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Home NEWS Bone marrow transplant cures 13-month-old boy of rare genetic disorder

Bone marrow transplant cures 13-month-old boy of rare genetic disorder

Mohammed Khizr from Hyderabad was affected by extreme mixed immunodeficiency

Bai Jerbai Wadia Hospital for Children in Mumbai has efficiently carried out a life-saving bone marrow transplant surgical procedure on a 13-month-old boy from Hyderabad with extreme mixed immunodeficiency (SCID).

Mohammed Khizr was born to Raziya Sayed (29) and Karrar Ali (33) on December 24, 2019. Their happiness was short-lived as Mohammed would get fever and cough each alternate day. After giving drugs prescribed by a paediatrician, his fever and cough would subside for just a few days. Several assessments performed in an area hospital failed to supply an accurate analysis.

The affected person was referred to Wadia hospital the place he was recognized with SCID in September 2020, Dr. Prashant Hiwarkar, a bone marrow transplant doctor, stated.

Immune deficiency

“The patient was evaluated for immune deficiency in his first admission in August 2020 while in the paediatric ward. He was diagnosed with SCID on September 4. It is an inherited primary immunodeficiency disease that is seen in infancy; a genetic defect that affects T cells. Children with SCID have virtually no immune system to fight common viruses and fungus and die before their first birthday without a bone marrow transplant,” he stated.

On admission, he was recognized with pneumocystis pneumonia, a rare extreme an infection resulting in acute respiratory misery syndrome. He was on the ventilator for 14 days and developed life-threatening viral and bacterial infections. The affected person was stabilised with the assistance of anti-microbial drugs.

Biggest problem

“The biggest challenge in any SCID condition is that it is diagnosed late. Even in this case, by the time the baby reached us he was suffering from two life-threatening infections. So, our first hurdle was to control the infections. If the medical procedure of bone barrow transplant is done without controlling the infection, the transplant is less likely to be successful,” Dr. Hiwarkar stated.

Mohammed was shifted to the bone marrow transplant unit after reaching good management of infections. All the organs had been evaluated earlier than transplant and had been discovered to have regular perform. After treating with chemotherapy for 4 days, he underwent a profitable stem cell transplant at 11 months of age from his HLA-matched grandmother. The new immune system from the transplanted stem cells began to perform after 16 days, and he was lastly discharged on Wednesday after being within the transplant unit for greater than two months.

It takes about six months to a 12 months for full restoration of T cells. The dad and mom have been suggested to keep away from contact with those that have a viral sickness and cease going to crowded locations until full restoration of his immune system.

Dr. Ambreen Pandrowala, an immunologist with the hospital, stated, “This is the second child with SCID that we have successfully transplanted under one year of age. It is important for all children with immunodeficiencies to have an early referral to a centre familiar with immunodeficiency transplant.”

Ms. Sayed, a dentist, stated their world turned upside-down when Mohammed was recognized with SCID. “We were in tears and could not see our baby in pain. He is now achieving milestones of his age. He was 5.7 kg when he got admitted and now, he is 8.1 kg and much more confident as he tries to walk by holding onto furniture. Frequent fever and non-subsiding cough for three months was the major issue that we had been facing. We will go back to Hyderabad when he does not require frequent hospital follow-up,” she stated.

Lack of consciousness of main immunodeficiency problems amongst main well being care physicians contributes to the delayed analysis. When a baby develops severe an infection or a number of episodes of infections, a analysis of immunodeficiency disorder ought to be thought-about.

According to medical doctors, one in a single lakh folks will be immunodeficient and SCID is much more rare. Life-threatening immunodeficiencies will be fully cured with a bone marrow transplant.

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