Noa

One of my patients the first year I was an RN in the PICU was a little girl named Noa. I became one of her primary care nurses. The experience with that family was powerful and still affects me to this day.

Noa and her family were visiting relatives in northern California from their home in Sydney, when she first became ill. She was 18 months old at the time. Her father was still in Australia due to business, so only she and her mother were present when she was first admitted to the pediatric intensive care unit (PICU). Noa had the flu. But it was not this illness, which brought her in to the PICU. It was in the course of having the flu that she became too weak to sit up. This weakness did not leave with her flu symptoms. Studies conducted in our PICU came back with a diagnosis of Spinal Muscular Atrophy (SMA). Few children with this genetic, neuro-muscular disease live to adolescence.

The diagnosis was provided just as the father had arrived from overseas. Having not seen his family in several weeks, he was in a complete state of shock to hear the news. His beautiful baby girl had just been diagnosed with a disease with a poor prognosis. Noa’s father was not coping well. He felt out of control and was frequently emotional and would sometimes lash out angrily, finding blame with any medical staff that came near. Because of his length of time away during the initial stage of this disease, he seemed to place a lot of blame on himself as well. Noa’s mother, while obviously upset, was more accepting and focused on researching all the facets of her daughter’s illness.

Due to the severity of Noa’s disease and the need for therapies not available in Sydney, the family made plans to move and completely change their lives. Noa’s mother and older brother stayed by her side while her father went back and forth to Australia. Noa was discharged with a whole host of therapies to strengthen her.

A month later, Noa came back due to an apneic event and continued difficulty breathing. The SMA was now affecting her intercostal muscles. She was intubated and placed on a ventilator. While upset about this change in events, the family as a whole seemed stronger and better able to cope with the disease. They were no longer grieving the loss of their perfect, healthy child and were now focused on giving Noa as much quality of life as possible. This hospitalization was especially long and the staff came to truly bond with all members of the family.

As staff, we did what we could toward giving Noa as much quality of life as possible while in her PICU room and connected to the ventilator. I often assisted the mother with her daily “spa treatment” where we’d sit Noa up on pillows and place her feet in a basin of warm bubbly water so she could kick and splash with her feet. We invited the family to decorate her ICU room and her mother filled the bed with pink boas. We dubbed them “Noa boas”. Child life was heavily involved and we all participated in each activity. Story time was every afternoon by a volunteer. Pet therapy was once a week, where a dog came and sat on her bed. Music therapy was twice a week. This allowed Noa to use what little arm strength she had to shake a plastic egg filled with sand along with the music.

Despite being intubated, Noa was very expressive. She learned to communicate with her eyes and eyebrows. Two eyebrow lifts were for “yes” and either closing her eyes or shaking her head was “no.” Her mother had even taught her a few hand signs. This made it all the more enjoyable to spend time with Noa. We could do silly things with her toys and make animal sounds that would get her laughing so hard that her ventilator would alarm, due to the changes in air pressure. The family was now in California full time living with relatives and the father had not yet found work. While the unemployment was a cause of stress, it allowed all family members to be at the bedside regularly to spend time with Noa.

The day Noa had a code blue, both parents were witnesses. Looking back, I am so glad they were there, as it truly made a difference in the days to come. At the time there was no set policy in the PICU on having parents around during such events. If they had already been outside the unit, they likely would not have been let in. Yet because they were there, they stood outside the room watching us all work fervently to bring their daughter back. They saw the respiratory therapist bag with one hand while holding her little fingers in the other and they heard our pleas to her while we infused various medications and IV fluids during the resuscitation. Noa made it through that episode and her mother rushed to her side as we walked out of the room. But her father remained outside in order to hug each one of us. Seeing our dedication to helping his child cemented our bond.

Noa was soon given a tracheotomy and a G-tube. With her airway stable, she was able to be weaned off the ventilator and only required routine suctioning and oxygen at night. Through much family education, Noa and her family were discharged to go home.

Many months later I received a call at home from my co-worker, that Noa was dying. Noa had come in that week with respiratory distress and was barely responsive. It became apparent that her condition was worsening. After multiple family conferences, the family had made the decision to go home. They had watched their daughter go through so much the past year and did not want to prolong her suffering. The mother later explained to me how they lay her between the two of them and held her all night until she died.

The funeral was held in their neighborhood church, a three hour drive away, and almost all the PICU staff were there, wearing pink “Noa boas”. The father said wonderful things about our unit and set up a fund in his daughter’s name. After the funeral, we went to their home where her mother showed me the garden she had made for Noa and the little cement “Noa angel” she had placed there in her memory.

The family thanked me for all I had done for Noa. But I felt the experience with them as a family had given me so much more. I had watched them from the beginning of their crisis period and saw them grow together and strengthen their coping skills. Their faith and strength helped them through each hospitalization and worsening stage of her disease. The rapport between the family members and hospital staff built trust and allowed family conferences to be more productive, as they knew we also wanted the best for Noa. Their love and strength as a family continued to support them as they had to face the very difficult decision to let their daughter die. It was a decision that I had seen few parents make and filled me with the utmost respect and honor for them.