A Birthday Letter to Sydney

My beautiful baby girl, Sydney Jo:

Today you are three years old.  I can hardly believe it.
My gorgeous girl, where does the time go?
This year has been a rough one for us, in finding out that your heart is sick like Madi's was.  You are such a trooper, though, and nothing is holding you back at all.  I pray every day that your heart function will improve and that you can continue to live as normal a life as possible.
You are making such leaps and bounds with your vocab!  All your hard work with your teachers is really paying off, and I couldn't be more proud.  We will show everyone yet that us Rippy girls are fighters.
It's been so busy in our lives lately-you got to see Sesame Street Live again this winter, which you loved.  We were also fortunate enough to get to take you and your sister to see "The Lorax" in the movie theater a couple weeks ago.  It was both of you girls' first time at the theater, and we all had a blast.  Hopefully that's something that we can continue to do, as well as other events through HopeKids.
The weather has been beautiful here as of late-we even had a week of 80 degree temps in March!  You, Madi, and Mama have been having a great time outside.  You still love to dig in dirt and get messy every chance you get.  You also love to take walks and point out birds and puppies along the way.  On days that it's icky outside you like to watch "Mickey Mouse Clubhouse" or the movie "Tangled." 
Sydney, you have blessed my life in more ways than you can ever imagine.  I hope you know how wonderful and special you are to me.  No matter how old you get, you will always be my baby.

Love, Mama

Sh!t Heart Moms Say

A new trend has hit the internet.  Actors and actresses on YouTube doing parodies of stereotypical things that a certain gender/race/group say or do.  And let me tell you, as un-PC as they can be, I have found them to be hilarious.  My personal favorite is Shit Fat Girls Say.  It cracks me up every time, most likely because let's face it, I am not the skinniest person to ever walk the earth.  And I may or may not have said some of the things that are portrayed in the video.  You can watch it here but be warned, there are parts that are not for little ears.
After watching that video, and some of its hilarious counterparts-Sh!t Extreme Couponers Say, Sh!t Girls Say, and more-I decided that there should be one for us heart moms out there.  Being that there's no way on God's green Earth that I am going to be filming myself anytime soon, I thought I would just post some of the things that are said frequently in our little world that may not make sense to others, but cause that "aha" moment for a heart parent.  Feel free to contribute if you think I have missed some!  :)

"Are her lips bluer than normal to you?"
"Anyone know of any way to put extra calories in a diet without using olive oil in everything?"
"She was satting in the low-80s today, so I was a bit concerned."
"Can you believe how PINK her toes are?"
"Well, her ejection fraction is 25% but you could never tell by looking at her."
"I HATE when people tell me that she's 'repaired'.. she has freakin man-made material in her chest!"
"Please pray for pee!"
"Anyone out there have a trick to getting your two year old to sit through a blood pressure reading?"
"During her speech therapy today, she actually said a full sentence!"
"Ugh.  So tired of oral aversions."
"Yep.  We have pneumonia/RSV.  Again."
"Oh my gosh, we have made it 6 months without seeing the inside of Children's!"
"Yay!  They featured someone with HLHS on Grey's Anatomy tonight!  But they totally handled it wrong.."
"Well she has a moderate tricuspid leak, but most of us do, so whatever."
"I cannot wait till he gets off this sildenafil.  The 'unfortunate side effects' are a little weird in a three-year-old."
"Another stay at Hotel Amplatz.  (Hotel Children's, etc.)  Can someone bring me some food?"
"I don't want to say the h word yet, but.. we may get to go home soon!"
"I wish I could put her in a bubble so she could avoid germs all the time."
"Woo hoo! Extubation!"
"He's such a hard stick that they are keeping the IV in till they are sure he will stop dropping his pressures."
"Three a.m. and I am doing laundry.  Damn g tube leaked all over again."
"What's his INR?"
"Chest tube removal.. sounds like the perfect time for me to leave the room."
"Do they HAVE to check vitals every 4 hours? How do they expect us to sleep around here?"
"I swear I should a have a freaking nursing degree by now."


Sometimes, it's good to know you're not alone.

