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Eat small, frequent meals, minimize simple sugars and no ETOH.

Increase fluids and salt to maintain blood volume. Caffeine may be helpful in the AM
Perform pedal-pushes, leg-crossing and thigh contraction exercises
4-5 times daily. Swimming is excellent because isotonic exercises
produce less hypotension than isometric.

Wear elastic thigh stockings and/or abdominal support when out of bed

Raise the head of the bed with 4-6” blocks at night to reduce the potential effects of supine hypertension. The upright position diminishes the effect of supine HTN by reducing press-natriuresis in the kidney and increases rennin secretion, thereby restoring vascular volume.5
These rather simple, but effective strategies enhance the safety of the patient. Patients need continuing support and encouragement to adapt these new interventions into their daily routine.

Commonly used medications

Midodrine (ProAmatine) is usually the initial drug of choice. It’s an oral selective a-1 agonist which is effective within an hour, lasting 2-4 hours. Since it has the tendency to raise the supine BP, it should be taken early in the morning, 1 hour before arising, when the BP is naturally lower. The patient should take Midrodrine with plenty of water and remain in bed for 30-60 minutes. Additional doses may be taken before lunch and in the early afternoon. Midrodrine should never be taken after 6PM. Midodrine increases sphincter tone therefore urinary retention should be carefully monitored. The majority of patients benefitted from 10 mg of the medication.4
 
Pyridostigmine (Mestinon), a cholinterase inhibitor, acts by improving the ganglionic transmission, effectively increasing the standing BP with minimal effect on supine BP. The usual dose is 30 mg 2-3 times daily. The main side effect is abdominal colic.

Fludrocortisone (Florinef) retains salt and water, thus expanding plasma volume which raises the BP. At high doses, hypokalemia, hypernatremia and supine HTN may increase. It takes about 4-5 days to reach full effect and may be used in conjunction with midodrine.4

 

Risk for respiratory infection R/T aspiration

Aspiration may occurs due to autonomic dysfunction of the muscles used for swallowing and speech. Based on the speech therapist’s evaluation, which usually includes a barium swallow, diet recommendations will be made. Small, frequent feedings are used to minimize fatigue. Nutritional supplements are often needed to maintain a positive nitrogen balance. It is important to have the patient:

Maintain chin tuck, dry swallow 2-3 times after each mouthful
Eat meals in a chair or when in bed, maintain high fowler’s position
Remain in a sitting position 1-3H after eating/drinking
Drink thickened liquids, limit milk
Crush or use liquid meds
Minimize environmental distractions

As MSA advances, a repeat barium swallow may be ordered. Findings frequently indicate the need for a percutaneous gastrostomy (PEG) tube to compensate for fatigue, decreased muscle strength and weight loss. Research indicated that despite a PEG tube, patients may continue to have episodes of aspiration that lead to pneumonia.7

Jack, like many patients wanted to eat small amounts of his favorite foods. Thanks to the health team arranging the needed supervision, his wish was granted.

Ineffective airway clearance R/T muscle weakness and excess secretions

As MSA progresses, the deterioration of ANS leads to decreased muscle strength of thoracic and diaphragmatic muscles causing retention of pulmonary secretions, stridor and obstruction. Stridor is associated with a poor prognosis.7 Most common causes of death in MSA are pulmonary related; namely, apnea and recurrent infections. Mouth care before meals and at bedtime lessens the incidence of pneumonia. Increasing fluids, encouraging activity, deep breathing and expectoration of sputum is imperative. If there is any question of a respiratory infection, the patient should seek immediate medical intervention. In the terminal stages of MSA, the patient is too weak to cough up secretions and suction is required. A tracheostomy may be an appropriate option in some situations.
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