Sunday, January 27, 2013

52 Days

There is no place like home! And no better way to arrive home than to a sweet 4 year old screaming Daddy! Momma! and running across the airport to meet us and give us the enormous parrot shaped helium balloon she had picked out for us.  It was only 3.5 days - but it felt much longer to us!  We now have 52 days until we leave for the 2-3 month trip for Jacob's birth. 7.4 weeks, 52 days, 1248 hours.  Let the mental preparation begin.  Over the next few weeks we will be moving our spare room downstairs and completing Jacob's room because we are going to beat this heart defect and bring our baby boy home.  It may be June before he comes home, but he is absolutely coming home.

I'm feeling much more positive than I was when I wrote my last post.  Being home with Georgia, the amazing amount of support and love we are getting from our family and friends, and time to process has made a big difference.  I want to say thank you to those who keep reminding me - over and over again - that God will take care of Jacob.

I'm still tired of telling people that I am "fine" - I'm not fine.  But I am doing much better today than I was yesterday, and that is what I'm focusing on.  Jon is also doing much better today - we were quite the depressing pair over our time in Toronto, which culminated with me having a mental breakdown while on a packed plane that was about to land in Halifax.  Thankfully the other passengers did a good job of pretending to ignore the hormonal, crying, pregnant lady.  And my husband has a really good set of shoulders to cry on.

Jacob is now 2 lbs 13 oz, and has started getting the hiccups 2-3 times a day.  It's cute.  I can't wait to see his beautiful face.

Friday, January 25, 2013

Double Inlet Left Ventricle - DILV



Double inlet left ventricle

Definition

Double inlet left ventricle (DILV) is a congenital heart defect that affects the valves and chambers of the heart. Congenital means it is present from birth. Babies born with this condition have only one working pumping chamber (ventricle) in their heart.

Alternative Names

DILV; Single ventricle; Common ventricle

Causes

DILV is one of several heart defects known as single (or common) ventricle defects. People with this condition generally have a large left ventricle (the pumping chamber of the heart that supplies the body with blood), and a small right ventricle (the pumping chamber that supplies the lungs with blood).
In the normal heart, the right and left lower chambers (ventricles) receive blood from the right and left upper chambers (atria). Oxygen-poor blood returning from the body flows to the right atrium and right ventricle. The right ventricle then pumps blood to the pulmonary artery, which carries blood to the lungs to pick up oxygen.
The blood, now oxygen rich, returns to the left atrium and left ventricle. The aorta then carries oxygen-rich blood to the rest of the body from the left ventricle.
However, in those with DILV, only the left lower heart chamber is developed, and both upper chambers carry blood into this ventricle. This means that oxygen-rich blood mixes with oxygen-poor blood. The mixture is then pumped into both the body and the lungs.
DILV can occur with transposition of the great vessels (in which the aorta arises from the small right ventricle and the pulmonary artery arises from the left ventricle), or it can occur with the arteries arising from their "normal" ventricles. Blood flows from the left to right ventricle through a ventricular septal defect (VSD) -- a hole between the chambers.
Double inlet left ventricle occurs in about 5 - 10 of 100,000 live births. The problem most likely occurs early in the pregnancy, when the baby's heart develops. However, the exact cause of DILV is unknown.

Source: http://pennstatehershey.adam.com/content.aspx?productId=117&pid=1&gid=007327

New Diagnosis


We have completed a day of appointments at Sick Kids and Mount Sinai hospitals here in Toronto – and what a day! My head is still whirling and fuzzy from the information overload and the changes in our diagnosis.  Jacob now has a new diagnosis – it is NOT Hypoplastic Left Heart Syndrome.  Jacob’s new diagnosis is Double Inlet Left Ventricle (DILV) – it is still a single ventricle diagnosis, but the overall prognosis is about 10% better than it was with HLHS.  Essentially, Jacob’s left ventricle is normal sized and functioning well, but his right ventricle is small and not functioning normally.  I will post a technical explanation and diagram too.  DILV is even rarer than HLHS – the fetal cardiologist told us that at Sick Kids they might see 1-2 cases/year.  It is not genetic, and there is no known cause.  DILV occurs is about 5-10 of 10,000 live births.  That’s the good news. 

