Bennett takes Boston (Children's Hospital at least)

For our June trip to Boston, Bennett was at a tricky age. He couldn't walk yet but was ready to be on the move so containing him on the bed in the hospital room was challenging. We figured that it would be easier this time because of his ability to walk but we also thought maybe he would be old enough to present some challenges in the behavioral area. He was much much better than we expected! Yes he is more feisty than Nathan and does not put up with as much poking and prodding but overall I would say he was pretty cooperative! As anticipated it was much better with him walking. He was able to get out of the stroller and play so much more which made him a much happier kid!

Bennett's nurse for the week was Miss Courtney. She was just as nice as Miss Ivy but by the end of the week Bennett just cried when he saw her. Poor Miss Courtney. He wouldn't even get close for a picture. Hopefully she'll get the better patient next time.

Each trip to Boston gets a little bit easier. Knowing what to expect and knowing all the people is a huge benefit. For me there is even a certain comfort to being there. Mark disagreed but for me I think it is about being around people who have seen other kids with progeria. The staff at the hospital and the Devon Nicole House are all so sweet and we don't have to explain ourselves to anyone. They already know.

Getting to know Nathan

One of the benefits of this trip was getting to know Nathan a little bit better. Libby is an intense child and when she is around, her personality, emotions and bright ideas permeate every available inch of airspace. Of course we love her so much but it has left us wondering sometimes if Nathan is really in there. We honestly wonder sometimes because his choices and opinions rarely vary from his big sister’s. Think “I’ll eat whatever Libby is having?” Libby picks up on little things in conversation while Nathan appears to be oblivious about what is going on around him. We are never sure if this is a cognitive thing or a boy girl thing.
So, it was nice to spend some time with Nathan outside of the shadows of his sister. And it turns out, he is in there!! He remembered lots of things from last year, had opinions about things and most of all he talks nonstop!! Oh my gosh can he talk!! It is so interesting how kids are smart in such completely different ways. We taught Nathan the values of change pieces this weeken and he pretty much got it right away. But then Libby struggled for so long learning change but can learn her way around an electronic in no time! In addition we realized how much Nathan can do independently that we just ask Libby to do because it’s easier for her. He is so ready for more independence. Although he is limited because of his size and strength we need to be better about giving him responsibilities regardless of how slowly he does them. Since we got home I have been letting him pick out his own clothes. It is a lengthy and scarey process as he has to get the stool and stand on the stool while he is pulling out the drawers. It just takes some extra planning on our part, helping him get up and down the steps etc. but he is so thrilled with himself! We are so focused on his health that tend to forget how important this stuff is for his development. Or maybe its not forgetting, maybe we just don't have the energy!

I jokingly said we were going to teach Nathan to read this week so he is ready for Kindergarten. I took some Bob books and a Leapfrog tag reader but we spent little time on reading unfortunately. But he did learn how to swallow pills which is HUGE!

Two doctors can't be wrong.....

So, the good news is that both doctors were wrong. They were right that they heard rumblings in Bennett's neck but there is no narrowing of the blood vessels or blockages. The doctor at the vascular lab said that Bennett's blood vessels are curved just like the other kids with Progeria which is why they heard turbulence in there. So so happy and relieved about this!

Right before we were going over to the vascular lab for the ultrasounds, Nathan showed us his finger which he said was feeling weird and changing color. Sure enough it was white and then blue. I was in a panic and I sense that Mark was too but of course he tried to hide it. Fortunately we were going to see the cardiovascular people. So, I was hysterical and Mark asked her to look at Nathan's finger first and she did a test and determined that Nathan has.......Raynauds Phenomonen It is its own diagnosis and she believes it is unrelated to Progeria. The solution is to keep his hands warm (we were inside though) and if they change color to put them under his arm pits to resolve it. The only time we would need to contact a doctor would be if the finger did not return to normal within a few minutes. My concern (which I asked her about) is this: Nathan already looks much different and now in Kindergarten he is going to have to walk around with his hands tucked in his armpits??? At least I made her laugh!

So after hearing both of these results, we left the vascular lab on cloud nine! Its so ironic how things like this can put us in such good spirits. Meanwhile they still have a fatal disorder, but we dodged some bad news that day. I guess with something like this you have to find the small reasons to celebrate and the little ways to have fun. Small improvements like a pound, an inch or a tiny bit more strength makes us giddy!

