Four days ago, twins with a rare disease were born in Bikaner, Rajasthan. It has a girl and a boy. Their skin is like plastic. The skin is as hard as nails and cracked. These children are suffering from a rare disease called harlequin-type ichthyosis.
Doctors claim that babies born with Harlequin-type ichthyosis have come in for treatment before. But, this is probably the first case of twins in the country. This disease is included in the category of rare diseases. This genetic disease occurs in one in 5 lakh children.
According to doctors, it is not necessary that the parents are suffering from this disease. The disease is transmitted from parents to children due to chromosomal infection. That is, the parents are its carriers, but through which generation the disease is passing, it can be found only through medical history.
Children have plastic wraps on their bodies.
The skin is hard and cracked The twins, suffering from harlequin-type ichthyosis, were born on November 3 at a private hospital in Nokha, Bikaner. Their skin is like a light pink nail color which is very hard. There are cracks between them and torn from the inside. Due to his critical condition after birth, he was referred to PBM Hospital in Bikaner on November 5.
After two days of treatment in Nokha, both the children were brought to PBM Hospital in Bikaner, where they are undergoing treatment.
1 in 5 lakh people get this disease PBM Hospital Pediatrician Dr. GS Tanwar said – the family came here on November 5 with their children in critical condition. Since then, he is under treatment in the hospital's NICU. Harlequin-type ichthyosis is a genetic disease that affects one in 5 lakh children. This is probably the first case of twins in the country.
Children with this disease can live only up to one and a half years. Sometimes even for 25 years… But, life is not easy. For the first year, children's skin remains red, joints shrink and their limbs are delayed. Blood also leaks from the skin of babies at birth.
Pediatrician GS Tanwar said – the condition of both is not normal. They will be kept in the hospital until both are normal. Dermatology Department of Sardar Patel Medical College is also helping in treating these children.
Even if the parents are healthy, the next generation is at risk Dr. GS According to Tanwar, Harlequin-type ichthyosis disease is transmitted to a child through autosomal recessive genes. People can become carriers of the disease even without it.
For example, if one of the parents has inherited this gene, one or both of them will be carriers of the disease. This means that even if a parent does not have Harlequin-type ichthyosis in childhood, their future child may develop the disease.
That is, it cannot be said from which generation this disorder has come in genes. The parents of these children do not have the disease, but one or both of them are carriers.
Dr. GS Tanwar said – they are giving piped milk feeding along with vitamin-A therapy to keep the skin of both children moist. This is a very challenging task for us. Medical history, literature and research show that this disease has a one hundred percent mortality rate. However, every possible step is being taken to save the lives of the children.
Most babies don't live more than a week. Those who survive can live anywhere from 10 months to 25 years with high-tech healthcare.
Dr. Tanwar said- the child in this mother's womb had already died. Doctors believe that it is also possible that the future child will also suffer from the same disease.
Signs of disease at birth
- Deep cracks on the skin
- Eyes neither close nor open
- Keep your mouth open
- Ears are attached to the head
- Small, swollen hands and feet
- shortness of breath
- Dehydration
Image Credit: (Divya-Bhaskar): Images/graphics belong to (Divya-Bhaskar).
