Tetralogy of Fallot with Pulmonary Atresia - Jessica style

A friend asked me to help her as she is going to school and needed to interview someone who has experience parenting a Special Needs child.  She thought of Jessica and me and I'm so glad that I had the chance to help out.  I spent quite a bit of time on it today and am including a link about Jessica's heart surgeries so that I don't have to write all of that again.  Here is what I wrote in answer to these three questions:

1- What exactly was Jess's congenital defect?

2- How many surgeries did she have and what was done

3- How did her defect (and surgeries) affect her school and academic learning?

Jessica’s heart defects were: (I am using my own words to describe the heart defects)

1 - 4. Tetralogy of Fallot which consists of 4 heart defects:

    

     1. Ventricular Septal Defect (VSD): A Ventricular Septal Defect (VSD), is a large hole in the septum (heart wall) between the bottom two chambers (ventricles) of her heart.

     2. Overriding Aorta: The Aorta is a huge artery that takes blood from the heart to the body.  An Overriding Aorta is when that artery sits directly over the VSD and allows blood from both of the bottom two chambers to go up into the Aorta and out to the body.  A normal Aorta usually sits right on top of the Left Ventricle.  

    

     3. Pulmonary Stenosis:  Pulmonary Stenosis is where there is narrowing in the pulmonary artery (which takes blood from the heart to the lungs to receive oxygen).  Jessica’s narrowing was as severe as it gets.  It was narrow all the way through.  To top it off Jessica’s pulmonary arteries only branched out to 1/3 of each lung. 

    

     4. Right Ventricular Hypertrophy: Right Ventricular Hypertrophy is where the wall of the heart around the Right Ventricle (the bottom right side or chamber of the heart) is thickened because of the extra work placed on it to pump blood out to the body rather than just to the lungs.

Jessica also had the added complicated CHDs of the following:

5. Pulmonary Atresia: Pulmonary Atresia is where the pulmonary valve that comes up from the Left Ventricle to go out to the lungs is either not working, closed off or not even there at all… as in Jessica’s case.  Jessica wasn’t even both with the piece of Pulmonary Artery that hooks up to the heart; in other words, Jessica had no direct connection between her heart and lungs.  The huge VSD is what saved her life.

6. Pulmonary Branch Stenosis:  Pulmonary Branch Stenosis is where the pulmonary artery branches (where the artery divides into “branches” to take blood out to the lungs) are all very narrow.

7. Nonconfluent Pulmonary Artery Branches:  Nonconfluent Pulmonary Artery Branches means that the pulmonary artery branches don’t branch out to all parts of the lungs.  Jessica's only reached to 1/3 of each lung.  

8. MAPCAS: Major Aortopulmonany Collateral Artery:  MAPCAS: Major Aortopulmonany Collateral Artery(s) is where there are small blood vessels that come off the Aorta and take blood to the lungs that way.  The problem with these blood vessels (often just referred to as collaterals) is that they are taking blood that has the oxygenated & unoxygenated blood mixed together. 

To let you know how rare Jessica's combination of heart defects of just the two:  Pulmonary Atresia and VSD are, In a 2012 study using data from birth defects tracking systems across the United States, researchers estimated that about 1 out of every 10,000 babies is born with pulmonary atresia whereas 1 in every 100 babies are born with CHD.

I edited the image below to point out Jessica’s CHD.

 Here is the original image which includes a normal working heart, Pulmonary Atresia with Tetralogy of Fallot (lower left) and MAPCAs (lower right). 

I gave my friend Amanda the link to Jessica’s 5 heart surgeries but Jessica had quite a few other surgeries as well.  She had to have her extra 2 fingers and 1 toe removed; she had numerous cut-downs on her arteries when they were doing cardiac caths, she was kicked by a horse in 2008 and developed a massive hematoma which had to be operated on.  That left a huge gaping hole which had to be packed every day.  This took an extra amount of time to heal because Jessica’s body didn’t get enough oxygen to the cells for it to heal quickly.  Jessica endured 2 strokes during her first 2 surgeries.  That information is included in the link I sent you about her 5 heart surgeries.  Here’s that link again:  http://fancydancy.blogspot.com/2007/05/five.html

Jessica spent a good part of the first few years of her life at the hospital, doctor’s appointment, labs, going to OT, PT & Speech therapies, etc.  Most babies and young children learn as they do things.  Jessica’s heart was so enlarged and the function was so poor that she could barely sustain life at first, much less have the energy to do things.  On top of that she spent several months recovering from major surgeries which most children don’t have to do.  Jessica’s body was weak so even with the different therapies she wasn’t able to reach milestones at the same time as the healthy children her age.   She sat up on her own when she was one year old and she walked at age 2. 

Over the years her body got stronger… for a little while anyway.  She was able to learn but at her pace.  She seemed to stay on a learning curve similar to her peers except that it was lower than theirs… both physically and academically. 

We participated in a genetics study in 1989 and it turned out that Jessica had DiGeorge Syndrome.  This syndrome can cause many different learning delays as well as medical issues including CHD. 

