Tuesday, July 10, 2018

Never a thought

I'm so very behind on this blog, nothing I can say but sorry and move on.  I've got two teenagers and Declan, I'm a very busy lady.  Fair warning this post may contain some triggers for those with close calls experiences (CPR), or PTSD.  Just know Declan is ok, he is doing great.

       I was talking with someone recently, and she said "I just don't know how you do it, and I'm so proud that you are cause you know you didn't have to. You could have put him in a home or given him away."  Mind blown.  Yes, long time ago that's probably what people did.  Not us, never us. Declan I need you to know it was never a option.  Never a no, not even a thought process to not keep you, and love you.  We will always love you, no matter how many hospital stays we have to do or surgeries or never getting a good night sleep.  You are our baby boy and we will never give you up.
       I don't want to shame others if this is what they had to do, but the world has changed, and I hope they gave it a chance before doing so, because you never know what you are capable of until you are pushed to your limit time and time again.  The world makes you tough, but you gather your strength from your family.  Declan has changed this family for the better, and we will always fight for him.

      Now here is where I'm going to tell you about our close call.  It isn't pretty, it was terrifying.  You can skip this section if you want.

We almost lost him.
Many of you know we recently had a close call with Declan.  He had a respiratory arrest at the home, right in front of me.  911, EMS, Life flight all involved.  It was not pretty, not peaceful, it was so terrifying to me, I still shake when I talk about it.  

January 3, 2018
     Declan was still sick, after being sick since November and spending a week in the hospital, he was better from the flu, but still had a respiratory issue. Still coughing, still gunky, still not our happy boy.  All week I kept telling everyone he needs to go to the hospital he isn't getting better.  We saw his doctors, did the xrays, we were doing his breathing treatments.  Nothing was changing, how horrible is that to be sick for so long.  I was taking him back to the docs having our pedi doc talk to our pulmonary doctor to come up with a plan.  Something different something new because Declan was not getting better.  We had all the equipment at home.  This was his regiment for the breathing treatments.  Nebulizer with Albuterol, suction, cough assist 5 sets of 5 with suction in between, and manual chest percussion when needed (Declan calls it Tapping).  We were doing everything the hospital would do, so my response from everyone was "just keep doing what you are doing."  Declan was just taking a long time to recover, the illnesses are really bad this year, your doing everything the hospital would do.
     K happened to be home sick, fighting a bug, she was quarantined to her room. Normal sort of  day for us, Declan woke up, I started suctioning him, normal stuff.  Then I turned him onto his back after sleeping on his side.  He said "tap, tap" and started gasping for breath, started turning white.  I turned him onto his side and started pounding on his side (probably harder than I needed to but it worked), it must of dislodged something enough for air to get back into the lungs, it was probably only 30 seconds but it felt so much longer.  K came in to check on things I think I yelled for her, I was to rattled to remember.  I was again on the phone with the doctors, scared, worried, feeling everything.  Declan was back to normal (sick normal) talking, sitting up, alert.  Doc said we will check him at his appointment.   Got him dressed and off we were to the docs.  They listened to him, said he sounded fine, a little junk in the left lung, and just keep doing what you are doing.  I get it not wanting us to go to the hospital, RSV, flu are everywhere there, but Declan was not getting better then add this scare, I should have ignored everyone and just taken him then.  That night I had a talk with K, went over if Declan stops breathing again what I need her to do if she is here.  Made sure she knew our address to tell 911, to open the front door, and if things got really bad, go get a neighbor.  I didn't think she would need that plan so soon.  I thought this was one of my worst days, I was wrong.

