Haley Amber Hayes was born on November 28, 2005 at 12:34 am, just 3 hours after arriving to the hospital. The delivery went well and Haley's 5 year old sister, Britani, was so excited that she had a sister, not a brother. After a few weeks, Haley got colicky like most babies do, and despite many formula changes, nothing really helped. She seemed to outgrow it at around 4 mos. of age. Around that time Haley's Doctor was noticing that she hadn't gained much weight from 2 to 4 mos. We started going to the Dr. every 2 weeks to check Haley's weight and muscle tone. She would usually put on just enough weight to get by with, but at her 6 month appointment she wasn't even on the growth chart, weighing only 11 pounds, and the doctor was also concerned with her muscle tone. We were then sent to the local hospital for lab work. Haley's liver enzymes were very elevated and after talking with a doctor at MCV in Richmond, Virginia, both doctors decided to admit her for "failure to thrive."

Haley was admitted to the hospital on June 1, 2006. The first few days seemed like a normal home routine. Haley would eat 1-3 oz. every 1-3 hours, not being consistent with any feeding. Because she was admitted before the weekend, many tests were delayed until Monday. On Sunday the doctors ordered for Haley to have 5 oz of formula every 4 hours. After not being able to drink that much by mouth, the nurse inserted a nasogastric (NG) tube for her nourishment. On Monday Haley was taken for many tests, including an ultrasound of the heart or Echocardiogram (ECHO). When the cardiologist came back to tell us the results, he first ordered for the nurse to place an IV and then proceeded to tell us that Haley's heart was extremely enlarged and functioning very poorly. At that point, her father and I were extremely confused. Haley, for the most part, had seemed so normal and we were thinking maybe she had a simple gastrointestinal (GI) problem as to why she wasn't gaining weight. The doctor told us that many more tests and lab work would have to be done to determine the reason why her heart was like this. What seemed like a normal checkup had turned into a nightmare.

That same day, Haley began to get very sick from the high amounts of formula that were being forced into her body. She developed Congestive Heart Failure (and a bad ear infection) and was transferred to the Pediatric Intensive Care Unit (PICU) on June 5th, 2006. After getting many cardiac medicines and medicines to pull the fluid from her lungs, Haley's condition began to stabilize. A great deal of blood was also being drawn, (with many, many, many needle sticks to all parts of her body) to determine the underlying cause of her sickened heart. The doctors were giving us many genetic disorders that she possibly could have that would explain all of her symptoms, however we wouldn't know which one for days. We were able to research many diseases on the Internet but then just decided to stop researching until a diagnosis was given. On June 12th, the doctor came to us with good news and bad news. The good news was that Haley was stable enough to leave the PICU, and that they had found the reason for Haley's heart condition.

Here came the bad news. Haley had Pompe Disease. Those two words went over and over in our heads. Out of all of our research, that was the one disease we didn't want her to have. Earlier I had printed off some information about Pompe Disease and when I read the part that most infant's don't live beyond the age of 1, I put thepaper down. How could such a normal appearing child have this fatal disease? The doctor explained a little about the disease involving new clinical trials with a medicine called Myozyme. The medicine had actually just been approved April 26, 2006. Myozyme was to slow down the progression of the disease, it was not a cure, but was definitely promising. After all other initial testing, they did a skin biopsy on Haley which would give them the definitive diagnosis of Pompe Disease. Those results wouldn't be back for 6-9 weeks. Haley was then transferred back to the pediatric floor.

The medicine Haley needed was to be administeredimmediately, once MCV got approval from our insurance company and the medicine was mailed to MCV. The Myozyme arrived on June 19th and we took Haley back to the PICU so they could monitor her closely for signs of a reaction. However, after 8 unsuccessful attempts to get an IV, Haley needed surgery to place a Portacath under her skin, near her heart, that feeds through a major vein. This way she would have easier IV access and also could have most of her blood work taken through the Portacath. Haley was taken to surgery the next day, on June 20th. We were advised by the surgeons that surgery was very risky for someone with a heart condition like Haley's, so of course we were on pins and needles the whole day. Haley pulled through the surgery very well, but later that day a post-op fever set in. The Myozyme was due to be given the next day, but due to fever, it was once again delayed.

Haley began to get even sicker and looked worse than when she was admitted. She started to go into Congestive Heart Failure again due to the fluids she was given during surgery. She was sent back to the PICU on June 22 and given a blood transfusion on the 23rd. (Happy Birthday to Me.) She began to stabilize again and on June 26, she was able to get her first infusion of the gold drug, Myozyme. Once back in a normal room the next day, she started to develop another fever. Blood work showed that Haley's fever was coming from an infection in her Portacath. Doctors told us that if her fever got bad enough, the Portacath would have to be removed. However, Haley responded well to many antibiotics and we were thrilled she got to keep the Portacath. She then began to get really congested and was started on breathing treatments, chest PT (beating her chest), and was occasionally on oxygen when she slept.

The next few days the doctors were talking about DISCHARGE!! Yeah! After a month in a hospital and NOT in our own bed, we were excited! We had also only

walked out of the hospital a few times, mostly near the end of the month when Haley was more stable. We also missed her sister, Britani, so much, as she was taking turns staying with our parents, and having fun the whole time we were missing her. But oh how Haley lit up when Britani came to see her. What wonderful therapy.

More tests were ordered on Haley before she could be discharged (such as spinal X-rays, ECHOs, barium swallow, hearing test, sleep study, and many more!). We were also being taught how to care for Haley at home. She was still on antibiotics that were given through her Port and many supplies were required for home care. Haley was finally discharged on July 2nd, weighing around 12 1/2 pounds, with her NG tube still in place. Thank God I'm a nurse, because there were SO many things to be done at home. Her daily schedule was ridiculously hectic when we first got home, not to mention having appointments with different people and doctors. We had many medicines that were to be given throughout the day, including IV antibiotics through her Portacath. My husband learned a lot about her care and was able to do everything I could do. That helped a lot, because soon I was to go back to work, after being off for over a month and a half. My husband was going to school full time to be a teacher (with ONE semester left), but because of Haley's condition, has decided he is needed more at home at this time.

Haley has had 3 more infusions of Myozyme, with everything going well. We have also switched her care to Duke Hospital in Durham, NC, due to their increased awareness of infants with Pompe's Disease. We are impressed with their understanding of the course of the disease. For now we are just trying to keep Haley as healthy as possible and get back into our daily routine. We are also trying to spend as much time as we can with our other daughter, Britani. With so much going on, that's hard to do, and soon she will be going to Kindergarten!! Our schedules have allowed Britani to always be at home with both of us, so this will be a big change for her, but mainly for us.

We would like to thank everyone that has helped us during this difficult time. Whether it has been money, gifts, food, cards, thoughts, and definitely prayers, we are grateful for everything! Without our family, friends, and this special community, our existence would have been unbearable.