The piano keys ping underneath her fingertips, C-C-G-G-A-A-G, as her 11-year-old hands slowly play, “Twinkle Twinkle Little Star.” Nearing the end of the song her fingers grow heavy, she looses the rhythm and misses key. Christy gives a heavy sigh and lets her arms sink. Her music therapist touches her on the shoulder, Christy’s head rises, and she glances at her mother smiling softly at her side. Her mother helps carry her hands up to the piano; the specialist shows her where to place her fingers on the ivory keys. She blows out a weighty breath and starts again; there is no way she is quitting now.

Weeks earlier Christy’s fever was dangerously high; she dealt with irritating rashes, and a throbbing head that refused to subside. After general testing done by her pediatrician, they found markers for lupus and her pediatrician kept a close watch. Christy and her doctor continued to manage her symptoms, but as the day continued her symptoms worsened. Christy’s blood tests showed that her hemoglobin was half its normal rate. She was severely anemic and needed specialized pediatric care right away: she was referred to Miller Children’s Hospital Long Beach.

Christy arrived at Miller Children’s in poor condition, blood specialists were on her case immediately. As testing occurred Christy’s health quickly plummeted. The right side of her face and neck began to swell; blood was compressing her windpipe making her unable to breath.

Doctors rushed to help Christy breathe; she was given an emergency tracheotomy. Why was she bleeding? Doctors hurried to find an answer so that they could safely begin treatment.

Testing showed Christy’s blood was missing an important component called factor 8. Factor 8 is an essential blood factor needed to make their blood clot. After further testing doctor’s discovered that Christy had developed a very rare form of lupus – childhood systemic lupus.

Like adult lupus, childhood systemic lupus, is an autoimmune inflammatory condition where a trigger activates the immune system to change. The immune system does not recognize itself, begins to attack its own body and the person’s health quickly slips away. If Christy had entered into the hospital a moment later, or arrived at a hospital that was not staffed with pediatric rheumatologists and hematologists, her chances of being alive today would have been minimal.

Christy’s body had turned against her. Her immune system had totally destroyed her factor 8, and her body was bleeding out. Next her immune system made an assault on her skin and joints. In reaction she developed symptoms of arthritis and fever from overall inflammation. As time ticked by, her system wasted no time taking a hit on her kidneys, brain and demolishing different cells in her blood. All of her systems were inflamed, she was prone to infection.

That’s when the blood that flooded her lungs developed into a life-threatening fungus. As doctors worked on her she grew exceedingly sick, she was comatose, her joints were experiencing extreme inflammation and her body was swollen from the massive amount of internal bleeding. Unresponsive, her body began to shut down.

With every blow Christy’s immune system made, the pediatric intensive care team was there to simultaneously combat the condition and regulate her system. Christy was treated with multiple steroids and chemotherapy to cool her immune system down to a controlled state.

Two weeks after being in a medically induced coma, Christy finally woke up. She had a few set-backs during recovery, but she slowly began to heal. The bleeding had stopped.

Even though Christy had survived she would still need months of therapies, rehabilitation and a life-long regimen of medicines to manage her lupus. Christy would have to relearn how to talk, walk and care for herself. As she lay in bed her father would talk to her and urge her to try to start moving. Every night her father and her little brother would visit to give her support and keep her positive and her mother never left her side.

Rehabilitation therapists worked with Christy’s family to give her comprehensive care. Christy was able to heal by working with rehab therapists who utilized child centered therapy to help her gain her strength. For instance, Christy strengthened her arms by making friendship bracelets high over her head, as her therapist supported her and helped Christy follow through. She also went to the hospital gym and gained strength on the parallel bars.

Child life specialists were there with Christy to help distract and provide comfort for her. As Christy’s health progressed she was able to visit the playroom where a child life specialist would give her a needed distraction and break. Christy would sit at the piano with a music therapist, and with ease she learned how to play songs like Twinkle, Twinkle Little Star, Happy Birthday and others.

With the support from her family, the care from a comprehensive care team and her will to get better; Christy is now home and doing well.

Christy will have to learn to live with her diagnosis of systemic childhood lupus. This is a chronic life-long disease that is all about control and management. Christy is now taking fewer steroids, her blood is clotting normally, and her immune system is being regulated. Christy still has a comprehensive care team in which she sees a rheumatologist, blood doctors, and stomach doctors, and has made great progress.

Christy has gained a new passion for piano and music; her father even promised her a piano if she continued to give therapies her all and never give up. Thanks to fast acting, knowledgeable pediatric intensive care team and a little girl’s strength to survive Christy can continue to play her song.

     Twinkle, twinkle, little star,
     How I wonder what you are.
     Up above the world so high,
     Like a diamond in the sky.

     Then the traveller in the dark,
     Thanks you for your tiny spark,
     He could not see which way to go,
     If you did not twinkle so.