I am slowly working my way into the positive.  I have been getting calls from people asking me if I am ok b/c the blog is a bit depressing.  I guess people are not used to me sounding down.  So I am going to try to be more upbeat and positive.   Samuel's passing hit me pretty hard as well as many others in the mito community.  I cannot even begin to imagine the family's sadness.    

But today was nice b/c it has been a crazy couple of days.  Both Rick and I have not been feeling well and our schedules have been so busy.  He went to Avery's GI appt. with me today b/c it's difficult going by myself to a late appt. with a respiratory kid being stuck in Boston traffic.  So I am grateful that he was able to take the time out of work. Those are the times that we can take a minute and check in with each other.  Although the poor guy had a rough day at work and had to go to the appt. in his shirt and tie.  He had a change of clothes but the dress shoes and cachi shorts were not working together so he opted for the profesional look.  LOL.

Anyway, we talked about Samuel as he is being laid to rest today.  It just stirs up alot of emotions in both of us.  Here we have Avery who has had soo many issues and we wonder when? and how?... So here we are running late for the appt. and Rick is stressed and I am stressed about his driving and then...You look back at Avery who is pulling one of his Frankenstein type of faces (from the spasms) and we just start laughing.  I know it sounds mean but sometimes you just have to laugh. Because when Avery sees you laughing then he will laugh.  And for anybody who knows Avery and has seen the face that I am talking about then you know you are laughing right now too...

Anyway, I have to say that this was one of the first appts. in a long time that I went in for a specific purpose and got what I wanted. Shocking, actually...  My intention was to get the GI drs. to drop Avery's TPN from 7 days down to 5 days.  Mission accomplished!!! They also dropped his time from 16 hrs a day to 12 hrs.  Woot Woot.  This is awesome in so many ways.  Number one, everyone agrees that Avery is a big fat chunka munk.  While it is cute to see his fat cheeks, triple chin, and cellulite belly, it is NOT good for his health nor mine  He cannot use his diaphragm effectively to breathe with all that chub.  Plus with his already weak muscles it is just a bad combo.  I was glad that they were in total agreement.  The drs. were very happy with how Avery was doing.  It is always an accomplishment for them to be able to reduce TPN.  While it is great for weight gain it can be hard on the liver.  Luckily for Avery, his liver looks great.  He has been on TPN for almost 2 yrs as his primary nutrition and just 2 line infections in 3 yrs of having his central access.  So while he looks like a train wreck in their charts, he is actually doing well in other ways.  
The other positive is that the steroids did the trick.  He sounds sooo much better.  He sounded horrible last wk and this week you would never know anything was wrong respiratory wise.  The great thing about Avery is he responds really well to treatments.  The only thing we will change is the form next time.  For the dose he needed to get it was too much for his gut (volume wise) so he will get it IV next time.  I am hoping it is enough to keep him home over the winter.  He also got his flu shot so homeboy is all set. 

As you can see I am trying to get Avery squared away for before my surgery which is in two weeks.  I know I cannot predict when he gets sick but I can certainly try.  I would just like to be able to recover at home for a bit.  If I am not then I fear I will be in constantly with the same issue.  So while there has been alot of sadness lately, I am grateful that I have my boys here and they are thriving. I am going to try to focus on the positive things because their certainly are many blessing in our lives. If you can continue to pray for the Knight family as they lay their beautiful boy to rest. Thank you

Still.....

Sad, negative, heartbroken, fearful...Just so many emotions.  I know I have never met this little boy but if you are a parent there is no way you can see a video of him singing his ABCs and not cry.  What a beautiful boy Samuel was and he surely have left his mark in this world.  Yet I cannot help but look at my own beautiful boys with tears of sadness that I will one day face the same fate.  There is not a choice, it is what will be.  Moreso with Avery as he has racked up more diagnosis than some of my patients in the nursing homes.  All it takes it one illness and he is gone forever.  I am sorry to sound so sad but it is what is in my heart and it breaks everyday for the loss that will be upon us one day.  I find myself driving in the car and crying my eyes out and going to bed with tears.  This is not me at all but this week it is.  I think after what we went through this summer we feel that we "got lucky" but luck does run out.

