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Friday, April 24, 2015

RIP Dr. Derick Sheppard, Grey's Anatomy

It has been all over Facebook....people crying, people want him back. Dr. Derick Sheppard died last night on Grey's Anatomy. (Sorry if this is a spoiler for some). It was acting for Derick and Meredith, just a TV show, a job, but for some it is reality. It could be our reality. 

Accidents happen, people get hurt, people die, it's all part of life. It is a VRK1 mutation, PCH1A; for us, no accident, a genetic mutation. What are the chances, one in how many...no one knows. PCH1A is something you have never heard of before Ian, something we have never heard of before July 2012. Regardless of the name it has not changed our reality, it would still be the same.

Watching the nurse turn the life support machines off on Derick, was heart wrenching for fans of the show. For Brian and I, we saw our baby laying in that bed, envisioning what it would be like....are we going to have to take him off the vent or will it be something else? Last night was not the first time we have envisioned this or spoken about it, we are well aware the time will come when we may need to turn the vent off, take him off life support...it won't be fair to him to continue living the way he is...we are well aware. My emotions were a little out of sorts watching the show, to be honest they still are....my mind keeps going back to the scene from Grey's. What will it be like, is that how it will happen, how much reality do the writers put into the show? 

I know over time the image will not keep running through my head...it will get easier and I will forget, well at least for a time being, as the show did not put anything new into our minds, it just reinforced it. 

For now, we will try to treasure yesterday, dream of tomorrow but live for today.

Wednesday, April 22, 2015

Happy 12th Birthday and How We Are Doing

First and for most, I want to wish my sweet girl and boy a very Happy 12th Birthday. Both of you have given mommy and daddy, along with so many others such joy over the years. No one knows what their life journey will be and what a journey it has been..one which we continue to treasure, dream and live....thank you both for giving us special days, each and every day.








We have been doing great since being home. Ian continues to heal well, resting, sitting in his chair for a few hours a day, even getting outside on the porch a little bit. He has been more comfortable in bed and not needed to be re-positioned as often as he used to. His surgical site looks wonderful and continues to show signs of healing every day. We could not be happier with the way he is doing. Becca is doing well and back into the swing of school, hard to believe it is the last quarter of 6th grade. Girl scouts is coming to a close soon as well and then before we know it, she will be off to all of her summer adventures. She has her moments of dealing with things, of having to grow up to fast and being more mature then a lot of kids her age...of being a typical 12 year old...we are working hard for her to be able to do more things outside of the house with friends...after it is all about being a kid when you can be one...isn't that what we all want for our kids. 

We are looking forward to what this year hold for us on our journey through life with PCH1A....the ups, the downs, the good, the bad, the pretty and the ugly....one day and step at a time. 

Thursday, April 16, 2015

When Your Medically Complex Child is Never Really 'Out Of The Woods'

Originally appeared on Feeding Raya

Parenting is hard. There's so much to worry about. Throw in complex medical conditions and what's considered "hard" about parenting changes. The things at the forefront of the mind are different for parents of medically complex children. The stresses of parenting a child without medical conditions certainly exist, and they're real and valid. For parents dealing with medically complex children, those typical worries still exist, but they have to take a back burner to more basic, primal needs that keep the child alive.

The term "medically complex" is a vague umbrella term used to describe a wide range of medical conditions that present unique challenges and require special care on an ongoing basis. There are no official parameters regarding what conditions fall under the umbrella of "medically complex," but chances are if a child requires ongoing care with a herd of doctors and therapists, he or she is medically complex. Many medically complex children have diagnoses of genetic conditions, diseases of major organs and neurological disorders, and many have a collection of symptoms with no diagnosis.

Parenting a medically complex child means not only providing the usual care and nurturing but also becoming an expert on whatever medical conditions affect the child. It means learning how to operate medical equipment, perform medical procedures, use the various tubes and devices attached to the child and acquire an entire vocabulary of medical terminology. It also means preparing for any of he potential emergency situations that could arise and knowing that danger of these emergencies happening always lurks in life's shadows.

Parenting a child with any type of special needs changes the way the mind works. The blinders come off, and nothing can ever be looked at the same way. Acknowledging the darker side of life does not make parents of medically complex kids pessimists; it just means they're doing their best to cope with the often ugly and terrifying realities that accompany their children's medical conditions. From an outsider's perspective, it can be difficult to understand that no matter how good a medically complex child looks, he or she may never really be "out of the woods," so to speak. Medications can keep symptoms at bay, and special diets can help control the damaging effects of disease. Feeding tubes can ensure adequate nutrition and aid in overall health. Insulin pumps can keep blood sugars stable. Congenital conditions can be repaired surgically. All the medical interventions parents of medically complex children learn to administer can keep their children in a state of optimal health for their conditions, but all of that can change at any moment. In the blink of an eye, things can go from calm to complete disaster.

