Tricks of the Trade

Important note: The information provided in this website is intended to educate the reader about certain medical conditions and certain possible treatments. It is not a substitute for examination, diagnosis, and medical care provided by a licensed and qualified health professional. If you believe you, your child, or someone you know suffers from the conditions described below, please see your health care provider. Do not attempt to treat yourself, your child, or anyone else without proper medical supervision.

This Page Includes:
GT Feeds
Buttons
PT and Respiratory Care
Respiratory Fact Sheet

GT Feeds
In order to have healthy growth and development your child must take in enough calories to meet his/her nutritional and fluid requirements. Your child has had a gastrostomy tube placed into the stomach through the abdominal wall. This will serve as an alternative way to feed him/her, allowing your child to obtain the prescribed nutritional goals.

You will need

• Feeding tube and decompression tube if a Bard button

• Formula, no colder than room temperature

• Measuring cup with pouring spout

• Small container or cup of lukewarm water

Preparations

• Find a comfortable place.

• Head should be elevated at least 30 degrees during feeds.

• Assemble equipment: Catheter tip syringe.

• Wash hands.

• Check for residual.

• Open cap on button.

• Insert the tip of a clamped decompression tube into the button (feeding-tube Mic-Key button). Make sure the entire tip is inserted.

• Place other end of tube in collection container to catch any fluids or gas. During this step the collection container should be lower than the stomach.

• Open the clamp and allow to drain.

• Measure the drained fluid. If the amount is more than 1/2 the amount of the previous feed, give back the drained amount through the feeding tube followed by an ounce (30cc) of water. Remove tube and close button.

• Wait 1 hour and repeat.

• If there still is an excessive amount of residual, clamp the tube and skip that feed.

• If this continues at next feed call your medical doctor.

• If residual is OK, remove decompression tube.

• Connect the tip of syringe to clamped feeding tube.

• Place the other end of tube into a collection cup.

• Pour 1 ounce water into barrel of syringe.

• Slowly open the clamp allowing the water to displace the air in the tubing.

• Close the clamp when the fluid reaches the tip of tubing.

• Insert the tip end of the feeding tube into the button (making sure the tip is completely inserted into the button).

• Pour the formula into the syringe barrel.

• Raise the syringe above the level of the stomach and open the clamp. You can control the speed of delivery by the height of the syringe. Lower is slower, higher is faster.

• Pour the rest of the formula into the syringe until the last formula reaches the tip of the syringe.

• Pour 1 ounce water into the barrel to flush the tubing and button.

• Close the tubing clamp before air reaches button.

• Close clamp.

• Disconnect the tube from the button. Place two fingers on either side of button while pulling out the tip of the tube.

• Cap the button if it is a Bard. This is not necessary with the Mic-Key.

• Clean tubing and syringe with warm soap and water. Flush thoroughly with clear water after washing, making sure no residual formula or soap is left.
  

  Cleaning the Bard Vent and Liquid Feeding Tubes

• Remove the elbow/vent from the end of the tube (these can be easily cleaned with a pipe cleaner) and insert a small ball of cotton (this can have soap or alcohol on it). Using two or sometimes three wooden shishkebab skewers (with their pointed ends cut off) push the cotton through the tube. Once the cotton is through, the skewers should slide out easily. If they do not, you can always work them through a little at a time. Finish the procedure by running hot soapy water through and reattaching the ends.
  

• If you feel the ends are too loose, remove them, cut 1/2" off the end of the tube, submerge the freshly cut end into boiling water for 30 seconds, remove and reinsert the end. Be careful not to burn yourself. When the plastic cools, it will tighten around the nib and give you a solid connection.
  

Special thanks to Nelson Asinowski and Florence Fried who developed this technique.

