Grace

At her 12 week ultrasound on October 2, 2012, we learned that she had a large cystic hygroma (fluid buildup behind the head/neck) that might indicate a problem with development. A blood test revealed that baby Grace has Trisomy 21, Down Syndrome.

One month after the hygroma was diagnosed, fluid began collecting in her chest and compressing her lungs and heart, and in her abdomen. It was diagnosed as "hydrops fetalis," which is a condition usually given a fatal prognosis. Ultrasound also revealed an unbalanced AV canal defect, left dominant. Grace was expected to die before or shortly after her birth due to the hydrops from poor lung development due to pleural effusions.

As an answer to prayer we believe, the fluid levels corrected themselves and Grace survived and was born 7 weeks premature in February 2013 at 4 lbs, 12 oz. The fear of her lungs being underdeveloped from the pleural effusions was relieved when she announced her birth with a loud scream and required no respiratory support or ventilation after birth. To this day her lungs are healthy and strong, a huge blessing.

Over the next several months, it become clear that Grace's heart problem was more complicated than once thought. While AV canal defects are not unusual among all heart defects, the unbalanced element creates a considerable dilemma. There has been much discernment and consultation with experts across the country to decide how best to handle Grace's situation and what type of surgery is the best option.

Her 1st heart surgery was a palliative measure, banding the pulmonary artery and ligating the patent ductus artery (PDA) to reduce blood flowing to the lungs. The main result was to help keep her lungs from being damaged from too much blood flow.

Grace's second surgery took place in August, 2013. Her PA band was then too tight so she had a bi-directional Glenn procedure done. The surgery improved her saturations but the AV canal defect (holes between both upper and lower chambers) still needed to be closed. Open heart surgery had been delayed due to persistent "heart failure" or ventricular dysfunction and frequent illness.

The situation dramatically resolved itself early in 2014 when her medications and diet were changed. Since these changes, Grace has made tremendous progress, going from a baby always seeming to be on the verge of hospitalization to one that rarely goes in for sick appointments with various specialists. She quickly moved from GJ tube feeds to needing only thin liquids given via gtube and eating solids normally by mouth.

Also after coming off all heart medications heart function was, for the first time since 2 months old, "normal" and oxygen saturations were more stable and slightly higher (even post Glenn surgery). During a heart catheterization it was confirmed that despite so many obstacles to good lung function, Grace has no evidence of pulmonary hypertension or poor lung function despite so many reasons that she should have (prenatal pleural effusions, prematurity, unrepaired CVAC and severe vomiting & aspiration). Truly a blessing that has saved her from so much suffering!

Open heart surgery to close the CVAC was done on October 29, 2014. The Glenn was left in place but the holes in her heart were fixed and 2 valves were made from the one common valve. This surgery has given Grace a "1 1/2 ventricular repair" which is a hybrid of sorts between a Fontan and a typical Biventricular repair. God willing she can stay that way! Grace's pulmonary function is great at this time and the passive flow of the Glenn through the lungs reduces the flow through her smaller right side. So far she has been doing very well with this unique circulation and no other heart surgeries are planned.

Yearly respiratory infections are a great concern. This increases the chance of lung damage which would send us back to reassess heart status and very few viable options once again. We now spare no time attempting to get her immune system working as well as possible.

Having gotten past some major heart hurdles, Grace is loving life and bringing much joy to her family and friends! We keep a FB page up & running in the event that Grace's story might be helpful for others dealing with a similar diagnosis. https://m.facebook.com/PrayForBabyGrace/

We learned so much from others walking the same path and if it was not for the encouragement of other heart parents we would have ended up with a very different set of surgeries and possibly different outcome. It is from these parents that we also learned to seek out a second or even third opinion and that what is in our child's best interest may come through a lot of prayer, research & persistence (as opposed to the first thing proposed by one's health care team at the time). Doctors, like the rest of us, have to make judgement calls. Sometimes they are spot-on and other times far from the ultimate result.

Grace's life has been in Gods hands from the start. We were grateful that she made it to birth and survived numerous surgeries. We have received so much love and joy from having her in our lives!

 

Back to Down's Syndrome Stories

-The support, information and encouragement provided by the PPFL parents is not meant to take the place of medical advice by a medical professional. Any specific questions about care should be directed to a health care professional familiar with the situation.