Saturday June 20, 2015

Please join us this Saturday to remember Evynn. 

3415 Greenwood Rd, Greenwood, ON L0H 1H0, 1-4pm

Children welcome

Please no formal black attire; white or light summer clothing 

(Evynn's favourite colour is deep purple)

Please no funeral flowers.

 A donation information table will be in place.

Parking available at Greenwood Community Centre: 3551 Greenwood Rd. Shuttle provided. 

If further directions or information required please contact Diana McIntosh at 905-621-6357

Remembrance of Evynn this Saturday

Hello Family & Friends,

We are so grateful for all your love and support.  We are planning a Remembrance of Evynn's Life this Saturday June 20, 1-4 pm at our home in Greenwood (north end of Pickering).  Details will be posted her on the blog soon, just wanted to let you all know now.  This will be an open invitation to everyone, as she has touched so many.

Thank you,
Carol

Sorry

Evynn is no longer fighting this fight...

BIG step in the right direction

These are the tubes that connected the ECMO to Evynn's heart.  We literally waved good bye as the beast left the room!  It saved her life last week... This week will be a different story.

ALTHOUGH we rejoice right now, we are STRONGLY warned that the next 12 hours are EXTREMELY critical.  We have been told that if she starts to crash she will not survive going back on ECMO (and this could be then end of her story).  Her heart remains stiff but is overcoming that for right now, and now being off ECMO she has the time her heart needs.  She remains chest open, and as we have seen that will be a major hurdle.  The inflammation of her heart needs to decrease and closing needs to not squish her IVC.  The stent is still in her IVC and her surgeon says she will not tolerate going to the O.R. any time soon, could be months.  Evynn is high risk for fatal infections, possible lungs issues and a whole host of other potential complications.

Today we give thanks to God and the amazing people he used.

Running out of time...

Evynn will be discussed again on Monday at surgical conference. Although she has made some small gains, we are running out of waiting time for her heart to soften enough to keep her blood pressure up.  Tomorrow her surgeon and others who have been caring for her will look for a plan to give her more time to soften that heart. Unfortunately, from our understanding now the risk of losing her personality and only keeping a body alive will increase with further intervention. We will be forced to weigh the risk.

Patience.

Yesterday, the suregons found two major sources of bleeding. One was an old vessel that needed a stitch, the other was in the IVC where the stent had eroded the wall of the vein. They had expected this could happen and that is why her surgeon didn't like the idea of the stent as a long term solution. They had expected it to last for weeks instead of 3 days though. They had fixed it (not sure how or how long it will hold). Since then, her bleeding has gone from over 100ml/hr to under 35ml/hr. She no longer requires blood product transfusions!  Her blood pressure has climbed to a point that they have had to reduce medication. She is now in a place where it is safer to try remove some of the edema (swelling).  They attempted to clamp off the ecmo and test her ability to maintain her blood pressure. She failed. Still because of the stiffening of the heart tissue. The message today was be patient...

Soften Thy Heart...

Yesterday the surgeons were able to locate a few sources of bleeding that they thought they could resolve.  For a few hours the bleeding had decreased by half, but because of the amount of anti-coagulant needed for ecmo she continues to bleed. She is back to needing over 100ml/hr of blood products. Her body only holds 1000ml so her blood is completely being replaced in less then a day or days in a row now. That alone is extremely hard on a body.

They were able to get a ectopic biopsy done without upsetting her heart. Results are not back 100%, so far seem as though there is no rejection.

The echo showed that her heart squeeze is back to where it was before the arrest which is good.  But the relaxation of the heart (how much it can fill up to then push out) is getting worse. They say her heart needs to 'soften' as it is still too stiff.  If they can get the bleeding under control, the stiff heart will be her biggest issue as the only thing that can help that is time and Evynn herself.

Last night we had the joy of seeing her off of muscle relaxers for an hour. She moved all her limbs, asked for water and nodded answers to questions. Amazing signs that her brain is still healthy!

Today they are investigating the bleeding again hoping to caughterize and resolve bleeding sources.  If they can't then she doesn't have a hope.  They are doing another echo and will clamp off the ecmo to see what her heart can to on it's own. They doubt they can take her off ecmo yet, but wish they could as that would also help the bleeding because of the anti-coagulants.

