scooting aroung the house now and tries to get into everything. We have to clean up Tubberware about 20 times a day. She tries to get into Britani's room any chance she gets. She'll knock on the door and if she gets in, she'll close the door too. Crawling may take some more time, but she is gradually getting better. She is trying to imitate many words and is finally saying "Dada!" She continues to use sign language for most of her wants, which is helpful. She still isn't eating, but we are working with her a lot, as they hope to do a swallow study the end of this month.

That's about it around here. We're still going through some crazy insurance issues, so please pray for us. And please continue to pray for Haley, as I can see how all the prayers have been working! Take care! (I also plan on putting pictures up soon. We've been having internet problems the last few months and it should be better now. Please check back!)

I sure didn't think it would be this long before I wrote again, but it's already 2008! The holidays were very nice, and hectic. We stayed busy with appointments, infusions, mild illnesses, and presents! For the most part Haley has had a good winter. She seems to be catching the bugs and viruses that last less than 24 hours, which is good. Nothing too major, so I can't complain. I've been off/on sick for the last month, but again, nothing major and I shouldn't complain.

Haley and Britani had a nice Christmas and we are still finding toys that haven't been played with yet, so we are very fortunate.

Haley is really doing so much more than she was many months ago. She is trying to imitate a lot, which is funny. She trys so hard to make sounds and words, which is a big improvement. She is continuing to learn sign language and sometimes I have no idea what she is trying to tell me. She gets kind of frustrated with us when she knows what she is saying, but we don't. Her fine motor skills are getting much better and she likes to put simple puzzles together. We are still working with her orally to try and get her mouth stronger so she will hopefully pass a swallow study in the coming months. She wants to eat so bad!

Strength-wise, I think she is starting to get stronger. We see things so gradually, but others seem to think she is getting stronger. Yesterdat, at physical therapy, she got on her hands and knees and actually kept herself up for a few seconds. A few months ago, she needed complete assistance. She is sitting very well and also trying to "scoot" to where she wants to go. Also at PT she rode a tricycle with much assistance, but she thought she was the biggest 2 year old around! Her feet and leg muscles are getting a bit tighter than they were but, after our appointment with the orthopedic doctor yesterday, they decided against casts again, and said to keep stretching her by hand. They said a last resort would be surgically releasing her "tight" tendons and tissue. But we will just continue to stretch her well so we hopefully won't have to go that route.

Other than that, just keeping busy with the usual. Glad things are finally getting back to normal after the crazy holiday schedules! I hope to put pictures of Haley from the last few months up very soon! If it takes a while, just email me and say, "Krystal, I'm waiting on pictures!!" It will probably remind me and hopefully won't take as long! Take care and I hope everyong is having a wonderful New Year!!

That's right. Haley turned 2 today. It's funny, because we keep telling her "Happy Birthday" and she is just looking at us like we are crazy. So, she doesn't really understand, and she can't enjoy any birthday cake, but we are excited. We are just so thankful for those that helped to develop the medicine she receives, because we know that without the medicine she wouldn't have made it to this birthday. So, it's a very special day.

To back track some, things have been up and down for the past month. Haley has been off and on sick. Mostly just having thick mucous which causes her to throw up after coughing. That lasted a week or so, then right after Thanksgiving, she got some virus or whatever that caused vomiting, low grade fever, increased heart rate, etc. She is just now getting over that one, after a few days and getting an antibiotic to help kick it. She almost didn't have her infusion yesterday, but the PA said to bring her on since she didn't have a fever at that time. However, once we got down there, she was out of it. She was doing okay when we left the house, slept most of the way there, and when we went to get her out of the car, she kept falling back asleep. We got to the room and laid her on the bed and she just kept rolling her eyes back and falling asleep, it was very weird AND scary! She had no fever and was just requiring a small amount of oxygen, so we gave her a little milk and they started the infusion. She started to "perk up" as the day went on, but still looked really tired. Anyway, I think she is finally getting over most of what she had, and hopefully she can enjoy the rest of HER day (once she wakes up from her nap.)

That's about it, I guess. Thanks for checking in! Hope everyone had a great Thanksgiving!

Everything's going well. Had an infusion, speech therapy, and physical therapy today. That's really about it. Haley continues to learn sign language, but she must slow down so that the rest of the family can learn. (So they know what she is trying to tell them!) Other than that, doing good. Quick note (for a change!) Take care and have a safe Halloween!

Things have been going very well. Actually Haley had her casts removed today! They decided against putting new ones on, so it only took 2 sets. I don't think her feet are at the stretch that they were hoping for, but she had some really nasty purple marks on her heel and the Dr. said that there wasn't much change from the first to the second set. So, we will try to put her AFO (braces) on to help stretch them more, and we will also have to physically stretch her feet. She didn't want us touching them tonight, but her feet were kind of swollen and I'm sure they were sore. But she sure was happy to get into the bath tub!!

The last infusions have all went well. The only thing I can complain about is the long day, but it really isn't that bad if Haley takes a good nap!

The speech therapist has been coming to our house about 2 times a week now and we are seeing some great progress. It's amazing how all of a sudden Haley has started picking up on things. You can definitely tell her brain is working fine! She has learned about 4 signs (sign language). Some of them, she will demonstrate after showing her once, and then she has it from then on. Right now she can do 'more', 'baby', 'all done', and 'water'. We are also working on a picture board to have her point to what she wants. Some times she picks both of the choices we give her, so that may take some time. We're also trying to get her mouth stronger because she just needs to pass a swallow study SOON! She wants to eat so bad! She watches us eat and smacks her mouth to pretend she is eating, it's kinda sad. So, hopefully it won't be much longer. That's about it for now. I hope to have some new pictures up very soon! Take care.

Haley has had 2 more infusions since I last wrote. Everything is still about the same, as we don't plan on seeing immediate results anyway. Since the last infusion, we have had to put casts on Haley's lower legs. The physical therapist recommended it, since we are unable to stretch Haley's feet and ankles, because she really dislikes it. It's called serial casting. They put on a cast that gives a gradual stretch and then they change it a week later to stretch a little more, and so on. They will continue to put new ones on until her feet get to the point that they aren't "tight". They figure that now is the best time to do this, since she really isn't putting weight on her feet. It looks like she broke both of her legs, so it's kind of sad. She's doing a little better with them now, but she still looks at me and points to them, like "Take these off!" The first night was kind of rough too, since she would fuss and try to kick them off in her sleep. The other bad thing, is that she is unable to take a bath, which she loves! So, we will all be glad when these are finally removed, anywhere from 3-8 weeks. I will hopefully add new pictures soon of her in her "new shoes".

So, that's basically all that's going on right now. Just dealing with many other appointments related mostly to physical, occupational, and speech therapy. Starting next week, a speech therapist will be coming to the house twice a week. She will try to help Haley's swallow get stronger, and also work on ways to help her communicate.

Thanks for checking in, and also for the continuous thoughts and prayers. Take care.

