Childrens Museum of Boston

Last Thursday we had plans to take advantage of some free passes and go to Great Adventure/Six Flags New England. Well the weather was not so cooperative. We decided to go instead to the Childrens Museum of Boston.  I always feel like we could go to a children's museum here in Philadelphia but there were not many other indoor options.  We grumbled to ourselves about spending money for admission on what was supposed to be a free to low cost day. We were skeptical but boy were we wrong!! We stayed for five hours at this awesome museum! Yes, there is a Childrens Museum in every city but we never take the time to go and especially not all five of us! My pictures are not so great because they were taken with my phone.  This is Bennett on the screen at the beginning of the visit. He stood there for more than ten minutes and we could not convince him to move around.  He seemed a bit overwhelmed.  The little girl in this video was so cute.  She was trying repeatedly to get Bennett to move around but to no avail. 

Libby of course had a great time climbing and doing just about everything.  There was a bubble room she liked as well as information about animals which is always something she enjoys.

This was an awesome room with tons of different activities exploring golf balls and physics.  They had ramps of every shape and size and spirals and more.  That is Libby up at the top getting ready to drop the golf ball down the channel.  They also had a magnetic wall where the kids could experiment and move PVC pipes around.  Honestly the kids could have spent all day in that room! 

This is one of the play structures in the Construction Room.  The sign said 5 and up.  When Nathan began to climb, I stopped him and then changed my mind.  He climbed up by himself and walked the whole way across the moving bridge and down the other side.  I was so proud of him!  It took him a very long time but he did it!  He has become so much stronger and confident in the past year!

On the second floor there was a room for kids ages three and under.  It took Bennett a little bit of time to warm up but he loved this area!  Mark took the big kids to some other areas and I enjoyed sitting there watching him play for an hour or more.  He often gets stuck sitting in the stroller while his big sister and big brother get to do things so it was awesome that there was a room full of things that were just his size.  He especially liked the trains. 

 

Bennett and Nathan can do most of what other children their age can do but it takes them a lot longer.  A typical two year old would hop right up there but for Bennett it took a lot of effort and thought.  Normally I help him so don't think I'm a horrible parent.  I also noticed at the beginning of the video that someone turned around to take a second look at Nathan.  Happens all the time but gets a little tiring. 

The Pool

I don't know why going to the pool puts me in such a funk.  I just got back from my first trip this summer.  All those little bodies running around with healthy skin...it puts me in such a bad place.  I want to go to the pool for my kids because they love it but I am beginning to have buyers remorse this summer.  It is not even the other kids looking at him them that bothers me there.  Even though they do.  It is more the longing for my boys to be healthy.  I long for them to flourish and grow like other kids to have that same smooth even skin with no veins showing and no bony knees.  I know that my kids have a lot, they are able to walk and talk and express themselves.  They are intelligent and affectionate but that skin and hair always reminds me what is going on within their bodies.  I know I take for granted what they can do and I'm sure there are parents that long to be able to tell their child "it's quiet time" or "don't run" or "don't back talk."  But I long to take all three of my kids for haircuts, have their skin tone even out and have them gain weight.  I don't know where I am going with this post.  I know it is a lot of rambling but I just needed to talk.  Again I don't quite understand why the pool is so much more difficult then other venues.  I guess because it is the place where skin is in and the contrast between my boys and healthy kids is so physically apparent.  I want more than anything for my boys to be healthy.  I do not want to miss out on life wishing for that but I do not want to stop fighting for it either.  I know that God is capable of healing Bennett and Nathan but I do not know if He will choose to do so.  I do know that He wants us to pray for what we want regardless.  So on days like this I pray for a cure so that our boys can live long healthy lives.

Mickey Mouse sunglasses

I have more to share about the conclusion of our Boston trip including some cute pictures but I will get back to that. For now I wanted to say thank you to those who purchased or had friends purchase sunglasses for Nathan at Disney World. We received several pair but are using them all. We are able to have two pair in the car and two pair in the house so that we are never caught on a sunny day without sunglasses. Today Libby even decided it was sunny enough for her put them on (she thinks Mickey Mouse is a baby show) Anyway I wanted to thank the people who got them for the boys. They love them and they are small enough that they stay on their faces. Like I said in my previous post, the sunglasses are a necessity due to their sensitivity to the light but the Mickey Mouse part just makes them fun. So thank you so much to those who went to the trouble to make telephone calls to friends and to those who searched for them at Disney World. These smiling faces (and their parents) really appreciate it.

