Hypoxic Ischemic Encephalopathy (HIE)

Overview
Hypoxic Ischemic Encephalopathy (HIE) is a brain injury caused by a lack of oxygen.  The injury affects the brain as a whole as opposed to a part of the brain.  There are many things that can cause low oxygen levels in the brain.  Most often HIE refers to new born infants.  HIE can apply to many other brain injuries as well (near drownings, drug overdose, respiratory failure, smoke inhalation, choking, blood loss, etc.).  Low oxygen levels may lead to an HIE diagnosis- not all low oxygen events result in HIE however.

1996 guidelines from the AAP and ACOG indicate that there must also be a profound metabolic or mixed academia in the cord blood (if obtained), the APGAR must be between 0-3 for more than 5 minutes, there needs to be evidence of neurological events (seizure, coma, hypotonia, etc.) and the event must involve multiple organs.   Some cases of HIE do not fit the AAP and ACOG guidelines.  There are many cases reported where the symptoms do not present until minutes, hours, or even days after the event.

HIE is scored on 3 levels, mild, moderate or severe.  The most commonly used grade is the Sarnat Staging System developed by Sarnat and Sarnat in 1976.  If you are interested in more details on how the Sarnat Stages work, please click HERE.

It is estimated that HIE is seen in 1-8 of every 1000 births.  The mortality rate is said to be 25-50% (most deaths occur within the first week due to other organ failures, aspiration pneumonias, or systemic infections).  Currently about 80% of infants that survive a severe HIE event suffer serious health complications.  About 10-20% suffer mild-moderate health complications.  

Symptoms
Symptoms vary for all HIE patients.  I recently heard someone say “We know more about the brain than any other organ- and still we know the least about what to do with it.”  This hits home with anyone dealing with a HIE.  Tests can show that damage to the brain occurred.  However, everyone reacts different and some brains seem to recover some of the lost or damaged areas while other brains do not.  Many HIE patients will experience some muscle issues (Cerebral Palsy).  It is often common to see many patients have problems with their complex reflexes (either the lack of reflexes, or over sensitivity).  These reflexes include gag, suck, swallow, blink, startle and light sensitivity.  Seizures, blindness and deafness are also often seen in HIE patients.  Many patients will also suffer from heart, GI, and pulmonary complications.  The severity of these symptoms will vary, and many patients have additional health complications as well (some secondary- some from the initial event).

Testing and Diagnosis
There are many tests that can be done to diagnose HIE.  Some of these include Brain MRI, Head CT Scan, EEG, Head PET Scan, cord blood gas, and a handful of others.  As previously stated however, diagnosing HIE is only the beginning.  Each patient has to be monitored and fully examined for any type of medical prognosis. 

Treatments
In the past 3-5 years there has been a lot done to improve the expectations for brain injured infants.  If the event is caught early, and treatments start within the first 6 hours, recent studies show that cooling (hypothermia) is giving these babies much better prognosis.  Just as you ice a sprained ankle, if you cool a brain while it is swelling (or other organs swelling as a result of the brain swell) the cooling reduces the swelling leading to less global necrosis (cell death).  Originally the cooling was done just on the brain by use of cooling caps.  Now there are also cooling blankets used in NICUs to cool the entire body.  The NICU staff has to maintain the core temperature very closely.  Not all NICU’s or ICU’s have the cooling equipment.  Most level IV and some level III NICU’s across the US are now able to cool babies that have suffered a brain injury.

Often after a full medical work up, HIE patients are given very poor expectations.  Doctors don’t always know what to tell parents. A lot of this is due to advances in medicine.  Doctors are able to revive and save many children that would not have previously survived.  The life saving skills have improved, but the rest of the medical field left to support the survivors are still catching up.  As time goes by there will hopefully be more statistics and data for HIE patients.  As the data becomes available hopefully there will be a more consistent, productive approach to the treatment, prevention, and rehabilitation of HIE patients.

Currently doctors treat the symptoms.  HIE patients often see a team of specialists.  These specialists can include just about any area.  Orthopedics often help patients dealing with muscle issues.  The ortho may recommend medications to loosen or stiffen muscles (such as Botox injections, baclofen or clonazepam to name a few).  Some may suggest surgery to help with range of motion, relieve pain, or help with positioning.  Pulmonologists are usually members on the medical team.  Many HIE patients have issues with swallowing with leads to aspirations and other airway issues.  The gag, suck and swallow issues usually lead to a gtube or some other form on non-oral feeding adding a GI to the team.  Seizures are almost expected with HIE patients.  A neurologist is usually on board to monitor the brain as the child develops as well as to manage seizures.  In addition to a team of medical specialists, most HIE patients also see a team of therapists (occupational, speech, physical, vision, etc.).

There are a few non-standard treatments.  Non-standard is also non-covered.  These treatments can be expensive, often require travel, and depending on the relationship with the current medical team, the patient may or may not get a lot of support for related follow-ups and/or possible complications.  These treatments are at the discretion of each patient (and his/her caregivers).  If you decide to pursue these treatments, please let your medical team know. 

Hyperbaric Oxygen Therapy (HBOT) is one option.  The patient is put into a pressure controlled tank with high levels of oxygen.  The idea is that the increased oxygen will increase blood flow to the brain.  Just like HIE is due to a lack of oxygen, many people feel that the increased oxygen through HBOT will help repair some of the damaged cells.