The Faces of CHD Part Fourteen: Torn between two loves

Today is CHD Awareness Day.
It's Heart Day.
And my heart is heavy.
All these angel babies gone way too soon.  All these parents grieving, dealing with their own broken hearts. 
Instead of posting a heart child's story today, I am honoring the angels.  I hope you will take the time to say a prayer for them, and for their families.

"It's a beautiful day up in heaven. Jesus is rounding up his tiniest angels, to go live on earth, and be born.
One of the sweetest angels says to Jesus "I don't want to leave, I like it here, and I will miss you". He reassures the scared little angel that everything will be okay, and that she is just going for a visit.
She is still not swayed on this idea. So Jesus kneels down, and says "how about if you leave half of your heart here with me and take the other half with you, will that be okay". The angel smiles and says "I guess that will work".
But the little angel is still a little scared. She asks "will I be okay with only half of my heart?" Jesus replies, "of course you will, I have other angels there that will help out, and you will be fine."
Then Jesus gives the angel more details about his plan, he says "when you are born, your mommy will be scared, so you have to be strong, and when you feel weak just remember that I have the other half of your heart". "Enjoy your time with your family, play and laugh everyday."
"And when its time to come back to heaven, I will make your heart whole again.
Always remember that you are not broken, just torn between two loves"

CHDs are ugly.  Scary.  Sad.  Destructive.  Selfish.  Non-discriminating.
This is why we fight.
For Tru, Nathan, Pierce, Ewan, Easton, David, Parker, Olivia, Hazel, Kayden, Lucy, Tommy, Joshua, Amayah, Lorenzo and Andrew.  For all the angels.  And for their families, torn between two loves.

The Faces of CHD Part Thirteen: HAILEY

Story taken in part from the U of M website/Jennifer Helstrom, Hailey's mom.

On March 25, 2010, Jennifer went into labor. As Jennifer and her (now ex) husband, Scott, drove to the hospital, labor progressed much quicker than expected. Scott was speeding when a police officer pulled him over. A few minutes later, that police officer delivered Baby Hailey — along County Road 5 in Cambridge, Minn. — in the back of the family van.

Jen immediately noticed that Hailey’s lips were blue and were concerned about what impact the 35-degree weather was having on her newborn. An ambulance transported Jennifer and Hailey the rest of the way to the hospital. Hailey was admitted to the hospital’s neonatal intensive care unit due to respiratory distress. A week later, Jennifer and Scott learned that their baby’s lips were not blue from the cold but because she had truncus arteriosus, a serious congenital-heart defect.

When Hailey was two weeks old, doctors implanted a donor heart valve and at three weeks, added a pacemaker. At four months, Hailey’s doctors implanted a new pacemaker and referred her to University of Minnesota Amplatz Children’s Hospital for specialized care. Hailey’s condition worsened — her heart was failing due to underlying dilated cardiomyopathy (decreased heart function because of a weakened and enlarged heart) that had evolved separate from the truncus arteriosus. Hailey would turn blue during normal infant activities such as eating. In September 2010, she was added to the transplant list.

Two weeks later, Hailey was comfortably asleep in Jennifer’s arms when the phone rang. “Something made me get up,” Jennifer recalled. “I took a deep breath and answered. It was our transplant coordinator, Tracy Demars. She said, ‘Jenny, we have a beautiful little heart for Miss Hailey.’ I remember those gentle, kind words like it was yesterday.” Jennifer then embarked on one of the most nerve-wracking drives of her life.
Hailey was able to get a heart quickly due to her age, blood type and size. The transplant however, was higher risk due to Hailey’s weight of only 5.5 kilograms (12 pounds). The surgery went well and there were no complications.

Post-transplant

Hailey was discharged two weeks after the transplant and has shown no signs of rejection.

Her cardiologist, Rebecca Ameduri, M.D., says that Hailey’s prognosis is very good since infants have the best long-term transplant outcomes. Since the transplant, Hailey has begun talking, and is eating well and growing. She requires a strict regimen of anti-rejection medications, but Jenny feels so blessed to have her, and is extremely thankful for the donor family that gave Hailey another shot at life.