Praying for a softened heart...

Update... Found more bleeding sources and attempted fixes, so far (only been hour) really good. Heart still stiff.

Biopsy & Bleeding

Evynn remained 'stable'. Her underlying heart rate has gone from 50 yesterday morning to high 90s and is a sinus rhythm (normal). Even though for her that is an appropriate rate, right now it is not enough to keep her blood pressure up, so she remains on the pacer at 130. She has had excessive bleeding and is being infused about 100ml of blood or blood products and hour. At 10am, surgeons will investigating if the sutures for the ECMO are what's causing the bleeding.  They will also test her heart function and do a biopsy to eliminate the possibility of rejection. Doing the biopsy is high risk in her state but necessary. Later today they will also do another 'awake test', by stopping the muscle relaxer.

ECMO.....

Evynn did not have a successful heart catheter, although they did manage to place the stent in her IVC.  She arrested 4 times and had many more complications. The last arrest was over 20 mins but full chest compressions with good cardiac output. Her heart was not beating on it's own. She was put on ECMO extra corporeal membrane oxygenation, portable heart by-pass. The chances of her surviving because she is a 'failing Fontan, post transplant on ECMO' is very slim. No one is sure if she still is in that category or in her own as they have not had to put anyone on ECMO 14 days post transplant. When she was brought back to CCCU her heart was still not beating.  ECMO is keeping blood going the her organs and brain. Withan hour it her heart began beating but at an extremely low rate of 50.  Her heart is being paced at 130. She is being treated for fungal uti, which was the infection that cancelled her surgery and added in an unsecessful procedure.  She remains on many medications. She is being muscle relaxed. They allowed her to off the relaxer for a few hours and she moved her limbs and mouthed 'water'.  Small victory. For now the plan is to wait and rest her heart. It will be a few days before anyone can give us a better sense of survival. Her surgeon has promised if he feels we are in a prolonging death situation he will tell us. For now he says we need to wait. Wait...

Going for heart catheter today...

To help stabilize Evynn the team has decided to do a heart catheter to gather information and to put in a temporary stent while waiting to see if her blood cultures declare infections.

Just to make this more difficult...

This morning we were called at 3am as Evynn was struggling with her blood pressure. She had a low grade fever and her limbs were extremely cold.  The staff struggled to keep her blood pressure up by increasing her ini-trops(meds) and giving her blood volume with albumin. There are a few theories as to why all this has occurred. One is that she just needs the surgery, another is more clotting, or that she may be septic (infection).
She was scheduled to go to O.R. at 8am, but now her surgeon is concerned about the risk of her being septic.  They are currently debating other plans and possibly delaying surgery 10days to 6 weeks.

Back to O.R.

After surgical conference today, the team has decided it is best for Evynn to do surgery tomorrow. As of tonight she is booked for 8am. The plan is the re-route her inferior vana cava (IVC), using a conduit (gortex vein) behind her liver. She will need to be opened right to her pelvic bone so they can remove her liver to access the route planed. Please pray for all the risks she faces again...

Set backs....

Friday, Evynn was able to tolerate a CT scan. In order for Evynn to safely go the CT she had to have a cardiovascular surgeon go with her and hold her chest strings up until the very moment the scanner was on. She had to stay stable for 1minute unsuspended. It was quite a production.
The team was hoping that using a stent in a heart catheter procedure would correct the problem with her IVC. Unfortunately, Evynn's suregon feels the stent would not work as a long term solution. So instead tomorrow Evynn will be discussed at surgical conference and they will decide when she should go back to the O.R. for another open heart surgery. The debate is balancing minimizing the risk of infection by doing it sooner vs giving her body more time to recover from the massive trauma of having a transplant. The CT also showed more clots have formed in her jugular viens, which could explain the swings in blood pressure.
For now Evynn remains chest suspend, vented and fully sedated. Her journey is going to be much longer before she is safe and home.

Still suspended and having issues....

