It was certain that Brooke Kennedy would be tested for SMA shortly after birth, given her sister’s diagnosis.  Bloodwork confirmed Brooke’s SMA diagnosis one month after birth. 

The early diagnosis was at least somewhat fortuitous: being as yet symptom-free made her eligible for admittance to a clinical ‘single key-drug therapy’ study being conducted by the University Of Utah School Of Medicine. Funded by FightSMA and led by Dr. Kathryn Swoboda, the study is called the “Prospective Phase I/II Study to Evaluate Effects of Sodium Phenylbutyrate in Pre-symptomatic Infants with Spinal Muscular Atrophy” or “STOP SMA”. By beginning treatment pre-symptomatic, Dr. Swoboda is setting out to prove that the earlier treatment begins, the greater the potential benefit in delaying onset and ultimately lessening severity of SMA symptoms. Read more about our clinic visits here.

At 2 years old, Brooke had yet to show the signs of deterioration her sister experienced at the same stage. She crawled extensively, pulled to stand and walked with a gait trainer. Starting around 12 months of age, Brooke’s greatest challenge was her inability to gain weight. As is often the case with experimental treatments in small children and infants, it is believed her medication, so apparently successful at delaying onset of SMA symptoms, may indeed have been the culprit for these complications. At 15 months of age, she weighed only 15 pounds and her doctors determined it was critical to abandon her current study medication regimen and start her on the same path as her sister. While this was an unappealing option due to the extreme toxic nature of the cocktail of drugs she must take instead, especially to children under age two, it was the only presently viable alternative. 

Brooke’s inability to gain weight resulted in emergency laparoscopic nissen fundoplication surgery and insertion of a gastrostomy tube (G-tube). Brooke is fed a bolus feeding three times a day and is hooked up to a feeding pump (continuous drip) all night.  She has endured many occupational therapy sessions to work on oral feeding to cut down on the bolus feeds during the day.

At first glance, Brooke has all the characteristics of a normal, healthy child. With a smile on her face at all times and a penchant for mischief, she shows everyone she meets that SMA does not define her. Brooke, like her sister, has a cheerful but indomitable fighting spirit that propels her forward despite the medical challenges she has already faced.

Stitches For Brooke

This is what sucks about SMA. Brooke and Brielle’s muscles are weak. Brooke was crawling and her arm gave out and her head fell hard, right on the metal strip that is in between out carpet and tile. We took her to the ER right away and she ended up needing 2 stitches.  She did great! The child life specialist came in and showed her a Mickey Mouse movie while the doctor did the stitches. The doctor said she even smiled at Mickey and Minnie while he stitched her up! So proud of my brave girl!


Brooke is an Ipad grant recipient from GSF's Project Mariposa!

The Gwendolyn Strong Foundation (TheGSF.org) created “Project Mariposa” to make the world more accessible to those with severe disabilities through targeted technology product grants.

“Project Mariposa will initially focus on granting iPads to those impacted by Spinal Muscular Atrophy (SMA) — 50 iPads in 50 weeks. These incredible devices, coupled with the growing application library that accompanies them, are literally life-changing in assisting with communication, education, and independence. The iPad is opening up new avenues that were previously impossible. It’s large screen size, light weight, portability, and featherlight touch screen make access to education, entertainment, and independence possible and in some cases it is allowing children to communicate for the first time in their lives. Its impact is truly incredible. In fact, the New York Times wrote an article and produced the below video about a 7-year-old boy with SMA and how the iPad has empowered him. Project Mariposa’s goal is to provide that same life-changing positivity for a large number of people.” TheGSF.org

We filled out an application for Brooke and we are just beyond thrilled that she was the second grant recipient from this wonderful initiative!

“GRANTEE #2: Brooke Kennedy, Michigan :: Brooke has SMA Type 2 and has a love for technology at the ripe age of 3. She likes to play on mom’s iPhone and already knows how to look for her favorite apps. She would be so happy to have her own iPad. The iPad would be helpful to have in stressful situations to help calm her down and redirect her attention. And we feel the iPad will help Brooke develop a longer attention span for learning and learn to communicate with us better than she currently does.” - thegsf.org

…and here she is opening her package that just came in the mail!

Thank you so much Gwendoly Strong Foundation, this gift is going to be invaluable for Brooke!!!!

Brooke Turns 3!!

Wow, it’s really hard to believe it’s been 3 years already since Brooke decided to make her first debut into the world! 

I remember it like it was yesterday…  The night before Brooke was born, Brielle and I baked Christmas cookies and after I put her to bed, I assembled our new double stroller with anticipation of filling that second seat.  Oh… and did I mention Eric was out of town for military training that he needed to complete before he would be deployed to Afghanistan in just 4 short weeks (Jan 2009).

