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We just couldn’t stay away…

Just when we thought we were done with hospital visits for a while… BAM, Ethan’s port is the size of a golf ball!

We infused Ethan on Friday as scheduled, and everything went absolutely perfect. I hit the port in the first try and immediately got blood return (which is exactly what is supposed to happen).

After that, things were great and nothing happened out of the ordinary over the weekend – at least not that we are aware of.

Ethan and I were getting ready to attend his little friend’s first birthday party on Sunday afternoon. As I started to change his clothes, I noticed his port was swollen. Everything was fine up until Saturday night, and I knew this was not normal. Peter immediately grabbed the phone and called the Janeway to speak to the hematologist on call. She assured us things were okay and that we could go about our normal plans for the day. She just wanted us to touch base with her later that evening if things got any worst; otherwise she would talk to us the next morning.

So, off we went. Ethan had a blast at the birthday party and everything went great. He had so much fun he slept for almost two hours when we got home!

That evening as I was getting Ethan ready to have his bath, I noticed the area around his port had swollen even more! This time it was more than double the size… Maybe even triple. It was easily the size of a golf ball. The skin was so tight that the scar from his surgery was starting to stretch. I panicked at the sight and immediately called the Janeway again!

I have to say, I have been really laid back and calm about everything we have been through so far, but this time I was legitimately worried. I felt sick to my stomach just looking at it. Oh dear! I was ready to scoop Ethan out of the tub then and there and take him directly to the hospital.

Thankfully, the hematologist assured us that things were fine and that there was no need for us to rush in with him. Aside from the noticeable swelling, there were no other signs of a bleed. He was still his normal, happy self. She advised us to keep an eye on it and to make a visit to Medical Day Care in the morning whenever we were up and straightened away. She wanted to see Ethan not so much because she was concerned with the swelling, but because she knew we wouldn’t be comfortable accessing his port to infuse him.

This morning when we woke, although Ethan’s port was still very swollen it had decreased significantly – which was a really good sign!

We made the hour trek over the highway to visit the doctors and nurses at the Medical Day Care unit once again. He really does love those nurses, ha ha.

After they checked Ethan out and assessed him, it was determined that he had a bleed around his port… For no known reason, other than the fact he is a little boy with hemophilia that has a port. He may have bumped it, we may have hit a small blood vessel while accessing his port (where his body isn’t used to it), and it may have just happened on its own.

Thankfully it was nothing too serious.

They decided to give Ethan his Advate while we were there, and rather than giving him the usual dose they doubled it to be safe. Since the swelling was significant, they left the needle in place so they wouldn’t have to re-access the port tomorrow. They just covered it with gauze and Tegaderm to secure the area.

For now Ethan has to go back again tomorrow for another dose of Advate, and again on Thursday for a checkup. Hopefully the swelling will be gone down by Wednesday when we are scheduled to do his next at-home infusion.

The area has, however, started to bruise. The hematologist warned us that it will likely bruise very badly before it fully heals.

Aside from the bleed in his port, the hematologist also wanted to do some bloodwork to make sure Ethan hasn’t developed an inhibitor. While this test was done just before he had his port surgery and came back negative, they want to be certain things haven’t changed since he has been on prophylaxis (and has been receiving factor treatment much more frequently than what his body is used to).

Hopefully this is not the case.

If it is, we will cross that bridge when we get to it. And, if by some chance Ethan has developed an inhibitor (which essentially means his body blocks the Advate from working), there are treatment options available. Ultimately the goal would be to get his body, and immune system, used to the infusion of Advate so that it no longer rejects it. This would be done by infusing Ethan with high doses of factor for long periods.

I will be sure to let you know how tomorrow goes. Fingers crossed for no inhibitor and that the swelling has gone down.

Kerilynn

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Factor Days

This past Friday was a big day for us! It was the first time Peter and I did an ‘at home’ infusion with no nurses or doctors walking us through the steps. We both thought we would be nervous, but we weren’t!

Everything went great, and the fact that Ethan didn’t mind us picking and poking at him certainly helped!