You know I went to the Mito symposium over the weekend and it was sad to see so many different people affected my mito.  There were adults in wheelchairs, parents, grandparents and relatives all affected by someone with mito.  I even had a man in his 70s come up to me after my presentation tell me that he lost his wife 10 yrs. ago from the disease.  It can affect anybody of any age and can overtake the whole body.  He told me how his wife suffered for yrs. from mito and she wanted to die b/c the pain and illnesses were too much.  It was dainting to hear from someone that can verbalize it.  So I did feel honored to be able to talk about my beautiful boys and show pictures of their smiles and how brave they are.  I also talked of how we cope as a family.  I think we do a pretty good job, but still there is no getting away from the ache in our hearts. 

You know I am normally a positive person but I am human.  I am not feeling it these days.  I know it will pass but I have so many friends that will be in the Knight's family shoes (some that don't have mito but other genetic disorders) and it is awful.  My heart goes out to all these families.  You are supposed to get married and have kids and they live long lives.  But in many of our situations, the kids live a life of pain and illnesses and are only here a short time.  It's just not fair.  So these days, it is really hard for me to hear people complain about the small things in life.  Maybe next week I will have more tolerance. 

So I take the tears of joy when I can and this was certainly one of those moments.  Isaiah trying out his new ride.

Today has been an extremely hard day for the Mito community.  Another beautiful child has lost their battle to this horrible horrible disease.  A little boy Samuel  who has fought hard with this disease passed unexpectedly.  He had a line infection which quickly took his body.  Please visit the family's site and send prayers to his mom Missy, father Ben and sister Lauren.  They will surely need them during this very difficult time.

This has hit me real hard.  I just can't help thinking about how sadden the family is at this unexpected loss.  It hits so close to home.  You can't help but think that your number is up next.  This is why I freak out at the thought of Avery getting a line infection.  You just never know if their already tired body will be able to fight through it.  It is just so devastating.  No family should have to go through what we all will.  I am especially saddened b/c I have 2 children with this disease who are severe.  I am cherishing every moment with my boys b/c you don't know when it will be taken away. 

As of now, Avery is really struggling respiratory wise and I don't know if he will make it at home over the weekend.  We are surely going to try.  Please make sure to stop by the Knight family's blog.  Thank you.

It has been an overwhelming few days but there is NOTHING like hearing giggles from the boys to take the stress of the days away :)

I feel like I have had the same suitcase packed in my car for the past few weeks.  Avery constantly has you on your toes.  His respiratory status can go from critical to totally clear within a few hrs.  I honestly don't know what is causing him to have so many secretions but when he does he is a total mess.  Last night was one of those times.  I just could not get his sats up no matter how much suctioning and chest pt I did.  Your heart starts racing like crazy.  I mean you have this child's life in your hands and the next move you make can decide a lot.  Luckily, the interventions I tried worked and he was finally able to settle on 3 liters of O2.  Not that 3 liters is good but it is better than when I had him on bipap and he was sitting at 79-82%. 

I often wonder if I am avoiding the inevitable in not going to the hospital.  But then again he can go from near death's door to giggling as if nothing ever happened.  Once he settled in on the O2 last night he had a good night and woke up a bit junky today but had a great day.  Go figure.  Meanwhile I called the dr. that came out two wks ago and he called in a steroids. Don't know if the steroids already kicked in or it's just Avery but he is not on O2 or bipap for the first time in a long time.  So hopefully he can kick whatever is going through and I can finally unpack my suitcase.