Since I last wrote, we got the news that Haley would be in the clinical study in which they will double the dose of medicine she is receiving. She had her first infusion, under the study, yesterday. It was basically like a regular infusion, but we had to go to a different part of the hospital. It's a floor that only does clinical research on many different things. Anyway, she was randomly picked to be in the group that goes weekly. That means she will get (close to) the same amount of medicine she had been getting, only she will get it weekly, instead of biweekly. There are only 12 people in the study (kids and adults). I believe there are three that are getting their infusions at Duke, but we didn't get a chance to meet anyone since yesterday was pretty hectic. We got there around 8:30am, and didn't leave until 5pm...very tiring.

They told us about a week ago that Haley was accepted into the study, but that they also needed some tests before the study could start. We had to go for another ECHO and that turned out to be awful. The day before the ECHO, Haley had a check-up with the Pediatrician and had to get two vaccinations. She was not very happy after that and didn't want the nurses to come near her. The next day we went to Duke for the ECHO, and as soon as we got into the room, Haley started to freak out. We tried to lay her down and start the ECHO, but she didn't want anyone to touch her and she was screaming so hard that she was getting more snotty and mucousy by the second. She ended up coughing really hard and brought up this big glob of really thick mucous and then she was unable to breathe. She turned almost completely blue and they were calling the Dr. in there while we were trying to suck the mucous out. The bulbs and catheters we were using wouldn't get it out because it was so thick. We ended up getting it out and then she was much better (and pinker). By then, she was exhausted. We decided to try to get her asleep and then we'd try the ECHO again. We strolled her around for about an hour and she would not give in. She was seeming happier, so we took her back, but left her in her stroller. As soon as they would come near her, she would start screaming again. It wasn't even a loud scream; if you weren't looking at her, you wouldn't even know she was crying! But she had tears rolling and her face was really red. We decided that we just weren't going to get that ECHO today.

After that appointment, we had to go to physical therapy. Even though Haley was exhausted by that point from crying so much, she did really good (she was used to the therapist). Her therapist really just had to fill out some paperwork answering what Haley could and couldn't do. I guess just to show a baseline before the study started. She slept the whole way home.

A few days later, we had an occupational therapy appointment in South Hill. That was going pretty good until a lady walked in with some machine, and Haley immediately turned towards me and started the silent screaming with lots of tears. As soon as the lady left, she was fine, but each person that walked in, she was watching them like a hawk, and screaming at some. We eventually had to move to a different room.

She pretty much did the same thing yesterday at the infusion. She was really afraid of the nurse and didn't want her to touch her. They had to weigh her and check her length three times for each to be accurate, and she was screaming the entire time. It also took a while to get her port accessed, and she wasn't happy with that either. She did the mucousy thing again, but this time it came out much easier. She ended up getting used to the nurse (thank goodness!) because she stayed in the room almost the entire day. We had three othe appointments that afternoon, but we didn't get to any other them. Actually, PT was supposed to come see her around 12:15, but she couldn't make it. Then, we had a speech therapy appointment at 2:00 and didn't make it to that because her infusion didn't start until almost 11am (had to wait for EKG and for her to pee first). Then we thought we would be able to leave 30 mins. after the infusion stopped, but they told us around noon that she would have to stay for 2 hours after each infusion for at least 6 months. Then we had an appointment at 2:45 that had to be cancelled. She was going to see the Orthopedic Dr. about her feet. The physical therapist wanted her to see him and they are also thinking about casting her legs to help them stretch out better. So, we had to reschedule that for next week too. Anyway, that was the end of our hectic hospital stay, we were glad to get home! Hopefully, next week will go much more smoothly!

Other than that, Haley had been doing pretty good. She still has a lot of thick mucous that is causing her to cough and need to cough a lot. Sometimes that will make her throw up, but other than that, she is doing pretty well. She is sitting up for a good while now, and beginning to put more pressure on her hands while sitting. She is such a drama queen and is really started to mentally pick up on things. We have some books, etc. that we will ask her to point to the giraffe or ladybug, etc. and most of the time she will be right (if she's in the mood!) She will also lay on the floor looking for any small speck of anything to put in her mouth. If we tell her not to, she will put her finger in her mouth and pretend to chew something. Also, if we sternly say "No" to something, she thinks it is really funny, in turn causing us to laugh! Basically, she's got us wrapped around her little finger! Well, better go for now! Take care!

Sorry it's been so long. I actually planned on writing sooner, but a lot has been going on lately. After I last wrote, Haley had an EMG and a pulmonology appointment on the 30th. The EMG (electromyography) was early that morning and was not fun, to say the least. They also did a nerve conduction study. Basically they are trying to see how her nerves respond to stimulation and the EMG measures the electrical impulse of the muscle at rest and during contraction. The lady that did the nerve test said everything looked good. The doctors that did the EMG said that they will send their report to Haley's doctor. So I actually haven't heard anything from that, but I think it's a test that they just want on file for the kids with Pompe Disease to show the progression of the disease and the effect on the muscles. But to do the test, they stick a (small) needle into different muscles for approximately 15-20 seconds. They did her arm, leg, and the back of her neck. She was not happy at the end of that appointment. After that, we had a few hours until the next appointment, so we just ate and cruised around.

The next appointment was speech therapy where Haley got to drink more water from a spoon. She was also able to try some peaches and pears. The speech therapist thought that Haley was ready for a swallow study, so we scheduled that for the next week.

Next was her pulmonology appointment. She had a pulmonary function test (PFT) which is never fun. They basically want her to cry and scream into a mask to measure her lungs during inspiration and expiration. The doctor was pleased with the results and said they looked better than the last time. So, he changed one of her nebulizer medicines to 'as needed', instead of around the clock.

The next week, school started for Britani. She didn't have homework all week, so school was easy and fun for her. We had a few more PT and speech therapy appointments that week and we also met with a lady and her daughter that were in town from Rhode Island. She also has Pompe Disease and reminds me a lot of Haley. (I believe there may be a picture of her on the gallery page-her name is Mia.) Anyway, it was really nice chatting with her (and her mother-in-law) and letting Haley and Mia interact.

On Friday, the 7th, Haley had a swallow study. She didn't do so well. To begin with, she slept on the way down and I'm not sure she got her entire nap. After that, she was very unsure of the radiologist in the room. Not to mention that everyone in the room had on these huge vests that had to be scary. Anyway, she swallowed some thin liquids, but they silently went into her lungs. We tried something a little thicker and it did the same thing. By time we got to the thicker substance, she was very upset and sobbing. She never would take a swallow, so we stopped the test there. So, basically we still can't give her anything to eat or drink until another test is done, whenever that may be. My concern is that I feel she will almost always be scared of that room and not give us an accurate test. Hopefully this very independent and controlling stage will soon pass.

After the swallow study was the 29th Annual AGSD Conference (Association for Glycogen Storage Disease).We stayed in Durham on Friday night for the conference (with my Mom and Britani) and were able to get to know some other families with similar diseases and spend more time with Mia's family. (Pompe Disease is Glycogen Storage Disease type 2. There are many others) Britani got to meet and spend time with kids, some of which where affect by a GSD, and some that weren't. There were many well known Doctors there that gave some great presentations. The doctor that actually "made" Haley's medicine was there----I wanted to yell THANK YOU SO MUCH! Because when you really think about it, without him, Haley wouldn't be with us right now. I am so grateful to him and the team that helped him.