Hailey & mom, Jen, Christmas 2011

The Faces of CHD Part Twelve: LUCY

Written by Melissa, Lucy's mom

 

We found out at my 20 week ultrasound that Lucy had a heart defect. My OB noticed it on the ultrasound so she sent us to a cardiologist in St. Cloud who did an echo and confirmed that Lucy had Tricuspid Atresia with a hypo plastic right heart (read about that here). We had then started going to the U of M to see the fetal cardiologists. They had a plan for us to be induced one week early so that they could have the doctors on staff that Lucy would need. Lucy was born September 12, 2011 at 7:49 she was 10 lbs 5oz and 20.5 inches. It was a long labor that ended in a c-section due to her size.

 

Lucy was brought to the NICU immediately so that they could do an echo to confirm what they saw prenatally. Things were as they believed. They waited a couple of days for her PDA to close to see how her heart would circulate her blood. Things seems fairly well so they decided they could do the Rashkind procedure (A cardiologist threads a catheter through the belly button or a vein in the leg, to the heart, and creates a hole between the upper chambers. The hole allows oxygenated blood from the left side of the heart and deoxygenated blood from the right side to mix, increasing the amount of oxygen in the blood pumped to the body) instead of having to put in a shunt. This was a good thing since that meant not having to open her chest. After a few days she seemed to be doing very well. She was eating like she should and her oxygen was in the high 80's to low 90's which was wonderful. They had moved us to another room because they had planned on sending us home.

 

Lucy started to not eat as much which they thought was ok due to what she had gone through with the Rashkind procedure. They told us she could not go home until she ate a certain amount every 4 hours. On the 20th we had gone home for the night when we got a call at 4am that her oxygen was dropping dramatically into the 40%. They were giving her meds to keep it up. They ended up putting in a central pick line to get meds directly to her heart. This took hours because they could not get it in they finally had to take her to radiology to get it done. After another day and no improvements they move her to the PICU so they could watch her better. On the 22nd her pressures started to drop dramatically. It was like a circus in her room trying to give her meds to keep it up. At about 11pm that night the surgeon came in saying our only option was ECMO and it was very risking because she was in heart failure and very sick with only half of a heart. We signed the papers and we watched them take her away not knowing if she would make it back. She got back around 1 am and things went well. The doctors were not sure why she went into heart failure. We had multiple doctors coming and going looking at her and doing tests. They sent her tests to the children's hospital in Cincinnati for their input as to what they thought may be wrong. They were not sure if it was cardiomyopathy or myocarditis. Lucy was on ECMO for about a week and things were not really changing they decided to try the Berlin heart which would be another risky procedure due to how sick she was and that she was on ECMO. They put in the Berlin heart and things went well during the surgery but she only had it for less then a day. Since she had only half a heart they had to also put a shunt in for the Berlin heart to work and place it in differently then they would normally and it did not work for her. Lucy went back onto ECMO. They told us she would have to be put on the transplant list immediately as a 1A recipient in order to have any chance but even with a transplant they told us she had a very low percentage that should would make it thorough because she was so sick. At this point Lucy still had the doctors unsure as to what was wrong with the left side of her heart. Lucy went on the transplant list September 28th. They had attempted to close her chest on the 6th which would be the first time since the 22nd. They did get her chest closed but she was bleeding more then she ever had. They did not know if she would make it through the night. At 1pm our surgeon Dr. Bryant called me to say they had a heart!! We were so excited but they told us getting her through this was not going to be easy. She got her new heart October 7th. She made it through the surgery great but when they tried to taking her off of bypass her pressures kept dropping again. They tried 3 different times for at least 4 hours and finally they had to put her back on ECMO. They believe that the heart was good but was on the small side and Lucy was so sick that it could not catch up. They kept her on ECMO for a week then they tried taking her off and the first time it did not work so they tried again two days later and she was able to do it on her own. Now the problem was her kidneys took a hit while being on bypass so she was full of fluid and was not peeing so they put her on dialysis. So they had to put her through another procedure to start the dialysis. They would fill her with the fluid and let it sit for 30 min then drain the fluid. Every time they would empty the fluid her pressures would drop. So they had to stop the dialysis for a couple of days and try it again. Once they started again it worked better and did not seem as hard on her. They were getting rid of some of the fluid but still not enough to close her chest. On October 19th they told us her liver tests were coming back extremely elevated so they were going to be watching it more closely. So now it was her heart, kidney's, and liver. October 20th I was waiting for the tests to come back to see if it had gotten better. One test stayed the same and one was a little better. This was also my husband's and mine 10th anniversary so after we got the results back we decided to go out to eat. When we got back Lucy was doing fairly well just going through her dialysis. She was starting to have some urine too so things seem to be looking good. But at about 10:15 when they were draining the fluid her pressures dropped as they had been but they always went back up right away. This time they dropped and did not seem to be going up as normal. They gave her meds and some blood products and it went back up for about a second but i stood there watching the monitors as her pressure suddenly dropped in half and never went back up. They hit they code blue and the room instantly filled up. They worked on he for about 45 min and she never came back. The surgeon told us that they could try ECMO one more time but he gave it about a 1% chance to work since she had already been on it 3 times and her chest had been open for over 4 weeks so anything we did not would be for us and not for Lucy anymore. He also told us that because her oxygen had dropped so low for a long time that she may have brain damage if she were to even make it through. My husband wanted to be the one to tell the nurses to stop so we went back into the room and he asked them to all stop. She never came back to us. It was the hardest thing we ever had to do.. we always knew this could happen but always before she was having another procedure not just what seemed out of the blue. I miss her so much. We only were able to hold her that first week but she was able to hold our hand and she would squeeze our hand every time we held it. She would always open her eyes and move her head when she heard me it was wonderful. The nurses loved her and I think the whole staff was amazed at her strength and how long she actually hung on. She was a fighter!! 

