Avalanna Routh, 6 year old “Mrs Bieber” Loses Brain Cancer Battle

The 6-year-old who became known as “Mrs. Bieber”, Avalanna Routh, has died. Avalanna lost her battle with a rare brain cancer called an atypical teratoid rhabdoid tumor (AT/RT). She has undergone numerous surgeries, chemotherapy, radiation therapy and even a stem cell transplant.

Avalanna first came to national attention last year at the Jimmy Fund Clinic pediatric cancer center at Boston’s Dana-Farber Cancer Institute. The staff knew that Avalanna was a huge Justin Bieber fan, so they threw her a make-believe wedding, complete with a cutout of Justin, flowers, a wedding cake and even a singer.

Justin heard about Avalanna through a special Facebook page- “Get Avalanna to Meet Justin Bieber” and arranged to meet them in New York. The two had a wonderful time cuddling and playing Candyland. Afterwards, Justin tweeted:

Avalanna was featured on this month’s Stand Up to Cancer telethon, and although she was too sick to attend, her story still got her a standing ovation!

Yesterday her parents sent out the following Tweet:

Our darling Avalanna went to Heaven this morning. Oh Avalanna, the brightest star - you took our hearts with you, our greatest Love;(

Justin Bieber’s reply was immediate and heartfelt: ” just got the worst news ever. one of the greatest spirits i have ever known is gone. please pray for her family and for her.”

What is an Atypical Teratoid Rhabdoid Tumor (AT/RT)?

Central nervous system (CNS) atypical teratoid/rhabdoid tumor (AT/RT)is a very rare, fast-growing tumor of the brain and spinal cord. It usually occurs in children younger than three years of age, although it can occur in older children and adults.

About half of these tumors form in the cerebellum or brain stem. The cerebellum is the part of the brain that controls movement, balance, and posture. The brain stem controls breathing, heart rate, and the nerves and muscles used in seeing, hearing, walking, talking, and eating. AT/RT may also be found in other parts of the central nervous system (brain and spinal cord).

Who is at greatest risk for getting AT/RF?

Atypical teratoid/rhabdoid tumor may be linked to a change in a tumor suppressor gene called SMARCB1. This type of gene makes a protein that helps control cell growth. Changes in the DNA of tumor suppressor genes like SMARCB1 may lead to cancer.

Changes in the SMARCB1 gene may be inherited (passed on from parents to offspring). When the SMARCB1 gene change is inherited, tumors may form in two parts of the body at the same time (for example, in the brain and the kidney).

What are the symptoms of AT/RF?

Because atypical teratoid/rhabdoid tumor is fast growing, symptoms may develop quickly and progress over a period of days or weeks. Symptoms vary and depend on the age of the patient and where the tumor has formed. They include:

• Morning headache or headache that goes away after vomiting.
• Nausea and vomiting.
• Unusual sleepiness or change in activity level.
• Loss of balance, lack of coordination, or trouble walking.
• Increase in head size (in infants).

What is the prognosis?

The prognosis (chance of recovery) and treatment options depend on the following:

• The age of the child.
• The amount of tumor remaining after surgery.
• Whether the cancer has spread to other parts of the central nervous system (brain and spinal cord) or to the kidney.

Because of the aggressiveness of the tumor, the two-year survival is less than 10%. The average survival after surgery is 7 months. Avalanna certainly lived with the disease for over 5 years!

(Source: National Cancer Institute)

RIP little “Mrs. Bieber.” Our thoughts and condolences to your family and friends.