Intravenous Lipids; What Are They Good For?

Intravenous Lipids

Intravenous Lipids have been used in the clinical setting for over 3 decades. However, the use of these intravenous fluids have had a widespread element of controversy. Some healthcare providers question whether we should be using intravenous lipids due to the potential compromise of patient outcomes. Over the past 15 years new lipids that include olive oil and/or fish oil have been introduced with some remarkable findings seen when these new lipids replace the more traditional ones.   The most widely available intravenous lipids are prepared from soybean oil or a combination of soybean and safflower oil. All of the emulsions are emulsified with egg yolk phospholipid, with glycerol added to achieve an isotonic solution.  The issue of Lipid function and use in pediatric, adult surgical and critically ill patients were covered in a Swedish Symposium with the aim of developing a clearer picture of the current situation. 

What Are Lipids Use For?

• Drug Overdose
• Cardiotoxicity
• Nutrition Replacement

Anesthetic-Induced Cardiovascular Collapse

On the other hand, Intravenous lipids emulsion is an established, effective treatment for local anesthetic-induced cardiovascular collapse. The main theory for the mechanism of action is that by creating an expanded, intravascular lipid phase, homeostasis is established that drives the offending drug from the patients tissues into the newly formed "lipid sink". The theory of using IV lipid emulsions have been considered a generic form of reversal of toxicity caused by overdose of treatment in non-local anesthetic overdoses across a wide spectrum of drugs: beta blockers, calcium channel blockers, herbicides and several varieties of psychotropic agents. IV Lipid therapy is gaining acceptance in emergency rooms and other critical care settings as a possible treatments for drug toxicity. 

Cardiotoxicity that results from bupivacaine and types of anesthetics has been the subject of laboratory investigation for over 30 years.   Long acting anesthetics such as bupivacaine were implicated in several fatal cardiac arrests reported in 1979 by Albright [4]. 

Nutrition Replacement

"Energy deficit is a common and serious problem in intensive care units and is associated with increased rates of complications, length of stay, and mortality. Parenteral nutrition (PN), either alone or in combination with enteral nutrition, can improve nutrient delivery to critically ill patients. Lipids provide a key source of calories within PN formulations, preventing or correcting energy deficits and improving outcomes."[6]

Damage to Patients Veins

"Infusion of intravenous lipid and parenteral nutrition in the same line may protect against phlebitis and loss of the intravenous vein, possibly due to physical coating of the vein with the lipid particles or the reduced osmolality of the infused solutions. The osmolality of intravenous lipid solutions, irrespective of the lipid concentration, is 268 mOsm/L, making them safe for both central and peripheral lines."[5]

Learn more about Intravenous lipid infusions to help your patient have a better outcome.

References

1. http://www.ncbi.nlm.nih.gov/pubmed/23459217
2. http://www.sjtrem.com/content/18/1/51
3. Singer, P., Theilla, M. and Cohen, J. (2015) Use of intravenous lipids: what do the guidelines say? World Review of Nutrition and Dietetics. 112, p.163-71.
4. Albright GA: Cardiac arrest following regional anesthesia with etidocaine or bupivacaine.  Anesthesiology 1979, 51:285-287.
5. Innis,S:Lipids in Parenteral Nutrition.2013.
6. Intensive Care Med. May 2010; 36(5): 735–749. Published online Jan 14, 2010. doi:  10.1007/s00134-009-1744-5

What is Sepsis?

Sepsis is a consequence of infection that is difficult to predict, diagnose, and treat. Patients who develop sepsis have an increased risk of complications and death and face higher healthcare costs and longer treatment. 

CDC is working to increase sepsis awareness and improve treatment among the public, healthcare providers, and healthcare facilities. It kills more people than HIV/Aids, prostate cancer and breast cancer combined.

What is sepsis?

Sepsis is an illness that affects all parts of the body that can happen in response to an infection and can quickly become life-threatening.

In severe cases of sepsis, one or more organs fail. In the worst cases, sepsis causes the blood pressure to drop and the heart to weaken, leading to septic shock. Once this happens, multiple organs may quickly fail and the patient can die.

Sepsis is a serious illness that is very difficult to predict, diagnose, and treat. Patients who develop sepsis have an increased risk of complications and death and face higher healthcare costs and longer treatment.

What causes sepsis?

