Report from Doctor in Houston about Joshua's testing

I know so many of you are curious about the results of the tests Joshua had just before Christmas in Houston. I just got an email from one of the doctors there with these results/plan of action and wanted to share them with you:

----- Forwarded Message ----
From: "Pepiak, Derek"
To: james wooten
Sent: Tuesday, February 24, 2009 2:35:21 PM
Subject: RE: Joshua Wooten

Dr. Koenig has finished reviewing all of Joshua's labs, MRIs, EEGs, etc. Here's a copy of here most recent note in his chart for you and any of his physicians there:

"Copies of medical records from San Antonio and results of Joshua's evaluation have been reviewed:

Medical records from Joshua's hospitalization in 2005 clearly demonstrate a picture of encephalitis or ADEM. He has an elevated CSF protein with a pattern on the MRI showing focal abnormalities in the right frontal lobe with diffuse meningeal enhancement.

Metabolic studies were normal, including: CSF neurotransmitters, Plasma amino acids, CSF amino acids, Urine organic acids, CSF cell count, CSF protein, ammonia level, Chemistries, plasma lactate. CSF protein was slightly elevated and plasma pyruvate was elevated. The significance of these results is unclear at this time.

EMG and Nerve Conduction Studies were normal.

MRI brain was reviewed with neuro-radiology (Dr. Hochhauser). It demonstrates some areas of gliosis, particularly in the right frontal lobe that are consistent with a prior diagnosis of ADEM or encephalitis.

24 hour Video EEG was reviewed with Dr. Gretchen Von Allment. It demonstrates occasional generalized bursts consistent with global brainstem dysfunction.

Based on the above findings, I believe that Joshua is suffering from central hypoventilation, likely secondary to the insult that occurred in 2005 (ADEM versus encephalitis) and agree with placement of a diagphragmatic pacemaker.
Mary Kay Koenig, MD"

Well that's all fine and dandy, you might say, but what does it mean? Basically what she is saying is that she believes that that he experienced an episode in 2005 of either encephalitis or ADEM that caused permanent damage to his brainstem and is currently resulting in central hypoventilation. Some people think that there is a genetic component to late onset CCHS or ROHHAD and that may be the case. However, in Joshua's case, it seem more likely that whatever occured in 2005 damaged his brainstem resulting in his current symptoms. Regardless of how it happened though, we think pacers are the way to go in the hopes of freeing Joshua from the ventilator, at least while awake. It is a slow process as the pacers are usually implanted one at a time (as Dr. Cox probably explained to you) and he would need further sleep studies for titration once the pacers are implanted and activated. Also, the Sleep Medicine physicians feel he may need to have another sleep study to truly assess his baseline as there were some problems with the study we did here. I will discuss with them further whether or not this is truly necessary.

Either way, we'd like to shoot for the beginning of June to get this done, if you'd still like to go forward with it. When is Joshua's last day of school? I will check with Dr. Cox to see if Joshua needs to be seen in their surgery clinic before proceeding or if his previous consultation was sufficient. Maybe by the end of the summer, we can decommission his ventilator cart!

Happy Mardi Gras!

--Derek


So what is ADEM?
The following is taken from the National Institute of Neurological Disorders and Stroke website:

What is Acute Disseminated Encephalomyelitis?

Acute disseminated encephalomyelitis (ADEM) is characterized by a brief but intense attack of inflammation in the brain and spinal cord that damages myelin – the protective covering of nerve fibers. It often follows viral infection, or less often, vaccination for measles, mumps, or rubella. The symptoms of ADEM come on quickly, beginning with encephalitis-like symptoms such as fever, fatigue, headache, nausea and vomiting, and in severe cases, seizures and coma. It may also damage white matter (brain tissue that takes its name from the white color of myelin), leading to neurological symptoms such as visual loss (due to inflammation of the optic nerve) in one or both eyes, weakness even to the point of paralysis, and difficulty coordinating voluntary muscle movements (such as those used in walking). ADEM is sometimes misdiagnosed as a severe first attack of multiple sclerosis (MS), since some of the symptoms of the two disorders, particularly those caused by white matter injury, may be similar. However, ADEM usually has symptoms of encephalitis (such as fever or coma), as well as symptoms of myelin damage (visual loss, paralysis), as opposed to MS, which doesn’t have encephalitis symptoms. In addition, ADEM usually consists of a single episode or attack, while MS features many attacks over the course of time. Doctors will often use imaging techniques, such as MRI (magnetic resonance imaging), to search for old and new lesions (areas of damage) on the brain. Old “inactive” brain lesions on MRI suggest that the condition may be MS rather than ADEM, since MS often causes brain lesions before symptoms become obvious. In rare situations, brain biopsy may show findings that allow differentiation between ADEM and severe, acute forms of MS. Children are more likely than adults to have ADEM.

Is there any treatment?

