Cath=Glenn surgery time

Maddie's cath went REALLY WELL this morning. I did a lot of worrying and borrowing trouble for nothing! We gave her to the surgical nurses at 7:45 and were paged at 9:00, so it didn't last long. Dr. Jones came in to talk to us shortly after and was smiling as he came through our door (always a good sign.) His first words were "Well, she did just fine," what a relief. From her cath, it was concluded that she needs surgery, the Glenn, which is the next phase in the 3-stage surgeries. Her pulmonary artery looks small in one part, the middle where it's attached to the shunt but the ends that go out to the body look normal. That was such good news to hear. Her shunt also looked very good so no ballooning was needed there either. Basically it was just an "investigative" cath, taking pictures in order to make preparations for her Glenn surgery. Now we're waiting to hear when she'll be scheduled. We're told it could be this week or in a couple weeks...she's not urgent, and Dr. Jones did say waiting a month or more isn't going to present any new changes or help. She's not going to gain that much more weight in that amount of time, so she's ready for the Glenn anytime. His last words to us were the best..."this is what I'd hoped for in Madison's case." What a beautiful phrase to hear, brought us much HOPE. God is good. Again, He's shown us His love, mercy and grace with our little Maddie. Here's the pictures we were given of Maddie's cath this morning:

This is the pulmonary artery (long tube running horizontal) and the narrowing is just in the middle where it connects with her shunt (skinny tube running vertical.) The sides of the pulmonary artery are larger (kind of looks like an ant) which are good and of normal range. The skinny loopy things are the catheter wires strung up through her heart.

A quick sketch of the Bi-Directional Glenn that Maddie will have next, hooking the superior vena cava to the pulmonary artery.

Maddie's right ventricle. It's very small in comparison to the left. In a normal heart, these are relatively the same size or are in proportion to each other.

Her left ventricle- enlarged since it has to compensate for her right side not functioning.


Since starting this post, Maddie arrived. She looks great and is ready to eat since she was cut off of feeds since midnight. Her little voice is hoarse from being intubated and she's coughing some but other than that, she looks beautiful! Back to her cute self I'd say!!!

Pre-Cath Day

Maddie loved the balloon and giraffe stuffed toy she received today from her heart buddy Teagan and her sweet family...very fitting since we're on the giraffe floor!

Sucking her thumb...a new favorite!

Feet are so fun to kick!

Cords keep me preoccupied too!

Maddie had an echo this morning and consent forms have been signed for tomorrow's cath procedure. She's the first case, 7:30 am with our fave, Dr. Jones. Anesthesia team will be in to take her around 7 am. She's had a pretty good day, just not too energetic about feeding orally. I've been having to gavage more through her NG tube than she can take orally, which is a disappointment after having months of good feeding. This has slowly been coming on, just proves that she needs some "fixing" in order to eat well again. Apparently with oxygen deprived babies, they give up some of the easiest things first, for Maddie, this is her feeding. Also, when you lack oxygen, you feel really crummy, so eating is the last thing you feel like doing. She's also been working a lot harder (her breathing)and I see more signs of her being uncomfortable and irritable, but do you blame her? Being in a hospital with a nose tube, cannula, 3 chest leads and a pulse oximeter attached is not so fun. I'm relieved we're here in the hospital though and just hope that we timed this about right...staying at home any longer may have presented a very scary experience. She's been weaned down to .5 liters of oxygen and they just want to leave her at that to keep her comfortable. She's been a real trooper, still smiling now and then and she's been kicking up a storm, which is so cute.

The plan for tomorrow's cath is to see whether they can buy her some more time before the Glenn by ballooning or stenting her shunt or pulmonary artery open. From the echo, it appears that the pulmonary artery they attach for the Glenn looks like it's on the small side. The doctors' words were "we're disappointed it didn't grow more". Since arriving at Children's on Saturday night, we've also learned that she has collateral arteries which could have attributed to this problem. The problem with collaterals as I understand it, is they steal the blood supply that should go to the pulmonary artery. It was Maddie's natural way in utero to help compensate for her closed pulmonary valve: the blood created its own way to try to get to the lungs. Complicated to understand, but to sum it up, the ones Maddie has are not good to have. So if her pulmonary artery looks like it's adequate size for the Glenn, I believe they'll just go ahead and do the surgery soon. So in the cath, they'll be able to get a close look at what her shunt looks like, get better pictures of her pulmonary artery as well as measure the pressures in the heart. We'll know after her cath what the plan is. So they'll have to make a "game time decision" tomorrow during the cath. On a lighter note, she had a good weight gain since being admitted...up to 5.41 kilos (12 lbs.) so she's hit the minimum although I've been told the bigger, the better for the surgery. We spent lots of time playing today...she loves grabbing anything now and tries to mouth it.