I woke up at 1:00am feeling like I had to go to the bathroom… but I couldn’t hold it.  It stopped so I went back to bed and then it happened again.  How annoying! :)  I talked to the doctor on-call because I wanted her to reassure me that it was totally normal and to go back to sleep.  Unfortunately, she assured me that my water broke and told me to get to the hospital right away so she could admit me.  I started crying and she asked me what was wrong with a puzzled voice.  She probably figured a nine month pregnant woman would absolutely love to go into labor 2+ weeks early!  I said, “My husband isn’t here… he’s in Texas for training.  This isn’t supposed to happen.  He comes home in two days.”  She empathetically said, “I’m so sorry but you need to get in here as soon as you can.”  So I quickly gathered my hospital bag, called my mom hoping that she would pick up, frantically calling Eric, calling our friend, Norine, to see if she could come over and watch Brielle.  I was in a frantic state of panic…  but we all whipped into shape after seemingly running around like chickens with our heads cut off in the middle of the night. 

Thankfully, our friend Norine drove over to our house in the snow covered and icy roads so that she could be there for Brielle early that next morning.  My mom lives really close so she drove me to the hospital.  Then, at 4:40am Brooke was born!  I hardly had time to get settled in a room!  Brooke was ready to come into this world on her terms and she was not going to wait for when it would be convenient for us! 

Luckily, Eric was able to fly home the next morning and he rushed right to the hospital so he could hold his new baby girl.  It was a day I will never forget.  :)

 

This year, as Brooke turned 3, she still has that spunky spirit and expects things to happen on her terms!  She wanted to have a Cinderella themed birthday party so we invited friends and family over to celebrate and had a “real princess” visit to sing and dance with the girls.  Brooke’s eyes were as big as saucers as she watched Cinderella enter the room.  As she got closer, Brooke studied Cinderella’s hair, tiara, dress and “glass slippers.”  After they finished singing and dancing, everyone sang the “Happy Birthday” song to Brooke as she blew out her candles and made a wish.  It was a wonderful birthday celebration that I hope she will remember when she gets older.

 

On the actual day of her birthday, she received a phone call from the REAL princesses from the castle in Disney World!!  First she talked to Cinderella, then Belle and last but not least Sleeping Beauty.  She told them how she was 3 years old now and she blew out the candle on her cake by herself (something she couldn’t do last year!).  What an awesome birthday gift - thank you to the princesses for taking time out of their day to call Brooke!

Here is a special video they made for her too!

 

Later that evening, the Pediatric Outpatient Rehab Specialists (where the girls get their therapy) had their annual holiday party at Bounceland!  We decided to go and bounce, climb and slide down the air filled structures.  It was quite a workout to carry, lift, hold, bounce with the girls but it was totally worth it to see the smiles on their faces!  Brooke absolutely loved every moment and kept saying, “again! again!!!”  She even had her arms up and hands above her head on the BIG slide!!  She is such a daredevil and I just love her zest for life! 

 

There was a surprise visitor that made an appearance at Bounceland too!  Santa Claus!!  Brooke sat right on his lap with no qualms and when asked, she and Brielle told him that they wanted, “lots of toys” for Christmas this year!  :D  This was the first time Brooke was actually excited to see Santa and wasn’t afraid of him.  She wanted to touch his beard and talk with him. 

Our baby is growing up so fast.  It makes me reflect on how much she has gone through in the past 3 years.  We have been through so many difficult situations with the worst being the diagnosis of a terminal disease but you would never know it if you met her in person.  She is incredibly brave, has a strong personality and a perseverence to push the limits.  Yet she is the sweetest, most forgiving and loving daughter that a mother could ask for. 

I am so thankful for every single moment I’ve been blessed to share with her in these past 3 years.

 

Happy Birthday Brooke, I love you very much!  -Mommy

Fun Day At the Zoo!

We spent the afternoon at the zoo and had a great time! We decided to take Brielle’s big pink wheelchair so she can get more practice in public places. We usually take her manual wheelchair because it is only 7lbs and great exercise her her upper body. The electric chair is great for long distances so it was perfect for the zoo!
Everything was handicap accessible and very easy for Brielle to get around. She and Brooke had SO much fun!

Brooke is finally feeling better!

After two and a half weeks of high fevers, non-stop coughing, sneezing, runny nose, etc. Brooke is finally feeling better!  I feel like I haven’t heard her laugh for a long time.  This moring during breakfast Brielle tells Brooke to say, “nom, nom, nom” so Brooke decides to play along and says, “nom, nom, nom” and then they both start to giggle… then the whole process starts over, “Brookie - say nom, nom, nom” then a brief pause and Brooke says, “nom nom nom” and then they both giggle like crazy!  I thought to myself that is so cute I wish I had it on video.  Oh well missed opportunity but the point is that it was so great to see Brooke in good spirits and laughing again and that’s how I know she is on the mend.  Thanks for all the thoughts and prayers!

