Shipping him off....

Well, tomorrow is the big day. Bennett is starting at UCP tomorrow, the same school as Nathan. Nathan starts tomorrow too. Nathan will be going Tuesday, Wednesday and Thursday. Bennett will be going on Tuesdays and Thursdays. Next week Libby starts school and the following week I return to work two days per week. I have mixed feelings about it all. When I first inquired at UCP about having Bennett go there, they were not sure they had room for him. Since I cannot make decisions I had decided that if UCP could take Bennett, I would send him and return to work. The next day the director from UCP called and said they could fit Bennett into the schedule. I figured that was a sign! Anyway, now I am not sure. He seems so little. Plus I am nervous about juggling it all, three kids and work even though it is only a few hours a week. I really enjoyed being home with Bennett this spring, snuggling him when I had nothing else that had to be done. I feel a little bit like I am cheating him since I am not doing that this fall but I do miss the work. Despite the crazyness, I really enjoyed being home with the kids this summer. It was fun not having to be anywhere at any certain time. I will miss each of them so much. The school day is so long. We'll see if I can actually leave Nathan and Bennett there tomorrow. I considered just taking them to see the rooms, meet the teachers and drop off their stuff. Wish me luck!

forgot more

So sorry, but I remembered more from the trip that I forgot to share. We learned that none of the cells that were sent from CHOP to Texas continued to grow, including mine and Marks'. This is a good indication that all of the cells were contaminated and not that the boys cells won't grow. We were really happy to hear this because even though they told us it was most likely due to contamination, in the back of my mind I was concerned. Dr. Garg said that sometimes for some kids (I'm not sure what diagnosis) cells do not grow in the lab setting so it is a good sign that the boys cells were growing in the CHOP lab.

I also asked if there are any known genetic disorders that have been cured and Dr. Ahmad and Dr. Garg sited disorders such as Sickle Cell Anemia and Spina Bifida in which drastic improvements in the health of patients has occured through treatment.

My follow up questions for Livia or anyone else who will listen are as follows: What would Francis Collins involvement be in this whole process? He is founder of the Human Genome project in Baltimore MD and we were originally told he would be treating the cells. Also, will we be making the trip to Boston to learn about possibilities for treatment? Or is Dr. Garg correct in his thoughts that they are too busy with progeria?

We asked the kids what was there favorite part of the trip. No surprise, Libby said the pool was her favorite part and Nathan said his favorite part was "sitting beside Daddy without a seatbelt on the shuttle!" Both kids also said they enjoyed the museum "where the guy got shot" which surprised us. Maybe we have some budding history buffs! Neither mentioned the children's museum with the dinosaur bone dig, but maybe that was because it was earlier in the trip.

two pictures

I have only two pictures from our trip to Texas because my camera wasn't working properly. And no, it was not the operator. The battery wouldn't hold a charge because it was cracked. The first is Bennett hanging out in the new (to us) sit and stand stroller, which as I mentioned was a lifesaver for us. One of my beloved clips that hangs on the back of the stroller was broken when it went through the rollers of the x-ray machine in the Dallas airport. I have a claim form to fill out for my $6 clips-not sure if that will get done. The second is Mark and the kids in the pool. The indoor and outdoor pool were connected by these carwash things which the kids thought was really cool.

the morning after

I continue to think of details I forgot to mention about our trip. The first is that while waiting for my biopsy on Friday, there was a blue file folder with all of the information about the boys. It had grown since Tuesday when it had maybe a dozen papers and was now about an inch thick of papers haphazardly shoved in the file. Me being the nosey person I am, I started looking through the file. It's ours right? I believe that most of the information was sent from CHOP. In it was Livia's presentation on the boys entitled "Old Case, New Information" and a subtitle that said something like, 'a teaching tool to always follow up with your patients.' Then pictures of the boys that they had taken and my collages of the boys over time. All of a sudden the next page was a familiar picture of Libby and Nathan in our back yard. I don't recall ever giving them a picture that included Libby. I was really surprised and unfortunately the doctor and Claudia came in at that time and I had to explain why I was digging through the folder. I told them I was taking the picture out because I don't want photos of Libby being used or floating around. They were very understanding. What I'm assuming is that someone from CHOP found my blog and printed out the picture, then a secretary was most likely instructed to fax any pictures of the boys to UTSW and they included this one. I guess it was just a wake up call. Of course I know the blog is public information and I am happy to have anyone reading, professional or non-professional. I love including family photos; however, I don't want them to get in the wrong hands.


Also, a couple of plane stories. On the way too Texas going through security was a disaster. It is so hard with three kids and a stroller. You have to take the kids out of the stroller, fold the stroller and take off all shoes. The part that really puts you behind is not only are you required to put each pair of shoes in a separate bin, but then you have to move the bins down the rollers yourself as you are holding the kids. In the meantime the kids are not moving because they are mesmerized, dazed and confused about what is going on and where they are supposed to go. Some guy knocked into Nathan as he tried to get past us in the metal detector. The TSA guy said something to him. Anyway we all had sneakers on because it was our bright idea that we would be doing a lot of walking and we wouldn't have to deal with flip flops falling off repeatedly on the plane. On the way back we had it down pat. We all wore flip flops-or in Nathan's case his stinky Thomas the Tank sandals. Someone told Mark that we were the fastest family with young kids he had ever seen go through security!! Yay us!

After a delay in getting on the plane in Texas they informed us we would be sitting on the tarmac for an hour waiting for our turn to take off. I started to panic that perhaps we would be like those poor people on Continental that had to wait nine hours with no food or water. I guess Bennett would probably have started one of his piercing cries that seem to be much louder than one would expect from such a little guy and they would certainly let us back in the airport, regardless of security. I looked in our carry on bag and saw the Play-do Claudia had given the kids. That entertained the kids for at least half hour 45 minutes. It was great! They put their tray tables down and went to town. Libby made two snakes with little eyes-a Mommy and a Daddy snake. The little cup circle on the tray table was the nest and she made about 25-30 teeny tiny baby snakes and played pretend. It was so so cute. Sometimes they really surprise you!

finally home

We finally arrived home at 10:30 PM after leaving our hotel in Texas at around 12:30. There were several airline delays. We are so thankful to be home in familiar territory. We love our kids dearly but too much time spent with them in the close quarters of a hotel room and plane definitely took it's toll. Libby is very high energy and talks constantly, especially in new situations when she is unsure. It was difficult having a thought to ourselves. Lots of unpacking to do tomorrow but happy to be home.