Sepsis is a response to an infection. When you get an infection, your immune system releases chemicals into your blood to fight the infection. The chemicals sometimes cause body-wide inflammation, which can lead to blood clots and leaky blood vessels. This impairs blood flow, which damages the body’s organs by depriving them of nutrients and oxygen.

Different types of infections can lead to sepsis, including infections of the skin, lungs, urinary tract, abdomen (such as appendicitis), or other part of the body. Healthcare-associated infections, including pneumonia, central line-associated bloodstream infections, catheter-associated urinary tract infections, and surgical site infections can sometimes lead to sepsis. MRSA infections of the skin and soft tissue can also lead to sepsis.

Who gets sepsis?

Anyone can get sepsis, but the risk is higher in:

• people with weakened immune systems
• infants and children
• elderly people
• people with chronic illnesses, such as diabetes, AIDS, cancer, and kidney or liver disease
• people suffering from a severe burn or physical trauma

How many people get sepsis?

CDC’s National Center for Health Statistics estimates that, based upon information collected for billing purposes, the number of times people were in the hospital with sepsis or septicemia (another word for sepsis) increased from 621,000 in the year 2000 to 1,141,000 in 2008.1

Between 28 and 50 percent of people who get sepsis die.2

The number of cases of sepsis each year has been going up in the United States. This could be because of the following reasons:

• the population is aging
• people have more chronic illnesses
• people are getting more invasive procedures, immunosuppressive drugs, chemotherapy, and organ transplants
• increasing antibiotic resistance
• increasing awareness and tracking of sepsis

Are there any long-term effects of sepsis?

Many people who survive severe sepsis recover completely and their lives return to normal. But some people, especially those who had pre-existing chronic diseases, may experience permanent organ damage. For example, in someone who already has kidney impairment, sepsis can lead to kidney failure that requires lifelong dialysis.

To learn more about Sepsis; visit the following resources:

• CDC
• NIH
• UK Sepsis
• Sepsis in Scotland

Do You Know How to Use an Intraosseous Device (IO)?

Intraosseous Devices

The recognition of the intraosseous(IO) vascular access device in fluid resuscitation and stabilization of patients has been underrated for quite some time. Intravenous therapy alone has made significant strides in the past forty years. The need to administer a wide variety of fluids and medications to a patient and affect their circulatory system is critical in the resuscitation of patients in an emergent situation regardless of their underlying disease process. Rapid vascular access is required for many conditions to include but not limited to hypovolemic shock, trauma, anaphylactic shock and cardiac arrest . There have been several “leading national and international organizations” that have published position statements as well as performed research studies to advance the cause of the IO. The IO has served to change the standard of care in the emergency room in regards to the quick need for vascular access, but also is making strides to the way we care for patients in the intensive care units (ICU’s) and medical-surgical units.
Among the leading organizations who state that the IO can play a significant role in the care of patients in need of resuscitation is the American Heart Association (AHA), addressing vascular access in cardiac arrest patients¹, the Infusion Nurses Society (INS) ², the Emergency Nurses Association (ENA)³ and the American Association of Critical-Care Nurses (AACN) all endorsing the INS position statement.² The intraosseous device is recognized by these professional organizations due to the significant time savings that patients benefit from in an emergency situation, as well as the minimal complications in relation to central venous catheters and peripheral venous catheters. The Intraosseous device allows healthcare professionals to decrease the time required to achieve access as well as administer necessary intravenous fluids and medications needed for resuscitation or stabilization of a patient throughout the different areas of nursing. The AHA came to the conclusion that a peripheral intravenous catheter (PIV) and an intraosseous device (IO) can administer equally and predictably the same pharmacological effects and in fact the IO device has a direct correlation to the central venous system and fluids and or medications can be sent to the central venous system within seconds not minutes.Using an ESRD) type patients. The technique of placing an intraosseous device is rather simple, involving placing the device tip into the bone matrix with a dwell time of 24 hours in order to deliver crystalloids, colloids, or medications through this device that will have an immediate effect on the patient via their systemic circulatory system via the bone marrow cavities.


