Progress!

Gosh it’s been a while since I’ve posted. I suppose no news is good news! Since Isla’s cath in April, she has come off her pump, O2 and her inhaler. That leaves us with 2 oral meds. One is 3x/day and the other is 2x/day. Luckily she likes them!!! We went back for a clinic appointment in June. Those appointments always include an EKG, Echo and consultation with Dr. Ivy. At that appointment she completely lost her cool for the echo. She’s just too young to understand that it won’t hurt… And at this point she’s expecting pokes and prods, so it didn’t go well. Plus, I think it’s hard to get any 2-year old to lay still for 40 minutes. They said they needed to get a good read at the next visit and if she couldn’t stay calm they would have to sedate her. We definitely wanted to avoid that!

We scheduled the next appointment for November 6th and did everything we could to prepare her. We bought a kids boom box with a microphone on it and pretended that it was an echo machine. We practiced on her and her stuffed animals and kept emphasizing “no ouchie”.


We showed her pictures of other kids having echoes and pictures of her doctors and nurses. Then when it came time to head to clinic yesterday we asked Isla’s cousin to come for moral support. It all worked out and she did great for her echo! They were able to get a good read and no sedation was needed.


Unfortunately she got upset for her vitals and EKG so they couldn’t get that done but the more important part was the echo. We are hoping one day she can have a clinic appointment from start to finish without panicking. It’s so hard for me and Matt to see her get so upset.

The results of her echo were great. Her heart is looking very strong. Her doctor wants her to stay on both meds through the winter and come back in March for another echo. If everything looks good in March she can come off the one medication that has liver damage risks and requires monthly blood draws. That will be amazing!!! If that’s the case then she will only need 4 more blood draws!

We are definitely on the right track! It’s exactly 2 years since Isla was in a medically induced coma. It’s been a roller coaster, but we are so thankful that she is doing well and will hopefully be able to put this all behind her. Regardless we will always be a part of the PH community. It’s a complicated, dangerous disease and it needs more visibility and research. We’d love to see everyone at one of the PH races. Here’s a picture of Isla with Dr. Ivy from the race in October.


And I’ll leave you with one last picture of Isla being a kid… as she should be… enjoying some ice cream.

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Best Birthday Gift Ever!

On April 1st I turn 35! This year I already know what I am getting as a gift, and I am sooo excited. On April 1st, Isla will be coming off the pump! This is such great news because it means she is doing well and her heart is stronger. The plan is to check into Children’s on the 1st around 11am. We will unhook her pump and they will monitor her overnight. The next morning she is scheduled for a Right Heart Catheterization at 7:30am which will require about three hours under anesthesia. The medication in the pump has a 4-hour half-life, so by the time she goes in for her Cath, they will be able to tell if her heart needs to be back on the medication.

It will be such a strangely emotional birthday, made up of joy from her coming off the pump and fear of her going under anesthesia. We will focus our thoughts and energy around her making it through this procedure safely, and heading home pump-free shortly after. I can just see her now in a bathing suit, splashing around in a pool… Matt and I can’t wait for that!

Our visit will likely overlap with another PH family who we learned about when Isla was first diagnosed. The little girl’s name is Brooklyn and her story when she was first diagnosed was very similar to Isla’s. They made a video about her PH journey (below) which I encourage you to watch and share. It’s amazing what our babies have been through! I am really excited to get Brooklyn the Brave and Isla the PHighter together. There will be strength beaming from the room where they meet 🙂

Please send your thoughts, love, energy and prayers to our little Islabug on April 2nd. We will keep you posted. xo

 

 

On Track…

We had our follow-up appointment on Friday. Echo and EKG are still looking good! So the plan is to start diluting her pump medication and once she is down to 8ng/kg/min then she can come off (she is at 16 now). She will be admitted to Children’s when we take her off so they can monitor her. We will spend the night (ugh!) and the next morning she will have to have a heart cath (ugh!). We asked if there was any way around doing the cath, but her Doctor feels it’s extremely important to see what her pulmonary pressures are. We definitely want to make sure everything looks good so we can all be certain that coming off the pump is the right decision, but we are scared! We just have to stay focused on the end result which will be a pump-free Isla. We might just have to stop at a pool when we check out of the hospital! We will find out soon when all of this will happen, but likely end of April/beginning of May. Here are some pix from this last visit!

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Therapy for Mama Fordyce!

One year ago our lives were forever changed. We went from enjoying the joyful exhaustion of being new parents to the fearful exhaustion of being captive in a hospital. At Children’s we witnessed the powerful sight of suffering children combined with adults doing everything in their power to save these young souls. It’s really an indescribable experience to see so much love and so much pain all at once.

