Declan beginning of 2017

Since I haven't posted in a long awhile I wanted to give an update about Declan and how he has been.  He is turning 5 this month! I never thought we would get here, but it has gotten easier throughout the years.  He has plateau'd on some things and gotten worse in others.  We still quarantine ourselves during winter.  Everyone knows not to come near us if they are sick and I try to keep us as healthy as we can, but school and work don't help.  We have done great this season so far, only had the flu once and we got the tamiflu and kicked its butt quick.  The cold weather on the other hand has not helped Declan or us.  When the pressure is high it is the worst for him.  His whole body hurts and he can't sleep... which mean we don't get to sleep either.  We can't take him out when it is heavily snowing either.  Since I haven't learned how to turn into the hulk in order to carry his 300 lb wheelchair.  Maybe that can be my goal this year?  ha ha who are we kidding. So I am sure we are getting some cabin fever and may just go crazy.  So it was disappointing to hear the groundhog say 6 more weeks of winter.  I hope he is wrong.  I'm not sure if other kids like Declan have similar issues with the weather.  But cold is not our friend.
Declan is in his last year of preschool, and will be starting kindergarten in the fall.  Nervous is the word I would use, but I am positive he will do great.  He has been in preschool since he was three. Making friends where ever he goes.  He is talking much more but is still delayed so we have had speech therapy and are looking into getting some communication help with programs on tablets and such.  Look forward to those posts I'm hoping to get the ball rolling this year. We still have PT and OT therapies.  We are trying to maintain the movement he has, but those pesky contractors have given us a lot of trouble.  His right arm, elbow to fingers is where he has the most movement.  Due to an accident in August which broke his right leg femur we also found out both hips are dislocated.  They said it is normal for children like Declan that sit in a chair all day for this is happen. The pelvic bone flattens out and can't hold in the bone to keep it in place.  I still think it causes him pain and now he can not lay flat on his back so I wanted to talk to the doctors about putting them back in. Which mean hospital stay...ugh!!  His legs are still bad.  He is at a 45 degree angle and all we can do is stretch him and rub him down.  We've talk about surgery to cut the tight tendons but it will cause a lot of scar tissue so they don't like to do it until the child is 10 years old.  So we have some time.  We will be doing surgery on his right ankle though.  I don't know when but it is on the list to get done. Picture below:

right ankle turned in.

It isn't considered a club foot but that's what it looks like.  I don't know if this causes him pain, but we can't use the stander to help with weight baring and or the dafos on his feet.  It has progressed to an almost impossible location.  

His back, oh my his back I don't know if getting the Magec rods was good or not. I love the short 5 minute doctor's visits for the extensions every 3-4 months, but this poor kid can't keep his right rod connected to his pelvic bone.  Declan's bones are already soft and it doesn't take much for the clamp to get knocked off.  I've lost count how many surgeries this kid has had and I know we are in for even more.  The rods have helped a lot with his breathing though I can't argue that.  He has got a set of lungs for sure.  I also know if we didn't do something I think we would have lost him from all the pressure that was on his lungs.  The docs have us putting him on a bipap at night, and boy does he hate it!  We hate it.  It doesn't help him sleep better it makes him sleep worse.  Let me tell you almost 5 years of never sleeping through the night is rough and we value our sleep.  So when a machine makes things worse it is really hard to get on board to like it.  I think we will have a talk with the doc in the near future.  Some things you just have to say "stop, no more."  But I'm trying!!!  