Over the years it seemed that Jessica had leveled off at the age level of a 7 – 8 year old.  For many years she seemed to be stuck in her routines, activities she liked to do and learning new things became harder for her.  Towards the end of her life she started regressing to an even younger state especially emotionally.  Her anxieties got out of control especially at night.  She started crying more easily and getting her feelings hurt more easily too.  Her pain was way up so I’m sure that had a lot to do with it but she knew that she could die and that scared her too.  

On top of that in 2004 Jessica was placed into a home hospice because she was having lung bleeds.  Those MAPCAs (collaterals that come off the aorta and out to the lungs) were bursting and bleeding into her lungs and any one of them could have been fatal.  The hospice nurse came with me to an appointment with the pulmonologist and she (the nurse) recommended that we try morphine therapy to relax the lungs and it worked!  Jessica was kicked off of hospice because her lung bleeds had stopped.   Jessica had soooo much anxiety during that time… as she was coughing up blood she would cry out, “I’m not ready to die!” as she choked on the blood.  It took me awhile to help her calm down when it was over and then I would have to go and find my youngers sons because they were scared and were trying to comfort each other.  It was very upsetting to our whole family and went on for months where she would cough up blood daily, sometimes several times a day.

Jessica also had ischemia of the bowels which was where the blood vessels going to the gut were very narrow much like her pulmonary artery.  She also didn’t have enough oxygen in the blood that actually got to her gut so over many years her bowels were dying and becoming necrotic.  It was a slow, painful death.  It was very hard on all of us to watch her suffer through so much.

Jessica wasn’t ever able to get enough oxygen in her blood which made her look blue or even dark purple.  Of course that meant that her brain never received enough oxygen either.  Even when wearing oxygen much of Jessica’s blood wasn’t able to reach the lungs to pick up that oxygen.  The oxygen served as a relaxing agent on the pulmonary arteries to help them open up a little big to make her more comfortable. 

We will probably never know the exact reason why Jessica remained as a little girl, only able to understand things at a 7 year old level.  She had several individual things that could have caused it such as delays from being sick, strokes which affected 5 areas of her brain and lack of oxygen to her brain… all I know is that she was perfect just the way God intended for her to be.  

February is CHD Awareness Month ~ Heart Surgeries

Jessica following her 2nd heart surgery age 3

This photo was taken after Jessica's 2nd heart surgery. My 5 year old son was scared to go into the PICU to see her and the only reason they allowed him in was because he was going to stay with my parents and I didn't want to send him away without him even seeing his sister and knowing that she hadn't died. A nurse took this picture to show him before he entered the PICU so that he wouldn't be quite so scared. We brought Jessica's doll and stuffed animal and placed them around and on top of her so that he wouldn't be afraid of her incision and the tubes and wires on her chest. {Too bad we didn't know back then that he had Aspergers (autism spectrum) back then and that's why he would freak out so much when he saw his little sister with tubes and wires, etc.} This was the surgery from when she hemorrhaged, had a massive stroke, was blind and paralyzed for a while and had a blood infection. At the time of this photo Jessica had barely regained enough eyesight to focus on something for a few seconds and then her eyes would drift up to the right. She couldn't control where they were looking and we didn't know how much she could see because when she could key in on something sometimes she would cry out as if she was afraid of whatever it was she was looking at. 

Jessica following her 5th heart surgery - age 7

Jessica posing with Dr. Donnerstein and Dr. Goldberg

The first photo is after Jessica's 5th heart surgery which went VERY well.  The other photo is of Jessica posing with Dr Donnerstein (on the left) and Dr. Goldberg (one in the back).  Both of these photos were taken right before we left the hospital to go home.   Dr. D and Dr. G are two of the pediatric cardiologists who cared for her over the years. Dr. D was the one who diagnosed Jess when she was 2 weeks old and he followed her until he retired when Jessica was 20. We joked that Jessica put him into retirement from all the stress she gave him throughout those 20 years. He came to her PC visits even after he retired but he came as a friend. He was very kind and caring. He stayed at the hospital all night the night that Jess was hemorrhaging and kept going back into surgery. Once a patient is handed over to the surgeon they usually step back until the after the surgery and the patient is released but Dr. D felt as though we were family. He was a great source of support to us all those years.

I didn't take many photos of Jess when she was in the hospital, especially when she was in the PICU. It was so very hard on me that I knew I would never forget and I felt like I was invading her privacy if I took pictures of her hooked up to all the machines and monitors, so I didn't take photos... but now I wish I had. I want others to know what an amazing soul she was and what she endured. Jessica's legacy of hope and love lives on but I feel like I could have done her story more justice with more photos of her difficult times. I am thankful that I went camera-crazy and took a lot of photos of the better times because sometimes those good memories are all I have to push out the flash-backs of the bad times.

To read more about her 5 heart surgeries please follow this link:

FIVE Heart Surgeries

‪#‎CHDweek ‪#‎CHDawareness ‪#‎1in100

February is CHD Awareness Month ~ Diagnosis

I am going to be posting CHD (Congenital Heart Defects) facts and sharing Jessica's story as a way of bringing awareness to CHD. 

