Archive for the ‘IPAH’ Category

ImageA picture of G. Just because.

I’m finally to the point where I’m not feeling absolutely miserable from the mastitis, so I went to the hospital today to see G. When I walked in, they were doing an Echo on her. Naturally I was kind of curious, because no one had mentioned anything to me about one being done. Her nurse told me it was just to check to see if the hole in her heart (which was either an ASD or a PFO) had closed. So I just shrugged it off. Literally didn’t think anything about it.

Just did my brief-visit business: 1) Snap a pic of G. 2.) Do a short video. 3.) Give the nurse my milk 4.) Catch up on any changes that might have been made 5.) Give G a kiss and/or a cuddle.

Her CO2 levels have decreased a small bit, by the way.

Anyways, so I trade off kids in the hallway (the grandparents had L – and they were heading in to visit G), and we make a brief detour by the pharmacy to try for the umpteenth time to get L’s diuretic. Still no luck. Grrr. She’s not yet completely out, so I place a call to cardiology requesting the refill even though the pharmacy was supposed to have done it two days ago.

Call placed, we make a stop by dairy queen (side note: My 4 year old is totally feeling my boobs up right now. She’s way too fascinated with this whole pumping thing.) and then go home to hang out. I’m in the middle of a pumping session when the phone rings and its the hospital.

Ah hell. What now?

Its a cardiologist. He says that he reviewed the echo done today, and G does have a moderate-sized ASD, but that’s a good thing at the moment, because its keeping the pressures in her heart down.

If you’ve read the posts about L, you know what this means.

I now officially have two kids with Pulmonary Hypertension.


The PH speciaist who follows L will be checking G out next week.

Oh, and right in the middle of writing this, I got a call from the hospital. They’re placing G back on the ventilator to bring her CO2 levels down some more and hopefully help her heart.

I don’t even know where to begin on how much I’m ready to scream right now.


Just. read. it.

(Note by D: When I noticed how many people this blog seems to have reached, I asked Karlee to do a post about what it means to be a support person to try to continue the education thing that I try to do in most of my posts. I have not edited this in any way. I thought straight from her was the best.)


D asked me to write up a post as to what it means to be a support person. At first, I stared blankly at my computer. It seems like common sense to me to just be there and offer support. I don’t think – I just do. D made the comment that “I don’t know what its like to be in your shoes, just like you don’t know what its like to be in mine.” And that is true. It doesn’t matter how much I empathize with her and the situations with L and G, I cannot truly understand her side. All I can do is be there for her in any way I can or she needs me to be.

I sat down and made a list of things that came to mind in regards to being a support for a parent of a chronically and/or terminally ill child. I never sat down and purposefully set out to put myself in this role. I met D while we were both pregnant. We didn’t meet in person – we started out talking on a pregnancy forum and eventually Facebook and on the phone. It was after L’s open heart surgery that we really started talking daily. We didn’t meet in person until this last September. I didn’t think it was possible, but I fell even more in love with L. She is one heck of an amazing little girl.

D didn’t have the time or energy to really explain to me what was going on with L in the beginning. She was far more concerned with making sure that her baby was getting the care she needed. At the start, it was honestly curiosity that drove me to learn about L’s various heart defects and Pulmonary Hypertension once it was diagnosed. The more I got to know D and L, the more I was driven by compassion and a desire to help. I know that D said in one of her previous posts that I know probably as much about Pulmonary Hypertension as she does – she never asked me to. It was my choice and one I am glad I made. I have made it a point to help educate others as well. That brings me to my first point: Be willing to sit down and RESEARCH. Don’t rely on them to educate you. Take the initiative. Be prepared that what you will find might be terrifying and heart breaking – and you aren’t the one living the reality. Just think what it means to them.

Don’t say sorry, especially with any sort of frequency. I know it the gut reaction anytime a person hears bad news. IT DOESN’T HELP. I know this is one of the things I hear D say frequently. It gets irritating to just hear “I’m sorry”. Say it like it is – the situation sucks (depending on your language, there may be more then a few choice words added there). It SUCKS that L has a terminal disease and it SUCKS that G is still in the NICU and is on the vent. “I’m sorry” comes off as patronizing and weak.

This seems to be pure common sense to me, but I am still going to say it. Don’t make promises you can’t keep. Only make offers that you are truly willing to follow through on. They have enough disappointment and stress in their lives. You are supposed to be taking that away, not adding to it. If they ask you to do something, and it is within your power to grant, DO IT. It doesn’t matter if it is as minor as sending chocolate. It helps. I have lost track of how many packages of chocolate I have sent D.

Make them laugh. Even when things seem at their darkest, laughter helps. This was one thing I have been unsure of until recently. D and I both tend to take our conversations off on weird tangents, often with hysterical results. A few days ago, I stopped and asked her if it was okay to try and make her laugh. This was when G was really declining quickly, and I wasn’t sure if it was appropriate. The last thing I want to do is add stress. “Laughter is always a good thing.” Alright then. Bring on the boob jokes.

Finally, develop a thick skin. Chances are, some of the anger and frustration will be taken out on you. Sometimes frequently. Don’t take it personally. Move on.

I realized that I’d been doing a lot of blogging about G and the X-Linked Periventricular Heterotopia but I hadn’t talked much about L and the Idiopathic Pulmonary Arterial Hypertension.

Prepare to be educated.

Pulmonary – From Miriam-Webster: a : of, relating to, affecting, or occurring in the lungs<pulmonary tissue> <pulmonary edema>

Hypertension – From Miriam-Webster:  abnormally high blood pressure and especially arterial blood pressure

So, specifically, Pulmonary Hypertension is abnormally high blood pressure affecting the lungs (and heart).

