Archive for the ‘CHDs’ 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.

fuckfuckfuckfuckfuckfuckFUUUUUUCK!!SHIT!DAMMIT!

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.

http://www.friendshipcircle.org/blog/2013/03/13/15-reasons-why-its-hard-to-get-along-with-special-needs-parents/

Just. read. it.

IMG_0085

G is in her new digs. She got moved from one unit to another because of hospital renovations. The new isolation unit is much bigger and nicer. Even has a couch for parents to chill on!

She’s still on CPAP. They put her on a nose mask instead of the traditional prongsy-thing because the regular doodad (I wish I knew the name for it) wasn’t staying on her little snub nose and maintaining pressures like it should.

The new nose-mask makes her look like a little piggie.

She’s due to get her 2 month immunizations next week, so they gave me the stuff to look over. I think I’m going to request we space them out. Give her like a week in between each one. G has proven she’s super sensitive to things, so why risk a reaction by giving her a bunch of vaccines at once.

I asked when they last did an x-ray, and the nurse wasn’t sure, so she pulled it up and I read over her shoulder. We both got a shock when we read at the bottom of the report “Persistant Cardiomegaly.”

…Uh, excuse me? They’ve never mentioned that before. So we proceeded to go back through her x-rays. Not one place has cardiomegaly mentioned. We know G has a potential ASD, but that’s it.

So needless to say the nurse will be bringing this up with the doctor. Not that we think G’s heart is enlarged, but that’s not a nice thing to have in the reports if its not true.

Her CO2 level is back up to 74 BUT her PH level is fine, so they’re not too concerned about acidosis.

The x-ray did show minimal improvement on her lungs, by the way. I’ll take that over no improvement, or worsening, any day!

(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 http://www.phassociation.org/ . 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 http://www.kreationsbykarlee.com/. 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!

My initial thought as I go to type this out is ‘why would anyone that’s reading this NOT have had their baby tested already?” but I know that isn’t fair to think. Many people aren’t aware of how important or even how easy this test is. Its also cheap, to boot!! So I’m going to spell it out, and I’m probably going to repeat myself a bit. I’ve found that repetition helps drive the point home.

I’m not going to use technical terms. I’m going to make this nice and simple.

Pulse Oximetry Screening = Pulse Ox Test

Why is it important? Its important because the most common birth defect is a congenital heart defect (CHDs)and the pulse ox test is the first step towards detecting many CHDs. CHDs affect roughly every 1 in 125 kids born in the USA ( www.marchofdimes.com ). It can also detect some lung problems, too. (Think about it this way – it measures the level of oxygen in your blood, and your heart and lungs are both involved in making sure you have plenty of oxygen in your blood. So if something is wrong with your heart or lungs, and therefore you’re not getting enough oxygen to all parts of your body… the pulse ox test can help pick up on that.) It is not 100 percent accurate, but its still very effective.

What is it? Its a test that measures the level of oxygen in your blood. This level is called ‘saturation’. I will probably call it ‘sat(s)’ a few times in this post.

Your blood cells transport the oxygen to all the areas of your body. Without oxygen, bad things happen, right? So you need to have X amount of oxygen going through your body at all times to make sure that everything can work the way it needs to work! The pulse ox test measures that “x” and gives the doctor an idea of if your blood cells are able to carry oxygen like they should be.

So, they probably have to give my child a needle poke, right? They do that for a lot of other tests… No. The pulse ox test is about as invasive as sticking a thermometer under your arm pit to take your temperature. What happens is simple.

(Infants) They take this little thing that looks like a band-aid, which has a red light-emitting sensor in the middle of it, and wrap it around your baby’s hand or foot. The light from the sensor shines into the baby’s hand/foot, and they need the baby to hold still for like a minute (which can be achieved via swaddling if nothing else). The sensor instantly starts detecting the oxygen level in your baby’s blood. When they get the reading, they take the ‘band-aid’ off the baby’s foot and you’re done! Its just that easy!

from flickr.com

Dark shot of pediatric pulse oximeter around child’s foot

 

(Older kids) They use a fingertip monitor and your kiddo sticks his/her finger into it. Its padded, it doesn’t hurt. It operates the same way the above mentioned ‘band-aid’ works, but your kid is older and can be expected to be able to hold their hand still for a few seconds, so they don’t need to tape it on. They get the reading, they take the probe off the child’s finger, and you’re done! Could it be any more simple or less scary sounding? (This is how they test adults too, by the way.)