While it takes a little time to wrap our heads around the process of preparing for Ethan’s treatment, we are comfortable infusing him and know that in no time it will be second nature for all of us.

Here is a breakdown of our new morning routine on Mondays, Wednesdays, and Fridays. Excluding the 45-50 minutes Ethan has to have Emla cream on his chest, it takes roughly 20 minutes or so from start to finish.

When Ethan woke Friday morning, Peter put some Emla cream on his chest (over the port) and covered it with a Tegaderm patch. In case you’re wondering Emla is a local anaesthetic cream used to numb the skin, and Tegaderm is a transparent dressing that is used to keep the cream in place. The cream has to be left on Ethan’s skin for at least 45 minutes to ensure it will be fully effective.

Once that was done, we went about our usual morning activities – Changed Ethan, gave him a bottle, and played for a little bit.

While Ethan and Peter were playing, I went about preparing the supplies and getting the area ready for Ethan’s first treatment. Since it is recommended to keep everything above waist level to ensure sterility, we decided to infuse Ethan on our bed. We purchased a fold-out table to keep in our room to put our sterile field and supplies on to have next to us.

We have a huge storage bin with drawers that is full of supplies. To make sure nothing gets overlooked I organized supplies into large Ziploc bags. Each bag contains all of the supplies we need for one infusion, so when we are ready for Ethan’s treatment all we have to do is grab the bag and begin the process (rather than having to scan through the list of supplies to ensure we remembered everything). This really cuts down on preparation time!

For each infusion we need:

  • Emla cream
  • Tegaderm patch
  • Advate (factor product)
  • Heparin Lok solution
  • Adapter for Heparin bottle
  • Sterile field
  • Sterile gloves
  • Port-a-cath needle
  • 2 small empty syringes
  • 2 pre-filled saline syringes
  • 5 chlorhexidine swabs
  • 2”x2” gauze

With such a large list of supplies, it is obvious why I decided to pre-package them into Ziploc bags to make sure we don’t forget anything!

I will attach a photo of our storage container full of supplies, as well as a photo of all the supplies needed for one infusion so you can get an idea of how much supplies we actually have on hand.

Once all the supplies have been gathered, the next step is to wash our hands. This has to be done very thoroughly; all over the hands, fingers, and arms (up to the elbows). When our hands are washed we dry them with paper towel and then use the paper towel to turn off the faucet. That keeps our hands nice and clean.

When that is done, one of us can proceed to lay out the supplies.

On Friday we decided that I would infuse Ethan and Peter would hold him still. We both agreed it would be in our best interest to have Peter hold him just in case he decided to squirm and wiggle where he isn’t quite used to it. When he becomes more accustomed to the process, we will take turns infusing him.

While I prepared the tray with supplies, Peter removed the Tegaderm from Ethan’s chest and used a clean cloth to wipe away the Emla cream.

Before I open my sterile field and lay everything out, the first step is to mix the Advate. I open the bottles, clean the top with a chlorhexidine swab, and continue to mix it as directed. After that I wash my hands again and begin to prepare my sterile field.

When opening my sterile field I have to be very cautious that I don’t touch any of the inside edges. If I do, I will contaminate the field and have to start over. Once the field is unfolded, I open all of the supplies. I carefully tear open each package without touching the inside of the package or the item itself. Once the package is open, I turn over the item and let it fall on the field – again, to avoid contamination.

Once all items are laid on my field, I put on my sterile gloves. Even putting the gloves on is a tedious process as I am not allowed to touch the outside of the glove.

When my sterile gloves are on, I can now access the supplies on my sterile field. The next step is to prime my two saline syringes, prime my needle (with saline), and then draw up the Advate and Heparin in the two empty syringes. This part requires Peter’s help. Since the Advate and Heparin bottles are not sterile, I am not allowed to touch them with my gloves. Peter holds them for me while I wipe the cap with a chlorhexidine swab and draw up the solutions in the small syringes. I need 2ml of Advate and 3ml of Heparin. When all of my syringes are ready and primed, I double check for air bubbles and tap my syringe to remove any that are visible.

