Today, our sweet "Crew Man" is 7-years-old and in 1st grade. He has a twin sister Cecelia in his class and a 5-year-old sister Everly in kindergarten. Crew is aware that he has NF1 - that he has tumors and a hearing insert - but beyond that, he is a normal, energetic, silly little boy. He is in advanced reading at school with his twin sister. He loves Legos, swimming, and his dog Toby. Crew has NF1 by genetic mutation, and his twin sister doesn't have it.

At 12-months-old, we first heard the word ‘Neurofibromatosis’ from our pediatrician. At age 13-months-old, we found out our son Crew had a tumor near his brain on his trigeminal nerve. Crew was formally diagnosed with Neurofibromatosis-Type 1 (NF1) at 13-months-old. At age 26 months, an MRI scan showed many new and growing tumors in his mouth, tongue, cheek, and neck. These tumors can continue to grow all over his body and on his nerves. The shape of Crew’s tongue was affected by the tumors. When he speaks, his tongue favors one side of his mouth. His right cheek is noticeably larger than the left. Tumors running along his nerves and carotid artery are visibly seen on the right side of his neck. Due to the tumor on the trigeminal nerve, every time he chews food, red flushing occurs on his right cheek. The flushing started at 6 months of age when we introduced whole foods to his diet and still continues to this day at age 7.

Crew has an extensive team of doctors including a pediatrician, pediatric neuro-oncologist, NF specialist, ear, nose and throat doctor, and an ophthalmologist. Crew has had sedated MRIs every 3 to 6 months since he was 1-years-old. After the first scare of tumor growth affecting his tongue around 2 years of age, I knew we had to do more. I researched, emailed, called, and finally decided on one of the most experienced NF specialists in the USA, Dr. Bruce Korf at the University of Alabama at Birmingham. With the help of Wings of Mercy West Michigann, we flew down and met with him. We had genetic testing done to confirm Crew's diagnosis, and Dr. Korf helped move us in the right direction medically.

With his guidance, we connected with physicians at Ann & Robert Lurie Children's Hospital in Chicago. There, Crew qualified to start on a clinical trial for a MEK inhibitor called binimetinib. He was on this medication for about 18 months and had about a 36% reduction in his tumors. Eventually, Crew stopped responding to the medication and the side effects were so horrible that we chose to discontinue. Crew side effects such as skin rashes, nail bed infections, edema (swelling), diarrhea, nausea, vomiting, persistent eyelid sores and a change in hair color. 

Crew's tumor remained stable until his most recent MRI in November. Crew had stopped binimetinib around age 5, and now at age 7, he is about to start selumetinib to hopefully slow down or decrease the tumor size. This time around, his medication will be 1 pill, twice daily. We'll have to practice swallowing tic-tacs or mini m&ms!

His plexiform tumors continue to grow in his right cheek and jaw area (as you can see in the MRI images to the left). They have been in other parts of his body such as his sinuses, mouth, ear, neck, scalp and now further down his chest. While there is only a little information how the growth is affecting his right ear, visually we can tell there is more swelling there and we want to protect his hearing. Crew uses a hearing insert to help keep his collapsed ear canal open due to the tumors. If the tumors keep growing, it could eventually make him lose hearing in his right ear.

We expected some growth in this last MRI, and also expected we'd restart a MEK inhibitor, but it was still a jolt for me back into "Medical Mom" mode. For awhile, we haven't had any "big medical things." I've been able to disappear into a new career, fun plans with family and friends, and other parenting and life things. Now, I'm snapped back into day-to-day medical needs, a kiddo who will have yucky side effects, and lots of doctor's appointments.

It's a crazy juggling act! But, I was made to be this kiddo's Momma and let me tell you, Crew is the strongest kiddo I know.

Crew will have this disorder his entire life. We can’t fix it but, but we can seek the best help and do everything in our power to help him. This is also why as a "Medical Mom" who has her Bachelor’s in Social Work, I choose to be as active in the NF community as I can be. We participate with NF Michigan, a member organization of the NF Network, and other NF organizations. I plan to participate in the February 2022 NF Network Advocacy Program Hill Visit Days. We have also been on the local news a few times to help raise NF awareness. We hope to bring awareness to this disorder and help provide strength and hope to others on similar paths.

 

With your support, each year the NF Network is able to serve over 10,000 members of our NF community and connect with over 1,200 new NF friends and families, including Hilary and Crew's. Together with your help, the NF Network can continue to provide direct support to families like the Berens. Make your year-end gift here. 

For more information on Crew and his journey, follow his Facebook page: https://www.facebook.com/HisBattleIsOurBattle/