This is why we're stressed out and why we can't completely relax. This is why we may find it hard to listen to people talk about the more typical concerns of parenting and why we sometimes seem flaky or uninterested. This is why, no matter how good things are going, we have a hard time not living like we're waiting for the other shoe to drop. Even when everything appears to be safe, we never feel like we're out of the proverbial woods because mentally, we can never really leave the woods. It's all we've known since our medically complex children entered our lives, and we can't erase the memories we've made there. We see terrifying and devastating things happen to the friends we've met while living in the woods, and we understand those things could happen to us too. Next time, it could be us.

If we seem a little overbearing, overprotective or paranoid, know it's for a good reason. Just as any parent dedicates his or her life to caring for his or her children, we've dedicated our lives to caring for our children, but what that requires of us is different. Living in the woods is not what we planned for our children and certainly not what we wanted for them. Since it's beyond our power to change that, all we can do is do our best to enjoy the scenery, keep living and create a safe and happy life for them, even if it is in the woods.

Wednesday, April 15, 2015

The Birthday Presents

Today, Ian had school and he worked on a sheet called The Birthday Presents. He had to pick 5 presents on the sheet and write a sentence about each one. Here are his sentences:

1. Ian wants the DVD Thor: The Dark World because Thor is brave like me.
2. A spiderman sheet set would be a good gift because spiderman is my favorite superhero. 
3. I have always wanted a train set to build up a little town and watch the train go around. 
4. I would like to go to a baseball game to get an autographed bat and ball. 
5. A big surprise party would make me happy because I like being around family and friends. 

So here are the answers Brian and I gave to him.

1. Ok the DVD is something we can get him for his birthday. 
2. While we would love to get him a spiderman sheet set, they don't make them to fit hospital beds. So we asked him if he would settle for the blankets and pillow cases he already has. He said yes for his bed in his room but what about for his bed in the den.  We continued to tell him that his bed in the den is also a hospital bed. His response, oh man.
3. Train set: he has one....and has told us to give it away, so no need for a new one. 
4. The baseball bat and ball, since we were in his room this one was easy....we pulled out his 2 baseball bats that are autographed as well as his 2 baseballs that are autographed. One of the balls is the one from 2 summers ago when he threw out the first pitch.  So he has eclipsed the basic autographed bat and ball. 
5. The party: told him to forget he knows anything about his party and then it will be a surprise.

This was just classic....too good to not share as well as for me to document so we remember it. 

Sunday, April 12, 2015

FOR GOOD by WICKED

Last night, Brian and I saw WICKED, the song FOR GOOD, made me think of Ian. Here are the words... And while I may not always feel it or show it.. But because of him I am better and changed for good.

I'm limited
Just look at me -- I'm limited
And just look at you--
You can do all I couldn't do, (Ian)...

Because now it's up to you
For both of us--
Now it's up to you

I've heard it said
That people come into our lives for a reason
Bringing something we must learn
And at are led
To those who help us most to grow
If we let them
And we help them in return
Well, I don't know if I believe that's true
But I know I'm who I am today
Because I knew you...

Like a comet pulled from orbit
As it passes a sun

Like a stream that meets a boulder
Halfway through the wood
Who can say if I've been changed for the better?
But because I know you
I have been l changed for good...

It will may be
That we will never meet again
In this lifetime
So let me say before we part
So much of me
Is made of what I learned from you
You'll be with me
Like a handprint on my heart
And I know you have rewritten mine
By being my friend

Like a ship blown from its mooring
By a wind off the sea
Like a seed dropped by a skybird
In a distant wood
Who can say if I've been changed for the better?
But because I knew you...

Because I knew you...

Because I knew you
I have been changed for good...

And just to clear the air
I ask forgiveness
For the things I've done you blame me for

But then, I guess we know
There's blame to share

And none of it seems to matter anymore

Like a comet pulled from
Orbit/as it passes a sun/
Like a stream that meets a
Boulder/ half-way
Through the wood

Like a ship blown off its
Mooring/by a wind off the
Sea/ like a seed dropped by a
Bird in
The wood

Who can say if I've been changed for the better?
I do believe I have been changed for the better

And because I knew you...