Buttons
About 80% of children with FD will need tube feeding before they are 5 years of age. The patient’s growth and development, as well as respiratory status, will be considered in making the decision as to whether he/she will benefit from enteral (tube) feeds. If it would be of benefit to the patient, a gastrostomy button will be surgically placed, through the abdominal wall, into the stomach. Nourishment and fluids will be provided via this button by a soft, flexible tube, shortly after surgery. Improvement in the health of the patient post operatively is dramatic; increase in muscle strength and weight gain is quickly measurable. General, physical well being occurs with the decrease in aspirations into the lungs and respiratory infections.

Patients are often encouraged to continue oral feeding and just use the button for supplemental fluids and calories. A gastrostomy is a completely reversible procedure. Once the button is removed, the tract from the stomach to skin surface can close within hours. Therefore it is important to replace the button if it accidentally becomes dislodged. The care of the button is simple--just wash with mild soap and water to keep the skin clean and pat dry. The patient may partake in all normal physical activity with the button in place, such as bathing, playing and swimming. Two of the types of buttons that are used most often at the FD center are the MIC-KEY and the Bard.

The Bard Button is a soft silicone tube that has a mushroom-like dome on the end which is placed in the stomach. This dome which is inserted through the gastrostomy stoma is radiopaque (can be seen on a x-ray) and will hold the Button in place. The part of the Button which is seen on the skin of the child has two small wings which keeps the Button from slipping through the skin into the stomach.

The Button opening has a cap which is left in place between feedings. Inside of the Button tube is a valve that keeps the feeding from coming out of the tube. This is called an anti-reflux valve. Because of this valve, you will be unable to aspirate from the Button.

Percussion Therapy (PT) and Respiratory Care

Respiratory Care

Children with Familial Dysautonomia are often affected by increased incidence of lung disease. Misdirected swallowing and gastric intestinal reflux may result in aspiration into the lungs (fluid entering the lungs). This causes lung tissue irritation leading to pneumonia and disease. Weak chest muscles increase secretions, and the appearance of being well even though infected, requires that we be diligent in the respiratory care of these children. Breathing exercises, chest PT, inhalation therapy and periodic chest x-rays may be required for your child to be able to clear secretions and keep lungs healthy. Your physician will prescribe the frequency of need.

Important Note: Some of the more effective techniques are illustrated below. These should not be started without the consent of a physician in case there is some specific contraindication for your child.

Chest PT and Postural Drainage

• Postural drainage uses the force of gravity to help the body move secretions.

• Chest percussion is the clapping of a rounded hand over different areas of the chest to help loosen secretions.

• Inhalation therapy is the deliverance of a vapor to deliver some type of therapy directly to the lungs. This may be for the purpose of opening the airways, thinning the secretions or delivering an antibiotic.

• Suctioning is the oral nasal use of a small pliable tube connected to a vacuum type device to remove secretion when a patient does not have the body mechanics to express the secretions themselves.

• Breathing Exercises help strengthen chest muscle walls and giving lungs more room to expand.

How to Make Therapy Fun

An additional benefit of Chest Percussion Therapy (CPT) is that it promotes a special time together for you and your child. It offers a regular and specific time to enjoy each other’s company. You may want to enhance this time with one of the following:

• Spend time playing, talking or singing with your child before during and after CPT.

• Schedule CPT around a favorite TV show.

• Play a favorite tape of songs or stories.

• Encourage blowing or coughing games during CPT, such as blowing pinwheels or coughing the deepest or funniest cough.

• Minimize interruptions.

• Ask willing and capable relatives, friends, brothers and sisters to perform CPT occasionally.

Respiratory Fact Sheet
The expression of respiratory disease in the FD patient is extremely variable.  The signs of infection can be obvious with cough, fever, and congestion.  It can also present with subtle signs like lethargy, loss of appetite, weight loss or even failure to thrive. Radiopathic examination should be performed if disease is suspected. Any individual patient may experience any one, several, or none of the problems and symptoms in the following discussion.

The Respiratory Fact Sheet Includes:
Why do children with FD have an increased incidence of respiratory problems?  
What is the treatment for lung disease?  

Why do children with FD have an increased incidence of respiratory problems?

