Category Archives: Thoughts

Transplant Surgery Updates

Below are the updates we received during the night as our baby slept on the operating table. I apologize for not posting them here earlier but the blog is not a very effective tool for real time information.

9:30PM – Maddie is on bypass and the heart is in the room.

10:30 – The heart is in and beating. Please send a prayer of thanks, healing, and strength on behalf of the donor family.

11:07PM – The transplant is complete. That sweet, miraculous heart is beating strong in our precious baby girl. The doctors are pleased with what they see. They have some additional monitoring and wrap up to do before we can see her but our spirits are lifted knowing that the surgery is over. We are so incredibly grateful. Your prayers, every single one, carried us through these long horrible hours. It is only by the constant companionship of the spirit of God that we could pass through this trial. And it is only through His guiding hand that we will go forward still. Thank you all for your unending support. More updates to come.

Heart Failure

WaitingWe returned to the hospital on Sunday. In short, Maddie’s heart is failing.

After being discharged from the hospital last week, spending Thanksgiving with family, and starting a new regimen of heart medication and diuretics, we hoped that she would improve enough without further intervention. Headaches and poor eating by the end of the week led us to contact her cardiologist. Our hopes were dashed when she said we’d need to take her in to the ICU. Another echocardiogram confirmed the devastating truth that her heart function continues to remain poor. In fact, we can assume now that it will never be as good as it was last spring.

So here we are, on the same Cardiac unit where she spent the first few weeks of recovery after her Fontan Surgery, only this time there are so many unknowns.

It’s been a few days now and though I’ve intended to sit down and share this news with you, I just couldn’t. The reality of the situation has been too hard to face.

Here is what we know right now. The IV medication (Milrinone) she’s been receiving continuously has shown marked improvement in her well-being, overall. She is eating, sleeping, and running around (literally, whenever she can, including riding another tricycle, cords and all) better than she has in a long time. This “cardiac tune-up,” as they fondly call it, is making a difference in her pumping function, just as they hoped, allowing her other mechanisms to relax a bit. But these are only clinical improvements. This medication is not a long term solution. It’s only enough to give her cardiac system a break.

The reality is her heart is just tired. Her special little single ventricle heart was never meant to work this hard. It may have accomplished all that it can do at this point. Her cardiac team is beginning to sense that now. We’ve met with the Heart Failure Specialist doctors already. The reality is that Maddie may be facing a heart transplant in the near future.

There are some Plans A and B that we are clinging to before we truly commit to this Plan C.

Plan A: she continues on this milrinone IV drip for the next 5 days, giving her heart more rest, seeing that she is truly improving , before they begin slowly weaning her off to be replaced with strong heart failure meds she can take at home. A three week stay to assure the plan has worked and then we resume our “normal” lives and live it up until we see a decline again. Hopefully not for awhile. This is what we pray for continually.

Plan B: She responds well to the IV milrinone, but not well enough. We spend a bit more time in the ICU and get trained in how to administer the drug at home. She caries it around continuously in a little pouch and we become more intensively involved in her medical care. She would probably be visiting the hospital more frequently, as well. Life, as we knew it would change.

Plan C: she doesn’t respond well to weaning off milrinone or to the new heart failure medication. We’d then begin the long, painful (emotional and possibly physical) process of heart transplant evaluation with no guarantee that it will be successful at any stage of the process.

Reading the cold, hard facts must be shocking and difficult. I apologize. I needed to write it down. Make it official. It’s the only way that I can prepare myself for what is ahead. It’s only fair to be direct so that you know what we’re facing.

Plus, we need your prayers. Oh, how we need your prayers of healing, but more importantly, of comfort and support. We do not know what will happen.

We started this journey almost 6 years ago with a tiny, fragile baby who had no family. Her prognosis then was foggy at best. Her story was presented to us and we immediately knew in our hearts that she was special, even though our logic told us it was crazy to take a child with such severe problems. We weighed it out in our minds. We fasted and prayed. How could we make this decision?

And then one night I was talking to Julianna, my pseudo-sister.

“How can I take this baby?,” I asked her. “How can I go forward with this when I don’t know what will happen? I don’t know if my heart can heal from that kind of loss. What if she dies? What if she dies right away?”

“Then you just love her. You just love her for as long as you have her,” she said through both of our tears.

And we have. And we will.