I forgot a couple things. Dr. Garg did say that he thought there are more children with MAD that are not yet diagnosed. He mentioned two that we did not know about, one in New Zealand and one in Germany in addition to the girls in Japan. I asked if he could give our information to the parents of the child in New Zealand because I would love to talk to another parent. The boy is around the age of 14. I believe that Dr. Garg may know the outcome of the progeria study but did not give us indications of the results. He said he is doubtful that Boston will be willing to work with us as they are focused on children with classic progeria and have 40 plus willing participants.

I'm not sure why I feel so blaah about the visit. I guess because Mark and I did not like the area so much. There was not much around the hospital so maybe we just need to stay in a different area next time. Frankly I'm not sure what all those old country singers are talking about. Even several of the people we dealt with at the hospital were not thrilled to be living in Dallas even though they had lived there for years. So maybe I'm just not super anxious to go back. I know this has nothing to do with treatment but I am just trying to figure out why I am feeling this way. I also don't get the feeling that Dr. Garg and his team comprehend the urgency here. I understand that no one will be as anxious as us to begin treatment but I didn't feel like there was any rush for him. The focus did seem to be more on researching and following the boys rather than improving their quality of life. I'm not saying anything bad about them. We really liked them alot. I'm just still sorting through everything that occurred.

I just feel sad whenever I look at Bennett and Nathan and see their skin and other features. I am tired of feeling this way about my own kids. I just hope that they eventually begin looking healthier rather than worse. I wish treatment could occur before Bennett's face and skin changed so much but it looks like that won't happen. I would love the chance to have a baby again that would be a typical baby but that is just not possible for us.

Leaving Texas

We were supposed to check out a half hour ago but I wanted to update everyone on the past 24 hours. Mark and I are so anxious to be home again. We are also glad that we had a summer vacation at the shore because this was not a vacation for the kids, especially Nathan. Yesterday we had some time to go to the 6th Floor Museum, the Book Depository where Lee Harvey Oswald assassinated JFK. It was so neat. We could have spent hours there reading everything but not with three kids. They were really good considering. They both had the headsets too and listened for a little while. Our kids are already loud though so it did make for some loud talking from them. Fortunately everyone else had headphones on too. Like I said, it was really cool to see the spot where he sat with the gun, the area below where JFK's car drove. After the shooting they drove to Parkland Hospital which is actually where we spent our time this week. UTSW is connected to Parkland and they rent space from Parkland. We went and got t-shirts for the kids and then drove around trying to find a good BBQ sandwich. We did but it took alot of driving!

This morning Bennett and Nathan were seen by a dermatologist at 7:15AM. According to Mark and Claudia it was not productive. Then they did a few more pictures of Nathan, some more measurements and came back to pick up me and Libby. We stayed behind to pack up the room. Mark and I had our biopsies and spoke one more time with Dr. Ahmad. Nathan's dexa scan came back 2.4 standard deviations below the norm for his age. I know they don't have alot of data for dexa scans on children so interpreting that is difficult. Curiously his body fat was 24%. All his blood work was within the normal limits according to Dr. Ahmad. From here we just wait for the cells to grow and then wait for them to be treated. We had to do another urine last night for both boys which is a pain but we got it done. Nathan has used the bathroom several times this week which is awesome! Maybe we have turned the corner!

Speaking of turning corners, after our initial discussion with Dr. Garg, I was so full of hope. I thought maybe this visit to Texas would be the turning point in our story-of my blog. This is when it all changed and we started working towards better health for the boys. That still may be the case, but I don't feel it like I felt it on Tuesday night. I'm not sure why. It's possibly because it is at least a year from now that we will be able to begin. Maybe we should have had the discussion the last day or I could have recorded it and played it again. He said that the fact that the boys are alive and made it past infancy is a sign that the enzyme is working so all we need to do is make it work better in laymens terms. Dr. Ahmad and Dr. Garg are excellent at laymens terms in spite of their brilliance. I know the fact that Bennett and especially Nathan having symptoms of old age, stiff joints etc. makes me really sad. The fact that some of the things with Nathan, the structure of his shoulders, fused bones in his feet and lowset ears will not change. I am hoping for some improvement from this treatment, hoping that it will be enough that I will be so grateful to have them living and participating in life that I could care less about the little things. Then I'll be happier than I ever could have been had this not happened for us.

We are off to make the long trek back to Philly. We will be happy to be home. Libby was mad at the doctors last night because they have to do so much to Nathan and Bennett. She thought it would be more of a vacation. I'm not sure how much are her own ideas and how much are from what we have said. In a hotel with three children it is diffiicult to have a private conversation. I explained that the doctors are doing what they can to find something that will make Bennett and Nathan bigger, stronger and healthier but I understood her frustration. I also told her that now she knows what the trips to Texas or anywhere else will be like and she can choose to go with us or stay with someone at home. I know we have several very kind volunteers! I hope I am doing a good job with Libby.

Thursday...so far

Well once again Mark is on duty with the boys. He is so much better at this stuff. We are a good team because he does what he is good at and I do mine. We just haven't found my strengths yet. No that is not true. I am better at asking the questions and sorting through all the information. Dr. Garg is really good at explaining things thoroughly and was suprised at how much we already knew and had researched. He said he was "impressed" with my questions and asked which one of us had a medical background. Nice right?

