Courtney was admitted to PICU at RUSH Presbyterian St. Luke's Medical Center into the care of a very wonderful pediatric cardiologist named Dr. Barbara Santucci. She has remained her pc through all these years and we love her like a family member. Courtney was finally diagnosed the next day with Tricuspid Atresia (no valve, no right ventricle), Transposition of the Great Arteries, Pulmonary Stenosis, VSD and an ASD. They immediately did a procedure to enlarge both her ASD and VSD as there was no other way for her blood to circulate.
When Courtney was 2 years old, she had closed heart surgery for a Blalock-Taussig shunt. That surgery took her to age 6 when she had her big surgery, the Fontan. It was a very scary time for us. They tied off the previous shunt and everything went beautifully. At discharge we were told to give her a baby aspirin every day to prevent the Fontan from calcifying. We were also told the Fontan would probably need replacing in 8 to 10 years.
Everything seemed to go well over the next few yrs except that I noticed that whenever she cut herself she would bleed for a very long time. I took this up with her pediatric cardiologist, who ordered a bleeding time test that showed a 9 min bleeding time (normal is 3-9 minutes). Because she was still in the high normal range she didn't seem overly concerned. She suggested we cut the baby aspirin down to every other day and that is what we did over the next couple of years.
Courtney continued to bruise and bleed despite reducing the aspirin. When she was 12 years old I started noticing that her abdomen was getting quite enlarged. We decided to go and see her pc. She took one look at her and said that her liver was enlarged and that she was in congestive heart failure and probably had been for two months. She had gained 21 lbs from her previous 6 month apt. I felt so bad. I didn't know how I could have missed it. She seemed to be fine except that her waist was disappearing. I thought that perhaps this was normal when one hit puberty. She had no other swelling.
The echocardiogram showed that Courtney's mitral valve was leaking. It turns out that because of the defects on the right side of her heart, the left side of her heart was overworked and eventually her own natural mitral valve on the left side wore out. It was decided that Courtney should get a metal St. Jude valve so they wouldn't have to replace it over the years down the road, as she would have later surgeries in life for the Fontan. Her cardiac cath revealed a VSD that was very narrowed, and subaortic muscle that would need to be resected when her mitral valve was replaced. The cath was done on both the right and left side. The doctor tried to get into the right side but because of scar tissue from all the previous caths, that side was closed off. When the pc went to pull the sheaths out she hemorrhaged on both sides. We were told Courtney was on her way back from the cath lab, and then 2 hours passed before our doctor emerged. We were told that she and another doctor had to apply direct pressure for over an hour. They had her cath sites sandbagged and we were told she couldn't move until morning. The doctor seemed really concerned. Courtney had been off aspirin for over a week so no one could understand why she had bled so much. We went home the next day and when we tried to change the pressure dressings, Courtney started to bleed again and we had to go to our sandbox and make sandbags. This eventually did stop the bleeding. Thank God that the bleeding episode did occur because now they began to take me seriously that she did have some type of bleeding problem.
The next week she went for her mitral valve replacement which was a very long, stressful day. When the surgeon came out he said things had gone well but he had given her many units of fresh frozen plasma along with platelets to get her to stop bleeding and that it was very difficult to control the bleeding. We can only imagine the trouble they would have gotten into had they not known ahead of time that she was having bleeding problems. She recovered well, although her color was bad for a few weeks after the surgery which worried me. Three days post-op, she had to begin taking coumadin (a blood thinner) so that her new metal valve wouldn't get clots. This made us all a little nervous, worrying if she would bleed, but everything seemed to go well with no signs of bleeding. Courtney was finally discharged on day 18. She had had a fever due to pericarditis, which is inflammation of the heart lining. Our pc decided to call in a hematologist to consult before starting her on an aspirin, which was how they treat the inflammation from pericarditis. She decided that it would be okay for a short time. This worried me being on Coumadin and aspirin at the same time but the hematologist thought it would be safe. Finally, we were discharged on Saturday and we were to return to see the pc on Wednesday for a checkup.
Everything was fine until Wednesday morning when we were getting ready to go to Chicago. Courtney kept complaining of a bad headache across the front of her head. She also had a sinus infection so we attributed the headache to that. We saw her pc at 12pm that day. We discussed her headache, she examined her and took her blood pressure. Everything seemed normal and she told us to follow up with our general pediatric doctor if she didn't feel better in 2 days. We made plans to return to the pc in one month. We tried to stop and eat on the way home but Courtney's headache was too bad and we had to leave in the middle of eating. I tried to get her to nap when we got home but she couldn't get comfortable and just kept crying about the headache. I finally called the pc back at 5pm and she said to take her to the pediatrician in the morning and maybe she would treat her for migraines. By 9pm she was really crying in pain and began to kick at the walls. By 10pm she began to vomit. I mean really wretch. This scared me so I told my husband I was taking her to the ER. He stayed behind with our son. Thank the good Lord that I did. When we got there she was beginning to show signs of neurological impairment. They asked her to look at the ceiling and she said she couldn't see it. I jumped off my chair and pointed to the ceiling and then she said she couldn't see me either. I knew right then that something was terribly wrong. They did a CAT scan that showed she was bleeding in her brain. I couldn't believe it! I was in shock. I really thought it was only a sinus infection or the flu. She was transferred to RUSH and was in brain surgery by midnight. When we left the cardiologist's office that day we really thought that we would be back in a month. It turns out it was the same day.