History 

Intraosseous (IO) infusions methods were researched and developed as early as the 1920’s

by C.K Drinker , a medical doctor. Doctor Drinker studied the use and effects of the IO device on the sternum and developed a theory that access to the intraosseous space should be considered a non-collapsible vein which most likely gave direct access to the central circulatory system.(Vidacare) In 1940 Dr. Henning used the IO device successfully to infuse a patient with blood into the intraosseous space of the sternum.  These types of IO devices were a part of military medical kits during WW II; however, due to the lack of organized emergency medical personnel stateside and the development of plastics after WW II, there was a decreased interest in IO infusion and thus the IO devices fell to the way side until the late 1970’s and early 1980’s. The development of enhanced plastics led to the means for medical personnel to perform what is now commonplace of initiating an intravenous (IV) catheter by which medications and fluids are infused into a patient’s vein. 

The intraosseous device was still considered an important first line treatment method for the pediatric patient especially in Pediatric Advanced Life Support (PALS). During this time period IO infusions were typically performed by using a Cook or Jamshedi needle. Both of these types of IO devices required some sort of manual screwing or boring to enter the bone matrix.

Even though most IO devices in use at the time required placement into the patient’s tibia; the first IO device approved for adult use by the United States federal Drug Administration (FDA) was the FAST1 which could be placed in the patient’s sternum. The use of IO is endorsed by the American Heart Association, European Resuscitation Council, Association for Critical Care Nurses, Emergency Nurses Association, and many more professional agencies throughout the U.S. and the rest of the world. Most if not all of these organizations recommend the IO device as the alternative to a peripheral venous access device if it cannot be rapidly established on a patient needing vascular access. Many military medical personnel including the U.S. and Israel are trained in the rapid use of an IO infusion on the battlefield. This is documented in the Emergency War Surgery and Tactical Combat Casualty Care Guidelines.


Purpose of an IO
The purpose of an IO is to obtain circulatory access in order to provide necessary intravenous fluids, medications, and/or blood products for a patient needing an intravenous catheter. However, sometimes during emergent situations a peripheral venous catheter is difficult to obtain so an alternative is needed. It is recognized nationwide that a lack of immediate vascular access can lead to unnecessary morbidity or mortality for our patients. It is imperative to establish some form of vascular access; especially in emergent situations where time is critical.

Indications for Use
The Federal Drug Administration (FDA) has approved the EZ-IO, BIG-Gun IO, and the FAST 1-IO for use in specific bones and for specific patients. The FDA states that the EZ-IO and BIG-Gun may be used for adult and pediatric patients in the proximal humerus, proximal tibia, and distal tibia. The FAST 1-IO is approved by the FDA for use in the manubrium (sternum).The FAST 1-IO is for use in patients greater than eight years of age and should be considered for use in any seriously ill or injured patient in which a PIV cannot be established in a timely manner. The anatomical landmarks to insert the IO device are the same in adults as they are for pediatric patients. The device should be inserted just medial to the tibial tuberosity, on the flat portion of the proximal tibia. Most medications, fluids, and/ or blood products that can be given intravenously may also be administered via the intraosseous route.


To Learn more about Intraosseous Devices, how they are used, how to place them visit us and take our online course Intraosseous Devices and Earn CE's.

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Enterovirus D68 (EV-D68)

Enterovirus D68 (EV-D68) is one of more than 100 non-polio enteroviruses. This virus was first identified in California in 1962.

What are the symptoms of EV-D68 infection?

EV-D68 can cause mild to severe respiratory illness.

• Mild symptoms may include fever, runny nose, sneezing, cough, and body and muscle aches.
• Severe symptoms may include wheezing and difficulty breathing. See EV-D68 in the U.S., 2014 for details about infections occurring this year.

Anyone with respiratory illness should contact their doctor if they are having difficulty breathing or if their symptoms are getting worse.

How does the virus spread?

Since EV-D68 causes respiratory illness, the virus can be found in an infected person’s respiratory secretions, such as saliva, nasal mucus, or sputum. EV-D68 likely spreads from person to person when an infected person coughs, sneezes, or touches a surface that is then touched by others.

What time of the year are people most likely to get infected?

In the United States, people are more likely to get infected with enteroviruses in the summer and fall. CDC expects, as with other enteroviruses, that EV-D68 infections will likely begin to decline by late fall.

How common is EV-D68 in the United States?

In general, a mix of enteroviruses circulates every year, and different types of enteroviruses can be common in different years. Small numbers of EV-D68 have been reported regularly to CDC since 1987. However, this year the number of people reported with confirmed EV-D68 infection is much greater than that reported in previous years. See EV-D68 in the U.S., 2014 for details about infections occurring this year.

Who is at risk?