Wrapping Up

We are wrapping things up in Boston today. Yesterday after the infusions, we had a chance to talk to Dr. Gordon which is always enjoyable. She has such a unique perspective as a doctor, scientist and mother of a child with Progeria. The results of the first study (First trial was Lonafarnib only and our boys were not part of that trial.) are not yet published but according to Dr. G this will be happening in the very near future. Although she cannot give us any specific information regarding the results of the first study, the fact that they are continuing to administer the Lonafarnib to all the children in the study is a positive sign. Dr. G seems very happy with the results. So my mind is of course racing with thoughts of all this stuff. Here is a bullet list of some of my thoughts and things we learned. They are in bullet form because they are so random and cannot be organized into any semblance of order. This is my brain, constantly-no semblance of order-lol.

• Both boys received inserts for their shoes. They had molds cast of their feet so that the inserts could be fit to them perfectly. These inserts provide cushion for the bottoms of their feet as they do not have enough fat to keep the bone in their foot from pounding down through the bottom of the feet. It also fills up the gaps in their shoes as their feet are so narrow.


• I am pretty sure that we will not have any more children. Its a case of my two year old self wants what I want, a healthy baby, but my brain has to step in and say that this is not what is best for our family.


• I know I have mentioned several times about the embryos we have in storage. Although we are not having more children, we have not disposed of them because of the outside chance that we would change our minds or that they could be used to provide medical treatment for Bennett and Nathan. Every time we get the bill, I feel guilty-like we are wasting money on something that will not happen. Dr. Gordon was able to tell us definitively to hang onto them as one day they may be beneficial. It is a big relief to have a clear cut answer and we will not feel guilty when the bill comes.


• I'm disappointed that the boys stayed virtually the same in height and weight. Anytime we said anything like that about the boys, the doctors nod and say, "That is typical of kids with Progeria." For the first time we don't want to be typical, not typical of Progeria anyway. And because the manifestation of symptoms in classic Progeria are a bit worse, they are not the least bit alarmed by the symptoms in our boys.


• Cutting Bennett's gums will most likely not cause his teeth to come down any faster. It is not a problem with the gums, but simply the teeth being slow to come down in children with Progeria.


• We asked about the second trial results and how they would be written, would there be a second section for children with non-classic Progeria. Dr. G said that it will all be together, but after the main study is published we will be provided with additional information about the children involved in the study that have non-classic progeria. Of course no one in the study has the same mutation as our boys. This is where the major worry begins. What if these medications are working for children with classic progeria but not our boys? What if any treatment in the future works better for kids with classic progeria and not our kids? PRF is not looking for a cure for our boys, they are looking for a cure for children with classic progeria. Yes it is closely related but obviously the most subtle differences can make a big impact in symptoms and treatment. All these things are spinning around in my head. We are wondering if we are putting all of our eggs in the wrong basket but technically the only other basket was Dr. Garg and he was far from any type of treatment. So, we know we are the right place but thinking about all these things can make a person crazy-at least it makes me crazy! 99% of the time we know we are doing the right thing for our kids but 1% of the time you start to question ourselves.

So today is our last day at the hospital. We finished at around 3:00 and our plan was to do the kids version of the Freedom Trail. Well it started raining so Mark took the big kids to the movies to see Mr. Popper's Penguins while Bennett naps. It was beautiful for the last two days and we were planning to do the tour this afternoon and Great Adventure tomorrow. It looks like it is supposed to rain tomorrow too so we might go to the Children's Museum of Boston. We are staying at the Devon Nicole House tonight, a hotel in Springfield Mass tomorrow night and then homeward bound on Friday.

Mickey and Nathan

He has only grown less then a centimeter but the pictures look good. I tried to keep Mickey the same size in each picture. My but is that I wonder how much a taller a typical child would appear in pictures such as these. We have to celebrate the small gains though. Right now the boys are getting their infusions of zoledronate. The nurses have been so nice about answering all of Libby's questions. When the nurse explained how the medication goes through the tubing into the iv, Libby said, "so that their special needs get better....which for Bennett it seems to be working because he is really strong! " So sweet.