Bennett waking up

Okay, I have lots of good things to tell you about our trip! Things turned out very well in the end despite an additional last minute scare by Nathan. But this video deserves its own post so here it is. It is 2 1/2 minutes but well worth it. Just when you think he is awake.........he's not. It went on and on after I turned off the camera too but I felt cruel already.

More Pictures

Playing Ms. Pac-man- very fun and addicting game!

This ball thingy is in the middle of the lobby. The boys (and many other kids) could stand there for hours watching!



Bennett getting ready to drive the Cozy Coupe-lots of fighting over it this week!

Just Nathan


Nathan and his favorite nurse-Miss Ivy!

Mark posing-he knows he's handsome so no need to tell him the next time you see him!

Another ball thingy on a different floor.

Taking our first shuttle ride from the house to the hospital yesterday. We always walk which we love but yesterday was too too snowy!

Bennett getting ready for blood work. She wasn't doing anything yet but by Thursday he caught on and started crying whenever his nurse came in the room.

Okay, Mark and I were fascinated with these! They are little "trains" that travel alone through the hallways delivering meals to the kids. It makes a train whistle sound, turns corners, opens doors and moves around you if you get in the way!! The only thing we didn't figure out was how they get on the elevator. The sign on the back says to let them ride the elevator alone but we never saw it actually happen.

Thursday!

I am sitting in the sub-basement of Children’s Hospital of Boston (you guessed it, the floor below the basement). We are so far down that I can’t even get internet access so I am writing in Word to copy and paste. Nathan and I are waiting while Bennett is having his medical photography done in the next room. Watching the kids get blood work, sit still for long periods of time and get examined repeatedly is tough but having your two year old being photographed naked for a half hour by a complete stranger to be viewed by complete strangers is just creepy. The other things I can do but this I cannot. Mark is very sweet and does anything to protect me from these things that are so difficult for me so he is in with Bennett. He tried to send me back to the DNH so that I didn’t even have to be this close to the creepiness but I am trying to suck it up and deal (even though now Bennett is crying).

Yesterday turned out to be an okay day. We were anxious to venture out so we went to our occupational Therapy appointment for both boys at 10am and then had a very long lunch in the cafeteria. Everywhere you look in this hospital there are things to entertain the kids so it is easy to spend a whole day here. After lunch we went and had their x-rays done which took a while. Today we got our two shopping bags of medicine, more blood work, another urine bag for Bennett and now the photography. Tomorrow we have to be here at 7:30 am for the ultra-sound with cardiovascular people. This is what will determine the cause/extent of the rumblings in Bennett’s blood vessels. They will be able to determine immediately if there is a blockage but it will take longer for them to tell us about the extent of/if any narrowing (I can hope right?). Please pray for a miracle! They decided to go ahead and prescribe aspirin for Bennett based on the two doctors opinions that there is turbulance in there. I can't believe it!! Two years old!! Anyway, it will be nerve-wracking in the same way it is when you have an ultrasound during pregnancy-you know, vigilantly trying to read the tech's facial expressions and watching to see if they slow down or go back over certain areas. Anyway, then it is off to dentist appointments for both (rescheduled from today) and Dexa Scans for both boys. Then we are off to the airport for our 6:30 pm flight! Yayyy home!! It’s that time of the week! We are very tired.

almost wordless wednesday

Signs of Progeria

Well of course we have always known they had it but we thought it was a much milder version. Unfortunately we are seeing lots of signs that they are close to having the real thing. Nathan has a callous on one of his feet due to the lack of subcutaneous fat on the bottom of his feet. The bone on the bottom of the foot pounding continuously creates the callous. So we need to schedule an appointment with a podiatrist for both boys as this is going to be a "life-long issue" There is that term again, life-long medical issues. Its something that still comes as a shock to me. Something I am in denial about I guess. It slaps me in the face everytime. I don't quite get why I am still surprised when I hear the words, but I guess it is just like the physical symptoms for me. I see them everyday but every time I look at their fingers or bulging veins, it saddens me. How can that be?

Anyway, the very very sweet neurologist confirmed the "rumblings" in Bennett's neck which she heard on both sides and not just one. This is indicative of some narrowing of the blood vessel walls which can be confirmed by the ultrasounds on Friday. It is something that is typical of Progera but they as you can imagine it is pretty shocking for us. I asked if it is reversible. They do not believe it is reversable but do think that with aspirin, further narrowing could be prevented. Nathan has already been on aspirin for the last six months.