I can now take my primed needle (with attached saline solution) and access Ethan’s port. Once the needle is inserted into the port, the first thing I do is check for blood return. Once blood return is visible I continue to flush with 10ml of saline solution, clamp the tubing, and remove the saline solution.

I then top up tubing with saline, attach the Advate syringe, unclamp, and begin to give factor. It is recommended that patients be given 4ml over two minutes, so we time Ethan’s infusion of 2ml over one minute.

When the Advate syringe is empty I clamp the tubing, remove the syringe, top up the tubing with saline, and then add the second saline syringe for flushing. I clamp the tube when the flush is complete and attach the Heparin Lok solution for the last flush. Heparin ensures there is no buildup (or clotting) in the line of the port while it is not in use. When the Heparin flush is complete, I clamp the tube line again. I keep my thumb on the plunger of the syringe while clamping the tube to ensure there is a positive pressure.

When the last flush is done and the line is clamped, the needle can be removed. The gripper plus needle we use has a small handle to pull up on to ensure the needle is safely inside a guard when ready to be disposed. Finally, we wipe the area with a small gauze if there is a small amount of blood.

And we’re done!

During the entire process, I am checking Ethan for signs of a reaction: his breathing, signs of redness at the site, appearance of hives, etc.

Easy peasy lemon squeezy? Ha ha.

The hardest part is ensuring that everything is kept sterile to avoid contamination. Since we are using the port to openly access Ethan’s vein, we need to make sure there are no potential risks of infection. The last thing we need is a sick baby. If we ever doubt that we may have touched something on the sterile field or that we have contaminated something, we dispose of the item(s) and get new ones – it’s better to be safe than sorry!

It actually isn’t that bad. Although Friday was only my second time infusing Ethan, we had tons of training. And it will get easier as times goes on.

And that fact that we have to poke Ethan with a needle three times a week? Well, we just put that aside knowing that what we are doing will help Ethan stay safe as well as give him a much better quality of life.

Kerilynn

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At-Home Infusions

Since Ethan had his port surgery last Tuesday, he was stated on prophylaxis. As mentioned in one of my previous posts, this means Ethan will receive regular infusions of Advate three times per week. The main purpose of this method of treatment is to prevent minor bleeding episodes from occurring. Peter and I have access to Ethan’s port and have been trained to infuse him with factor at home.

Home infusions have many benefits. Not only does it make it possible for us to give Ethan quick treatment at the first sign of a bleed, it also allows us to more easily manage prophylaxis as well as help alleviate frequent visits to the hospital whenever he gets a bump or bruise. If Ethan were to injure himself, rather than travelling to the hospital at the first sign of a minor bleed, we may decide to touch base with the hematologist on call and allow them to make judgment based on talking to us over the phone. If they feel it is not an emergency, they may just recommend us to give Ethan an extra dose of Advate to help with the bleed and see how things go from there.

Frequently reoccurring bleeds can cause long-term damage to Ethan’s joints and muscles if they are not properly treated. Home infusions will allow Peter and I to treat a bleed at the earliest possible opportunity to ensure a quick recovery. It will also help us (and Ethan) to be more independent, and will reduce disruption in our daily activities.

Ethan will quickly get used to us infusing him three times a week. Since we started treating Ethan so young, it’s all he will know. As he gets older and has his port removed, he will continue with prophylaxis but will learn to self-infuse by the means of using a butterfly needle.

For now we will infuse Ethan three times a week. He will receive his factor treatment Monday, Wednesday, and Friday morning before we begin our day. Since Ethan is most active during the day, it makes sense to infuse him in the morning just as he wakes up. That way his levels will be the highest during the day. If we were to infuse Ethan before bed, for example, he wouldn’t reap the full benefits of the increase in his factor levels. He would be sleeping while they were at their peak levels, and by the time he woke they would have significantly decreased.

Although Ethan has only just started prophylaxis treatment, Peter and I have already become more comfortable and confident in caring for him, allowing us to feel a greater sense of control over his safety and well-being. Ethan’s most common bumps and bruises will diminish and he can have more freedom to explore because he factor levels won’t be as low.

