Gwen (2017)

Gwen's Story...

Age: 11

Diagnosis: AVSD or Atrioventricular Septal Defect (a hole between the top chambers and bottom chambers of the heart),  Subaortic Membrane, Post Pump Chorea, Mitral Valve Defect (cleft in the anterior leaflet)

Gwen was born on September 27, 2005 and at the time of her birth everything was perfect. 24 hours later an on call pediatrician heard a murmur and called in a cardiologist for a consult.  She was diagnosed with an AVSD or atrioventricular septal defect (a hole between the top chambers and bottom chambers of the heart).  She also had a cleft in the anterior leaflet of the mitral valve.  There were several other minor defects that were not life threatening.  She was surgically repaired 8/22/06 at 11 months old with open heart surgery #1.  

Over the next 3 years Gwen's heart developed a condition called a subaortic membrane.  There was also significant scar tissue on the back of the anterior leaflet of the mitral valve.  She had open heart surgery #2 on 12/8/09 in order to remove the membrane which was obstructing blood from going out of the left ventricle through the aortic valve.  She was quite fatigued and it was causing PVC's (premature ventricular contractions).

Gwen continued to improve after her second surgery, but started to build up excessive gradient pressure in the left ventricular outflow tract again.  She also had some accessory tissue on the posterior leaflet of the mitral valve and the condition was rare so her cardiologist performed a heart catheterization in order to get a better reading.   That was in April of 2014.  She went in for open heart surgery #3 on 5/7/14 to remove the accessory tissue.  When they took her off of the bypass machine and did a transesophageal echocardiogram they found out that the gradient pressure was too high.  They had to hook her back up to the bypass and go in through the aorta this time around.  The scar tissue had grown back and was thick and spongy, a tissue he called myxomatous mitral valve tissue and the surgeon was frustrated because he could not get it all without completely damaging the valve tissue.

I June 2014 I noticed Gwen was twitching and could not sit still.  After multiple visits to her doctor and 3 different neurologists it was determined that Gwen had a very rare condition called Post Pump Chorea, characterized by 3 separate types of movements:  hemiballismus (jerky, ballstic involuntary movements), chorea (an odd dance like movement) and hemiplegia (partial paralysis).  Her entire right side was affected from sight, speech and limb movement.  She underwent an MRI to determine if there was any noticeable brain damage.  She started Occupational and Physical Therapy, we went to 2 developmental optometrists, she started speech therapy.  Fortunately we saw improvements when we were told it was permanent.  

In May of 2016 we went to her regular cardiology appointment and her condition had worsened.  She was having difficulty with activity and it was determined that her mitral valve was not working properly due to the scar tissue on both leaflets and the myxomatous tissue.  On 7/20/16 Gwen had her mitral valve replaced with a mechanical valve.  Her heart was enlarged and the valve was not working  properly.  Blood was going back into her lungs and damage was imminent.   We had hoped she would have been much older as this was now going to require her to be on blood thinners for the rest of her life.  The surgeon discussed mechanical vs tissue valves with me and it was determined that the tissue valves would not be a good fit because they give out too soon and it would need to be replace every few years.

Gwen was having difficulty breathing in the hospital 5 days after her surgery and after 2 sets of x-rays and 2 sets of echos, it was determined that she had a tampanade or large clot compressing her heart and a pleural effusion (fluid in the pleural around the lungs) collapsing her right lung.  She had emergency surgery to remove a baseball sized clot from her chest and the excess fluid.

Since her surgery, Gwen has been doing well.  It is a constant battle to regulate her Coumadin levels and that requires continual blood draws and adjustments to her medication.

In the fall of 2016 we sought out an allergist/asthma specialist due to symptoms she had when she ate certain foods.   She was diagnosed with asthma and put on 2 inhalers, a high allergy to latex (developed after multiple exposures to latex during surgery) and many allergies as well as Oral Allergy Syndrome.  She is currently being monitored for both and is not able to take an epipen for fear it will affect her heart, so she needs to avoid latex.  

During the month of December Gwen felt pain and urgency when urinating and I took her to an after hours urgent care.   They felt it was a UTI after a dipstick found leukocytes, but the culture was negative.  She took a round of antibiotics which we have to be careful with because it will affect her blood thinner.  Upon recheck they said she still had it, and gave her a shot, but the culture was negative.  Eventually we made it to her pediatrician who found the UTI to be negative but there were high levels of red blood cells in her urine.  She was sent to a nephrologist who after many tests determined that all systems are working, the coumadin is not making her kidneys bleed and it is probably due to growth.  We have one more set of tests and then return in a year.  That brings us up to date health wise.

Day in the life: Gwen's hobbies and interests are far and wide!!  She just recently started to play lacrosse again (with a helmet), she loves to bake, she is a YouTube junkie which she got into when her body didn't work and when she was in the hospital and was trapped in her bed.  She is VERY social since she is an only child and really lives for her friends.   She loves to swim, be outdoors, boogey board, and have fun with her cousins on our trips to Ohio.