Isla had her check up at Children’s this week… and it went really well! Our Doctor said her heart looked NORMAL on the Echo and EKG… yes, you read that right- NORMAL! It was music to our ears! She seems to be getting better and better each time we go. Because we don’t know why she has PH and we can’t tell what the pressures are without doing a heart cath, the plan is to leave her on the meds for now. We will go back again in 3 months and if everything looks good at that time, then we will start decreasing the SubQ meds. Our Doctor thinks it would be best to keep her on the pump through the winter, just to be safe. So, the hope if to have her off the pump by March and then she will continue her oral meds for “a while”. One scary thing is we will have to do another heart cath within 2 weeks of getting her off the pump, which will require her going under anesthesia. We are dreading that, but know it needs to be done. It’s just hard to think about because of what happened last time, but she will be older and stronger by then, so we just have to focus on that.
Isla is still on night time oxygen, so they are going to do a sleep study on her for two nights. One night with oxygen and one night without, to determine if she can come off. We will do that after our beach vacation, so we have rented a concentrator to bring with us on the plane and to have while we are away. That should be an adventure!
We are definitely heading in the right direction, slowly but surely. I leave you with some pictures of Isla waiting for the Doctor and a picture from last weekend of Isla and her adorable cousins!
Isla has been on SubQ for 6 months! I’ve been meaning to post pictures of what the process involves, so here you go! You can click on the pictures and scroll through. It’s interesting to think that this is our normal. I see pictures of little ones swimming or running around naked and I definitely get jealous. Isla can’t do that because of her pump. I hope she can one day, preferably before an age where it’s not cute anymore 😉
These are the supplies we need for every pump change which we do every 3 days.
We unhook the pump so we are just working with the site. Getting the site in is a whole other process! This is a picture of a site in her arm. This one has lasted 3 weeks so far. The longest one has lasted is 9 weeks and the shortest was a couple of days.
Here we just clipped the tubing to her site. The tubing attaches to her pump on the other end which delivers medication to the site and into her skin.
We wrap the tubing around the site once before taping it down so we have a buffer in case the pump falls and tugs. The last thing we want is for the site to be ripped out.
We then cover the site/tubing with a 2″ piece of gauze and an adhesive called IV3000.
Here we are pulling the top layer of the IV3000 off.
This is what it looks like after.
We then run the tubing up her arm to her back. We tape it to her back and run it down her onesie to her diaper.
Then we loop the tubing in her diaper to help secure it.
Lastly, we clip the pump to her pants… so she is always wearing pants!
I have to say, Isla is really amazing when we do her pump changes. It’s like she knows we are doing something serious and she let’s us do our thing. Love our little PHighter!
It’s amazing how hard it is to get anything done when you have a little one. I’ve been meaning to update since Isla’s last PH Doctor appointment which was March 14… so here we are, almost a month later 🙂
The actual appointment was tough because we had to move her site again while we were there. The one in her arm had dislodged and started bleeding, so we moved it to her leg. That was the third site in a two week period, so it was not ideal. Her leg got very swollen and she was in pain for a few days, but everything went back to normal and so far the leg site has “gone to sleep” as they say. Really the only sucky thing (well not really the only thing) is that we have to give her a sponge bath because we can’t submerge her in water 😦
Here you can see how swollen her poor leg was-
Isla had an EKG and another Echo at her appointment. It was her first normal EKG. Woo-Hoo! The Echo looked the same as it did in February. Not better, not worse. Her Doctor said that normally he would increase her meds because she is growing, but he did not increase them. His hope is that she will keep growing and she will continue to improve and it will get to a point where she outgrows the meds. His goal is to get her off of the SubQ Remodulin, but she would remain on the oral meds for “a while”. We will take it! If we can get her off the pump, it would be absolutely life changing! Oral meds are NOTHING!
I keep trying to figure out why this is happening to Isla. There must be some sort of reason, right? I just have to remind myself what a good friend told me- “Don’t try to figure out why… just be with your daughter and love her unconditionally!”
