Parenthood

Last night I heard from someone I truly care about but haven't spoken with in years. I was asked the generic "how are you" question and for once I tried to answer honestly.

I had to really think about it. Sean and I haven't had the time, energy, or emotional capacity to spare to really consider ourselves in the last few months. I guess I can't truly answer for him but I am fairly confident he'll agree with me.

Obviously we aren't fine; we have a critically ill child. I overheard a nursing report the other day when Avelyn had to be reintubated. The day shift nurse discribed me as worried and in her room all the time. She said it with a negative connotation that I don't feel was appropriate. Of course the nurse doesn't have children of her own. Even so, to work in this setting she has to compartmentalize her emotions and she likely doesn't realize she does it. I get it. I compartmentalize my work too, it's the only way you can cope with such heartbreaking work day in and day out. 

However, I try not to judge the family members of my patients and think all nurses should do the same. Everyone copes and handles crisis differently. Yes, I am nervous; I should be. My baby may not make it out of this hospital. Any parent in my shoes should be nervous. Yes, I rarely leave her side. Why should I? Being away and knowing she's struggling is harder than being present for it. While I believe more than anyone Avelyn will turn around, do well, and live a long happy life- I also know she is very very sick. There is a chance that this may be the only time I get with her. I don't ever want to look back and think I could've spent more time with her or that I missed something. 

So how am I? I am not ok. I am not fine. I struggle to make it through each day-sometimes each hour. Yet our struggle as parents pales in comparison to our baby girl's struggle. So we can't afford to fall apart. We can't afford to not handle the stress and challenges. We can't afford to lose hope. Therefore we won't fall apart. We will cope with every challenge and stressor and we will never lose hope. 

Very few things in our lives are simple these days but this aspect of lives as parents of a critically ill child is. Our baby needs us. So we are here and will always be. There isn't another option. She deserves so much more than the life she was born into. We can't do much for her right now. We can't hold her to comfort her. We can't tell her it'll be better soon. We can't feed her or really even change her diaper without assistance. All we can do is give her is our stability. 

It's not the parenthood I hoped for or that I would wish upon anyone. But it's all we know and it's what our baby needs from us. So for now we take each day as it comes and we survive. Our copeing isn't graceful, we often struggle but we're here and we'll continue to be. We have to. As her condition improves her needs will evolve and so too will we. It's the least we can do. She's been through too much for us to not step up to the plate and help her meet her full potential. 

So, how are we? We're trying. We're struggling. But we're surviving.

Life

I feel like our life has become a constant state of bad news since we learned of Avelyn's congenital heart disease. Recently it's gotten worse. She has really decompensated over the last few days. She had blood in her stool Thursday and vomited up her medications. She was also more irritable and seemed to be experiencing more pain. Friday things continued to escalate through the morning. Her pain quickly reached an uncontrollable level. It seemed to be originating from intestinal ischemia or lack of blood flow to her intestines. She was unconsolable and had to receive multiple doses of morphine. Her work to breathe got out of hand and she had to be restarted on the positive pressure support nasal cannula. Her blood was acidotic from poor perfusion and working too hard to breathe. By late morning it became clear that she needed to be reintubated. 

All these acute changes were initially attributed to an intestinal infection or again possibly NEC.

Through the day she continued to struggle even while ventilated. Her CO2 levels remained high while her O2 levels were low and she stayed acidotic no matter what was tried. She also continued to be pretty uncomfortable. 

She had a fever through the night and into the morning, spiking at nearly 103 with Tylenol on board. While it's improved some today she is still somewhat febrile. Today she was also uncomfortable and it's been difficult to control her pain without bottoming her blood pressure out or over loading her with fluid. The docs also tried to get an arterial line without success. (3 hours and 15+ sticks later I don't want to go into my feelings about that).

This morning her X-rays continued to be clear of any signs of NEC, blockage or pneumonia. Her lungs look pretty clear but cultures of sputum were sent just in case. She also had blood and urine cultures sent yesterday and today. So far nothing has grown but it takes up to five days for possible bacterial growth to present itself. 