Because I knew you...
I have been changed for the good.

Thursday, April 9, 2015

Thursday, April 9

Happy Thursday....and that is what we are shooting for. Ian has already been out of bed for a few hours today and tolerated it well. Later today, he will get his chest vest, cough assist and his trache changed back to his home trache. They are going to do all of those things at different treatment times and in slow baby steps...better to make sure we will move forward then backwards. He will get out of his chair a few more times today. He is back on all of his home medicines and doses...along with an order for PRN pain medicines, just like at home.

We are on track for discharge tomorrow and looking forward to posting that we are out of here.

Have a happy Thursday.

Wednesday, April 8, 2015

2 West

Late yesterday, we moved from the PICU to the floor. All one step closer to coming home.  Tuesday night was fair for Ian, the respiratory therapists got some good secretions and a few plugs during respiratory therapy, the plugs are what made it fair, but over all he did get a good night sleep.

Wednesday has started off good for him. Physical therapy came in early this morning did his stretches. He says they are getting easier now that they are being done everyday. As of 12:50pm, when I am typing this Ian has been sitting in his chair for over 2 hours and going strong, they will be getting him back in bed around 1. He will need to sit in his chair again today for about 2 hours as well as get his stretches in again.  As soon as he was back bed, he was out.

Rounds were just done around 2:15, later then on the PICU. So the game plan is to increase his respiratory therapy and try some manual chest therapy. As long as he tolerates that well today, they may start his vest therapy tonight at a decreased rate then normal. Assuming he handles the rest of today well, tomorrow they will change his trache back to his home trache as well as start the cough assist therapy at a decreased rate from normal. If all this goes well, and Ian handles all of his home treatments with out any pain and he does not have any extra plugs we maybe able to split this joint on Friday. We can work on increasing his therapies back to his home rates once we are home. Ian has started to eat a little bit by mouth and continues to get feeds through his g-tube at night. He may need to go home on some g-tube night feeds but we will work on those going away once he is eating some more by mouth. Baby steps will get our superhero home.

Becca is doing well. A little bored from time to time. However, she is getting to spend lots of time with cousins she does not get to see often. We will have to make sure that changes once we are home, as I think all of the kids are enjoying each other.

So here is to the rest of today and tomorrow going well. Remember to treasure yesterday, dream of tomorrow but live for today.

Tuesday, April 7, 2015

Moving on Up

We are out of the PICU and on the floor, 2 west.

Monday Night April 6th, Plan for Tuesday April 7

The rest of Monday was good for Ian, he did well on the vent with his home settings, getting out of bed 2 times and just relaxing. He slept well last night.

The plan for Tuesday is simple, one which we have been doing for about 2 hours so far. To be on his home vent with his home settings. They switched him over around noon today. If he continues to do well and there is a bed available on the regular floor we will be out of the PICU later today. One more step to getting Ian and all of us home.


Monday, April 6, 2015

Sunday Niight and Plan for Monday (April 5th and 6th)

Yesterday, Ian ate a little bit of food, well about 5 sips of ice tea and 2 spoonfuls of rice and cheese. He slept well. Brian said that it was a quiet night for Ian...not for Brian.

Yesterday morning, Nana and Pop-pop came to visit and say good bye before heading back home today. Later in the afternoon, Nana and Pop, Aunt Susan and all came to visit and have dinner for Nana's birthday...happy birthday Nana, we hope you had a good day.

Another chest x-ray was done this morning and the unofficial word is that it looks much better then yesterday. So we are moving along with that and saying good by to collapsed lung.

So the plan for today, to get Ian back onto his home settings on the servo, we thought he was back there last night but he was not. So he will be decreased every few hours getting him there by the end of the day. As long as he does well on the servo with his home settings he will be moved over to the LTV (his home vent) tonight. Once we can get him on the LTV and he does well, we can get out of the PICU. Being on the servo vent is the only thing keeping him in the PICU. Next, will come getting his trache changed back to his 5.5 uncuffed but that won't happen until he is back on the LTV and stable with that.

The other day they needed to put an NG tube in to get extra air out of his stomach, that has been successful. The NG tube will come out sometime today. The hope is that with the tube out, Ian's appetite will increase and he will eat more. Getting him to eat more will reduce the need to increase his tube feeds.

Another change for the day, is to decrease his morphine to 3mg every 8 hours from 8mg every 8 hours. He is still on all of his normal pain medicines from home. They will be stopping the IV antibiotics today as well but keeping him on his home antibiotics. The morphine is the only extra medicine he is currently on.