The other news is that Jacob still needs the 3 stage Norwood surgery and although the initial surgery will be slightly different it is at the same risk level as originally thought.  So, we are still facing surgery in the first week of life, at 5-6 months and at 3 years – all at Sick Kids.  I am feeling thankful for the new diagnosis, because anything that gives my boy a better chance at life is wonderful.  At the same time, Jon and I are feeling slightly devastated by a new statistic that was introduced by the fetal cardiologist – We were told that approximately 75% of children with DILV live past the age of 10.  With HLHS, that number is 65%.  3 out of 4 – I guess those are good odds? I can’t bring myself to even consider the 1 out of 4 option.  I don’t know if I would survive that. 

Sick Kids itself is an amazing hospital – and I say that after having spent only one day there talking to the doctors, nurses and social workers.  The level of care that we received today was phenomenal, and each specialist took a large chunk of time to do the assessment, and then sit with us afterwards to discuss the results and answer any of our questions.  We left the hospital today with the cards for the fetal cardiologist, the fetal heart nurse, the obstetrician and the social worker – and with assurances from each one that we could email or call anytime if we had questions or concerns.
 
But right now…all I want is to hold Georgia and kiss her sweet face.  I want to take my time playing with her, and talking to her and to ignore the mess of the house or the busyness of work – that is completely not a priority for me now.  I just got a virtual hug from her thanks to Skype, but tomorrow I’m going to squish her when I see her at the airport. 

Please, please, please keep praying! There is a long road ahead for baby Jacob, but I still believe in my heart that he will make it and will have a relatively normal and happy life – for however long we are blessed with his presence.  

Thursday, January 24, 2013

Morgan's Story - Hypoplastic Left Heart Syndrome Video Series | The Children's Hospital of Philadelphia

I watched this video while eating breakfast this morning, and am taking comfort in the final lines of the video - This is from the Children's Hospital of Philadelphia, but the procedure is the same.

Morgan's Story - Hypoplastic Left Heart Syndrome Video Series | The Children's Hospital of Philadelphia

Wednesday, January 23, 2013

Toronto, aka Mars

We are in Toronto...the first of many trips over the next few years. And I have to say, Toronto might as well be on Mars for how alienated it makes me feel! The flights were fine, baby Jacob seemed to be awake and active the entire time - maybe he liked the noise and altitude! The pilot sitting next to me asked where we were going on vacation - and after I answered him he didn't say another word to me for the whole flight. I simply said that we were going to Toronto for doctor's appointments - but he apparently couldn't come up a response to that. I miss my sweet Georgia. We talked to her on the phone when we arrived, and she sounds happy and content with her grandparents. Thank you God for providing good caregivers for our girl while we can't be there. We are here with a day to spare, because we didn't trust the weather to let us out of the province. My appointments are on Friday morning, so tomorrow Jon is working from the hotel room and I'm doing nothing - not feeling comfortable enough to wander around downtown Toronto by myself. My mind and heart are very anxious. I'm not sure what to expect at Sick Kids on Friday, or what news we are going to hear after the ultrasound. It is incredibly unfair that we are here, and not at home enjoying a normal pregnancy. But there is a master plan that I can't see or understand. And with the amount of support from our family, friends and church family, Jon and I don't feel alone. Thank you for that.

Monday, January 21, 2013

Saving the Tiniest Patients

Jacob's Bracelet



Andrea from http://www.karlandedesigns.com/ has customized her Heart and Stroke Foundation bracelet to make Jacob's Bracelet - pictured above.  It's made with swarovski crystals, and is absolutely beautiful.  I have been wearing mine with pride every day since I received it.  Currently there is a limited supply available through Vanessa Pretty, but soon the bracelet will be available for order on the website.  If you live in our area, you can save the shipping fee by picking it up from Vanessa. Sizing and price is listed below:

Sizes
Sm 6 3/4"
Med 7 1/4"
Lrg 8"
$47.82 + tax

To order contact Vanessa at vanessa_pretty@nf.sympatico.ca , and I will post the website link when it becomes available.