During this craziness last night, the Metabolism dr. called me to let me know that he is just preparing the boys presentation for Sat. Mito Symposium.  He wanted to know if I could do a Power Point presentation on the boys.  To which I replied "sure, no problem".  I then get off the phone and say "WTH" did I just do.  I have not done Power Point since my thesis in grad school in over 17 yrs.  I decide to just jump into it and wing it.  Well then Rick comes home and looks at what I have come up with and it surely is not a Power Point presentation.  It looks more like an essay slide show.  Ha ha ha.  So again, he saves the day and helped me with a really nice setup.  I now can say that I am updated on Power Point.  Thanks hun :)

Definitely a little nervous but once I start running my mouth I am sure it will be fine.  I am hoping to keep Avery out of the hospital b.c I have some MAJOR work stuff going on.  I have over 30 hearing aid to dispense in the next few wks. and have to get it done before my surgery.  No wonder why I am stressed!!!!

IT'S MITOCHONDRIAL DISEASE AWARENESS WEEK

It is so important to spread awareness of this disease, the more people know the early kids are diagnosed and funds can be raised to find a cure for this disease.  Isaiah was diagnosed at 5 months of age when very little was known about this disease.  Because of an increased awareness they/we were able to find the mutation by the time Avery came around.  This is huge for our family and especially Janessa.  We can break the cycle.  So please help get the word out so the more children and adults can be diagnosed and treated.  For more information please visit umdf.org,

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I was able to attend the Mito walk with Avery and Janessa on Sunday.  Unfortanately, Isaiah was doing his typical weekend retching so he hung out with dad all day.  It was a beautiful day at Castle Island.  Avery did awesome.  He just loves being outside.  Janessa was a big help too.  She was bummed that we didn't bring Milo as there were so many dogs there.  Honestly, I could picture the scenario of Milo running wild and me chasing him down with Avery's wheelchair while Janessa is screaming.  So I opted to leave him at home, lol. 

While we were at the walk Rick was working hard :) I have to say I am totally impressed. Let's just say the mechanics is not one of his strongest suits. Well he gets a big pat on the back b/c he put together Isaiah's new bike.  Even the physical therapist was shocked and impressed, lol.  Needless to say I was very happy to see it and very impressed with Rick.  Isaiah just needs a different head rest and he will be racing down the street in it. 

 

Today was one of those days that I know I will look back on and say "wow, how did I do it?"........Both boys had appts with the Mito dr.  It is ALOT of work to get both boys packed in a car especially b/c we don't have a handicapped accessible van.  No wonder why I have a hernia?  So I finally get them packed in with suctions, O2 tanks, pulse ox machines, wheelchairs and the kitchen sink, WHEN........

Avery starts vomitting his guts out.  He started turning blue and just looked real bad.  We checked his O2 and it was hanging at 79% (very bad).  Once we got him stable there was no way he was going to tolerate the appt.  So off I go with Isaiah.  It was actually good b/c I was able to get things done in terms of med. changes for his migraines.  We start heading home and low and behold Isaiah starts retching his guts out.

Well this is all perfect timing and the boys should get Oscars for their performances b/c we had a meeting at 2p with Nurse case management.  They work for the state and decided how many nursing hours the boys get.  Avery gets 120 hrs a wk and Isaiah gets 70 hrs.  Because Avery gets the max hrs, the state is really scutinizes it.  Clearly Avery needs the hrs.  He is total nursing care and just ALOT of work.  But you HAVE to prove that there is a nursing need.  Basically, you have to give both arms to prove that your child is justified for the hrs.  Today was BAD with the boys.  Avery was desatting into the 80s and required lots of deep suctioning all the while Isaiah is near him retching his guts out and needing suctioning. It was really pathetic.  My poor nurse worked her tail off.  And still, they could cut the boys hrs.  It is absolutely crazy that people that have no clue about our lives will sit their and make a huge decision and it has such a great affect on our lives.  Heck I don't even think they will give my extra hrs. for the week of my surgery.  Maybe if I dropped the boys off at the hospital for that wk and sent them the bill they would change their minds.  Seriously!!!!! This is all thanks to Obama care and it will only get worse.

So far now I sit with my glass of wine and hope and pray that Avery is not brewing something.  He has been off for sometime and today was the worst.  This is all the nature of the Mito beast.  So if you could spare some prayers that the boys keep their nursing hrs. and Avery stays well I would appreciate it.