All but Occupational Therapy for tomorrow has been cancelled due to the two feet of snow they are expecting. I didn't think New Englanders got too excited about snow but apparently two feet gets them riled up. They are already talking state of emergency, non-essential workers kind of terms. Since the x-rays are a walk-in appointment, we decided to wait and do them tomorrow. That way we can get out even if OT is cancelled. Being in a 15' x 20' room with two little boys all day is not ideal either. There is a total of about 4 square feet of open space in the room so it is pretty tricky!

So far....

We have a lighter schedule than the first visit one year ago. We did not realize that we do not do the MRI and/or the Audiology appointment this time. They are only done at the initial and the two year. That doesn't seem like a big deal but it involved a huge chunk of time pre-MRI appts, sedation for Bennett and going to a different building for the audiology appt. It will make for a much easier week. So far we have only had examinations, EKG and now we are doing physical therapy evaluation. Bennett has some possible "rumblings" in his blood vessels in his neck so they will find out on Friday at the cardiovascular ultrasound what this is all about.

And I don't want you to be dissappointed. I already did it.....

We are here!

We made it to Boston! We flew this time and I am still not sure which is better, driving or flying. We left our house at 2pm and arrived at Devon Nicole House at a little after 7pm. We probably saved an hour by flying but it is quite a hectic process. We have been very fortunate not to have ever had any major delays either. I'm trying to convince Mark to drive next time but I don't know if he is convinced.

It looks like we have a pretty easy day today. We don't have to be at the hospital until 9:45 am. Its so much better now that Bennett can walk around. The last two visits were tricky trying to keep him on the bed or in the playpen rather than crawling around on the floor. Nothing else new and exciting. I just wanted to let you all know we arrived safely.

Down and out!

I have been very depressed lately about the boys. On some days it is a minute to minute decision to keep moving forward and not go in my room and hide. I am so proud of myself on most days for not returning to bed and hiding from the world. I am never sure what prompts these low times. I can only assume it is the continued loss of Nathan's hair and Bennett looking more and more like a child with progeria rather than Bennett. Something so little as the fact that Nathan and Bennett no longer needs haircuts can sink me for a day!

It is suffocating going through this second time around and knowing what is to come. It is a different set of feelings and emotions. Just when I think I have experienced every possible feeling, more complicated ones follow. I don't realize that Nathan is continuing to change until I look at younger pictures of him in which he has a little bit more hair and a little bit more fat on his cheeks and face. In the area of physical development, both boys really seem to be improving, but it is a weird thing to watch and help them improve while also seeing the negative changes working at the same time. And I feel like such a vain person that I am so focused on physical cuteness but lets face it, we are all that way. Bennett is just physically so cute right now and I know that will not always be the case. Nathan is certainly cute but probably just in a way that only those who love him can appreciate.

Boston

I know I have not updated in a while and the truth is that I have tried to several times and been unable to focus on what to write. There are so many aspects of my life and our story that I am never quite sure which direction I should come from at any given time. Right now we are getting ready for our trip to Boston. We leave tomorrow and I have been dreading it. I almost think it is worse knowing what a difficult week it is for the boys and for Libby. We are so grateful for all that PRF has done for the boys and continues to do for our family; however, the week is physically and emotionally difficult for our family. Bloodwork multiple times daily, CAT scans, MRI's, x-rays, measurements and repeated examinations are exhausting to watch your kids go through. I can see that this year will be more difficult with Bennett being a little person of his own. Last year he was a baby and although it was difficult, I anticipate that it will be more difficult now that he knows what is going on. And then of course there is hearing all the things that are wrong with them. In June when we went I had thoughts that the kids were improving but now they both seem to have plateaued. I'm thinking that maybe we will be pleasantly surprised because really it is about what is going on inside their little bodies. We have not had any hematomas, no major falls, no broken bones and no major illness in more than a year! How fabulous is that?

late pictures

Hang onto your hats ladies and gentlemen because I am writing several blog entries to make up for lost time. Here are a couple of pictures from Bennett's birthday last month. I'm a little late but thought I would still share them. This was the first year that Pop Pop Falcone did his own shopping for birthday and Christmas. Judging by the look on Bennett's face with the Elmo book, he did a great job!