A groundswell of support has been shown for a GoFundMe for a young child with an extremely rare and terminal disease.

Cody Smith and Dominique Rehrig of Harrisburg organized the GoFundMe for their son, Silas Smith, 3, on June 27.

As of Monday afternoon, $7,185 of the $7,000 goal had been raised.

Silas was diagnosed with CLN2 (form of Batten’s Disease), which is extremely rare and terminal with no cure.

In the next few years, Silas will lose his mobility, vision, and speech.

Silas will become bedridden and only live to about 10 years old.

Silas’ parents traveled to DC so Silas can start enzyme replacement therapy. This treatment is every two weeks for the rest of his life.

It is brain injections that will help replace the ‘bad’ enzymes in his brain to get rid of the waste and help keep his mobility and speech longer. Silas will undergo lots of services and testing.

The funds will help with travel, food, medical, and equipment that we may need for Silas. Anything would be greatly appreciated as we ensure he gets the best quality of life.

Diagnosed

Silas’ great-grandmother, Theresa Serfass, of Lehighton, said her great-grandson was born Aug. 8, 2019 and was a normal boy.

Serfass said that on Sept. 29, 2022, Silas had a seizure, so his parents took him to ER and the doctor said he held his breath, and to keep an eye on him.

On Oct. 27, 2022, she said Silas had another seizure, and just like the first one, he was out for five to six minutes.

Silas’ parents took him back out to the ER, and they did an MRI-EEG and everything came back normal, Serfass said.

She said the neurologist said he didn’t know what was happening, but to video everything in case it happened again, which it did on Nov. 7, 2022.

Serfass said Cody videotaped Silas and went back the ER, at which time the neurologist admitted Silas on did a series of tests and started him on meds for seizures.

On April 23, Serfass said they did a 24-hour EEG and genetic testing and suspected CLN2, otherwise known as Battens Disease.

“After several visits to Hershey Medical Hospital, the neurologist said he didn’t know enough about the disease to help Silas,” Serfass said. “He also told them to join a support group for terminally ill kids (through Hershey Hospital).”

Within two days, Serfass said a family from Nebraska contacted them and has been giving Silas’ parents guidance and support.

“(Silas’) parents called a different hospital in Pennsylvania, but none of them had the resources to help Silas,” she said. “They talked to a neurologist at the Childrens Hospital in Philadelphia, who was familiar with the disease, but new to the area and didn’t have the authorization to treat him.”

Serfass said the family then found a neurologist from the Childrens’ National Hospital in Washington, D.C., Dr. Gropman, who agreed to see Silas.

She said Dr. (Andrea) Gropman is the Division Chief for neuro developmental and neurogenetics and has experience with the diseases.

That was on June 19, at which time Serfass said Dr. Gropman assessed Silas and referred him to Dr. Myseros, their neurosurgeon, and they had a meeting on June 21.

Dr. (John) Myseros explained the surgery procedure, which is called a post placement, in which a hole was drilled in the tops of Silas’ skull and a port was inserted, she said.

“He said we have to act quickly, (as) time is wasting,” said Serfass, who added the surgery took place on July 3 and took about 30 minutes, but took three hours for Silas to wake up from. Silas was told the next afternoon he could go home.

Thankful for the support

On Monday, Serfass said Silas must go back to DC for his first treatment, called enzyme replacement therapy. After the needle is placed in the port in the top of his head, and it takes 4½ hours to do the procedure.

“During the procedure, Silas can stand, play or sit but may not run, jump or climb,” she said.

Serfass said the name of the meds is Brineura, which must be administered every two weeks to slow the whole process down.

“Eventually, he will be wheelchair bound, lose his eyesight and speech, and will need a feeding tube,” she said. “The seizures will become uncontrollably worse and will result in death.”

Serfass said the doctors have suggested Physical Therapy to help with Silas’ mobility.

“He now wears orthotics, and leg braces were ordered to help slow things down,” she said. “Usually a child with Battens loses most of (their) abilities and functions by age 6 to 8, but the doctors are optimistic that he will be here with us longer than most because they caught it early; fingers are crossed he will beat the odds.”

Serfass said that in addition to the GoFundMe, the family would like to hold several other fundraisers, the first being a hoagie sale in August, with the proceeds going toward medical bills and equipment.

“We want to thank our family, friends, and neighbors for all their support,” she said.