In general, infants, children, and teenagers are most likely to get infected with enteroviruses and become ill. That's because they do not yet have immunity (protection) from previous exposures to these viruses. We believe this is also true for EV-D68. Adults can get infected with enteroviruses, but they are more likely to have no symptoms or mild symptoms.

Children with asthma may have a higher risk for severe respiratory illness caused by EV-D68 infection.

How is it diagnosed?

EV-D68 can only be diagnosed by doing specific lab tests on specimens from a person’s nose and throat.

Many hospitals and some doctor’s offices can test ill patients to see if they have enterovirus infection. However, most cannot do specific testing to determine the type of enterovirus, like EV-D68. CDC and some state health departments can do this sort of testing.

CDC developed, and started using on October 14, a new, faster lab test for detecting EV-D68. Read more about what CDC is doing about EV-D68 in 2014.

CDC recommends that clinicians only consider EV-D68 testing for patients with severe respiratory illness and when the cause is unclear

What are the treatments?

There is no specific treatment for people with respiratory illness caused by EV-D68. Talk to your child’s doctor about the best way to control his or her symptoms.

Some people with severe respiratory illness may need to be hospitalized.

There are no antiviral medications currently available for people who become infected with EV-D68.

How can I protect myself?

You can help prevent yourself from getting and spreading EV-D68 and other respiratory illnesses by following these steps:

• Wash hands often with soap and water for 20 seconds. SeeHandwashing: Clean Hands Save Lives.
• Avoid touching eyes, nose and mouth with unwashed hands.
• Avoid close contact such as kissing, hugging, and sharing cups or eating utensils with people who are sick.
• Cover your coughs and sneezes with a tissue or shirt sleeve, not your hands.
• Clean and disinfect frequently touched surfaces, such as toys and doorknobs, especially if someone is sick.
• Stay home when you are sick.

Also, see an infographic that shows these prevention steps.

There are no vaccines for preventing EV-D68 infections.

What should people with asthma and children suffering from reactive airway disease do?

Children with asthma are at risk for severe symptoms from EV-D68 and other respiratory illnesses. They should follow CDC’s guidance to maintain control of their illness during this time.

CDC recommends:

• Discuss and update your asthma action plan with your primary care provider.
• Take your prescribed asthma medications as directed, especially long term control medication(s).
• Be sure to keep your reliever medication with you.
• Get a flu vaccine when available.
• If you develop new or worsening asthma symptoms, follow the steps of your asthma action plan. If your symptoms do not go away, call your doctor right away.
• Parents should make sure the child’s caregiver and/or teacher is aware of his/her condition, and that they know how to help if the child experiences any symptoms related to asthma.

For additional information, see “What Parents Need to Know about Enterovirus D68” in

• English
• Spanish
• Haitian Creole (translation provided by State of New Jersey Department of Health)

Surviving Sepsis with the Sepsis Protocol

Does Your Hospital Use the The Surviving Sepsis Campaign?

This campaign was created to help healthcare workers implement the recommended guidelines for Severe Sepsis patients in the Emergency Room (ER) and Intensive Care Units (ICU). 

The Surviving Sepsis Campaign kits include posters, pocket guides, bundle cards, lapel pins and a list of resources to aid your implementation efforts using the most recent edition of the international sepsis guidelines.  The Surviving Sepsis Campaign has developed pre-packaged kits to help clinicians within institutions to improve sepsis identification, management and treatment. 

"The Surviving Sepsis Campaign (SSC) partnered with the Institute for Healthcare 

Improvement (IHI) to incorporate its “bundle concept” into the diagnosis and treatment 

of patients with severe sepsis and septic shock. We believe that improvement in 

the delivery of care should be measured one patient at a time through a series of 

incremental steps that will eventually lead to systemic change within institutions and 

larger health care systems. "

Are You A LEADER??

L Learn about sepsis and quality improvement by attending local and national sepsis 

meetings. 

E Establish a baseline in order to convince others that improvement is necessary and 

to make your measurements relevant. 

A Ask for buy-in from institutional leadership and seek initial support from the emergency 

department (ED) and ICU staff and directors, quality improvement personnel, nursing 

staff, and others. 

D Develop an institution-specific protocol comprising all bundle elements. 

E Educate stakeholders in the ED and ICU and floors according to shift schedules. 

R Remediate errors and anticipate obstacles along the way. 

