Essential Fatty Acids, Lorenzo's Oil and Beyond

©Copyright 1997 by Patricia Kane, Ph.D., Millville, New Jersey, U.S.A.
(Explore Issue: Volume 7, Number 6)

With the birth of their beautiful baby boy, Stephen and Rebecca Vaughn were overjoyed and meticulously recorded through pictures, video and diaries every nuance of his developement. After an anaphylactic reaction to a routine MMR (measles-mumps-ruebella) vaccination Alex emotionally began to slip away into a world of his own. Due to Alex's close bond with his parents they noticed the change in his behavior and desperately tried to lovingly engage him in play and interaction with others, but one month passed his second birthday Alex was diagnosed with autism. Medical science has failed to determine the cause of autism, but we do know through specialized testing that the impact is systemic-affecting every system of the body.

Alex Vaughn

For Alex and two hundred other children whose blood chemistries and red cell membrane fatty acids we have thoroughly researched, there is gross aberration in the fatty acid metabolism and the electrolyte status of children and adults not only with autism but with seizure activity, developmental delay, traumatic brain injury and cerebral palsy. Alex's parents were determined to save their precious little boy. They began metabolic intervention within two months of his diagnosis.

The first course of action for Alex's condition was to obtain a complete blood chemistry (Chem-28 including a CO₂ and CBC with diff) and enter the results into the BodyBio data management system. The unique patterns of disturbances within Alex's biochemistry were boldly demonstrated and a course of treatment through metabolic intervention began as the suggestions within the system are all based upon peer reviewed medical articles. The strength of their application comes from the specificity to the undividual patient and the depth of the research substantuating the findings.

Alex had a serious disturbance in his electrolyte status (deep depression of sodium and carbon dioxide), profoundly altered nitrogen status involving every nitrogen marker, sharply altered hematology and skewed differential. Carbon Based analysis is cross referenced with an enormous data base that matches nutrients to alterations in blood chemistry in regard to specific nutrient need. There are, for example, ten different forms of magnesium in the BodyBio system as chloride, malate, sulfate, taurate, carbonate, salicilate, aspartate, lactate, glycinate, and citrate. These bonding angles on magnesium are just as important as the base metal magnesium and they are defined specifically in Alex's chemistry.

Alex's chemistry revealed that he needed magnesium carbonate/magnesium malate and along with an entire roster of nutrients (ammonium molybdate, inositol, ascorbate, Vitamin K, pyridoxine, niacinamide, biotin, lactoferrin, plant enzymes, zinc citrate, oral electrolytes, N-acetyl glucosamine, ferritin, creatine, pantethine, riboflavin-5-phosphate, folinic acid, free form amino acids, adenosylcobalamin, inosine, carnitine, multivitamin formula, salivarius, plantarum, ionic liquid minerals, aqueous liver concentrate, Bromelain, Viobin Prometol, Omega Nutrition coconut butter and sesame oil, sesamin, and liquid colostrum). With a complete revision of his diet (high nutrient density, removal of gluten and casein, carb-protein-fatty acid balanced meals and snacks) the Vaughn family began to fight back against the autism.

Within three months of nutritional support Alex went from a complete absence of speech to four dozen words! Alex's father, Stephen, would call weekly with updates on his son's progress almost shouting his excitement in the tremendous gains Alex made in social interaction, eye contact, imaginative play, general health, physical movement, speech and began to ask for and consume concentrated protein foods.

After eight months of nutritional intervention Alex returned for re-evaluation to the physician who had made his original diagnosis. The doctor was awestruck as he examined Alex and observed him laughing and playing and talking -- he stated that he had not seen a child with autism change so drastically in 17 years of practice. Alex was chosen by the Miracle Children's Network to appear on television as the poster boy in June of 1996. While posing for pictures for the event Alex firmly held up his arms and said, "I am Awesome Alex -- The Adventurer!!!"

As so many families agonize over treatment for intractable seizure disorders, developmental delay, disturbances in metabolism, autism, traumatic brain injury and neurodegenerative conditions they often find twisted, frustrating pathways that lead to more questions than answers for their critically ill loved ones. Often the label genetic defect or inborn error in metabolism etiology unknown is solidly placed upon children and adults who fail to conform to a rigid set of parameters that define a particular disorder or disease. The true query is: What shall we do now to address metabolic disturbances in the myriad of "hopeless" conditions that exist today? These are real children and adults that are being further damaged every day. They are frequently told to wait for the drug or gene therapy that will be the answer to their health condition. It will be a very long wait, too long for most unfortunately. Often the media focuses on a family who is fighting for their child such as Michaela and Augusto Odone who tried to find a treatment for their child with adrenoleukodystrophy (ALD) with the use of Lorenzo's Oil.