15% of the proceeds will be given to us to help with our journey to Toronto for the surgeries.  Today we were told by the social worker that we will not be able to go on the wait list for Ronald Mcdonald House until the day Jacob is actually born.  So we are looking at a minimum stay of a month (but likely longer) in a hotel or other accommodation.  On the day that Jacob is born we are to seek out the social worker at Sick Kids and start our application - this really frustrates me because obviously the LAST thing on my mind that day will be finding a social worker and filling out a form.  I cannot imagine myself being willing to leave my sweet baby boy on the day of his birth to handle something like that.

We are now anxiously waiting for our trip to TO - the first trip, for the ultrasound and to meet the surgeon and find out exactly what will be done with Jacob's heart in the first surgery.  We leave on Wednesday, hopefully with no delays from snow.  I will keep you posted.

Thursday, January 17, 2013

He is able


I’m sitting in my living room, feeling my baby boy kicking at the laptop where it connects with my growing belly, and I know that I am blessed.  But at the same time, I know that life has thrown my family a curveball that we could never have prepared for.  I feel the need to say thank you, thank you, thank you to every single person who has taken a moment of their time to say a prayer for baby Jacob or to book a picture session with Vanessa, to give freely of their own money to make a donation for us, or to stop and say an encouraging word to myself or Jon.  

It is my own personal struggle not to place blame on myself – when I know, and the doctors have told me over and over – that this heart defect has no known cause.  It is not caused by genetics, or by the food I eat, or the face cream I use, or that moment in time when a client blew cigarette smoke into my face…all these and many more scenarios have crossed my mind.  Tonight my daughter went to Singing Company for the first time at our church – and although she was too shy to sing a word, I was given a quiet reminder as the children sang the words to a song that I myself have been singing for years. 

He is able, more than able
to accomplish what concerns me today.
He is able, more than able
To handle anything that comes my way

I need to hold on to my belief that God is in control.  If I lose sight of that, than this journey will become too much to bear.  I have another 12 weeks until baby Jacob’s due date – and for the time that he is inside my belly he is safe and healthy.  So Jon and I have 12 more weeks to cherish this treasure and prepare for the weeks following his birth.  I need to enjoy this time, and take care of all the elements that are within my control so that I can give Jacob the best possible chance at life.  Then I have to sit back and trust God and the Doctor’s to do the rest.  I confided in someone this week that lately I often feel that I am swinging back and forth between hope and fear – back and forth like a monkey on a vine…Tonight I am choosing to hold on to hope, and to not allow the fear to take root.  My baby boy is perfect just the way God made him, and when the Doctors are able to fix his little heart than he will become an even greater blessing than he already is.  I love you baby Jake.  Hang in there.

Monday, January 14, 2013

Pretty Pictures Mini Session Fundraiser

Pretty Pictures is doing a fundraiser on our behalf - the poster is above.  If you would like to book a mini session please contact Vanessa through the Facebook Page "Pretty Pictures" or at vanessa_pretty@nf.sympatico.ca


The first trip to Toronto...


Since Jacob’s diagnosis I have been having an ultrasound every 2-3 weeks at the Maternal Fetal Assessment Unit and with the Pediatric Cardiologist.  At our last two ultrasounds the cardiologist has told us that from looking at the baby’s heart, she thinks two positive things are working in Jacob’s favour.  First, it looks like there is a hole in his heart between the two chambers that is allowing some blood flow (with standard HLHS there is no blood flow in this direction, or very minimal).  Secondly, it appears that the main aorta is larger than originally thought.  These two factors combined, if they are indeed the case, would make the first surgery slightly less risky because less reconstruction may be necessary.  We were strongly cautioned by the cardiologist that she did not know this for sure – the only way to be sure is to go to Toronto to see the specialist at Sick Kids and have further testing done.  So, we leave next week for a three night trip to Toronto to have a fetal echocardiogram and ultrasound at Sick Kids.  This testing day will determine if these two possibly positive factors exist or not.

These two ultrasounds that had potentially positive news came after we had reached out to our families, friends and church for prayer on Jacob’s behalf.  Each time I sat with the cardiologist and she had something good to say, there was a strong voice in the back of my head saying – this is happening because of all the prayer, and all the faith that we have in the Great Physician.  I have one more ultrasound before leaving for this short trip to TO – please pray that the cardiologist continues to see these positive elements in Jacob’s heart.    