January 4, 2018  
    It started like every other day.  Woke up at 7:15 drove D to school at 7:30, (K asked for one more day to get better), came home and back in bed by 8:10am.  Hubs went to work at 8:30.  Declan was still sleeping so I went back to sleep as well.  Normal everyday stuff.
10am Declan woke me up, he started calling for me through the monitor.  I rolled out of bed, grabbed my phone and went into Declan's room.  He was on his side, he sounded really gunky so I started suctioning his nose. I got a lot out, I went to move onto his mouth so I turned him onto his back.  He looks at me and starts saying "tap tap". Here we go again I thought. I was pounding on the side of his chest, he turned white, and his face went blank.  I must have been yelling at Declan because K came in. I said something on the lines like nope I can't get him back and told her to call 911.  It was probably seconds, felt like minutes.  I was trying to suction to see if I could get something, while pounding, I even tried the cough assist to try to move something.  Nothing was working.  The 911 dispatcher had me put Declan on the floor and start CPR.  30 and 2, 30 and 2.  Declan released a breath, but it wasn't a normal breath it was like the air was just leaving his lungs automatically.  I don't know how to explain what it was like.  Declan was pure white, the oxi meter was going off, the suction machine was on and loud, the cough assist was on as well.  Declan was not there, his eyes where half closed and lifeless, no response.  I was beyond terrified I was in survival mode.  Fight or flight right, I was there fighting for Declan.  We figured it out later the EMS got there 6 mins after the call.  I think that is pretty damn awesome for response time.  First guy came in calling for us. Later found out his name is Cory (Thank You Cory!!!).  His response was quick, "move", I jumped out of the way he continued chest compression's. We got off the phone with 911 (thank you 911), and told K to call dad, went back to watching Declan.  Hubby said later he got the call from K, she was crying said Declan stopped breathing and they are working on him.  He grab his bag and left work.  I am sure it was a very scary situation for him.  I would never want to get one of those phone calls either.  He tried to call me a couple of times but of course I couldn't answer.  As he hurried home, all he kept repeating is "breathe Declan, just breathe".  Cory EMS was the only one there so far, I asked him if I could suction, yes was his reply.  I grabbed the hose and worked on Declan it was within seconds I got a hold of a long thick yellow mucus plug, it just kept coming I ended up grabbing it with my hand and wiped it on a blanket.  I believe my response was "yuck". Still no change with the way Declan looked, white not responsive. Bam! Everyone else showed up, and I mean everyone, fire department, cops, county sheriffs, detectives. We found out later the EMS team went down a dead end road that is right by us, and of course couldn't get past the barriers, so Cory with all his gear jumped over the barrier and ran to our house while the team turned around. Pretty cool of him.  With everyone showing up I had to get out of the way, they wanted a statement.  I moved Declan's power wheelchair out of the way, and I looked back at Declan before I left the room.  On the floor, lifeless, and Cory the EMS saying to his team, "he has a pulse".   Hope, it gave me hope.  I can't explain it, but as soon as those first responders got there, it's was like taking a deep breath.  I was still very shaken up, and worried, but a little of the stress of trying to save my child's life was lifted.  Declan still wasn't getting a lot of air if any, but he was alive.  It gets confusing at this time because I wasn't in the room and I didn't see what was going on.  I've read the report, but it wasn't any help either.  We believe as soon as I was able to get the mucus plug out, that was the turning point, and Declan just had to have some help to get back to normal. Some oxygen, some stimuli, but I don't know if they suctioned more and got more out, or just gave him breathing support.  I was busy repeating my statement of what happened, answering questions, they really don't give you time to breath.  Just rapid questions one after the other.  Your time is skewed, seconds feel like minutes, minutes feel like seconds.  All the while K was with me, watching, on the verge of crying, shaking.  She really did an awesome job she did exactly what she was suppose to do.  All we could do is wait.  Some random guy would come in every once and awhile and ask a question about Declan, what's his name, what's his normal skin color, is he verbal, is his chest normally like that. Asked which hospital to go to, which of course had to be the closest children's hospital (45 mins away), we learned from previous hospital visits that Declan is too complex for just any hospital, no one else will touch him.  I don't know what they thought when they came into my home, room full of medical equipment, me spouting out medical jargon.  Somewhere in all of this they had called for life flight, they were on their way.  