If you read the beginning of Liam's Story then you know how Liam was diagnosed with A-T. You know that it nearly broke us all. And that it changed us forever, as it should. Being a parent is hard. Being a parent of a special needs kid is harder. Being the parent of a child that you know will die before you, the parent, is impossible. Impossible to wrap your mind around. Wrap your heart around. Wrap your faith around. BTW...we have NEVER sat down with Liam & told him about the expiration date that looms on his horizon. It is hard enough to get through a regular, atypical kind of life. It is even harder to get through a life filled with sickness, hospitals, Dr appts, blood tests, medicines, injections, etc... without knowing that you don't even get to live to a ripe old age, no matter how many of the former you spend enduring. Besides, we figure we are all going to die. Depressing right? But it's true too. None of us knows our fate. I could step off the curb tomorrow & get hit by a car. Or our house, with us in it, could get flattened by a tornado. We don't get to know when we die, why should we freak Liam out with one more depressing detail about his future? And since we don't know how long he actually gets, it seems pointless to speculate. He doesn't need that on his shoulders too. Hell, we wish we didn't know it & we're the parents. Ignorance is bliss is a true sentiment. However, he is an intelligent kid. He's intuitive & far more observant than I'd like. Does he probably know on his own, maybe not the whole ugly truth but enough, yeah he probably does. And if he ever asked us, we would sit down & have the conversation, honestly, with him.
But there is more to his story. Liam was officially diagnosed on Labor Day Weekend 2005, he was 4 years old. The initial grieving process for his parents took until spring of 2007, he was now 6 years old.
This is what happened next...
One day I was looking for a group I could join online of other A-T families so that we wouldn't be alone. We were at that time the ONLY A-T family in all of Arizona. I wanted to ask questions, & while Liam's neuro at Children's was familiar with A-T from a Dr's view he didn't have any other A-T patients & not having it in his family could not help me/us. I needed someone I could reach out to for support. So I went back to the A-T Children's Project website. The page has changed a lot over the years. From there I learned there was a man who was going to be running a bunch of marathons to raise awareness & donations for the ATCP. But he wasn't going to be in my area. Eventually, frustrated by the website & it's lack of information I simply called the clinic. Where I was introduced to Aletia. Just the most wonderful voice on the other end of the phone. A lifeline that saved us a million times over from falling into a deep depression. I'm so thankful for Aletia.
Fundraising:
She told me all about Tim Borland, sent me to YouTube to watch the trailer for a documentary film he was working on about A-T, called 'Feat'. You see Tim was an ultra marathoner, yeah I had no idea there was such a thing as an ultra marathoner. He ran lots of marathons regularly. Crazy man! He befriended an A-T family in California & was inspired to run for our kids. He ran 63 marathons in 63 days. Literally a marathon a day every day for 63 days. We signed up to be one of the stops along his route & went to Disneyland on Labor Day weekend, on Liam's 2 year A-T diagnosis anniversary, for the 1/2 marathon, he ran it twice, once by himself before the marathon started, & then again in the actual 1/2 marathon. While there we met at least a dozen other A-T families, kids of all ages & for the first time in 2 years we didn't feel alone. We even had runners signed up running for Liam in the Disneyland 1/2 marathon. It was a life altering, profoundly moving experience for our family.
Surprise, AZ, where we were living at the time, was Tim's 13th stop of the Cure Tour. That man ran in 110-degree temperatures around the Surprise stadium for 2-3 hours. The night before we hosted a great big fundraising dinner. With local pizza places having donated to our benefit dinner, and a bunch of AZ based businesses donating services, products, memorabilia to our silent auction, that ran during the dinner. There were speeches given, and auction items awarded, our friends, family, community, church members rallied around us for months in preparation for the big event. I had a committee of friends who helped me every step of the way to make this all happen with only 4 months notice. From donations to logistics, to flyers, to selling heart papers outside grocery stores on on Saturdays during the long hot AZ summer to raise awareness & donations, to the school selling the hearts as well, to newspaper articles, to local coverage. One of our friends spent days making & baking 100s of desserts for the dessert table. We raised over $6500.00 on our day of the Cure Tour. It was amazing! We hosted Tim, his wife, & their 2 beautiful kids in our home, only for one night but were so grateful & privileged to be of any kind of support during his 2-month journey for our kids.
