Children’s Hospital of Pittsburgh of UPMC



Mother donates part of her liver to save son’s life in living-donor transplant procedure


A 7,000-mile journey to Children’s Hospital of Pittsburgh of UPMC enabled 8-year-old Saleh Al-Sada from the Persian Gulf country of Qatar to receive the vital organ transplant needed to save his life. Yet the curative solution was always as near to him as his mother.

Young Saleh was suffering from heart failure, a consequence of a rare inherited metabolic disorder. The transplant that would restore his cardiac health involved not the heart, but the liver, and his mother Manal, a viable candidate, stepped forward to provide a portion of her own organ for her son.

Mrs. Al-Sada’s gift has enabled Saleh’s heart to heal and his metabolic condition to improve, and her own liver function is now fully restored. It is an example of how a living-donor liver transplant can provide a life-saving alternative for young children with serious metabolic conditions.

Metabolic disorder
Saleh and his fraternal twin, Mohammed, who does not have the condition, were born in 2007 in Doha, Qatar. Doctors quickly diagnosed Saleh’s condition, known as propionic acidemia (PA), a potentially life-threatening disorder that presents quickly in stricken infants through poor feeding, lack of appetite, vomiting, lethargy and weak muscle tone.

As a recessive genetic disorder, PA occurs when a specific faulty gene is inherited from both parents. In people with the condition, the body is unable to break down certain amino acids and fats, which can lead to a buildup of harmful organic acids and toxins in the blood.

Through the age of 4, Saleh’s health and nutrition issues required frequent hospital visits. By the time he was 5 years old, Saleh stabilized, started to eat, gained weight, and attended school. Until at the age of 7 he had an inexplicable episode of rapid deep breathing that required medical attention. Doctors said his lungs were good, but found he had developed cardiomyopathy caused by his metabolic condition.

“One of the complex complications of propionic acidemia is cardiac disease, which has been reported to get better with liver transplantation,” said Children’s Hospital’s Director of Pediatric Transplantation, George Mazariegos, MD, FACS, an expert in the field of liver transplantation as a cure for metabolic disease, whose team ultimately performed Saleh’s liver transplant.

Doctors advised the family that a liver transplant might benefit Saleh. Aware of Children’s reputation in liver transplantation for metabolic disease, Saleh’s doctors consulted with Dr. Mazariegos and then recommended that the Al-Sadas bring their son to Pittsburgh.

Concerned about her son’s frail condition, Mrs. Al-Sada asked if she could serve as the donor to expedite transplantation. Although Mrs. Al-Sada, like her husband Badr, is a carrier of the defective gene that resulted in her son’s PA, she does not have the condition, and her own liver functions normally.

Transport for transplant
In 2015 the family arrived in Pittsburgh. Saleh and his mother were evaluated, and preparation for the transplant procedure began. A portion of the left lobe of Mrs. Al-Sada’s liver was removed and rushed to Dr. Mazariegos and the team at Children’s. Saleh’s surgery was more complex, and recovery took longer, requiring temporary use of a ventilator to assist his weak lungs while his heart got stronger. However, signs of cardiac improvement were already evident after the first week. After two months, Saleh’s recovery had progressed to the point where physical and occupational therapy were started to help him regain strength.

The path home
Six months after surgery, his cardiac condition and breathing were significantly improved, and his liver function was normal. As PA is a systemic disease, liver transplant does not cure the patient of the condition. “However, the pathways of the organic acids are present to a large degree in the liver, so treatment of it significantly improves with liver transplantation,” Dr. Mazariegos explains, adding that Saleh’s metabolic control in terms of his dietary restrictions has improved with the transplant, and he is being weaned from his medications.

Mrs. Al-Sada’s left lobe will completely regenerate over time, and the portion transplanted into Saleh will grow and develop as if it was his original liver.

Liver transplantation as a treatment for pediatric metabolic diseases is increasingly proving to be a viable option – one where Children’s Hospital has a proven record of success. With related livingdonor transplantation, parents and family members can often be the donors, resulting in more positive outcomes due to shortened wait times and better genetic matching from healthy living donors.

According to Dr. Mazariegos, Saleh’s case was innovative from the standpoint of using the liver of a donor who was also the carrier of a gene that caused the metabolic disorder, even though the mother did not have the condition.

Today, Mrs. Al-Sada’s hope for her son is that he may grow up to become a doctor – like the many who were so instrumental in saving his life.

 

 

Date of upload: 20th Jan 2017

                                  
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