Hubby called, he was almost home, no word yet if Declan was okay. Every once and awhile I would hear Declan cry, it was soft and weak, but it was there.  Sitting and waiting, that's all we could do, asked if they could call anyone.  I know it seems weird, but I didn't want them to call the grandparents, and I didn't want to talk to anyone until I knew Declan was stable.  So I put it off but I did call my sister, it was short, come and get K right now, Declan isn't breathing, they are working on him.  They did tell me my neighbors were outside if we needed them, which I appreciated, again I didn't want to talk to anyone until I knew Declan was going to be okay. One gentleman came up and said life flight is here.  So to me I was a little confused we live in a suburb not tons of room.  I asked where they landed, they said right out front. I was surprised it fit.  At this point I was still in my pj's so I excused myself so I could get some pants on at least.  When I came out hubby was there, trying to see into the room.  I called for him, and he hugged us so hard, and just said I'm sorry over and over.  We stood together, talking, trying to fill him in.  I asked if I could go on the flight with him, and didn't want him going alone, and there was no way I could drive, I was still shaking through all of this.  I don't think I stopped till hours after everything was done.  We finally got the voice saying " mom, dad do you want to come see Declan."  I remember releasing a long breath, and walking into Declan's room.  He was on the floor where I left him, much different picture.  He was awake, with a oxygen mask on, still a little pale, but not like before.  We walked in and he saw us and said "mom, dad" such great words to hear.  We knelt down by him and just talked to him.  Declan was a little dazed and groggy, he would look around and you could see it in his expression "why am I on the floor?"  It made me laugh, "yep your on the floor, you scared me, but your going to be okay."  Things moved quickly after that.  We were getting ready to go.  We made sure to grab Declan's favorite blanket and stuffed animal that he could take with him.  I got on my shoes and coat and one of the flight team members walked me out to get into the helicopter, while dad stayed behind to help load up Declan.  I remember walking out and seeing for the first time all the help that came, and the life flight that landing in front of my neighbor's home.  I believe they were able to land cause my neighbor had not yet planted his tree's in the park strip which gave enough room for the helicopter's blades, I teased him about later to not plant any trees cause it is now my life flight landing zone.  I saw my neighbor's bunched together a house down, don't worry I saw you, I did a half wave to acknowledge you, but I was going for a ride.  Walking to the helicopter was... I don't know the feeling, I believe I was in a state of shock and everything was rolling off.  All I knew was Declan was breathing, it was running through my head, we're good, we're good.  They got me strapped in, let me tell you there is not a lot of room in those things, it's tight.  They left me in there and went to go get Declan.  That's when I pulled out my phone and sent a small text to family, it was short and to the point. "Declan stopped breathing.  Being life flighted to hospital. Breathing again. Still groggy." I sent that at 10:45am  Forty-Five minutes later, that's all it was. I'll never forget those 45 minutes though.  A few minutes later and they were loading Declan into the helicopter, he was calm.  I thought he was going to be crying, but he was just eerily calm.  Then we were off, rising up, and up.  It was a very smooth ride, and the team was great, super nice to me and Declan.  Before we knew it, we were at the hospital, and unloading Declan.  Again I was repeating my story, filling in medical history, it was a fast paced conversation.  Every little beep, every deep breath Declan would take would set me on edge.  At one point it looked like he was turning white again, and I just stopped what I was doing and said to someone, "watch", but Declan recovered quickly and I started to breath again myself.  They brought in Xray, and put in an IV.  At some point dad, and grandma showed up all kind of blurring as to when.  Someone asked me if Declan needed a life saving procedure did we want that.  There was no hesitation, yes, do everything you can.  The staff was working on getting us to the PICU.  Now that things seemed to be calming down, they showed us the xray his left lung was completely collapsed, or filled with junk.  Best guess was that a mucus plug was blocking his right lung and since his left wasn't working it caused a respiratory arrest.  His viral panel showed negative for any illness. It was some kind of respiratory illness, this year has been so bad with the flu, rsv, etc that Declan was just hit with one after the other and couldn't recover on his own.  After they all left I was able to sit down and take a breath.  I asked hubs how I looked? His reply was beautiful, grandma said horrible. lol.  I may have agreed with grandma at the time with how I felt, but my son was alive and if everyone needed to see me without make up and not dressed then it's a small price to pay.