Anyway, to get back on track, we came home on Saturday, I worked Sat, and Sun. and inbetween that time, Haley began to get sick. We were very worried that she was getting pneumonia, because that's what happened after the last swallow study she failed. We watched her closely, because basically she had every symptom, except the high fever. We ended up getting an appointment with her pediatrican on the 10th and also got a chest x-ray. That showed that she possibly had viral bronchitis, bronchiolitis, or pneumonitis, but not pneumonia. The Dr. said her ears were a little pink, so she gave us a prescription for an antibiotic to help "kick out" what's going on. She's getting better, but she still has an awful cough and that causes her to vomit and then feel yucky for a while.

On the 11th, we had her Infusion and everything went well with that. We heard a little more about the study. It's looking more like she may get in, so we signed the consents, and we are now waiting to hear if she 100% got in, and which random group she will be in. Ya'll please pray she gets in this study, as I am having awful insurance issues, and if she makes it in the study, then her medicine will be free for the next year. I pray what happens, will happen for the best, either way.

Well, that's it for now....told ya we've been busy!! Better run...school bus comes early.....

Haley had her infusion today, along with a Cardiology appointment. The infusion went well and the cardiologist told us that her last ECHO looked great. She didn't go into specifics, but said her heart function looked good and that her heart was still a little thick, but close to what's normal. She took her off one of her heart medications and told us to wean another one of her heart medications down next week. We will do that for about 2 months and then she will reevaluate her to see how she is doing. So, good news!

They told us today that they weren't sure about Haley being in the clinical study where her dose would be doubled. They need to submit some more information to Genzyme and they were being especially picky, since only 12 were being chosen. Initially, Duke had thought they would give the names of those they wanted in the study and there wouldn't be a problem, but they are finding out otherwise. I think Haley is doing better than most people that they are chosing for the study, which is great, but it is also keeping her from the study. It's weird, a month ago, they told us her next infusion would probably be under the study, now we're not sure if she's even getting in it. I pray that whatever happens will be for the best.

Other than that, Haley's had a few speech and occupational therapy appointments. They are still saying to wait a few more weeks for the swallow study. We are giving her water by spoon to get her mouth muscles stronger and teach her how to swallow. Thursday will be a day full of appointments, so I'll be glad to get them over with!

That's about it. School starts, for Britani, in ONE week. I can't believe the summer's gone already!

Hello all. Haley had her infusion yesterday. Everything went well. She actually had the infusion plus 3 other appointments in the same day. Before they started her infusion, she had a hearing test. They said everything looked normal. She was pretty happy during the test until some dancing animals scared her, then she was afraid. Next was a speech therapy appointment. During that appointment they evaluated her feeding abilities. They gave her some water with a spoon, which she loved. She got a taste of some bananas, which she made a funny face at and then wanted more water. They said we could do a trial with the food and give her water with a spoon and also try small amounts of baby food. However, they called today and said to wait on the baby food and continue the water. They hope to get her mouth and throat muscles a little stronger, and repeat the swallow study in the next few weeks. It's weird because when she first failed her swallow study it was very hard to picture her not eating and drinking. However, I think over time we have got so used to her not eating that now it is strange to feed her. It's almost scary to feed her, when before we never thought about it that way. Anyway, hopefully soon she will be eating.

Next, Haley had an ECHO. We're not sure yet of the results, but will hopefully find out soon. We have an appointment in 2 weeks with her Cardiologist, so we'll definitely know by then. We are hoping to hear that she can come off of her cardiac medicines, which will be nice. That night we actually stayed in a hotel in Durham. It was nice because they gave us a 'medical rate' because of Haley's appointments and also a company, called PSI, reimburses for lodging related to appointment dates. It was nice to be somewhere else and not have to worry about cleaning or doing this or that around the house. Haley and Britani also got to swim in a pool, which they both LOVE. They didn't want to get out. We really enjoyed staying there, however it was almost more hassle to pack and unpack everything but the kitchen sink for only one night. Haley was also really bored and fussy inside of the hotel room. Not sure if we'll do that again.

The next morning (today) we got up, ate breakfast, and went to Haley's physical therapy appointment. Haley hadn't seen the physical therapist in a little while due to scheduling conflicts, so she was impressed with how well she was doing. To begin with, Haley sat on her own for almost 10 minutes. Before that, we could only prove (via stopwatch) that she was sitting alone for like 2-3 minutes. This time she was trying to turn her body towards a toy, while sitting. She got on her hands and knees for only a few seconds, but showed more strength when doing so. She also tried to ride a tricycle. She liked it at first, but then got a little tired and was done. Anyway, her therapist was very impressed with her today.

When we got home, we unpacked, hung around, and then we were invited to go swimming at a family members' house down the street. Again, Haley and Britani swam like little fish. You know, it's really good therapy for Haley and I think insurance should approve us putting one at our house, don't ya think?? =)

Well, next we came home, got cleaned up, and waited for the phone NOT to ring. (Tonight I'm on call from 7pm-7am.) That's about it. Hopefully I'll have more news soon. Take care!

One last thing, if you are reading this PLEASE write in our guestbook. I'm really interested in finding out who's checking out this website and keeping up with Haley's progress. Thank you so much for taking the time. Haley is so lucky to have such wonderful family and friends. Deep down, I know Haley knows how many people are thinking about and praying for her. Thank you.

Woah! This is the longest I have gone without writing in this journal. Thankfully, things are going really well. Haley is having a good summer. We are all staying busy and healthy. We were able to go the beach for 6 days with my family. Haley loved the waves! Britani's been busy-actually right now she's spending a few days in Richmond with my mom (getting spoiled before school starts). We'd love to get outdoors more, but this heat is awful!! Hopefully soon Haley can spend more time out there.

Haley's feedings are going pretty well. She is still only getting milk through her G tube, but we hope to have a swallow study in the near future to determine if she will finally be able to eat and drink. We actually have a lot of appointments coming up, from cardiology appointments to something called an EMG. (They have to stick a needle into her muscle and it determines muscle function somehow.) They say it's painful. She had one at MCV a long time ago, but I know she doesn't remember it and actually we stepped out of the room, so neither do we. But hopefully we can get that over with, along with all of her other appointments. At one point, we were scheduled to go to Duke 3 times a week. But now we are trying to rearrange appointment times so that they are more spread out.

We are also still waiting to hear if Haley will be accepted into the clinical study in which they will double her medicine. We've actually been waiting since the beginning of the year, but Duke said they have also been waiting for approval from the company. During the last infusion, they mentioned that her next infusion could be the start of the study; but we haven't heard anything yet.

Haley's really beginning to look like a little girl. Especially with the tube off of her face, it's hard to even tell something's wrong with her! She's also been picking up on things a lot more lately. Most of the time she will follow instructions to what you say like, "Push the button," etc. She's saying Ma ma a lot more, even though I'm not yet sure if she's just mumbling. Sometimes she will say it and look at me and smile, so I think she knows what she is doing. She is still rolling on the floor to anywhere she wants to go. She is trying harder to get on her knees, but the arms are the problem, even though they are better than they used to be. She is also sitting for a long period of time, but we still have to stay right beside her, cause she'll flop over in a second.