It’s hard not knowing what the future will hold. I just hope that Isla’s Doctor has the right hunch and that she will outgrow this. He has to be right! He just has to!!! We will keep chugging along, slow and steady… we will get through this…
The last couple of weeks have been tough for me. I am really trying to stay positive with this whole PH thing, but am struggling! Isla is responding to the meds and is doing well overall, but my mind frequently goes to the worst case scenario. I have connected with some other parents who have children with PH which has been really helpful… but it also is hard because I hear their stories, and they are NOT good! Many of these children are on the transplant list (double heart and lung- only cure!), or are about to be, or already had a transplant. Some parents talk about how their kids have become ‘angels’ because of PH and I hear that and FREAK out! My mind is running round and round in circles and I am hoping that maybe a little vent session here will let me get it out and I can focus on Isla getting better and being healthy… be positive, be positive, be positive… that’s the secret, right?
Last week in particular I had a lot of anger towards this stupid disease. We had to switch Isla’s SubQ site… it had been 6 weeks and it was starting to go bad. It had been in her left arm, so we put the new site in her right arm knowing that within 24 hours she would be in pain and stop using her arm for at least a day. Well, within 12 hours she started to show signs of pain and within 24 hours, she was in excruciating pain and her whole right arm was extremely swollen. She had a bad reaction to the site, so we had to place another site back in her poor left arm and switch it over. Within 24 hours she stopped using her left arm and was clearly in pain but she started to use her right arm again. Luckily, the site pain in her left arm lasted for just about 24 hours and she was back to her old self by the weekend. Needless to say, it was a ROUGH week! When we lay a new site, we have to give Isla additional medication- histamine blockers and also motrin and tylenol for pain. Here is a picture of JUST her morning meds:
It sucks to have to give her all of that. It sucks to see her in pain. It sucks living with the reality that she is sick. It sucks that she has to wear that stupid oxygen around her face. It all just plain sucks…
Okay- but here is what I HAVE to focus on… Isla is amazing. She is a PHighter and she is going to beat this damn disease. She is getting better and, minus the site changes/pain, she is a happy little girl. Matt is the most amazing and supportive husband and father… he takes such amazing care of our daughter, it’s just unbelievable. He is my rock, my support, my love. Our families and friends and co-workers are by our side, ready to help and listen and be there for us any time we need it. We are surrounded by love on all sides and we WILL get through this!
“We can’t direct the wind, but we can adjust the sails. For maximum happiness, peace, and contentment, may we choose a positive attitude.”
Ah, CADD pumps… you may be wondering, what is a CADD pump? Basically, Isla’s IV medicine is delivered through a pump. Her current pump is the CADD legacy. It contains 100ml of diluted medicine and has to be changed out … Continue reading →
Today we had an appointment with the Pulmonary Hypertension team at Children’s. Isla had another echo today. The results were really encouraging. The right side of her heart is smaller and the left side of her heart is going back to it’s normal shape. Our Doctor said that sometimes it takes years to see this type of progress… and sometimes they never see it, so it is very promising. Their aggressive treatment is definitely working! She still needs to remain on all of the meds (4 oral meds and one IV), but it’s possible that it won’t be forever. We are really excited but also want to stay slightly conservative with our reaction. We don’t want to get our hopes up, so we’ll take days like today as a small win and just hope that we continue in this direction. We still just need to focus on one day at a time!
Here’s a selfie from today at our appointment:
Dad having fun with Islabug while we wait for the Doctor:
We really filled the day… worked 8 hours, bought a new car, went home and made dinner, went to ER, got pulled over, went home… phew! Isla has a picc line in her right arm which has been there since the beginning of November. Her IV meds are delivered through the line, but on Tuesday night, we noticed the line was coming out of her arm and was slightly kinked. Here you can kind of see:
So we paged the PH team and they told us to go to Children’s ER to get an X-Ray to see if the line had moved too far out of place. Luckily it was okay, so we were able to go home. Had it moved, we would have been admitted and they would have needed to put in a temporary IV which would have been traumatizing for everyone. It’s going to stay in for another week or so, until we move to a more permanent option.