She had an echocardiogram this morning too. The results aren't good. She looks to have pericarditis or an infection in her heart-one of her tricuspid valve leaflets. The damage is severe. She'll need surgical repair or replacement of the valve and soon. We don't know if the surgeon can repair it on top of the other surgical repairs she needs. This might mean an additional open heart surgery in her near future. However the risk of taking her back with an active infection are very high. However she is only declining at this point. We seem to really be in a tough place. We don't know what will happen over the next week. One thing is clear, she'll need to be on very powerful antibiotics for a couple months after her surgery and will need to remain hospitalized for the duration.  So our time here will likely stretch into the spring. We're so ready to take our Avelyn home and let her be a baby. She needs to catch a break. She's been through so very much and has endured so much pain. She's never known anything but pain, fatigue, suffering, sedation and conscious altering opiates. We're doing our best to stay positive but when the doc apologizes for "only giving bad news for a while now" it gets more difficult by the day. Please keep Avelyn in your thoughts. I'll try to do better about blogging more regularly. 

Living on the Edge

Avelyn has been struggling with withdrawals all weekend and continues to today. I see the symptoms on a daily basis at work but seeing each in my month old baby strikes a nerve. It's sad to watch someone so little go through withdrawals-to see her heart race; the sweat bead down her face; her breaths so fast and shallow; and the twitching of her little limbs. She's restless and irritable whenever her detoxing meds aren't covering her symptoms. Due to her anantomy and physiology her blood pressure naturally runs quite low-often times too low. This complicates what can be given for withdrawals because all the medications cause decreased blood pressure or hypotension to some extent. To combat the impact of the detoxing medication she has gotten blood, fluid and increased dosages of epinephrine. For now it seems to be working. 

Friday was a rough detoxing day as well as in general for Avie. Her perfusion to her intestines wasn't the best throughout the day due to the withdrawals. That evening she had a grossly bloody stool, which is indictive of not processing her feeds well and sets her up on the edge for future surgery. 

If she can't process feeds and grow she needs her unifocalization surgery sooner rather than later. Over the weekend the attending was suggesting the blood in her stool again would likely be the final straw that sent her to surgery. However new days bring new attendings a with new treatment plans and ideas. The current attending just started her back on feeds after another 48 hour NEC rule out. We still haven't heard from Avelyn's surgeon yet and may not until Thursday, unless something significant comes up. In the mean time we are trying to keep Avie as comfortable and relaxed as possible-which isn't easy given she's going through withdrawals. If she gets upset her body will naturally shunt more blood to her lungs and not her brain or periphery. Meaning she's at risk for decreased gut purfusion and NEC as well as another stroke. 

It's not an easy line to walk. We want her to grow and be as large as possible to give her the best chance at a successful repair but we risk her having another lactic acidosis episode, NEC, or stroke with each day we wait. Needless to say life is stressful. I doubt any parents have ever been more attuned to their child's cries as we are given these circumstances. 

To make matters worse I am nearing the end of my leave at work. Currently I am able to calm her more quickly and easier than anyone else. I guess all the time she spent in utero she became accoustumed to my voice and lack of rhymic movement-poor child lol. But anyway considering the potential consequences of her not settling-I rarely leave her room. It's often just not worth the risk. Granted some days, when she's doing quite well I'll venture out for a couple hours but even then it's never any longer. I can't even begin to consider going back to work now and being 5 hours away from her. Hopefully she'll make amazing headway in the coming weeks. In the mean time we'll be looking into other options. 

Sean and I are blessed that we've be able to be with her as long as we have. Many of these kids often have no one at their bedside. The nurses here are wonderful but can't attend to every cry every time. It hurts my heart to know any cries go unanswered but especially those of such sweet fragile little babies. 

CHD is Endured

A lot has happened since my last update. Most tragically a sweet little heart warrior named Skylar passed. She was born a couple days prior to Avelyn and was a true beauty. While we haven't really grown close to any of the other families here at Vanderbilt, we saw Skylar's sweet parents often in passing and traded updates about our girls. Skylar's passing has had me thinking a lot of how these kids battle each day for life and how they are often described as losing their fight when they don't make it. 

I think this sends the wrong message. Little Skylar and all the other kids who have passed with CHD have not lost their fights. To suggest such belittles what these kids have been through. CHD is not something to be beaten or won. It is a battle that must be endured. Some kids are born with more complex defects than others and must endure more if they are to stay with us. Sometimes it's just too must to ask a little body to endure. To suggest these kids lost their battle suggests there was something they could've done differently or that they could've fought harder. If I make anything clear I hope it's this, these babies can't fight any harder than they already are. They're the purest among us, no alternative motives exist for these sweet babies. For each and every one of them the fight for life is all they know. Each needs to be remembered and respected for such. Even survivors will forever live with the effects of CHD. There's no cure and surgeries don't completely "fix" defects.

Each child who passes from CHD is a true loss, to their families, communities, and society. The future they would've had is a true loss. The lack of research for CHD is a true loss. How can these kids lose a fight that cannot be beaten. No, each is a little wonder all hit/her own. They just had the misfortune to be born in painful circumstance. 