                                                     At the hospital

        We had many concerned visitor's, neighbors, friends, family, even Declan's Pediatrician brought his family and came to visit him.  We spent 15 days in the hospital.  Dad and I took shifts, me the weekdays, dad the weekends, unless I really needed a break and a good night sleep then he would come up and take over.  For the first week, we keep up with the breathing treatments every 4 hours around the clock, one day/night every 2 hours.  Oh and of course either mom and/or dad had to do it, cause Declan won't just let anyone touch him.  We were exhausted, and we weren't seeing improvement.  Using everything, a vest that shakes the chest, cough assist, suction, percussion therapy.  It wasn't working.  We were at wits end, we needed a new plan.  Looking into getting a bronchoscopy to check his airway, it came with a lot of complications.  A lot of "could happens".  It was risky.  They made us go through planning in case of those what ifs, learning about the trach, what our decision would be.  Counseling, a lot of talking.  Declan's life plan, and just in case what we wanted for him if he passed.  I would have to say this hospital stay was one of the most stressful, emotional, exhausting stays we have ever had. 

     Pulmonary came in with one more option before the weekend and if it didn't work we would go ahead with the bronc procedure on Monday.  It was called the Metaneb.  Think of it as inflating a tire. It pushes air into the lung as a pulse.  Sounds like a train, choo choo choo choo.  They are usually used on CF patients, but thought it might work on Declan.  I really do love this machine, it is the only thing where we started to see improvement.  It took the weekend and most of the week, but by the end of the week we were finally coming home.


     Being home was a wonderful feeling, but created other stresses.   Since that horrible day, I was having nightmares, and panic attacks.  I had PTSD.  I believe Declan was suffering it as well.  I prepped the home, just in case it happened again.  Kept the oxygen tanks close by, even got an ambu bag set up under his bed, so it could be easy to grab.  Just in case.  I filled out CHIRP forms, and put those all over the house. 
     The CHIRP program is a free service provided the Utah Emergency Medical Services for Children program within the Utah Department of Health. The goal is to ensure that up-to-date information is easily available for emergency medical services providers if you have to call 9-1-1 to assist your child with special health care needs.   You can go here : CHIRP INFO  to learn more about the program.  
     I watched Declan like a hawk.  Before going to bed, I made sure there was a clear floor and path just in case we had another incident.   We pulled him from school, and signed up for a home/hospital teacher to come to the house twice a week to teach him.  He didn't go back to public school till May.  My rules were simple, You can't be sick if you visit.  If you were around someone sick, don't visit.  Wash your hands before seeing Declan.  My family can attest to me going crazy, I would give anyone an evil eye if someone coughed near Declan. 
      I did find some help with PTSD through groups online, but I think what really helped was writing it all down.   Every feeling, every detail.  I still get anxious, and can go over board with protecting Declan, but can you blame me.  No more nightmares though. 
     Declan was better.  He was back to a healthy little boy, who was happy and not in pain.  It had been so so long since we saw him this well.   We didn't go out, we hardly went anywhere, except doctors.  The flu season was still going around and I couldn't chance him getting sick again.   I just wanted to wrap my family up with a blanket and hold them all.  The older kids were very understanding with having a crazy mom, and just rolled with it.  A supportive husband, that put in hours and hours of work with Declan, and on top of working his normal job as well. 
     Friends helped me clean the house, neighbors brought food, and support.  Everyone was very concerned about our family.  I think we were the talk of the neighborhood for awhile.  We didn't mean to scare anyone, but look at it this way, have you ever seen life flight that close before?  What an experience!  I feel more prepared for if it happens again.  Let's hope not.  I can do this!  I now know I will fight, I won't stop, I won't back down.  I've got this! 
     It's taken me about 6 months to finish this post, I wanted to get it just right.  It's helped me heal, and express what I was feeling.  I don't mean to upset anyone, this was for me, and I wanted to share our story.
Thank You.