What is the First Step??

"The first step in improving the care of patients with severe sepsis and septic shock is 

making a solid commitment to improving that care. This commitment includes a strong 

and well-worded aim statement that sets an aggressive global aim. It is critical that the 

overall aim has a measurable objective and a specified time frame."

What Can You DO??

1. Time from ED triage to presumptive diagnosis of severe sepsis is less than 2 hours
2. Time from ED triage to all patients’ meeting severe sepsis criteria having a serum lactate is < than 3 hours
3. Time from ED triage to appropriate antibiotics given is less than 1 hour
4. If hypotensive or if lactate > 4.0 mmol, immediate fluid resuscitation is started (at least 30 mL/kg normal saline or lactated ringers solution within 1 hour)
5. If MAP < 65 mmHg and not responsive to adequate (at least 30 mL/kg) fluid resuscitation, vasopressors are started immediately
6. If blood pressure or serum lactate not responsive to fluid, a central venous pressure monitor is instituted within the first 6 hours

What About Bundles?

"​The Surviving Sepsis Campaign Bundles are the core of the sepsis improvement efforts. Using "bundles" simplifies the complex processes of the care of patients with severe sepsis. A bundle is a selected set of elements of care distilled from evidence-based practice guidelines that, when implemented as a group, have an effect on outcomes beyond implementing the individual elements alone."

Bundle Implementation

Your hospital's sepsis protocol may be customized, however, it must meet the standards created by the bundle. Enhancing reliability of these bundle elements allows teams to focus on aspects of the changes they are implementing to create a reliable system that achieves the goal of 25 percent reduction in mortality due to sepsis called for by the Surviving Sepsis Campaign.

Hear His Story??

To Learn more and get all of the Surviving Sepsis Campaign resources visit their site here.

References

http://www.survivingsepsis.org/Pages/default.aspx

I Got Your Breach!! Where is My EBOLA!!

SO I have been thinking about Nina Pham.  Who is Nina?  Well if you have to ask; then I think you should stop reading and go check your heart and soul.  The Center for Disease Control and Prevention (CDC) has a poster that has been put out that shows you exactly how to put on and take off your Personal Protective Equipment (PPE) in case you have a patient in isolation for whatever reason.

However, I have been studying this poster and thinking about all my years of training; not only in the civilian world; but the military as well.  What is missing from the poster?  Can you guess?

The Poster provided to us by the CDC is shown as follows:  

Well, have you guessed?  What about the healthcare provider cover their hair or head?  What is a patient with the EBOLA virus is having a coughing spell; or having massive diarrhea and you are the one having to help them and care for them?  What if those small particles get on your head and hair?  No, not possible?  Then look at the next part of the CDC poster when you are to remove your PPE.

Look at numbers 2, 3, and 4 in the photo below.  You have already taken off your gloves the way you have been trained; but then you reach up to remove your goggles; then untie or pull off your mask and at anytime in these steps it would be easy for a healthcare provider to brush against the back of their hair.  TRUE, the next step is to wash your hands; however, what if you are hot and sweating and you forget and accidentally rub your face without even blinking and eye.

The average person touches his or her face 2000 times a day or more without even recalling that we did it.  Just like driving home in the morning after a long twelve hour shift and cannot even recall what we did or saw because it has become automatic.

Well what does the World Health Organization (WHO) say about EBOLA and how to protect yourself?  Well, they say that a provider should use rubber boots if at all possible; but if not available, then shoe covers.  Did you hear me?  Rubber boots!!  This is what we used in the military during chemical warfare training.   They also go on to say that "If an impermeable  gown is not  available, place waterproof  apron  over gown."  Where is that in the CDC Protocol?

What about the products we use to clean and disinfect.  The "Purple Top Super Sani-Wipes."  How effective are they at killing the EBOLA virus?  Have they been tested on this virus?  Did you know it can live on inanimate objects outside the body for up to seven weeks?  What is the kill time when using these products on this specific virus? Well, there is a lot of information out there to try and protect yourself; the only issue is there are a lot of conflicting protocols and standards and no one seems to be on the same page.  Speaking of page; read page 29 in the CDC's Viral Hemorrhagic Fever Manual and you can see the conflicts I am referring too..

The WHO is much more specific in removing the PPE especially the mask, headgear, 

and face shield.

