On July 17th, 2008 the most beautiful baby girl came into our lives, Presley Drew Shalala. It was love at first site for my husband, four-year-old daughter and myself! Presley was the most beautiful thing I had ever seen. She had big brown eyes, Angelina lips and street sweeping eyelashes!
Presley was a little blue at birth and required some oxygen at first. She got a 7 on her APGAR score and there was a debate between two nurses as to her having clubfoot or not. She also had a short umbilical cord.
Four hours later began the ride of our lives! Presley was not feeding well and was sent to the NICU for low blood sugar. We were later told she had fluid in her lungs and may have an infection, all things that were not too uncommon however, she was immediately put on antibiotics. Presley had a hard time maintaining her temp and had minor jaundice. She failed her hearing test twice as well. After 8 days in the NICU, a few scares and no answers they let her come home.
Presley was home for 2 weeks when we noticed the first seizure one night after bath time. She was lying on the bed and her left eye began to twitch. We took her to her pediatrician and he witnessed the same thing. She was once again admitted to the same hospital she just spent the first 8 days of her life in. Doctors did a CT Scan, MRI, blood work, and most importantly an EEG. The EEG confirmed the seizures, but MRI, CT Scan and blood work all came back normal.
After many meetings with many doctors, we did not feel we were getting enough answers, so after 3 days, and the work of some great close friends we were able to get Presley transferred to Sunrise Hospital, a specific pediatric hospital. There we met an amazing young doctor who took control of Presley’s situation. He knew what needed to get done and moved fast to make it happen. Another 7 day work up, cardiologist, optimologist, and the list goes on.
This time we were released with a little more information. Presley’s head measured small and there was a discussion of Microcephaly. The neurologist said he wasn’t too worried. She had a smaller than average head but not small enough to put her in that “Microcephaly” category. Presley also was diagnosed with Tracheomalacia and Laringomalacia, both are the result of under developed esophagus, which would explain her hard breathing. Presley seem to struggle to breath, every night it would keep us up and sound like we had a goat in the house…a super cute goat! Additionally, she had severe reflux, which made it hard for her to hold down her food. We had to put her on special formula. As we left the doctor this time around she was put on Phenobarbital for the seizures, a very strong anti convulsive medication and Zantac for the reflux.
We were once again sent home with very little answers. Kids have seizures we were told! Not buying this, we hopped a plane to Boston. Boston Children’s Hospital is the best in the world. Once again through some amazing family and friends we were put in touch with the best doctors. We spent a full week in Boston Children’s, from 7am till 10pm at night, never leaving her side, sleeping in chairs, taking shifts and every once in a while my husband causing havoc on the floor thinking he could do it better than the nurses. We were determined to take this opportunity and fix our baby!
Presley was now 2 months old. When we met with the neurologist at Boston we were told Presley’s MRI was anything but normal. You can imagine our frustration as we were told all along by Vegas doctors everything was normal. Hearing this news was devastating. What did it mean? What do we do to fix it? Our world was turned upside down. We had to hold strong and stay focused for Press. She needed us to take advantage of every valuable minute to make sure we absorbed every piece of information. They did not have a diagnosis for her, but according to the doctor her brain had not developed properly. They told us they didn’t know if she would ever walk or talk. This was the hardest thing I had ever been told in my life. But she is perfect I told myself, how could this have happened?
We came home from Boston and began to start everything we needed to do to maximize Presley’s development. The doctors could not give us specific answers and prognosis but we knew we needed to do everything we could to help her develop. We knew she would be delayed but we were determined to prove everyone wrong.
After a week we came home and saw a foot doctor who confirmed Presley had clubfoot. In order to fix it, they plaster cast her legs from the toes to her waist. Presley’s case was mild so the casting was done after about 2 months. What a gruesome process though. Every week they re-cast her in a more extreme position then the week before. She would cry all night until she got use to her new legs. Then when the casting was finished she got a brace with little white leather shoes that would connected her legs together and turn her feet outward. She needed to wear brace for 22 out of 24hrs a day.