Day 1 of CHD Awareness - Diagnosis:

Jessica almost died as an infant due to her undiagnosed CHDs. Miss Jess was born on 6-3-88. My labor was fast and furious! In fact, the nurse had to grab a doctor out of another room to come and catch her. lol I had a scan just two weeks prior to her birth and the technician seemed to spend quite a bit of time looking at the heart. We asked her if there was something wrong and she said no. I vaguely remember her going out of the room and then coming back with someone who looked at the scan and said that everything was ok. I was sent home and nothing more was said. After Jessica was born and sent to the nursery a doctor came in and said that she heard a heart murmur but it was most likely caused by the ductus which usually closes at birth and hadn’t closed yet. We were told "DON’T WORRY" many times but just be sure and take her to her 2 week appointment. All the concern was focused on Jessica’s polydactyly – she was born with 2 extra fingers and 1 extra toe – just like ME! We had no insurance at that time so we were given the phone number to a clinic that helps children born with different types of defects. We had no idea that we would practically live at that clinic and the hospital for several years to come. Jessica and I were sent home just 24 hours following her birth. We were told that they had done a pulse-ox test and it was low but in normal range and again to take her to her 2 week appointment.

A few days after Jessica was born she was jaundice so I took her in to see the pediatrician and was sent home with instructions to put her in the sunshine for a couple of hours each day. That pediatrician said NOTHING about a heart murmur and I was quite overwhelmed with having a 2 year old, a newborn, worrying about surgery to remove the extra digits and managing apartments while trying to recover from the delivery that I didn’t even think to mention it. A couple of days after that I went back to the hospital for breastfeeding support. Jessica was having a hard time feeding and I thought it was my fault. Granted none of the support staff were doctors but none of them seemed to notice that she was breathing hard and that was why she wasn't able to eat well. Even the day before her 2 week appointment when Karl accidentally inhaled chlorine gas when working on the pool at the apartments where we were managers and we stayed in the ER most of the day to get his O2 sats up, NOBODY, even the doctors and nurses who ooohed and awwwed over our baby girl, noticed that she was in trouble! That night Jessica would latch on and frantically try to nurse. She would pull back gasping for air and then fall into a deep sleep. She would wake up again only to repeat the previous scenario. If I hadn't already had an appointment the next day I would have taken her to the ER.

Karl was still recovering from his chlorine gas experience so I took Jessica to the appointment alone and a friend took my 2 year old son, Justen for me. When the nurse weighed her I noticed that Jess had gained 2 whole pounds so I tried to calm my nerves by telling myself that she really was gaining weight so she must be fine. That calmness was shattered when the first thing that the pediatrician (different one) asked, “Is she always this blue?” He did an exam and said that she was breathing too rapidly and that the murmur was really loud. He left the room for what seemed like forever to return and told me that Jessica had an appointment with a pediatric cardiologist in 2 hours at the hospital and for me to NOT MISS IT! The doctor, who I found out was a resident, had just done his peds cardiology rotation and told me that the PC (peds cardiologist) was a really good one and very nice.

Jessica shortly after she was born

**THE OFFICIAL DIAGNOSIS**

The Pediatric Cardiologist, Richard Donnerstein, did an exam and then had a technician do an echocardiogram. Dr. D. came in while they were doing the exam and then he brought in another doctor and yet another one. In the meantime Karl was just trying to stay awake and breathe (his lungs had been burnt out by chlorine gas the previous day). I couldn't grasp what everyone was saying or what they were looking at. Jessica had been sedated for the echo and I just looked at her wondering what was going on with my beautiful baby girl.

Dr. Donnerstein finally told me that Jessica had what they called Tetralogy of Fallot which consists of 4 defects, Pulmonary Atresia and Pulmonary Stenosis. There was absolutely NO connection between her heart and lungs that they could see. They suspected that the ductus had been open to allow some blood flow to her lungs but had recently closed causing her to be in severe congestive heart failure. They needed to do a cardiac catheterization to see exactly what was going on before doing surgery TONIGHT! We were told that Jessica may not survive the night without surgery so we needed to prepare for it immediately following the cath. 

It was about 6 pm or later when Jess was finally taken in for a cath. I didn’t know if I would ever see her again because just a few years prior my mom had needed a cath and had stopped breathing during it. Thankfully she was resuscitated and was doing well but I worried that my tiny baby who was in congestive heart failure wouldn't make it. A kind nurse took me under her wing and comforted me. I’ll always be grateful that she was there.

At about 11 pm Dr. D came and informed us that Jessica was not going to have surgery after all. They found hundreds of collaterals that were taking blood to her lungs and she was so sick that she probably wouldn't survive surgery. They had started her on heart meds and would see if they could do surgery the next day. “Go home and get some rest” is what we were told, she was “critical but stable” at that moment. My heart broke as I looked over my baby who was still sedated from the cath. She looked so sick, helpless and barely even alive. In my brain I kept hearing the words, “Jessica may not survive the night without surgery” so I was worried that she still might not make it. The nurses in the PICU reassured me that they would call me if anything happened during the night so we went home exhausted and emotional. Thankfully we only lived 10 minutes from the hospital.