Thank you to everyone who has sent e-cards. If you would like to send Ian an e-card, you can go to www.nemours.org. In the bottom right corner of the main page click on send an e-card. Choose the Wilmington, Delaware location. Ian is in the PICU room 25. I know he will love to hear from everyone. 

Here's to another day of moving forward.



Sunday, April 5, 2015

Rest of Saturday, Saturday Night and Sunday Morning

Ian slept most of the day on Saturday. They did a chest x-ray around 3pm and the pneumothorax (collapsed lung), appeared to get a little bit better than the morning xray. But because of this, the lung is not able to expand all the way out to the chest wall. They will repeat the chest x-ray at 10pm. The 10pm chest X-ray showed a little bit of improvement. His night was pretty good considering he was woken up every few hours for his treatments. Once that happened he would cluck for something, hence my sleep was not so good.

In addition, he started running a slight temperature, which caused them to run a culture from his central line and started him on antibiotics. They don't want to culture his trache because it could make the collapse worse. At this time all his cultures are pending.

So the current plan is to the get the servo (hospital vent) onto his home vent settings. Once he is stable on the servo for a little while they will move him back to his LTV (home vent). Once he is stable on the LTV, they will change him back over to the uncuffed trache. They will only get X-rays once a day in order to really see what is happening.

Within the hour, all of his medicines will be converted back over to through his g-tube except for his as needed morphine. We can start him on clear liquids, really we were told that once we got him to drink a little bit of ice tea and he handled that well we could begin giving him food again. So hoping to do that later this afternoon. The NG tube they placed yesterday will stay in for the time being.

So we are really back to where we were on Friday....here is to more steps forward and no more steps backward. 

Saturday, April 4, 2015

I LOVE MY SON by Brian

THIS.....mister sleeping beauty.  Im just sitting there in silence, dumbfounded that he had the audacity to fall asleep on me just like that.  Marci sees me sitting there and asks whats wrong.  I dont respond and then she asks if he is sleeping and I just shook my head yes.  Marci starts to laugh like its the funniest thing she has ever seen or heard.  Then precedes to tell me that I have bored my son to sleep.  I think I have now hit an all time low and new record with Ian.   Not only did he apparently not want to build the thing, he fell asleep within MINUTES!!!
Therefore, as the title says....I LOVE MY SON!!!

Mommy, Daddy and Sister

Many of you have been asking how mommy, daddy and sister are doing. We are all holding our own. I think once we got through surgery on Tuesday, we could all breath a little bit better and relax a little bit. Brian and I have been taking turns sleeping at the hospital and the Ronald McDonald House. It has been nice to get a good night sleep there as well as breakfast before coming over. During the week, they have a very simple breakfast of cold or hot cereal. On the weekends, they have volunteer groups come in for a hot breakfast.

Becca has been spending most of her days with us at the hospital and sleeping at the RMH with whoever is there. After Tuesday and Tuesday night, she seems to be doing much better. You can tell that she is relaxing a little bit but very happy to see Ian each morning.

We have been having family and visitors each day and we as well as Becca have been spending time with them. Becca got a treat yesterday to spend some time with cousins who live in Wilmington...she came back with a smile on her face.

We will continue to take each day as it comes on this part of our journey. We would love visitors but please check in with us before making the drive. Thank you for all the e-cards as well as the other gifts you have been sending. I love reading all of your messages on facebook as well as comments on the blog. Thank you for all of the support you have shown us.

Happy Passover and Easter to all those who celebrate them.

Remember to TREASURE YESTERDAY, DREAM OF TOMORROW BUT LIVE FOR TODAY.

Saturday

Friday night was a relatively good night. His hemoglobin levels had dropped again a little bit so they did give him one unit of blood. When they checked them this morning they were around 12.5, where they want them to be. They will check again tomorrow morning, unless they see a reason to do so before hand. 

Around 8am, his morning turned a little rough. Ian started having some breathing issues. A chest xray showed a little bit more collapse on his left side. So they changed him back to the other vent again for now.  While on this vent he can't move out of the PICU but this is where he needs to be for now. 

In addition, his belly is still so rigid they took xrays and don't see anything, except for a lot of air. To help alleviate the gas in his stomach they put a tube down his nose and in to his belly to help it faster. I asked if this is normal and was informed it can be normal especially with all the increased pain medicines he is getting. They changed the morphine back over to IV from G-tube. Since the x-ray did not show a blockage, all other medicines will go back to his G-tube. 