Evynn remains suspended. After another echo, there is concern about the spot where the IVC (vein) from new heart connects to old. It is tight and she needs a CT scan because she will most likely need a stent put in to widen it.  But they couldn't do CT today because she was too unstable to not be suspended. She has also been having massive blood pressure swings from hypotension of to hypertension of 181/110. They have tested her iV lines to see if they are the issue. Could have been medication delivery issue, but all line, pumps, syringes are good. No one really understands what is causing the fluctuations. It's going to be a long night.

Chest closed but suspended.... (picture warning)

Evynn was able to have her chest closed this morning.  She tolerated it well but later in the evening her blood pressure started dropping too much and they needed to "suspend" her chest.  They literally pull up on her chest by attaching thread to her ribs and that hang from above.  Since then her blood pressure has been stable.  They also found clots in her right groan from a femoral line.  Her pain management medication continues to be increased as she is becoming tolerant to the effect.  Evynn doesn't do recovery the easy way.

Retractor out.

Over the past few days Evynn has been weaned off most the blood pressure support meds. She continues on the durectic sand was able to get enough fluid off that yesterday they were able to get the retractor out of her chest. Now she is only partially open.  Her surgeon thinks they will try tomorrow for a full closure. If her blood pressure tolerates a full closure then she will be able to be extubated and we could hold her! They have increase her comfort management as yesterday she was quite aware of all the discomfort. Today she was finally able to sleep restfully. She continues to improve one day at a time.

Bumpy road

Evynn has had a bumpy few days. Chest is still open with retractor. Still on ventilator. Still on ini-tropes and vasopressin, which had been weaned but needed it back after her blood pressure dropped. It has been a tricky push/pull trying to reduce the fluid in her tissue without drying her inside out so much that her pressure suffers. They say her main issue is being vasoplegic (weak/large veins). Yesterday morning she was in a better position and they were going to trying close her chest today but now they are thinking they won't be able to close for at least 5 more days.  She is completely off the muscle relaxer and has been complaining of pain. Her morphine has been increase and they have added other meds to help make her feel comfortable. She has been awake a lot and mouthing the words. Even mouthed 'I can't talk' when we were asking her where she felt pain. Hoping with the increase in comfort meds she will get the proper rest she needs. Over being a 'failing FONTAN' going in, they are happy with where she is now.  It's going to be a long slow road for her.

Stable

Evynn had a quite night. They attempted to wean the vasopressin (vein & artery constriction med) more but with the intoduction of lasix (durectic) her blood pressure dropped a bit too much. The plan for today is to continue with the iV lasix and wean slowly the support meds. They will do a transesphogile echo (scope to picture heart) to get better images of her heart function because the regular echo is unable to get proper images due to her chest being open. She is moving in the right direction, she is just going to take her time recovering.

Baby steps

We haven't been allowed in her room most of the day because there was a procedure with another child. (Rooms turn into O.R.s if they need)
We just got to see her again. They have removed the pacer, weaned one of the meds and her urine out put has increased. Her blood pressure is remaining stable. All good news!

Rough Night

After having a stable day on what we were told was the maximum dose of the heart support medications, Evynn had a scary night.  Her blood pressure & heart rate were dropping and increasing the medication and pushing fluid was not helping.  Surgeons had to open her chest even wider to relieve any pressure on the heart and they put her on the external pacer to increase her heart rate which in turn increases her blood pressure.  She has now stabilized on twice the medication, wider chest and pacer.  She needs to remain stable all day before they will attempt to wean any thing.  If there is any decline, she will require ECMO, which at this point could greatly reduces her chance of success.

THE BEST BIRTHDAY GIFT EVER!!!!!!!!! (includes CCCU picture)

Evynn received the best birthday gift she could ever hope for!  Sunday night she was given a new heart, it first started beating just after midnight on her actual birthday.  We are so gratefully and truly understand the meaning this gift.

She is currently recovering in the CCCU.  Her chest is still open because of the inflammation.  She still requires a long list of medications (Epinephrine, vasopressin, isoproterenol, norepinephrine, milrinone) to stabilizer her.  She is on muscle relaxant and morphine.  She is on the ventilator.  The plan is to just let her rest.  The team did not expect her to recover quickly as her last surgery, the Fontan, makes adjusting to transplant more difficult.  For now she has avoided ECMO (portable bypass machine).  Although the picture looks like her eyes are open, she is not fully awake.  She does have moments of demonstrating that she is aware of touch and my voice.