There is no doubt in our minds that Maddie was meant to be in our family. That answer to prayer back then continues to be a sweet blessing as we face each challenge. God knew that we’d be here, at this very moment, looking down a very difficult road. He knew we’d be okay then and I feel Him strengthening us each day, reminding us that we will be okay now.

And we will.

Maddie is hospitalized

Strong Maddie

When I was a teenager, I’d pour all my angst into my journal. Every crummy thing that transpired was recorded with detail. I had many happy moments and much to celebrate but those times would always get left unwritten because I was too busy enjoying the good. I didn’t mean it to be that way. It just became the best method for me to vent my frustration with life.

It seems that this family blog has met the same fate as my high school journal. I have many wonderful memories of our summer’s activities and autumn doings that I’ve meant to share but have been hard pressed to find a free moment to relay them. Instead, I’m writing today with bad news.

Maddie was hospitalized late Tuesday night. Bringing her into Children’s Hospital with suspicion of pneumonia turned into the discovery that she was experiencing heart failure. Her liver was extremely bloated (4 cm) and her body was under severe stress.

Let me back up. All four kids had been sick with a viral illness for several weeks. Maddie was actually the least affected one. Jake and Joe started antibiotics for sinus infections and Maddie started the same medication just to be on the safe side. Her complex anatomy and recent heart surgery keep us erring on the side of caution. Warren’s asthma was bad enough to visit a specialist who started him on heavier medications. They all completed their course of antibiotics, everyone seemed to be improving, and we moved on with our busy schedules.

It’s been fairly cold here recently. Some days were in the low 30’s. Little feisty Maddie often refused to wear proper gear when going outside. Her bluish lips didn’t seem that out of place as she stood at the school bus stop with minimal coverage.

When the third day passed with a progressively worsening cough, prolonged cyanotic symptoms (blue lips, etc.) even after the blankets were piled on, and decreased energy, we decided to take her back to the doctor. There we were told that her chest x-ray showed some fluid accumulation. It could be a pneumonia. We’d better go to Children’s Hospital’s ER for further testing.

Upon arrival, we learned that her INR level (used to measure the thickness of her blood while on Warfarin) was through the roof. Her normal range is 1 to 2, where she’s been since the Fontan surgery. The lower the number, the thicker the blood, the higher the number the thinner. Both ends of the spectrum have their problems, especially for Maddie’s circulatory system. That night she was at a 12. My jaw hit the floor.

Meanwhile, we’d watched her oxygen saturation levels lower on the monitor. They started her on oxygen and tested for bacterial and viral infections. Nothing was showing up and after a few hours of monitoring and a bit of sodium chloride to help her dehydration, they were considering letting us go home. When the ER doctor tried to wean her off the oxygen her vitals stopped looking good. At 2:30AM, she was admitted for closer observation.

Everything took a turn for the worse the next day. The doctors and medical team held “rounds” that morning and were still debating on the symptoms. It didn’t seem like pneumonia, though they continued new antibiotics just in case. An echocardiogram ordered for the afternoon would provide more information about her pulmonary veins, which are always problematic for her, and of course would see what was going on with her special little heart.

The results were like a blow to the head. She was experiencing heart failure. Because her heart muscles were not contracting properly, fluid was backing up causing increased pressure in her Fontan circuit and bloating to her liver. Nobody really had answers for why it was happening. She’s always a challenging cardiac puzzle to piece together.

Did the virus she contracted along with the rest of the kids become too taxing for her heart to handle, making her circulatory system weak? Or was she already experiencing failure slowly over time and we just had no idea?

It’s Friday (technically Saturday since I’ve finally gotten the emotional strength write this down at midnight), and we think that we’ve gotten things figured out as best as possible.

The most likely case is that she was already deteriorating. This virus was just too much for her to handle. The fact that finding her veins during blood draws for INR checks had become increasingly more difficult over the last couple of months may have been a sign we just didn’t recognize. The IV fluids she received probably pushed her over the edge, since pumping extra fluids directly into an already taxed blood stream only causes backup in the system. In other words, it was only a matter of time and we just didn’t know. At least it was sooner rather than later.

But things are looking up. Once they began draining the fluid with our old friend Lasix and starting her on Enalapril for her heart muscle contraction she’s been showing steady improvement.

In order for her to go home she needs to be completely off the oxygen, have little fluid in her lungs and liver, and show stable vitals: good heart rate and respiratory rate. They’re hopeful for the beginning of next week. We’re hopeful that it’s at least before Thanksgiving.