Well, I guess that's all for now. Thanks for checking in. I hope to write sooner next time. I also plan on putting more pictures in the picture gallery very soon!!! Take care!

It's been a little while since I have written. We got home from the hospital on the 8th. Haley was vomiting some right before we left, but they still let us go later that evening. She was rattling a lot and every time she coughed hard, it made her throw up. I convinced the doctors that Haley does this often, so they would know it wasn't surgery related. Since the surgery, Haley has continued to do that off and on. It got better for a few days and then she started with some sort of bug on Saturday night. On Sunday, she threw up many, many times, and looked awful. She also needed more oxygen and had a mild fever. With all the throwing up she did, we were just hoping that she didn't aspirate anything, which could give her pneumonia again. After talking to the PA at Duke, we were going to take her to the ER that afternoon, but right after talking with her, Haley went to sleep and her need for oxygen was better. We also started Pedialyte at that point and that helped her not to throw up so much. Today she was a little bit better. She didn't throw up any, and she also went some time without using oxygen. The only thing was that she still looked terrible. She only smiled occasionally and laid around and slept most of the day. Her infusion for tomorrow is postponed and if she still acts sick tomorrow then we will take her to the Dr.

Other than that, we are still trying to get used to her G-tube. I don't think it's bothering her as much as it used to, but it's still different. Hopefully over time we will all love it. Sure looks nice not to have anything on her face!

Haley's tube on her face is finally gone!! She had her g-tube placed yesterday and everything went pretty well. We've been in the hospital since Tuesday morning, so we're ready to come home! That should be happening tomorrow as long as everything continues to go well. She was very groggy and fussy yesterday after the surgery, but she slept most of the afternoon and night, so I think that helped. We took her strolling to see the fish a little bit ago, and she really liked that. Britani will be coming to see us tonight, so I'm sure Haley will be thrilled about that. Well, just thought I'd give a quick update. Take care.

We've had an extremely busy weekend with 2 fundraisers for Haley. Both turned out excellent. I hope to be putting more information soon on the fundraising page. I wish I could get it all done today and also add the pictures that I took, but we are getting things together for our upcoming hospital stay.

Well, not much to say, other than please pray for Haley as she undergoes surgery and hopefully will recover quickly. We hope to keep everyone informed as much as possible. Please write in the guestbook if you haven't already. I love to hear from people, it really warms my heart. Take care.

Well, it's been a couple of weeks since I have written. Everything has been going pretty well. Thankfully, everyone has been healthy. Also, on Mother's Day, Dave graduated from college!! (Yeah!) It was a wonderful day and he graduated with Honors with the 3rd highest GPA in the graduating class.

Haley also had her 24th infusion on May 22nd. Everything went pretty well and they also gave us a date for her surgery. We will have her next infusion on June 5th and then she will be admitted that night for pre-op stuff. She will get her G tube the next day, on the 6th. That's IF between now and then she's stays well. So, we are just praying she will stay healthy for a while. (a long, long while if possible!) We should go home the day after the surgery if all goes as planned. Well, that's all the news I have for now. Thanks for checking in! Take care!

Haley had her 23rd infusion yesterday (finally)! She was a lot more active today, which was great. After the infusion we had to get a holter monitor for Haley. Basically it's like an EKG, but it records the data for 24 hours. So, it was kind of tough to let her do too much today because she was hooked up to lots of wires, with a machine that we were not supposed to move or bump much, Ha! Good thing it was only for 24 hours. At the infusion, they didn't mention much about the G tube operation, only that she needed to get back to normal from being sick. I was hoping we could go ahead and plan a date, but no such luck. She even said that it may be June or July, but I mentioned that the sooner the better. I don't want to spend a lot of the summer in and out of the hospital doing procedures, especially with Britani out of school and vacation (if we're able). So, I guess we will know more later about that.

Britani was better today. No fevers or vomiting. Although she was still a little tired. She stayed at home today and got some rest and energy and also worked on schoolwork/homework. Hopefully she'll return to school tomorrow after 3 days off. That's it for now, I guess. Hope everyone has a great week and a wonderful Mother's Day this weekend. Happy Mother's Day to all those special mothers out there!!!!

Well, it's been an interesting few weeks. Since I last wrote, Haley kept a fever that would come and go and she eventually started with the vomiting and was barely able to keep anything down. When she started acting like she did with the pneumonia a few weeks ago, we were very concerned. The doctors did a Chest x-ray and that showed that she didn't have pneumonia, but labwork (and the fever) proved that there was something going on, we just didn't know exactly what. She was put on antibiotics and steroids last Thursday to help her get over the "bug". Anyway, she was pretty weak and lazy for awhile because she wasn't getting very much nutrition, and I'm sure not having her infusion was causing some of it also. As soon as Haley started to perk up some, Britani started with her "bug". She started a fever on Saturday afternoon (right before I left for work) and Sunday morning she woke up still with a fever and also not being able to keep anything down. At one point Sunday, her temperature was registering between 105 and 107 on my temporal thermometer and about 103.5 rectally. During that time, she was acting okay, but extremely hot. Motrin and Tylenol were bringing the fever down some, but only to 100-102 degrees. After consulting with a doctor before I had to, once again, go to work, he said to keep giving the Motrin and Tylenol and bring her in the next day if the fever continued. He also said he was seeing a lot of the GI "bug" and it caused extremely high temperatures. We took Britani to the Dr. this morning and everything checked out okay, so that meant she just had a nasty virus and there was nothing she could take to kill it. She's gotten a little better today, but still having high fevers occasionally and just feeling pretty crummy.

Well tomorrow we are planning to have Haley's infusion that she missed. She's not back to normal, but she definitely needs to get her medicine. I hope that will perk her up some. We are also supposed to talk with PA and Nurse Practitioner about Haley possibly getting a G tube (gastrostomy tube). It would be surgery, but it will be a tube that comes directly from her stomach for us to give her nutrition and that way we don't have to stick the NG tube down anymore (yeah!). It's definitely a better way to go, but of course her going for surgery is scary. We'll see tomorrow if and when that can be performed. Please continue to keep our (entire) family in your prayers. Thanks so much for everything.

Today was supposed to be Haley's 23rd infusion, but it had to be postponed because Haley had a fever this morning. It was weird because Britani had a fever yesterday and I was checking on her this morning to see if she should go to school. Before I went into Britani's room, I looked in on Haley and saw that her heart rate was up a lot for her to be asleep. So I felt her and she felt pretty warm. Britani was just fine and went on to school. When Haley woke up she vomited some, so between that and the fever, she didn't feel well. We talked to the Nurse Practitioner at Duke to let her know what was going on and she said we will try to get the infusion rescheduled for next week. Haley spent most of the morning laying on me, but by noon she was feeling pretty good. The rest of the day she seemed normal and happy. Dave and I joked that she wanted to play hooky today. Well, we hope and pray next week will be better so that she can get her infusion. Thanks for checking in.