Anyway, we went this morning at 9:30 again. We did urine samples overnight for the boys which was not fun. Attaching bags to a baby and a four year old is no fun task. Mark and I had bloodwork this morning as well as Nathan. There was more examining and the doctors talking amongst themselves this morning which is always sad for me. Then Mark took the boys for photos, an EKG for each, more measurements and a check of their vitals. They are still there at 12:45 but should be done soon. Hopefully when we pick them up, we are going to drive into the city. We may go to the JFK museum if we have time and then out to dinner. It will be our first dinner out here as the hospital has been feeding us.

Claudia continues to be really sweet but on the wifty side. She had a bag of prizes for me, Mark and Nathan following our bloodwork. She noticed that Libby and Nathan were fighting over the Wizards of Waverly Place wand she bought for Libby so she also bought one for Nathan. She continues to apologize for everything and calls everyone dude. The kids love her! She took them for a walk and made up a whole adventure. She told them she can't jump so Nathan calls her "Miss Claudia that can't jump" It must be her Indian name. Tomorrow morning we have to take the boys over at 7:15 am so that one doctor can look at their hair. Then Mark and I need to get biopsies again. They want us to have the biopsies but gave us the choice. Of course we are going to comply. I think our flight is at 2pm so we will hopefully be home at a decent time, plus we are gaining an hour. Hmmm, that reminds me. We need to pay online for our bags to return to Philadelphia with us. Otherwise it will be more expensive at the counter tomorrow.

Wednesday

Well as you can see, I am summing up Wednesday on Thursday. We have been much much busier than I expected and spending much more time at the hospital than I anticipated. Wednesday we went to the hospital at 9:30AM. Nathan had another biopsy in case all the cells from CHOP are contaminated. He was then poked and prodded and measured. It's a bit strange because they are not quite as professional and polished as the staff at CHOP, not wearing gloves when they should, questioning themselves and having us sign consents after a procedure or test. They also have no baby scale so they weighed Bennett by weighing Mark and then Mark holding Bennett. Mark said it was the most unscientific thing ever and that he and I could have done a better job. Bennett weighed only 12 pounds according to their measurements so lets hope they are wrong.

We left at about 11am and headed to the Childrens Science Place which was really fun. They have a huge sand pit in which the kids can dig for dinosaur bones which they loved. They had alot of fun. We could have spent all day there. We headed back to the hospital at 1pm and I dropped off Nathan, Bennett and Mark. Nathan had a dexa scan and both got more detailed measurements. Mark said it was pretty awful. The used little clamp type things to pinch skin and fat in order to measure. Mark said both boys were fussy and generally uncooperative. I met them in the hospital room and we discussed taking photos of the boys on Thursday. I started to get upset because I hate the thought of my little man (Nathan) standing there naked having his picture taken. It's so humiliating and not what any four year old should be doing. I also picture other people looking through medical journals and seeing this little body with the eyes and genitals blurred and that is my child! Claudia did not understand this at all. Mark is able to take the boys tomorrow for the pictures as I cannot handle the whole process. The other thing that bothers me is that we will have to go through all of this every few months in order to obtain treatment for our boys. Like I said, this is not the way little boys should be living their lives. I was crying, Libby was repeatedly inquiring as to why I was upset which made me feel guilty. I was able to turn it off and think about it later. I felt guilty about bringing my son to this place where he is getting polked, prodded and hurt. I know it is for treatment but I still feel badly. I finally realized that I can't do a darn thing about this. I am doing my best with a bad situation. I also feel guilty about Libby having to deal with all this and having a mother who is depressed but once again, there is nothing I can do other than my best to meet all her emotional and physical needs. As far as the pictures, I have to realize that there are other moms out there, just like me who will benefit from the research that is being done on my boys and students and doctors will learn from their pictures. Obviously there are other moms prior to me that have sacrificed much more so that we can be where we are today in the field of medicine.


We finished up at around 4:30 and came back and went in the hotel pool. It is really really nice because we were the only ones down there at the indoor pool, outdoor pool and the hot tub. Nathan and Bennett loved the hot tub so we are trying to figure out if we can find a way to have one paid for as a medical expense for the boys. Libby loves the pool of course and we had a fabulous evening. I am very grateful that we are able to do these things as a family. I understand that there are children with special needs that prohibit or make it difficult for their families to do things together.


Mark and I are ready to be home and glad we will have the weekend at home.

Day two in Texas

Well, we have had a busy 24-36 hours. We arrived yesterday at 11:30am. The hotel is okay. I chose it based on proximity to UTSW and having an outdoor pool. We are just surprised that they charge for everything now! From the plane where we had to pay for our baggage, breakfast and when I asked for a pillow, the flight attendant informed us they don't give out a pillows and blankets any longer. You have to purchase the "nap snack" for $7. We declined. At the hotel we have to pay for parking each day and coffee in the morning! a pillow (we did without the pillow)etc. First we met with Dr. Ahmad, a fellow and Dr. Garg at UTSW (University of Texas Southwestern). Claudia is the nurse and has been our tour guide of sorts. She had gifts for each of the kids yesterday which was really sweet, and then when we spoke to Dr. Garg and Dr. Ahmad, she took the kids for a long walk. We talked alot about possible treatment for the boys which would probably be at least a year out. We first need to treat the cells then get everything approved and then start treatment. We finished up at the hospital at about 7:00 and headed back to the hotel for some hospital food dinner. It was actually pretty good and saved us a significant amount of money!

The area around here is pretty strange. About the only things in the general vicinity are hotels, hospitals, liquor stores, strip clubs and bail bonds. We searched high and low for a Walgreens to no avail. So far we have only been to the hotel, hospital and liquor store. Mark came out of the liquor store with two six packs so we may be hitting one of the bail bonds places soon.

