One Mother’s Story of Thanksgiving

By Lora Beasley

Lora and Meadow

My daughter Meadow was born with a condition so rare that I did not find another child with the same diagnois until she was 2 yrs old. It was a lonely journey filled with many unknowns, and no one to relate too. I now know a total of only 7 other children living with an extremely rare condition called basal encephalocele in the USA. All the children have some different features, but for the most part they are very similar.

Meadow in early 2007

Meadow was born September 23, 2004, after a 39 week pregnancy. Maybe mother’s instinct had prepared me that something was not going to be quite right. At about 11-13 weeks gestation I began spotting. I thought I was having a miscarriage, and my husband took me to the E.R. The ultrasound tech said there was a heart beat present, and the baby was alive. All follow-ups with the OB doctor were normal, and no abnormalities found during the remainder of my pregnancy. During the second trimester I had experienced early contractions, and by 8 months I was 4 cm dilated. I just had a gut instinct that my body was trying to tell me something.

The doctor scheduled an induction for Meadow’s birth. During labor, things took a turn once again to scare me: the baby’s heart rate continued to drop. After having an epidural it was very hard to move into a position that would bring the baby’s heart rate back up. The nurse would rush in and reposition me. Eventually I was given oxygen through a mask. When the doctor ruptured the amniotic sac, he told me I had Placenta Previa where the placenta is tearing away from the uterine wall, a condition that can cause serious complications. Nevertheless, we proceeded with the vaginal delivery without complications.

Meadow and her big sister Madison

When baby Meadow arrived, I remember the look on the doctor’s face. It was sort of a look of shock. I didn’t hear Meadow cry, and immediately I panicked. I thought she was not breathing. He told me “she has a little cleft lip.” It didn’t seem to sink in at first. I was only given a brief glance at my new baby, and she was rushed to the nursery. Meadow stayed in the nursery for 4 hours. A nurse came from the nursery and told me that Meadow was doing fine, and she was breathing without oxygen. I asked if I could please hold her; after receiving permission from her supervisor to release Meadow from the nursery, she brought her to me.

Meadow’s features were quite distinct: wide set eyes, tiny cleft lip, and a flat nasal bridge. The cleft lip in itself was very different. It was directly in the middle of her upper lip. I later learned that it is called a “true” midline cleft, and is very rare in terms of cleft lips. Perhaps the most peculiar feature was her large open cleft palate. A small pink sac was present in the roof of her mouth where her palate should have been. I had thought of every possible idea of what the soft, pink mass protruding through her open palate could have been…a lymph node, a gland, sinus stuff or just normal tissue. I had no idea what was in store for my daughter.

Meadow in March 2008

Two weeks after her birth, we traveled 2 hrs from home to a Children’s Hospital for referral to a cleft clinic. There we were swarmed by medical specialists. She saw geneticists, endocrinologists, cranio-facial surgeons, and the doctors I most remembered, neurosurgeons. I knew Meadow’s distinct features were quite different, wide set eyes, small cleft lip, flat nasal bridge, but I was not at all prepared for what we were about to learn.

Behind all these unique features lay a neural tube defect called a basal encephalocele. At some point during early gestation, the skull did not completely close, leaving a gap just big enough for brain tissue to herniate through. The encephalocele fell through the base of the skull, hence “basal”, behind all the masking features, into the roof of her absent palate. It was the small, soft, nodule in the roof of her mouth. At birth, I could have absolutely never imagined I was looking at a herniation of brain in my daughter’s mouth. This is where Meadow’s encephalocele differs from the other children I have come to know. Meadow’s encephalocele, or “herniated brain”, was visibly present in her mouth.

Meadow on her 3rd Birthday

Encephaloceles are the rarest form of Neural Tube Defect (NTD). Spina Bifida and Anencephaly account for 90% of all Neural Tube Defects. Encephaloceles make up the remaining 10%. Encephaloceles are characterized by sac-like protrusions of the brain and the membranes that cover it through an opening in the skull.