After hearing today's news from her echo, I'm stressed. Hearing that her pulmonary artery may be too small is scary. When I look at Maddie and reflect on what a great 4months she's had, I'm in disbelief of her complex heart. But when the doctors talk to us about it, reality hits. Of course they LOVE talking about the complexity of these babies hearts, it intrigues them, drives them, it's their passion. But it's my baby that has this problem. I've learned that her journey is only getting more difficult for me as she gets older. She is such a beautiful little soul and I can't imagine life without her in it. When they have you sign consent forms, they rattle off all of the risks: stroke, bleeding, blood clots, death, after that word, I just hear "blah, blah, blah." It's tough to hear when we don't have any options for our daughter. Again, I find myself in a dichotomy: I want my daughter to live yet in order for her to do that, she has to endure pain and suffering which I don't want to inflict on her. So I pray tonight that I will feel His presence more and I'll be able to renew my faith in the Lord's plan for Maddie. Again, I have to hand her over.

Please pray for a safe and successful cath for her tomorrow and that the doctors will be able to make the best decisions for our daughter's heart's future. Pray that Maddie will do wonderful through her cath procedure and will be able to come off of the ventilator right away. And please pray that we will have the strength to endure the day and what it will bring.

We're here...early

Yes, Maddie's in the hospital. Yesterday after her refusal to eat, extreme sleepiness and her dusky look, I called her Cardiologist and the decision was made to get her over here. So Bryan and I drove over to Seattle to Children's ER. Her sats were 60 when we got here then hung in the upper 60's to low 70's. Maddie's color was very poor and she had a severe mood change- FUSSY! I've been lucky with my laid back baby girl, so this was a signal that things were not quite right. Over the last week she'd really tapered off of her eating and was having more low sat episodes. I'm thinking our last ER visit was the brink of what we're seeing now. So she's been fighting this off and on for 2 weeks.

Many tests were done last night (urine, blood, chest x-ray) that all came back fine. A culture was also grown to rule out any of the "5 bad viruses" and that also came back negative. She'd been sneezing and coughing but nothing has really manifested from it. Overall, little sis just doesn't feel well. So she's on 0.8 liters of oxygen with a nose cannula to help bring up her sats and that good ol' NG tube was put back for feeding after 6, yes 6, attempts (3 different nurses, even the "big dog") at getting an IV in her arms/legs failed to help get her hydrated. It was horrible to watch!!! I've never seen a baby cry harder and consoling did not work. We've now learned that Maddie has really crummy veins that blow every time they'd get the IV threaded in. We were admitted to the floor and the perk of our stay has been we're in isolation since she could be contagious with her teeny cough and sneezing. So we have our own room: G4001. I'm so happy about this part.

An echo has been scheduled for tomorrow morning and we are in the books for her cath on Tuesday morning. If she's not feeling well though, I don't think they'd do the cath. It could be a minor virus that's causing this or a heart problem. Hopefully the echo can show if it's heart related and the cath will be the best way to see what's going on. So we're playing the waiting game.

On another note, Maddie is 4 months old today! Not so fun spending her 4 mo. birthday in the hospital, poor baby. She's looking a little beat up too... She did gain pretty well, even with her sporadic feedings the last few days, she's up to 11 lbs. 10 oz. which 5 kilos is the minimum weight they like them for the Glenn and she's hit that. So if she has to have surgery sooner than we thought, I think she'll handle it fine. We just need to get her healthy and feeling good again. Keep our sweetie in your prayers. We'll keep you posted.

The ball is rolling...