Dr. Garg was very nice too, much more personable than we expected. He has only a folder with 10 papers on us which I found interesting. CHOP has yet to send more information. The hospital is interesting too, nothing like CHOP. It is a bit dingy and old. It is such a long walk from the parking lot to the actual hospital so we are happy to have the sit and stand. The inside of the hospital reminds me of some 1940's hospital where illegal research is being done or something. You pass by compression tanks and underwater weight stations, all with big metal doors and dials on the outside. It's a little creepy. They have us actually in a hospital room in which there are two beds. There are other patients who are staying in the hospital on our floor. I may have to take some pictures so you can get the full affect.

I am so excited about possible treatment but a bit bummed that it will be a year until it could possibly begin. When I came back to the hotel I was so happy and told Mark that any day we talk about treatment is a good day. We have no idea what to expect over the course of the next couple days. Claudia is unable to givew us any kind of schedule. Tomorrow we know we have to be there at 9:30 for and then again at 1:30PM.

we are here...

We made it! It was a comedy of errors on our part but that is typical for us. Left 10 min late, then wrong airline counter, then "misplaced" e-tix and kids birth certificates. Kids were great on flight! We got on the wrong shuttle, then finally to car rental and installing rented carseats in minivan! It all went as well as can be expected. Thank goodness for new sit n stand stroller I found at a consignment store just in time for our trip. It would have been awful if we had to carry Nathan as well as all our bags! Meeting dr today at 3pm. So far so good!

Not one but two...

I think the thing that gets me every time is having not one, but two children with special needs. I honestly think if Bennett had been a "typical" baby it would have balanced things out for me, for us. I have been trying to find some books on parenting special needs children, how to take care of yourself, your other children etc. They always discuss how difficult being a parent of a special needs child.

Anyway, we are off to Texas tomorrow. I am praying mostly that I have a good, positive attitude, that my boys aren't poked and prodded too much and that Libby has a good time. I guess we will have to be explaining a lot more to her about the boys. Of course I am praying that we get some positive information about possible treatment for the boys. I know we won't be leaving there with medication or anything but I would like to have a time frame and a better understanding. Your prayers for one, two or all of these things would be appreciated.

I'm off to finish packing, print some directions and information and copy birth certificates so that the kids can get on the plane.

Oh and this is an interesting bio about the doctor we are going to see. The end is interesting bc it mentions mandibuloacral dysplasia. I am worried about a possible language barrier when we are already talking about things I don't understand. Yes, I know this is not politically correct and yes, I am a social worker. See this is why I'm asking for prayers that I have a positive attitude!

Libby, Nathan and Bennett

Libby-finally agreed to go to children's worship during church. They only have this during the summer when there is no Sunday School. Libby has not wanted to go for the last two summers because she does not like being mixed in with the big kids. It turns out they had popcorn! She had a great time and was so excited! We still haven't gotten her to go up front for the children's sermon but, baby steps. After church I launched into a "moral of the story" lesson about trying new things...yada yada yada. I'm sure I sounded like the teacher in the Peanuts cartoon because she had that glazed over look! Oh well. I tried to make it a teachable moment but maybe I could have just left it alone.

Nathan-still has his hematoma. It's about the same size. I've still been really anxious about him falling. Every little noise has me jumping and waiting for a cry. Everytime Mark opens the gate at the basement steps I wait to hear it close behind him. He has been on a bit of an eating spree, (Nathan, not Mark) eating a whole piece of pizza for lunch and half of a hamburger for dinner. He was not a very good boy in church this morning. We sit in the balcony and there is glass in front of us. He was experimenting by breathing on the glass and then writing in the condensation. He was also using the pew and window ledge to swing his legs.....and pretending the pencil was an airplane. It is really really difficult to reprimand him for stuff like that because I am just so happy he is doing things, experimenting and being a boy. I'm sure he gets away with alot more than Libby.

Bennett-has a new excersaucer that I bought at a local church rummage sale for $5! I was so excited! I have been concerned because he won't put any of his weight on his legs. When you try to stand a baby up on your lap they usually bear some weight and push themselves back up. He does not and I thought the exersaucer might help him strengthen those little legs. Anyway, in the excersaucer his legs are pulled up really tight and don't touch the bottom. We put a game in the bottom of the excersaucer so that his feet could reach but he still wasn't putting his feet down. So today I put him in there for a few minutes and pulled his legs down from underneath with his feet flat on the bottom and showed him how it bounces. Well...he was so thrilled! He bounced himself for a little while, tiny bounces but just adorable, very pleased with himself! It was so cute I had to call Mark in from mowing (and listening to the Phillies on his ipod**) to see him.

**At one point Mark yelled so loud I came out thinking he had run over something with the mower....oh no, just a bad play!

more damage than I thought

Well, reading Libby's journal hit me harder than I originally thought. The reminders of what Nathan "should" be able to do are everywhere. Even the delivery of our sit and stand stroller reminded me that we should not be needing a sit and stand. When Libby was four years old, Nathan was two years old and Libby was already considered big. It feels like 99% of parents have typical kids and it stinks being the 1%. I guess the older Nathan gets, the wider the gap becomes and knowing that Bennett will be the same is an overwhelming thought. I keep hearing Livia saying, "it's not a cure, but a possible treatment." I have to remember that because in the back of my mind I think it is the miracle cure! The results of the progeria clinical trial that was completed in Boston is due to be pulicized in October. I keep hoping for good results, but the truth is it's not like the children who are going through it have gone through major improvements in their appearance. I keep thinking that Nathan will miraculously become normal in muscle tone, weight, appearance and skin. I guess I am living in a fantasy world. And why am I the only parent of special needs children who is still whining? This is why I don't think I was cut out for this job. I'm too much of a complainer.

pictures

Bennett and Nathan were sitting on the couch together and when we went to take a picture, Libby had to get in too! We could not get them all looking the same direction at the same time.

uggh someone kick me off Facebook!

Honestly, I love being back in touch with everyone from high school, college etc. but why do I have to click on all the kid pictures. I am happy that everyone has healthy children but it just reminds me that I do not. Especially the pictures of kids Nathan's age playing sports or babies the same age as Bennett doing 8 month old things. It's just torture. And of course they all look like their parents. I wonder if parents with typical biological children can see that their kids look so much like them. It seems so important to me because it is something that is out of my reach but maybe it's not that big of a deal. Please if you are one of those posting pictures, don't stop. I just need to learn not to look at all of them and examine their teeth, size etc.

