Dear Son Medical Update~Things are a Mess

This is a picture the day he got released from the hospital, earlier this month. It's not the greatest picture, with his long hair and all, but we were happy to be going home after two weeks.

Things have been difficult this week, to say the least. Dear Son is yelling/crying out a lot. I have removed his patch, put half the patch back on and then put the full patch back on and yet nothing seems to help. All of this has been to try to stop the crying.

Last night, Dear Son was up a minimum of once an hour from 2:30 a.m. on. I am exhausted. This went on for hours. Finally, I got up and tried to sit him in the recliner for a while but he began crying out so much that I had to put him back in his bed. The fact that he can't sit up at all is concerning to me. I ended up paging the doctor to see if there was anything I could give him to stop the crying. He told me to give him some Risperdal.

The good news is that the courier delivered the Scopolamine gel today. The gel replaces the patch and allows us to adjust the dose so essentially, he gets a dose that will last 4-6 hours to help dry up secretions at night and then conceptually, he won't need it during the day. As you may recall, he began to have the side effect of urinary retention with the entire patch; his bladder was huge and he was in pain trying to urinate. Hopefully, this resolves his issues.

I thought it might be interesting to talk about the types of questions that I ask when he gets a new drug. Many parents ask me what types of questions should they ask so I thought I'd share some of the questions that I ask and talk a little bit about why I ask them.

In this case, we have a drug from a compound pharmacy in a form we've never used. Since the drug was delivered by courier, I didn't have a chance to speak with the pharmacist directly but he did tell me to call him when I recieved the medicine. In this case, I was not able to check out the pharmacy, but in cases where they are making the drug, I would have liked to have seen the pharmacy, specifically, to see how clean they were. In this case, while I didn't get to see the place, Dear Son's physician specifically recommended the pharmacy so I had some assurance that they would be o.k. In addition, their automated voice recording states that they are a preferred provider of the largest insurance firm in our state.

This is how the gel arrived. This is a three month supply.

Prior to calling the pharmacist, I am going to get my questions ready. After seeing the packaging, I could tell that the prescription should be stored in a dark area and it looked like it might need to be refrigerated. I would have assumed it would have come in a refrigerated type package, but it doesn't hurt to ask. I'll confirm both of these issues. Next, the Scopolamine patch that Dear Son was using was a 1.5 mg patch meaning it delivered 1.5 mg of Scopolamine over three days or .5 mg per day. So if the goal is to use the drug at night, I assume that the dose would be .25 mg or half of the daily dose of the patch. Although the directions that came with the gel state to use .1 ml (.25mg) to .4 ml as directed every 4-6 hours, I will have him explain this to me. Next, I need information on how it should be applied. Over the phone, he mentioned to Dad that it should be applied on the wrists and rubbed in. Next, I wanted to know if the gel had a half life and finally, I want to know if there are any contraindications of the gel meaning what are the signs that I should stop using the medication asap. You should always know that for each medication as well as know the major side effect of the drug.

So to summarize, prior to my call, these are my questions:

• Does the gel need to be refrigerated?
• Does it need to be away from light?
• The Scopolamine patch was 1.5 mg patch for 3 days or .5 mg/day or .25 mg for 1/2 day or 12 hours; with the Scopolamine gel, is my goal to use the same amount as before for x number of hours?
• How do I apply it?
• Does the gel have a half life?
• Are there any side effects or contraindications of using the gel?

In this case, I am looking to speak with the pharmacist himself and not a pharmacy tech, to get the answers. I call the pharmacist and we discuss the following:

• The gel can be kept at room temperature and does not need to be refrigerated. Refrigeration could break it down. (This is good information and that's why you want to ask these questions. Also, if it were summer time and it was warmer inside, you need to know if it should be refrigerated.)
• It needs to be stored in a dark place at room temperature.
• Regarding dosing or how much to use, he explains that the Scopolamine patch works differently. It delivers .33 mg/day but the penetration rate is higher than the gel and it also has a slower release into the bloodstream. The advantage of the Scopolamine gel is that it is an immediate release into the bloodstream.
• Dosing-He goes on to say that it will be trial and error with the gel to get the dosing right. Each person absorbs the gel differently and there isn't any way to tell how fast or slow that a person will metabolize the gel. (We know from past experience that Dear Son is a hyperutilizer of medications.) The pharmacist has us start with .1ml (.25mg) at bedtime and see how he does. It should last 4-6 hours. The recommended dose is 2-4 times per day (24 hour period) however that is if we wanted the same coverage as the patch; in our case, we are looking for partial coverage or coverage at night and then no coverage during the day. He explains that the advantage of the gel is the ability to adjust the dose and it's a different release mechanism; the gel can be more cumbersome to administer in higher doses however if we get to the point where he is using a higher dose or the same amount that the patch delivered, then we need to go back and talk to the physician.
• In terms of application, he states we can apply it to the wrists, the tops of the feet or at the base of the neck, anywhere where there are blood vessels where it can be absorbed. (In Dear Son's case, we know that he has edema in his feet so his feet would not be the ideal place to apply this gel.)
• Does the gel have a half life? Yes, the gel's half life is the same as the patch, 3-6 hours.
• Are there any side effects or any situation where I should stop using the gel? The gel is hyperallergenic; the only contraindications would be urination (or lack thereof) and if his breathing gets worse.

After our call, I decided to try out the gel on Dear Son. First, there is the practical application of this gel. The goal is to use it at night so I am planning on giving it to him at midnight with his other medications so it will last hopefully until 6 a.m. Now then, here is the syringe and I am supposed to give him .1ml, or a tenth of this syringe. I can barely read the syringe in broad daylight and with reading glasses on, lol, let alone do this at night in the dark. Next, I'll put it on his wrists and rub them together. Sounds easy until you see Dear Son. His arms are tense and it's not the easiest thing to do.

This is the picture of the syringe. It is a white gel with white letters. I will be giving him one tenth of this syringe for a single application. I think black letters might have been easier to read considering the gel is white.

Around ten p.m. tonight, I changed his diaper and he was choking on his saliva. I decided to give him a second dose of the gel since the goal is to keep his airways clear. This time, I use rubber gloves and put the exact dose on the glove under the kitchen lamp, so I can see. This way, I won't need reading glasses. When I do that, .2 ml squirt out. Yikes! I use the syringe to put .1 ml back in so hopefully, that occurred. With the rubber glove on, I rub it into his wrist and then try to rub his wrists together. I may end up rubbing this in on both wrists myself instead of trying to rub his wrists together when he's sleeping. (The rubber glove is extremely important! You do not want to get Scopolamine in your eyes or on you since it can have the same effect. If you get it in your eye, it will cause your pupils to dilate and blurred vision. I know this because it happened to me once. I must have got it on my fingernail when I removed his patch. Although I am a diligent hand washer, stuff happens. My pupil was dilated and it took three days for my vision to return in that eye. )

So hopefully, tonight will go well and he will sleep. The last three months have been really challenging for him and for me. I think it's probably been the most difficult for me namely due to all of the crying and lack of sleep. I sincerely hope that this gets resolved. My nerves are about shot trying to make him more comfortable.

The great news is that it will resolve the urinary retention issue with the patch. Regarding new prescriptions, you certainly don't have to ask all of these questions but I like to understand how drugs work. As an advocate for Dear Son, it's helpful to learn these things. Now certainly, I've grown into this role over time. I can't imagine that when Dear Son was a baby and he got medications that I would be asking how they worked since I am sure I'd be overwhelmed with everything and the process. But now that we are in the end stages of his disease progression, and now that he is really medically complex, it helps to understand how things work since there are so many issues going on.