Our friend kept our 2 year old son, Justen, overnight so Karl and I went home to our apartment. Inside were an empty crib and empty bassinet. We held each other and cried as our world came crashing down around us.

The following day we were told that Jessica was doing much better. The heart meds were really helping her and they wanted to postpone surgery one more day so that she could get stronger. I mentioned that I was worried that she had lost some weight and I was told that it was a good thing! She had excessive fluid from the congestive heart failure and that she needed to get rid of it in order to breathe better. I remember being floored as I realized that the 2 whole pounds that Jessica had gained (she was only 6 lbs 12 oz when she was born) was all fluid from CHF.

I also remember watching her in her hospital crib later that day and she moved in a familiar way. It surprised me because I had felt as though the baby that I dreamed about for 9 months and had taken home and loved for 2 weeks had died. That baby who I dreamed of growing up healthy who would do all the fun things I had enjoyed as a child had died. The baby I brought home from the hospital with me 2 weeks ago was gone and she had been replaced by this new baby who had a time bomb inside of her. This baby would probably die too and my heart couldn't handle that.

The next several days were a blur as my husband had to go back to work and school so I had to be at the hospital during the day with Jessica and take care of my son at night. Members of my church took turns caring for Justen so that I could be at the hospital with Jessica. The story was the same every day, “we need to wait until tomorrow to do surgery because Jessica needs to gain weight and get stronger before we do it”. On Friday the doctor asked “How would you like to take your daughter home tomorrow?” WHAT??? Up until that point I was told that Jessica would stay in the hospital until she had her surgery. Suddenly they wanted to send a little time bomb home with me? ME? The one who almost let her die? We had been told that if I had waited even just one more day to take Jessica to a doctor she would have died. Now they wanted to send her home with me? I was terrified! It didn’t help that the discharge nurse said, “If you mix these two up you will kill her” when she was going over the meds with me. Ugh! Thankfully she labelled the tiny oral syringes with tags which marked the exact amount that was needed to be given & the medication name on each syringe. She wrote down the exact times that each medication needed to be given so that I would have that to refer to.

I had a hard time grasping everything that was wrong with Jessica’s heart because I didn’t even know how a heart was supposed to work in the first place. I didn’t have time to go to the library and the internet hadn’t even been invented yet. I felt alone and helpless. Jessica spent almost as much time in the hospital as she did at home the next several months. She would catch a cold which immediately went to her lungs and she would be hospitalized fighting for her life. Poor Dr. D. must have gotten tired of drawing diagrams for me and writing down words for me to study and memorize. I’m not usually a slow learner but living on no sleep, caring for a 2 year old and a very sick baby while supporting my husband who was going to school full-time and working 2 – 3 jobs was very stressful… but I eventually started to catch on. Before too long I was explaining everything to ER doctors and drawing them diagrams!

Knowledge and experience gave me the confidence I needed to make the difficult decisions for my daughter. At first I felt like God had made a mistake by sending Jessica to me. I thought that she deserved parents who had some type of medical experience or at least had insurance, but as I sought God’s guidance I came to realize how fortunate I was to be Jessica’s mommy. I’ll forever be grateful that God had more faith in me than I had in myself.

  Jessica and her older brother, Justen

Jessica had surgery on her leg

Jessica's hematoma has been continually getting worse so I took her to a doctor last week on Thursday. Actually, we weren't able to get in to see her primary card doctor so we saw a nurse practitioner. She numbed an area on the hematoma and took a sample. She wanted to make sure that Jess didn't have an infection. It seemed to be fine so she referred us to a surgeon. She said that they may want to make a small incision to drain the blood but a surgeon would have to do it.

I called the surgeon's office and they said, "You need to see our vascular surgeon" so we set up the appointment for Monday afternoon. The surgeon took a look at it and kept saying that she needed to have it drained. There was an area that had been a small scratch but Jess had kept picking at it and we were all concerned that it would become infected. Karl and I both felt that it needed to be done and when he said, "Let's do the surgery tomorrow afternoon", we decided that sooner was better than later. Jessica had been keeping me up all night every night since her appointment on Thursday because she was so afraid of what the surgeon might say! So let's just do it and get it over with. Karl called in sick so he could be there with me. Thank goodness! (Gosh I love that man!)

The surgery itself, which was Tues at 1:00pm, went well. We arrived at TMC at about 11:00am. We were so glad to have two very special visitors from our Tu Nidito group. First Amanda came bearing gifts! Jess got a gift card to Target. *woo-hoo!* She was a great distraction for Jess while I went over Jessica's medical history with the nurses. Just as Amanda was leaving, Susan came to stay with us for a while. She is also from Tu Nidito. We were so glad to have them there. Susan was able to stay with us until just after the surgeon came out to talk to us post surgery. She is so sweet to stay with us and just be there.