We are leaving for Texas on Tuesday morning. I wasn't going to post the date in case there are strangers reading who may want to rob our house. If you are one of those people, please don't bother because we have nothing of monetary value in the house. We have no cash stashed anywhere and no antiques. I will be wearing my watch and rings and the rest of my jewelry is from Kohls Department store, no family heirlooms. I doubt you would be able to get the television in the living room unattached from the wall and the television in the kitchen has to be turned on and off by remote bc the power button fell into the television. Oh, and we have a large orange attack cat who does bite (ask my mom).

Anyway, we are leaving tuesday morning. We still did not call to get a seat for Bennett. I think we have to purchase one for him because he needs his carseat. We also have to call to make arrangements for carseats for Nathan and Libby for the rental car and a crib for the hotel room. I haven't flown since 2003 so I need to check out what we are allowed to take on the plane. It should be interesting! I have already accepted that we will most likely cause a scene in the airport and getting on the plane. We also have to figure out if we are going to ask someone drive us down in the morning and pick us up or paying to park our car for four days. Lots to do! I had to break down and buy a sit and stand stroller which should be on it's way. Nathan cannot keep up walking and carrying him and luggage is just not an option. I am looking forward to watching the kids experience flying for the first time, swimming in the hotel pool with them and trying out a minivan! I am not looking forward to talking about the boys symptoms and having them be poked, prodded and photographed. I am hoping that the vibe is not sheer curiosity and research but also treatment for the boys. Claudia was so sweet and asked what the boys like so it sounds like they really try to make it as easy as possible.

I have a journal in which I have been writing to Libby since the day she was born. I had not written in it since April 2008 so feeling guilty, I decided to do so today. Of course then I started looking over the entire journal which is now filled. Libby loved hearing all about the things she did and said when she was little. It's amazing how quickly she developed. She was crawling at the age of 7 months and climbing steps and pulling herself up at 8 months. She had her first two teeth at the age of 6 months and was racing through the baby foods at lightening speeds. It was so nice having a typically developing child, without physical therapy, stretching and worrying about injury. In my writing, I was so happy and amazed at every new milestone. I wish we had another chance to experience that again. I often feel bad about myself because I am so frustrated with the boys development but when I read the journal today I forgave myself a little bit. We are definitely doing things the hard way with them.

If I could have one small prayer answered, it would be for Nathan to be able to go up and down the stairs by himself. It is wonderful that I can send Libby to get her own pajamas or send her to her room. With Nathan we have to walk him up or down for everything. Many times it is just easier to carry him or get whatever he needs ourselves. Plus when Libby and he are upstairs we have to close the gate and then Libby is trapped too. It would make my life so much easier if he could do the steps by himself.... or we could get a one story house. At the beach, Libby loved having the bedrooms on the same floor as the living room and said she wants to live in a house with one floor. Too bad that when we were house hunting that Mark's one criteria was that he has to go upstairs to go to bed!

Small Setback

So here is the update. Livia finally called me back today telling me there are no new updates. She was off for 2 1/2 weeks and has not heard from Leslie Gordon at Progeria Research Foundation to know if they are willing to work with us but admitted she needs to call them. I asked how the cells were responding to the treatment and she said we will find out when we go to TX.

Then I called Claudia from TX to find out if everything is still on for our trip. Claudia said the cells did not grow so they need to have more fibroblasts sent to them from CHOP. I asked if it had anything to do with the disorder/cells and she said it was most likely a problem with contamination in transferring the cells. I asked if that means the rest of the cells from CHOP would be contaminated and she said she didn't know. I asked if we could just have the boys "re-biopsied" when we come to TX because I don't want to wait to find out that these cells won't grow either. She said we can do that, but she wasn't sure about Bennett because they typically don't treat children under 1 year of age. So basically we are back to the June 6th date when we had the biopsies. I spoke with Livia again (twice in one day!) and she said she has some frozen and can send them to TX. I am confused because I thought the cells were sent to Baltimore for the medication treatment. Claudia didn't know anything about Baltimore and I forgot to ask Livia. I feel like I don't understand alot about this process.

I told Livia I am haunted by the memory of Dr. Zackai saying to me, "You've got to get progeria out of your head." I can hear her voice saying those words and can see her gesturing with her hands. Meanwhile Nathan did have a form of progeria all along. She said they didn't know there were so many different forms. I told her that I had searched the internet high and low for other types of progeria, even sending an email to Leslie Gordon, the director of research at PRF. I know it is futile to think about the past but it is something I have a hard time getting out of my head.

Then there are times I just can't believe this is happening. I just can't believe our family is dealing with something even rarer than Progeria. When I was talking to Livia, I honestly felt like I couldn't breathe. It's just all so life-changing! I hope and pray that this is just a minor set-back. I know that just because the cells did not grow, it does not mean the whole thing is doomed. It does feel so familiar though. It reminds me of he ups and downs of infertily, hope and then dissappointment. Why do some people go through life with none of these issues and we have been through so many. I don't get it, but I guess that is the age old question that we will not know while here on earth.

the Bike

Here is Nathan on his new bike. He loves it and was so excited! He spent about 5 minutes on it before giving up because it was too much effort. I remember Libby doing the same thing when she got hers at age four so I think he will get the hang of it eventually. Nathan enjoyed his birthday week. We didn't want to do a big party since our families had just gotten together for Bennett's baptism. Plus I told him he couldn't have a big party until he is potty trained. It resulted in several small parties though so I guess he won again! This morning he asked when he is having his next birthday party!