GOOD NEWS! Haley had an ECHO of her heart yesterday and it showed improvement from the last ECHO! The cardiologist said that back in August her heart was functioning very poorly. Her ECHO in January showed that it was moderately functioning and yesterday the Dr. said that Haley's heart function was on the low side of NORMAL. Just to hear the word 'normal' is so wonderful when you look at what she was at last August. She also said the heart wall was thinning, which is great because it was very thick. She thought she looked well, and we were excited with the news. She also had a bone survey yesterday, which is where they take x-rays of all her bones. We'll hear more about the results of that later, but you would have thought they were poking her with needles the way she was screaming during the x-rays. Nothing was hurting her, she just didn't want to lie down in that room and when they needed like 7 x-rays, she just got angrier the longer it took. She slept very well after that.

Haley also saw her pulmonologist last week and he also thought she was doing well. Her lungs sounded good and he also repeated an x-ray to make sure the pneumonia had cleared up. We didn't hear anything about the results, but he said not toworry unless he called. The only bad news I guess from the past 2 weeks is that Haley had a few days where she had a low fever and was vomiting quite a bit. Of course that worried us because we were scared she was going to get pneumonia again, but so far she's doing well. We hope everyone's enjoying the nice (hot!) weather! Take care.

Well everyone, Haley had her first hospital admission since last July. The good thing is that she has gotten through the fall and winter without an admission. The bad thing is that she had pneumonia and was at Duke for 4 days. Last Tuesday she woke up vomiting and had a mild fever and upset stomach. We just watched her really close throughout the day, but then overnight her temperature got higher and she started needing more and more oxygen. We were suspecting pneumonia at first, due to her just failing her swallow study, but her temperature wasn't really as high as you'd expect. The next morning on the 4th, we called the PA at Duke and updated her on how Haley was overnight and she told us to go to her Pediatrician to get checked. She pretty much slept on the way to the Dr. and by time we got her weighed, etc. she looked just awful. Her fever was over 100 and she was just lying around looking like she was asleep, but she was just spaced out. If you lifted her arms up- they just dropped. The nurse took cultures to see if she had the flu, RSV, or strep, but they were all negative. Next, was to do an x-ray, but by that time, Haley looked really bad so we decided to hit the road to Duke. We got to the ER there around 11:30 am and were seen by the nurse a little after noon. We saw several doctors (residents, interns, attending) and they decided to do some blood cultures, urine cultures, IV, antibiotics, and an x-ray to start off with. Her temperature had went down on it's own on the way to Duke, but by time we got in a room in the ER, it was close to 103. She got some Tylenol and her tests began. After her x-ray came back, we found out that she had pneumonia in her lower left lung. They said we would be going up to the Pediatric floor but it would take a while. We got to the floor around 7:30 that night and she pretty much slept that whole day and night (and was restless some). They weren't giving her any milk at all in case she threw up again, but they had an IV going, along with antibiotics. She was also requiring about 1.5 liters/min of oxygen. We stayed until Saturday night when they told us that morning we could either stay until Sunday or Monday, or leave that night if we felt comfortable. We decided that she was doing much better and that we could do all that they were doing for her at home, so we left that Saturday night and came home. They changed her antibiotics to go in her NG tube and we got home in time for the Easter bunny to come! The bad thing is that this week is Spring Break and on Monday (the 9th) we had planned a vacation with my father, stepmother, and sister to go to Washington DC for 3 days. We had booked the hotel room a few weeks ago, and even though we knew there was a possibilty Haley (or anyone) could get sick, the rooms were non-refundable (and much cheaper!). So, Dad, Kathy, and Laura took Britani and they went to DC on Monday (and met Kathy's mother and nephew there.) We've been checking up on them often!! It's really awful being here at home after getting so worked up about finally getting to get out of the house and do something, but Haley's getting better (and DC is really cold right now) so, I'm thankful for that. Well, I'd better go. We have a Drs. appointment in the morning to follow-up after leaving the hospital, but she's doing much better. (Hopefully she won't have Strep again!) Take care everyone and thanks for the continuous prayers. They definitely helped because pneumonia usually causes the little ones with Pompe to need a ventilator. Thanks so much and keep them coming!

Haley had her 21st infusion today. She weighed in today at 21 pounds and 6 ounces. She fought sleep most of the day, but the infusion went well. We also had the swallow study this afternoon, and that didn't go so well. It showed that Haley was aspirating the thick and the thin liquids. So basically everything they put into her mouth, she was sending to her lungs, instead of her stomach. It didn't help much that she got really mad during the test and was pretty much screaming the entire time, which could have been the reason she didn't swallow the way she was supposed to. So now we have to stop all of the bottle feeding and the baby foods. They did say that we could try some water every now and then, which at least won't take the bottle away entirely. They probably won't repeat the test for 2-3 months, so this is going to be a big change for Haley, and us. You never realize how comforting that bottle of milk is until it's gone. And Haley was finally starting to sit at the table with us and enjoy some baby food. Well, that's my news for the day...wish it were better. Will try to write again soon. Thanks for checking in on us!

Well, it's been almost 2 months since I last wrote! Sorry for the delay, but life's hectic as usual. With Dave student teaching, the Hayes' home is very chaotic, but thankfully everyone is doing well. I'm having to look through my calendar to see what has gone on since I last wrote! Nothing too exciting has happened, but Haley had her sleep study the middle of February and we are waiting to hear about that. She's has been doing okay with oxygen lately. One week she may not need it at all and then next she will sound a little congested and need oxygen every time she sleeps. So, we should know soon about the sleep study results, just praying for good news. Haley's been having a lot of physical and occupational therapy lately, both at Duke and at home. I think she is doing well with that. She's sitting for longer periods, still unsteady at times, but doing well. She's rolling a whole lot and trying to pull her knees up under her, which we are thrilled about. We hope that's a sign that she'll want to crawl soon. She's just got to get those arms stronger and learn how to do it. Haley also got a piece of equipment, known as a stander. It's hard to explain it, but it puts her body in an upright position to help her bear weight on her legs and feet. I'll try to post a picture soon.

We've been through infusion numbers 18, 19, and 20. They all went well. They did take labwork a few months ago to check for antibodies to the Myozyme and it did show that her body was making some antibodies. They told us that the number they go by is still low, to not worry, but they will test it every now and then to see it (hopefully) going down. (When some kids have high antibodies, their bodies are pretty much trying to "attack" the enzyme in the medicine and most of the time it causes reactions during the infusion.)

The girls did go through the whole "norovirus type" symptoms about a month ago. Britani had it first and only missed one day of school. Haley got it about a week and a half after Britani and was sick for a few days. They both did well though, and seemed to get over it quickly.

This week we went to the pediatrician with Haley for her routine vaccinations and ONCE again, she was positive for Strep throat!! Yes, this is the 3rd time in the last few months. She really didn't have any symptoms, only a red throat. She's being treated with antibiotics at this time and, of course, has the lovely upset stomach that it causes.

We've been enjoying this wonderful weather lately. Haley loves to go outside, so we have been strolling down the street some. She gets so tickled when she spots a cat. She would like to stay outside all the time, but the sun and the allergens in the environment begin to bother her eyes because she's not used to it.

