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!
That pretty much sums up this last year. Having a child is one of the most rewarding, fun, exhausting, challenging, amazing, did I say exhausting? experiences. Everyone says it’s life changing, and people try to prepare you for it… but the only way to truly understand is to go through it. Although I did read a good article with some preparation tips such as- get an orangutan and put it in a car seat and try to keep your eyes on the road, set your alarm every 2-3 hours at night, handcuff one hand behind your back and go about your day, etc. Good pointers for sure 😉 I digress… what I was going to say is- Isla definitely threw a little curveball into the “normal” (is there such a thing?) parenthood experience, but it has certainly taught us a lot and made us all much stronger. I absolutely love being a mom and I absolutely love our little Islabug!
I cannot believe Isla is turning one! We are sooo excited that we can actually light a candle on her cake without the risk of an explosion (no daytime O2) Yay! It’s the little things:) Isla’s birthday is on Wednesday June 25th, but we had a few friends over today to celebrate.
We made a zebra cake for Isla because a zebra is a good representation of PH. Doctors are taught, “if it sounds like hoof beats, think horse”… so for example if someone is having trouble breathing, think asthma or anxiety. PH sounds like so many other common diseases, but it’s not… just like a zebra looks and sounds like a horse, it isn’t a horse!!! And it’s quite rare. Anyway, this looked like a normal cake, didn’t it?
Isla’s next appointment is in August, so keep your positive thoughts headed her way. In the meantime, one of Isla’s Respiratory Therapists, Abbey Smith, from Children’s Hospital is riding in the Courage Classic which benefits Children’s Colorado. Because of Children’s Colorado and all of the people who cared for Isla, like Abbey, we are able to celebrate Isla’s first birthday! They saved her life and we are forever grateful. Consider helping Abbey meet her goal. Any little bit helps.
This time last year, we had NO idea what was coming! The day after Memorial Day I went to my Doctors appointment and was admitted to the hospital on bed rest- the reason I started this blog in the first place! In the last year we definitely had some life changing experiences. I was thinking about one experience and (now that some time has passed) I was laughing about it… Isla was in the hospital in her medically induced coma and she needed a blood transfusion. The Doctors came in with the paperwork and waivers and went over all of the risks with us. There was a page long list of things that could happen but were highly unlikely, such as 1 in 50,000 chance or getting Disease A and 1 in 200,000 chance of getting Disease B. The Doctor was trying to reassure us that it was very safe and clearly the chances of anything on the list were slim because of the statistics. What this Doctor didn’t realize was PH affects 1 in 1 million people, so our odds weren’t on our side. When I remembered that day recently I just thought to myself- Holy Shit- we have been through some major stuff this year! Nobody should have to have those conversations… Nobody!
I know for me, it has really put things in perspective. I have always been pretty even keeled… but now I feel like I am even MORE so! I just don’t sweat the small stuff… maybe it’s because we have so much BIG stuff… or maybe it’s because we got a reminder that life is fragile and it’s important to make the most of every day because tomorrow is never assured. Interestingly enough, my Meyers Briggs personality type changed. If you are familiar with it, I was an ESTJ and am now an ESFJ. Typically, personality types don’t change… but I would imagine that a life changing event can make an impact!
Isla is doing really well. She is enjoying her night-time only Oxygen! She is a joy to be around, and I cherish the moments that all three of us- Matt, Isla and I can be together. I was just thinking… we need family picture! Here are a few…
This is right before we found out we were having a baby!
This was taken in the hospital, before Isla was born.
This was taken when Isla was 2 months old.
And we don’t have any more family pictures since then… just one of us taking a picture of the other… so we need a family photo 🙂
Today we had a follow-up appointment at Children’s Hospital. Both Matt and I feel sick to our stomachs every time we have to go back there. There is just so much unknown, plus the fact that twice in 2013 we went to a Doctor appointment and were admitted to the hospital for over 5 weeks… so I have a feeling we will be forever traumatized. Nevertheless, despite our fears… today’s appointment went really well!
Isla had an Echo and an EKG. Compared to her last appointment, her heart looks better… and the Doctor even said that it looks almost normal! He gave the okay to go from 24/7 Oxygen to night time only!!! That will make life so much easier… we won’t have to worry about this anymore:
He also said that we can stop one of her (many!) daily medications. It isn’t a PH med, but still, one less med works for us! He isn’t increasing her SubQ pump meds which essentially means he is decreasing the dosage since she is growing. He is hoping that she will be able to get off of the pump completely by the end of the year. Oh how amazing that would be! We are overjoyed with the news. We are holding onto hope that all of this will have a positive outcome. It just has to! Please continue to send well wishes Isla’s way. She isn’t out of the woods yet, but she is definitely heading in the right direction.