Sunday, January 13, 2013

Hypoplastic Left Heart Syndrome


What is Hypoplastic Left Heart Syndrome?
Hypoplastic left heart syndrome (HLHS) is one of the most complex cardiac defects seen in the newborn, is uniformly fatal without surgical intervention and remains probably the most challenging to manage of all congenital heart defects. It is very rare, diagnosed in only 600-800 births annually.

In a child with HLHS, all of the structures on the left side of the heart (the side which receives oxygen-rich blood from the lungs and pumps it out to the body) are severely underdeveloped. The mitral and aortic valves are either completely "atretic" (closed), or they are very small. The left ventricle itself is tiny, and the first part of the aorta is very small, often only a few millimeters in diameter.This results in a situation where the left side of the heart is completely unable to support the circulation needed by the body's organs, though the right side of the heart (the side that delivers blood to the lungs) is typically normally developed. Blood returning from the lungs to the left atrium must pass through an atrial septal defect (ASD) to the right side of the heart. The right ventricle must then do a "double duty" of pumping blood both to the lungs (via the pulmonary artery) and out to the body (via a patent ductus arteriosus (PDA)). The patent ductus arteriosus, a normal structure in the fetus, is often the only pathway through which blood can reach the body from the heart. When the ductus arteriosus begins to close, as it typically does in the first days of life, the blood flow to the body will severely diminish resulting in dangerously low blood flow to vital organs and leading to shock. Without treatment, Hypoplastic Left Heart Syndrome is uniformly fatal,
often within the first hours or days of life.


What is the treatment for Hypoplastic Left Heart Syndrome?
The management of the newborn with Hypoplastic Left Heart Syndrome can be divided into the initial stabilization period and the operative / post-operative period. Even while diagnostic tests may be going on, the rapid stabilization of infants with Hypoplastic Left Heart Syndrome must begin as soon as the diagnosis is suspected. Catheters are placed, usually in the umbilical blood vessels, which allow medications to be given and blood to be obtained for testing. An infusion of prostaglandin, a medication that prevents the patent ductus arteriosus from closing, is begun, thus maintaining the pathway for blood to reach the body from the right ventricle. Even though the infant may have low oxygen saturations, supplemental oxygen is avoided since it tends to promote more blood flow to the lungs which may steal blood flow from the body and place excessive demands on the already stressed single right ventricle.

Manipulations of medications and respiratory treatments (including possible mechanical ventilation) are performed to optimally balance the flow of blood to the body and the flow of blood to the lungs. Close monitoring is essential to detect any organ dysfunction and maintain cardiopulmonary stability because infants with this anomaly may be very unpredictable and undergo quite sudden changes. There are essentially three treatment options that have been proposed for children with Hypoplastic Left Heart Syndrome. In the past, due to poor outcomes with available treatments at that time, no treatment was often recommended. Today it is rare that a family may choose not to treat a child with Hypoplastic Left Heart Syndrome, though in cases when the infant is unable to be satisfactorily stabilized no treatment may be advised.

Cardiac transplantation in the newborn period is performed as primary treatment for Hypoplastic Left Heart Syndrome at some centers in this country. While transplantation has the advantage of replacing the very abnormal heart of a child with Hypoplastic Left Heart Syndrome with one of normal structure, this treatment is limited by the scarcity of newborn organs available for transplantation and the life-long need for anti-rejection therapy. Additionally, although outcomes for transplantation continue to improve, and the incidence of rejection is lowest in patients transplanted as newborns, the average life span of the transplanted heart is limited (currently less than 15 years). The most commonly pursued treatment for Hypoplastic Left Heart Syndrome is "staged reconstruction" in which a series of operations, usually three, are performed to reconfigure the child's cardiovascular system to be as efficient as possible despite the lack of an adequate left ventricle. These surgeries do not correct the lesion, and are instead considered "palliative".
The first operation in the staged approach is known as the Norwood operation and is typically performed in the first week of life. With the Norwood operation, the right ventricle becomes the systemic or main ventricle pumping to the body. A "new" or "neo" aorta is made from part of the pulmonary artery and the original, tiny aorta, which is reconstructed / enlarged to provide blood flow to the body. Finally, to provide blood flow to the lungs, a small tube graft is placed either from an artery to the lung vessels (called a modified Blalock-Taussig shunt) or from the right ventricle to the lung vessels (called a Sano modification). Because of the extensive reconstruction of the aorta that must be done, this operation is one of the most challenging heart surgeries in pediatrics. The subsequent operations in the staged reconstruction plan are the bi-directional Glenn procedure, typically done at 3 to 6 months of age, and the Fontan operation, typically done in children older than 2 or 3 years. These operations are described in more detail in the Heart Encyclopedia chapter on "Single Ventricle Cardiac Anomalies."

