Bill and Lilly Ann

Lilly Ann and her parents.

Other than my wife Beth, nobody has written here on this blog.  Until today.  Bill Brooks is a fellow NF Dad who has amazed me since the day I met him. He is passionate, compassionate, smart, funny and NO time for BS.  His daughter, Lilly Ann, has NF just like Jack but has had many challenges and has approached them with a grace and dignity that astounds me.  I have met her a few times and I can easily say she is one of my heroes.  He is too.  The rest of the words now are his about her and really, in some ways, about all NF parents and their children and "the battle".  PLEASE, PLEASE READ.

 

Sorry for an epistle but I had to get an update out to everyone who's prayed, called, texted and commented.

Lilly Ann Brooks started her Friday, June 24th at 7AM in radiology with her second successful UNSEDATED MRI at National Institutes of Health Clinical Center. Yay! Then met at 9 with her wonderful Patient Care Coordinator Kara, then at 9:30 AM had her Pulmonary Function Testing (Oscillometry and Spirometry), then a 10:30 EKG, then an 11:00 ECHO cardiogram. Then a brief lunch at NIH of their finest pizza (no chicken fingers for two days!). 
At 1 she had her blood / lab work drawn, then a 2 PM bone density DEXA scan. We thought she had a break til 6, but one of the MRI's didn't capture enough of her shoulder and arm (which is where they chose to "target" or track a tumor for the trial) so it was back at 3:30 for her 3rd unsedated MRI in 24 hours, which took 1.5 hours! 
So at 5 we rushed back to the Children's Inn for one hour and back to NIH for the inpatient admitting process for a sleep study. By the time we got into the hospital at 7 they had stopped bringing hot meals for dinner so she had an "Uncrustable" PB&J and chips. Then up to 7th floor for the sleep study- hooked her up to 30 wires on her head, face, chest and legs, plus an oxygen hose in the nose! (She made me promise not to post a picture). Got up at 6, unhooked the wires, discharged and back to Children's Inn at 6:45 AM. Florida Highway Patrol thinks they have Troopers? Meh! Lilly Ann defines a trooper and a hero! What a day!

Candee had arrived in the Children's Inn during the night. Sat AM, we all headed out via the Metro for breakfast at Original Pancake House in Bethesda, then to the National Zoo to see the pandas and gorillas and ...lions and tigers and bears! (Not gonna say it). After that much deserved and wonderful zoo trip, we ate a fabulous dinner at Founding Farmers on Pennsylvania Ave. and then back to the Children's Inn. With great intentions to go out for the Monuments by Night tour, we passed out at 8 and woke up Sunday at 8!

Reluctant to miss a chance to leave the NIH campus, pretty and nice as it is, Lilly Ann pressed us to head out on the Metro to Old Town Alexandria for a great brunch at Vermilion, to sight see and shop where her brother's girlfriend Lizzie works at the local Lily Pulitzer store, and to go in other "Boutique Row" shops my girls would "just be a minute" in. Before we knew it, we had to rush back to Children's Inn and back out to Georgetown for dinner at Filomena's with Lizzie and her parents, absolutely delightful and wonderful people. Made it back at 11:30. Great day away from all the medical and research environment.

Monday began with an 8 AM Occupational Therapy analysis, followed by a long meeting with our lead Research Nurse Trish Whitcomb, to discuss giving our informed consent to all the statistics, protocols, risks and the possibilities (shrink and slow tumor growth!) that go along with submitting her to an experimental drug clinical trial. And there's those "Possible side effects"...something all over the counter drugs list but no one ever reads. Well, you read them when your child is in a clinical trial. That's when it hit home, right in the gut. My mind started questioning. Are we doing the right thing? The "What if's" ripped into my heart. There's some highly unlikely but possible stuff that's really scary. And now she's old enough to understand what she's hearing! Her questions are incredible, one causing Trish to hesitate and take a pass on an answer for a later meeting with the trial's Principal Investigator. "What if the one tumor they are tracking and targeting doesn't shrink enough to qualify to stay on the trial, but the other tumors do? Can I still get the medicine?" "That's a very good question Lilly Ann. I'm amazed at your maturity and analytical mind. I'm not totally sure so let's ask Dr. Widemann." The tears were welling up but you stop them from coming when she's sitting right there.

Nearly through the consent discussion our coordinator Kara called to say we're late for her Physical Therapy analysis. Off we went to finish the consent talk later.