After talking with our doctor in Seattle a few times this week, it was decided this afternoon that Maddie will have her Pre-Glenn Catheterization done next week. We had a cardiology appt. scheduled for next Wednesday but Dr. Seslar (and we) believes that Maddie's behavior and sats show that she's ready for her cath procedure and she's just telling us that surgery time is close. A cardiac catheterization procedure is done prior to surgery to help the doctors determine the best way to treat more complex conditions. The information gathered during the cath is used to figure out timing for surgery or to plan for the operation itself. In Maddie's case, the doctors will be able to clearly see her shunt and how it's functioning, as well as the rest of her heart. From this, the shunt may need some tweaking which would hopefully buy us some more time before surgery by bringing her sats up as well as her energy level, eating, and her color. They would do this with a balloon procedure in the cath lab. If no tweaking is necessary then she's clearly outgrown her shunt and ready for the Glenn. So scheduling will be calling first thing Monday morning with our date, time and details. Looks like the ball is rolling now!

FAQ'S about Maddie's Heart

Thought I'd answer some questions that I get asked frequently about Maddie and her heart condition. Plus this is a good way for me to remember this part in future years.

*What does Maddie exactly have?
Maddie's CHD diagnosis (Pulmonary Atresia) has affected the right side of her heart. Because her pulmonary valve does not open (she does have one), blood can not get to her lungs to be oxygenated. Because of this, her right ventricle is small, hard and the muscle that surrounds it is thick. It does not pump efficiently since the blood has no where to go but back up to her right atrium, where it just came from. Here's a picture of what Maddie's heart looks like.

Her right ventricle also receives little blood flow to it because her tricuspid valve, the one between the right atrium and right ventricle, is malformed as well. This malformation is another CHD called Ebsteins Anomaly. So it doesn't work properly and the blood just flows between those chambers freely. Amazingly enough though, her pulmonary artery is of normal size which leads our doctors to believe that at one time, in utero, her pulmonary valve worked and blood pumped through there in order for that to develop. She also has an Atrial Septal Defect, ASD, which is a hole between her right and left atrium. This is actually helping her right now, although the blood is mixed which causes her cynosis (blueness). She also had a Patent Ductus Arteriosus (PDA) that allowed the blood to mix between her pulmonary artery and aorta. When her shunt was put in, this was closed in surgery. Lastly, she is classified as a Hypoplastic Right Heart Syndrome (HRHS) baby because the right sided structures of her heart are underdeveloped. It's easiest for me to just describe her as HRHS. So the clinical diagnosis to describe Maddie's heart condition is: Pulmonary Atresia with IVS, Intact Ventricular Septum, meaning she does not have a hole between her ventricles or a VSD. BTW, Maddie's left side of her heart is good and will function normally (as it was made for) and this is a blessing in her case.

I've learned that her defects go hand in hand and in some way are all present because of a domino effect. We can't tell which caused what or really even know if one caused the other, but it's hard to believe that they're all present without some sort of relationship. In the range of right sided defects, Maddie's is a major problem. She is considered in the complex heart defects category and is on the severe end of the spectrum when talking about defects. I found that her particular diagnosis is 3% of congenital heart defects. There are over 35 known congenital defects and they're present in about 1% of live births. So Maddie's is very rare, which makes her that more unique! Her nurses' recording sheet at the doctors office just says "Complex CHD" in the diagnosis box, since there are too many to list...but from what I've researched, this is "normal" when you're in the severe range of heart defects.

*How can her heart defect be treated?
When we received her diagnosis, we were given 3 options: terminate the pregnancy, comfort care (take Maddie home after birth and let her pass away) or a series of operations. I don't recall this conversation with our doctor since we had just gotten the official news of our baby girl's heart and I was completely overwhelmed with so many emotions, I totally blocked this part out. We did quickly learn that her heart defect is fatal without treatment and she would only live a few hours or days after birth without treatment. So, we opted for the open-heart surgeries. These are done in stages and Maddie's passed stage 1. She has the B-T Shunt, a bypass to get blood to her lungs since it can't flow normally through the right side of her heart to get there. The next surgery (4-6 months of age) is called the Bi-Directional Glenn. In the Glenn, the superior vena cava (major vein from the systemic circulation to head/upper body) is connected to the right pulmonary artery. This reduces the workload of the heart since it only has to pump blood to the body, not the lungs anymore. Now blood just flows to the lungs instead of being pumped. Her shunt will be taken down in this surgery. The last stage of surgery is called the Fontan procedure. This is done between age 2-5 and it reroutes blood flow in the inferior vena cava to the pulmonary artery (plumbs in the blood that goes to her body/organs). This would be the last repair. Her defect isn't correctable...but is palliative. When I looked up what this meant, I was down for days. Here's the definition: Palliative care is any form of medical care or treatment that concentrates on reducing the severity of disease symptoms, rather than halting or delaying progression of the disease itself or providing a cure. The goal is to prevent and relieve suffering and to improve quality of life for people facing serious, complex illness. Wow, I know. It's tough to read this. I always tell people she's in for 3 surgeries but there could be more, just depends on whether Maddie's heart likes it or not. Our last option if these surgeries don't work is a heart transplant. I don't even go there. Getting a transplant is not considered "easier" than the surgeries, believe it or not. Having a transplant is a very complicated thing...tons of meds, uncertainty if the heart will be rejected and a transplant lasts only 10-15 years then another one is needed. Yes, it sounds great "just get a new heart" but it's not. It would be the last resort. So Maddie is on the single-ventricle route of surgeries with her half of heart!

