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Megan Liberty |
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Six days past my due date, I finally went into labor. Three hours later we had a beautiful baby girl! She weighed 8 lbs. 6 oz. and was 21 inches long. It took her a minute to pink up, but then she seemed to be fine.
That evening I was up and around, watching the Olympics on television. I kept having these feelings that something was wrong with her. That night, I dreamed that I was holding Megan in my arms and she just got smaller and smaller until she disappeared. It was very strange.
When she was three days old I took her to my pediatrician because I was sure that something was not right, I just could not put my finger on it. He examined her and then went to get the pulse oximeter, saying that her color was "just a touch off." Looking back at the pictures, I realize that she was quite blue. Her oxygen saturations were in the 70s (normal being above 95). My heart began to race. Our pediatrician immediately called for a transport team to take her to Primary Children's Medical Center in Salt Lake City, Utah, about an hour from our home in Provo.
By the time we arrived, her saturations were in the 50s and she was a very sick baby. I remember saying to one of the nurses while they were trying to get chest x-rays and EKGs, etc. "here's her binky (pacifier), she will probably stop crying if you give it to her." The nurse's reply was, "if she's crying, at least I know she's breathing." My heart sank.
The cardiologist came in to do the echocardiogram and when he was finished he said, "well I know a lot more now." That was the first time I cried. And I didn't stop for almost three days. He told us that Megan had transposition of the great arteries (TGA), a moderate ventricular septal defect (VSD), an atrial septal defect (ASD), and an unusual coronary artery arrangement. She was started on prostaglandin (PEG1), and admitted to the pediatric intensive care unit (PICU). An umbilical line was started and she was further stabilized.
The next day she had a Rashkind procedure (atrial balloon septostomy) and her sats were slightly better. She was scheduled to have an arterial switch (Jatene) in four days, on her one-week birthday. Unfortunately, on day 6 of life, Megan began to go into septic shock and DIC. Megan had developed omphalitis, a rare, and very deadly infection of the umbilicus and its vessels. The mortality rate for this type of infection is extremely high, and that is with children who have normal hearts. Further complicating matters, a belly ultrasound showed that Megan had an additional birth defect, called patent urachal cyst. This cyst was the most likely source of infection. When her cultures came back, she was infected with group B strep, group G strep, and a "rare" staph.
My husband was at work and my mother was caring for my other children, so I was alone at the hospital when they came in to tell me that, at most, Megan had 12-24 hours to live. My husband reached Primary Children's in under a an hour. It is a 50 mile drive. I was given a moment alone with Megan and then they took her to surgery. The photos from that moment are still painful to look at. I asked them to do everything possible to save her life, so they agreed to attempt this surgery. My husband, Nate, arrived after the operation had begun. During surgery, the surgeon, Dr. Matlak, removed the cyst, and the umbilical ring as well as all of the umbilical vessels.
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Megan returned to the PICU, but was so swollen that I didn't recognize her at first. One of my most vivid memories of that day is her nurse, Peggy, pushing large 60cc syringes of fluid (I later learned that this amber-colored liquid was called Albumin) into Megan's lines, just to give her a blood pressure. She was on 18 medfusion pumps (it would go up to 27 after heart surgery). Her MAP (mean arterial pressure - she had an arterial line in) should have been somewhere around 40- 45. Peggy said that she would be happy with 38 or 39, but Megan's was hanging between 30-33. Without adequate blood pressure, she wouldn't have enough blood flowing to vital organs, especially her brain. For 3 days Megan had even more nurses than normal, and was constantly attended to by at least two PICU nurses. An intensivist (a highly trained doctor who specializes in pediatric critical care medicine) was in her pod space at all times. They said that even if she did recover (which they doubted) she would be brain damaged from the lack of blood pressure.
I prayed like I have never prayed in my life. I begged for her life.
After the third day (day 9 or 10 of her life), there was some very cautious optimism. She was still critically ill, but they changed her status from critical-unstable to critical-stable. It meant that she was still ventilated, still requiring lots of support, but we were down to one nurse, and she had a decent blood pressure. Most kids in the PICU have one nurse to one patient, and the nurse stays by the bed the entire time. So, things were starting to look more normal, well, the PICU's version of normal!
They started checking her heart to make sure the infection hadn't damaged it too much. She had been in mild congestive heart failure this whole time, but now it was starting to get worse. On day 13, they told me they would have to do open-heart surgery the next day. The cardio-thoracic surgeon told me that normally they quote a 95% success rate for this surgery (Jatene Switch, ASD, VSD closure), but with Megan the odds were not so good.