Tomorrow kicks off the start of Mitochondrial Disease Awareness Week Sept 19th-25th.  Many children and adults suffer from this disease daily.  This month alone we have lost 2 beautiful young children from this horrible disease.  There is no treatment or cure but through awareness and fundraising researchers are able to find new mutations and medications.  For more information visit www.umdf.org We are attending the MitoWalk in Boston tomorrow.  I can't wait to meet other families and some that I already know.  It's going to be a beautiful day out too :)


In other news, I did see the surgeon yesterday and I do have a hernia.  Ugh!!!! Surgery is scheduled for next month.  I want to get it out of the way before things get crazy with basketball session.  It just really stinks that I have to go through this again and now it will be worse b/c I will have lifting restrictions.  I got off easy the last time but not so much this time around.  I am hoping that I will be able to recover at home rather than in the hospital with Avery. 

Avery is hanging in there.  Thanks to home bipap, he has been doing well respiratory wise.  He is using it both for naps and bedtime and I can really see a difference with him.  He saw immunology and I think they just suck as a whole.  It was a big waste of my time.  I knew they were going to up his dose of IVIG due to his low numbers.  Of course they did and it was supposed to be at my house for today but it was not.  So I had to give him the old dose.  I have never met a department with such poor follow through on things.  I also let them know we never got the order for bloodwork, although they claimed it was sent it.  Whatever... I really wish they would get their act together.  Grrrrr.   I am just hoping we can get his levels up enough so that he has more protection from viruses that could really make him sick.  They also put him a request for him to get the monthly RSV shot.  I doubt number one that they will follow through with that and number two that Avery will be approved.  It is very costly and reserved only for NICU babies and other specific diagnoses.  But it is worth a shot.  I won't hold my breath. 

My hope in the next few wks it to get caught up with things for work and keep Avery as healthy as possible.  I don't know if it will take me longer to kick back from this procedure.  Although knowing myself, I will be back before you know it. 

Since I last posted things have been very busy around here.  Avery has been suffering from respiratory issues for over a wk now.  At times he is requiring 4liters of O2 and desatting over his bipap.  He is requiring lots and lots of suctioning and chest pt.  If any time goes by without any of the intervention then it quickly settles into his lower lobes of his lungs.  I am nervous especially with his Igg levels being low. There are also alot of sick people lately with the flu, UGH.  I have had my bags packed since the weekend just in case but am hoping not to need them at all.  I would like to take longer than a month off from the palace.  I am finally catching up on alot of work and I don't want to break that grove.  Thankfully I have had alot of nursing b/c he was definitely be in the hospital if it were not for them.  The thing that would send him over the edge would be a fever.  The other thing is PACT is trying to wean down his methadone which is NOT working.  I think he is fine where he was and I don't want to mess with things if they were working. As it is he is not falling asleep until at least 10p and yet he is so exhausted.  I know he is at a high doses but if that is what he needs then leave him be.  So he certainly has alot going on but one thing at a time.


Isaiah is still not himself these days.  He is back to having retching cycles every weekend.  It just really really sucks.  It can be so crazy around here when Avery is respiratory and Isaiah is retching.  I truely feel like it is a hospital ward on days like that.  I am hoping things will settle down with Isaiah but we just cannot seem to find the magic potion to fix these migraines/cycles.

As for me, I have been back to the gym on a regular bases since I was not able to do it all summer.  Been feeling great, UNTIL.....It looks like I have an alien head sticking out of my stomach.  Honestly, lol.  I am not sure if it's truely a hernia this time or scar tissue that has decided to hang off my abdominial wall.  But in any case it just does not look good at all.  People are noticing it.  I have an appt. with the surgeon on frid.  I am hoping and praying it does not require another surgery.  Maybe I can get away with just wearing a girdle, haha.  No seriously.  Not to mention the post traumatic from the last surgery where Avery ended up being intubated just days after.  So let's see what she will come up with.  I like the surgeon alot b/c she really gets my situation and will do what is best for me and my crazy lifestyle. 

I will keep you all posted.  In the meantime, please continue to pray for Eithene.  She is a strong little girl who has been through alot.  She is slowly improving but will continue to need prayers along the way.  Thanks.