I am really banking on this treatment for the boys. Everytime I feel sad about their skin, lack of eating, small size and developmental delays, I comfort myself with the thought that it will improve with treatment. If that is not the case I'm in trouble! The thing is we are so close to normal but so far. We have three kids, they are 6, 4 and 9 months. We live in suburbia, I stay home with the kids, we do playdates with friends and hang out at the pool. We enjoy a toy cluttered house, sibling rivalry and lots of hugs! But we are so different. Our daughter is normal but came to us in a different way than most kids. We will soon have to answer many questions and deal with many issues regarding being "given up for adoption." Our four year old can't run and jump like other kids his age. He can't walk up and down steps by himself. He has had several skull fractures and wears the same size shoes as an 18 month old. Our nine month old cannot sit up yet or hang out in the excersaucer. Two of our three kids have physical therapy and see a specialist for every part of their body. We are dealing with a progressive disorder that only four other people in the world have ever had. We are soooo not normal.

Sometimes it makes it difficult to know where we fit.

I have such trouble with the little kids at the pool who run around and are the same age and younger than Nathan. Their skin is such a nice healthy color and they have full heads of hair. It makes we wonder how a preschool biological child of Mark and me would look. We will never know. I wonder if that is something I will ever get over.

We have two injuries in our house. Libby fell off her scooter yesterday and has brush burn on her arm. Nathan fell in the pool today and started bleeding from his mouth. I thought it was from inside his mouth, but it's a brush burn on his cheek.

heart attack

Nathan is seriously going to give me a heart attack. My nerves are shot. Tonight he fell in BJ's Wholesale Club on the concrete floor. Honestly, I am seriously thinking he should be wearing a helmet. I heard him crying from a couple aisles over and when I got there Mark told me he slipped and fell but "it wasn't bad." He did cry for a while though and said it hurt. Every time I hear a thud in the house I am panic stricken, my heart starts racing and I wait for the tears and the "report" from Libby if he fell or is just throwing a fit. When I am away from him I am a wreck too-What if the babysitter forgot to put the gate up at the top of the steps? What if he fell on the back steps while Mark was dealing with Bennett? It is an awful part of this whole situation. I haven't been taking Nathan to physical therapy at Theraplay because I just needed a break while all three are still home. I actually miss it because it gets me out of the house and gives me some structure.

Nathan and Bennett are doing alright, neither of them are eating to break any records. It is still such a battle to get Nathan to eat his meals. Before he even sees his food he starts saying, "I don't have to eat it all, wight?" He is getting a two wheel bike for his birthday. I didn't want him to have a big birthday party because I used that (unsuccessfully) as incentive for going in the potty. Instead of one big party we are having several little ones in order to celebrate with everyone so once again, he won. He is getting his bike tomorrow so I hope he is excited. It is red of course, his absolute favorite color! It is from the Cars movie which he is not really that into, but it's difficult to find plain bikes anymore (i sound old!).

Nathan went on the potty today which was very exciting! Libby gave him some pointers that would be too much information to share. It worked so I can't complain!

I booked our Texas trip! It is pretty expensive for all of us to fly down there and stay for four days. Maybe if this were actually the treatment it wouldn't feel so bad spending all this money, but it is really just to talk with them, have them meet the boys and go through some testing. It would be nice if treatment actually occurred in Boston because that is drivable but obviously we will go wherever. It goes without saying that I hope this works. It would really change our lives and the lives of our children. I paged Livia the other day and she did not return the call. Honestly, I didn't know if I wanted her too. I'm always worried there would be bad news of some kind. Mark says "What bad news could she possibly have?" Oh Mark, do you know how my brain works? Texas backed out after we spent all this money....the cells are not responding to the medications.....Boston said no.......There is always a chance the bottom could drop out on this. I guess after it has happened several times to us I have become very pessimistic and Mark remains optimistic. Please continue to pray for guidance for us.

Tomorrow morning me and the kids are going to Lancaster to my sister Susan's garage sale. Libby is selling juice boxes for $1. If you happen to be in Gap, stop by! She is so excited so I hope she sells some!

Nathan is Four!

Today is Nathan's fourth birthday! I can't believe it has been four years since he was born! It has gone so fast but so slow since it has been filled with so much trauma for us. Happy Birthday Nathan! I hope we have many more birthdays together!

I have several blog entries started but none finished. I have lots of thoughts running through my head and can't seem to get them organized on "paper." I booked the trip to Texas, airfare, accomodations and a rental car. This is a very expensive trip for us, so I hope it is not in vain. I have alot of questions about God, prayer and what the future holds but like I said I just can't seem to get to them right now. I just wanted everyone to know that we are still here, still alive and moving right along. I will update later today or tomorrow.

Haircut

Bad Bad Day

I had a really bad day today. Honestly I feel like it has been building and building and then it just all caught up to me. Nathan has a hematoma from his recent fall off the stool. Discovering it put me over the edge. I was in tears. I just can't imagine what all these hematoma's are doing to our poor little man. With 4 in 4 years, how many more will he get? The fall off the stool was unpreventable. One minute he was standing on a 12" high stool and the next he was falling backwards onto the floor. Honestly sometimes it's like he can't stay on his feet! I so wish he could be more independent like other 4 year old's, up and down the stairs to get things, upstairs playing without the gate up and outside playing in the driveway or on the swing set. He climbs the ladder of the slide which he has been doing very well, but what if he falls while doing that? If a fall in the kitchen results in a hematoma a fall from the swing set ladder would be devastating. Do we stand behind him at all times while he's outside? Do we take him to the doctor after every fall? And the worst part is the thought that we have to deal with this with Bennett yet too. I thought that Nathan might be able to play some sports some day but what was I thinking? No Little League or soccer seems so minor but it is one of the things I am really enjoying about Libby's age. It is the way of meeting other families and being members of the community. It is the way that little boys socialize. Four seems to be the magic age in which kids can start doing these things and the gap between Nathan and his peers becomes more and more evident. To have Libby be our one and only chance to experience these childhood rites of passage is sad. Who knows, maybe as they get older this stuff becomes less important as they find other things, music and art. I hope so.