I don't remember if I ever posted about Declan receiving his Gtube. We had to bite the bullet and take the plunge. When he got sick with pneumonia and we stayed in the hospital for a week they had to insert an NG tube through the nose and we couldn't get him off of it.  So we decided to go ahead and do surgery for the Gtube.  We've had a few ups and down with the it but overall it worked out well.  It is still very hard for Declan to gain weight and he is still on his own curve.  Following his own path.  Recently the hubby got a new job so that meant new insurance.  Turns out for some stupid reason United Healthcare has decided to not pay for any formula anymore, including those meant for gtube children.  Yeah stupid is a nice term.  So when we were looking at $280 a month for his formula out of pocket I went in search of something else.  I don't even remember how I came across it or why I remembered it.  A blended diet.  We use everyday foods, most of which I already have cause we eat them ourselves and use an industrial blender to puree the food and do bolus feeds throughout the day.  Well thanks to my mom and dad they gave me an early christmas gift, a Blendtec.  I love it!  It does a wonderful job.  It takes me about 10-15 mins to blend and cook Declan's food and put in a mason jar in the fridge.  Which we use throughout the day.  He gets 60-120 mls each feed and we feed him about every two hours.  It take about 5 mins to push it into his extension and we flush the line with warm water and done!  Over night we run water through his pump so he doesn't become dehydrated. There is a ton of help with support groups on facebook and I found a website called: www.blenderizedrn.com They can help anyone interested in starting a blended diet for their Gtube child.  Before we started the blended diet we could only get three cans of formula into him.  But with the blended diet we can get a whole jar into him throughout the day.  Therefor he is actually receiving more calories than before and I'm not going to be upset about that.  He can also eat what the family is eating spaghetti, meatloaf, chicken alfredo... doesn't matter, we just blend it!  I feel like he is eating healthier and staying healthier.  He will outlive us all.

one blended feed vs. it's equal calorie intake with formula

blending his food.

Even though we struggle we love this kid.  He is both frustrating and hilarious.  This is one wild ride we are on, and it is teaching us so much.  We have become a better family, we have become better people.  I may not be able to hang out with friends as much as I use to, or go to every party, but don't stop asking.  I'm still here just with a lot less free time.  Drop off some Dr. Pepper and a romance novel and I will love you forever!  
If you have any questions comment below.
Thanks for hanging in there with me!

Spinraza (A treatment for SMA)

Please note: I am not a doctor, I'm not telling you this treatment will work.  I am just providing information about spinraza. Talk with your doctor about the pros and cons of this drug for your child.  Do your research! 

Spinraza, it is a beautiful word to SMA families.  It's not a cure, but finally a treatment.  I'm so glad I was able to see this happen in my lifetime.  This means so much to the SMA community.  I'll try to post some videos of patients on this drug, and of course links.  Do your research, and I hope it will help you.
Unfortunately for us, Declan will not be taking this drug.  Since he tested negative for SMA but positive for one mutation with SMARD, this drug will not help him.  I'm hoping since we provided a treatment for SMA, one is close for SMARD.  May this treatment help the many, but our goal always and forever will be to come up with a cure. 

INFO:

• SPINRAZA is a survival motor neuron-2 (SMN2)-directed antisense oligonucleotide indicated for the treatment of spinal muscular atrophy (SMA) in pediatric and adult patients (1)
• SPINRAZA is administered intrathecally  (Intrathecal administration is a route of administration for drugs via an injection into the spinal canal, or into the subarachnoid space so that it reaches the cerebrospinal fluid (CSF) and is useful in spinal anaesthesia, chemotherapy, or pain management applications.)
• Initiate SPINRAZA treatment with 4 loading doses; the first three loading doses should be administered at 14-day intervals; the 4th loading dose should be administered 30 days after the 3rd dose; a maintenance dose should be administered once every 4 months thereafter.
• SPINRAZA is an antisense oligonucleotide (ASO) designed to treat SMA caused by mutations in chromosome 5q that lead to SMN protein deficiency. Using in vitro assays and studies in transgenic animal models of SMA, SPINRAZA was shown to increase exon 7 inclusion in SMN2 messenger ribonucleic acid (mRNA) transcripts and production of full-length SMN protein. (meaning it replaces a missing protein.) 
• Cost:    Spinraza, will not come cheap — and, by some estimates, will be among the most expensive drugs in the world.       Biogen, which is licensing Spinraza from Ionis Pharmaceuticals, said this week that one dose will have a list price of $125,000. That means the drug will cost $625,000 to $750,000 to cover the five or six doses needed in the first year, and about $375,000 annually after that, to cover the necessary three doses a year. Patients will presumably take Spinraza for the rest of their lives. (I am unable to find at this time what the actual costs are.)