I spent quite a bit of time talking to the anesthesiologist about Jessica's unique cardiac anatomy. He thanked me several times for explaining it to him so clearly. He then asked me about Jessica's living will and if we wanted to waive it for this procedure. I told him no. He explained that the medication could possibly send her over the edge temporarily but could be reversed. I asked him if that involved compressions and intubation. When he said yes I shook my head and said no. You can't do that to her. So he then asked if he could talk to Jessica about it. I said sure but you have to understand that she is at about the level of a 3rd grader. So he started talking to her and I could tell that she didn't understand anything he was saying. When he was done I asked her if she wanted the tube in her throat. She adamantly said NO! "And if you do, you'll be in big trouble! And my daddy is a police officer" hehe... He then told Jess that there would be NO tube and Jess was satisfied. The doctor turned to me and I told him that I would be in big trouble if Jess were to wake up intubated "or brain dead" the doctor finished my sentence. I told him that Jessica's heart can't be repaired, her health is deteriorating and now she is experiencing bowel ischemia among other things.... there's no way she would want to survive another stroke or even wake up intubated. He seemed to really understand. Oh yeah, what was interesting is that when he was trying to talk to Jess, he started off by saying that it's very rare for him to work with people with the same type of heart problems as her because none have survived this long without being repaired. He knew she had pulmonary atresia but didn't know to what extent... when I told him that there was never a connection between her pulmonary artery and right ventricle of her heart and that there still isn't one, he was very surprised. She is my miracle for sure! I told Jessica that she is unique and special. That got me a smile.

We had to keep reminding everyone that Jessica needed a huge dose of antibiotics before the surgery and it was actually the anesthesiologist who ordered it and got it going. He was also the one to start the IV since everyone else apparently had other things to do. *gah!* I love that doctor because he took the time to talk to me about Jessica's unique cardiac anatomy and what her oxygen sats are. He really listened and cared. I don't remember his name but he is German. He had an accent but I could understand him just fine.

The surgery went well. The surgeon told us that he was able to extract a lot of blood from the hematoma. The problem is that the surgeon didn't mention to us that Jessica would have an open wound that would have to be cleaned and packed every day! He said one thing and then did another. He told us what kind of wound she had AFTER the surgery. Karl and I started asking him questions and he seemed flustered and said, "We went over this yesterday". Excuse me... we aren't stupid. We would know if he mentioned, "open wound" and he didn't say anything about it during our visit on Monday. Every time we tried to ask him questions he would say, "you are getting ahead of yourself" and kept saying, "She will be fine". So after the operation the surgeon said, "I'll have to check my schedule but I want you to come into my office on Thursday so that I can look at it and change it. I don't trust anyone but myself with this. Then we'll set up wound care" So we were sent home with the idea that his office was going to call us and set up an appointment for Thursday.

Meanwhile, Jessica has been having a lot of pain. We finally got her pain level down and she went to sleep at about 2:15am - and that's when I went to bed. At 4:00am she got up saying that her leg was really hurting and something about her bruise... I thought she had opened up the bandage and panicked. Karl and I both got up with her and the blood had soaked through the bandage. She was crying because she thought I had accused her of doing something she hadn't done. We got her settled down and Karl sent me back to bed. Karl was able to get a hold of the surgeon (at about 6:00am is when he called us back). The surgeon said, "bring her into my office so I can look at it and change the bandage. I will be there between 9 - noon." Jessica had finally gone back to sleep so Karl let her sleep (and slept a little himself) and then got her up and took her in at 10:00am. Nobody at the office knew Jessica was coming in and get this: the surgeon called in sick! What the....??? He had told Karl at 6:00am to go into the office and then turned around and called in sick - and didn't tell anyone that Jessica was coming in?!

The assistant changed the bandage and cleaned out the wound and then repacked it. By the way, after surgery, the doctor had said that the incision was only about "this" big - using his fingers to indicate about an inch long incision. Well, Karl says that the incision is about 3 inches long and that you can see all the way into the bone! Poor Jessica had no idea that it was open and she was shocked at seeing her own bone and tendons, etc. She said that is really stung when they cleaned it and it hurt really badly when they packed it. The assistant said, "I'm surprised that TMC didn't send you home with wound care. They usually do." Well, the doctor didn't set it up so how could the hospital do it? Also, the office said that the surgeon never comes into the office on Thursday and they didn't have an appointment set up to see him then either. They almost acted like we were crazy and didn't know what we were talking about. I'm just blown away at how nonchalant this doctor has been with us. Poor Jessica has been traumatized and keeps saying how her leg didn't hurt this bad before surgery. We are trying to keep her down and her leg up. She has soaked through her bandage twice already tonight. (Weds night) They gave Karl a package of gauze to put on top of their dressing so we have been doing that. We have been trying to get wound care set up through the nursing agency. If they don't have it set up then we'll have to take her back to the surgeon's office and have the assistant change it again. Jessica wants me to learn how to do it. I've always been so queasy about these kinds of things. I've had to take care of her wounds before but nothing like this. I've had to make myself NOT throw up or pass out while caring for her post heart surgery or cath and all the while praying that God gives me the stomach to do it. I have been so sleep deprived lately and emotional thinking about how Jessica got the hematoma in the first place, knowing that she doesn't have a very good quality of life in the first place and now for this to happen to her. I've been thinking about Marcus and how sick he is becoming and now I have to take care of this very nauseating wound... and hearing my daughter crying because she is in pain and has been traumatized.... I've been a wreck. Thank goodness Karl was able to take Jess in today and I was able to get some sleep. I'm feeling a bit better tonight and even dealt with her bandages.