Lorenzo Odone

The story of Lorenzo, who is now 17 years old, is sadly not a successful one. Medical trials have proven that the elevation of very long chain saturated fatty acids (C24:O lignoceric acid and C26:O hexacosanoic) are normalized with the administration of Lorenzo's Oil (C18:1 oleic acid and C22:1 erucic acid) but the degeneration of the condition persists and is relentless. Use of the oil causes gross deficiency of omega 3 and omega 6 fatty acids and lowers the platelet count. Amazingly, researchers have finally (we pointed out the association of omega 3 and omega 6 fatty acids years ago but it was ignored) now discovered that children with adrenoleukodystrophy are deficient in docasahexaenoic acid (DHA) and are conducting trials on the administration of this fatty acid derived from algae (Martek Biosciences Corporation). DHA is available in marine oils but due to the fishy taste researchers shunned using it due to difficulties with patient compliance. It is crucial that medical testing of red cell membrane fatty acids be performed so that application of therapy is specific to the individual's requirements. Children with adrenoleukodystrophy have low docasahexaenoic acid, low nervonic acid and high lignoceric acid while children with autism, seizures and brain injury also may exhibit high lignoceric acid but have high docasahexaenoic acid and high nervonic acid. Arbitrary administration of specialized fatty acids is inappropriate due to the unique patterns of fatty acid derangement in specific medical conditions.

Charlie Abrahams

The movie producer Jim Abrahams spoke out publically when his son Charlie responded positively to the ketogenic diet (low carbohydrate, high fat) at Johns Hopkins. Charlie had suffered from an especially difficult form of epilepsy, Lennox-Gastaut syndrome, characterized by numerous types of seizures and often unresponsive to anticonvulsant medication. After $100,000 worth of tests, drugs and surgery the convulsions continued to rack Charlie's body 100 times a day. In desperation Jim went to the medical library in search of an answer and found the book Seizures and Epilepsy in Childhood: A guide for Parents by Dr. John M. Freeman. Charlie was one of the lucky 30% of children whose seizures stopped on the ketogenic diet and he remained seizure free until the diet was stopped. After two years when the diet was discarded, the seizures returned with a vengeance and are now intractable. Dr. Freeman states that he does not know the reason that the ketogenic diet works -- or does not work. This is not surprizing since many clinicians do not have time to view the medical literature and blend their findings with that of other researchers.

The biochemical basis for the success of the ketogenic diet is simple. The cessation of eating stimulates the beta oxidation of very long chain fatty acids that not only occurs in adrenoleukodystrophy but in epilepsy, autism and developmental delay. The ingestion of carbohydrate also stimulates the release of insulin which depresses delta 6 desaturase and stimulates inflammatory prostaglandin two series and ultimately causes derangement of the fatty acid metabolism. Excess carbohydrate ingestion, which is so common in our present American diet, diverts and compromises the synthesis of prostaglandin series one. The modulation of fatty acid metabolism, however, is quite complicated and the lack of knowledge in regard to the coenzymes, the individual child's metabolism, the balance of electrolytes and the recent addition of hydrogenated fats to the ketogenic diet makes the outcome of this treatment shaky at best.

The Odone's and Abrahams' as well as many, many parents have been through excruciatingly painful ordeals with their children and have searched the ends of the earth to come up with treatments that would relieve their child's suffering. Often there is a promise of hope if the child stabilizes with a manipulation of the fatty acid metabolism but lack of knowlege of fatty acid metablism has grossly hindered our progress in treatment of neurodegenerative conditions.

