HUD – PAP DEVICE Asklipios Model for the therapy of patients affected with Friedreich Ataxia

 

Friedrich Ataxia (FA) is a rare genetic neuromuscular disorder (autosomal recessive). The prevalence is 1 in 50,000 in the United States and risk factors include family history. A total of about 6000 people are living with this rare condition in the US and less than a hundred patients are diagnosed every year.

Being FA a rare disease, the full development of the device for this indication is not possible due to the small number of patients that can be benefited. Therefore the FDA created a process called HDE (Humanitarian Device Exemption) where the manufacturer can apply though the Humanitarian Device Designation process for marketing of the device for the therapy of the rare disease allowing patients to benefit on new technologies and therapies otherwise unavailable.

The symptoms of FA usually begin between the ages of 5 and 15 but can, on rare occasions; appear as early as 18 months or as late as 30 years of age. The first symptom to appear is usually difficulty in walking, or gait ataxia. The ataxia gradually worsens and slowly spreads to the arms and then the trunk. Foot deformities such as clubfoot, flexion (involuntary bending) of the toes, hammer toes, or foot inversion (turning inward) may be early signs. Over time, muscles begin to weaken and waste away, especially in the feet, lower legs, and hands, and deformities develop. Other symptoms include loss of tendon reflexes, especially in the knees and ankles. There is often a gradual loss of sensation in the extremities, which may spread to other parts of the body. Dysarthria (slowness and slurring of speech) develops, and the person is easily fatigued. Rapid, rhythmic, involuntary movements of the eyeball (nystagmus) is common. Most people with Friedreich's ataxia develop scoliosis (a curving of the spine to one side), which, if severe, may impair breathing.

Other symptoms that may occur include chest pain, shortness of breath, and heart palpitations. These symptoms are the result of various forms of heart disease that often accompany Friedreich's ataxia, such as cardiomyopathy (enlargement of the heart), myocardial fibrosis (formation of fiber-like material in the muscles of the heart), and cardiac failure. Heart rhythm abnormalities such as tachycardia (fast heart rate) and heart block (impaired conduction of cardiac impulses within the heart) are also common. About 20 percent of people with Friedreich's ataxia develop carbohydrate intolerance and 10 percent develop diabetes mellitus. Some people lose hearing or eyesight.

Current standards treatments

As with many degenerative diseases of the nervous system, there is currently no effective cure or treatment for Friedreich's ataxia. However, many of the symptoms and accompanying complications can be treated to help patients maintain optimal functioning as long as possible. Diabetes, if present, can be treated with diet and medications such as insulin, and some of the heart problems can be treated with medication as well. Orthopedic problems such as foot deformities and scoliosis can be treated with braces or surgery. Physical therapy may prolong use of the arms and legs. At present, the treatment of individuals with FRDA remains supportive. Treatment of associated cardiac disease may also help improve the quality and duration of life of individuals with FRDA

This device is intended to be used to help control symptoms of the disease as muscular weakness and unsteadiness as part of a complete physical and general therapy.

Scientific rationale for its use as proposed

 

This device is a special ion magnetic inductor which produces short, complex, fast rising, bipolar magnetic pulses which induce an alternating electrical filed of instant duration. These parameters seem to be more effective to produce a high intra and intercellular bio-energy and are the least heat inducer in human and animal tissue creating a ‘subdiathermic effect”.

 

Every molecule has several internal degrees of freedom characterized by its structural and atomic composition and energetic status. Bioenergy is the activation of those internal degrees of freedom of a molecule. PEMF promotes the activation of the internal degrees of freedom seemingly without altering the molecular excitational state and therefore without measurable heat generation. The main difference between bioenergy and heat energy is that bioenergy appears to promote biosynthesis and homeostasis of the cellular environment. On the other hand, the diathermic effect seems to promote decomposition and destruction of complex bio-molecules.

 

All the cells in the body have a weak natural electric current flowing through them. Those currents are caused by electrically charged particles called ions. The ion concentration, distribution and flux will affect the homeostasis of the cell and therefore of the entire body. The application of a magnetic field (PEMF) around the affected tissue should prompt the cell to respond with the generation of weak micro-electrical currents that would influence the concentration, distribution and flux of ions. Promoting a potential more efficient ion flux prompts the cells to exchange nutrients and “heal” more rapidly. On the other hand PEMF therapy was associated with bone repair, neurotransmission intensification and DNA synthesis. All the molecules and atoms in the body are in transitional ionic state where the ionic charge may flow as in recent studies is shown that DNA can conduct electrons along stacked bases withtn the double helix  acting as a conductor. It has been suggested that the weak electromagnetic fields initiante mRNA transcription by accelerating electrons moving through DNA. Furthermore Nobel Laureate Albert Szent-Gyorgy established that structured proteins behave like solid state semiconductors or rectifiers. Cell membranes can rectify an induced voltage and this rectifying properties exerted by membrane proteins can cause changes in the intra and extracellular ion concentration stimulating the activity of the Na+/K+ pump. The activation of such a bimolecular process may restore intra and extracellular homeostasis.

 

The application of PEMF to damaged cells has been shown to help accelerate the reestablishing of normal cell potentials therefore increasing the rate of healing an reduction of tissue swelling (Rosch and Markov, 2004).

 

Friedreich Ataxia is a progressive neurodegenerative disease of genetic origin that slowly affects neuromuscular function. That neurodegenerative effect was potentially attributed to the increase in the concentration of iron at sub-cellular levels. Iron that is not bound to hemoglobin behaves as a positive ion that might be affected by a magnetic field potentially exposing it to naturally occurring oxidizing agents in the body and “neutralizing” its toxic effects.

 

On September 6, 2005 PulseDynamics applied for the Humanitarian Device Designation in this indication. Please check in the page News and Future developments for more information.

We are expecting a response form the FDA to this application.