Now, in full disclosure, our day didn't actually make it into the finished movie, but there were a lot of stops along the way & thankfully we were still able to see this footage from the daily updates along the way. There aren't sufficient words to describe the elation & gratitude we felt, & still feel, for being a part of the Cure Tour. That year raised the ATCP over a million dollars. It is these grassroots fundraisers that make up more than 99% of the monies raised for the ATCP that goes on to find life extending therapies & ultimately, hopefully, a cure for this awful disease. That year also changed how we felt & looked at Liam's disease. It woke us up. Gave us something to fight. Helped us see we didn't just have to take what this disease threw our way. We did another fundraiser a couple of years later, not nearly as big or as successful, monetarily, but it gave us that feeling again. It was a Rim to Rim hike across the Grand Canyon, in one day. My husband got behind this fundraiser. But again our friends, the community, & even complete strangers signed up to participate in the hike. Logistics for hiking the Grand Canyon btw are a nightmare. CA, and one of our neighbors Heath, were the last 2 to come out of the canyon that night. And it was terrifying waiting for them to emerge. With both of them having strained/injured themselves during their trek. Ask him if he would do it again, he'd say, 'In a heartbeat'.
Have we done any other fundraisers for the ATCP since then? No. Sadly the economy hit our family hard at the time & it involved a new job, & a great big cross country move. Then Liam's health began to deteriorate. And we just don't have the contacts we once had, nor the support system we had in Surprise. Would I like to organize another fundraiser for the ATCP? Very much so. Liam's health is better, our finances, while still strained due to the plethora of medical expenses, are better. Alas, I've become something of a hermit. Afraid of germs that could expose Liam to more hospital time. And I have forgotten how to make friends in the real world. I am not on FB, nor do I have any desire to be on FB. Liam has a personal FB profile page for close friends & far away family to keep in touch with him, and he has an Official Stubbornly Liam FB page to help spread awareness about A-T & to help those who have met through the years to keep up with his health. And while I do have one form of social media, Instagram, and of course this family blog. I am not big on the social media world. As an introvert, I find it draining trying to keep up with everyone now. I don't have the energy to constantly update my status or to constantly check everyone else's status. I'd rather talk in person than over the internet.
School Issues:
In the middle of fundraisers, I fought the school district for a 1:1 aide for Liam. This was a battle for which I was unprepared. I fully expected to walk into his IEP meeting expecting them to approve the request that he get someone assigned to him that would be with him every day all day at school. The district refused to spend the money on someone to be with him. He came home daily in tears because he was alone, he couldn't keep up with the school work, with his classmates, he had no one to talk for him when he was tired. No one to sit with him in patience & help him as he struggled more & more with his muscle ability. He never has learned to read more than a few words, simply because he cannot control his eye muscles & track the words for more than a few seconds. He cannot write more than a couple of words because he cannot control the pencil long enough. He was losing his ability to walk as well. Life was becoming harder & harder for him to physically keep up with his friends.