This is only the tip of the Iceberg as when dealing with such a virulent pathogen; there really should be a designated spot between the clean and dirty side of the decontamination area as well as nurses should be using the buddy system.  Did Nina have a buddy?  What is the patient to nurse ration in Texas? Do they have one?  These are all important questions that should have been raised before Nina stepped into the room.  However, as nurse for over 20 years, I know that if one were to ask such simple questions you may get an answer of; "well if you do not like it, find another job."

Well, I am not advocating for nurses to walk off just yet; remember you took a pledge when you were pinned as a nurse.

"I solemnly pledge myself before God and in the presence of this assembly, to pass my life in purity and to practice my profession faithfully. I will abstain from whatever is deleterious and mischievous, and will not take or knowingly administer any harmful drug. I will do all in my power to maintain and elevate the standard of my profession, and will hold in confidence all personal matters committed to my keeping and all family affairs coming to my knowledge in the practice of my calling. With loyalty will I endeavor to aid the physician in his work, and devote myself to the welfare of those committed to my care."

Stay Safe and Speak Up!  We are all in this together and we must rely on each other to get the truth out and save our own lives as well as our patients.

Dr. D.

Central Valley Medical would like to introduce "Heather Connor." 

 Heather Connor is originally from The Unites States; however, she has spent the last 11 years in Canada. Two years ago, after a long search that yielded no answers for the cause of her boys mystery symptoms, she gave up her career as a Surgical Device Rep, to focus on getting her kids the care and treatment they needed. Since then, both of her children and husband have been diagnosed with Dystonia, a little known neurological movement disorder that causes painful twisting and contractions of any voluntary muscle in the body. She has found her voice as an advocate for dystonia through her blog "Raising Dystonia".

by Heather Connor October 13, 2014

He was younger than I imagined. Maybe forty...tops. He was tall and attractive with light brown hair and glasses. He was Yale educated with a B.Sc, an M.Sc, a Ph.D and an MD. My research revealed that he is a pioneer in the field of neurogenetics and our  Pediatric Neurologist had told me he is the most brilliant diagnostician he had ever met.  Needless to say, his reputation preceded him.  His list of medical discoveries and publications was shockingly long.  It was the kind of resume I had expected from an older seasoned  physician.  Not from the young, unassuming man standing in front of me.
He entered the room with an entourage that made him seem more like a celebrity than a neurologist.  He quickly introduced himself and the team he brought with him. The doctor then sat down and immediately started asking questions.   He had clearly read the gigantic chart in front in him.  It took up a whole binder and contained the medical transcripts of my boys from their last eight specialists.  He asked mostly new questions, questions not a single doctor or nurse had asked us in four years, which amazed me.  I thought we had been asked everything possible, but apparently there were gaps in the previous assessments. It gave me hope that he could be the one to confirm that their issue was indeed neuromuscular.

After he examined the boys, he listened to us talk for an hour, as we explained the symptoms and events that led us to this appointment. I kept trying to rush. Experience had taught me that specialists were always in a hurry. But it was hard, there was just too much information between both boys and so many tests and different doctors. However,  this appointment was different. In an action so simple yet so powerful, he became the first doctor to never once look at his watch. And I loved him for it.

After we were done, he leaned forward in his chair placing his elbows on his knees.  He looked at me most sincerely and said,  "I'm not sure why anyone ever thought this was neuromuscular, It's not.  I'm sorry. I know you've been given the run around and I can only imagine how frustrating this process has been."  My heart sank.  It was not neuromuscular, and he was going to pass us on the the next specialist just like all the rest.  I could feel the lump forming in the back of my throat and I tried to swallow the urge to cry.  The search for answers was going to continue.  "But," he went on, "for reasons that professionally frustrate me, I can only say that I'm sorry their Dystonia went missed for so long."

I froze.  I thought I just heard him say something that resembled a diagnosis, but I was too afraid to be told otherwise to ask.  Instead I sat frozen and stared at him as he explained, "There are many forms of the disorder so more tests must be done.  I wasn't sure what was wrong, so I brought the whole team. I didn't want you to have to make a separate appointment to get your questions answered."  I couldn't believe what I was hearing.  Here, in this room, were the answers we'd been searching for years to get. I was beyond surprised that he was opening the door for more questions and not getting ready to kick us out for taking so long.  I felt immediate relief.  I had something to call what was happening. Dystonia. I also felt validation for all the years I spent feeling like I was crazy.  But mostly, I was confused. How could he diagnose so quickly? It seemed so easy and it left me wondering why it had been so hard for everyone else?   My husband and I said nothing. We were  too overwhelmed.  As we stared at him, the doctor leaned forward and placed his hand on top of mine.  When he spoke it was as though he knew exactly what I needed to hear,  "Ma'am, you are NOT crazy.  You are right. Something is hurting your boys.  It's called Dystonia. I want you to know I am confident that I can help them."