Pulmonary or Lung Infections 
Pulmonary or lung infections in an FD patient are most commonly caused by repeated aspirations.  "Aspiration" is the intake of foreign material into the airways.  Some of the contributing factors for aspiration are:  

Difficulty in Swallowing: FD children often have difficulty forming a bolus of food in their mouths and moving it to a proper position for swallowing. They may misdirect fluid and food into air passages to the lungs or even up toward the nose.  This can cause ear infections, sinus infections or pneumonia. Liquid is more apt to be misdirected than food.  The thinner the drink the more difficult it will be for the patient to control and direct it to the correct pathways.  

Dysfunction at the Stomach and Esophogus Junction: If the junction is too relaxed, stomach contents (food and acid) can back up into the esophagus, mouth, and possibly the airways.  This is called gastro-esophogeal reflux (GER).  Symptoms of GER may include pain, “heartburn,” nausea, vomiting, choking, color changes and infection. GER can also have relatively silent symptoms.  It certainly should be suspected with recurrent infection of chest, sinuses and even ears.

Mechanical Restrictions
Scoliosis: There is a high incidence of curvature of the spine in children with FD. If it is going to occur, it is usually apparent by ten years of age.  Scoliosis can modify the shape of the rib cage and therefore prevent proper expansion of the lungs when breathing, which makes the patient more prone to atelectasis and pneumonia.  Poor coordination and muscle weakness may also reduce the FD patient’s ability to take deep breaths and cough effectively.  

Atelectasis: Atelectasis is a partial or total collapse of the lung. Obstruction and/or hypoventilation may cause these unexpanded areas of the lung.  A decreased respiratory effort and ineffective cough and poor secretion clearance predisposes the FD child to mucus plugs and unexpanded airways causing atelectasis.  

Autonomic Nervous System Dysfunction 
Children with FD do not respond normally to changes in the level of carbon dioxide and oxygen in their environment.  This can result in:  

• Breath-holding until the point of losing consciousness.  This usually occurs in children under 5 years of age.

• Loss of consciousness when flying or traveling to high altitude.

• Abnormal breathing patterns during sleep.

• Drowning – the child can be unaware of his/her need to come to the surface and breathe.

What is the treatment for lung disease?

Treatment for Pulmonary Lung Infection
Treatment is both preventative and supportive.  Not all of the following are indicated for every FD child with pulmonary involvement.  Treatment is individualized but many of the following techniques have been useful.  

• Feeding and swallowing therapy

• Antacids

• Gastrostomy button for feeds (prevents aspiration into the lungs of fluids and food)

• Esophageal fundoplication (surgery that tightens the base of the esophagus in order to prevent reflux)

• Antibiotics

• Aerosol treatments  to deliver various types of medications directly to the lungs  

• Corticosteroid (controls inflammation)

• Oxygen

• Yearly flu shots

• Synergis treatment for prevention of Respiratory Syncytial Virus (RSV) in children under 5 years of age

• Frequent evaluation monitoring for the subtle clinical signs of aspiration, unexplained fevers, chills, loss of appetite, fainting, weight loss, or fatigue or listlessness

Chest Physiotherapy

• Clapping/percussion

• Vibration

• Postural drainage

• Aerosol treatments

• Suctioning

• Incentive spirometers

• Positive pressure respirators

• ABI VESTS airway clearance system

Treatment for Mechanical Restriction
Prevention and early intervention are the focus of treatment. 

• Breathing exercises maximize lung expansion and strengthen accessory muscles.

• Physical therapy concentrating on upper body strength and the back.  

• Awareness of body position.

• Monitoring braces, making sure the fit is correct.

Treatment for Autonomic Respiratory Dysfunction
Autonomic Respiratory Dysfunction is treated by being aware of the FD patient’s insensitivity to change of environmental oxygen and carbon dioxide and then taking appropriate measures.

• During air travel supplemental oxygen should be available and g-tube should be vented during take off and landing.  

• Vacations at high altitudes should be avoided.  

• Swimming should be supervised and underwater activities such as breath holding contests, diving for items at the bottoms of the pool, acrobatics, etc., should be avoided.