This is a set-back to her overall health but a manageable one once she’s stable. Her echocardiogram today showed improvement in pumping function but also a new issue with leakage in her tricuspid valve. We don’t know how that will resolve yet. At the very least she’ll be taking the Enalapril medication indefinitely.

We are exhausted. It was all so sudden and unexpected. Everyone, especially Warren, Jake, and Joe, have felt the emotional impact of this situation. They miss their sister incredibly. Maddie is holding up but would give anything to go home and be with her brothers. I could barely bring myself to write this story down because it’s so raw and lengthy and unbelievable. But as I was watching “Grey’s Anatomy” to zone out and realized that I understood about 95% of the medical jargon and procedures they were rattling off, I figured I was ready to share.

And I am ready to share. This has been a roller coaster ride with many unknowns but we have already witnessed miracles and felt the strength of endless prayers. You, our dear family and friends, have stepped in once again to provide support and encouragement, meals and babysitting, and so much faith and love.

One day I want to share with you the happy times that we lived this summer as we watch our fighter girl Maddie run and play like she always wanted to do but never had the energy for pre surgery, and post pictures of all the laughing we did as we had adventures and breathed in the summer air. But right now I will share with you our gratitude for our precious girl’s life, for our happy family that has unified under the strain of this trial, and give our unending thanks for the kindness that you continue to show to us.

We appreciate all of your prayers and good thoughts.

Fontan Recovery: Day 30

Hospital room

One month. We’ve been here one month.

It’s not completely shocking to still be at the hospital, but when we were so close to finally leaving, only to take a step backward in recovery, it has started to feel claustrophobic. Like we’re never going to escape.

Especially yesterday. Yesterday, Maddie had not just one but two tubes placed back in her chest cavity. They are smaller than the previous ones placed in during her Fontan— almost like straws– but they still hurt. They removed several hundred mL of fluid from her left pleural cavity, as well as some air from the right.

Going to the OR

She was scheduled for 2PM surgery but the cases before her ended up being more complex than they hoped, so she was pushed back until 5:30PM. That was 18 hours of fasting. No food, no water. She didn’t eat until 9PM. She kept telling herself throughout the day that when it was all over she would get to eat her dinner. Chicken noodle soup. That’s all she wanted.

Yesterday was painful in every way imaginable. Seemingly endless waiting filled with frustration and anxiety. Over and over she would repeat the steps they would take in surgery, to get it straight in her mind and cope with her fears.

“First, they’re going to put the mask on me?”

“Yes,” we would say.

“And then they’re going to do three things? They’re going to cut me, then they’re going to put the tubes in, and then they’re going to put the bandage on?” she’d whimper. “And they’re going to be quick?”

“Yes,” we’d reassure her.

“And I’m not going to feel them cut me because I’m going to be asleep?”

“Yes,” we soothed.

And then she would cry because, above all, she was terrified of the anesthesia mask. Calming her fear while stifling our own anxiety is thoroughly exhausting.

The procedure went well. They did what they needed to do and we don’t doubt the necessity. It is just unbelievably awful to revert back when we seemed to be making such progress.

And it’s in these moments that I feel the sinking weight of bitterness try to consume me. The whys and the “how much more does she have to endure” questions creep in to discourage. Maddie feels it too. She woke up this morning angry. Angry that she is pain, that she has tubes again, and that she doesn’t get to go home yet.

Because, you know what? This sucks. I hate this. I try desperately to remain positive, but today I’m feeling trapped. It’s horrible to watch your child suffer emotionally and physically over and over again for 30 days. The pouring rain outside, in contrast to the past week’s warm sun, reflects my dark mood quite nicely.

And yet, it’s this same little girl who is suffering through these pains who teaches me how to endure. The morning was rough until she said, “Mama, I’m sorry I was mean to you,” and then she moved on. This afternoon she laughs. She finds energy to play with her toys. She continues to wow the nurses with her self-sufficiency. How can I have a bad attitude when she clearly hasn’t given up?

Before the first surgery, Colin gave me a blessing of comfort. The words still echo in my mind. He said that the burden of the emotional pain of this trial would not be removed but that I would be able to endure.

I look at my strong little girl feeding herself, sitting up in the chair, breathing room air all on her own and know those words are true.

We can do this. Just a little longer.

