acyclovir – beatingcowdens

It’s June. It doesn’t feel like it. At least the weather doesn’t. It’s cool and rainy. I guess that’s OK for now – while we are still wrapping up school.

It has been a long week.

I don’t usually leave my blog unattended for so long. I am behind at writing. I am behind even further at my reading. It seems the days just blend together lately.

It is June, and when you are a school teacher, this is a month of eager anticipation, and volumes of paperwork to be settled. There are boxes to back and things to carefully put away in preparation for the fall. There isn’t much time to be still.

Well – in another 2 weeks it is!

It’s June, and when you are an advocate – you do take a few minutes to celebrate the victory that put speed bumps on the street where you had the accident that damaged your back forever and ever. You are grateful for the citizens, and politicians alike that fought relentlessly. You are thrilled by speed bumps, but you still want that stop sign. You celebrate with a glass of wine – or two.

It’s June though, which means that damaged back has to ache longer in between trips to the chiropractor, and the PT you promised yourself seems like it may never happen. You are the mother. You will get by.

It’s June and when you are a Mom of a kid with Cowden’s Syndrome you spend 2 or three afternoons a week at physical therapy to make her chronic pain bearable. Not totally sure the pain is related to the Cowden’s, but sure it’s related to SOMETHING, you scoff at the denial for school based PT and wonder what they would say if one of them could spend a morning in your house watching your 9 year old walk like she’s 90. You balance those PT appointments with swimming lessons, all in preparation for the team she will join. The team she is desperate to swim on successfully, and God willing – pain free.

It’s June, so you balance the breakthrough of the virus on that adorable immune compromised 9 year old’s face with increased doses of the antiviral medicine and extra trips to the pediatrician. It’s June so when it’s not pouring – you make sure she has a hat to keep the sun off her face. And when you look at the dose of antiviral medicine you start to feel a bit guilty, nervous maybe, about her liver – and all the prescription medicine. So, you take a chance and toss the Celebrex to the side. Hoping maybe, just maybe she can get by without it.

It’s June and its raining. You feel a little guilty about “forgetting” to tell her you stopped the Celebrex, but each day you hear the complaint of another joint, another ache, another pain. Ten days later you abandon your hopes of relieving the stress on that young liver, and you relent. Too many Tylenol – not cutting it. Celebrex it is.

The war rages – all the months. The battles are won and lost on a regular basis – but the war looms large. I don my armor – a large binder of medical facts, bloodwork, and reports. I gather my inner strength.

It’s June. Summer vacation is coming, but there will be no camp in our house. It doesn’t fit in with the schedule.

Every six months. Every doctor. Forever. Mine, and hers. Different doctors. Different times. Different facilities.

I am getting better at the scheduling. I have learned to bunch them together. So, we go in February and again in July.

For Meghan it’s the thyroid first. That foreboding nemesis. Ultrasound, appointment… and we will see what comes next. Then its the AVM follow up, and the genetecist. That’s just the last week in June.

Mom has an MRI to schedule to look at that spleen, some more surgical follow ups…

There will be 15 appointments before the 2nd week in July. That’s if every one goes well.

This is how it has to be. We have to work, she has school. We can’t have the appointments all throughout the year, so we must endure them all at once.

It’s June. I am already tired. Wrapping up one full time job to focus on another. I feel my anxiety rising.

Getting all my rest. Gathering my inner strength. Armed and ready. Kicking Cowden’s to the curb…

We can… WE WILL!

I have this delusion in my head of what a Saturday should be. I think sometimes about getting up – not at the crack of dawn, but at a reasonable hour. Getting a quick breakfast and jumping in the car to head… well wherever we want to go. I think about spending Saturday as a family. Worry free. Relaxing together. Sharing an adventure.

Then there is reality. It has a way of biting you in the behind when you aren’t paying attention. (Or when you are practicing active ignoring.)

Reality is that we have two working parents in this house. That same reality that allows us to pay for the medical bills that creep up, and the fun stuff like vacations, is the same reality that means Saturday afternoon adventures are uncommon. Well, almost unheard of anyway.