A sigh of Relief

I am so glad I took matters into my own hands and made the 4:30 appt. with hematology.  Firstly, I wanted to thank you so much Joanne b/c I could not have gone without you.  With Avery being so junky these days you cannot travel alone anymore b/c he constantly needs suctioning.  It would be unsafe for me to travel myself with him b/c I never know what his sats are going to be so thanks Joanne for all you do for us we love you. 

Now backing up to the day before, I had drawn labs on Avery to check his blood counts and Igg levels (which tells us how his immune system is).  Well we needed an order from immunology to process the labs.  Guess what??? They never sent them (no big surprise there).  Honestly, I dropped off the labs and they still ran them, I'm thinking that they went off the previous dr. from last wk.  Who knows, as long as they were done.  Just goes to show you what slackers that department is.  Anyway, the results came in just minutes before leaving for the appt.  His platelets were the same (low 112) but more concerning was his Igg (532) and the range is 590-1700.  In order for Avery to fight infections he really should be at least 700-800 which previously he was at 900.  This is why he was out of the hospital so much last yr b/c he was hanging around 900.  So now I am worried b/c  I still have lower platelets but it sounded like Avery really needs the IVIG and a higher does.  Eeek....

I was impressed that the hematologist was well prepared for us will with all his graphs on Avery's blood counts.  He actually heard me out and agreed with my concerns.  Finally...  It quickly put my mind at ease.   While he was unsure why Avery's counts are lower with IVIG he feels it is still ok.  He actually has kids walking around with counts of 23-10 and they are ok (wow).  Although I did express my concerns about how quickly he dropped the last time and it put him in DIC.  He still did not know why that happened and all the circumstances around it but felt Avery would be better served continuing the infusions.  The crazy thing is they give IVIG to pts. with low platelets to increase them and yet Avery is doing the opposite.  He was pretty stumped on that one.  But we know Avery, never the norm.  So I felt soooo much better and it was well worth sitting in 5pm Boston traffic.  I just needed someone to hear my concerns and give an honest opinion and I am good to go.  I will be giving him the infusion every 7 days and not looking at bloodwork anymore.  He really needs the immunities especially with how junky he is lately.  His risk of infection is so high right now and I would to do everything I can to keep him healthy.

Also the pulmonologist and NP stopped by yesterday.  I had a real open talk with them about a trach for Avery and the pros and cons.  Still no real decision will be made yet b/c it is so complicated.  They did express that if Avery only had his respiratory issues then it would be much easier to trach him.  Where he has so many other issues going on that would be just helping one system.  Also it will only makes some things easier but will not prevent him from getting the pneumonias.   We are very aggressive at home with his respiratory stuff and as a whole do not feel that the trach would greatly improved what we are doing.  So the jury is still out on that one.  But it was great b/c if Avery does get sick at home the team will be able to prescribe home antibiotics and adjust bipap settings.  So hopefully it is another step to keeping him at home more.

Isaiah though is having ANOTHER migrane.  Poor kid is starting up every friday and lasting until Sund. or Mond.  I feel so bad for him and just wish we could fix this one thing for him. 

I am definetly overwhelmed medically but feel at peace with things now.  Hopefully things are quiet for a bit. 

Our little friend Eithene fromthebanksofjordan.blogspot.com really needs your prayers right now.  Please visit her site and send some prayers to her.  Thanks

Round and Round We Go....

Here we are again, just two days before Avery's next IVIG infusion and still no direction.  So I had to take matters in my own hands of course which is beyond frustrating.  It truely amazes me that we are at the best hospital in the country and possibly the world and yet I am still struggling to get some direction from medical professionals.  I feel with a kid like Avery who is super complicated he is fixed up a when he is inpatient but you are on your own when you are home.  It is me making all the phone calls to different drs. and trying to get ordesr for blood work.  It's something else when I am starting to know which labs need which color test tubes.  Crazy huh! But necessary.

