Diaphragmatic Paralysis

I remember when I first heard that Liam's left diaphragm was paralyzed due to CDH and/or Repair. I was freaked out. I didn't know what to expect. The specialist would skate over the issue and just say that he would be ok but they'd keep an eye on him. When we found out that Liam also had a defect in his right diaphragm, therefor he was rediagnosed as bi-lateral CDH, they said they wouldn't touch it unless they absolutely had to because of his left diaphragm already being paralyzed. When you get the news sometimes the info goes in and right back out. So I researched it so that I could refresh my memory:

* Diaphragmatic paralysis, whether it occurs in one or both sides of the diaphragm, is uncommon.

*Whether the paralysis occurs in one (unilateral) or both (bilateral) sides of the diaphragm, all patients will experience some amount of reduction in lung capacity, particularly noticeable when lying down.
Liam's left diaphragm is paralyzed and due to that and pulmonary hypoplasia his left lung hardly functions. He requires daily breathing treatments to keep the lungs open. One treatment is a steroid.

There are many reasons Diaphragmatic Paralysis occurs but for CDHers its usually because:
*The phrenic nerve didn't fully develop or develop properly
*incurred surgical trauma

Patients with diaphragmatic paralysis may experience shortness of breath, headaches, blue lips and fingers, fatigue, insomnia and overall breathing difficulty.  Also:

• Unilateral diaphragmatic paralysis may go undiagnosed. Often patients compensate for the discomfort of reduced lung capacity by sleeping in a semi-upright position or reducing physical activity when there is shortness of breath.
• Bilateral diaphragmatic paralysis presents more severe symptoms, which leads patients to seek medical attention. The shortness of breath is more severe, even with mild exertion.

Newborns and children with unilateral diaphragmatic paralysis may experience more severe respiratory distress than an adult, due to weaker muscles and a more compliant chest wall. The newborn may have a weak cry or show signs of gastrointestinal distress, with frequent vomiting.  Children with bilateral diaphragmatic paralysis require immediate medical attention and ventilator intervention because the condition can be life threatening

The tools used to diagnose diaphragmatic paralysis include:

• Pulmonary function testing while lying down and again while upright.  Lung capacity is often reduced about 10 percent when a person is lying down; patients with bilateral diaphragmatic paralysis may experience a 70 to 80 percent reduction in lung capacity while patients with unilateral diaphragmatic paralysis may experience a 50 percent reduction.
• Chest X-rays or an upright, inspiratory chest radiograph.
• A blood test to measure the amount of oxygen in the blood.
• Measuring transdiaphragmatic pressure and thickness.
• Phrenic nerve stimulation testing.
• Electromyography, a test that evaluates and records electrical activity produced by skeletal muscles.
• Computed tomography (CT) scanning of the thorax and/or abdomen.
• Magnetic resonance imaging (MRI) to determine if there is an underlying condition involving the spinal column or nerve roots.
• Ultrasound to see the activity of the diaphragm and to identify any unusual movement or lack of movement.

Treating Diaphragmatic Paralysis

Physicians take into consideration the overall health of the patient, the severity of symptoms as well as any underlying cause for the paralysis:

• Diaphragmatic plication, a surgical procedure that pulls the diaphragm down, is commonly used in patients with unilateral paralysis.  The surgery allows the diaphragm to moves so as to expand better and improve ventilation.  Patients with more severe symptoms, such as a respiratory infection, asthma or COPD (chronic obstructive pulmonary disease), can benefit from diaphragmatic plication.
• If the patient has no symptoms, or the symptoms are mild, and the patient is in otherwise good health, no treatment may be necessary.
• Breathing pacemakers may be used in patients who have functioning phrenic nerves, such as patients with ALS or spinal cord injury. The devices may result in improved respiratory function and lower infection rates.
• Thoracoscopic diaphragm plication may be an option for some patients, resulting in a shorter hospital stays than other techniques.
• In severe cases of patients on a ventilator due to bilateral diaphragmatic paralysis, the diaphragm might be plicated to help get patients off the ventilator.
• A tracheostomy, the surgical formation of an opening in the trachea, helps allow the passage of air.  This approach is commonly used for patients with a life threatening disease or a diagnosis of high quadriplegia.

Liam had been on a ventilator at birth and slowly weined to CPAP, then a nasal canula and off. He's required oxygen on and off for the last year. A common cold or flu and his lungs need the additional support of oxygen. Since a plication on his already repaired left diaphragm, we know that doing another one will not help. His right diaphragm needs a plication but in Liams case it is too dangerous to do for several reasons:
*They cannot risk his right diaphragm from becomming paralyzed because it will cause more lung issues than he already has. With him having Chronic Lung Disease his chances are lower of being able to breathe without a vent.
*Liam's body creates alot of scar tissue and because of all his surgeries his abdomen is full of scar tissue. Scar tissue if harder to heal and work with. Because of all the scar tissue they cannot do a plication laprascopically. They would have to open his chest cavity and that is a risk no surgeon wants to take.

Prognosis for Diaphragmatic Paralysis

The prognosis for unilateral paralysis is quite good, providing there is no underlying pulmonary disease.  Sometimes, patients recover without any medical intervention.The prognosis for bilateral paralysis also depends on the overall health of the patient but surgery may be the best option for patients who continue to have a poor quality of life.

(Follow the link to read the full article from Cedars Sinai http://www.cedars-sinai.edu/Patients/Health-Conditions/Paralyzed-Diaphragm-Diaphragmatic-Paralysis.aspx )