Brooke has Respiratory Syncytial Virus Infection (RSV) :(

Respiratory syncytial (sin-SISH-uhl) virus, or RSV, is a respiratory virus that infects the lungs and breathing passages. Most otherwise healthy people recover from RSV infection in 1 to 2 weeks. However, infection can be severe in some people, such as certain infants, young children, and older adults. In fact, RSV is the most common cause of bronchiolitis (inflammation of the small airways in the lung) and pneumonia in children under 1 year of age in the United States. In addition, RSV is more often being recognized as an important cause of respiratory illness in older adults. - Courtesy of CDC.gov

Friday, April 15th, Brooke was diagnosed with RSV.  She’s been sick for about a week now and it all started with an ear infection.  We started her on an antibiotic right away and we’ve been waiting for this to pass but instead of getting better, she’s been getting worse.  She’s lost her voice and has had a non-stop runny nose and ‘wet’ cough.  Our last scheduled clinical trial visit was supposed to be this week Wed-Fri. so they suggested we get a virus panel to see what kind of virus we are dealing with and the results came back with RSV.  Brooke also had a chest X-ray but that came back clear, thank goodness!  Since she has RSV, the doctor said she should not be doing any traveling for at least 6 weeks!  So we will just have to reschedule for another time.  I was kind of looking forward to going out there and putting all this behind us but things happen and we’re doing our best to get Brookie better.

She has been home and getting lots of rest, fluids and nutriton through her feeding tube.  She’s been entertained by Disney movies and her favorite is Cinderella!  We’ve been using the cough assist machine to help her clear secretions from the lungs.  When she breathes in, the machine gives her air to help expand the lungs.  When she blows out, the machine creates a sucking force that pulls air out of the lungs.  This rapid change in pressure during breathing helps make the cough stronger and better.  It is something we should be using daily but is essential in times like this when she needs the help with her cough and preventing pneumonia.

Brooke coughing in the cough assist mask

Please say a little prayer for Brooke that she gets over this virus quickly!  Thanks for all of your support!

One Year Ago Today...

One year ago today Brooke and I made our routine visit to the University of Utah for her Stop SMA clinical trial visit.  We had been sending weekly weights to the dietician and trying so hard to get Brooke to eat and gain weight.  Every single ounce was a small victory because they were so hard to come by.  After months of trying to get her to eat and gain weight, the decision was made while we were in Utah that it was crucial that Brooke have a feeding tube for survival.  FAILURE TO SURVIVE.  That was the technical term on all the paperwork that always hit me like a ton of bricks making me feel like an inadequate mother who couldn’t feed her child.  It was our choice to have the surgery in Utah.  We wanted to have the team that knew Brooke and who have followed her since she was 3 months old be there for her surgery and recovery.  It one of the hardest things I’ve had to go through… watching her be taken down the hallway for surgery, giving her that last kiss before she entered the surgery room and the wait for them to call me and let me know it was over and how she is recovering.  I knew that she was in good hands, she had the best surgeon and we were surrounded by our usual SMA clinical trial team and Dr. Swoboda was right there for everything!  I mean, seriously, everything.  My words cannot express how much respect and gratitude I have for her.  She was comforting, always there when I needed her, answered every question I had, spent time with us every single day after surgery, talked with each nurse that we encountered, figured every single kind of dosing, made phone calls, called in favors, etc.  It was just what I needed during such an emotional time.  Looking back it is still a very emotional thing for me but I look across the room at Brooke crawling and pulling to stand and it puts a smile on my face and makes everything worthwhile.  Having the gastronomy tube and nissen fundoplication surgery was the best move we made and Brooke is thriving and doing SO well, she’s gained weight, started eating by mouth again and asking for food telling me she’s hungry!  It’s like music to my ears! 

At the same time… one year ago… the Friends of Brielle & Brooke Organization and Facebook Fan Page were formed by friends and family.  The Friends of Brielle and Brooke Organization put on two big fundraisers to help our family with the intention to cover the costs of a wheelchair conversion for our van.  We were able to have the van converted last August (2010) and it has made our life so much easier transporting wheelchairs, gait trainers, and a mobile stander.  We are so grateful to our family and friends for all the wonderful support you have given us!

The best part is that we are so fortunate to have a surplus!  That extra money has gone to:

  •     Brooke’s electric wheelchair – our portion of the medical costs.
  •     24 Hippotherapy sessions 
  •     We are using up the remainder of the funds to buy Brooke a mobile Standing Dani!!

We thrilled to have these life changing pieces of equipment for our girls and we would like to thank everyone who was involved.  This summer will be lots of fun exploring the outdoors and I envision Brielle and Brooke playing “tag” in their mobile standers and lots of giggles and belly laughs as they experience something they have never been able to do before but only dreamed about.

Thank you from the bottom of my heart for all of your support over the past year.   

 

 

Brooke's New Permobil K450!!

Wow, what an exciting day for our family and Brooke! 

She is now the proud owner of a Permobil 450MX Power Wheelchair!!  This piece of equipment will be life changing for her.  It will give her the freedom to move independently …and quickly!


This K450 model has it all.  It is a very impressive chair with lots of functions! The best function is the ability to lower all the way to the floor so Brooke can crawl out and in all by herself.

   

THE chair! At home in the sunshine to show the color.

 

First time in the chair.

 

Lowering it to the floor.

Climbing back in!

 

turning and getting into a sitting position.

 

She’s off and “running!”

Brooke will still need lots of practice driving and we can’t wait for spring to get outside so we have lots of space to move!

After a 3 hour fitting appointment, she is exhausted and ready for a nap!

Naptime!