I have a grocery shopping routine – down almost to a science. About every 3 weeks on Friday night, I go on the marathon. I stop at Costco, and run home with what we need. Then I head to Wegmans in Woodbridge, and Whole Foods in Millburn. The whole journey is about 40 or so miles, and it takes about 6 hours from door to door. Then there is the unpacking…

Why?

Well food sensitivities have kept Meghan gluten, dairy, and soy free since she was about a year old. She is also very sensitive to dyes, preservatives, nitrites, food coloring, and the like. Her diet is pretty much pure and organic.

Local shopping is not as plentiful as that over the bridge, and prices are flat out better there. So, last night as we undid the (ridiculous amount of money) worth of groceries, and I put a soup in the crock pot for today, I was about to fall over from fatigue.

The piles of laundry already covered the basement floor. The beds would need to be stripped in the morning. The bathroom was in dire need of a cleaning after my husband repaired a pipe that burst under the bathroom sink. I thought about shutting down the alarm and just letting my body do the talking this morning. I thought about it for about 3 minutes.

Then I took a flashlight into Meghan’s room. I covered her eyes and looked at the sores on the right cheek of her sleeping face. My heart sank. I turned the alarm on. I knew where we had to be in the morning.

In addition to having Cowden’s Syndrome, Meghan suffers with an immune deficiency. Well, technically speaking probably more than one. She is IgG subclass 1 deficient. Her body doesn’t seem to remember how to fight infections. She is also Mannose Binding Lectin deficient – a whole other element of the immune system – just absent.

Usually she holds her own. She takes a HOST of vitamin and mineral supplements. She eats that pure and largely organic diet I spoke about, and aside from seemingly chronic fatigue, she does OK most of the time. She can’t be running around too long or too often. She can not be over stressed. These things wear her out, and then we have trouble.

When she was just 6 months old she was first hospitalized with an infection of Herpes Simplex 1 (fever blister) on her right cheek. It was odd the location it started. It was in the middle of her face. Before we knew what it was it had become cellultis, and we spent a week in the hospital. The first 2 days were the scariest as the blister kept increasing in size. Eventually my pediatrician at the time called in an infectious disease specialist (my pediatrician now,) who swiftly diagnosed the herpes simplex, and began IV acyclovir. Within 24 hours things calmed down. A week after we were admitted, we got to go home.

However she never, despite a few miserably failed attempts, got off the acyclovir.

She spent another week in the hospital at about 20 months, treating an aggressive outbreak.

At one point we slowly weaned her to 500 mg a day only to have her develop a very painful trigeminal neuralgia which the oral surgeon wisely explained was caused by the herpes virus taking up residence in the trigeminal nerve in her face.

We promptly went back up to full dose, only to watch the pain fade away in a few days.

We have seen minor outbreaks through the years – usually nothing more than a red mark to indicate its time to do something. We have slowly watched her dose of acyclovir be increased. Currently at 80 pounds she was taking 1400mg a day.

We worry about the liver. About whether there are long term effects. But there really is not a choice.

That’s why as I shined the light on her face last night I knew this morning would involve a trip to the pediatrician.

We arrived bright and early and as he greeted us warmly he sized up her face in one brief glance.

“I see our old friend is trying to make an appearance. Raise the acyclovir to 1600mg. Move to 3 times a day to help it get through the system better. Take a picture every morning, and call me if it doesn’t improve. I will see you in 2 weeks.”

We stopped at CVS. We texted a friend’s mom for a play date. Then we got home at about 11:30 – drained.

Felix was working to bleach the bathroom – a job my asthma doesn’t allow.

There were human beds and dog beds to wash. There were floors to clean, and dishes to do. There was spaghetti sauce and some gluten free cookies to make.

As I heard the sound of giggling girls from behind Meghan’s closed bedroom door – I took solace in the fact that at least she had an adventure today. Even if it was in the house.

Cowden’s Syndrome and the immune system don’t seem linked on paper. But, anecdotally I hear of issues from every Cowden’s patient I correspond with. What goes on in our bodies to make it just so hard to put a virus to rest?

One child. One diagnosed genetic disorder on the tumor suppressor gene. A mannose binding lectin (a protein) that is also missing. An IgG subclass deficiency. Metabolic errors still being unearthed.

And I was looking for an adventure?

I should be happy and content with the Saturday cleaning. It is the most rest we seem to get!