Image may contain: sky, cloud, tree, outdoor and nature
Life flight taking off.  Photo by: KFD

No automatic alt text available.
Declan giving a thumbs up in the hospital.
Image may contain: one or more people, people sleeping and people sitting
Declan with therapy dog.


Image may contain: 2 people, including Megan Fink, people smiling, selfie and closeup
Image may contain: 1 person, tree, outdoor and nature
healthy again!!

Friday, March 31, 2017

I caught a Man Cold!!

No seriously I caught a cold this week and it knocked me flat.  Sick all week, exhausted, hurting, coughing, sneezing, nose that would not stop!  I was miserable, and probably a baby, which made my hubby come right out and say it:  "Honey, Dear, You have a Man Cold just chill the hell out...."
(laughing hysterically)

Now lets put things in perspective here.  The last time I got sick was 3 or 4 years ago during Christmas the whole family caught the flu.  That's how many years of me not getting sick, and being healthy for the family?  Yeah, okay so I believe it was time for me to get sick.  I didn't want to, but my body said hey there pretty lady I think you're over due for a cold.  I just didn't read the fine print where it said they are going to give me a special cold... a man cold....

I had a horrible 4 days of sneezing, and coughing, and I hate to say it, but in order to just have my nose stop for 5 flippen minutes I shoved tissues up my nose.  Yep I did, up the nose!  I would walk around the house or just sit there with tissue up my nose.  Of course as soon as I sneezed they came out...

During this time, I slept, laid around, I didn't do a damn thing.  Didn't cook, didn't do laundry, didn't drive my kids to school.  It was great! I slept in, and rested.  I can see why men do this!
Now my poor hubby, he had to put up with me, and take care of the kids. I tried really hard not to touch much and not be around especially Declan since I didn't want anyone to catch it.  But that left it all on hubby to get it done, karate, dinner, Declan's dinner, diapers, homework, cleaning, everything....

Now now I was probably being a baby, I made him check my head, more than once, and listen to my lungs for pneumonia.... maybe more than once....   But I was coughing so hard I think I bruised my lung!

Today I feel much better, some coughing but definitely got my energy back. So no worries I will be back to normal very very soon..... or will I?   Having a week off was really nice. ;p

Below I will post the video my husband showed me for my man cold, it is really funny, but I will have you know he didn't cook me any soup, he only had to make dinners...

Also I wanted to say a big thank you to my husband, he has worked hard, and is a great dad.  We love you very much!

man cold video

Monday, March 6, 2017

what is it like being undiagnosed?

Being Undiagnosed sucks... no easy way to say it.  We can't be apart of clinical trials, treatment or cures for a disease we don't have.  We are similar, but not the same.  SMA (Spinal Muscular Atrophy) just got a treatment, it is extremely expensive, but showing wonderful results. I am ecstatic that SMA families are receiving it.  I doubt we ever will be able to use it for us.  Why? Because the test results came back negative for SMA.  Five years into this new world of special needs and we are still undiagnosed.  There are support groups for us, families just like us. Traveling the unknown future. Not knowing what to expect, not knowing how much time we have.  We find groups with similar symptoms and it helps, it really does, but it isn't the same. We are still on the edge unable to jump in.  We come up with names to try to help the doctors and nurses understand what he has.  "congenital myopathy, muscular dystrophy, something similar to SMA or SMARD, but not really cause the results came back negative..." I have said that to so many people, medical professionals, teachers, friends, family.  God Damn It! All I want is a name! A name to curse, and name to fight for a treatment and a cure!