______________________WARNINGS AND PRECAUTIONS ____________________
 • Thrombocytopenia and Coagulation Abnormalities: Increased risk for bleeding complications; testing required at baseline and before each dose.
• Renal Toxicity: Quantitative spot urine protein testing required at baseline and prior to each dose.


VIDEOS:

There are many more videos out there about how this treatment is working.  I love it!!!


LINKS:

Farrell Family
spinraza
prescribing-information.pdf
curesma
Biogen’s SPINRAZA
https://www.nytimes.com

Celebrating the little things

Here we are 19 months into the hardest, depressing, joyful, and educational 19 months I've lived so far.


   That was the sentence I saved from 3 years ago.  It's so amazing Declan is turning 5 in less than a month!  We can't help but to do a little dance and say HA! to all those doctors that said to prepare our selves. Like there was some sort of handbook for parents. "Best way to grieve..."  (face palm).  I don't think one can actually prepare one self to say goodbye to their child.  We never should out live our children, but it happens all the time.  Mom's and Dad's try to hold onto their children, their souls, and I think our souls actually cracks when we say goodbye.  So many things run through my head late at night.  How will I grieve? How will I love again? How will I still hold onto my other children?  How will my marriage survive?  Five years of me, fearing every cough, every hospital stay, every doctor's visit with more bad news.  But I will take the fear over the loss.  Because even though I fear daily for Declan, I can fight!  I would like to think in the flight or fight scenario I would fight till the death.  I'm just praying that Declan can tell me when he is done fighting so I never have to make that decision.  Until then I will fight, my husband will fight, our children will fight for their brother.  He isn't going anywhere.  
   Life is hard! It's depressing, and exhausting.  Parents try and try to make our children better than ourselves, while still adulting. The world is turning to shit and we are just trying to hold on to our little family, hoping that nothing will change.  It can consume you.  The hate especially, it's like a virus that spreads.  Now everything is online and it spreads faster.  Some change is good, some not.  I'm not getting into the world today, it would fill a book and who wants to read that!
   The title to this post is called celebrate the little things.  How do you celebrate the little things with your children?  Each child has something different to celebrate.  An "A" in science, yellow belt in karate, hearing your toddler say mom for the first time.  I had to wait four years to hear Declan say "mom", and it is still the sweetest sound.  I try really hard to celebrate the small things with D&K, to make sure they know I love them.  It is easy to get over shadowed with all of Declan's problems.  So some days the only thing that I am able to say to them are "I love you".  Everyday, day or night, I hope it's a habit we never get out of.  With Declan most things seem little, but are big for us.  Drinking out of a straw, and now he has moved onto drinking out of a cup, you have no idea how much work went into that for Declan.  Navigating through a store without hitting one thing... or person.... seems small, but it was a big deal for us.  How healthy he has been.  We celebrate what we can, and never give up.
   It feels like I'm painting this perfect picture of our family, and trust me when I say we are not.  We fight, we argue, we are lazy.  We have stepped it up for Declan, but he is actually easy to take care of.  Just different.  When I lay Declan down for a nap, he can't crawl out of bed time and time again.  Declan doesn't want to eat dinner? Okay that is fine, I just put it in his G tube.  He still has his four year old qualities, tantrums and all, it's just different.  We are not perfect, I'm not perfect.  We just don't quit.

Sorry this is short I'm trying to get back into the habit of blogging. Pictures below:

Owen Grady riding a raptor 2015

Yep we broke a leg... how does that happen?

you'll be cool but never as cool as me.

2016 a knight riding his horse.

companion animal Jack. 

I am batman! 

j/k I'm a fireman.