So we rolled her into the Physical Therapist's room who unhesitatingly asked Lilly Ann to do a 6 minute walk test to see how far she can go up and back in laps of the 50 meter tile corridor. It was tough just to watch, but I kept encouraging her each pass, "you're doing great baby!" while watching her hold her side and her arm in obvious pain. She did 4 1/2 laps, about a 1/4 mile. Back in the PT room she turned down a drink of water and sat in the chair in silence, looking at me, tears streaming down her cheek, in serious pain. She said "Dad you know me, I don't complain about pain, but I'm really hurting." When offered a Tylenol she declined, just wanting to "sit a while before whatever is next".

Next was Ophthalmology, for a full battery of exams and retinal and iris photos. Her whole life she has been extremely light sensitive. The pupil dilation drops and numbing drops only exacerbate it. The bright blue, green and white lights and camera flashes were brutal. But she persevered through it. Two techs and multiple photos later, it was back to the waiting room. She napped in the wheelchair. Then 30 minutes later she was examined by two fellows / research eye doctors and then she was done.

It was time for some more fine NIH pizza!

Then back at 12:30 to the peds clinic to meet with Research Nurse Marielle. Needed another lab sample.

Next it was time to finish the consent discussion with Trish. We'd all thought of other questions and Trish honestly answered them all wonderfully. She has a great patient and family "manner" in our talks. All the possible side effects again were described. "Hair lightening and thinning, acne, rashes, BP increases, blood cell issues, nausea, diarrhea and vomiting, constipation, and heart issues are possible. But a lot of participants have only few, and many adjust after a couple of months and it gets better."

Upon leaving at 5 pm, we no sooner stepped into the Children's Inn that Lilly Ann wanted to go back out and eat a good dinner and see the National Mall monuments! OMG! So we headed to the Metro, went to eat at The Hamilton, went to the Rooftop Lounge in The W Hotel to see the White House and Washington Monument at night, then I showed her the historic Round Bar in the Willard Hotel next door (OK that one was for me.) Then back to the Metro to Children's Inn for me to pack for departure at 6 am to leave them behind with Tuesday appointments for her body photography, video, and nutritionist and the meeting with the Principal Investigator Dr. Brigitte Widemann and her team, which I called in for. Lilly Ann was equally impressive to Dr. Widemann as they all discussed a lot of the same questions, risks, side effects and possibilities of the trial that we'd already been over with Trish and Marielle. The emotional walls I'd built were starting to crack.The reality is they don't know or yet fully understand the what, why, who, when or lots of things in biomedical research. That's why they do these trials, to figure that out. And one sure thing about clinical trials is they must follow protocols exactly to not compromise the trial's data. And the truth be bluntly known, she is a human participant in research (I cannot use other terms) whose volunteering (by us) may benefit her and others with NF and it may not. Only God knows. 
My mind wandered off and I only heard "Charlie Brown adult" sounds on the call for a couple of minutes. I snapped out of it when she said through the phone "Dad, we're doing this." I managed to get a "yes"'out and went back on mute so I could cry. Every expert and parent I've consulted on this trial has convinced me, this is the right thing right now. And it's also the only thing. I've prayed it through. So we're on board. Like she's told me a lot lately, "I've got this Dad".

As I sit on a 7 AM plane Wednesday morning from Orlando to Atlanta for a supposedly "important" all day meeting, my wonderful daughter and her mom are inside a hospital room in The National Institutes of Health in Bethesda. Lilly Ann is getting her first dose of the experimental drug Selumetinib, and getting an IV needle stuck in her arm to allow blood samples to be taken every couple of hours to monitor her body's vitals and tolerance of the drug. And Lilly Ann can't wait. Can you imagine?

I talked to her three times today. So far she has tolerated it well and has kept the food she's eaten down. They let them discharge at 6 and she headed back to The Children's Inn to take the second dose at 6:45 and will eat at 8.

She sounds so happy and grateful on the phone. I thought about that. She's "happy" that she's got this "opportunity", she's "grateful" to finally have something to at least try. Not anger or bitterness or pity or selfishness. Just happy and grateful. Where does that come from? And she smiled at every person who examined her, even if she knew it would probably not be a pleasant experience.

As she told her 96 year old Pop Pop Friday, "This is going to make me get better. I'm going to be fine. Don't worry."

God please bless this child and answer our prayers and make this a successful trial for her. Shrink, slow and stop the growth of these tumors!

Did I ever tell you she's my hero?