 Ian seems to be relaxing and comfortable at this point.

Friday, April 3, 2015

Thursday Night and Plan for Friday

We slept pretty well last night, up every 4 hours for treatments but back to sleep pretty quickly. They have been checking out his lungs with x-rays, they are looking nice and stable and expanding back out to the chest walls. All normal following this procedure. The goal from a pulmonary standpoint is to get him back onto his home vent settings and his home trache. Today they will change his vent settings on the hospital vent and if all goes well with that later today will change him back to his home vent with changing the trache tomorrow.

His blood pressures are still a little high, however, Ian is still on  steroids which can increase your blood pressure. Steroids will be discharged today and his blood pressure will be watched.

Hemoglobin, the protein molecule in red blood cells that carries oxygen from the lungs to the body's tissues and returns carbon dioxide from the tissues back to the lungs. Ian's hemoglobin is low, they will continue to watch it but he may need a transfusion in the next day or so.

Pain management has been pretty good. They are going to put in a referral pain management team so when the time comes for him to come to the floor they will know about him and come see him. In addition, he is currently on on IV morphine, they will be weaning him to morphine through his g-tube. They intend to wean him off of the morphine and back onto his home pain medicines, only.

Ian has been on very little g-tube feeds however they have oked him to start on clear liquids...now to figure out what he will eat.

The hope is to get him out of the PICU on Sunday or Monday.

And for the big surprise of the day:
SITTING MAN





Thursday, April 2, 2015

End of 2nd Day

Today has been good, made some progress and one backwards move. He is off of the fentanyl medicine but now on morphine. He has gotten some lasix to help get rid of the extra fluid he has retained over the last 2 days. In addition, they tried to change his trache from the cuffed one to the uncuffed one (which is his usual one), however, Ian was not ready for that. He handled that for about an hour however, his breathing was labored and increased so the decision was made to change him back to the cuffed trache. Once this was done, Ian's breathing was not as labored...slowly getting back to normal. In addition, his pain was out of control for a little bit this evening but with an extra dose of morphine he is good to go...I keep asking if the mother can get some of the good pain meds...but they frown upon that.

For all of those Camp Simcha Special people out there....we have a wonderful doctor taking care of us tonight....Dr. L is in the building. 

Have a great night, we are hoping for one here.
 

2nd Day and Night

Wednesday, April 1, 2015 was a good day. Ian was up on and off, maybe a little bit too much up with all his company. His pain seemed to be under control yesterday but that was with lots of medicine. Brian stayed at the hospital with him last night and said it was up and down but nothing to worry about. His blood pressure was up and down so they increased his medicine again.

From rounds this morning, they are planning on decreasing his fentanyl and starting him on round the clock morphine. By the end of today, he will be off of the fentanyl. They will be finishing the antibiotics they have him on and he will begin his home antibiotics. As long as PT and the orthopedic agree, they are going to work on getting him up into a chair today. Can't wait to see him sitting up straight. They are not concerned about his blood pressure, his nurse said that the up and down is very normal after such a traumatic procedure.

Everyone says he is making good progress. We are hoping for a much quieter day today...

If you would like to send Ian an e-card, you can go to www.nemours.org. In the bottom right corner of the main page click on send an e-card. Choose the Wilmington, Delaware location. Ian is in the PICU room 25. I know he will love to hear from everyone.

Wednesday, April 1, 2015

After Surgery

Yesterday went much better than expected. Ian rocked the surgery. He lost less then 300cc of blood, did not need a transfusion, and as you can tell from the photos below gained some inches.  

His night was good. He is still sedated with pain medicine. His blood pressure dipped so they have started him on medicine for that, in addition to pushing some extra fluids. We have been assured this is all normal for a day post-op. They seem to have it all under control. 

Rounds were good this morning. His carbon dioxide level was elevated to 58 but has since come down to 47. They like this to be between 35-45 but will continue to monitor and change his vent settings to adjust. The hope is that he will get back to his normal vent settings. In addition, this will improve as they begin some of his pulmonary toileting. The plan is to start IPV every 4 hours as he will not be able to handle his normal toileting. 

As for his pain, that is under control. They will decrease his fentanyl pump today in order for him to be more awake today. It was up last night in order for him to sleep and to stay pretty much sedated. He was just up for a few minutes and the first thing he asked for was TV and his glasses. 

The physical therapists came in this morning and did some range of motion on him. She discussed having someone come in and modify the wheelchair tomorrow, or at least begin the process.

Looking forward to a calm and easy day for Ian.
Before                    After