I found myself wrapped around the doctor in a hug entirely too large to be appropriate.  He hugged me back and  comforted me as I cried.   "Thank you!" I said over and over again.  His lack of surprise and perfect response to my flood of emotions told me I was not the first mom to flip out on him after receiving a diagnosis.

After I pulled myself together I couldn't help but ask, "How can you be sure it's Dystonia?" He just smiled, and informed me that it was as clear and classic a presentation as they come. He had no doubts.  All seven members of his team nodded their heads in agreement.  "If it's so classic, how did eight specialists miss it!? How did neurologists miss it!?", I protested, still confused how the answer was seemingly so obvious. He looked at me with compassion on his face and his answer broke my heart, "It's one of those things that professionally saddens me," he explained, "They don't really teach much about Dystonia in medical school.  It's the third most common movement disorder and yet, somehow, even movement disorder specialists sometimes miss it. Your boys went undiagnosed for so long because there is a lack of awareness within the medical community.  I'm sorry."  I nodded to let him know I understood. But I did not. How was it possible all those doctors could not have known!? How could they not be aware of it?! It made me both angry and sad.
After we asked all our questions, he handed me a requisition for DNA tests, prescriptions to help the boy's pain, and the personal contact information for everyone on the team.  As he opened the door to leave, he said to me,  "I really think I can help your boys.  I'm going to use all my resources to make sure I do."  I smiled at him, and with tears rolling down my face, said, "You know you just changed our life, don't you?"  He just nodded and said, "I hope so ma'am. I really hope so."  He closed the door behind him as he went to see his next patient.

With each new interaction with our neurologist he has continued to impress. His reputation for brilliance is well deserved.  But what I was not expecting was his bedside manor.  More than any other specialist he understands the importance of our relationship with him.  He believes that we are a team and he knows that I see day to day what he cannot. When I tell him what is happening, he believes me.  Most importantly, he never forgets that I am a mother. When I email him freaking out late at night because I'm scared that my child is convulsing, he replies right away.  Even if he is not "medically" concerned, he knows that for a mother, waiting till the morning is waiting an eternity. 

 No longer do I wrestle with unending fear and helplessness.  He has changed our lives in more ways than he could know.  We sleep, eat, play, and live better because of one person's compassion and awareness. My boys have never done so well for so long.  Aside from the medicine and information he has given us, it's his ability to diffuse our fears and believe in us as parents that  has made the biggest impact on us.  I feel like he has given me back my power as a mother.  I feel like for the first time, I have support from someone who can actually help. The feeling is amazing.

It is a feeling I wish more families facing our challenges could experience. Tragically, our experience is a microcosm of what the Dystonia community experiences.  But, we are luckier than most. More often than not, patients have to live without ever finding that life altering, compassionate specialist. Dystonia is more common than ALS, Muscular Dystrophy, Parkinson's and Huntington's Disease. Yet the medical community is not familiar with it. There are roughly half a million families in North America fighting the same battle as me.  It saddens me that the system is ill equipped to diagnose and treat them and that they must endlessly struggle with the fear and helplessness that has defined our past four and a half years.

It is my hope that one day families with Dystonia do not have to constantly explain to doctors, nurses and first responders about the cramping and twisted posturing that is Dystonia. And it my prayer, that they will understand Dystonia well enough to have compassion for a disorder that is simultaneously so silent and debilitating. I can only dream that there will be more people with Dystonia hearing, "I know what's wrong," and forever have both their lives and families transformed.

So to you Dr. D, thank you for caring about Dystonia! Please know that you are changing the lives of patients that are desperate for answers to questions that so many of your peers are unfortunately unable to answer. My kids will probably never understand just how much you have done for them, but I will never forget.  Thank you for your patience, kindness and for not treating me like a crazy mom.  But more than anything, thank you for giving my boys back their childhood.  It's the most beautiful gift anyone has ever given them, and I will remain forever in your debt.