2012

IMPORTANCE OF THE UNINTERRUPTED ADMINISTRATION OF TOCOTRIENOLS AND EGCG-CONTAINING GREEN TEA EXTRACT (GTE) TO FD PATIENTS UNDERGOING A SURGICAL PROCEDURE – WHAT WE HAVE DISCOVERED:
An article that represents a collaboration between our research group and a team working at The Children’s Hospital of Philadelphia (CHOP) has been published in the # 1-rated anesthesiology journal (aptly called “Anesthesiology”) in the world. This article describes the importance of the uninterrupted administration of tocotrienols and GTE to FD patients undergoing a surgical procedure. It also emphasizes the importance of assuring that no medications are administered to individuals with FD that are contraindicated for individuals with reduced monoamine oxidase levels. This is the first report that demonstrates: 1) the essential uninterrupted administration of these therapies to maintain autonomic stability during surgery with anesthesia in an FD patient and 2) the importance of giving medications that are not contraindicated for those with reduced monoamine oxidase levels. Much of what has been learned about FD, from therapies developed to the knowledge of low MAO levels in FD patients to medications safe for individuals with FD, culminated in the ability for this surgery to be successful.

WHAT PARENTS AND PHYSICIANS OF INDIVIDUALS WITH FD NEED TO LEARN FROM THIS COLLABORATIVE EFFORT:
The article that was written by Dr. Scott D. Cook-Sather, an anesthesiologist at CHOP who specializes in anesthesiology and critical care medicine, in collaboration with Dr. Rubin and others at CHOP, describes the ability of the combined administration of tocotrienols and GTE to restore lacrimation (tearing) to normal levels and for this combination to eliminate the occurrence of dysautonomic crises in a child with FD under his care. The article also describes the impact of the uninterrupted administration of the tocotrienols and GTE on the surgical outcome of this child while undergoing adenoidectomy. The five year old child was maintained on a tocotrienol and green tea regimen through the morning of the anesthesia and surgery. This child was taking 300 mg of the tocotrienols in the a.m. and 300 mg in the p.m. as well as two GTE capsules each day, one in the a.m. and one in the p.m. Throughout the surgical procedure, this child’s clotting function was unimpaired. Dr. Cook-Sather further reports that unlike the nausea, vomiting, crisis and hemodynamic instability this child experienced following surgical procedures that took place prior to being on the tocotrienol and GTE regimen, this child exhibited none of these symptoms during or following this surgical procedure. Based on the literature that describes autonomic, respiratory and cardiac instability in FD patients following a surgical procedure, a pediatric intensive care unit bed was reserved for this child for her recovery after the surgery. In light of the fact that this child’s vital signs following the surgery were stable (blood pressure: 88/40; heart rate: 75 beats per minute; pulse ox: 99%; temperature: 36.3°C), the intensive care unit bed was not needed. Two hours following the surgical procedure, the child was transported to a regular hospital room. A post-operative examination of the child revealed no nausea, vomiting or dysautonomic dysfunction.

The last paragraph of the “Discussion” section of this paper provides an excellent overview of the surgical event. It reads as follows:

“Our patient demonstrated normal hemodynamic variability associated with anesthesia and surgery for this procedure. She experienced none of the most common intraoperative problems reported in FD patients (hypoxemia, hypotension, hypertension, bradycardia, dysrhythmias, heart block, and/or cardiac arrest) and, later, none of the associated postoperative complications (intractable vomiting, excessive secretions, atelectasis, pneumonia and respiratory arrest requiring reintubation). We attribute our patient’s perioperative hemodynamic stability to the continuation of her tocotrienol and GTE regimen. Before this therapy and shortly after its introduction, she likely had insufficient drug and low IKAP levels, resulting in two difficult postoperative courses that included apnea, hypotension, and severe, refractory vomiting.”

The final section of the article entitled “Knowledge Gap” provides valuable and critical information for parents of children with FD and the physicians that treat these children. This information should be shared with physicians caring for individuals with FD.

ANOTHER SUCCESSFUL SURGERY SINCE THE ONE DESCRIBED ABOVE:
It is interesting to note that the publication of this article in Anesthesia coincides with the recent release of a child with FD from NYU who had corrective back surgery. The parents vigilantly follow the protocols we have developed. This child awoke from surgery stating that she was hungry and six days after the surgery, she was back at home. She did not retch even once during her recovery period at NYU!
Publication: Case Scenario: Perioperative Administration of Tocotrienols and Green Tea Extract in a Child with Familial Dysautonomia (PDF)