If only we knew all the details we probably would have insisted on having a surgeon who goes to UMC and we could have prepared Jessica and ourselves for post-op care. We also would have had home wound care set up.

Tomorrow will be better. It just has to be. Good night.

Typo???

I just got a note from the new Peds Cardiologist. I'm going to have to get used to the way he does things I guess. Dr. D would call usually call me and give me the results from tests... although there were a lot of times that I would call him first. LOL I should call Dr. V because in his notes he said that Jessica's holter showed 1100 PVCs. What? Is that a TYPO or is this medication really helping???

Jessica has had multiple holter monitors since August of last year and most of them showed between 8,800 - over 10,000 PVCs. This one said 1,000! WOO-HOO!!!! I'm so happy that this medication is helping her and it doesn't seem to be aggravating her asthma. We are soooooo blessed!

Lately I've had many opportunities to talk to Jessica about having faith. It's nothing less than a miracle that she survived her 2nd heart surgery... a Cardio-thoracic surgeon stood there, looking at me in the eye and said so. It's nothing less than a miracle that she got her eyesight back after the stroke she had during that surgery. Many times she has cheated death including 4 years ago when she was having so many lung bleeds that she would carry a bowl around because she would cough up blood at any time. We prayed for answers and ours came in a little bottle of blue liquid. Morphine. It was a long shot but we tried it and it worked. Hospice was very helpful in the process of finding our miracle and it was very hard when they left us. But Miss Jess is only rarely - I mean, EXTREMELY rarely coughing up blood anymore. Jess said, "But now my heart is having 10,000 PVCs... and I told her to have faith that we would find another miracle to help her with that. I was soooooo excited when I got the paper in the mail and told her about it. I tried to tell her that 1,000 PVCs was 10 times better than 10,000. Blank stare. Being a 7 yr old in a 20 yr old body is a little hard... so trying to grasp the difference between 10,000 and 1,000 was kind of hard for her. So our conversation went something like this:

J: "Is 1,000 better than 10,000?"

Me: "Oh yes! MUCH better!"

J: "Is 1,000 better than 9,000?"

Me: "Yes!"

J: "Is it better than 8,000?"

Me: "Yes! And it is better than 7,000 and better than 6,000, and better than 5,000, and better than 4,000 and better than 3,000 and even better than 2,000!"

J: smiling from ear to ear: "Wow, that is good!"

Jess is scared of dying and also feeling very sorry for herself that she isn't like everyone else. She REALLY wants to have a boyfriend and get married. She wants someone to love her and take care of her. I've tried several different approaches with her since she has set in her mind that since she is 20 then she is old enough to have a boyfriend - so where is he? LOL So the last week or so every time she brings it up I remind her of all the miracles on her life. I ask her if she has faith. Does she really believe that Heavenly Father and Jesus really want her to be happy? She says Yes! Then she needs to have faith that they are watching over her and are going to make sure that she is happy.... but she needs to try to be happy NOW... and not waste her life away wishing for something. She needs to have faith while she waits and God will take care of the rest. (As I've mentioned here before that in our religion we believe that we will all be resurrected - but not reincarnated.) I'm positive that those who don't have a chance to marry and have children in this life will have that opportunity in the next - after being resurrected. Wouldn't that be wonderful for her to have a body that is perfect? One that can walk across the room without getting winded? One that won't need oxygen or a wheelchair? One that will be able to do anything she wants it to? So she needs to learn to have faith and patience. Oh my... that is a hard one! I've been telling God that I've learned patience, it's time to move on! hehe... I'm joking of course but I tell ya, some days I really feel like it. I've been taking care of Miss Jess - who has been very ill her whole life - for 20 years. I'm tired. BUT I wouldn't trade her for anything. I don't want her to leave ... but she does deal with a lot of pain. Every single day. That's hard.

We are so blessed though. We have so many people who love us and support us. I had better get to bed since I'm taking Miss Jess to have lunch at her fav restaurant: IHOP. Our SUV is out of the shop and so I can take her/ the wheelchair/ and oxygen to have a nice lunch with one of our friends from "Tu Nidito". FUN! I hope it's not too hot - nor rainy... the wheelchair lift if on the outside of the SUV and we don't want her motorized wheelchair in the rain!

Anyway, I want to keep up with my blog more often and not leave you all in the dark - so turn on a light already! ;)

Coming up in the next week: Brandon's birthday on Saturday, Sunday is always busy with church stuff (usually Karl and the boys go but I may get a chance to go too - IF I get some sleep before then), Monday is Brandon's actual birthday - 15 yrs old!!! (*yikes!), Tues is a court of honor - both Brandon and Austin have 5 merit badges that they worked on at scout camp and Brandon is advancing in rank!, and Wednesday Brandon has to have ORAL SURGERY! None of us are looking forward to that day. Poor Brandon - please keep him in your prayers.