Treatment for ADEM is targeted at suppressing inflammation in the brain using anti-inflammatory drugs. Most individuals respond to intravenous corticosteroids such as methylprednisolone. When corticosteroids fail to work, plasmapheresis or intravenous immunoglobulin therapy has been shown to produce improvement. Additional treatment is symptomatic and supportive.

What is the prognosis?

Corticosteroid therapy can shorten the duration of neurological symptoms and halt further progression of the disease in the short term, but the long term prognosis for individuals with ADEM varies. For most, recovery begins within days, and half will recover completely. Others may have mild to moderate lifelong impairment. Severe cases of ADEM can be fatal. Some individuals who initially diagnosed as having ADEM will later be reclassified as MS, but there is currently no method to determine whom those individuals will be.

What research is being done?

The National Institute of Neurological Disorders and Stroke (NINDS) and other institutes of the National Institutes of Health (NIH) conduct research related to ADEM in laboratories at the NIH, and also support additional research through grants to major medical institutions across the country. Much of this research focuses on finding better ways to prevent, treat, and ultimately cure demyelinating disorders such as ADEM.

EEG Abnormalities and House Renovations Continue

We've been waiting for weeks to hear what the doctors found from Joshua's testing just before Christmas. Today, I got word that there WERE some abnormalities found in his EEG. EEG stands for electroencephalogram. It is a test that measures and records the electrical activity of the brain. It helps to diagnose conditions such as epilepsy (seizure disorder). We have not yet been told what kind of abnormalities Joshua has. The doctor is conferring with an epileptologist first.

Before Joshua had the test, the doctor mentioned that if the cause of Joshua's medical problems could be pinpointed in his brain, they may be able to do a surgery to correct it. We have been hoping and praying for that so Joshua would no longer need the ventilators, trach tubes, many medications and growth hormones. We hope it would help him be able to get back to a normal life style. If doctors DO find out the cause and can correct it, that could also help the 30 or more others in the world also suffering from the same disorder.

As soon as we hear anything definite, I will pass it along.



Meanwhile, we have been preparing our home for the market. I've written about some of the changes we made starting in December.... including changing out light fixtures and door knobs, staining the front porch and re-arranging some rooms. We have also put up wainscotting (beadboard panelling) in all three of our bathrooms. And this past weekend (Valentine's Day) we invested in wood laminate flooring and ripped out the carpet that was in our living and dining rooms.

We will be laying the wood laminate in our kitchen, laundry room and downstairs bathroom, too. It is a lot of work and it is already looking great! By the time we finish (hopefully within another week or two - after my sister moves out and into her apartment) the place should be fully market ready.

Please pray with us that the doctors will find a way to help Josh and the others with ROHHAD. And pray that we are able to sell our home for enough to pay off all our debt and find the new (one-story) house that God has planned for us.


Oh, one more thing. I am an aunt again. My brother and sister-in-law had a baby boy last night. He weighed in at 9 pounds 4 ounces and was 20 3/4 inches long! I can't wait to get to go see him!

Sweat Test and Showering Nude Near Alamo

I took the day off work today to take Josh across San Antonio to Methodist Hospital for a sweat chloride test. We scheduled it last week when he saw his pulmonologist. It was actually recommended by the pulmonary doctors in Houston. They wanted to rule out Cystic Fibrosis because Joshua showed signs of inflammation in all his sinuses in his MRI. They first prescribed 2 weeks worth of antibiotics, which Joshua finished a week or so ago.

The clinician who performed the sweat test today told us that our doctor should have the results by tomorrow.

On the way home from the hospital, Joshua and I turned on the radio to get ready to listen to Dave Ramsey on AM 550. His show would be on in just a few minutes (from 1-4pm). But as we got out onto the highway, we heard a reporter interviewing people and telling of something strange happening downtown near the Alamo. Crowds were gathering there in anticipation of an event scheduled by PETA representatives. PETA stands for People for the Ethical Treatment of Animals.

A couple of women were going to be showering naked in front of the Alamo in protest of something. We couldn't understand what the protest was supposed to be about. And before long, the Dave Ramsey show was on. So, we forgot about the crazy women of PETA and their live event.

But as I signed onto my website, VWootenWorld, I saw my live picture I keep on here of the Alamo, and that brought to my mind the event we'd heard about hours ago. I looked up the story online to see if I could figure out what the protest was all about. And here is what I found:




http://qik.com/video/976410

and

http://www.ksat.com/news/18642119/detail.html



Apparently, the idea was to try to convince people that it is not only animal friendly to be a vegetarian, but also environmentally friendly - somehow. Anyway, I still love meat too much to become a vegetarian. And no matter how many crazy people get naked and take showers in public, that is not likely to change my mind.

Oh, and we are having Pot Roast for dinner tonight. In fact, it should be done now... and my tummy is already grumbling... bring on the beef!