Here is Isla right after we took off her oxygen. She had an imprint from wearing a nasal cannula for the last 7 months and she even has a slight tan line. We are so used to seeing her with a nasal cannula, it’s like looking at someone who normally wears glasses that got contacts.
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.”
Having a sick child sucks in many ways, with one exception… it brings out the giving, caring, nurturing, supporting side of people. We have been in awe with the out-pouring of love since Isla’s diagnosis! We have never once felt alone. This whole situation has taught us a lot- to be present, to love and cherish every day, to not dwell on why things happen, to appreciate all that you have no matter what, to love unconditionally, to accept help when you need it and to pay it forward when you have the means. Last year we spent a total of 9 weeks(!) in the hospital, and although most people reached out, I know there were others that wanted to do something but didn’t know what to do. If you ever have a friend or friends child land in the hospital, and you aren’t sure what to do…here are a couple of ideas:
1- Just be there! Take time out of your busy schedule to check in on them regularly. Show up in person, call, text or email. Whatever you are most comfortable with. Keep doing it even if they don’t respond. They can hear you and feel your love but may not have the time or capacity to reply. You aren’t bothering them, you are helping them, so keep doing it.
2- Try to avoid saying, ‘Let me know if I can do anything.’ It’s kind of an automatic response… It is a very nice gesture, but even if you really will do anything, most likely they aren’t going to let you know. Choose something to do and tell them you are doing it, and just confirm that it’s okay. Some ideas- go to their house and rake the leaves or shovel the snow or take out the trash. Ask for a key so you can take the dog on a walk or to the kennel. Arrange a food train, just ask for email addresses and set it up at mealtrain.com. Raise some money to help offset costs. Send them a care package. Donate money to their cause in their name. Send them a gas gift card. Hire some house cleaners. Get them a massage, etc. (All of these are things people did for us. We were astonished and relieved… it helped so much!)
p.s. Isla update- she is still responding well to the meds. She has a follow-up appointment on 3/14, so more to come!
Matt and I have always been planners… plan activities for the day, plan meals for the week, plan vacations for the year, etc. When we got pregnant, we did what most couples do- we set a birth plan. It was informal… not even written down actually… but we knew the plan was to have a natural birth. People always tell you to be flexible because often times you can’t follow your birth plan, but quite honestly I thought we would be fine since our plan was pretty high level. Well, that didn’t happen… and if you know us or have read earlier posts, we had a scheduled C-Section instead.
After Isla was born, we set a plan for her care. I would stay home for 12 weeks and then we would have family come and help for 6 weeks and then she would go to daycare. We made it two weeks into her daycare stint before she got sick. Since then we realized that Isla is just not a good candidate for daycare, and really with all of her meds and SubQ and Oxygen, a nanny isn’t even realistic. One of us has to stay home. Based on our current situation, it makes more sense for Matt to stay home, so he recently informed his employer and is officially a stay at home dad!
We have had family and friends in town for a while to help out, but just yesterday our last house guest left and it was Matt’s first official day home alone with Isla. I have always known that he would be such an amazing stay at home dad, and on day one he blew my expectations out of the water. I came home to a list of what they did together and an amazing home cooked meal. Isla and I are so damn lucky!
The switch to SubQ went really well. We put the SubQ site in her left arm the day before the planned switch. They recommend doing it one day in advance to make sure there is no site reaction, and luckily she didn’t have one. Here is a picture of the site in her left arm and you can also see the PICC line in her right arm.
We had one day to go until we could get that PICC line out, but Isla decided we needed a little excitement, so she pulled it out that night. It had been working it’s way out, so we weren’t too surprised, but we had to go to the ER to get an IV put in and hook her medicine back up. Not too fun, but we all survived!
The next day we hooked her medicine up to the SubQ site and they monitored in the hospital for 24 hours. Everything was fine until about 24 hours later she was favoring her right arm and was extra fussy, so she was clearly having site pain. That lasted 24 hours, and since then she has been doing really well. The new pump is so much easier to manage. It is about the size of a pager and it comes with a case which we clip to her pants. It already feels like less baggage. Now… if we can just get her off the Oxygen.
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 →