As Ethan grows up, he will be able to more easily participate in ‘normal’ activities. While he is currently receiving Advate therapy three times a week, this can be tweaked to suit his needs depending on his activity level. If, by chance, Ethan becomes an avid athlete (for example), the hematologist may choose for him to be treated more frequently. This is something that will be accessed and decided by the hemophilia team as they feel necessary.

Kerilynn

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We are home… And we couldn’t be happier!

We are home… And we couldn’t be happier!

As I sit to write this entry, I stare around at the bags that have to be unpacked and the copious amounts of stuff that has to be put away… But, I know it can wait until tomorrow. After a long, tiresome eight days I need to take some time to myself – time to relax, and time to look back on what we have been through this past week.

The last eight days have seemed like forever, yet I can’t believe Ethan’s port surgery is DONE. It still seems so surreal. It feels like we have been waiting forever for him to have his surgery and now he is home, recovering, and doing amazingly well!

If there is any such thing as having a good experience in hospital, we did.

I have to (once again) commend the staff at the Janeway, especially the nurses on J4 Surgery. They were absolutely incredible and made our eight night stay as enjoyable and as comfortable as possible.

The hemophilia team is amazing as well: The three hematologists that we frequently meet with along with the hemophilia nurses. They have become a second family to us over the last nine months, and more specifically over the last eight days. We have met with them and talked to them every day and they have been there to support us every step of the way!

The doctors at the Janeway surprised us with a day pass on Valentine’s Day which was so nice! We were so thankful to spend the day off the surgery floor, and Ethan enjoyed his freedom being able to crawl around and play like a normal nine-month-old. We spent the day at the Ronald McDonald house, had some visitors, and even went out for a nice supper. It was great!

The next day, we were lucky enough to get a day/night pass and were able to spend the entire night at the Ronald McDonald house. It was the best! Having a ‘normal’ bed to sleep in, being able to cook and eat ‘normal’ food, and having ‘normal’ people to have a conversation with (as opposed to all the medical chatter that had happened over the last several days). It was a real treat and we thoroughly enjoyed it!

Yesterday we trekked back to the hospital to finish up our port training. It was the start of a new journey for us… The first day we would begin to infuse Ethan with Advate ourselves. I am pleased to say everything went perfect.

I surprised myself with how calm I was about everything; it went extremely well! Ethan was really well behaved and didn’t mind the needle or all the picking and poking at him, which really helped the situation. What a good boy he was (not that we expected any different). Thankfully Peter was standing next to me supporting me though the entire process. When I was done, a big group hug and high fives all around reassured me that I did everything right. I am so grateful to have such an amazingly supportive husband… I’m not sure if I would ever get through all of this if it weren’t for him!

After everything was said and done, I told Peter I needed to have a ‘big cry for myself’ – but this time a happy cry! Peter makes fun of me for sporadically needing to cry now and then when I let my feelings build up for too long. I was so proud of myself; all of a sudden it hit me and I felt a wave of emotions. I did something that many parents would never dream of having to do to their children… I successfully did my first factor infusion (of many) on Ethan.

Yay! What an accomplishment!

I know it will get easier from here on in, and in no time it will be second nature to us.

Of course we needed to celebrate after we were done, and what better way to celebrate than a trip to Menchies for some yummy frozen yogurt. Even Ethan enjoyed the celebratory treat!

Peter will get to do his first factor infusion on Ethan tomorrow. We have our last appointment with the hemophilia team tomorrow morning at 10:00am. They want to make sure both of us are comfortable and confident infusing Ethan before they officially send us home with all of our supplies.

I know Peter will do great as well. And it helps that both of us are here for each other – to talk to and walk each other through the steps. Not that we need reminders of how it is done, but as a support system for one another. And that is what’s important!

Peter and I have always had an amazingly strong, supportive relationship, but this has certainly brought us closer together and made us even stronger.

This is certainly not the end of our journey – if anything it’s just the beginning.

Kerilynn

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Valentine’s Day on J4 Surgery

Ethan has been doing amazingly well since his port surgery – he bounced back to his silly, high-energy self in no time. Now it’s just a challenge for us to keep him occupied all day long in such a small room. He is such a busy boy and loves to play and move around.