I stole the photo below from Skylar's sweet momma.  While it doesn't reflect our children's struggle with CHD if definitely reflects ours.

Grow Baby Grow!

The last week has been a roller coaster. Our girl had her heart cath and we were told that she needed to undergo a "unifocalization" procedure to bring the MAPCAs into her left pulmonary artery sooner rather than later. Sean and I expressed doubt, as its well known that kiddo's do better when they are larger. We trust the surgeon though and last week he seemed to think it was best to go forward with the surgery. It was a major blow. Sean and I were both terrified especially given her small size. 

As the weekend played out the blows kept coming. Avelyn failed extubation on Saturday, and when I say failed extubation-I mean she didn't make it five minutes. We're still not positive as to why but it was likely a combination of factors. These being atelectasis, pulmonary edema and thick mucous plugs dislodging and blocking her airway.  

Sunday brought it's own hard knocks. We learned that at some point in the last month our sweet girl has suffered an ischemic stroke. She has two small infarcts on her basal ganglia. Supposedly the infarcts are small enough and she is young enough that neither should affect her. However we won't know for sure until much later in her life due to the basal ganglia contributing to procedural/habitual knowledge and fine motor skills. These are things babies obviously won't express anyway. So that was a hard pill to swallow and makes our fear of her decompensation events that much worse. What if the next episode is worse and she suffers a more severe stroke? 

Moving into the Monday we learned the PCICU filled up so all the scheduled surgeries for Tuesday had to be canceled because there wasn't a PCICU bed to put the post op kids in. Given Avelyn already has a PCICU bed, moving her surgery up a day was tossed about. 

Then out of nowhere, Monday afternoon her surgeon switched gears and decided to give her one last shot to grow before attempting the unifocalization. Sean and I were thrilled. I literally felt exhilarated, sometimes you just know things will get better. Granted she was still intubated at this point but I felt a peace and knowing come over me in that things would start to turn around for our baby. Sometimes God's sends us signs that are just much more obvious than others, the canceling of her surgery felt like one of those obvious signs that I had been praying for.

Since then she's been doing better. She was extubated yesterday and has tolerated that well. 

Unfortunately it isn't all smooth sailing. Avelyn is suffering from withdrawal symptoms from her sedative medication that she needed while she was intubated. She has been tachycardic, restless and overall acting like a little jitter bug. For once I felt within my nursing element seeing the withdrawal signs. I may not know much within the ICU nursing realm, but I know psych nursing. I recognized the detox symptoms from the get go but again had to advocate my concerns to the team. It turns out that she must be pretty sensitive to already have detox symptoms because she was only on the precedex five days. It usually takes kiddo's a week to develop a physical dependence. Her providers seem to be doing better about listening to me and Sean's concerns. I guess if you call it right enough you get a bit of credit around here. 

Anyway so that's been our major concern today. I never thought in all my life that my acute psychiatric nursing skills would be put to use on my month old child. Life can have a sick sense of humor sometimes.

Despite the challenge of detoxing our sweet little baby we are so thankful that she is extubated, doing well and not currently staring down the barrel of an open heart surgery. If we can buy her a couple months she'll be in such a better place physically/anatomically to handle her upcoming surgeries. 

Please pray she continues to do well and grows like a champ! 

And the Blows Keep Coming

Avie's cardiac cath ended up being  purely diagnostic. She has two arteries coming off her aorta and going to her left lung that shouldn't be there (MAPCAs). However she needs the blood flow they provide so her next surgery will also have to include removing those from the aorta and grafting them to the left pulmonary artery. Finding and grafting these arteries is very tedious and time consuming, it makes for a much more complicated surgery. The one bit of good news is left pulmonary artery looks to have maybe grown a bit-which means the shunt is working in that respect.

The two MAPCAs coming off the aorta are stealing blood that's supposed to be perfusing the body, instead it's going back to the lungs or "over-circulating". It isn't good news to have these MAPCA's but it was expected that she had at least one, we're glad it's not more.

She's got a long road ahead of her, she's pretty sick. The MAPCAs are what's causing her to be so unstable right now. Avelyn's cardiac surgeon believes she won't stabile as long as she is in her current state-meaning  she needs another surgery before she can go home. He doesn't feel confident in her ability to truly stabilize without the danger of having another episode at home. The surgery would be next week and would likely be just finding and connecting the MAPCAs to her left pulmonary artery. Which has had some growth. He may be able to place the conduit to creat a right vebtricle outflow tract but probably not. He definitely won't be able to close the hole in her heart between her ventricles. That's because there's no guarantee the MAPCAs will survive the surgery let along grow afterwards, so the VSD would serve as a sort of pressure release pathway if they didn't. This means another surgery would be needed later in the future. We wouldn't know when until seeing how she does. 