In our struggle to direct the biochemistry of the human system we have fallen into an abyss in regard to our understanding of lipids and therapeutic manipulation. Virtually every aspect of metabolism is completely dependent upon the fats we consume yet current opinion leads one to believe that the etiology of the catastrophic illnesses of our time all have an intimate connection to the consumption of butter, eggs and meat. The entire spectrum of autoimmune disorders, endocrine imbalances, gastrointestinal disturbances cardiovascular difficulties and central nervous system involvement are all rivoted in derangement of fatty acid metabolism. The conflict is so deep that cardiologists and oncologists have completely opposing statements in regard to lipid consumption, one stating that fats be avoided completely and the other the exclusive use of polyunsaturated fats. American and European homes abound with polyunsaturates, unfortunately they are in the form of hydrogenated or trans fatty acids that are lifeless lipids that cannot support nutriture with anything more than calories. The use of trans fats literally shuts down the fatty acid metabolism. The synthesis of prostaglandins-local hormones that control all cell to cell interactions within the body-are completely dependent upon the ingestion of high quality, unaltered fatty acids. The body requires specific fatty acids to create gastrointestinal integrity, bilipid membranes, hormones, neurohormones, prostaglandins and immune modulators all derived from fatty acids and these fats must be supplied and trans fats avoided if modulation of the faulty metabolism is to be achieved.

In our struggle to direct the immune, endocrine, cardiovascular and nervous systems we have neglected to use the very substrate that is basic to all biochemical interactions -- fatty acids. Instead we have shunned lipids, removed them from our diets, blamed them for the ills of society-obesity, cancer, heart disease, degenerative illness-when in fact they are the critical substrates to the immune (cytokines, prostaglandins), endocrine (hormones, neurohormones) neuronal (myelin) components we attempt to manipulate. The administration of hormones, for example, often fails when an individual exibits very low serum cholesterol which is the base substrate for the cascade of regulatory hormones. Thus when an individual is loaded with a hormone they are often unable to properly utilize the hormone due to a deficiency of substrate (fatty acids, coenzymes). Recently the addition of DHEA has been suggested but this often further complicates the patient's chemistry rather than corrects the hormonal imbalance because the basic raw materials are missing and the ingestion of trans fats literally blocks fatty acid metabolism and hormonal production. The very availability of fatty acids (found in unrefined, cold processed oils, coconut butter, raw nuts and seeds) is often a challenge in the United States and now across Europe as our oils are hydrogenated to prolong shelflife.

Traditional medicine and complimentary medicine are now on a collision course as research has revealed the catastophic ignorance in regard to human fatty acid requirements and the relationship to disease. Fatty acids are receiving a tremendous amount of attention in the medical literature due to positive outcomes in studies of cardiovascular disease, rheumatoid arthritis, inflammatory bowel disease, eczema, immune disorders, inborn errors in metabolism, autism, attention deficit disorder, premature infants, dementia, alcoholism, dyslexia, diabetes, pregnancy and lactation. It was encouraging to hear as I was present in November 1996 at the International Conference on Highly Unsaturated Fatty Acids in Nutrition and Disease Prevention in Barcelona, Spain that medical science is finally taking some bold steps towards addressing fatty acid disturbances in relation to disease. We still have a long road to travel.

The work of individual medical researchers are strong avenues for indepth exploration into fatty acid metabolism but often their work is of one tiny aspect and the symbiotics of the entire picture is lost along with some very basic courses of action. Resolving lipid imbalances requires, of course, attenuation to the patient's dietary intake but of further critical concern are alterations in the blood chemistry and characteristic changes of red cell membrane fatty acids in regard to specific health disorders.

Physicians now have available a new data management system through BodyBio Corporation that transforms lab data (blood chemistry, red cell fatty acids, organic acids, amino acids) into a clear, concise format that takes the individual blood chemistry and matches the disturbances in their metabolism to appropriate nutritional intervention, disease patterns, and drug interactions all supported within the medical literature.

In my search for answers to "hopeless" medical conditions I found myself practically living in the bowels of medical libraries and at the bedside of critically ill infants and children in intensive care units in an effort to glean the associations within available laboratory tests, its application to nutritional biochemistry -- and data that could be entered into a system leading to answers that could be applied and be effective immediately. BodyBio (45 Reese Road, Millville, NJ 08332, 609-825-8338) now offers clinicians appropriate biochemical intervention by organizing and disseminating the medical literature into a format that has direct impact the upon the lives of individuals and creating a better prognosis in the long term outcome of those who were once imprisoned in an unyielding haze of degenerative conditions.

For Alex Vaughn the future is bright as the autistic features have slipped away with individualized metabolic support of his immune, endocrine and nervous systems. His parents meticulously ensure that he continues to receive appropriate nutriture for the disturbances as revealed in his blood chemistry and fatty acid metabolism. Underneath the pain and fear a ray of powerful light has emerged for Alex's parents welcome their little boy back into the world.

 

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