 

They did not find out what was wrong with her native heart till after it was removed for the transplant. They discovered it was Hypertrophic cardiomyopathy (. Due to Lucy being born with only half a heart it put to much pressure on the other side which caused the heart failure to progress so quickly. 

I miss her and love her so much but am thankful for the time we had and glad she no longer is suffering.

 

The Faces of CHD Part Eleven: GIANNA

Written by her mom, Margit

 

When we found out in December 2009 we were expecting twins, we were beyond ecstatic, yet hesitant.  We lost one of our twins (Gabriel) at 27 weeks in utero due to Trisomy 18 in our first pregnancy, but were blessed with Isabella.  We were so worried going up to the 20 week ultrasound.  I remember the confirmation that it was two girls and everything was perfect.  Wow, nothing wrong, that is amazing I thought.  Then at our 24 week ultrasound, the tech spent a lot of time on Baby A's heart.  I had flashbacks to the amount of time they spent on Gabriel's heart.  Tom assured me it was fine.  I asked what was wrong.  The tech said I think she might have Transposition of the Great Arteries.  I was heart broken, I couldn't lose another child.  She said it was "a good heart defect" to have because the outcome after surgery is favorable.  That didn't help me much, but I knew she was trying to help me stay positive.  We then met with the perinatologist and cardiologist who confirmed her TGA.
Thank God, I carried the twins to 38 weeks.  This gave Gianna the best chance for surgery since she was able to grow more.  Gianna and Reagan were born 8/18/2010.  Gianna needed a procedure, balloon septosomy, within 4 hours of birth to allow more oxygenated blood to circulate. 

 

She had difficult heart anatomy with her coronary arteries being inverted, so she had two more heart cath procedures  to ensure they had her anatomy correct before her open heart surgery.  She had her open heart surgery to correct the inverted coronary arteries and to correct her aorta and pulmonary artery, which had grown in the wrong spot. Gianna did amazing, but had a lot of bleeding after her surgery.  

 

Our surgeon told us it was one of his toughest surgeries ever for him.  She had a lot of swelling in her tiny little body, and her chest was left open for 4 days.  Her stay was on the very long end for this type of surgery.  She had issues with feeding, gaining weight, and retaining water.  After 5 weeks to the day, Gianna was able to come home to be with her family.  She was in the less than 3rd percentile for weight height when she came home, and 80% tube fed.  Within 6 weeks, she was 100% bottle fed and by 6 months old she had moved up to the 10-25 percentile for weight and height.  She still has a VSD  and  narrowing of her pulmonary artery, and may need surgery in the future, but we know she is a fighter!  I thank God every day for the University of MN Amplatz Children's Hospital, Dr St. Louis (her surgeon) and the wonderful staff and volunteers.    