Obviously Sean and I are having trouble taking all this in. Avelyn is having trouble recovering from her heart cath procedure now, or at least is recovering as quickly as expected. However it's likely due to her specific anatomy working against her. Maybe she'll do better recovering from a surgery that would alter her anatomy theoretically to her benefit. While the doctors believe she can handle another surgery so soon, I can't help but question it. She's still so small. She hasn't even gained back up to birth weight yet. She's so fragile and decompensates so easily. Granted a lot of this is because of the MAPCAs which the surgery will be addressing. It's all so terrifying. How much can we put her little body through and expect her to recover from. I don't know but I think we're likely reaching that limit. She's not even a month old yet and will have been through two major open heart surgeries.

Yet we don't seem to have a choice.  We can't risk taking her home in her current state. She's a prisoner here until we can help in some way. Is this the right path. I don't yet know but our hands are tied. All we can do is pray it is and that she'll finally get some relief and stability. We trust her surgeon whole heartedly but our faith is surely being tested.

Please pray Avelyn finds relief and peace. Pray for our and her medical team's guidance. Pray her surgeon can locate and incorporate the MAPCAs well. Pray for his steadiness of hand and clear mind. Pray for Avie's little body to heal and for the MAPCAs to grow after incorporation. 

I have faith that these are all possible outcomes for our sweet baby. We appreciate the prayers and support already lifting up our little Avelyn more than I could ever describe but please don't stop now.  Much love and blessings to you all.

Handoffs

I just handed my child of to surgeons once again. It's only a heart cath this time and I thought that would make it easier. It doesn't. She started to cry as I let her go and she was wheeled from the room. What do you do when you can't protect your child? She doesn't understand why I let her go. She doesn't understand why she can't be picked up and soothed. She doesn't understand why she is woken every couple hours. She doesn't understand why she is in pain. She doesn't understand why she can't catch her breath. The struggle is all she knows.

I can't make it better. Sean and I would do anything to ease her pain and suffering but we can't. She alone has to fight this war if she is live. We can't do it for her even though we would in a heart beat. It's not fair. It's not right. 

She deserves the life of every other baby on your news feed. She deserves peaceful sleep. She deserves comfort and love when she's scared, tired, in pain, or hungry. She deserves reassurance that her situation often prevents us from providing. She deserves a full belly, not to be  hungry and without food for half her life. She deserves vitality and strength not lethargy and stagnant growth. She deserves her biggest annoyance to be a diaper change not multiple surgical interventions and recoveries. 

Her short life has brought mine into perspective. I am sure all parents feel that way. Yet most can't really value health until it's absent. I see and hear so many complaints and worries that seem so insignificant.

So your kid made a mess while playing and expanding his/her cognitive abilities. So you're sleepy because your baby was up to eat every three hours. So your kid is a picky. So you can't get anything done for holding your baby. 

I would love to be shower-less, sleepless, at my messy home covered in spit up and have my girl in my lap breathing easy with a full belly. Instead my girl has never been outside of the hospital, she fights for breath, is on total parental nutrition because she can't tolerate taking food by mouth, and we're in constant fear she'll decompensate and end up being reintubated.

And what's more sad is a lot of babies here have it worse-some have it much worse. It's so hard to look into the faces of the parents who's kids are sicker than mine. What's there to say? They may never get the mundane moments at home we all long for. They may never send their babies to kindergarten or see them run and play. While so many parents long for excitement, we pray for the mundane. We pray for peace. Our daily wish often is just the hope of being able to hold and rock our babies. It doesn't seem like much to ask but in these circumstances it is a mighty blessing. 10ml of milk by mouth is a blessing. Diapers without blood are blessings. The sound of our babies crying is a blessing. I know that sounds crazy. But until you've watched the silent struggle of your child trying to cry while ventilated-you just can't understand. 

In the last four weeks I have learned that often times our blessings come in such tiny packages they are easily overlooked. Therefore you have to dig to get past all ridiculously superficial and materialistic fluff to find true blessings. Blessings of health, love, comfort, reassurance, peace, and the joy of just being with the ones you love. 

I encourage everyone reading this to take the time to dig. Don't wait until you're forced into a handoff off to appreciate th mundane moments. Don't take small blessings for granted. Love earnestly and wholely. It's all that really matters.