Baby suffering from a rare genetic disease, born without skin, in a "critical" state

A baby born without skin due to a rare genetic disease has spent its first four months in intensive medical treatment and in the treatment of pain in the hope of saving his life.

Ja'bari, Swahili name meaning "the brave", was born on January 1 and weighed just 1.36 kg (3 pounds). He missed most of his skin, except for the head and legs. He suffers from a very rare genetic connective tissue disorder called epidermolysis bullosa (EB), which results in fragile skin that easily tears or forms blisters, even in the event of friction, rubbing, or scraping. very soft. Minor cases may simply cause blisters on the hands, feet, knees and elbows, while the most severe cases, such as Autosomal Recessive Dystrophic Epidermolysis Bullosa, Hallopeau-Siemens type (RDEB-HS) , can cause widespread loss of skin by events that occur during birth – and blisters on the body and inside the mouth and digestive tract.

Priscilla Gray, the mother of the child, writes that her pregnancy was "going well" until an ultrasound showed that her baby was not gaining weight. Doctors at a hospital in San Antonio, Texas induced it at 37 weeks and performed an emergency caesarean section when the child's heart began to fall. Last week, the child was transferred to the Texas Children's Hospital, where specialists are better equipped to treat him, reports the San Antonio Express-News. Currently, Ja'bari is taking medication to relieve pain and frequently receives dressing changes, as well as topical ointments to reduce the risk of infection. It has taken 2.26 kg since birth and is fed by a tube in the nose.

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"He spent all his life in the hospital," Gray told the newspaper. "I was able to hold it twice, but you have to wear a dress and wear gloves. It's not skin-to-skin. It's not the same thing. "

Texas Children's doctors had to take steps to cut the scar tissue that was melting the baby's chin in the chest. Her eyelids are fused since birth. IFLScience spoke with a representative from the Texas Children's Hospital who confirmed that the child was in a "critical" state. The hospital stated that it could not provide further comments on the patient's health because of HIPPA's privacy laws.

"We want to find an answer now, but everything is in the air," said the child's mother, Priscilla Gray, to the publication. "They are really focused on keeping him comfortable now."

These life-saving procedures barely took place when the family's insurance provider claimed that the transfer and treatment of the child to Texas Children's were off-grid. Medicaid then reversed the decision and the family created a GoFundMe page to help offset the associated costs.

"Every day is a blessing that he is still fighting to live on this earth," wrote the mother.