His teacher, bless her heart was stuck between a rock & the district. Not allowed to say anything in front me or to me about how she desperately needed help in the classroom, she finally told me one day, she wanted so much to be able to do more & be more to Liam in class but that meant less time for the other children, which wasn't fair. He had great friends who wanted to help him but that meant their own work suffered, which wasn't fair to them either. Fed up I finally decided, since I am legally allowed to call as many IEP meetings as I deem necessary that I would do just that. I called for one IEP meeting a month. Finally in late October, while my family, myself included, were all fighting a really bad stomach bug, for about the 3rd time in 6 weeks, it just kept going through the house over & over & over, I walked into the conference room with my husband, on one hand, sick toddlers on the other & a bunch of paperwork in between. It was 7 am. And I didn't even bother sitting down, even though my body was begging me to curl up in a ball on the floor & throw up. I slammed down, on the big conference table, my list of rights from the state education board, then letters from various specialists that oversaw Liam with their medical advice that he be assigned a 1:1 aide immediately, a letter from the ATCP, stating the statistics of A-T kids w aides, as well as a letter from the A-T Clinic at Johns Hopkins Hospital stating the reasons he needed a 1:1 aide assigned asap. Finally I slammed down, on top of all of this, the local newspaper article & the contact info to the reporter who covered the Cure Tour when it came to Surprise & explained that I was positive this reporter would love to cover why the little boy, that pulled the community together just 6 weeks earlier, was being denied basic human rights by the elementary school he was attending. Two seconds later the district administrator who had attended the meeting asked me to calm down, that they had intended the whole time that at this meeting it would be approved that Liam receives the aide as requested. I didn't care how much BS I was wading through, all I cared about was that aide. I was told that they would have to hire & train someone before they could assign them to Liam & that the interviewing process would start immediately but that I would still need to patient. I acquiesced & by the return to school after winter break Liam had his 1:1 aide. We loved Mrs. Jodi & were heartbroken 2 years later when we had to move across the country & leave her behind. He's been blessed since then with contained special needs classrooms & lots of paras working at each school he's attended, until this last year. Once again I find myself having to circle the wagons, gather my paperwork & organize the request for a 1:1 para to be assigned. It is a long drawn out process & one I dislike. But I will be a force to be reckoned, should they fail to cooperate & act on my request this time.
Physical Issues:
Liam received his first walker, a vibrant electric blue walker at the age of 6. within a year he was in his first manual wheelchair. He & I, with the accompaniment of grandma were able to fly back east in April of 2007 to Johns Hopkins & the Kennedy Krieger Institue, where he was poked, prodded, studied, examined by 12 different specialists in 3 days. By the time Liam was 10 he received his first electric wheelchair. and by the time he was 12 years old was having a PEG tube placed to give him a chance to live. His immune system was gone. He weighed 40 lbs, at the age of 12 & was diagnosed, 'Failure to Thrive', which means he needed medical intervention in order to keep living. Right now he is going through another type of decline in the disease. It's part of the premature aging process. His joints & muslces are stiffening, his bones are losing density. His body is failing him as if he were of geriatric age and not a teenage boy.
The Health Decline:
In July of 2013, we went our first family vacation in 3 years. We had planned a big trip that would take us to the Wisconsin Dells & then to Mackinac Island, MI. The morning we left, Liam woke up throwing up. We had prepaid, non-refundable reservations, & Liam is prone to throwing up so we figured it would take a few hours but he would be alright. In fact, he threw up so much at this point in his life that he had a regular prescription for Zofran, an anti-nausea med. Our first stop was the Dells & the kids were so excited to see the water park capital of the world. Liam & I spent pretty much all day just lazing in the shade, him throwing up occasionally into a plastic bag, sipping water, nibbling on crackers, the same thing he would be doing at home, while CA & the kids ran through the park. By the next morning though, he still wasn't feeling well. He just looked miserable. And the kids weren't all that excited about the parks anymore because the lines were long & because with only one parent available to take them around it got boring really fast. So we left a day earlier & headed to Mackinac Island. The drive gave Liam more time to rest but he just couldn't shake the stomach issues. We arrived in Upper Michigan & left Kier in charge of everyone resting in the hotel room, while CA & I had a quick dinner date. We arrived back to the hotel, where Kieran was just about to call us to come back asap. Liam had burst into tears, thrown up, screaming he wanted mommy. I checked him out, he had a fever of 102. But I couldn't see anything actually wrong with him. No swollen glands, no scrapes, or bruises, or anything bad. I gave him Ibuprofen & laid down with him & prayed. By the next morning, he felt fine again. We went over to the Island & walked all over the place, ate yummy food, delicious ice cream, the famous Mackinac Island fudge & got back to the hotel room by 7 that night. Everyone had a great day! The island was beautiful. We arrived home a couple of days later & the fever came screaming back. 103 fever, vomiting, this time accompanied by severe ear pain. One emergency trip to the pediatrician later & we discovered he had a double ear infection, throat infection, & bronchitis. Bronchitis with no coughing seemed so weird but it was Liam so... a month later he had the same thing, but instead of bronchitis pneumonia. A month after that he had it all again. We finally got in to see a pediatric neuro, this was a long process as almost all of the ped neuros at Children's in Milwaukee had retired or moved out of the area. We ended up going to Chicago children's for a neuro, who used to be at Milwaukee Children's & knew the staff there really well. She sat down with me, Liam was hanging with the nurses while she & I had a terrifying heart to heart. She explained how worried she was about Liam's health & especially his weight. I agreed with her. I was terrified. She explained to me that Liam was in a great deal of danger & that CA & I needed to prepare ourselves for his demise if we couldn't get him healthy & now. She gave me all the names & direct numbers to the specialists at Children's in Milwaukee. Who to ask for & what to tell them, in particular, that SHE was the one who told me to call them specifically. By the beginning of December, we had appointments with EVERYONE we needed to have appointments. It was during one such visit with Immunology, that Liam happened to have a high fever again & he was checked into the hospital.
