Success Story: Hydrocephalus; Hemophilia B


Hydrocephalus Success Story (NPH) with Hemophilia B

(From Post c. June 30, 2010)

 I've posted previously regarding my father's condition of NPH (Normal Pressure Hydrocephalus) and his resulting success.

 In 2000, my dad began a shuffling-of-the-feet and slowness of gait (walking slowly). During the next couple of years, he also evidenced hand tremors -- a quivering of his joints.

 When his symptoms intensified, he agreed to see a neurologist who diagnosed him in November 2000 with Parkinson's Disease. The doctor prescribed the gold standard Parkinson's medication, called Sinemet. The dosage was gradually increased for a year and a half -- but our father never showed signs of improvement. When the neurologist then told him that his Parkinson's was progressing faster than average, our mother sought a second opinion.

 In 2003, our dad was examined by Dr. Neal Hermanowicz at the University of California, Irvine. Dr. Hermanowicz actually suspected NPH (Normal Pressure Hydrocephalus) and ordered an MRI blood flow (also called an MRI flow test) at one of the few facilities in the area with a specialized machine that could perform that particular test.

 The test was inconclusive. Our father displayed two of the triad symptoms of NPH, poor balance and incontinence. He evidenced no noticeable dementia except for normal forgetfulness that a 72-year-old might have. He spent an additional two years of treatments, medication and physical therapy ordered by Dr. Hermanowicz.

 We enrolled him in a wonderful Balance and Mobility class directed by Dr. Debra Rose at one of the California State University Centers for Successful Aging. This kept his muscles strong and taught him how to prevent falls. In late 2003 he began a series of weekly six-hour gamma globulin infusions at the UCI Medical Center in Orange, CA. This is a treatment akin to chemotherapy, for what Dr. Hermanowicz then thought might be an autoimmune disorder. Following the twelve-week series, there was no improvement in my dad's condition. He was losing hope and becoming depressed. Finally, in 2004 Dr. Hermanowicz referred us to neurosurgeon Dr. Marvin Bergsneider, M.D., at UCLA.

 After his first appointment at UCLA, he spent five days at the UCLA Medical Center in July 2004 for a procedure called the lumbar drain, a precursor to shunt surgery. His doctor was Dr. Paul Vespa, M.D., at UCLA. Cerebral spinal fluid (CSF) was drained from his spine and measured hourly. The procedure did not bring noticeable improvement in his balance, as was hoped. Shunt surgery was postponed.
 
Several months later when he realized that his gait disturbance was advancing rapidly, he envisioned he would soon be wheelchair-bound. He finally decided to call Dr. Bergsneider to request shunt surgery.

 In 2005, he finally had an adjustable shunt implanted in his brain at UCLA. (see here, here and here and here for info.) The shunt implantation, along with periodic adjustments over a year, brought about significant improvement in our father's gait, joint motion, mood, confidence, and memory.

 We thank God for blessing us with this life-changing surgery and are so very grateful that we were led to Dr. Bergsneider. And we are grateful that there are medical devices that can make our father's life extension possible.

 Only a handful of manufacturers make shunts. Our father was given a programmable shunt. This means that when difficulties arise in the patient, such as urinary incontinence, the patient may ask that the shunt be adjusted. The doctor may "tighten" the setting on the shunt, and the patient can be given relief of the symptom, such as urinary incontinence.



A Word About Hydrocephalus
  • Adults can acquire hydrocephalus. Our father first had the symptoms in his 70's. It took about four (4) years from his symptoms to the date he was given a shunt implant. Since then, he's had several adjustments of his shunt.
  • And one thing was rare about his shunt. Our father is a hemophiliac. That means he risks significant losses of blood. That means that if he bleeds, depending upon the bleeding, it could get serious. Up until the very last minute, the medical exams apparently could not positively confirm that he had hemophilia. At the very last minute, the physician team concluded that he did, positively, have hemophilia.
  • And that means that he had to be given infusions during and after the surgery. A concentration was pumped into his wrist area to make sure that he did not suffer significant loss of blood.
  • Hydrocephalus is not only diagnosed in adults, but also in children. We've had the opportunity to meet people who had hydrocephalus from birth. Treatment and operations are available for those of different ages, including young people. For instance, CHOC, the Children's Hospital of Orange County, is staffed with neurosurgeons who have performed shunt implants on children. We've had the opportunity to meet with one of them.

----------------------------

June 11, 2010:
The Daily Decibel salutes Dr. Marvin Bergsneider: Best Doctor In America


 According to the UCLA Neurosurgeons listings, Dr. Marvin Bergsneider is Professor, Co-Vice Chair of Clinical Affairs. Dr. Bergsneider specializes in minimally-invasive and advanced endoscopic techniques for the treatment of pituitary tumors, skullbase tumors, and hydrocephalus.
Dr. Marvin Bergsneider, M.D.
Dr. Bergsneider has earned Best Doctor In America, 2009-2010. See here for details on Dr. Bergsneider's award for Best Doctor In America.

 We have personal experience with Dr. Bergsneider and his work in our family. Our father, for a period of many months, had a serious disorder. It's called Normal Pressure Hydrocephalus, or NPH. A better name would probably be Abnormal Pressure Hydrocephalus. In simple language, it's "fluid on the brain."

