Yesterday CA, Liam, & I had a very busy day at Children's Hospital of Wisconsin (CHOW). It was Liam's annual getting ready to go back to school check ups with his main specialists. We had 4 appointments starting at 10:30 in the morning & ending at 4 in the afternoon. This is a normal hospital day for Liam & I, but his dad was able to take off work to accompany us. Thank goodness because getting Liam to the bathroom is getting harder for me as he is just about the same size I am, height wise. We also see the specialists every spring (end of the school year). It used to be everyone wanted us in there every 3 months so they could keep a close eye on his frail health, but we usually ended up seeing them once a month because he was constantly sick or at least talking to them at length over the phone. But it has been nearly 2 years since Liam's last hospital stay. Right at that point, they decided that IgG infusions weren't enough to keep him healthy so they added a prophylactic antibiotic 3 times a week to help with inflammation in his lungs. And the 2 combined have made a huge impact on his ability to stay healthy...so far. (knock on wood)
We started the morning off with Immunology. Immunology is THE main dr for a kid with A-T. They are the one who watches out for his main system, & watches for signs of cancer. They tell you who you need to see for everything that comes your child's way. For the first time in, I cannot even remember how long, it was nothing but positive take aways. Healthy weight, healthy skin, eyes, nose, throat, lungs, blood pressure. No questions from me, nothing that concerned the Nurse Practitioner. She ordered labs for his IgG blood panel to check on his T-cells, lymphocytes & IgG; which are typically low & to make sure it was time to up his gamma globulin dose from 8 grams to 10 grams as he is bigger & they want to make sure he is getting a large enough dose to keep him safe, especially as flu season is coming. The results from the blood work should be in next week.
If you are wondering what all those things they were looking for mean? I can give you the 411, lymphocytes are white blood cells... made up of T Cells & B Cells. They are what determine your body's ability to fight off different types of infections. B Cells fight off bacterial infections & T Cells help fight off viral/fungal infections. When the T Cells are low, like they are for Liam, (which btw is more common than a "too high" T Cell count), then you're at risk for yeast infections, especially around his g tube site & in his mouth and Influenza. Which is why it is imperative that our family get the flu shot, which is a dead version of the virus. Not the mist, as that is a live version of the virus, and is more likely to give him the flu. Now, having a low T Cell count can also lead to cancers like Lymphoma, Leukemia, & Hodgkin's Disease. So we watch those T Cells regularly. And every check up includes a lymph node check as well.
IgG is the main component of your immune system. Years ago when we first went to the immunologist he described it this way... You know how your computer has a main operating system? IgG is that main operating system for your immune system. You have other Ig Factors in your blood, just like you have other programs on your computer, & together they make up your system. But IgG is the basic OS that allows your computer to turn on & add all those other programs. If your OS goes down you cannot turn on your computer. Well, if you don't have enough IgG to run your immune system you are in a similar situation. So every other week Liam gets an infusion, it takes about 90 minutes, into his abdomen, just under the skin. This is called SubCutaneousImmunoglobulin, SCIg. As it goes just under his skin, in his case he gets three small needles w tubing & an automatic pump that infuses the immunoglobulin into his system. And yes I am the one who has to stick him. So much fun. (I really would love for some one to invent a sarcasm font) The medicine he gets is called Hizentra. It's the part of blood plasma that contains antibodies. So it's plasma that is washed & separated into just IgG. Which means we get to keep a Sharps container in Liam's room for disposal of needles & anything else that comes into contact with the plasma, tubing syringes & the empty bottles of Hizentra. Yay us...again sarcasm. Since he has grown in both height & weight it's time to up this dose of IgG. So my main takeaway from the first appointment is he is doing so well & growing so well because he is healthy & strong that it's time to get more medicine to keep on this awesome path. It is so rewarding to see all the hard work keeping him healthy reflected in the eyes of the Drs & nurses when they say, 'good job', to us. Feels a lot better than the pity looks & hugs they give on bad visits.
Next up was the Pulmonary Clinic. He always has a lung function test first. Then the appt with the actual pulmonologist. This test includes a great big machine w a long tube & an attached mouth piece that he has to breath into really hard for a sustained period of time (as long as he can) & then suck in air, really big too, so they can measure how strong his lungs are at the moment. They do 4 or 5 breaths without albuterol, then they give him albuterol, wait 10 minutes & have him breathe into the machine again 4 or 5 times. This is, for Liam, akin to running on a treadmill. All that heavy breathing in & out & sustained breathing is exhausting & usually drains him for the rest of the trip so I always try to make sure this is the final appointment. Unfortunately, that wasn't possible this time but we did schedule it so that we could get a 2-hour break before the next appt & get lunch.