I couldn't sleep last night so I just started writing.  It began with words and became sentences, which became a story.  Don't fault me it was 2:30 in the morning.  Hopefully it will help you understand a little what undiagnosed families are going through.

too cute.


     Here you have a pen, but you don't know that it is a pen. All you know is it is an object.  You know what it does.  It clicks and writes with different colors of ink, but you don't know what to call it.  You know deep deep down it has a name and all you want to do is to know its name.   

     Here is Sara on the otherwise of the world.  Sara has the same object. Hers clicks and writes with different colors too. But Sara knows what to call this object.  It's called a pen.  She can say to someone "hey I have this pen.  Hey can you hand me my pen..." and everyone knows what she is talking about.  

      Do you know how hard it is to say to some one; hey hand me my thing that clicks and writes with different colors and have them look at you and think you're crazy?    
Now change the pen with a disease name. SMA, SMARD, MS, Cancer...  

My son is one of the many that are undiagnosed.  I know his disease has a name, someone out there already knows the name of it.  I just need to find them so we too can call it a pen.   

     Five years and still no name.  The docs are supposedly getting approval for exome gene sequencing. Its been, I think, two/three years maybe longer.  In the beginning all the doctors wanted to see him, now they are stumped so they move on.  
Well I'm tired of not knowing the name that has hurt my son.  Not knowing what to expect.  Not knowing his future.  Just... not knowing. 

We may never get a name or it may be so rare that my son is the first.  But it doesn't mean we should stop looking. 



for serious....

Thursday, February 2, 2017

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. 


  • 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.


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


Farrell Family

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. 

Saturday, August 9, 2014

August SMA Awareness 2014

SMA Candle lighting 2014

August is SMA Awareness Month 2014

                Spinal Muscular Atrophy it was such a big scary word when we first heard it.  Number ONE genetic killer of infants under two, 1 in 6,000 babies are born with SMA.  Spinal Muscular Atrophy (SMA) refers to a group of inherited diseases of the motor nerves that cause muscle weakness and atrophy (wasting). The motor nerves arise from the spinal cord and control the muscles that are used for activities such as breathing, crawling, walking, head and neck control, and swallowing.   SMA affects muscles throughout the body. In the most common types, weakness in the legs is generally greater than in the arms. Sometimes feeding, swallowing, and respiratory function (e.g., breathing, coughing, and clearing secretions) can be affected. When the muscles used for breathing and coughing are affected and weakened, this can lead to an increased risk for pneumonia and other respiratory infections, as well as breathing difficulty during sleep. The brain’s cognitive functions and the ability to feel objects and pain are not affected.   