During this visit to the hospital, all the specialists came to Liam. We didn't have to wait for appointment after appointment. It was at this point the immunologist informed us of his lack of immune system, specifically he doesn't make any IgG, which is the basic makeup for your immune system & that he would have to start immunoglobulin therapy. We were visited by the Special Needs Team at the hospital & were informed Liam would be added to their roster of kids with complicated diseases that required many specialists. That they were the ones who become his case managers. He saw the GI specialist, the one who would ultimately place his PEG tube & then switch it out with a more permanent Mini-One Button. We were also introduced to his pulmonologist who would put him on a bunch of different breathing devices over the next year. The list goes on & on. On February 3rd, 2014 Liam had his PEG Tube placed. It was a quick 30-minute procedure, that should have only kept him in the hospital for 24 hours. We had worked so hard to get him up to the 50lb requirement they had in order to even place the tube or it would be too much for his body to handle. But the 24-hour stay was extended to 5 days. He spiked fevers that made me panic. He couldn't get up the strength to leave his bed. He was fading. It was too much & we were seriously close to losing him. What pulled him through? What put the light back into his eyes? A dog. CA had been talking about getting a hunting dog for a while & on the way to the hospital saw a sign for Vizsla puppies for sale. He stopped & took a look at the litter & fell in love with one. Almost completely weaned from mama too. When he told Liam he was getting a puppy but that Liam would have to name her so he had to get better to meet the puppy Liam perked right back up. Animals are his favorite thing on the planet. That little puppy, without even knowing it saved Liam's life.
Ruby came home the same night Liam did & she was the reason for his smile. Full of far too much energy for him to play with her for at least a month, she made him laugh & smile & keep going. You can see in the video she wanted to get to him so badly. Because we had stairs in our rental house, Liam & I slept on the couch for a couple of weeks while he recovered. Ruby was in heaven with the boy sleeping right there in the same room as her, she would have preferred to sleep on his head/body, but as his body was recovering from surgery it wasn't going to happen & instead she slept in her crate.
He was in & out of the hospital with infections, fevers, issues over & over again over the next year. I even had a piece of my luggage packed & ready to go to the hospital any time I needed to, my "go bag" is still in my closet, although I haven't needed to go in over a year, which reminds me I need to go through & make sure nothing has leaked & that none of the items in it need refreshing or replacement. It was once they put him on Azithromycin 3 times a week prophylactically, plus allergy medicine, plus immunoglobulin infusions, plus steroids (these ones were temporary, & as needed) that we finally noticed an improvement. He has a lot of equipment in his room; a Vest Clearance System, a cough assist device, a nebulizer, his Joey Pump (for his formula feeds through the Mini-One Button located in his abdomen, & last but not least, a hospital bed. Soon he will have a Stander (for physical therapy) in his room as well. I am sure there will be more & more equipment needed, as he ages through the disease.
This is the life of an A-T kid. This is what happens after the diagnosis. This was not the life I had planned out for any of us. But therein lies the foible...I tried to plan life. Something I have learned is a ridiculous concept.
Liam's Morning Routine:
Go to the bathroom
Get dressed
Use Nebulizer (w saline solution) breathing treatment
Use 3 puffs (8 breaths each puff) orange inhaler
Use 2 puffs (8 breaths each puff) red inhaler
20 minutes on Vest treatment
5 minutes of cough assist
Brush teeth
Put on SMOs (over socks)
Put on shoes
Gather supplies for school
Gather lunch & a bottle of formula for gravity feed
Load up Liam & power chair in van & head to school
Pick up 2 pm (regular school day for high school is 7:30 to 3 pm.