It is not the blood pressure the nurse measures in the doctor’s office. It cannot be managed through ‘diet and exercise’. It is not cheap to treat – medication costs can easily top 100k a year.

It is a terminal illness. That means it will kill you. It is often misdiagnosed with asthma until its almost too late to do anything about it.

The Go-To website to educate yourself about Pulmonary Hypertension is . There’s all sorts of information on there from all parts of this life.

I will hit some highlights though.

– You will often see people say just PH or Pulmonary Hypertension, but please understand that it is a general term. There are many types of PH. What we deal with tends to be specifically Pulmonary Arterial Hypertension.

– Anyone can get it, though its more common in middle-aged women

-(Again) Its often misdiagnosed as asthma because it presents very similarly at first.

-There are three classifications – Idiopathic/Primary, Heritable/Familial, and Associated/Secondary .

Put simply – Idiopathic/Primary means unknown cause. They know that someone has it, but they don’t have an explanation as to why  they have it. 

– Heritable/Familial means that you got it because it runs in your family. There’s got to be a couple people diagnosed with it before its considered this.

– Associated/Secondary means that you have it because something else is wrong with you. Some examples are: CHDs, connective tissue diseases, HIV, etc.

Untreated, the lifespan in adults is generally three years from the time of development.

Untreated, the lifespan in children is generally 10 months from the time of development.

It. is. freaking. scary.

There are three main methods of treatment and often methods of treatment are combined. Remember, these are treatments, not cures. There IS no cure.

Oral – You take a pill (such as Tracleer), or an inhaled medicine such as Ventavis.

IV – You have a continuous flow of medication being pumped into your body 24 hours a day, 7 days a week. If your pump breaks or malfunctions for any reason, you have a limited amount of time to get it fixed before things can get really, really bad. Medications include: Flolan, Remodulin, and Veletri. Flolan and Veletri are basically the same medication, but Flolan has to be kept on ice, whereas Veletri is stable at room temp.

Subcutaneous – Remodulin (It can be administered both ways.)

Imagine having your 3 year old hooked up to a IV pump 24 hours a day.

Imagine having to insert a needle under your 5 year old’s skin, and make sure it stays there for weeks at a time.

Probably can’t imagine, can you? I hope not. I sincerely do.

There’s more that I probably should mention, and I have no doubt that this is hardly the last education post I’ll make on Pulmonary Hypertension. So now, for the personal.

L is a red-headed, freckle-faced little ball of energy. She very much meets each day with an enthusiasm I would love to have. Her perfect day begins and ends with cuddles.  She is amazing. (She’s also a demanding little brat, but that’s beside the point!) We were so scared with her, and at first treated her like a fragile little doll.

Not anymore.  Yes, L is sick. Yes, her disease is considered terminal. But, no, she is not fragile. She’s a stocky little munchkin with too much energy and not enough time in the day.

And yet every morning, if she doesn’t wake up before I do, I’m terrified. Because, you see, her condition could worsen dramatically in the blink of an eye. There is every possibility that if she’s not up before I am that she’s passed away during the night. And I didn’t know. I wasn’t there to try to save her because I was sleeping. Its hard enough to deal with normally, but putting her to bed when she has a cold or other respiratory thing going on just makes me sick to my stomach.

I’m in a group for Family of Children with Pulmonary Hypertension on Facebook. It is simultaneously the best and worst group that I’ve ever been in. Its the best because it is filled with people who know exactly what each other are going through. Its the worst because we share each other’s pain. We are there, virtually holding vigil, as one child after another gets sick, and fights for life. We are there, wishing we could be by another parents side, holding them, when they have to say goodbye to their child. We are there in heart and spirit even if we can’t be there physically.

And it hurts. Oh lord, does it hurt.  Even just thinking about it makes that hot, sick feeling wash over me. We’ve said goodbye to so many kids in the past year alone. I’m ashamed to say I’ve lost count of how many children we’ve lost since I joined the group.

We lost a little one who was doing well, then got sick with a cold… three days later she was gone. From a COLD!!! You cannot believe how ridiculous and scary that is. She died from complications from a flipping COLD! Three days. It took only three days for her to be gone forever.

We lost another little boy who began getting worse gradually. I believe from the start of his downturn until his passing was about 6 weeks. I could be a bit off though.

We lost Molly. Molly is the only one I will name because you can look her up easily because of the Flat Molly project. Click here to see the youtube compilation, and if you’re the crying sort – be warned. Have tissues nearby.

I could go on and on… we lost, we lost…we lost. We’re always losing. We do gain – but what we gain is another PHamily  member that we know we’ll eventually have to say goodbye to. We don’t want to gain any more members.

It gives you nightmares. It breaks your heart.

I’m lucky in the fact that I’ve surrounded myself with a wonderful group of online friends who do their best to understand what I’m going through (even though they can’t), to learn all they can about L’s disease, and promote awareness. They know who they are, and how much I love them.

In particular, though, I have to give special thanks to a great friend named Karlee. I’m naming her because I’m going to shamelessly plug her business in a moment. We were pregnant at the same time. We didn’t really know each other well during the pregnancy, but after L was born, Karlee sort of took up the flag and really was there for me when I needed someone to be there. She still is, to this day. She knows probably as much about pulmonary hypertension as I do. She is always trying to raise awareness on her business page. She makes things and donates them to people who could use them. She fundraises. You name it, she does it. She’s even made hats and scarves for L that were tailored to what she needed at that time.

So if you are looking for handmade goods of the knitted and crocheted variety, you really need to check out She makes pretty much everything you can think of, and she has reasonable prices too.

Here’s an example of one of her cuties.

Custom knit triceratops stuffie in camo - front view.

…and I’m written out. Writing about L’s disease just makes me mentally tired. More to come!