From wikimedia.org

They might look a little different, but this is it basically..

How much does it cost? Because if its not a required test, I’ll probably have to pay for it…

From http://www.cdc.gov/ncbddd/pediatricgenetics/pulse.html

Costs of pulse oximetry screening include screening equipment, supplies associated with screening (e.g., probes, adhesive wraps), and staff time needed to perform screening and track results.

  • Screening has been estimated to cost less than $15.00 per infant.

 

You might have to pay more than that if billing is being a jerk, but… consider this:  Even if it cost fifty dollars… fifty dollars  to potentially save your kiddo’s life versus a couple thousand  to bury him/her.  Which one would you prefer? (I pray you didn’t have to actually think about that one…)

How do I ask for it in the hospital? I recommend that you actually call ahead of time to the hospital you are going to deliver at (you have 9 months to do it…) and ask if it is standard practice.  Also, discuss it with your OBGYN or CNM. Make sure he/she knows that you insist on the test being done. Last: if you have a written birth plan then write it down!

What if  I forgot to ask for it in the hospital, or didn’t know to ask for it? What if I’m not sure if it was done or not? I will give you the same answer for all three. The next time you go to the pediatrician, ask for a pulse ox test to be done. Your pediatrician might say that its standard procedure to have it done at whatever hospital you delivered at. I would ask to have it done again anyways. Just so you know that everything is okay. Sometimes standard procedures become so routine that things can get missed by the people who do them over and over and over again.

What if I ask, but my pediatrician says no. That they only do it on sick kids, or kids who have a family history of a problem. (One of my mommy friends actually had this excuse given to her…) Slap him. Kick him in the manly bits. Tell him he’s a flipping idiot. If your pediatrician is a butthole, which can happen, I would skip straight to “If you don’t do it at my request, and my child would happen to fall seriously ill or die from an undetected heart or lung defect that could have been picked up by this test, I will sue you for every dime you make for the rest of your life and I will make sure everyone knows that you said no to a test that could have saved my baby’s life!” and then I’d probably find another pediatrician, because you don’t need a butthole taking care of your kids.

However, if your pediatrician is reasonable, then I would simply say that “I know  that sometimes just because children don’t look sick, that doesn’t mean that they aren’t sick. I know CHDs are the most common defect, and that even if I don’t have a history of it in my family, that doesn’t mean my kid is perfectly in the clear.” Some variation thereof, and if your pediatrician has two brain cells to rub together, they will do the test.

What if my pediatrician’s office doesn’t have a pulse ox test machine available? ARE THEY STUPID?! Okay, seriously, while I will always recommend that you have the test done by a medical professional, the truth is the pulse ox monitoring is so simple that you can buy a tester off the internet and do it yourself for about $60.00! I did a quick Google for ‘pediatric pulse oximeters’. This is one of the first links that showed up. http://www.walgreens.com/store/c/choicemmed-children-oxywatch-c53-fingertip-pulse-oximeter/ID=prod6060764-product?ext=gooPersonal_Care_PLA_Oximeters_prod6060764_pla&adtype={adtype}&kpid=prod6060764&sst=1fd5b48a-4ac6-a308-82d6-00006ea0c12c

I’m well known among my friends for being a bit of a pushy broad about the pulse ox testing. I have outright told my facebook friends that “If you know me, then you know L’s story. If you know L’s story, if you’ve heard me speak on the importance of pulse ox testing, and you do NOT get it done… if you know someone who is pregnant and you don’t tell her how important it is to get it done..if you don’t spread the word to people who need to hear it… and something happens to that baby that could have been prevented if they had known to ask for a pulse oximetry test then its on YOU. You will have that guilt. YOU could have potentially saved a life.”