Stem Cell Therapy is another option.  Currently Stem Cell Therapy for brain injuries is not an FDA approved treatment.  If you chose to get stem cell injections you will have to leave the US.  There are a few countries (Mexico, China, Russia to name some) that do offer Stem Cell Therapy.  The idea behind stem cells is that stem cells are smart cells.  When stem cells are injected into the body they travel through the body looking for cells that signal distress.  When they see an area of distress they basically set up camp and work to replace those cells.  There are many different types of stem cells as well.  The controversy is over embryonic cells (cells from a fetus).  However, stems cells can also be harvested from umbilical cords, sharks, fat tissue, and even some studies have shown successful harvesting from menstrual discharge.  The source of stem cells is growing every day.

There are occasionally stem cell studies done in the US for brain injuries.  These studies do not guarantee acceptance for all that inquire.   Currently Duke is doing some studies.  If you are interested in participating in one of these studies, please contact Dr. Joanne Kurtzberg, director of the Pediatric Blood and Marrow Transplant Program at Duke University.

Resources
Brain Injury Association of America
National Institute of Neurological Disorders and Stroke

2 HIE Yahoo support groups-
HIESC@yahoogroups.com
HIE@yahoogroups.com

Facebook-
Hope for HIE
Newborn Brain Cooling
Hope for HIE
Beautiful Faces of HIE (Secret Support Group)
For those interested in joining, please message one of the Admins. (Lori Sproul, Shannon Rice Bourdeaux, Kristi McConnell, Candice Lindley or Sam Lees) and give a brief reason for wishing to join their group

Casey’s Story
Casey was born on April 23rd 2006 just short of 37 weeks.  Casey came early, but not easily.  I had a uterine rupture.  Some ruptures are really just tears; however mine split the uterus completely in half.  When the uterus ruptured, Casey and her placenta left the uterus and floated in my abdominal cavity.

Luckily we made it to the hospital very quickly.  The doctors and nurses worked very fast to get Casey out.  Based on the blood gas taken from her umbilical cord Casey went about 30 minutes with limited to no oxygen.  The APGAR was 0 at birth, no breath, no heart beat.  After 5 minutes the resuscitation team had a heart beat; after 10 minutes they had gasping breaths.  Casey was taken to the NICU where she stayed for the first 2 months of life.

The official diagnosis of HIE (Hypoxic Ischemic Encephalopathy) was given.  Casey has many side effects/complications.  Casey has Quadriplegia CP (Cerebral Palsy).  Casey's CP is a stiffness in her arms and legs.  The stiffness in her legs has lead to a deformation in her feet as well as a dislocation of both hips and elbows.  The stiffness in her hands lead to cortical thumbs (pointing inward).  Casey has a barrel chest that adds to complications in positioning, airway management and gtube positioning (so LEAKY).  In addition to CP, Casey also has all of her complex reflexes missing.  Casey is not able to suck, swallow, gag nor blink.  Without the ability to swallow Casey's air way is in constant risk.  Suction equipment is needed to remove secretions, and anything else in her mouth/throat, in order for her to breath.  Also, without the ability to swallow Casey cannot eat by mouth.  All of Casey's nutrients and calories come in liquid form through a G-Tube.  Without the ability to blink, eye safety is another issue.  Moisture is supplied often with drops and lube, and Casey's eyes have to be covered when there is high risk for foreign objects near the eye.  Casey also has moderate hearing and vision loss.  She is legally blind, but the eye doctors believe with help she can eventually see.  Her eyes work, she is just not able to comprehend everything she sees.  The same can be said for her hearing.  She hears some, and with hearing aids she hears a little better, but the loss is more about her brain not comprehending the sounds than it is about her ears not working.

Over time Casey has aspirated fluid into her lungs a few times due to the lack of swallow.  These aspirations led to a few hospital stays with very serious pneumonias that were escalated to Acute Respiratory Distress Syndrome - ARDS.  The ARDS has put Casey into a category of lung and respiratory related issues as well. Even though she was not born with respiratory issues, they can still be considered complications of HIE, just secondary.

When Casey was born the cooling cap method was in trial in 1 US hospital- not the hospital we were in.  In the first year we tried 40- 1 hour sessions of HBOT as well as an injection of 1 million umbilical stem cells.  The second year we went back for a second round of stem cells where 2 million were injected.  We saw some improvements around these times, but it is too hard to say for sure how much (if any) the non-standard treatments contributed.  Sadly this is part of the reason these treatments have not been able to pass FDA regulations yet.

Casey has many serious complications, and has had a very hard start to her life.  Regardless of the challenge or the pain Casey has fought through it all.  She is without a doubt the strongest and most amazing child I have ever met.  Doctors originally gave a very grim expectation for Casey.  Some said she would never go home at all.  Others said if she did go home it was most likely she would not see her first birthday.  Casey has proven them all wrong.  She has a huge, wonderful personality to go with her amazing will and strength.  She may be disabled; she is definitely determined.  

Marty Barnes, mother of five year-old Casey, is one of the admins for Mommies of Miracles.  She is a stay-at-home MOM and wife.  The Barnes family live in Austin, Texas.  Marty has a background in IT (specifically databases).  She uses this experience toward maintaining her daughter's site- Caseybarnes.org as well as her grassroots inCLUsion Campaign- CLU.  Marty is on the Parent Advisory Council at the local children's hospital and works with local organizations such as Hand to Hold and Texas Parent to Parent.