Respiratory problems are, unarguably, the most serious of medical complications in ALS. Breathing difficulties occur from the gradual deterioration of breathing muscles, i.e. the diaphragm and the intercostals. The course is as shown below.

_{SIGNS OF RESPIRATORY INSUFFICIENCY}

1. General fatigue, drowsiness, lethargy.

2. Sleep disturbances such as nightmares, night terrors, sleep apnoea (interrupted breathing during sleep) or sudden awakening.

3. Morning headaches.

4. Daytime sleepiness.

5. Confusion, disorientation, anxiety.

6. Poor appetite, weight loss.

7. Excessive yawning or hiccups.

8. More laboured breathing, especially when lying down.

9. Rapid, shallow respirations with increased heart rate.

10. Weakened or softened voice; speaking in short phrases; inability to sing or shout.

11. Difficulty coughing and clearing the throat (weakened abdominal and throat muscles also contribute to this)

Monitoring for respiratory problems

1. Pulmonary function test (PFT)
2. Regular monitor of SPO₂(Normal SPO₂ should be more than 98%)
3. Spirometer reading

Managing Breathing Problems

1) Breathing Exercises

• It is very important to start breathing exercises regularly even before the symptoms start.

• This prevents fatigue and delays muscle weakness.

• Deep breathing exercises are recommended to improve the lung capacity. Yoga can be very beneficial for eg: (Anuloma Viloma) breathing exercises.

• Spirometer is a device that can be used for breathing exercise

2) Medications

• Bronchodilators: Dilate the airway to help breathing, but otherwise ineffective due to muscle weakness. This can be used by nebulization.

• Expectorants: To thin out the secretions and make them easier to cough out.

• Saliva management: Weakened breathing muscles and weaker muscles of the mouth can cause drooling. Medications can be prescribed by the physician to control drooling (glycopyrolate).

• Supportive medications: The physician may prescribe other drugs for symptomatic relief such as for pain, depression, sleep disturbances, constipation, etc.

3) Chest physiotherapy:

• Chest physiotherapy can be provided by a specialized trained therapist.

• They may teach breathing maneuvers to prevent atelectasis ( lung collapse) and aspiration.

• They can also teach breathing exercise to improve the lung capacity. Chest physiotherapy can help to clear secretion from the base of the lungs. It can help to cough out when the patient has weak cough.

4) Cough Assist Device:

• Automatic Mechanical cough assist devices such as the Philips Cough assist or Hillrom Vital cough helps those with an ineffective cough by breathing through a mouthpiece or masks

• The device gradually applies positive pressure to insure a deep breath, then shifts to negative pressure to assist with pulling secretions upward, simulating a deep natural cough.

• High frequency chest wall oscillation involves an inflatable vest that is attached to a machine.

• The machine mechanically vibrates at a high frequency. This helps to loosen and thin mucus and clear the airway.

5) Noninvasive Breathing Support

A BiPAP Machine, is often prescribed in ALS.

• BiPAP is short form of Bilevel Positive Airway Pressure and delivers air at two pressures, one for inspiration and one for expiration (inhalation and exhalation).

• A number of nasal or face masks and attachments are available, can be customized for the best fit.

C Pap Machine continuous pressure assisted. Positive ventilation.

Pressure-cycled vent machines, which deliver air at a set pressure level with a variable volume of air on a timed cycle.

Advanced respiratory care in ALS

• As ALS progresses, the person living with the disease may become increasingly dependent on ventilation and ultimately, will require invasive ventilation with tracheostomy. This will provide more efficient ventilation and better control of the upper airway and secretions

• Ventilation through tracheostomy

• A tracheostomy is a surgically created hole in the trachea (windpipe) through which air is forced. The tube through which the air is delivered also is called a tracheostomy (trach) tube.

Caring for the Tracheostomy

• Its normal for the patient to have small amount of mucus around the tube

• Hole in the neck should be pink and painless.

• It’s always beneficial to carry an extra tube in case of plugging of the tube.

• Hold a tissue or cloth to catch the mucus coming from the tube.

• Consult a doctor to know how to prevent the plugs in the tube as the patients’ nose will no longer keep the air moist.

• One of the strategies to keep the breathing air moist is to put a wet gauze or cloth outside the tube.

• A few drops of salt water (saline) will loosen a plug of thick mucus. Putting a few drops in the tube and windpipe, then taking a deep breath and coughing will help bring up the mucus.

• Protect the hole in the neck with a cloth or tracheostomy cover when outside. These covers can also help make the breathing sounds quieter.

• During showers, cover the hole with a tracheostomy cover. No swimming is allowed with a tracheostomy tube.

• To speak, the patient will need to cover the hole with a finger, a cap, or a speaking valve.

• Once the hole in the neck is not sore from the surgery, clean the hole with a cotton swab or a cotton ball at least once a day to prevent infection.

• The bandage (gauze dressing) between the tube and neck helps catch mucus. It also keeps the tube from rubbing on the neck. Change the bandage when it is dirty, at least once a day.

• Change the ribbons (trach ties) that keep the tube in place if they get dirty. Make sure the tube is held in place while changing the ribbon. Two fingers should be fit under the ribbon to make sure it is not too tight.

LTV^® 1150 ventilator

• Provide your adult and pediatric patients pressure control and pressure support without a high pressure oxygen source

• The LTV 1150 ventilator provides portable, advanced ventilation for adult and pediatric patients at home or a post-acute care facility

• At only 14 lb, the ventilator provides a wide range of ventilation therapies to meet demanding patients needs, including volume control, pressure control, pressure support and spontaneous breath types.

_{Advantages of invasive ventilation:}

1. It provides for much longer survival

2. It provides a secure connection directly to the airway for suctioning secretions.

3. It leaves the face free, without headgear, straps, and skin pressure problems on the pace.

_{Decision making regarding invasive care}

The decision to choose invasive mechanical ventilation is a very personal one. People with ALS who choose Invasive Ventilation can live for years. Some of them are cared for in nursing homes while others are able to remain at home.

So, in making the decision, one should consider his family support, level of independence and financial resources.