Angelica's Story--Chiari 111
Our journey started in February, 2009, when I was scheduled for a test that involved radio-active material, so I had to make sure I wasn't pregnant before I went in for the test. I had been very ill and things hadn't been working as they should, so imagine my surprise when it was positive. Two weeks later I was diagnosed with Type 1 diabetes and there was a 50/50 chance that our baby would have some serious birth defects.
Our first ultrasound at 10 weeks didn't show that there was anything wrong. When we went back for our follow-up ultrasound at 16 weeks I could see that something wasn't right. This was our 12th pregnancy and I'd seen enough ultrasounds to know that something was wrong with the head. After a quick review, another doctor came in and told me that our daughter had anencephaly and that the ultrasound was over and I could go home.
I didn't know anything about anencephaly, so I went home and did some research on the internet. I was devastated. I had survived my illness just to find out my pancreas no longer functioned and I had diabetes and now this? By the time of my normal OB appointment I had gathered all of my baby items, had a rummage sale then delivered the rest to a local crisis center. My heart was so torn that I swore to my husband that I wouldn't have any more children. Being Catholic and having lived life accepting life on God's terms, this was obviously a rash and emotional decision on my part. After all was said and done, however, because of my health I am no longer able to have more children.
Our Physician set up an ultrasound with a specialist and a genetic counselor at 20 weeks. Turned out that the ultrasound technician was the wife of one of the deacons at our church. That immediately made me feel more comfortable. Then another pleasant surprise, our daughter didn't have anencephaly. She still had a terminal birth defect, Chiari III Malformation, but it wasn't lethal. Dr. O'Day was all prepared to discuss anencephaly with Tony and I. As for Chiari III, she had a single paragraph in her medical book. Because of the rarity of the defect, there just wasn't anything more to find. Chiari I, II and IV had definite information, but Chiari III seemed to be that mystical stage where little was definite.
We did decide to have the amniocentesis done to determine the possibility of other issues or heredity issues for our other children. Everyone around us knew that our pregnancy was going to go to term and we wanted to be as prepared as possible. Through the amnio we discovered that we were having a girl. We decided to name her Angelica Joy.
Next we met with a children's neurologist. The info he had for us was the same info we had found on the internet. He was able to expand on it more which was helpful for us to understand what was happening to our daughter. Many things were dependent on what was in the encephelocele (bulging sack formed at the base of her skull where the neural tube didn't close). He explained that the malformation of the brain was degenerative and that after her birth, the brain would begin deteriorating until it stopped functioning. We, without a doubt, now understood why there was nothing that could be done. It would be like stopping the aging process. Our bodies age and degenerate until they give out. There was no way of knowing how long she would live, or even if the breathing and heart-beating functions of her brain would even work after her birth.
At her next ultrasound I found out that both her brain stem and her cerebellum were in the encephelocele. This brought us to the reality that she probably wouldn't survive outside the womb. We had already made the decision to AnJoy as much of life as possible while she was still with us. The news made that decision more necessary. We wanted to create special memories during our pregnancy so we could look back and say, "We did that with Angelica."
From the 16-week ultrasound, we had been open with all of our children as to what was happening with their sister. When it became apparent that she wouldn't be coming home, they were all for creating those special memories. Tony and our two oldest boys took a helicopter ride together. As a family we visited the Extreme Makeover: Home Edition build site in Wisconsin during the wee hours of the morning. As a family, we built her casket and planned her funeral. We developed a birth plan and invited a few close family members to be with us at the hospital for her birth. The hospice team helped us a great deal during the last few months of our pregnancy as well as providing us with a professional photographer at the hospital.
Around 6:30 p.m. of the second day of trying to induce labor, success! I let Tony know that it was time to call the family to make sure they had time to gather everyone up and be at the hospital. We walked the halls until the photographer arrived at 8:40. Angelica Joy was born at 8:50 p.m. blue and not breathing. Photographs were taken immediately after the cord was cut with Tony, myself and Angelica. A wonderful nurse wrapped her up and cleaned her face, handed her to Tony to bring her to the family that was waiting to see her. The doctor informed me that she had passed, but 20 minutes later when Tony returned she was breathing and her color was much improved. (Angelica was now the 31st live birth of a Chiari III case in the U.S.) Everyone gathered in my room and Fr. Tom Foster performed her baptism.
I held Angelica through the night and after the MRI the next morning she didn't look very well, so we gathered everything up and took her home with the prognosis that she wouldn't make it through the day. In the weeks to come, our home was full of love, family, friends, neighbors and anyone who wished to visit with our little saint before she left this earth. Five months of weekly birthdays were celebrated in our humble abode.
Angelica was unable to maintain her body heat for the first 2-1/2 months, she was never able to cry, by Christmas she could no longer see, her movements were just wiggles, she was unable to nurse because of her lack of ability to suck, but she was incredibly content and rarely appeared to be in any discomfort. I slept in a la-z-boy for those first 2-1/2 months to keep her warm and because she didn't cry I relied on her wiggling to wake me when she needed to eat through the night. When she started sleeping through the night we wrapped a heated blanket around her bassinet to keep her warm as she slept.
She was home with us through everything. Hospice visited us weekly with love and compassion. When the seizures started they versed me on the medication, but the seizures were never very long with most just being a second or two. On Tuesday, February 9, 2010, Angelica fell asleep for the last time. In those final days Angelica was surrounded with family and love. Thursday she received the Annointing of the Sick and a blessing from our Priest, Fr. Kuriakose Nediakala and Shirley Baker, DRE. Sunday was our church's children's Mass, so we took this opportunity to bring Angelica to church with her siblings one last time. Late Sunday evening we called our hospice team because she had stopped breathing in my arms. I held her for some time, then Tony took her for one last dance and she suddenly gasped for breath. We prayed and talked with our little girl. We let her know that we'd be just fine knowing that she was in the arms of our Blessed Mother and Our Lord Jesus. 7:00 a.m. on Monday morning our son's girlfriend, Kelly, came to the house to have one last opportunity to hold Angelica because she was going out of town for the week. Shortly after she left our home, Angelica passed away in Tony's arms at 9:36 a.m.
Fr. Tom Foster who helped us start our journey with Angelica at the hospital came to our home with Deacon Ted Windus to administer last rites for Angelica. We kept Angelica with us until all those who were at the hospital with us when she was born had a chance to come and say a final good-bye before we called the funeral home.
At her funeral our church was overflowing with people and love. She touched the lives of an incredible amount of people through our Caring Bridge site. Her case was so rare that she was the awe of a great many more. She taught us to slow down and AnJoy the moments that we have.
From Angelica's Obituary:
Angelica Joy Salls, born Sept. 15, 2009, was received by Heaven on Feb. 15, 2010, from the arms of her parents. Diagnosed with a rare neural tube defect, Angelica was not to survive birth. She was born and baptized surrounded by family. This cocoon of love surrounded her throughout her 21 weekly birthdays and ended with her celebrating her five month birthday with her brother, Stephen, and the angels.
Angelica, messenger of God, you have taught so many to "AnJoy the Moment," to celebrate and embrace life with courage. We will AnJoy life with you in spirit. Your parents: Tony and Kristi Salls; your brothers: A.J., Thomas, Joshua, Tris, Lucas and Michael Salls; your sisters: Patricia, Brittany, Bethany and Rebekah Salls all of Duluth, MN.
With our love and thanks for allowing us to share our story,
Tony & Kristi Salls
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-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.