The next day, she was the first case. They had us walk down the hall to the operating room doors with her. She was still on the vent, still so sick and the thought of her going through MORE was just too much. As they turned to go through the O.R. doors, my legs gave way. Nate caught me and carried me into the private waiting room they had set up for us. Later her nurse that day (Mary O.) said that she "didn't know how we were able to say goodbye at that moment" and later she told me that she, along with the surgeon, respiratory therapist, and O.R. nurses had tears in their eyes as they took her into the O.R.
She survived surgery. It was 7 am when we left her, and after 7 pm when we saw her again. She had "bled out" during surgery, and her total blood volume was replaced several times. After the surgery the surgeon said that he "watched her bleed for 3 hours." Lovely. When we saw her in the PICU, her nurse said "I can't talk to you right now" because she was so busy running around, doing things for Megan. She was back up to 2 nurses just for her little self (two of our favorites, Mary O. and Lori McB.) Her chest was open, she had 3 chest tubes, still the ventilator, pumps, lines everywhere, etc., etc. She was also on an external pacemaker
It was a Friday. They closed her chest the following Wednesday. They were hoping to extubate her (take her off the ventilator) by the weekend. For some reason she just wouldn't breathe and her chest tube output actually increased dramatically. It also turned this funny whitish color. They sent a sample for testing to the lab and found out that she had chylothorax, a nasty, slightly unusual complication after open-heart surgery. Her lymphatic system had been somehow damaged during surgery and was leaking fat into her chest cavity, this liquidy fat would then collapse her lungs. No wonder she wouldn't breathe. The treatment for this was to make her totally NPO (no food/drink by mouth) for several days, and then a fat-free diet, and then a special formula called Portagen that has no long-chain fats. And you have to stay on it for at least 6 weeks, probably longer. Megan had been on TPN (total parenteral nutrition - basically nutrition and fats through a central line) since she showed up at the hospital. They had started feeding her tiny amounts of breastmilk through a feeding tube only the day before. And I mean *small* amounts. 1cc per hour (and for those who don't know, it takes 5cc to make 1 teaspoon, so she was getting just under 5 teaspoons per day.) They also told me that she wouldn't be able to have any breastmilk for at least 6 weeks. As a mother, and as a Lactation Consultant, I was just devastated. It was the only thing I could really do for her and it was being taken away.
As I was adjusting to that news, two ladies from the Health Department showed up. I remember one was named Faye. They told me that my baby had PKU, and not only would she not be able to have breastmilk for 6 weeks, she would never be able to breastfeed...I could give her, maybe, 1 tablespoon of breastmilk per day. The PICU quickly turned off her TPN and called the pharmacy and had them make a new batch without any phenylalanine. The problem was with PKU and chylothorax, she would have to live on TPN alone until the chylo resolved.
The next day I walk into the PICU and there are 9 people standing around her bed. I knew something was wrong. I walk over and they are doing an ultrasound of her heart (an echo) and they all look pretty grim. I saw the echo tech write something down on a piece of paper and hand it to the cardiologist. Then the cardiologist took me aside and said that Megan had clotted off completely her SVC (superior vena cava) and 90% clotted off her IVC (inferior vena cava) And he was seriously concerned. They had her heart surgeon, John Hawkins, come over and he said "your daughter scares me". He said that these kind of clots have a 50% chance of killing her. The choices were to start her on TPA (a clot-busting drug) or take her to surgery and remove the clots manually. The problem with the TPA was that she had already bled out during surgery and her liver had taken a big hit w/ the sepsis, etc. They didn't know if she would just start bleeding everywhere w/ the TPA, including into her brain and around her heart, or if she would be okay. And the problem with surgically removing the clots was that she would have to go back on bypass, and risk further brain damage. She had been on bypass for more than 3 hours the week previous and we were not anxious to put her back on. We decided to try the TPA.
Megan began a TPA infusion. After a day or two, the SVC clot dissolved, but the IVC clot decided it was happy where it was (and is still there to this day). It starts about the level of her kidneys and goes all the way down to her legs. Her venous return is facilitated by collateral vessels.
We would deal with several more venous blood clots during her PICU stay. And they would always change her pressures within her chest and cause an increase in her chylo. She currently has 3 major DVTs (deep vein thromboses—blood clots), the IVC one, plus one in her right internal jugular and her right innominate artery.