At four months old I took Presley to Cedars-Sinai in LA to see a Geneticist. This was actually our second time to Cedars. My husband and I took her at about 2 months (after Boston) to see a Nero surgeon. He told us right away there was nothing that could be done for her. The Geneticist said he has seen kids like Presley before and they don’t do well. He told me they are easy to take care of as long as the seizures can be controlled with medication. This guy needed a smack and almost got one!
One week later I took Presley back to Boston to do a follow up MRI. This time I had to go alone. My husband had to stay home with Jaden and couldn’t take off anymore work. The bills were beginning to pile up. Presley was just 5 months at this point. After this trip we came home with a diagnosis, Polymicrogyria. She had it bilaterally. The perplexing thing with Presley is that her genetic make-up was all normal, these babies usually have a deletion on the #22 chromosome and Presley did not, so she was put into a sub category. Another perplexing scenario we constantly battled with. Still unsure of Presley’s hearing, she got little baby pink hearing aids at 6 months of age. First few days she was responding to everything. Such a minor step forward but a huge reason to celebrate!
The next few months were filled with weekly physical therapy sessions. We found Missy Cowan…the most amazing Physical Therapist. She would come to the house and work with Miss Presley on the yoga ball and matt. Presley would always protest with whimpers of annoyance, but Missy was able to make great progress with her.
During this time we were always switching anti-seizure medications. One medication would get a seizure under control, Press would develop nicely for a few weeks, then her brain would re wire again and start a new type of seizure. We were always trying a new medication keeping our fingers crossed every time.
Part of her doctor’s visits included an Opthamologist. After being told Presley could see, at about 8 months old we were told Presley had cataracts in both eyes and was going to need surgery. Another strange diagnosis no one could understand, infants don’t get cataracts?
This time we were off to LA Children’s Hospital. There was only one surgeon that could operate on babies this young. We were back and forth to LA for 3 weeks. They can only do one eye at a time with a week rest period in between.
The weeks following Presley’s eye surgery were extremely difficult. She started a new seizure type, most likely from the stress of the surgery. This was something we were used to dealing with, but these were violent and scary to watch. I would just hold her real tight and try and talk to her until they were done. She would always let out a HUGE cry when they ended. They were so scary for her.
With her neurologist 2500 miles away I was desperate for someone closer. With a seizure disorders they can have up to 3 different kinds of seizures at once and a seizure type can change at any time for any number of reasons.
We found a wonderful local neurologist willing to step outside the box for us. We started Presley on a steroid called ACTH. It’s a very expensive drug, but insurance company’s can’t refuse it, usually because it’s considered a last ditch effort. When you have tried as many drugs as we did and nothing controlled the seizures we felt this was our best option. There were risks involved but we decided as a family the benefits out weighed the risks.
The next two months an in home nurse came to the house to administer Presley the steroid. She would get a shot in her thigh in the morning. When we would travel Dad would do the shot, which made me nuts as you can imagine. We had to have her blood drawn regularly to make sure the steroid wasn’t damaging her liver or kidneys.
The steroid worked wonders for Presley. She had her 1st normal EEG of her life during the two months she was on the steroid. She was making vast improvements in her Physical therapy, and eating like a champ. She would smile and roll over. I was the happiest mom on Earth! Yet the reality was she couldn’t be on a steroid forever.
The week of Presley’s 1st Birthday she had her last injection. With in 24 hrs the seizures were back in full force. She was struggling to suck on a bottle and was back to sleeping a lot.
One week after Presley’s 1st Birthday on July 26th, 2009 Presley became an angel. During her short time with us, she faught so hard and gave us glimmers of hope that one day she would beat this. We could not have prepared for this. We spent a year chasing answers all over the country. In my heart there would always be an answer and a fix to whatever ailment my daughter had.
My heart goes out to all those parents going through similar struggles on a daily basis. PMG is becoming more common and research and treatment is limited. If there is any way to make your path a little easier, that is our goal!
back to top^