Fontan Recovery: Day 24

Colin and I have, what they call, “Hospital Brain.” Confined to the same hospital room, day in day out, with interrupted sleep, constant beeping from machines, hours of waiting around, and moments of intense stress we have been pushed beyond fatigue. We are so sleep-deprived that our sluggish brains cannot function anywhere near to normal capacity. We’re at about 30%, if we’re lucky. Sometimes that’s combined. Think early stages of caring for a new born baby and crank it up at least 5 notches, then remove the joy and wonder of cuddling and first smiles that make it all worthwhile and you’ve got the picture.

Madds on wheels

But this Maddie girl. She continues to astound us with her fierce determination and resilience. This girl is beyond tough. She is the very definition of strong.

Who else could be days out from her third surgical procedure and, with three chest tubes hanging down, ride a tricycle around the hospital floor? Multiple laps, mind you.

(This fine video brought to you by Google + courtesy of the Auto Awesome function.)

Is she jolly? Does she laugh readily? Does she cooperate easily? Does she even interact when friends stop by to visit. No. But she is worlds better off than earlier this month.

Somehow, despite the pain, the anxiety, the fear that come with healing on the surgical floor, Maddie manages to forge onward. She gets up and moves around, walking from the bed to the chair to the commode. She swallows pills and deals with the fluctuations in all the medications, the blood thinners, the diuretics they’re prescribing to dry up the fluid drainage. She suffers through the dressing changes as tubes and catheters continue to be removed, skin red and sometimes blistered and always the adhesive too strong for comfort. Every day she bravely faces the blood draws required to obtain just the right INR levels for her now life-long dependency on Warfarin.

But here we are, three weeks and three days into this thing and at long last we are finally seeing the end of the tunnel. On Friday, they removed her PICC line and the last of her IV medications. Yesterday, they removed the mediastinal tube, which leaves two remaining chest tubes.  Her total drainage output on Monday was 700-something mL for all three. This morning, it was just under 100 mL total. Steady progress. We’ve watched her improve right before our eyes.

Which brings me to my last thought. We are only able to be here with her everyday, watching and caring for her through these long hours because of all of you. Your prayers, your kind words of encouragement, your visits, your babysitting care, your meals, your gifts, your thoughtful treats. All of them have made it possible for us to see our little girl return to health.

Several nurses have commented on how saddened they are by the children they care for here who are without their families because of work constraints and distance, some residing in far away states. But we are able to be here every minute because of the love of our family and friends.  Your support is an answer to our prayers. God has answered them directly through you. Our gratitude is immeasurable.

We are nearly there.  Please bear with us just a little longer. We can hardly wait.

 

Fontan Recovery: Day 12

Yesterday, I was completely dejected.

Maddie is still tachycardic (in a JET rhythm), she may need a permanent pacemaker, is still draining quite a bit from her chest, and has been resistant to anything improving her mood. No toys, games, shows, movies, interactions, not anything has put even a smile on that girl’s face. In short, she was depressed and has been since she woke up from her surgery twelve days ago. It was almost as if she was choosing to be unhappy. Recovery has taken it’s toll on this silly, feisty girl.

Watching your child suffer from pain is unbearable. Watching your child sink into depression is almost worse.

And then a miracle happened. I came to the hospital today to switch Colin and as I pulled into the parking lot, he sent me this picture:

laughing again

Maddie was laughing. Not just a little either.

This image. This image brings me more joy than I can express! My prayers have been that she could find peace and comfort during this process. I feel like those prayers have been answered.

Today, I am grateful. I’m grateful for a nurse that could find ways to interact with Maddie to elicit smiles and laughter. I’m grateful for family members that visit and call regularly to cheer her up. I’m grateful for friends that support us in numerous ways. I’m grateful for a little girl who fights and doesn’t give up.

She’s still tachycardic. She’s still in the ICU. She still has a long way to go. But today I am focusing on the good.

And this smile is good enough for me.

It Takes a Village…

neighbors

When we bought the Pig House, we had no idea how much of a blessing that decision would become. You can view the home’s structure and the landscaping and even the layout of the street before you close the deal, but there’s no way to really know what your neighborhood will actually feel like.

It took me most of the winter and even the early spring of last year to work through the grief of leaving our last home, but when I did, I emerged to find a set of neighbors that I now call friends.

Our children feel at home not only in our house but on the entire street.

Light Sabers on the Street

There are kids everywhere. They pour out onto the street every afternoon as soon as school is done. In the summer, they hardly go inside at all. Age makes no difference. The older ones look out for the younger ones and the little ones look up to the older kids. They are creative and active and enjoy running around exploring the world together. When squabbles arise, they work it out themselves.