So now that you’ve read this and know how easy and quick it is… Its on you.

In September of 2008, I found out I was pregnant.  I did not want to be, and to be truthful I did consider abortion and adoption, both. I was in a bad place, it was a bad time, and I just was not ready to be a mom. But I couldn’t abort. Then adoption fell through when I was supposed to meet with a potential family, and got a call that day saying that they’d had a child placed with them. I took that as a sign that I was obviously meant to keep the fetus growing inside of me.

I remember one of the ‘moral quandry’ questions I’d discussed with classmates at some point had been “If you knew your child would be sick, would you still make/have made the choice to give birth to it.” It was an interesting question, and being the somewhat snobby youth that I was… I said “of course not.” Why would I ever choose to have a child that I knew was going to be chronically or terminally ill? Or even mentally disabled? What kind of person knowingly brings a child like that into the world? What about their quality of life? Besides, the world is overpopulated as it is!

I didn’t think it would ever be an issue for me anyways.

Hah!

I faithfully attended my doctors appointments and got all the tests done that I was supposed to have done. The ultrasounds showed no abnormalities. I was told to expect a healthy baby.  At this point I’d even picked out a name for her. I was probably 7 months along before I actually started to *want* her.  I mean, she amused to me, the way she’d go nuts inside my abdomen but I still wasn’t ready to be a mom. Finally, though, a little bond did start to develop.

It was a rough pregnancy. I had contractions almost every day it seemed like. I had random bleeds. The doctor wanted to put me on bedrest. My response was to hold out my hand and say “Pay my bills for me and I will!”

Then, on March 6, 2009, I went into labor. I won’t give you any grueling details, because there really weren’t any. The most interesting thing that happened during the process was driving myself to the hospital. I will say one thing though: Good drugs. GOOD drugs. Between the nubain that I got because they didn’t think I could get an epidural, and then the epidural that I ended up getting… I was in a happy, care-free place.

I regretted that afterwards. I still do. It was just me and the nurses/doctors in the room, and I know some stuff happened. I know I was put on oxygen. I know the doctor made a comment about the placenta. That I was practically dry when I went into labor although my water had never obviously broken.

Anyways, I had my baby. She was ugly right then. I know, I know. I should go on and on about how I looked at her and fell instantly in love. About how beautiful she was. But – that would be lying. She reminded me of Yoda, minus the green skin and over-sized ears. I just hoped she’d pretty up at some point.

We attempted to breastfeed, but it didn’t go too well. So we supplemented almost right away. Didn’t particularly bother me because I thought I’ll just bf her 2 weeks to give her that colostrum stuff and be done with it. Breastfeeding squicked me out!

She looked fine to me. She turned purple when she cried, but I thought “Well, hell, I’d turn colors too if I screamed as much as she did!” and didn’t think anything about it. The nurses didn’t either, it seems. Then my mom came to visit, and right away she was like “Honey, that’s not normal.” She mentioned it to a nurse, who said she’d mention it to someone else.. and then the matter was dropped. I had L at 1:14 in the morning on her due date of March 7.  7:30 that night, the pediatrician came to do the discharge exam with her. At 8:00 pm the pedi was in my room, telling me that she had a hole in her heart. A big one. She knew it was a big one, because she almost didn’t hear it. L’s heart sounded almost normal.

Suddenly everything turned upside down for me. L was put into the NICU. I wasn’t even allowed to hold her. I got yelled at for taking a picture with my phone.  Visiting hours even for parents were strict. It was ridiculous!!

But a few days later, she was transferred to Children’s Hospital and put into the NICU there. So much different. God bless Childrens Hospital!

Test after test was done. Its mostly a blur.

