2017 Update

IMG_0238A friend recently reminded me that it’s been a while since we updated this. Well, I suppose no news is good news. We went in today for Isla’s follow-up echo. She did great! She was able to do everything without her cousin by her side, and was very brave!

Results show that her heart is 98% normal. Dr. Ivy wants her to stay on meds until it is 100% normal. The fact that we are talking about WHEN it becomes normal is so amazing! Until then she will remain on one medication 3x/day, but we won’t increase the dose which technically means she is weaning.

The only negative thing is when she finally comes off meds, they will need to do a heart cath about 6 months later to check the pulmonary pressure. That scares us considering how she responded the first time, but we will cross that bridge when we come to it.

Our next appointment will be in 9 months! Longest break yet. The picture above is her amazing Nurse and Doctor, and below is Isla playing with the pile of toys they gave her today. 💕

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Spring 2016 Echo Update

 These posts have become fewer and farther between, which is the sign of a good thing! Since the last post in November, we’ve dropped one more med! We are now down to Sildenafil (Viagra) 3x/day, which she will likely stay on for a while. We scheduled Isla’s follow-up echo for the end of February. Unfortunately, that appointment didn’t go so well. Isla’s cousin Lila wasn’t allowed to come to the hospital with us because of respiratory season, which turned out to make a pretty big impact. When we tried to do the Echo, she wasn’t having it. The Doctor wanted to sedate her to get it done, but we negotiated with them to get a little more time to work with Isla. We made a plan to bring her to the hospital once a week for a month to play, get used to the surroundings, and learn to have a positive association with the space and people. We also practiced using sticker charts so we could use one the day of the echo so she would know what to expect. We scheduled the follow-up echo for the beginning of April and agreed that we would resort to sedating her if our plan didn’t work. Also, because Lila made such a big difference, they let her come with us in April. All of that combined worked! One month later, Isla was able to have a full check-up- Echo, EKG, blood work, etc. All without sedation. 

Having her get through the appointment was one goal. The other was having good test results… which we did! Her Echo looked “normal” and her labs looked good with the exception of one elevated number which might not mean anything. We got the go ahead to wait 6 months until her next Echo. The will be the longest break we have ever had. 
On another note, Isla started preschool in January and is loving it. She has been exposed to lots of germs and has had a cold on and off since she started, and she has been handling it so well. It’s great to see her be able to run around and play like a “normal” kid. We hope and pray that everything continues in a positive direction and we can report great results after every visit. Our Doctor has some patients that have improved and then back-tracked, so that will always be a fear. But we just have to hold on to every bit of hope and enjoy every positive and healthy moment. Lots of love.

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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.

Cath Update #2

Isla was a champ! We were able to go back to post op as she woke up. We had to keep her flat for an hour which was hard because she woke up pretty quickly. We are now back to the floor and will hopefully be able to go home today!

Her cath results were really good. It’s all quite complex, so the quick version is- she still has mild PH but her pressures are much closer to normal… Not quite normal, but close! We have the green light to take her off the pump and keep her on oral meds. We will continue to do echocardiograms and ekgs in clinic regulary and just hope that she continues to outgrow this. Our doctor is hopeful but also prepared us for the fact that since we don’t know why she has PH in the first place, there is a chance it could worsen later. However, the outgrowing theory seems to make sense and that’s what we will focus on.

Thank you all for for love, support and prayers. This was a really stressful couple of days and you kept us strong. We are joyous that we can report out good news. xo

   

   

Cath Update

Overall yesterday went pretty well! Being in the hospital brings up a lot of emotion for me, Matt and now Isla. We checked into the hospital around 1pm. As soon as we walked into the room, Isla started panicking. Just getting the pulseox and heart rate stickers on her was a struggle. They informed us that she would need to have an IV before coming off the pump… Just in case. Getting the IV in was tough. She started freaking out when the nurse walked in, before he even touched her. Both Matt and I took turns holding her and it took 2 sticks to get it in. When I was holding her I noticed her diaper get warm… I definitely think it was a fear response. But man, I have to say… In moments of fear, she fights!!! What a brave little girl! The plan was to get an Echo and an EKG yesterday as well, but after the nurse witnessed her IV experience, they decided to wait until she was under anesthesia for her cath.

We headed to the pre/post op area around 6:30am today. They ran through the details of the procedure which was unbearable. Then they let us go back with her until she fell asleep. She fought that too. They gave her something to take the edge off and then put on the mask. It was nice to be in there with her, but also really hard. Our nurse just came out and told us that she is under and her breathing tube is in and she is stable. He said she is in good hands since the anesthesiologist wrote the book on anesthesia and pulmonary hypertension… Literally… He had lots of information on the topic published. Isla’s procedure will be a part of 2 research studies as well, so her hard work back there will help lots of other people with PH. Please keep her in your thoughts and prayers. We are excited to see her in a couple of hours. I will update again after… xo

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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Winter (almost) Update

Isla had her PH follow-up appointment on Friday. She is getting to an age now where she knows something is going on and is wary of all of the doctors… Because of her PH she gets monthly labs and also a month RSV vaccination (during the winter) so she is poked and prodded a lot, so I don’t blame her for being wary! Results from her echo looked good. Her heart continues to look better so we started to wean her pump medication!!!!!! The hope is to have her off the pump by the end of March. We are going back for another check in January, so fingers crossed it continues to look good. Our doctor wants to do another heart cath once she is off the pump which I can’t even think about. I’m terrified to put her under again… Ugh! One day at a time. I took a couple pix at her last visit but they all turned out blurry because she was running around so much!

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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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