Surgery lasted until 6 or 7 am. I can't really even remember?I was in such a fog. The neurosurgeons said things had gone fairly well but there was a lot of swelling and that they had had bleeding problems as well. In the ICU they ordered a bleeding time and it was now >30min (normal 3-9min). This means that if you haven't clotted in 30 minutes they just call it and stop the test. That alarmed everyone, of course. She had been taken off of the coumadin and aspirin to prevent more bleeding. But now we knew she was at risk for getting a clot in her new valve. All that day she remained on the ventilator and morphine. At 4pm that day she didn't pass her neurological test and they feared she was bleeding again. I argued that they had just given her morphine but they said she should still be able to wake up. They brought her down with ventilator and all for another CAT scan. I couldn't bear to go this time so my husband went down with the neurosurgeon. I had been so strong through the cath, heart surgery, and the first brain surgery but I just couldn't bear her going back to surgery. I went to the restroom to get away from everyone and prayed and pleaded while sobbing, begging God to spare this tiny 12 year-old from any more surgery or complications. When I finally emerged and went back down to the waiting room filled with our family I was confident that when they came back from the CT, it would have been a false alarm and everything would be okay. Several minutes passed and when my husband and the neurosurgeon passed through the doorway I took one look at my husband's face and I knew she was bleeding again. I have never seen him look so pale or so vulnerable.
Courtney went back to surgery but this time the bleed was much deeper and surgery lasted much longer. We were told afterwards that the swelling was massive and that they were forced to leave the whole left side of her skull out. They also told us she was in a coma and that if the swelling continued or she bled again the only thing they could do to save her life would be to resection her brain which would leave her severely mentally impaired. They had put in an intracranial pressure monitor. Every hour, for days I watched that monitor. They told us that if the pressures got over 20 they would have to operate. Twice the numbers did get to 23 and 21. They held off a little longer prepping her for surgery and we just prayed and prayed and miraculously the numbers would go down. After 5 days of no sleeping or eating I really just couldn't take it anymore. We were in a waiting room right next to the PICU. Finally on the 6th day I just had to lay down at 4am. I prayed to GOD that he would either help her to get better or just take her. She was so critical at the time. I couldn't stand living on this roller coaster anymore, hour by hour wondering if she were going to die. I asked him for some kind of sign that things were going to get better. I told him I didn't care how long recovery would take but that I just needed to know. Well, what they say that the Lord takes over when you really give all your problems up to him is absolutely true. I woke up at 6am and panicked. I couldn't believe I had fallen sleep for 2 hrs. I ran to the PICU and leaned over her bed and went to kiss her, she opened her eyes and with her good hand (she was now partially paralyzed on the right side), she reached up and patted me on the back. That was the sign I needed. From then on things only got better. She was in the hospital for a 9 week recovery. Her stroke caused her to loose all fine and large motor skills on the right side. She also lost all her short term memory. She had to relearn her everyday words. She had what is called receptive/expressive aphasia. They put back her cranial bone 5 months later when the swelling finally went down. She went through rehab in our home for 1 year with both occupational and physical therapy. She also spent 6 years in speech therapy, from which she just graduated at 18 years old. She also missed her entire 6th grade year. No one still knows for sure why she bled. She still takes Coumadin every day so we have to keep a close eye on her blood values. We have a Coagucheck machine in our home and we test her blood each week to make sure it is not too high so as to make her bleed but also not too low so she develops a clot in her valve.
After mitral valve replacement Courtney developed atrial arrhythmias due to scarring from previous surgeries and enlargement of her right atrium. They were easily controlled with anti-arrhythmics for 5 years, but in November of 2000 they became chronic. She seemed to be in chronic fib for all of 2001. She was cardioverted (that is, her heart was shocked) 25 to 30 times that year. We did two radiofrequency catheter ablations and she had a pacemaker implanted in December of 2000. But nothing seem to help. Finally in January of 2002, at Mayo Clinic, she had her Fontan revised and converted over to the new circulation now being used with Fontans. They also resected her enlarged right atrium and did a MAZE arrhythmia procedure which involves making many tiny incisions in the atrium and then suturing to promote scarring to interrupt the abnormal pathways and stop the arrhythmias. They did have quite a bit of bleeding during surgery and post-op which was controlled with the transfusion of many blood products. She was hospitalized for 4 weeks due to a few complications, mainly from a pleural effusion. Overall she did well this time around. It is yet to be seen if she will have a cure from her arrhythmias. She has only 45% to 50% heart function and we know that someday she will need a heart transplant.
Today, Courtney is doing remarkably well. Everyone always says that they can't believe what she has been through. She looks like any other normal teenager. She is a beautiful, sweet and kind young woman with a lot of faith. She has regained total use of her right side and has overcome all of her word retrieval difficulties. She is defiantly a miracle! When I look back on everything that happened I think of the "Footprints in the Sand" poem. After the second CAT scan, when they were bringing Courtney back to surgery I felt for just a brief moment that God had abandoned us. But I tried to keep my faith. I knew that God has a plan for everything and everyone. As we look back now I realize that when we thought he had abandoned us, is really when he was carrying us. We can't say why her complications after her mitral valve surgery happened, but we don't ever try to question it either. The Lord has a plan for all of us, and he will test us, that I am certain. How we commit to our faith during those times is what really matters. We are truly blessed!!