HAPPY McHAPPY DAY Instead of Home... admitted, discharged & back

Since the discussion of heading home, Evynn's heart failure symptoms increased and she ended up needing to be admitted to SickKids on Thursday night.  The team increased her milronone (iV med).  She responded well and has been discharged back to the Ronald McDonald House Toronto just in time for McHappy Day.  The whole family was asked to represent families staying at the house during breakfast this morning at a local McDonald's with the B.T. crew.
Check out Ev's photo, go out and buy a coffee TODAY!

Home maybe.....

After 13 long weeks of being away from home,  the Transplant Team feel that we could "try" going home.  Evynn is still on the heart transplant wait list.  She is still on iv and oral medications to manage her heart failure.  She still has again decreased feed tolerance (more vomiting) because of too much edema (fluid) in her belly.  But despite all this the team feels that they can predict her better and that we should try be at home before she declines more.
Sunday, we had to visit Emerge because Evynn was vomiting everything, but after some bloodwork and medication adjustments, she was back to the Ronald McDonald House.  Monday, her picc line was also giving us trouble, resulting in another visit to SickKids for TPA.  She could be home as soon as next week as long as she doesn't pull anymore tricks.

Discharged!!! Moved to the Ronald McDonald House Toronto

After 6 rough weeks, with many ups and downs, Evynn finally has been able to leave SickKids.  She is not ready to be too far from the hospital, as she is back almost every day, so we have moved to the Ronald McDonald House Toronto.  Evynn loves being able to be with her siblings when they visit.  She is still in heart failure but her IV medication and diuretics are keeping her as stable as possible.  Now we wait the unpredictable wait and hope she remains stable...

Adjusting to life in Hospital

Evynn has not been discharged yet.  After getting her picc, it seems that the original dose of milrinone is not doing the trick any more.  Over the past week, she has struggled to keep her feeds down.  Vomiting some days as much as 800 ml total.  Her stomach is just not digesting and she has been getting puffier (edema).  Her team decided to increase her diuretics and her milrinone.  They won't be talking about discharging her until she is stable on her medications and increases her feeding tolerances.

Her cardiology team has also decided they need to discuss the option of replacing her tricuspid valve again.  They fear the 2 year wait for a heart transplant may be too long of a wait...

In true Evynn style she is fully enjoying all the attention, and loving the play opportunities that Child Life and her nurses provide.

PICC in!

Evynn had her PICC (peripherally inserted central catheter) inserted this morning.  She was put under a general anesthesia. Every thing went well and she is now recovering. The plan is to discharge her tomorrow but we won't be able to go home until the CCAC nursing managers stop their strike. So instead we will go to the Ronald McDonald House, truly a home away from home as they say.

A Long Over Due Update

So much has happened since the last update.  After weeks of tests & discussions, we decided we preferred to list Evynn for a heart transplant again (3rd time in her life).  She was officially listed in October.  Since then she has had an increase in her heart failure symptoms.  Evynn has been struggling to tolerate her g-tube feed,  she has been vomiting far more often.  The thought is that she has so much fluid in her belly that she isn't digesting her food.  In effort to decrease that edema she was given another diuretic but this caused her become extremely dehydrated.  We ended up at SickKids emerge.  Evynn's heart rate was 54 and her blood pressure was extremely low and she had junctional escape heart rhythm.  The feeling in the room was that they were anticipating her to code at any moment.  They immediately gave her a dose of calcium which instantly raised her heart rate.  She was then admitted to the CCCU, her electrolytes were completely out of whack and her kidneys had quit working.  She received a blood transfusion along with other interventions.  They were able to get her kidney function back and all her electrolytes have almost recovered.  

Evynn has now been moved to the cardiac ward and is receiving a med iv continuous infusion called Milrinone.  Usually, once a child is on this medicine they are unable to be discharged from the hospital.  Occasionally, if a child responds well and stabilizes they can go home and have nursing visits to care for the PICC line (more long term iv, running close to the heart).  She will need to be on the Milrinone 24/7, while she continues to wait for a heart transplant.

On a positive note, her personality is returning...