I’ve never known all of my neighbors before. It’s both a foreign feeling and one so easy that I can’t believe it’s taken us this long to find such a sense of community. One neighbor opens her garage to the best playroom you can imagine. Another neighbor holds weekly Friday/Saturday night garage movies. Others let Jake and Joe follow them around in their gardens, “helping” them water and dig up worms. Conversations are frequent and easy and there always seems to be some excuse for a party.

Labor Day BBQ

When we told them all that Maddie would need this surgery, every single one of them immediately offered help. They’ve provided meals, babysat the boys, come to visit in the hospital, given numerous gifts, prayed for us, and have otherwise been an unbelievable source of support.

Warren, Jake, and Joey are feeling the stress of this situation and miss both Colin and me when we are gone but they are able to find peace in the normalcy of life on our street. Our good neighbors have looked out for them and cared for them throughout these last few days and everyday. We are so grateful to know that they are happiest when they are home.

“It takes a village to raise a child,” the old adage says. Or four, in our case. And we couldn’t have found a better one to raise ours.

Fontan Recovery: Heart Catheterization

Sister and brother

Days 4 and 5 have not been very good.

Maddie’s heart rate has been consistently high over the last few days. Her tachycardia, arrhythmia, and the continual drainage from her lungs have led the cardiologists to believe that something is obstructing the Fontan system.

There are three possible reasons for this blockage: 1) A closed fenestration, 2) a blood clot, or 3) the new Fontan hardware could have been pinched during the final closure of her chest cavity.

The fenestration, or hole placed in the new tube they engineered next to the atrium, is possibly closed. The hole acts as a release if the pressure from the fluid change becomes too high. All signs point to closure as a first cause.

Another reason could be a blood clot has formed somewhere in the Fontan circuit. This, of course, is a particularly problematic situation. Clots travel through the circulatory system and can end up in the brain causing a stroke. Blood flow through the pulmonary veins show that this case is unlikely the cause right now.

The final possibility, that her tubes have been pinched, is the worst because it will require surgery to repair. The likelihood of this scenario is far less than the others.

She is absolutely miserable. She’s sweating but has cold extremities and poor profusion.  Her vascular system is dry in that the output of fluid through the lungs is greater than the amount of hydration going in or being replenished. Overall, she is not progressing.

Today we were informed that a heart catheterization would be necessary to go in and examine the Fontan and the fenestration to see if the hole needs to be reopened and widened. That is the easiest and most likely scenario and solution.

She is going in sometime in the next half hour (5:30PM Pacific) and will be in the cath lab for up to 3 hours. Of course she will be fully sedated and will come back into the ICU for recovery.

I know this post is extremely technical but I wanted to write this all down to work it through in my own mind. Hopefully, you’ll get a sense of where she is at, clinically speaking.

She has had heart caths before but this time the risk is increased because of the higher potential for dislodging a clot somewhere in the Fontan system. The 5-10% chance of stroke is weighing on my heart and mind so greatly. We’re asking, once again, for your faith, prayers, and positive energy.

We will post more once she is back in the ICU.

 

Surgery Is Over!

Silly girl

Maddie is out of surgery! Everything went well. Her surgeon was very pleased with what he found and with the repairs they did. I will try to post soon the details of the procedure. They are absolutely astounding.

We haven’t seen her yet. They need to get her settled into the ICU first. Most likely that will be within the hour. Just knowing that we can soon hold her sweet hand again is overwhelmingly joyful.

What an absolute relief to know that she is alright. I’m not even sure there are enough words to express it.

Thank you. Thank you all for your constant support. Your strength helped carry us.

Prayers were definitely answered today.

Waiting

Waiting

It’s 11:36AM. We’re still waiting in the pre-op room. We haven’t even met with the surgeon or anesthesiologist yet. I can’t keep my body from fidgeting. The waiting is consuming me.

Madie, on the other hand, is calm. She’s thrilled to be watching “The Nightmare Before Christmas”  on the touch pad. Mind numbing technology can be such a blessing.

We’re not sure what’s causing the delay. The other case must have been more intense than expected. When I start feeling resentful, I just remember the thoughts my dear friend and fellow heart mom, Patty, shared. Some day it will be you in the long, unexpected haul and another family will need to have patience with you. I’m thinking of that other family right now.