Matthew’s Story

Matthew Evangelista was born on October 14, 2001 at Flushing Hospital in Queens, New York. Premature at 34 weeks, he weighed in at 6 lbs and 2 ounces. At birth, Matthew was found to be extremely anemic, jaundiced and in respiratory distress. Doctors informed the family that Matthew was suffering from a condition known as Hydrops fetalis and explained to mom that this was a life-threatening condition and that Matthew was living on a day to day basis.

Matthew stayed in the neonatal intensive care unit for 3 months after his
birth. During his stay, he continued to have problems with his liver, spleen,
and breathing. He had multiple surgeries and was tested for multiple disorders.
Nothing had come up positive except for a thalassemia test. He was
subsequently discharged home after this three month stay. He was not without
problems, however. The months following his discharge he was admitted several
times for blood transfusions, respiratory problems, and was even recommended to
receive a liver transplant for liver problems. Fortunately, he responded to
liver medication and did not require the transplant.

In these first few months of life, he was slowly reaching developmental milestones. Although he did not reach them as quickly as others, he was crawling, sitting and standing with assistance from early intervention specialists, physical therapists, and occupational therapists that visited his home weekly. He was always a very happy baby, despite all the hospital admissions and respiratory problems.

In March of 2002, while playing in his crib, Matthew fell and lay stiff in the crib. Unable to move his arms and legs, Matthew was taken to the emergency room at Schneider’s Children Hospital in Long Island. It was at this point that his mother decided to change his physician. A resident physician in the emergency room recommended a genetic doctor by the name of Joyce Fox. She subsequently did a genetic workup and diagnosed Matthew with Sly Syndrome. After months of not being diagnosed, the family finally had something to learn about and to work with. It was at this point that Matthew’s mom began looking into the disorder and investigated its cause, progression, treatment, and cure.

Simultaneously, Matthew underwent spinal surgery to correct his paralyzed state. Steroids had helped significantly to restore movement in his limbs but surgery was indicated as a more long term solution. The surgery was long and complicated, lasting 8 hours. Matthew’s narrow airway made the surgery risky and doctors worried about his survival. But he pulled through and within 3 weeks was cleared for discharge! He wasn’t without further problems though. The spinal fusion he underwent didn’t cure his condition and he would be required to wear a full head and body brace for an undetermined amount of time.

Matthew’s family was advised by Dr. Fox during this hospital stay to undergo a bone marrow transplant in the following year. She stated that it was one of the only possible treatments for his disorder. His mother contemplated this heavily. Pediatricians urged her to take this route. She called another mother whose son had Sly Syndrome and who opted against the bone marrow transplant. It was proven successful with less severe forms of mucopolysaccharide disorders (MPS) but was never really proven with those afflicted with Sly Syndrome. The risks seemed too high. Matthew was severely immunocompromised and his multiple hospital admissions left mom worried about his ability to withstand a bone marrow transplant and its immunocompromising side effects. She had a strong feeling that it would not be the best thing for him and after a long and hard contemplation period, she decided against it.

At this time, she also spoke with a scientist in Missouri, Dr. Sands, who was working on a cure for MPS disorders. He stated that the drug he was working on wasn’t currently approved by the FDA and that he was still testing it on animals. He took her information and suggested that Matthew would be a fine candidate once the drug was approved. In light of this, Matthew’s mom opted to deal with whatever came Matthew’s way and hope that a cure would become available soon.
Unable to meet his needs at home, Matthew was brought to St. Mary’s Hospital for Children in Bayside, Queens. This was a huge support to Matthew’s family, who would be unable to care for all his medical needs on their own. It was a rehabilitation facility for children with disabilities that would provide nursing care around the clock, as well as therapies, support, and education. It was there that the family met other families in similar situations. At St. Mary’s, Matthew flourished. He started learning sign language, began speaking, and was eager to learn more despite the restrictions on his breathing and movement. He became a popular child there and his smile and happy demeanor left an imprint on all those who cared and continue to care for him.

Despite the leaps and bounds, Matthew was still suffering greatly because of his respiratory problems. The full body brace restricted his ability to breathe deeply and further complicated his respiratory status. He was still being admitted frequently for this and finally contracted a parainfluenza in June of 2004, which prompted an emergency room admission to Schneider’s Children Hospital. It was then that doctors rushed Matthew to the operating room at North Shore University Hospital in Manhasset to place a tracheostomy. Matthew’s mom resisted this and didn’t want her son to undergo this procedure but physicians insisted it was necessary for Matthew to live and mom finally agreed. He recovered quickly from surgery and the tracheostomy proved to assist Matthew greatly with his breathing. But it was also determined during his stay in the hospital at this time that Matthew would also require placement of a gastrostomy tube, since swallow studies found that eating by mouth would only increase his risk of aspirating his foods and would cause frequent pneumonias in the future.

After a month in the hospital, Matthew returned to St. Mary’s Hospital for Children where he lives currently and is receiving all his services. He is now two years old with a tracheostomy, gastrostomy tube, full head and body brace, and oxygen dependence. He has fought through multiple surgeries and frequent hospital admissions, conquering infection after infection. In his two years, he has undergone what those of us may not experience in a lifetime and yet he remains happy, smiling, and always quick to learn about what goes on around him. Caregivers in all the hospitals where he has been treated have all fallen in love with him and remember him as a happy, content child. Matthew’s undying strength and fighting spirit have inspired all those he’s touched and it is that moves us to help to find a cure for Sly Syndrome.