We have infusion #21 this Friday. My dad is going with us. (Because I need a driver, his truck, and an extra set of hands. It works nicely.) My mom went to the last one and it's been nice to show everyone where we spend our time every other week. This Friday we also have a swallow study scheduled to make sure nothing she swallows goes to her lungs. They've been a little concerned with the rattle she has in her throat lately. Hopefully she'll do well, because if she doesn't, she won't be able eat or drink anything until she gets stronger and passes a swallow study. Please pray for her. (As I know so many are doing, thank you....)

Well, that's all for now. I won't try to wait so long to write again. Oh! Dave will be finished with his student teaching FRIDAY!!! Yeah! He may substitute some for the school, but his school will basically be finished. He will graduate in May!

Well everyone, finally we have wonderful news!! Haley had an ECHO of her heart on the 31st of January and we got the results during her infusion on the 2nd. The ECHO showed much improvement in her heart function!!! Her left ventricle mass, which was FOUR times the size it should be, is now TWO times the size it should be. Also, in the ECHO previously, her heart function was classified as "severely decreased function," it is now classified as "mildly decreased function"!!! Finally some proof (on paper) to show the Myozyme is helping. We know that without the Myozyme she wouldn't be where she is right now, but it's so nice to finally have some proof! They are expecting the heart to continue on this path and eventually she will have more room for her lungs to hopefully work a little better for her. (ie, less oxygen and easier to clear her congestion) Her infusion also went well on the 2nd and so far Haley is doing okay the last few days (beside a few episodes of vomiting). Well, hope you enjoy the good news as much as we did!!! Take care!

Finally, I get a minute to sit and write about the last month! Can you believe it's almost February?! Well, I hate to cram all of the events that have happened this month into one date, but I'll try to be short. =) Haley had her 15th infusion on the 5th and that went well. We took her back to her pediatrician on the 11th for her immunizations that have been put off for 2 months. She did finally get them, however, they did another strep test because her throat was red, and it was positive. So, once again, Haley was put on antibiotics. Six days later, on the 17th, Haley was supposed to go for her sleep study, but that was postponed due to fever and vomiting. Her infusion on the 19th was also postponed due to her illness. After 4 days of on and off fever and vomiting, Haley began to get better. On January 22, Haley had her clinic appointment with the geneticist. Basically Haley will have an appointment in their clinic every 6 months to check on progress of her treatment. They will usually review any labs or tests that have been done recently to chart her progress. Since Haley has been so ill lately, not many tests were followed up on. She did see the nutritionist, the nurse practitioner, and the Geneticist and I believe they were pleased with Haley's progress at this point. They mentioned that the key right now was to keep her out of the hospital, as far as admissions go. The Geneticist was happy with her sitting and some of her improved muscle strength. They mentioned wanting to get very aggressive with occupation or speech therapy for feeding issues. They don't want her to lose the desire to eat food, which has been extra hard lately due to her vomiting and not wanting to eat. They also talked with us about the possibility of Haley being involved in a study that Genzyme (the company that makes her infusion medicine) is going to conduct soon. They want to increase the amount of medicine she gets (pretty much double it), to see if it would be more effective than what she receives now. There have been studies like this in the past that didn't show any difference when doubling the dose, but they say that the number of patients was very small at that time and those that were involved began treatment at less than 6 months of age. They tell me that Haley will be a good candidate for this study and Genzyme will be paying for the extra medicine that will be administered, which is amazing. Hopefully we will know in the next month or so if she meets their criteria, then they will decide if she will get double the medicine in a longer infusion day, or get her infusions every week instead of every 2 weeks. Will be interesting!

Now to the 29th...today! We had a physical therapist from my hospital make a home visit to evaluate Haley on the possibility of receiving some physical therapy at home--which will be very nice. We still plan on going to Duke for PT also, but this will help with us traveling so much. Now I just have to figure out the secret insurance codes to see how much PT and occupational therapy we are allowed....that'll be fun =)

Well, better go. Thanks for checking in on us. We appreciate everyone's love and support so much.

Well, so much has gone on since I've written! There's Christmas and not to mention it's now a NEW year!! Britani got so many things for Christmas that I'll be finding some place to put everything for the rest of the month. We finally took down the tree today...how depressing. Christmas is gone til later this year. Anyway, we have been staying very busy with the Holidays, work, and Haley. Around the 18-19th, Haley got a fever that just didn't want to go away. The congestion was also still there, so we took her to a Pediatric Nurse Practitioner in South Hill on the 20th. Her throat was red, but the strep was negative and one of her ears were pink. Because of her symptoms, they put her on Augmentin, an antibiotic. The next day she had her 14th infusion and she still sounded very congested. They did Pulmonary Function Tests (PFTs) on Haley and they turned out to be a little worse than the previous ones. So Haley was put on steroids. It took a while, but she began to clear up a little bit. We got through Christmas okay, but Haley was still very fussy and began to get more congested around the 30th. After speaking with the Drs. at Duke, we decided that we should take Haley to the ER to be evaluated. That's where we spent most of our New Year's Eve. They did a Chest X-Ray that ruled out pneumonia, and they also did more throat cultures, since the previous one at South Hill had ended up growing something called Pseudomonas. However, they weren't that concerned because she really had no other symptoms, such as fever, etc. They also did a nasal culture to rule out flu and RSV. Everything turned out okay and we were discharged home basically doing they same things we had been. They said it was probably just a virus that was taking Haley a long time to get over. After calling in a wreck that happened right in front of us on Interstate 85, we made it home safely and enjoyed the ball dropping with Britani, Dad, Kathy, and Laura. Haley was asleep so Britani and Laura blew those noise things on the front porch. (Pictures coming soon!)

Haley went back to the Dr. yesterday (the 3rd) for a follow-up and was going to get her 12 mo. immunizations. But those were delayed again due to her just finishing the steroids. Everything looked okay, even though she was still wheezing some and they also did a CBC and another strep test, that was negative. We also changed Haley's milk on the 1st to Pediasure. It's vanilla flavored and I think she likes the taste. It's better than soy formula, I'm sure. Anyway, we are also trying to introduce baby food again since she hasn't done any while she was sick.

We are going to Duke in the morning for Haley's 15th infusion. We hope everyone had a wonderful Christmas and a Happy New Year!!

We will be posting pictures on the gallery in the next few days, so please check back. Thanks so much for keeping up with Haley's progress and keeping her in your thoughts and prayers.

It's been a while since I have written. Guess I can blame that on the craziness of the Holidays!! I am trying to think about what has even went on in the last few weeks. We took Haley to the Doctor about 11 days ago for her 12 mo. well child checkup (or so we thought). She ended up having Strep throat! So, she went on antibiotics for that and we had to get tested to see if we were carriers for the Strep. Her flu and RSV vaccine had to wait a few days, and she still hasn't gotten all of the usual vaccines for 12 mo. olds. Anyway, she has pretty much been really congested the last month, so we have been doing breathing treatments like every 3-4 hours and some during the night. She has also been need oxygen lately during her sleep and during naps. Speaking of that, her pulmonologist talked with us about her last sleep study and recommended doing another one soon. That's sooooo not fun! (See pictures below!!) Anyway, there is a possibility that she might need something called BiPap, which is a mask that we put on her during sleep, and there's a machine that helps to make her breathing more effective. I'll go into more description later if she needs it, but we are praying that she won't. Other than waking up this AM throwing up and with a fever, she has been doing okay. She is getting stronger and even sitting for like a minute at the time. I hope to post more pics soon. Well, take care, gotta run!