*What is Maddie's long-term outlook after repair?
Of course none of us know our life-expectancy but when you have a child with a critical condition, you look at this more closely. The statistics that are out there are new, since the 1970's. The surgical procedure she's having has only been around since then and has been "revamped" as well over time. The oldest living survivor with her same 3-stage repair is 31 years old and he is a Hypoplastic Left Heart Syndrome diagnosis. I have never found anyone with Maddie's same defect yet. So her "repair" is still fairly new in the medical world. She will be followed her entire life by a cardiologist and will be part of the statistics. I do have faith that with medical advancements, there will be a better way to someday fix her heart for long-term. Our doctor gave us the "assurance" that there should be no reason that she can't live a "normal" life. This always makes me laugh because what is "normal"? Of course, I'm elated to hear this. I do know that with Maddie's heart condition, she will lack physical endurance and will tire easily. Our cardiologist said that she probably won't be the fastest kid on the playground. Little does she know, her mother wasn't either!!! One heart mom friend I've talked to has a little boy with the same repair surgeries as Maddie. She can't imagine what her 1 year old little guy would be like if he had a whole heart! He's a go-getter and she can hardly keep up with him!!! Since Maddie has defied so many odds already, I do believe she may fit in that "go-getter" category. Plus she'll have two big brothers to keep up with. So, I focus on the positives of her...not so much her defect. SHE has proven to us already that she is a fighter, she's tough and the heart that she was born with has HEART. It may not be structurally correct, it may be complex, but Maddie's will and her HEART has been remarkable! I thank God everyday for the heart that he gave my baby girl.

So excited to swing this morning!!!

Better Days

Just an update that Maddie is feeling much better. These last couple of days have been good. Her sats are running normal for her again and she's making progress with her appetite each day. Her symptoms are hardly visible! She's been discovering lots of new sounds that she can make...so close to giggling as well (with the help of tickling her under her chin). It's so fun watching her new tricks!

Feeling Yucky

Maddie has officially caught the bug that her big brother Caden has had. I'm thinking its a viral thing: fatigue, coughing, hoarseness, stuffy head (no drainage yet)and sneezing. Cade has ran a fever with it but Maddie hasn't shown this symptom yet. This explains her past few days of ups and downs. After talking with the doctor and the cardiac nurse, Karen, over the last couple of days, they are not concerned with Maddie's heart. She's running pretty low sats (68-74), she's much duskier in color, so I've been putting the blow-by oxygen on her which has helped them come up and be more stable. They commented that if she doesn't get over this within a week then we may need to get her over there. She had no appetite yesterday, which was scary and she was completely out of it. Today she's eating much better and she even likes some Pedialyte in her bottle. The thickness of her bottles is probably not the tastiest right now so this helps that out. My mom, mother-in-law, and sister-in-law helped out and took the boys yesterday and overnight so I could get some much needed sleep (when it was possible!)

I do think I'm going a bit delirious... I seriously thought the pulse oximeter screen was talking to me last night. My nerves and anxiety are completely shot. It's so hard watching our children when they're sick and even more difficult when your little one is already taxed! I worry about her heart function enough as it is and since this is her first time getting sick, I'm just on-edge of what to look out for. We do know she's a fighter and should whip this in a couple of days. So please keep our little Maddie in your prayers to beat this "summer complaint" bug.