Hannah’s Hope

When Shannon and Jon Hall of San Fernando Valley, CA, heard their daughter, Hannah, cry for the first time at birth, it was the most wonderful sound in the world.

Just about one year earlier, their first daughter, Alexis, was diagnosed with autosomal recessive polycystic kidney disease (ARPKD) before she was born. Alexis never cried and was never taken off her incubator.

Like countless other parents, the Halls had never heard of ARPKD, and they were told that Alexis would probably not survive long past birth. Doctors at Children’s Hospital tried to help save the baby by putting her on an EKMO machine that reduces pressure on the lungs and heart. Unfortunately, Alexis died two weeks after birth.

Hannah, who was also diagnosed with ARPKD prior to her birth, survived and is now 2 years old. However, the path through her birth and early years has not been particularly easy.

After Alexis died, Jon and Shannon received help dealing with their grief by joining a hospital program for parents who had lost babies. They also reached out to others experienced with ARPKD and received support from family, friends and their church. Although there was a significant risk that any future child might also have ARPKD (research shows there’s a 25 percent chance with all pregnancies in individuals carrying the ARPKD gene), they wanted another child and did not want to delay that possibility.

By the time Shannon became pregnant again, she had done quite a bit of research and was much more informed about the disease. Although Jon and Shannon did not even know if the baby had ARPKD, this second pregnancy was treated as high-risk, and they discussed a strategy with their doctors. Their medical team also consulted with ARPKD experts to determine the best course of action including the decision not to use the EKMO machine again.

Shannon’s amniotic fluid level was extremely low at 24 weeks, and by 25 weeks, the kidneys showed cysts. Although they had hoped to hear if the baby had ARPKD much earlier, confirmation that Hannah had ARPKD occurred during Shannon’s 26th week of pregnancy.

Although the prognosis for Hannah’s survival was much more hopeful and doctors were proactive throughout the pregnancy, the outcome was not certain. An expert perinatologist in Los Angeles recommended trying a rare and risky procedure called an Amnio Infusion, which involves inserting saline into the uterus to simulate amniotic fluid. Only a select number of doctors are willing to perform Amnio Infusions, but since their doctor had experience and recommended that it be done, the procedure was scheduled almost immediately. Shannon underwent six Amnio Infusions, with a ruptured membrane occurring between the third and fourth procedures.

The Halls were nervous and frightened until the baby’s birth, but tears flowed when Hannah was born and screamed out loud for the first time. It meant tremendous hope. Since Hannah was six weeks premature and had underdeveloped lungs and high-blood-pressure issues, she stayed in the hospital for five weeks before going home.

Hannah is now 2 years old  shy at first, but then outgoing. She can count to 15 and knows her ABCs. She is tinier than other two-year-olds, receives physical therapy, needs to build muscle mass and takes two types of blood-pressure medication each day. However, Hannah is doing well, and her prognosis is positive. Shannon has celebrated that each stage of her development (crawling, sitting, walking) has taken more time than average and can be enjoyed that much longer.

The Halls have persevered over the past few years with a lot of support. For Shannon, the scariest thing has been the loss of innocence about having a baby. Her friends all had had babies with no problems, and she had not expected anything different for herself. She and Jon now realize how easily life can be lost, but they do not feel sorry for themselves. They believe things happen for a reason.

To others facing a similar situation, the Halls encourage reaching out to anyone who will listen and understand. Get involved with the PKD Foundation’s ARPKD Chapter and learn from others. Make sure that you have doctors willing to consult with other ARPKD experts if they do not initially have enough information. As a result, your experience may not be as frightening. Shannon and Jon are willing to share their knowledge with others. Please contact them at 818-523-2421 or shanon_frost@yahoo.com.