The pulmonologist, like the immunologist, had nothing but good things to say. His lungs are about the same, with just a small improvement from our last visit, so the take away is they aren't drowning in fluid like they were 2 years ago. They are healthy...for him. He still needs both the steroid inhaler & the albuterol inhaler, as well as all the machinery mentioned in my previous post, "This is what happens next...". She also said, provided he does well this winter, then next spring when we see her again she'll consider taking Liam off some of the equipment & the steroid inhaler for the summer, giving him a 3-month break. YAY!
Following the Pulmonary Clinic, as previously mentioned, we stopped for lunch at the Subway in the hospital. It was a chance to sit & veg for a good 45 minutes & just breathe. Immediately after lunch, we walked next door to the lab for blood work. After that, I needed to drop off paperwork from the high school at the GI clinic for his feed schedule/prescription from the dr for the school nurse's office. Then it was back into the main hospital, we walked all over the place, with Liam in the lead. Yeah, when you're 16 years old & know your way around the hospital as though you are an employee there, that's not right, but that's our normal. Once in the hospital, we headed down to the lower level for Liam's 3 years check up on his cerebellum, via MRI. They keep a close watch on the degeneration in his brain so that if something happens they have an updated control image at all times. Plus, he was recently diagnosed with ocular migraines so they wanted to double check & make sure there wasn't anything physically causing the migraines in his eyes. Again, we'll get the results back in a week or so.
Because we arrived about 90 minutes early they were able to get us in earlier than our scheduled appt. So instead of his imaging starting at 4 pm, we were able to start at 3:30. Thankfully we were done & out of the hospital by 4:45. After a long day like that leaving the hospital always feels like being released from prison. And it was a very long day. We arrived home about 6 pm. And by that point, Liam was pale, drawn, & just completely done with everything & everyone. What an amazing kid. Not one meltdown, just a quiet request, once I got him to bed, to turn off his light, close his door & let him watch TV in silence. His normal bed time in the summer time is 9 pm during the week. He didn't make it past 7:30.
CA & I were asleep by 9, I think. I don't really remember. Once I had taken my shower & had a cup of Nighty Night tea I was out for the count myself.
My total takeaway from the day's appointments was; Liam is in extremely good health, for him. He still needs the G Tube, lung apparatuses, & medicines & in fact needs to up a dose or two but he is doing beautifully. 3 years ago the doctors had him at death's door & quite frankly I think he was there too. It's been a quiet, uneventful 2 years, but we know that this too shall pass & another trial awaits us just around the corner. How long until that corner arrives we have no way of knowing. Just that it is there watching & waiting for its turn. Until then we savor each moment, each memory, each hug, each laugh, & thank God for giving us this time to spend with our boy. And pray we have the strength to deal when it's time for our boy to leave us. Because until a cure is found for A-T, this boy will leave us. That is a guarantee. And he'll leave us far sooner than we want him to go. Outliving your child is a horrible concept but for us, it is our normal.
As the month of August comes to a close, I am reflecting on what it has meant to be able to spend this month spreading awareness via all the social media I have at my fingertips. It has been a blessing to be able to share our daily lives as a special needs family but it hasn't been without pain as well. Being a special needs family doesn't make you or your family better than or worse than everyone else, it makes you different. In a hard but at times very beautiful way. It's very complicated & things do not get easier, they get more complicated as your child grows. It's hard being sick or different when you are little, but enduring it all throughout puberty is enraging. And yet he handles it with his character like humor & a humility & patience with which I don't believe I was blessed.
I know a lot of special needs parents. Of various special needs too, not just A-T. And I am always amazed by the grace these parents show in the thick of battle against the diseases their kids are battling. And I am even more amazed at the attitudes of the siblings of these kids. Being a parent of a special needs kid we tend to get the most attention but it's the siblings that really deserve the praise. My kids hardly ever fight. They regularly show love & compassion, not just to each other which is enough to make my mama pride skyrocket, but to other kids they meet & interact with at school & in the community. Now they are not perfect. :) They get sassy, frustrated, tired & hangry too. ;) They are human after all. But in general, I think they are positively angelic & so inspiring. I am so proud of all of my kids. Liam for his fight, and Kier, McKenna, & yes even ADHD Nolan, for their positive, loving, compassionate, funny attitudes. I couldn't do this without my CA, & my amazing kids!
With all that said, if there were a chance for a cure I'd take it in a heartbeat. So here is my last plea for A-T Awareness Month, please donate to the ATCP to help fund a cure for Liam & all the other A-T kids out there. Help our family fight this disease. Use these hashtags to spread our message...
#LivingwithAT #ATWarrior #HelpStopAT #KidsHopeCure #FightAT #AugustATAwarenessMonth #ATVolunteerSquad #ATCP #CureAT #BeTheHope
And thank you for your patience, love, support, time, energy, money, prayers. They and you are always appreciated!
All my love,
Jess aka Liam's mom