The last few days with all the stress, planning the Texas trip, the cost of it and unanswered questions about this treatment I have had no patience with the kids. I pride myself on being pretty patient and not yelling but I have not been so good lately. After discovering the hematoma today, I cancelled a play date which sent the kids into hysterics. I just couldn't get it together to go out and face the world. I couldn't even take them outside to play. I had no good explanation other than that Mommy was sad about Nathan and Bennett being different. I feel bad that I am crying in front of my own kids. We did make some brownies and peanut butter cookies in the easy bake oven (yuck x two) and I also have to remind myself that I do not spend my days in bed crying. This was one day that I couldn't put on a good face and make it out of the house. Hopefully they won't be scarred for life but I do feel very guilty. I don't want them to miss out because of me. I also don't want to isolate myself from friends, family and the world. I have not had the strength to talk to my sister since she had her baby. There are many others with whom I would love to be spending time. I am a social person who currently cannot bring myself to socialize and cannot risk the sadness these interactions often trigger.

Further isolating is the fact that there is not one other mother who has a child with Mandibuloacral Dysplasia. We don't really fit anywhere. I would love to have progeria kind of be our cause; however, we don't really even fit there.

I ache so badly that my children are not healthy typical children. It is an ache so deep, in the depths of my heart and soul. I wish I didn't have to worry about hematoma's and skull fractures. I wish I didn't have to hear myself and others say, "Watch out for Nathan." I wish he was a rough and tumble boy. Then there is the added stress of trying to explain this to Nathan and Bennett who will understand they are different. I understand this treatment is not a cure, but how much improvement can it bring and at what cost. I wish we could be a normal family like I see everywhere I turn.

....and saving the good news for last.....drum roll...Bennett rolled from stomach to back! There was a report of this occurring yesterday; however, my only eye witness was Libby's six year old friend so I needed to see it for myself! Yayyyy Ben-Ben (that's what Libby & Nathan call him)! I'm so excited! He is really coming alive, crying when you take something from him and fussing when he wants something. He is eating the whole first jars of baby food (the teeny tiny ones) and we can't feed him fast enough! I love it!

Angry Momma

We had PT for Bennett and Nathan this morning, trying to get Nathan to ride a scooter so I don't have to be envious of the neighbor kid. Speaking of envy, we also went to the pool today. I am noticing that I am very angry with the "stay at home" mom crowd. (I know I am one of them, but you know what I mean.) There were tons of (skinny) moms there chatting it up and not watching their kids. I wonder how much of my anger was that they weren't watching their kids and how much was jealousy of their healthy kids. I don't want to be a bitter person, honest. Nathan had his intertube taken away by a little girl who took it right up over his head!! He also got hit in the head with a football and was knocked over by a boy who was way too old for the baby pool. Now, granted it doesn't take much to knock Nathan over, but still! Libby was awesome! She was right there to pull him up and defend him! I am so proud of how she looks out for her brother. We went to McDonalds for dinner (woo hoo) and kept an eye on him there too. There was another little girl there and Nathan and Libby initially didn't let her play with them. I quickly made sure they included her; however, it was interesting to see them as their own little unit. Anyway, I need to work on my anger and bitterness before I embarrass my children. I was ready to rip that innertube out of that poor little girls hands!

I got the kids haircuts tonight. Nathan looks so much cuter with a fresh haircut.

as promised

Here are some pictures from our trip to the beach!

Packed and ready to go!
Deer Memaw & Nathan

This is about as far as we got with Nathan.

He didn't really want to try too much. Maybe next year.

Bennett-apparently not a water bug.

Libby, our water bug!

No explaination needed!

Nathan playing in the sand-his favorite thing to do!

This was my sister's idea, Libby holding Bennett.

It made me very nervous but he survived!

On our porch of the beach house!

I saved the best for last.

Mark made me crop it so you can't see his six pack abs.....why are you laughing?

Waiting for a Hematoma

Nathan fell straight backwards off of a stool today and hit the back of his head on the kitchen floor. I am so worried he is going to get another hematoma. He seemed fine after he got done crying. Honestly, just when I think he is more stable he goes and falls so quickly and suddenly. I am a nervous wreck during his waking hours. Sometimes I am so relieved when he goes down for a nap because it means I don't have to watch his near misses. Today he and Libby had the couch cushions off and were building a fort, nothing too crazy but I was so nervous that he was going to fall and hit his head on the table in the living room. If Libby fell and cut herself requiring stitches or broke a bone, we would take her to the ER, get her fixed up and go on our way, chalking it up to one of the childhood rites of passage. But with Nathan everything is more serious. Sometimes I long for him to have the physical abilities that other kids his age have so he could play freely.

picture

Yes, two posts in one day. I was taking pictures of this crib set for ebay today. I had to put Nathan in time out for something. He was mad so he stuck his foot out to ruin my picture! He is a typical almost four year old in some ways!

Trip to the mall

I went to the mall yesterday to do a little ebay shopping and walking around all I see are families with typical children. I wonder what it must be like to not carry this burden around all the time. I envision these families flitting through life with not a care in the world. I know this is totally inaccurate because everyone has their burdens and battles. But I wonder how many have one as truly deep as ours, one they carry with them every day. The pain of having two children with this beyond rare disorder is deeper and more painful than I could ever imagine. I have prayed that God would lift this burden, even if this doesn't mean a cure; however, I still I carry it with me. If this situation had never happened would I still be restless, unhappy and discontented? I don't know, but I sure wish I could give it a try.