Day One in Boston

We are done with appointments for today. The day was easy physically but always tough for me emotionally. The boys have not gained any weight in the last six months and Nathan has not gained weight in the last year. Nathan weighed 28.8 pounds today and actually weighed more back in January. Bennett weighed 19.58 pounds today which is up less then a pound in the last six months. Nathan and Bennett continue to change in so many physical ways but not in the ways they are "supposed" to be changing.

I was giving myself a hard time this morning when the "weigh-in" put me in a funk. Why is this getting to me? I know it is part of Progeria? It's not a surprise, I knew that they are not outgrowing clothing! So I tried to think about why I cannot get over this seemingly trivial matter. Why such strong emotions tied to not gaining weight? So I backtrack to emotions that accompany when your child does gain weight(Libby and for the first few months of Nathan's life). Remember that feeling of walking out of the pediatrician's office and knowing that your baby gained a pound or two since the last visit. I would proudly call Mark followed by either my Mom, my sisters, friends or all of the above. The pride was in the fact that we were feeding, nurturing and providing an environment for our little one to blossom and grow. It is the confirmation of what we secretly already knew, that our children are superstars! So with that much emotion pinned on weight gain and growth, it is understandable that the opposite would trigger the opposite emotions and feelings. Its just a struggle to repeatedly pick onself up from those emotions (at least it is for me.)

There is so much encouraging and positive research that is happening right now thanks to PRF and the NIH (I "heart" Francis Collins). I have such hope for the future for those diagnosed with this disorder and I am so grateful for the opportunity to participate in these clinical trials. As a mother all the research in the world does not mean anything until my boys start gaining weight and filling out their clothes. I hope that having a "but" does not make me sound ungrateful and negative. I am just a mom who is anxious to have my children's health restored!

Hoping for a better day!

Tonight has been one of the worst nights for us. I'm being dramatic because there is nothing seriously wrong. It took us eight hours to get up here to Boston because of traffic around New York but getting here was the least of our problems. We arrived at 10pm to the Devon Nicole House which is the place we always stay. It is a great place, a Ronald McDonald type house. This is our fourth stay here and Libby's second. We have always been given the same room but this time it is a different room. It is actually bigger than the one we have always had but that did not suit Libby. She is "creeped out" because she is not used to it and on and on. Then they did not have a cot and instead have a blow up mattress for them which caused even more hysteria. For the last two hours Libby has been screaming crying and throwing fits about where she is sleeping, being completely irrational. We have tried everything short of giving her our bed and have done it all in a (mostly) calm manner but to no avail. It is now 12:15 and there is some hope in sight but still a very discouraging night for us. Getting three kids to sleep in one room is never easy but this has truly been the worst ever. I dread seeing the other guests tomorrow because I am truly embarrassed because of the crying from all three of our kids.

So here is hoping for a better day tomorrow at the hospital. It is technically later today so I better get to sleep! Everyone should be in great moods tomorrow (I mean today)!!

Kindergarten

So nothing really terrible happened at kindergarten orientation over a week ago. It just made me sad. It started before we even got into the building. Walking across the parking lot I was carrying Bennett and Nathan was walking beside me. His peers were skipping and running along and when they saw him they did a once over look, looked at me, looked back at Nathan and then walked a little bit closer to their parents. This happened repeatedly throughout the hour which felt more like three hours. Everyone was delightful. The teachers and staff were so kind and welcoming. We met the woman who will be his aid in the classroom. Someone offered us the key to the elevator (we declined.) In the hallway a family from Libby’s softball game shouted “Nathan, Nathan” which was so nice and someone said they knew Nathan from the neighborhood water ice store.

So everything went well but in the deepest places of my heart and soul I wish that Nathan’s appearance matched his inside five year old self. As I watched the other five year olds jump up and down on the steps of the stage, run through the halls and roughhouse with each other I have never wanted something so deeply. It is difficult for me to describe because it was not a ‘feel sorry for myself’ depressive, hopeless kind of feeling. It was more of an aching sadness for Nathan and for what he does not yet fully understand. And the overwhelming part is knowing that we will have the same experience in three years with Bennett. Just more reminders that this is not merely a bump in the road, a “this too shall pass” but this is our life. This is how we send our kids to school, with Aids, IEP's, curious stares and sympathetic smiles. These types of milestones are no longer fun, carefree events in our lives.