One morning when I came in, Megan had spiked a fever (again) and they were culturing her. Since she was in the PICU and had central lines, she needed a "septic workup" which includes blood work with cultures, urine culture and a lumbar puncture (spinal tap). She had had several septic workups during her stay. This day, November 1st, her blood was, yet again, positive for bacteria. She was septic again. They also did an echo to check for endocarditis (an infection on one of the valves in the heart—potentially devastating). I wasn't too worried because we had been through this before and her echo was always fine. This day it was not. She had staph aureus endocarditis. One of the most aggressive bacteria, one of the most damaging to the heart, was growing and damaging her aortic valve. This meant 6 weeks of IV antibiotics...6 more weeks of central lines, etc.
The central lines should have been the least of my worries. She had developed some life-threatening arrhythmias. They still use her strips for teaching tools.
She was started on IV amiodarone and again we prayed. She started having what we call the "arrhythmia salad" every kind of thing you can think of...PVCs, PACs, aberrantly conducted PACs, SVT, and then the bad ones...you know what I mean.
She never really got over the arrhythmia thing and was on meds for it for 2 years.
We finally brought her home. Just before Christmas. We went right from the PICU to our home. She was on 14 meds that had to each be dosed at different times throughout the day. Several of them were "every 4 hours" or "every 6 hours" so we were up at all hours. She was also on a feeding pump, getting continuous feeds 24 hours a day. When we brought her home, she was nearly 3 months old and weighed less than she had at birth. She was a skinny little thing (still is!)
We had a great Christmas and then she was readmitted on New Years Eve with arrhythmia and respiratory distress. We were "frequent flyers" for that first year or two. She has also had surgery for ear tubes, a g-tube and other more minor things.
We later learned that Megan had/has a T-cell deficiency. It explained a lot of her pneumoniae, and other bacterial infections. She has been treated with PCP prophylaxis and briefly with IVIG.
Megan is now three and a half years old and doing beautifully. She has no delays at all and had her g-tube removed about 6 months ago. She is a spunky kid with a lot of fight in her (obviously!) and we expect her to do very well.
I can't say enough good things about Primary Children's Medical Center. The doctors, surgeons, and nurses there cared for my daughter the way I would and took care of us too. They are a truly amazing team.
December 2005 Update
Megan is currently in kindergarten and doing very well. In June of 2005 we had some neuropsych testing done to determine if she was ready to enter kindergarten. We were thrilled when the doctor said that Megan had "no neurological deficits whatsoever, despite a very long pump run" and "very high IQ". I started crying in her office, after all she has been through, we were virtually guaranteed that she would have at least some learning problems, if not significant brain damage. We are so thrilled for Megan. After 6 hours of neuropsych testing, which checked everything from language development to fine motor skills to visual-motor integration, she is completely, totally normal.
She is into all things girly and loves her baby brother. She gives her older brother and sister a hard time, but they still adore her. She is feisty and intense and demands attention in ways that her siblings do not. Megan is popular with classmates and enjoys reading, writing and painting. She takes a ballet class once a week and LOVES to be on stage.
Her last cardiology check up was in November of 2005 and her heart looked pretty much the same as it always has. Some residual pulmonary stenosis, but for at least the next little while, we are good to go. She does seem to fatigue earlier than her peers, but she paces herself. At school she will take short breaks on the couch in the reading corner, but bounces back pretty quickly.
She has turned out to be a normal, bright child. She makes us laugh everyday with her antics, she brings joy and humor to our family. We couldn't be happier.
March 2009 Update
Megan did pretty well in kindergarten and it was obvious that the neuropsych testing was accurate when it showed that she was very bright. However, we noticed that she had a very hard time paying attention in a classroom setting. In the middle of second grade, Megan was diagnosed with ADD. I know that some heart kids have ADD, but I wasn’t sure if it was part of the defects themselves, early and often surgeries or just the luck of the draw. On medication, Megan does very, very well. Off medication, she is able to compensate, but tends to have a very hard time staying focused on what is happening in the classroom.
Heart-wise Megan has done quite well. She is on aspirin and midodrine. Her activity in PE or at recess is based on what she thinks she is able to do at that moment. She does have a 504 plan in place.
Today, Megan is in the 3rd grade. She is getting good grades and has lots of friends. She is a happy, singer-dancer child who has told us that she has no doubts that she will be famous one day. She has been full of an unbelievable will-to-survive since the day she was born. I'm sure that will help her become exactly the person she wants to be.