Liam's Nightly Routine:
Shower
Pajamas
Probiotic pill
Nebulizer
Orange inhaler
Red inhaler
20 minutes Vest
5 minutes Cough Assist
Brush Teeth
Allergy pill (by mouth)
M, W, F get Azithromycin (through tube)
Set up overnight feed w Joey Pump
Go to bed
These routines are for when he is healthy. When he is sick they take place every 4 hours. The meds would change, depending on what he is fighting off. He also has to have at least 30 ounces of water on top of his 4 bottles of formula daily. He does eat by mouth daily as well (because he gets his real nutrition from the formula, he is allowed to eat when he wants, & whatever he wants; to make eating enjoyable again, because it wasn't enjoyable for him a long time, before the button was placed) but when he is sick he mostly lives off the formula as sickness tends to rob him of the ability to swallow safely. His brain is just too tired & overwhelmed to coordinate the muscles needed for swallowing without aspirating his food or drink. Showering, changing, going to the bathroom, eating requires a great of help &/or assistance from the rest of us.
He now weighs 95.6 lbs. It has taken us 3.5 years to go from 50 lbs to 95.6 lbs. He's 16 years old. He will never drive. Never dance. Never be able to date without a parent present to assist. Never marry. Never have kids of his own. Most likely he will not be able to attend college, his health is too precarious & his strength is fleeting. If he even lives to college age it will be a miracle. Drs tell us at every appointment that we need to start preparing for the end of life decisions now that he is in his mid teens. This is what happens next...and it sucks. But it is our reality. It is Liam's reality. It is what it is. If I could heal him, I would. If I could carry the disease for him, I would. It doesn't work that way. Liam, even though he is surrounded by family & friends who love him dearly, is essentially alone on this journey. He has to get through these trials alone. He has his faith. And he has our faith. And he has our love. And they do offer comfort but ultimately he is alone. There are great blessings awaiting him in heaven for the things he is enduring so beautifully here on earth, I truly believe that. Those blessings are hard to wait for though when you are in pain, afraid, sick, and so very tired.
This is what happens next...
Our A-T kids are our heroes for a reason. They endure so much. They deserve so much more than they have been given. This is why we beg, some times at the top of our voices, for awareness, for donations to find a cure. We want so badly to make a difference in their lives for the better because all we can do is sit next to them & watch them suffer & endure. As a parent, it is the most torturous thing to endure. Watching your child suffer knowing there is nothing we can do to stop it or change it. We treat each symptom as it appears, we comfort them when they struggle, we fight for them & advocate for them & raise funds & awareness for them, but we can't miraculously heal them.
Combine the worst symptoms of Cerebral Palsy, Cystic Fibrosis, Multiple Sclerosis, Cancer & Immune Deficiencies & that is Ataxia Telangiectasia. Most kids are wheelchair bound by the age of 10, Liam has been in a wheelchair since he was 7. If they don't die of cancer (leukemia or Lymphoma being prevalent of the types of cancer) then it is typically pneumonia that takes their lives. It causes premature aging, affects their heart & kidneys as well causing heart disease &/or diabetes. The average lifespan is now, thanks to the research that our fundraising has made possible, the early twenties to mid-twenties. 12 years ago when Liam was diagnosed it was late teens, meaning if not for the life prolonging therapies that research has provided Liam would be at the end of his life right now. So when I say your dollar could be the dollar to fund a cure I mean it. Literally.
Go to the A-T Children's Project to learn more about the disease, to help raise funds yourself by starting your own grassroots fundraising project, you don't need to know or live near an A-T kid to help, or by simply donating & helping to spread the word to others to donate their time, their energy, their money. That is what happens next... You. You can make a difference between life & death for an A-T kid. To alleviate the suffering of another A-T family from the loss of their A-T child at far too young an age. Please help us help our children. So that together we can change the this is what happens next from a young death to a long life lived fully. You can change what happens next.