L was diagnosed with:

–          VSD

–          ASD

–          PDA

–          Bicuspid Aortic Valve

–          Enlarged Heart

–          Malformed Lung

–          Arachnoid Cyst

–          Gray Matter Heterotopia

–          Malformed corpus collosum

–          Pulmonary Arterial Hypertension

I don’t think I’m forgetting anything, although its very possible that  I might be . After a while, everything just starts to blur together.

From the time of diagnoses on, it was a downward spiral for the longest time. They had discussed the possibility that she might need open heart surgery at some point, but seemed to be at least somewhat optimistic. They let me take her home on March 14th. Unfortunately, after only like a week a home, L was admitted back into the hospital. I think we were actually put back into the NICU. She was sleeping all the time. And I could not wake her up. That was a symptom of heart failure I was soon to learn. Suddenly it seemed to go from she might need heart surgery a couple years down the road to she could need heart surgery tomorrow, next week, or … well, yeah. We no longer knew.

Her heart problems took precedence over anything else. We no longer worried about the stuff that was going on in her brain, especially once it was established that I had some of the same defects that she had, and as one of the geneticists put it “are apparently doing fine, so we’re not that concerned”.  I do remember the doctors talking about the fact that she had high blood pressure in her heart and lungs, but I didn’t think much about it. I just figured, hey, they can put you on meds for that, right?

After another week in the hospital, L was released and came home again. This time she was out of the hospital until June 18th. There were weekly trips to the pediatrician and many scares, but we managed for that long. We had actually been in talks with the pediatrician to put her in the hospital before her open heart surgery, which was scheduled on June 25th, to ‘tank up’ because she hadn’t been on the growth charts since birth, basically.

That doctors visit on the 18th, though, we saw that her pulse oxygen saturations were in the 70s. Needless to say, shortly thereafter, we ended up at the hospital and they decided to admit her. A few days later (still in the hospital), she had an episode where her sats plummeted to the 60s. It took some work to get them back up. A couple days after THAT, they took her down to the cath lab because they said they had to get a reading on the pressures in her heart and lungs. If the pressures were too high, they couldn’t do surgery because it would kill her. Well, they never got a chance to do the cath because when the anesthesiologist put her under, it was discovered that one of her lungs had collapsed.

And just that quickly, surgery had went from one of those ‘its routine, we do this all the time’ type of things to ‘hope for the best, but preparing for the worst wouldn’t be a bad idea’. Needless to say, it was a very traumatizing time.

Finally, on June 30th (after a couple scares with fevers and such), she finally had surgery to repair the VSD and the PDA. They left the ASD open to serve as a pop-off valve for if the pressures in her heart got too high. The rest of the stuff we were just going to monitor. It took a while for her to leave the hospital after that. In fact, the total length of stay was 46 days.

She came home on a feeding tube, and she would keep that in until she was 2 years old.

After that it was a lot of follow up cardiology visits and there was more and more talk about the high blood pressure in her heart and lungs.  Finally it was determined that we needed to start seeing a pulmonary hypertension specialist. That is the first time I really remember being introduced to the term, and getting the message that it might actually be a big deal.

So I did that thing that all doctors wish you wouldn’t do. I googled. I had to do it. I needed to start educating myself. What I found terrified me.

I learned that Pulmonary Arterial Hypertension (the type L had – which is more specific than just Pulmonary Hypertension) was considered a terminal illness. That there wasn’t a cure, and there were very few treatments. That there were no treatments approved for use in children.

Then it got a little more specific (and I’ll go into more details at another time) and eventually I learned that L had Idiopathic Pulmonary Arterial Hypertension . Idiopathic means “unknown cause”.

Life was, understandably, a bit distressing from there on out as we learned to deal with her illness, and began the battle to find the line between wanting to protect her, and wanting to preserve a high quality of life for her.

We managed, though. And eventually we decided to have G. Our thought processes were that surely we couldn’t get this screwed a second time. Maybe something would go right for once and we’d have a healthy baby. After all, as far as we knew, L was… just extremely unlucky.

Uhm.. not so much.

G was born on April 6th, 2013 and the next chapter in our journey began to unfold.