Dear Project Shui,
My child with Juvenile Tay-Sachs continues to struggle with overloaded secretions. We give 2 teaspoons of Robinol twice a day and she gets Albuterol once a day or as needed and uses the Vest Airway Clearance System. Our pulmonologist is suggesting Pulmicort, but I was told by another mother that Pulmicort made their child's eyes blood shot red. Also he is suggesting Botox for the salivary glands. What are your thoughts on this? Do you suggest Scopolamine (patch) over the Botox? I'm getting mixed reviews from families. One Tay-Sachs family believes we should hold off on unnecessary anesthesia and the Botox in the salivary glands has a lot of potential risks. I'm a bit overwhelmed and would appreciate your thoughts, feedback and help.
Sincerely,
Concerned Tay-Sachs Parent
*****
Dear Concerned Tay-Sachs Parent,
We have been extremely aggressive with pulmonary care since Rachaeli had aspiration pneumonia before she was even diagnosed with Tay-Sachs. I have always thought that one reason she is still here with us is because she was so sick at such an early age that we were forced to start aggressive respiratory prophylaxis from the beginning. What we do every day is what most people do only when their child is in a severe/acute respiratory episode. Our first pulmonologist always spoke to us about preparing for death and considering DNR orders very early on, but he was also very willing to do as much as possible to protect her lungs, which would most likely be the organ that ultimately failed and caused death.
So, to answer your questions and respond to the medications you are using:
1. We have never used
Robinol though many/most families have used it and found it very helpful.
--Because Robinol is taken by mouth/g-tube, it acts systemically, not locally, and so the salivary glands and lungs are not the only things that dry out.
--The organs we didn't want to get dry - the Eyes, the GI Tract (constipation was bad enough already), and the Urinary Tract (to avoid urinary retention and the need to catheterize) - are all effected by an oral drying agent (known as Anti-Cholinergic medications or medications used for their Anti-Cholinergic side effects).
--Also, too much of these Anticholinergic agents can affect the brain and cause confusion, or with Tay-Sachs, possibly more seizure activity. In normal people, too much of this anticholinergic action can cause delirium (Eric and I are shrinks and we've seen this too much).
2.
Albuterol and Xopenex are essentially the same medication. We used Xopenex from early on and so we stuck with it (it's more expensive, so if Albuterol works, I'd stay with that).
--These are rescue medications in that they work rapidly to open the airway and improve breathing. They also help loosen up secretions that may be settling in the lungs.
--For Rachaeli, we use these meds for maintenance and prophylaxis, not just for emergencies. We use more of them when Rachaeli has a respiratory crisis.
--We use Xopenex - half a vial mixed with saline - every 3 hours by nebulizer; if Rachaeli is super-stable, I will give a double dose, or a full vial, for a 6-hour duration.
--Early on, we used this medication at least every 8 hours. We switched to every 6 hours to fit the schedule of her other medications and treatments better.
--After her hospitalization at 2 years of age, when she aspirated blood that she vomited from a GI Bleed, we switched to Xopenex every 3 hours around the clock. We believe that this has played a big role in keeping her lungs clear and keeping her out of the hospital these past 6 years.
--I don't know anyone else who uses Xopenex or Albuterol this way or this often, but this is what we've done and I do believe it works.
**NOTE: Nebulizer treatments every 3 hours is what is used for acutely ill kids with pneumonia, asthma, etc. It is a lot of medication and it keeps the caretaker even busier since treatments usually mean lots of suctioning after the treatment.
However, I feel that this regimen really keeps the lungs clean and clear. It won't prevent aspiration, but I think it could help prevent a bacterial pneumonia from setting in.
3. We have used
Pulmicort since Rachaeli's first aspiration pneumonia. To me, this is a no-brainer.
--I don't know about blood shot eyes as a side effect, but I do believe it may be worth treating the blood shot eyes in order to use the Pulmicort.
--Pulmicort is used every day, usually twice a day, to Protect the lungs. It is an inhaled steroid.
--It is not going to help for an acute episode (It's NOT a "rescue" med), but I do believe it has been well-proven that inhaled steroids help prevent asthmatic/respiratory events.
--The only thing to be sure you do is to brush/clean the teeth, gums, tongue, etc., after each treatment because it can cause oral thrush (candidiasis) if it sits around on the mucous membranes of the mouth and throat.
**NOTE: Pulmicort will NOT decrease secretions. In fact, it will likely increase them. However, the reason for using it is not to treat or worsen excess secretions, but to Protect the lungs, to prophylax against reactive airway symptoms.
4. We have used
Scopolamine Patches since Rachaeli was about 2 1/2 or 3 years old. We started off using 1/4 of the patch (you can just cut them and save the rest for later) every other day. Now we are up to a full patch which we change every other day. These have been the best things we have ever used.
--It is essentially an Anti-Cholinergic medication (like Robinul) in the form of a transdermal patch.
--This mode of delivery is meant to act locally, and not on the whole body. It is placed in the neck region, like right behind or below the ear lobe. We switch sides (right ear then left) with each patch change.
--My nurses and I all know if it's a "patch day" or an intermediate day. When we put on the new patch, secretions dry up within 6 hours and stay dry until 6 - 12 hours before the next patch change.
--Some kids get an eczema like rash on the site. Rachaeli already has eczema. We'd rather have the eczema than the secretions.
5 -I looked into the option Botox injections into the salivary glands a few years ago. Another family had it done on their child They spoke about it in the medical session at the conference.
--They say that it worked great and they highly recommend it. Their doctor, however, seemed to be a specialist at this. I think that only ENT doctors do this procedure and they are not all expert at it. However, for some reason I think it was the child's Neurologist(not an ENT) that did the procedure.