The Norwood operation is the most complex and highest risk procedure in the sequence of staged reconstruction for Hypoplastic Left Heart Syndrome. Current management at major pediatric heart centers has resulted in survival rates of 75 percent or better. The recovery period in the hospital following the Norwood operation is often unpredictable and complicated, averaging about 3 to 4 weeks. A small percentage of patients who leave the hospital may continue to experience significant problems in the first months of life. Occasionally, the right ventricle does not function well following the Norwood operation and in some case, cardiac transplantation may need to be considered.

If a child with Hypoplastic Left Heart Syndrome reaches the time for the second stage (about 4 to 6 months of age) without major complications, the survival through the Glenn and Fontan operations are much better, exceeding 90 percent with current methods. Almost all children with Hypoplastic Left Heart Syndrome will continue to need some cardiac medications to maximize the efficient function of their heart, and all will require regular periodic follow-up visits with their cardiologist to evaluate their cardiac function and detect late complications such as arrhythmias.

While the prognosis for the series of surgeries has greatly improved over the past 20 years, HLHS children still have a long road ahead. Life expectancy to date is unknown, but there are many early patients already in their mid 20's and enjoying life.

Source: http://www.hopeforbabybennett.com/p/hypoplastic-left-heart-syndrome.html

Baby Jacob's Diagnosis


I decided to start this blog as a way to keep friends and family updated on baby Jacob’s progress.  If you are reading this blog and you have a baby with the same diagnosis, please feel free to follow along on our journey and don’t hesitate to make contact with me.  It has been 7 weeks since Jacob’s diagnosis, and there have been many hard turns and learning curves in that time.

Our little family of 3 had been through a lot in 2012, and we were glad to see the calendar turn to 2013.  This year will hold many challenges for our family, but we have hope and faith that God will see our little boy through this year and many more to come.  It all began at my 19 week ultrasound, when the technician was having difficulty getting a clear picture of the baby’s heart.  We were asked to come back a week later for another ultrasound, and left with no indication that something was wrong.  At my 20 week follow up ultrasound it was a much different story.  I remember lying on the examination table while 2 technicians, a doctor and a resident all peered at the screen, trying to make sense of what they were seeing.  No one was communicating with me or Jon as we waited, but it was clear from the demeanor of those looking at the screen that something was wrong.  By this time I was crying, the tears were just pouring out, and still no one was communicating with us.  Finally, that examination ended and the doctor gave us a minimal amount of information – our baby was a BOY! and the left side of the baby’s heart was much smaller than the right side.  The term the doctor used was Hypoplastic Left Heart Syndrome, and we were told to come back two days later to see the Pediatric Cardiologist, Janeway Genetics and the Maternal Fetal Assessment Unit. 

The visit two days later is forever etched in my mind as one of the most painful days of my entire life.  We received an overload of information on HLHS from each specialist, and I had an amniocentesis to rule out chromosome abnormalities and other defects which would make Jacob’s diagnosis instantly fatal.  Termination of the pregnancy was suggested as an option by each specialist – and each time we turned around and told them that it was NOT an option for this baby.  We were told that 60% of couples who receive this diagnosis terminated the pregnancy and that if we were to terminate we only had 5 days left where that was an option.  We left that appointment knowing three main things: 80% of babies born with HLHS survive the first three surgeries, the list of possible complications for our baby was horrifically long, and that we would have to head to Sick Kids in Toronto to have the baby.  Jon and I were devastated as we walked out of the hospital.  All we wanted to do was go home and wrap our arms around our daughter Georgia and never ever let her go. 

We spent the following week asking impossible questions: Why is this happening to us? How are we going to deal with this? How are we going to handle the financial implications of this? And my own personal battle – What did I do to cause this to happen?