Scary right?  Let me tell you what SMA means to this family.
For starters how about determination: We do everything we can to help Declan, we quarantine ourselves during winter, we feed him as many times as we can, we get up all night long to turn him.  We are determined to keep him healthy.   Declan is two (and a half) and is still exceeding the doctors expectations.  I think he is just as stubborn as his parents and no one will tell him how his life is going to be.
Knowledge: Before SMA came into our lives we were blissfully unaware of the struggles of others in the world.  There wasn't much information on the internet and what you did find was all bad.  But with the sadness came newly formed friendships, and help from other struggling families.  I believe the knowing of  " YOU'RE NOT ALONE" helped us more than anything.  Declan is different with his symptoms and his mutated gene.  He doesn't match everyone out there.  But I know if I have a question or I need to rant I can count on these families to understand.  They understand the exhaustion, the fear, the humor and love that comes with SMA.  Now we know how blessed we are.
Humor:  You wouldn't think anything about these kids struggling with SMA would be funny, but holy cow Declan makes me laugh daily.   No he can't run up to me and tickle me, or laugh while saying mommy.  But Declan loves to laugh, and with how dark SMA can be we need to laugh.  Declan has a war cry.  His swords are straws and he always has to have two, but he will raise his hand up and let loose a loud and long war cry "AAAAHHHHHH!" before swinging down that mighty straw.  Or giving his sister kiss after kiss, or saying a word out of the blue, or he starts singing with the music on the radio.  This kid is magical, where ever he goes people smile, and if you're really lucky you will hear him laugh.  It's a beautiful sound and so very dear.  
Patience: I talk about this a lot, and there is a reason for it.  I am "short fuse mommy", I like things done quickly and correctly.  I love my sleep, and love my caffeine even more.   So when Declan came along and I gave up my sleep, and gave up the clean house, and yes even showers got postponed, I was a bit on edge.  Then I would look into these bright blue eyes that have so much love in them.  It's like looking into a soul of an angel so pure, so clean.  I would do anything to make sure those eyes stayed bright.  (don't get me wrong Declan has his devil moments, he has a temper too, and we don't always see eye to eye with one another, usually at 3am in the morning...)  I began to listen, and wait.  I've come to terms that my house will not be magazine worthy, and that my hair will always have that wilted "I haven't been to a salon in years" look, and my eyes will have that tired look to them.  I will tell you what I got in return.  Two years worth of physical therapy and Declan can move his right hand and arm all the way up and even lift his shoulder off the floor.  He is wiggling his toes, and working on kicking his legs.  He is reaching for me!   He has taught us anything can be accomplished with TIME.  We don't have to rush, we just have to experience every painstaking small feat along the way.    
Strength: I'm not just talking about the added arm muscle I am gaining from carrying this boy around.  I am talking about adding strength to my soul.  In the beginning we were pretty shocked, and I know I cried a thousand tears.  With each passing day we became stronger in our core.  Instead of just sitting there waiting for death to claim our little boy, we acted.  We dealt with things we could see and let go of what we couldn't.  Our son doesn't have any muscle, ok then lets start physical therapy, then we added occupational therapy, developmental therapy, and speech therapy.  I learned baby massage, and continued to act.  Declan doesn't need a mom that can't handle hard things, he needs a mom that will fight for him.  I argue with the doctors, and medicaid, and make phone call after phone call to make sure he gets what he needs.  I'm not afraid to stand up for my son.  My son doesn't have the strength to fight for himself yet, so we will.

I'm sure I can go on and on about what SMA means to us, but I will just talk about one more, and it is the most important one.
Love:  Oh my goodness what a powerful word.  My heart just bursts thinking about how much we love this kid with SMA.  I truly believe these kids come down to teach us how to love bigger and better.  We are connected, mind, body, and soul.  When he hurts I hurt, when he is happy I am happy.  Declan and I have a bond, everyone who knows us, knows this.  He is my baby, and I am his mother.  The love just pours out of him.  I hope everyone gets to feel this way someday.  It is magical. 

Spinal Muscular Atrophy... yes it's a horrible, unforgiving disease, but I'm not going to talk about that side today.  Today is a day for hope, and joy, and to remember those that lost the battle, but left behind a lasting impression.  I am terrified of losing Declan, but I can only deal with the now.  Declan is healthy, growing, and laughing.  The end of September he will be going in for back surgery where they will be putting in a Veptr like rod into his back.  We are staying positive and are very interested on how this will effect Declan.  Our hope is he will be able to breath easier, eat more, and sleep without pain.  The doc said he would probably gain about four inches after the surgery.  I will try to keep you posted.
I want to say a huge thank you to our family and friends that put up with our crazy needs.  We know we don't get to see you all that much, but hopefully you understand we still love you guys and we are always willing to help where we can.  
Thanks for reading about Declan, and you can help spread the word about SMA by sharing.  If you feel like donating you can always go to:

Declan in his stander