--My Pulmonologist brought it up as an option, but told me it was a one-time procedure, and they either got it right that one time or they didn't . He made it seem like one wouldn't go back for a second try.
--We didn't go that route b/c the Scopolomine was working and, like you mentioned, we didn't want the anesthesia risk.
--However, I'm pretty sure that when neurologists use botox in their office, they don't use general anesthesia. My guess is that they just numb the area locally.
--I think it is definitely something to consider if general anesthesia is not needed.
6 -Another method of drying secretions, and which I feel is completely Under-utilized, is inhaled Atrovent (Ipratropium Bromide), which is given by nebulizer.
--Rachaeli first used it in the hospital when she had a pneumonia years ago. I don't think it is used by many people at home.
--However, we had it at home for years. At first, it was just sitting around in case of emergency - it's NOT a rescue medication, but it is often added on in acute episodes to dry secretions.
--We had it ordered on an "as needed" basis, but we always forgot to use it. Eventually, we started giving it routinely (1/2 vial), once a day, mixed with one of Rachaeli's Xopenex treatments.
--Now we give it every 12 hours, or twice a day, (1/2 vial each time) to keep secretions down. If she's really wet, I'll give it early or just give an extra dose.
--Atrovent is an Inhaled Anticholinergic agent (like Robinul or the Scopolamine patch, but in a nebulized form). Because it's given by nebulizer, it acts locally in the lungs and should not affect other organs.
--Like other drying agents, it could over-dry, and that's not good either, because dry mouth could result in sores or dental carries.
--So, like I said, we use this on a regular schedule and also as needed. If she is really dry, I will not give the Atrovent at the scheduled time. On "patch days" -- when we change the Scopolamine patch -- I will sometimes skip one of the Atrovent doses that day.
7 -This next intervention should be Number 1 on the list of interventions for excess secretions. I usually forget to try this until I've tried everything else.
--It's Pedialyte. I now call it the Miracle Treatment for most of Rachaeli's ailments.
--I use it for the first line of treatment for Fever (before trying Motrin/Ibuprofen or Tylenol/Acetominophen), Diarrhea (what it's meant for), and Excessive Oral Secretions.
--I use Pedialyte before adding any extra Atrovent.
--Sometimes I overdo it when I try everything at once: change Scopolamine patch, give extra Atrovent dose, and give a Pedialyte flush.
8 -Also, don't forget to consider "normal" reasons for increased secretions, like a Cold or Allergies. We discovered that Rachaeli gets seasonal allergies and I give her Zyrtec. If she goes out during pollen season, her nose will run uncontrollably, no matter what the intervention. But I may give her some Pseudafedrine and/or Pedialyte and/or as needed Atrovent.
9 -One "abnormal" cause of increased secretions (or that pneumonia that is not getting better) is Constipation and/or Excess Gas causing Abdominal Distention.
--This connection is more anatomical than chemical -- big belly pushes up on lungs.
--I have found that a good clean out from below can cure many respiratory problems.
--Years ago we used Miralax laxative as needed and then tried fleets enemas when things got really bad.
--For years now, Rachaeli has taken Miralax on a regular basis. At first 1/2 cap full every other day -- now we're up to a cap full (17grams) each morning.
--Some people will be concerned about building up a sort of tolerance to the laxative, which happens, but given the short life expectancy these kids have, abdominal comfort and improved breathing won out over the risk of laxative dependence when it came to Rachaeli's treatment.
--Until this past May, I used digital rectal stimulation on Rachaeli to ensure that she emptied her bowels fully. It's kind of like using the enema tip without the enema. The tone on her anal sphincter used to be very tight and she would retain a lot of stool even when she had a large bowel movement -- or, it would be so tight, she couldn't have a bowel movement.
--With a gloved pinky, a little stimulation down there would often release a massive amount of stool, and her comfort level would improve tremendously.
10 -We also use the VEST Airway Clearance System twice a day for 20 minutes (set at 14 hz). This gives the child a really good round of CChest Physical Therapy, which loosens up lung secretions and helps you to suction them up.
-We use the Cough Assist Machine every 6 hours, after a Xopenex treatment and before her feedings. This machine pulls up the secretions from the lungs and back of the throat, which is really important because these kids lose their ability to cough early on.
- It goes without saying, but we also use a Suction Machine, which has become a standard piece of equipment for these kids very early on.
-We have an Oxygen Concentrator in case oxygen saturations start to drop and she needs to be on oxygen.
*****
So, if Rachaeli is unusually wet, I will check the following:
-Did I forget to change her Scopolamine Patch?
-Does she have an obvious cold or upper respiratory illness? If so, try a little Pedialyte and/or Pseudafed.
-When was her last Bowel Movement? If none lately, find a way for her to go - ie. digital rectal stimulation or enema.
-Does she have a fever? If so, try Pedialyte. If that doesn't help, add Motrin or Tylenol with more Pedialyte.
-Is it allergy season? If so, try Pedialyte and/or as needed Atrovent and/or as needed Pseudafed
-Is she wheezing as well? If so, bump up the VEST and Cough Assist usage and do more Chest PT and repositioning.
If that doesn't help give more frequent Xopenex treatments.
To go even further:
-Is there Fever with the Wheezing?
If Wheezing continues with every 2-3 hours of Xopenex/Albuterol treatments and there is a Fever, ask Doctor about starting Antibiotics. Even if there is no documented pneumonia, sometimes a course of Antibiotics is needed (I like a simple course of Amoxicillin but lately we've bumped it up to the more potent Zithromax) especially if oxygen saturation readings are dipping and oxygen is needed.
Sincerely,
Project Shuimommma