 

Gianna is a miracle.  She is my angel.  Her name means God is gracious.  She truly is a gift from God and shines on everyone she meets!

The Faces of CHD Part Ten: NATHAN

Nathan's mom, Michelle, knew that the baby she was carrying had a heart problem.  She just didn't know what kind yet.  As she waited for the week to go by until her next OB appointment, when she would find out just what was wrong with her little guy, she went into labor and had Nathan at 29 weeks.  It was December 19th, 2008, and it was the middle of a blizzard.  Mayo One, the lifeflight helicopter used to transport patients, was grounded.  In the ambulance on the way from one hospital to the children's hospital at St. Mary's in Rochester, MN, her pediatrician and OB (who were along for the ride) told her that Nathan had Hypoplastic Left Heart Syndrome-that his left ventricle was pretty much non-existant.  The family was told that he would need surgery as soon as he hit 5 pounds, and that was if they chose to try to save him.  Otherwise, they could do hospice care and wait for him to slip away.
Michelle, of course, knew she had to do whatever it took to give her beautiful boy the best chance she could.

On March 13, 2008, Nathan was not quite 5 pounds but he needed to have surgery.  He had the first of his three planned surgeries that day and did well.  (Read about the three-stage surgery used for children with HLHS here ).  In July of the same year, Nathan was once again a very sick little boy.  His kidneys and liver were not doing so well, and it was found that his shunt had closed.  Since he was so tiny and so fragile, a catheter procedure couldn't be done to put in a new shunt, and Nathan had to undergo another open heart surgery to replace the closed shunt.

At the end of October of 2009, Nathan went in to have his second stage surgery, the Glenn.  He did very well, and was at home within 2 weeks.  After being home for a week, it was discovered that he had a chylothorax, a leak in his lymphatic system that was going into his pleural cavity.  (Chylothorax info in detail), so he spent another two weeks inpatient.  Nathan was the star of the cardiac ward during those two weeks.  In spite of his chest drainage tube, he was having a good old time with wagon rides and playing with all the staff.

In late November of 2011, Nathan went to St. Mary's for what was to be the last of his planned surgeries, the Fontan.  While he flew through the surgery itself, the next four days were not so great.  His body was so traumatized and tired that he needed to be placed on ECMO to get some rest.  Just as he was getting hooked up to ECMO, he coded for four minutes.  They were able to bring him back, and in the next three days, Nathan went back to the OR four times to have more heart surgeries.  At some point during all the calamity, he developed a large blood clot in his leg, and the family was told that when he was recovered from his heart surgery, a part of his leg/foot would need to be amputated.
After coming off of ECMO, Nathan's kidneys didn't want to function, so he was placed on dialysis.
Late in the evening of January 9th, 2012, Nathan's pressures dropped considerably and he took a turn for the worse.  It was found that he had grave intestinal bleeding, and it was inoperable due to complications with dialysis and kidney function.  Rather than prolong his suffering, the family chose to let Nathan go.

Nathan and family w/Santa 2011

Nathan & Mama just days before he went to Heaven

He passed away peacefully in his mom's arms on January 10th, 2012, in the wee hours of the morning.
Today Nathan has been gone for a month.  His family does their best to get through, and they know that Heaven is a beautiful place.  But it doesn't change the fact that he is not here on Earth with his family.
CHDs are monsters.  They affect not only a heart, but a body's whole operating system.  They are so much more than "just" a heart problem.  Hopefully hearing Nathan's story (and all the others featured on this blog) will help people to understand just how much is needed for research and funding.  We need to fight these monsters.
Today, please take a moment to pray for Nathan and his family.  And then, do something that benefits CHD awareness.  For all the children in pain, for their families, for the kids gone too soon. 
For Nathan.

Rest in peace, sweet boy.