Symptomatic Treatments
Excessive saliva and drooling occur in ALS due to weakness in the muscles in the face and the mouth. Excessive secretions may cause difficulty in speaking, and may lead to aspiration. Anticholinergic medications, such as glycopyrrolate (Robinul) or scopolamine, and medications with anticholinergic side effects, such as tricyclic antidepressants (Elavil- amitriptyline), are often used to reduce the amount of saliva and help to control drooling. The most common side effects of these medications, however, are drowsiness and constipation. As a result of the latter, it is often recommended to increase fluid intake to help maintain the motility of the intestinal tract and to also help maintain some moisture in the mouth. Furthermore, if the mouth becomes too dry, saliva substitutes, such as Salivart, may be used to help maintain moisture in the mouth. If medications are ineffective, botulinum toxin or radiation may be helpful but may not be covered by insurance.
Thick mucus is treated with fluids, guaifenesin (Robitussin or Mucinex), or Mucomyst by nebulizer. Milk products aggravate thickening of mucus. A suction device or cough assist may also help.
Emotional liability, the loss of emotional control (pseudobulbar affect) , is one of the problems associated with the bulbar symptoms of ALS. Although it is not actually a symptom of depression, it improves with the antidepressant Elavil (amitriptyline) that also helps with all of the following symptoms: increased saliva, depression, and poor sleep. If Elavil does not work, we recommend Nuedexta (dextromethorphan 20mg and quinidine 10mg). Starting dose: One capsule daily by mouth for 7 days. Maintenance dose: After 7 days, 1 capsule every 12 hours.
The most common adverse reactions (incidence of ≥ 3% and two-fold greater than placebo) in patients taking Nuedexta are diarrhea, dizziness, cough, vomiting, asthenia, peripheral edema, and flatulence.
Muscle/joint stiffness (spasticity) is treated with baclofen and possibly physical therapy. Medications may be less effective than in other conditions such as multiple sclerosis.
BACLOFEN (LIORESAL)
- Class: skeletal muscle relaxant, centrally acting
- Dose adjustments: kidney impairment, geriatrics
- Adverse effects: drowsiness, slurred speech, hypotension, constipation, urinary frequency.
Valium is another option, as is Zanaflex.
Constipation can mean different things to different people. It can mean the difficult or infrequent passage of stools or can refer to the hardness of stools. Some people feel "constipated" if they do not pass a stool daily, while others may go two to three days before feeling constipated.
It is first important to note that it is not necessary for every person to move his or her bowels every day. A regular bowel routine is individual from person to person. Some people may move their bowels more than once a day, while others may move their bowels every other day. Both instances are perfectly normal. To determine what your normal bowel routine is, think back to a time before you noticed a problem, and make that your goal to return to a similar schedule. A person should move their bowels at least once every three days.
Constipation can be caused by many factors. Amyotrophic Lateral Sclerosis increases the likelihood of developing constipation in many people because of decreased activity. Diet, medications, delay of transit time through the colon, incomplete bowel emptying, lack of fluids, and lack of exercise are a few other causes of constipation.
How do we prevent constipation? First, we should look at our personal habits, and see where we can improve them. Some tips to follow to ensure regular bowel habits are:
- Increase your fluid intake! Eight glasses of water or juices per day are essential. Adding prune juice daily may also help. (Prune juice maybe more appetizing if you drink it warm.)
- Increase fiber in your diet. Vegetable fiber is largely indigestible and non-absorbable and increases the bulk in our stools. Bran and other sources of fiber may be found in cereals. Read the labels to find the highest fiber sources.
- Attempt a bowel movement at a regularly scheduled time each day and be sure that you are not rushed in any way. Establishing a routine often helps to "retrain" our bowels.
- Add some form of exercise to your daily routine. Inactivity may add to your problem.
- Bulking agents such as bran, psyllium, and methylcellulose may be added to your routine. Although they are considered laxatives, they act slowly and gently and are the safest agents for promoting elimination. Be sure to increase your fluids along with these agents.
- Daily stool softeners such as Colace (sodium docusate) may be added.
If constipation still occurs you may need to add a laxative to correct the situation. There are many over-the-counter laxatives available today, such as Senekot or Dulcolax pills or Dulcolax suppositories. Miralax is often helpful too. Ask your family doctor or pharmacist to help you select the appropriate medication for you.
Cramps may occur in patients with ALS. For Charley horse-type cramping symptoms, it is important that you are well hydrated and do not have any electrolyte imbalances. If the cramping is a significant problem, we may try gabapentin (Neurontin). If the cramping is due to spasms or spasticity, then we would utilize baclofen. Prior to starting these therapies, stretching is important. Sometimes quinine in tonic water also helps with cramps.
Pain is fairly common in the later stages of ALS for a number of reasons. Joints may become stiff and patients may have musculoskeletal back pain due to decreased mobility. We recommend starting pain treatment with simple measures such as acetaminophen, ibuprofen or Naproxen (Aleve) along with moist heat and massage. For moderately severe pain, we may recommend Tramadol and severe pain may require narcotic usage sometimes in conjunction with a pain physician or palliative care doctor.
Bladder problems are relatively uncommon in ALS, but sometimes they occur. If you are having problems with urination such as incontinence or difficulty voiding, we do recommend an evaluation by a urologist first to rule out other causes and to help guide treatment which would generally be “symptomatic”.
Swelling/Edema – This problem is common in weak limbs due to muscle weakness and decreased lymphatic drainage. It is not usually necessary to treat except by intermittent limb elevation. TED hose can also be used. We generally do not recommend water pills. If there is pain, redness or fever, call your PCP to be assessed for cellulitis or a blood clot.