I put a call into immunology again with my concerns that Avery's platelets are on the lower end (111).  While this is not terribly low it is lower after 6 days of having his infusion and getting another one the next day.  See, when he went into the hospital the last time his platelets were 120s 3 days after his infusion and by day 6 he was down to 23 and bleeding out.  So you see my concerns!!!!! Despite what anyone wants to believe from their medical books it just doesn't fit my kid.  I have such post traumatic issues from this last hospitalization and if I can prevent it from happening again then I will.  But what I got from the dr. today was 111 is not bad especially if he is not bleeding anywhere.  For which I reminded him that Avery constantly bleeds from in and around his g tube.  Well no answer to that one right?  I took it upon myself to get an appt. with Hematology on frid. (although it sucks b/c the appt. is 4:30 pm on a friday afternoon).  I am sure I will be kicking myself on the way home, especially if I don't come out with some sort of plan.  I am just hoping that can give us some type of parameters for continuing with the infusions.  At the end of the day, I need to do what is best for Avery.  If these infusions are hurting him more than helping then it is NOT worth it in my book. But drs. see it completely different. 

As you can see from my last few posts I am beyond frustrated.  I am scared for my little boy.  There is so much pressure to keep giving him these infusions from the medical community but for me all I see is what it has done to him.  While I know it did great for him prior to all of this for me it is not worth the stress of constantly worrying about his labs.  The other thing is he looks like he is retaining fluids in his face. I don't know that this means for him.  Although his labs look decent. Others that have not seen him in awhile are asking if he is on steroids which can give that appearance.  I am so used to seeing him that I don't notice it only that he is SUPER heavy now.  So I have made another appt. to see if we can go down on his calories b.c it cannot be good for his respiratory issues which have been horrendous lately. Not to mention he won't fall asleep to after 9-10pm which is amazing with all the meds he gets.  UGH!!!!

Poor Isaiah had the absolutely worse migrane I have seen over the weekend & through Monday.  He slept almost the entire time.  I hope it is not getting worse, I just cannot even imagine it for him.  Especially since his bike just came in.   

Not to mention our "cute" little dog who I am ready to sell.  He is so much worse than a baby.  I know it will pass b/c he is only a pup and overall he is a good dog.  BUT!!!!!!!! TOTALLY not helping things right now.

This has totally turned into a complete *itch session but that is what this blog is for right?   I am obviously in a funk but it will pass. Although I did forget to mention last posting that Rick totally made my birthday on Friday.  He spoiled me to death and I love him for it.  We are also lucky to have each other as well as good friends and family.  It just feels like my life has caught up with me a bit these days is all. 

Well, I guess I am done for now.  I promise to be more positive in the next post, just not feeling it today. 

I know it has been awhile since I have last posted.  I just really needed a break from the medical stuff after all that Avery had been through this summer.  Honestly, I feel like I had a hangover from the whole experience.  You find yourself living on the high of how well you kids are doing but then something like that happens and it brings the reality of our lives right back to us.  So I have sort of shut myself down for a bit in order to cope with things.  I had cancelled all drs appts and am only doing the necessary stuff for a bit. 

 There still are many unanswered questions in terms of what to do with Avery's IVIG.  We have continued to do it sub q (through the skin) at home and yet he is still dropping his platelets and gets respiratory and even today he has a fever.  I am frustrated b/c I feel like I am not getting any direction from drs. on what to do.  It truely is a very tough spot to be in.  If Avery does not get the infusions then he is at risk to get sick constantly which could be just as bad but then again I CANNOT go through what we did a few weeks ago. 

In other respects Avery looks amazing.  It's as if nothing ever happened.  He is one hefty boy now.  I think he only weighs a few pounds less than Isaiah right now which is sad b/c they are four yrs. apart.  Isaiah has some exciting things going on with his bike coming in and getting him prepped for a power wheelchair (should be very interesting :)  

Milo is still eating everything in site and driving us bonkers but he is a cutie. 

Below are some pictures of the boys session with the pallative care music therapist.  They both really enjoy it and you can see Isaiah getting into it.  Very cute.

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Avery's loving all the attention as always