Urgent prayers for our friend, Paige - and great update on Lindley

I had asked for prayers for our friend, Paige, last week who had some complications following her surgery. Paige has not done well and has been going down-hill. I just found out that she was flown to another hospital to receive a heart transplant because her heart is doing so badly. She is in critical condition and desperately needs a heart soon. My heart goes out to her family and loved ones. My friend Cathy has been updating me about Paige. Cathy had a son, Jason, who passed away under similar circumstances on Aug 9, 2001. I made a webpage honoring Jason here. Cathy and I had become good friends online before Jason passed away and we finally got to meet in person a few years later - the same time that I met Jenn (Paige's mom). Fortunately Cathy and Jenn live only about 2 hours away from each other and have been able to meet up more over the years. Cathy and her husband were able to go visit Paige just over a week ago. What great friends they are to support others when they have been through so much themselves. In fact, Cathy volunteers her time to the CHD quit project making quiltblocks honoring other children born with CHD. She made Jessica's quilt block not long after Jason passed away. Sorry I got side tracked, but I just love these families so much! I wish I could be there in person to hold a hand, lend a listening ear or give a hug when needed. I appreciate any additional good thoughts and prayers for Paige, her family and those who love her.

On a positive note, our friend Lindley did very well through her surgery and is at home! Of course she is still healing but how happy she must be to sleep in her own bed - or one of her sibling's beds! She is keeping her momma on her toes of course. Hopefully they will be able to settle in to a new routine and that Lindley will not be as nervous about everything. (((hugs))) to you sweetie! [You can read more about how she is doing on her mom's blog JennyHaHa Flaw and Disorder. ]

FIVE

Jessica has had FIVE heart surgeries.

This is a picture of Miss Jess when she was three years old post-surgery.
This was the hardest recovery of all.

First, a little background information about Miss Jess.
Jessica was diagnosed with pulmonary atresia/VSD when she was two weeks old. We were told that she had very narrow pulmonary arteries and that the heart was not directly connected to her lungs. Blood flows through a large hole between her two lower heart chambers, mixing both the oxygenated blood and the unoxygenated blood. Then the blood flows up the aorta to the body. Jessica grew some collaterals from the aorta out to the lungs and that's how she was getting blood from her heart to her lungs. Jessica was very ill and fragile so the doctors wanted her to take the heart meds to stabilize her and get her to put on a little bit of weight.

Jessica had surgery number ONE when she was five months old. The surgeon put in a shunt between her pulmonary artery and her aorta - forcing blood through the pulmonary artery in hopes that it would make the artery grow. A blood clot formed and went to her brain and Jessica suffered a stroke which weakened the right side of her body and affected her speech. She has to learn sign language in order to facilitate speech. She was very slow growing and we rejoiced at ever ounce she gained! She was delayed and didn't sit up until she was almost a year old and didn't walk until she was 2. She had OT, PT and Speech weekly for months! Pretty soon it was time for another surgery.

Jess was back in surgery just a month after her 3rd birthday.
During surgery number TWO the doctors accidentally cut the shunt and Jessica hemorrhaged out. She went 8 minutes without getting blood to her lungs and in turn no oxygen to the brain. Once she was on bypass the doctors were able to replace the shunt with a larger one... tucking it out of the way. The first shunt had been pushed into the sternum by Jessica's enlarged heart and that's how it ended up in the way and was cut. Jessica remained in the OR for hours bleeding out of control. Finally at 1:00 am we were told that she was stable enough to bring up to the ICU but she was in critical condition. She remained in the ICU for a couple of weeks experiencing more bleeding, seizures and a life-threatening blood infection. Several times she was not expected to make it. Once she was off the ventilator and was able to wake up we realized that her left side (previously her strong side) was paralyzed and she was blind. The CT scan showed 4 areas of her brain had been affected. Over a short period of time Jess regained enough vision to recognize us and was no longer terrified as she had been for several days (during which time we prayed and prayed... prayed that we would accept HIS will and that she would be comforted. HE gave her the eyesight. What a gift!) During this time is when the picture (above) was taken. It is one of two pictures that were ever taken of Jessica in ICU. I just couldn't bear to photograph her with all the tubes and wires, etc. I know that I will never forget and I didn't want pictures to remind me of how hard it was and how much she went through. Jessica had to relearn how to crawl, sit up, walk and all the other things we had struggled so hard to learn the first time. She had OT, PT and Speech again. She was very determined and regained almost all that was lost.

Surgery number THREE took place when Jessica was 5yrs old. The surgeons did what was a new surgery at the time called unifocalization of the collaterals on her right lung. Jessica suffered a pneumothorax post surgery but that resolved by it's self. Brandon was just 2 months old at that time! Thank goodness Jess did not suffer another stroke or hemorrhage.