Haley is officially not a baby anymore. Yesterday was her birthday and she is now ONE!! Very hard to believe, but then again it feels like she was born a long time ago. We had a nice Thanksgiving and we also had a birthday party for Haley this past Friday. She got many presents and had a good time playing in her cake. I got MANY pictures!=) This is just such a big milestone for Haley. We are so lucky the medicine she receives came out when it did, or we wouldn't have made it to this point in Haley's life. Once again, so much to be thankful for.

Let's see. What else did we do this past week? We went to Duke yesterday for Physical Therapy. They actually didn't do any "physical therapy" with Haley. However, they made casts of her legs so that she can get some splints (or leg braces) to help her correctly align her feet. They want to make sure that when she does stand and walk, she is walking with the correct position as much as possible. We should receive them in a few weeks and hopefully they won't bother her as much as the hand splints do.

One more exciting thing. Haley (and Dave and I) had our first TV interview today. It was very interesting!! All that I know is that the Discovery Health Channel is doing a program on an organization, called PSI (Patient Services Incorporated). PSI is a national non-profit organization that provides assistance to many with specific chronic illnesses. They help pay for insurance premiums, travel reimbursement, etc. (Check out www.uneedpsi.org for more info on what they do. The Discovery Health Channel is doing a show about the founder of PSI, along with clients that receive help, to provide awareness, and to hopefully increase donations and funds that will in turn help people. (From what I understand.) We had our interview today and as soon as we know anything about air time, etc, we will let everyone know. There's no telling how it will end up as far as our interview goes, but we'll have to wait and see. Very exciting! Well, that's all I have to talk about today....will talk more later. Haley's 13th infusion is next Friday and we will just pray she stays healthy for that.

Happy Almost Thanksgiving Everyone!! It's been a while since I have written, so I figured I'd drop a quick line or two. I can't believe Thanksgiving is almost here!!!! It's amazing how fast time flies! Anyway, Haley has been through a few infusions since the last time I wrote, and all have gone well, THANKFULLY. She is also getting stronger, gradually. She is trying to sit by herself. She stays up for a few seconds (like 8-9 the most), then tumbles sideways, but she's getting it. When she is in the walker, she is rocking her body from front to back and today she did walk towards me some. All things are happening slow, but nonetheless happening! I am trying to feed her more solid, when she will take them. She's been sick on and off a lot lately, mostly more on than off, but it hasn't been too bad. Just the usual congestion and occasional fever. Other than that, we can't complain. We have so many things to be thankful for when we look back at the last year. We are so lucky to be celebrating Haley's first birthday in this following week!!! Britani has also been loving school, and Haley still continues to light up everytime she lays eyes on her. Well, better get going. Turkey day tomorrow! We wish everyone a Happy Thanksgiving and hope everyone is safe during the holidays.

Today was Haley's 10th infusion. We went to the Pediatrician one week ago and she was put on antibiotics. Since then she has been without fever, and for the most part, pretty happy. The infusion went very well and while we were there Haley met with a speech therapist and the physical therapist. The speech therapist evaluated how Haley was taking the bottle and other foods. The therapist gave her a cheetoh and a graham cracker, and she was soooo excited!! She did well with both, pretty much just gnawing on them, but loved it!The therapist gave us tips on ways to make her mouth muscles stronger so she will be able to eat more foods. Also, the physical therapist stopped by to see Haley and was very impressed with what she was doing. Lately she hadn't been feeling so good when she saw her, so today was a great day to "work her body". We're thinking that it might not be too long before she can sit on her own, just a little more work...and patience, I guess. Well, I'm hoping to get some pictures on the picture gallery of her eating, and other things, so please check back there soon! (Oh yeah, Happy Birthday to Dave!!!)

Haley had her infusion on Tuesday the 17th. Despite waking up that morning with a temp of 102+, it went away with Tylenol and her infusion went well. Later that night she started back with the fever and has pretty much kept it until tonight. (Hope I'm not ginxing myself.) Anyway, Tylenol would help some, but not as much as usual. However, she still has remained quite active, which is good. We're hoping she's done with the fevers for awhile. During the night she is so restless and wakes up many, many times. She'll cry so hard that her heart will go up to 190 beats per minute. And sometimes she'll cry so much she throws up her milk and then we have to stop her milk pump. Anyway, needless to say, I got my flu shot yesterday and soon the rest of the family will follow. For tomorrow, we may go to Duke for physical therapy depending on how our night goes, or we may go get immunizations! =)

Haley was doing pretty good until this morning when she woke up with a fever of 102. We then got her out of the bed and she threw up lots of formula. We tried Pedialyte a few times, but she just couldn't keep anything down. However, the fever got better after giving her some Tylenol. Later in the day I called Stephanie (the Physician Assistant at Duke) to see if we should come tomorrow for her infusion. She said we should reschedule the infusions and let Haley rest for a few days and she also mentioned that she may need some IV fluids if she doesn't keep anything down. We got ready to try some more Pedialyte to see if it would stay down, but then Stephanie called back because she had talked with Haley's cardiologist. They decided that because of her heart condition it would be best for her to come to the ER for IV fluids. She said it could be for a few hours, or maybe overnight, depending on how Haley's doing. We quickly packed and drove to Duke. We arrived there a little before 5pm and got into a room around 6 or so. To make a long story short, it took them a few hours to get the ball rolling, and by time they were going to start her IV, Haley had keep two small feedings down.Because of that, the ER Doctors decided to continue to feed her through her NG tube and also give her some medicine to help with the vomiting. Dave and I fed her every hour and she started feeling and looking better. They decided to discharge her around 1am and sent us with a prescription to be filled at the Eckerd down the street. We left the ER and got to Eckerd to find out that they didn't have that medicine there. The Pharmacist spent 30 mins. trying to find somewhere that did. We ended up having to drive across town to some place we have never been at 2 am to get her medicine. Needless to say, we didn't get home until after 4 am. We all spent much of the next day trying to "catch up" on our sleep. Haley did well the next day. The vomiting had stopped, but her stomach was still very upset. She was also going all night without any oxygen! That's our wonderful ER story, but we are thankful she did so well on her own.

Haley's congestion is almost gone. She actually went all of last night without any oxygen! Her 8th infusion this past Friday also went well. However, she does have an upset stomach, so we have been through many, many diapers today. But, other than that, she is finally getting over the cold. It seems as if it takes her about two weeks to get well from a cold.

Back to Duke this week for Physical Therapy. Should be nice as long as Haley will cooperate.