Yesterday we gave Ethan a little treat by putting his helmet, knee pads, and mittens on (to avoid all the yucky germs) and let him crawl around the unit. The nurses couldn’t get over the cuteness overload, ha ha! He went absolutely silly, racing around the floor giggling.

The first day post-surgery was a little challenging. They were using the port for his IV and lost it at two different occasions. There was an occlusion in the line, but thankfully when they removed the needle there was no issue with the port itself. Pheww! Everyone was a little worried for a while.

The site is healing nicely and the swelling is starting to go down.

Ethan’s IV dislodged again this morning, which meant the nurses had to access the port and insert a new needle. He was such a good boy having it done, he didn’t even cry. What a trooper he has been!

Aside from that things have been really quiet, which is good!

We are so glad that he has stayed on a good sleeping and feeding schedule, which was one of the things we were worried about. The little boy who is normally very independent and refuses to cuddle has turned into a snuggle monster – but we have been taking advantage and thoroughly enjoying every minute of it! For his naps he has been snuggling down with Daddy and for bedtime he snuggles into Mommy. He is quite the cuddle buddy – We love it!

To no surprise, he has stolen the hearts of ALL the nurses on J4 Surgery. Even when they aren’t working with him, they always come to visit. He is quite the charmer, as you can imagine. He always manages to smile, giggle, or just show off for the nurses when they are around. He really has it figured out!

The nurses and doctors at the Janeway have been nothing short of amazing! They have been so compassionate, kind, and caring; genuinely interested in the care of Ethan and his needs – not just because it’s their job but because they care. They have done everything they possibly can to make our stay here comfortable and enjoyable. We are very thankful for that!

We’re spending Ethan’s first Valentine’s Day in hospital, but since we are all together, we are happy enough. Wouldn’t change it for the world. Plus, he doesn’t seem to mind!

We are hoping to get a pass today or tomorrow so we can go to the Ronald McDonald house for a break and to have some play time… Just waiting for the hematologist to come visit. Fingers crossed.

If that doesn’t work out, hopefully he will be discharged from the floor on Monday and will be able to stay at the Ronald McDonald House for the rest of the week so we can come back and forth daily for his prophylaxis and port training.

Thankfully, there wasn’t much in the way of updates. No news is good news I guess; hopefully it stays that way.

Happy Valentine’s Day everyone, xo!

Kerilynn, Peter, and Ethan

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Ethan’s Port Surgery

I just wanted to write a quick entry to update you on Ethan’s port surgery.

Today has been a very challenging day to say the least!

We started off with Ethan having to fast for his surgery, so his last bottle was 10:00pm last night. He was scheduled for surgery at 10:00am, so we thought all was well. He woke up 7:30am, had a dose of Advate to boost his factor levels, had bloodwork done an hour later, and then headed to the OR waiting area to prep for surgery around 9:30am.

Low and behold when they received Ethan’s bloodwork results, minutes before they were ready to take him in for surgery, his levels weren’t high enough!

Essentially they needed his levels as close to 100% as possible to avoid any potential bleeds. His levels were only at 60%, so that wasn’t satisfactory.

We felt so disappointed. They were postponing his surgery again… and even tossed around the idea of canceling it for a while. My heart sank. We knew this surgery would give Ethan a much better quality of life. Not to mention the fact that this was our second time mentally and emotionally preparing ourselves for him to have this procedure done.

Initially when they received his factor levels they were concerned he may have an inhibitor (which essentially means his body blocks the Advate from working)… Thankfully that wasn’t the case.

So, after fasting since the night before, we trekked back to the surgery floor where we had to wait and wait (and wait) for Ethan to receive another dose of Advate before they would make a third attempt.

So, here we are with a nine-month-old that hasn’t eaten since 10:00pm the night before, and he still has to fast. Not only does he have to fast, but we have to infuse him with more factor and take more blood to retest his levels. Oh dear!

What a trooper he has been. Words cannot describe how well behaved he has been through all of this so far. The nurses couldn’t get over how good he was (I think he may have stolen their hearts, too). He quickly reminded me how resilient babies are when it comes to stuff like this; they are definitely stronger than we give them credit for.