And the milestone of kindergarten is also an indication that time is moving forward. Time is passing and we need a cure for our boys.

Daddy's boy

Bennett is quite attached to his Daddy. He cared for Bennett a lot in the months preceding and following the diagnosis two years ago. When I pick up Bennett from the church nursery he is happy but quickly cranes his neck to look for "Daddy." When I leave him at home with Mark he says, "Bye Mommy" but when Mark leaves he cries. It is really cute how attached he is to his Daddy. Last night Mark took Libby to her final softball game of the season and I stayed home with the boys because of the heat (and we have lots of gnats for some reason this year). Poor Bennett got his sneakers out to go with them. He proceeded to cry for almost a half hour, carrying around his shoes and crying "Daddy." Nathan and I tried ice cream, books and hugs but were unable to console him. Finally we were able to distract him by playing several rounds of Hullabaloo!

Here is a very fuzzy picture of him I took with my cell phone. I had to text Mark and show him what he was missing!

Animoto

So last night as I was trying to create a slideshow I discovered Animoto! Here is my first. Some of the captions are at the wrong points but oh well.

Phillies!! {warning-many exclamation points}

As many of you have heard by now, we had a great night at the Phillies game last night! A friend of a friend, Kate Sheilds, contacted someone she knew at the Phillies and and our family was invited to attend a Phillies game and go on the field for batting practice prior to the game. First of all, it was a beautiful night weather-wise. We arrived at 5pm and the kids were given some gifts, hats for the big kids and a plush Phillie Phanatic for Bennett. Then it was off to the field. We saw several of the players take batting practice, Victorino, Utley and Howard. It was amazing to be on the field, behind home plate and see these figures that you see on television, walking around in front of you! It sounds so silly but the green of the field, red of the clay and the bright uniform colors still take my breath away.

Sorry they are out of order. I spent a lot of time trying to find a free site to create a slide show without people having to go to another site. I finally figured it out and didn't have the energy to reorder them.

Our One Tooth Wonder!

Bennett brought the pretzel jug to me the other night with a cup saying "pretzels please cup." I was downloading ebay pictures at the time so I had my camera right next to me.

Bennett still only has that one tooth. It does not seem to hold him back to much and bothers me more than him. Mark took him and Nathan to the dentist. He offered and insisted because he knows that these kinds of appointments often bring me down. Yes, I am a very lucky girl in the husband department! Although I sent along some books for him to have Nathan learn to read (Bob Books) while they waited. Instead Nathan informed me that they played the video games that are in the waiting room and only read a couple of pages of one book!

Anyway, his teeth are indeed under there but the dentist said his gums are very thick. If more of his teeth have not erupted in the next six months, they may consider cutting the gums. I could not find anything on the internet that describes this procedure or gives it a name.

Bennett is becoming quite the little boy. He is talking a lot, saying lots of phrases such as "need help please-stuck" or "Daddy come here." He also sings some version of his ABC's but will do none of this on demand. He is quite affectionate and has to hug each of his teachers before he leaves school. He also reportedly kissed another little girl in his class on the lips! Yikes! I am really enjoying Bennett. Two is definitely one of my favorite ages.

I also forgot that I took Bennett for an ear recheck last week. The ENT at CHOP recommended that we come back in three months to see if Bennett still had fluid in his ears and if so, they would consider tubes. Instead of waiting several hours at CHOP, I opted to go to see our regular pediatrician. Dr. L said his ears looked pretty clear but since he has a cold, he wants to see him back in one month to make sure it is resolved. So he does not appear to have fluid in his ears any longer but does have some junk in there from the cold. Good News!

Friday is Nathan's kindergarten orientation for which I am very nervous. I will be taking him (and Bennett in tow) to tour the classrooms, meet some teachers and the principal. I'm not sure what I am so anxious about. I guess other children asking about Nathan or other parents avoiding me because my kid is different. Every parent wants their child to fit in, not stand out and be accepted by their peers.