I am grateful that we have three children who are all functioning normally cognitively. I am grateful that two of my children are ambulatory and one will be at some point. In order to make myself feel better, I remind myself that there are families who are not so fortunate, families who have lost children, couples who long for children but have none, families with children who have multiple physical and mental disabilities. This makes me feel better temporarily; however, then I look around and realize that this such a small percentage of people. The majority of people have children who are healthy and "normal." I look out and see the 3 1/2 yr old neighbor boy riding his scooter with ease and I wish Nathan could play so freely and painlessly (and without me hovering over him). Then there are families with one child with a disability and two or more other children who are typical children. At this point I feel like I could handle that too, but having two children with a disability and one typical child just feels so unbalanced. I know I should be happy with what we do have but it is so hard for me. As I write I'm realizing that in my head, I envision some kind of heirarchy with every family falling somewhere on the scale. People with healthy children are at the top and we are somewhere near the bottom. Sometimes I mistakenly believe that the people at the top are better than me. Sometimes I envision the scale as some type of fortunate/unfortunate-those at the top being super fortunate and those at the bottom, not so much. We are not members of the club at the top. We are at the bottom worrying about skull fractures, our kids not gaing weight and how many trips to TX/Boston we will need to make for clinical trials. I know this must all sound ridiculous but I am just attempting to describe and determine the distortions that go on in my mind. I realize that there are people in the world don't have homes, food to eat, supportive loved ones in their lives or fear for there safety on a daily basis. I just don't happen to live with or hang out with them. I hang out in suburban USA where every child has a house, two parents, food on the table and matching siblings. Every four year old I know has subcutaneous fat, a full head of hair and is able to run, jump, walk up stairs independently. I guess I'm comparing myself to the wrong people.

PS: I wrote this early this morning. I am feeling much better now but wanted to post it anyway. One thing that cheered me up...I took the kids to a doctor appt. As we were leaving an older gentleman in a car said "You got your hands full. Are they all yours?" me "Yep! They are all mine!" gentleman "They must keep you busy!" me "Yep, it's a great kind of busy!"

Ten Years (long...really long)

I haven't written in a while so this will be long! Take frequent breaks, feel free to skim! We are back from the beach and had a great time! None of us wanted to come home with perhaps the exception of Bennett. He was merely drug back and forth to the beach. Though he never sat in the sand, he managed to have sand in every crack and crevice at one point. Thank goodness we crashed my sister's family vacation so there were plenty of hands to hold him on the beach. Nathan and Libby had a great time. Libby loved boogie boarding and was fearless in the water. Though Nathan was no match for the strength of even the tiniest waves, he also loved the water. It's hard to determine what other almost four year olds would be doing at the beach. I don't know that they would be boogie boarding, but perhaps they would be able to be trusted wading in the water a bit more independently. With Libby being the oldest, she is often able to do more than Nathan. Because of that we sometimes do things separately, ie Mark will take Libby to the pool or for a bike ride. I know part of it is her age, but part of it is Nathan's physical delays. I hate this and hope the gap doesn't widen. We have to meet the needs of our typically developing child as well as our developmentally delayed children. Did I mention that I attempted boogie boarding for the first time? It wasn't pretty but it was fun! I kept trying and managed to get it right 3-4 times. My six year old was telling me when to hop on the board! I have pictures to post, but I will get to that later. (None of me boogie boarding. It was the one time I was glad that Mark never takes pictures of me) You don't want to miss them as there is one of Mark trying on Nathan's turtle goggles!

Libby spent Friday at her cousin Sarah's house. I had a friend and her little boy over for a playdate and the little boy also has developmental delays. I thought it would be fun for Libby to do something else and nice for Nathan to be able to spend time with his new friend. Plus I thought she could be spared at least one time from the two hours of weekly physical therapy. She had a great time and I appreciated not having to juggle it all for a while. Both Nathan and Bennett are doing well with physical therapy. It's difficult for us to see the improvements because we are around them all the time. I should video tape them more, especially Nathan so we can see the changes. Nathan is getting braver in trying new things. I was so surprised that he enjoyed the beach so much because typically he does not like to be out of his element.

No news on the treatment front. We are still planning our trip to Texas. There is no news from Boston yet. My latest question is if there has ever been a cure for any genetic disorders. I tried to google this question but came up empty. If anyone knows the answer to this question, I would love to know.

Last night Mark and I went to see Elton John and Billy Joel for our tenth anniversary! It was awesome! I do think about our situation alot even when we are out. I see boys everywhere and feel sadness that my boys are not going to look typical and may deteriorate physically. Mark can put it out of his mind for a while, but I have a tougher time. Add young married couples to the list of people who cause sadness for me. I have no desire to be them, it just takes me back to the time when Mark and I were first married and full of hope. We knew none of the painful things that lie ahead and were hopeful for a typical family with typical healthy kids. It is too late to unwind things now and we will never be the same. We will never completely heal and our hope of a typical family is gone forever.

Anyway, the music was awesome! I love concerts. They are one of my favorite things to do and I had not been to one in forever. I think the last one I went to was Pearl Jam before Libby was born. I actually love anything where crowds come together for one common cause, sporting events, concerts, shows etc. They are so energizing! The first song was Your Song....and I cried. The lyrics, "how wonderful life is while you're in the world." made me think of my kids, but especially Nathan. He is such a joy and I wish for nothing more than for him to have a long and happy life. I hate that he and Bennett will have to endure more physical and emotional pain than most kids. The next song was "Don't Go Changin'" and again I cried (This will all be a surprise to Mark as I had on my sunglasses). It reminded me of our marriage. We have been through so much. "I would not leave you in times of trouble, We never could have come this far. I took the good times, I'll take the bad times, I take you just the way you are...."

So I thought I was in good shape, no more emotional upheavel and then several songs later, Elton John sang, Daniel. I'm not sure why, but it reminded me of the price that Libby will pay for having two brothers with special needs. "Daniel my brother..........do you still feel the pain of the scars that won't heal. Your eyes have died but you see more than I. Daniel you're a star in the face of the sky." Will she be scarred from being drug around to various doctors while we attempt to find treatment for her brothers? Will she be embarrassed at school that her brothers are "different"? Something tells me she will set people straight in that department. Anyway, the rest of the show was happy. Elton John played my theme song, "I'm still standin." It was a great night and a great way to celebrate ten years of marriage. I can't imagine a better person with whom to spend my life.