Encephaloceles are generally classified into three different categories, primarily based on location and structures involved. Occipital encephaloceles which acoount for 75% of all cases are most common and are typically found on the back of the head. Frontal encephaloceles, account for 15%. Basal encephaloceles account for the remaining 10%. Basal encephaloceles, the rarest of this rare form of NTD, are typically harder to diagnose because of their location. They are not generally visible at birth like the other encephaloceles. Basal encephaloceles protrude through the floor of the brain behind the eyes and nasal structures and sometimes, as in Meadow’s case, into the oral cavity.

Meadow at Muir Woods

Meadow had surgery to repair the encephalocele at 7 months old. The doctors took a team approach with a cranio-facial surgeon and a neurosurgeon. It was a difficult decision to make, but ultimately we knew she could not live any other way. Having the protruding brain in her mouth at 7 months old was in itself risky, but it didn’t seem to make the choice of surgery any easier. I knew I risked the chance of losing her with this massive operation into the deep brain structures. We were also told with surgical removal of the basal encephalocele, there was risk of damaging, or worse, loosing the pituitary gland. I was informed that she would most likely loose her sense of smell, as the olfactory nerves were also located at the site of the basal encephalocele.

Meadow in 2006

Twelve long, grueling hours after Meadow was taken back for surgery, the neurosurgeon came out. With a tremendous sigh of relief I learned that she was alive, and I hugged his neck. He then explained that she may have suffered some neuro deficits. I was just anxious to see my baby who had been in surgery all day. The days following the encephalocele removal were filled with tears and mixed emotions. I was so thankful that strong little Meadow had made it out of surgery alive. However, her journey was far from over. We learned that Meadow’s pituitary gland had been lost. Meadow began a series of very important hormone replacements to give her body what the pituitary gland no longer could. Her sodium levels fluctuated in critical values. Urine flowed clear like water out of her catheter, in amounts much too large for such a tiny child. Her anti-diuretic hormone was replaced through IV Vasopressin to try to obtain normal sodium levels and normal urine output. This was very critical, and labs were monitored every couple of hours.

We also learned that Meadow had suffered a stroke. I just couldn’t believe it! It didn’t make sense…babies don’t have strokes…do they? Days later Meadow had another stroke, this time affecting the opposite side of the body. She lay unresponsive for 4 days. I truly thought the second stroke had taken my baby from me. Meadow remained in the ICU on a ventilator, tubes mangled all around her little body, in and out of every orifice imaginable. Her head and face were massively swollen, and her eyelids were like balloons filled with fluid. Her head was shaved and stitches sewn across the top of her head, from one ear to the other. Meadow experienced uncontrollable seizures for 5 days during this period. She was given IV medications around the clock to try to obtain seizure control.

Meadow dressed up for Halloween 2006

Seven days following the first operation, Meadow began leaking cerebral-spinal fluid out of her left nostril. We were informed that Meadow needed to be taken to the O.R. for exploratory surgery to find the source of the CSF leak and repair it. She also needed replacement of her external CSF drain which had collapsed and was no longer functioning. I called my family to tell them they might want to come and see her. She had not responded in 4 days and had continual seizures; I was afraid this was the end. I did not think her little body could withstand another procedure. Meadow went once again to surgery, this time for 4 hours. She returned after a successful surgery in which the leak had been repaired. What a strong little baby! Who knew she would come out of surgery better than when she went in?

When Meadow finally opened her eyes, the nurse took an instrument and rubbed the bottom of her tiny foot. She got an immediate reflex. It was at this point that I knew Meadow was going to be okay, and I was going to take my baby home. Her final battle was meningitis. Thankfully, they caught it early, and administered IV antibiotics. After a one month stay at UAB Children’s Hospital, I got to bring Meadow Grayce home.

Meadow now receives a number of therapies, including physical therapy and occupational therapy for the strokes, speech therapy for her cleft palate, and vision therapy for her visual issues. She overcame the odds! The worst possible outcome that could have happened didn’t…because I still have her with me. Now we can live without the fear of the encephalocele rupturing and causing meningitis, and probable death.

With all Meadow has been through, she still remains one of the happiest children I have ever known. Her huge smile and beautiful dimples can light up any room. Meadow has taught me the true meaning of appreciation…in all things. Through her I have obtained a greater sense of compassion and patience. I have learned to deal with life a day at a time. We never know how quickly our lives can change…forever. She has truly changed my life for the better.