Once they received the results of his bloodwork in the afternoon – his levels finally went up to 97% – they scheduled his surgery for 2:30pm.

So, we managed to get him down for a short nap and keep him occupied for the rest of the afternoon… which ultimately meant us walking, and walking, and walking the floors to keep his mind off how hungry he was.

2:30pm came and went, and still no sign. We watched the clock and waited until around 4:00pm. That’s when they called and said they were ready to take him back down.

Finally, after everything was said and done they took him into the operating room at 4:30pm.

He was such a brave little boy. Off he went in the nurses arms and didn’t mind leaving Mommy and Daddy behind! It definitely made it easier on us.

It was a long wait until we heard from anyone. Two hours later the doctor came up to talk with us and let us know how everything went. Ethan’s surgery was a success! By that time Ethan was back in the recovery room and doing well.

He was back in my arms around 7:30pm. It was like holding him as a newborn all over again – I was so excited to see him! He stayed snuggled in my arms for about an hour or so before he woke (and of course I really enjoyed the extra cuddles). At that time we were able to give him some water and Tylenol, change him into his sleepers, and then get him settled away for a bottle.

Poor little guy, it was the first bottle he had in 22 hours. Boy did he ever enjoy it!

Now he’s sound asleep snuggled into me as I write this entry. We are all so relieved that he has his surgery over with so he can recover and get back to normal.

Now that we have that out of the way, we will begin prophylaxis and start learning to infuse Ethan through his port in the next few days. Something that is new to us, but will be a part of our life from now on.

Kerilynn

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WE ARE READY! Well… As ready as we’ll ever be.

I haven’t posted in a few days. I feel like Ethan’s hemophilia has been pretty ‘agreeable’ lately. As busy as he is, there have been no serious bumps or bruises… Thank goodness!

His knees are still covered in bruises from crawling – even though he wears knee pads on a regular basis. And just a few nights ago he slipped when crawling and bumped his head on the floor. Although he had his helmet on, he still managed to make contact and develop a bruise. Thankfully, it was nothing serious and no factor was needed. Phewww!

I figured I would take a few minutes to myself and write a short entry before I get back to packing.

Ethan just went to bed for the night. Tomorrow is the day we head to the Janeway to be admitted for his port surgery.

Tomorrow he will spend the day on the surgery floor primarily to receive factor to ensure his levels are up nice and high before heading into the operating room the next day. If everything goes as planned he will have his port-a-cath put in place on Tuesday.

As scary as this may be for us as new parents – and while we still worry about him having the procedure done – we know it’s best for Ethan. We have had quite some time to think about this, weigh out all the options, and mentally prepare ourselves for what’s to come.

We recognize the struggles we have had trying to access Ethan’s veins every time he has needed factor. With his chubby little hands and feet, it’s always a fight to find a vein for his treatment. The more he grows the more active he will get, which will likely increase his risk of having a bleed that requires additional treatment.

WE ARE READY! Well… As ready as we’ll ever be.

I am pretty sure I won’t feel ready when Ethan leaves for his surgery Tuesday afternoon. As a mother, I will likely question whether or not we are doing the right thing as we wait for him to return to the recovery room.

I just wish it was over with, really. The hour or so that he will be in surgery will undoubtedly seem like the longest hour ever. And while I know the first few days after his surgery will be challenging, it will get easier.

As I have said before, Peter and I have done days of training for this.

This week, we will do something with Ethan that most nurses only experience once they have received their nursing degree. And even then, some of them still may not be familiar with accessing a port. I feel like over the last few months Peter and I have learned enough to earn our very own medical degrees.

As soon as Ethan is out of surgery and the site starts to heal, we will begin accessing his port for prophylaxis.

Anyway, as I get back to my packing and preparing for tomorrow, I ask that you keep us in your thoughts and prayers. Ethan is a tough little boy, and we know he will be fine. We just pray for a speedy recovery and that things go well enough that we are back home in no time.

Here’s to starting another new chapter in our walk with hemophilia.

Kerilynn

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