Our Physical therapy appointment for the 19th was postponed due to Haley's cold and congestion. Instead, they wanted Haley to rest for a few days to try and get better.We came down today for her therapy. When we got down there you could still hear the congestion when Haley was breathing. The Physical Therapist didn't want to work with Haley until she was checked out. The Physican's assistant, Stephanie, came to see her and after talking with the Pulmonologist, they decided to send Haley to the Children's Hospital at Duke for a Pulmonary Function Test, or PFT. Depending on the results, they were thinking about admitting Haley overnight to observe her. During the test, I held a mask over Haley's face to make her scream. The point is to see how effectively she inhales, while screaming. (IF that makes sense.) She then gets a breathing treatment and then we make her scream again. The results of the test turned out to be good and the pulmonologist decided to let us go home! =) They put Haley on a steroid (kind of like Prednisone), again, and told us that if she acts any worse to come to the ER. They also mentioned that it would be a hard winter (and I guess Fall). Once getting home we were doing breathing treatments every 3 hours, with chest PT, to help clear the congestion.

Haley is doing better today. (See entries below) Her fever has been gone for a few days, but she continues to be congested. We are still doing breathing treatments often and also chest PT. We talked with the PA at Duke today. She was checking on Haley and also told us that the ECHO from the other day was unchanged from the last ECHO. Her heart was still the same size and had the same function. We were hoping it would be smaller, but I guess it's a good sign that it didn't get worse, like some do. We go to Duke tomorrow for Haley to get Physical therapy and Occupational therapy.

Haley had her 7th infusion today. She slept a lot of the day, since she didn't get a full night of sleep from the sleep study. We got there early and they started her medicine at 9am. It was finished around 1pm and then we took her to get an ECHO (of her heart) and an EKG. The team at Duke have found that after 12-24 weeks of being on Myozyme, most infant's hearts got a little worse around this time frame. They all ended up getting better in the end, but during this time frame they sometimes see a decline in the heart function. This is why we were doing an ECHO and EKG this day. After all of the testing we went back to the infusion center, they did vital signs on Haley, and then we were free to go home. We left around 3:30 or so. Here is a picture of her sleeping during the infusion.

Haley had begun a mild cough at the hospital and a little later that night she had a runny nose. Her temperature started creeping up a little and by the time she went to bed it was around 103 degrees. We gave her Tylenol and that helped her fever, but she was restless the whole night. Dave and I slept in her floor and took turns comforting her the whole night. The next day we checked with her doctors and they recommended that we wait it out until the next day, unless she got worse. They also said we could take her to an urgent care center to make sure she didn't have an ear infection. Luckily, we got in touch with Dr. Bailey, in Bracey, and he met us at the office and took a look at Haley. Her ears looked fine, but she was pretty congested. We were already doing her breathing treatments more often, so as long as she didn't get worse, we just had to wait out the virus.

Today was me and Dave's anniversary! (6 years!) However, we had to go to Duke at 8pm for Haley to have a sleep study. We weren't looking forward to it at all. The first one that was done at MCV was absolutely torture for Haley. There were so many wires and tape attached to her body she was just not comfortable. This one turned out a little better. She fussed some, but was able to go to sleep not too long after they applied all of the wires. Throughout the night she woke up more than usual, but went right back to sleep. It was very strange because we are in a very small room and outside of the room is a sleep technician. There is a camera inside the room that tapes everything and they can also hear any sound from the room. The next morning they took off all of the wires around 5:00 and of course Haley woke up. She went back to sleep and then we ate breakfast because her infusion was scheduled for that day. I am putting a picture below to show what Haley looked like after they got her "hooked up."


All hooked up	Giving us a little smile

Britani, the 5 (going on 21) year old, has started Kindergarten this week. I guess she is having fun. It's like I'm pulling teeth to find out what she did at school! When I ask her, she's like "You know, everything." That's about all that I get! Anyway, she's liking it, but not the getting up at 6:30 am part. Haley's been doing pretty good this week. At the beginning of the week she was doing wonderful. She was going the whole night without oxygen and she was keeping down all of her feedings. That was great! But yesterday she began to get a little "rattle" in her throat (sounds like she's purring) and I guess because of that she needed oxygen last night. She also wasn't able to keep down all of her feedings because she would cough really hard and gag a little. Her lungs still sound clear, which is the good part. She is also still coughing hard, just not hard enough to make the purring sound go away. But all in all we had a good and very eventful week.

Today we went to Duke for Haley's 6th Infusion. We were worried about Ernesto first thing this morning, but everything turned out okay. Just a little wind and rain, nothing too bad. We brought Britani with us because she has never been there and soon she will start school and won't have a chance to go. While the infusion was going, a Psychologist came in to do a developmental test on Haley. She was able to play with many toys during that time. I don't know whether she "passed" or not, but she had fun playing. Anyway, the day went by fairly quickly and we were out of Duke by 4:15pm. The infusion went well and Haley slept on the way home.

Today we went to the Museum of Life and Science in Durham. It was Dave, Britani, Haley, Nana (Arlene, my mom), and me. It was very informal and most of the families from Thursday (see below) returned today. Today we had more time to talk with each other. We also had lunch and was able to roam throughout the museum. The "Pompe Team" from the hospital were also there. This included Dr. Kishnani, Stephanie (the Physician's Assistant that we have paged about a hundred times regarding Haley), Joanne (nurse Practitioner), Dr. Kravitz (Pulminologist), Dr. Li (Cardiologist), Laura (Physical Therapy), and also a lady from audiology (whom we hadn't met yet). I name these people because every single one of them are amazing. We have only had 2 treatments there and met some of them only once, and we just love them all. They also had their families there. It was a relaxing day to speak with many families to ask questions over and over about their child, like: when was she/he diagnosed, when did she start treatment, how's she doing now, does she have a stomach tube, heart problems, etc. There were families there from South Africa, Peru, and around the U.S. Every single one would talk to us like they had known us forever. It was amazing the connection that everyone in that room had. Anyway, to get more on the subject of what we did. We ate, talked, watched a slide show with many kids that have Pompe Disease, and went around the Museum. We saw a baby black bear that was just adorable and Haley rode on her first train ride (that was really fast). We had a good day and hated to leave, but people had flights to catch, and we had to head home because I had to work that night (Ugh!) All in all it was a wonderful time and I am so glad the people at Duke decided to have unite us families.

Dave, Britani, Haley, and I (Krystal) went to Durham to the Sarah P. Duke Gardens for the first ever Pompe Reunion. We arrived at 5pm and were able to meet with a few families before the presenters started at 6pm. There were many who made short speeches, with some of them being the President of Genzyme (the company that makes Myozyme), and also the President of Duke Hospital. They spoke about Pompe Disease and the clinical trials that were started many years ago and the trials and tribulations of getting the medicine approved this past April. There were a few families that spoke about how amazing Duke has been in their lives. The families had children who currently had the disease, or either children that had already passed away from the disease. It was sad to hear the families that had a child diagnosed and at the time there wasn't enough Myozyme to treat more than a few infants. They were basically told to take their child home and spend as much time with them as they could, and they did. On the upside of things, we saw some children that were able to start the medicine so early in their lives that they appeared normal. We saw the 7 year old boy that was one of the 3 children accepted into the first clinical trial at Duke. He looked amazing. It was so nice to speak to these families that had already gone through the things were are going through now. To also see a child doing so well gives us much hope about Haley.