Surgery number FOUR took place just 7 months later. The surgeons did the unifocaliazation of the collaterals on her left lung and they placed a conduit inbetween her right ventricle and pulmonary artery. The thought was that it would establish continuity between the heart and lungs and they would also be able to do a cath to measure the pressures in the pulmonary artery. Again Jess suffered a few complications but nothing as major as the prior strokes. Unfortunately this was when the surgeon came out and told us that looking at the pulmonary artery he could see that it has not grown. He felt she would never be able to have complete repair and went on to mumble something about most kids living off shunts like she has don't live past their mid-teens.... they either can't fight off an infection or they get too "blue" to survive. (meaning that's how they usually die.) He also mentioned that most kids haven't been through the strokes and hemorrhaging difficulties like her and how sorry he was that they couldn't do more. He mentioned transplant but said that Jessica would need both heart and lung transplant and with the severe difficulties she's had (strokes and bleeding), he felt that she wouldn't make it off the operating table - she would have lesions everywhere. We went home to cry. Cry and pray. Jessica came home about 8 days later. I cornered one of the pediatric cardiologists and asked him exactly how much time he thought we had left with Jessica. He had been avoiding this question and finally he looked me in the eye and said, "We never expected her to last this long, Mrs. Jensen." Since we almost lost Jess several times when she was 3 years old we were already feeling like we were living on borrowed time. So we called Make A Wish and she wanted a computer. We did some things as a family that we wanted to do. We were done. We were done with surgeries and done having children. I had been put on bed rest with my pregnancy with Brandon and I just couldn't take care of a dying child and be on bed rest again.... so we thought!

Surgery number FIVE took place almost a year after the fourth one. Jessica wasn't doing very well after the fourth surgery and I kept trying to tell the doctors that something was wrong. She seemed more "blue" and tired more easily. She was needing oxygen more often. Finally they decided to do a cath and find out what was going on. During the cath they found that the conduit that they had put in was causing more blood to back up into her right ventricle and she was in congestive heart failure big time! They had to either a) go in and close off the conduit or b) give comfort care - she was dying. The doctors left it up to Karl and myself. They were worried to subject her to another surgery and potentially causing another stroke or other complications. So we prayed again. We decided to do surgery ONLY if they promised not to open mid-line. She had been opened there 3 times already (the other surgery was under her right arm and around her back). She always had complications and it took months for her to get back to feeling ok post surgery. They agreed. She went in for her fifth surgery and they were able to go through two ribs to close off the conduit. Oh, did I mention I was 6 months pregnant with Austin when she had her last surgery? I don't think I will ever tell God what I can and can't go through again. ha! It was really something... but we did it and our sweet girl came home 5 days after surgery!

Now Jessica is about to turn 19 YEARS OLD!

What a miracle! We are so blessed.

For those who have asked, Jessica is still considered terminally ill. Her body is deteriorating and new problems are arising from the fact that her body is not able to get very much blood to her lungs, therefore not getting enough oxygen to her body. She is suffering from hypoxia. She is experiencing digestive problems, acid reflux, headaches, body aches, fatigue, depression, irritability, a lot of anxiety as well as other problems due to her body never having enough oxygen in the blood. Some things that I didn't mention here is that she is prone to developing blood clots but we can't put her on blood thinners because she also experiences hemoptysis (coughing up blood) due to the collaterals bursting and bleeding into the lungs. Fortunately the morphine therapy is keeping the hemoptysis under control. Jessica doesn't get out much but that doesn't stop her from enjoying her life as much as possible. She is at about the level of an 7 or 8 year old. She loves to color, trace pictures in coloring books, bead necklaces and bracelets, and play video games. She is a joy to everyone who meets her. She is so innocent and loves everyone. She is especially interested in animals (she has her own pet bunny - a netherland dwarf), she loves Disney Princesses, Lord of the Rings, Pirates of the Caribbean and Harry Potter. In fact, Jessica has written a book about a new character that she invented for Harry Potter... his long-lost twin sister, Mary. Of course a lot of the book rambles on and most of the spelling is wrong. On occasion you will see punctuation and random capitalizations but she wrote the book all by herself! And she kept up with it for 4 years until it was done. There are little drawings in there too. It is a book I will cherish forever. Jessica continues to amaze everyone. We don't know how much time we have left but we try to cherish each day. Each hug. Each smile. I try not to take anything for granted. Each day is a gift.

Marcus update 10-03-06 at 10:30PM

I was able to talk to my sister this evening. Marcus came out of surgery after about 5 hours. He is extubated and doing pretty well. He is responding to them but they will know more when he wakes up tomorrow. The results of the biopsy will be in by Friday but the doctors are quite sure it IS cancer. My sister and her husband are able to stay at the hospital so that is good. Tomorrow evening my brother-in-law is going to pick up his sister at the airport and take her home. She has offered to stay at their house and take care of the younger boys. What a sweetheart she is!

We are amazed at how quickly the doctors got Marcus into the hospital and into surgery! Two doctors have said that it is very agressive tumor so that makes us nervous, but they think they got it all. Of course we are praying for a full recovery and that the tumor doesn't return or show up someplace else.... One day at a time.... that's how we will beat this thing.

Thank you all for so many emails of support and prayers. I told my sister about them and she is very touched. She is going to tell Marcus tomorrow when he wakes up more. It's so wonderful to have so many people who care.

I will update here as I can.