Here is Isla just a few days before our new lives started:

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I have never written in detail about our hospital experience, but I would like to share some of what I personally felt as our new prescribed adventure came before us. This will be therapeutic for me, so thanks for listening… and pardon the language 🙂

The first night we were in the hospital we were under the impression that Isla had a virus and they were going to keep us there for monitoring. It seemed like everything was going to be fine, so Matt went home to let the dogs out and planned to return in the early morning. Had we known what was about to happen, he would never have stepped foot outside. Doctors and nurses kept coming in and asking questions and doing tests. Finally at 3am I was rocking Isla to sleep and all of a sudden she started crying. But it wasn’t a normal cry… you could tell something was terribly wrong. She was hooked up to monitors for heart rate, oxygen and blood pressure and every number went the wrong way. Everything was beeping and the nurses in the room took her from my arms. Suddenly they pushed the ‘code’ button on the wall and at least 20 people came in the room. They bagged her and got the crash cart out but luckily didn’t have to use it. I thought she was going to die. I was in shock, so much that I couldn’t physically call Matt. I handed my phone to a nurse who called him for me. He didn’t answer because he was exhausted from not getting any sleep the previous few nights because Isla hadn’t been sleeping. (In hindsight, we now realize that she had been having Pulmonary Hypertension issues.) After a few minutes I called our friends and hysterically said “Isla is not okay and please go and wake Matt up.” They did… oh how lucky we are to have such good friends. Once Isla was stabilized they sent her to the Cardiac ICU where she would stay for the next month.

The next day we learned of her diagnosis of Pulmonary Hypertension and that the previous night she had experienced what is called a Pulmonary Hypertensive Crisis. The next step was to have a heart cath to get the formal diagnosis and to see what her pulmonary pressures were. We had to wait a couple of weeks because she was getting over a cold and they didn’t want to put her under when she was sick… so we waited and waited (in the ICU). We spent her first Halloween there as you can see from her hat. Here arms in this picture were wrapped up because she had IVs in both arms.IMG_0694 They put her on one oral med (Viagra) to help with the pressures in the meantime. The day before her heart cath our nurse told us that in order for insurance to cover and approve her being on any PH med, Isla would have to have the cath med free… meaning she needed to be off the meds for at least 24 hours before hand. If you ask me- this is so messed up. They could tell loud and clear that she needed this medication from her echo (ultrasound of the heart) but we had to do this because without insurance coverage, the medications cost over $100,000/year. Anyway, she came off the meds and had her cath which did not go well. Her pressures were supra systemic- very high/very bad, and she did not respond well to the anesthesia. I know being off of her meds didn’t help either, but don’t get me started! She had to be intubated, paralyzed and sedated and brought to ICU. Seeing your child in this state is unimaginable, unbearable and quite frankly fucked up. Within a couple of days she was extubated and was doing well. Matt went home at this point to take care of the dogs and this was the day we realized we needed help with the dogs because… he left and I was holding her again and suddenly I heard the same cry. Not good… the nurses grabbed her from me and it was déjà vu. Twenty people in the room, the doctor telling me to sit down, the social worker asking who she could call… and the room was spinning. I got up and went into the hallway and started screaming. If you know me, you probably know that I can keep my composure in most situations and it can take a lot to rock me. But not then, not there. I hysterically started yelling at the world, at whoever would listen (which was everyone since I was yelling!) “What the fuck? Why the fuck is this happening to her? She is a fucking baby… this isn’t supposed to happen.” One of our PH nurses heard me and ran over and started talking me through it. Another nurse called Matt and he turned around and came back. A mothers number one job is to protect her child, and I wasn’t able to do anything for her. I felt helpless and lost and empty. Look at her…

At that point, Matt and I set up camp in the hospital. Our parents came to help support us and help us with the dogs. It was a heavy, dark time not knowing what was going to happen. I remember the nurses took all of us into a room to talk about next steps. They were talking about ‘when Isla gets out’ but all I could think about was that my little girl was intubated, paralyzed and sedated and was going to be like that for ‘a while’… (it was about a week, although it felt like an eternity). We were sitting in this room together and the nurses mentioned that she was going to need 24/7 medication delivered through an IV. My previous blog posts talk about how that all works, so I won’t go into detail. I lost it. What do you mean she has to be on an IV? I was still in the ‘what the fuck’ mode. That was the beginning of the end of my denial stage that this was happening to us, and slowly I switched over to acceptance.

The amount of emotion I experienced during that time was unimaginable. The other thing I so thankfully experienced was the sense of support and community. So many people reached out to us in so many different ways (which I have talked about in other posts) but I have to say- that has made a huge impact on me. I think it’s easy to wake up every day and run through your routine which typically is all about you and your loved ones. Which makes sense, right? But our experience shed light for me that we are not just here for ourselves, we are really here for each other. Volunteering your time, your money or your experience in ways such as participating in events, assisting non profits, giving advice, doing research, helping a friend or stranger, whatever it may be. It doesn’t just have to be the formal/literal volunteering. The question I now ask myself is- did I help someone today? Did I make someone’s day better? Because I know that I survived my worst nightmare because of the help and support of my husband, my family, my friends and perfect strangers who did exactly that. Because of them, because of you- I survived.

Thanks for listening.

p.s. November is Pulmonary Hypertension Awareness month. If you don’t remember what it is exactly, visit phassociation.org and tell at least one person. xo

This is when Matt and I got to hold Isla for the first time after she was extubated… and in closing, this is a recent picture of Isla the PHighter:

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Repite, Por Favor!

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!

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The New (getting kinda old) Normal!

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 😉

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 was the best of times, it was the worst of times.

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. 

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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?

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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. 

http://www.couragetours.com/siteapps/personalpage/ShowPage.aspx?c=9pLILSPuEcLWH&b=8862253&sid=dpIRK4MBJfIJLNMyHsH

Happy 1st Birthday Islabug! 

Slow and Steady Wins the Race

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-

 

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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…

 

 

I Need Some Wind in My Floppy Sails

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:

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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.” 

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