 In the year 2000, our father began shuffling and walking with an abnormal gait. He was in denial that it was a serious issue and he refused to see a doctor for several months. When his symptoms intensified, he agreed to see a neurologist who diagnosed him in November 2000 with Parkinson's Disease -- prescribed Sinemet, the "gold standard" medication for Parkinson's Disease.

 The dosage was gradually increased for a year and a half, but our father never showed signs of improvement. When the neurologist then told our father that his Parkinson's was progressing faster than average, we sought a second opinion.
Dr. Neal Hermanowicz, UCI
We've had the opportunity to be involved in support groups that discuss subjects which doctors often never seem to have time to address. Through one of our PD (Parkinson's Disease) support groups, we were given the name of Dr. Neal Hermanowicz, M.D., a movement disorder specialist at the Gottschalk Medical Plaza at the University of California, Irvine.
In March 2003, during our father's first examination, Dr. Hermanowicz told us he didn't think our father had Parkinson's at all. He suspected Normal Pressure Hydrocephalus (NPH). He ordered an "MRI Blood Flow" test at Long Beach Memorial Hospital, one of the very few facilities with a unique MRI machine.

 (For more information on "MRI Blood Flow Test", we recommend contacting UCI Healthcare or Long Beach Memorial Hospital in California.

The test was inconclusive. Our father displayed two of the triad symptoms of NPH, poor balance and incontinence -- but no noticeable dementia except for the normal forgetfulness of a 72-year-old. He spent an additional two years of treatments, medication and physical therapy ordered by Dr. Hermanowicz in the quest for a solution. He found himself leaning more and more on his cane -- and on our precious mother.
An MRI machine
At one point, Dr. Hermanowicz reported that our father's previous tests showed some small strokes and some degeneration of the spine.

 In 2003, he began using a walker. That same year, his driver's license expired, so he applied for renewal. He passed the written test 100%, but during the behind-the-wheel test, the unthinkable happened. While the vehicle was stopped at an intersection, his foot slipped off the brake and onto the accelerator pedal, causing an accident. A new license was, of course, denied.

 For a total of five years, our mother made it her mission to research the cause of our father's disorder and to find a cure -- and to take him out of the house every single day just so the four walls wouldn't close in on him, so-to-speak. That took some creativity. We enrolled him in a wonderful Balance and Mobility class directed by Dr. Debra Rose at the Cal State Fullerton University Center for Successful Aging. This kept his muscles strong and taught him how to prevent falls. He was also able to share some time with his fellow sufferers in the class. In the course of his suffering, our father did seem to benefit from the physical therapy he received.

In late 2003, he began a series of weekly six-hour gamma globulin infusions, a treatment akin to chemotherapy, at the UCI Medical Center in Orange, CA for what Dr. Hermanowicz then thought might be an autoimmune disorder.

 At the conclusion of the twelve-week series, there was no improvement in his condition. He was losing hope and becoming depressed. Finally, in 2004, Dr. Hermanowicz referred us to neurosurgeon Dr. Marvin Bergsneider at UCLA.

 After his first appointment at UCLA, our father spent five days at the UCLA Medical Center in July 2004 for a procedure called the "lumbar drain", a precursor to shunt surgery. Cerebral spinal fluid (CSF) was drained from his spine and measured hourly. The procedure did not bring noticeable improvement in his balance as was hoped, so it was decided that he abandon shunt surgery.

 But several months later, our father realized that his gait disturbance was advancing rapidly -- and he envisioned he would soon be wheelchair-bound. So, he decided to call Dr. Marvin Bergsneider regarding shunt surgery.
Photo of an MRI machine

 We were all relieved when Dr. Bergsneider came out three hours later to tell us that the surgery to implant the "programmable shunt" (also known as a valve) went well. Dr. Bergsneider had already performed a few thousand such surgeries, although our father's surgery was rare in that he had Hemophilia type B.

 Our father didn't go home in two or three days as most patients do. He remained in the hospital for 11 days because he has a rare form of hemophilia -- a condition associated with rapid bleeding. Hemophilia B is a hereditary bleeding disorder caused by a lack of blood clotting factor IX. So, a blood concentrate -- for blood clotting factor IX -- had to be administered constantly to prevent possible bleeding in his brain. It was crucial that UCLA was able to verify that he had hemophilia, because if they didn't have such verification, they wouldn't have administered the blood concentrate into his wrist area.

 We were advised that improvement in his balance could occur in a few days or longer -- or not at all.

 In our father's case, it took a year -- with four periodic "shunt adjustments" -- to bring maximum results. The results have been -- as our mother has testified -- miraculous.

 He is performing chores around the house and yard. He's shopped, socialized, and traveled throughout California and even has traveled overseas. He was given new physical strength and new confidence, and a new attitude. Friends who hadn't seen him for some time have told him how amazed they are at seeing the complete turnaround in his condition and behavior. As of 2011, he's 80 years old -- and his mind is sharp and he enjoys his avid reading and interacting with people.

 We thank God for blessing us with this life-altering surgery and are so very grateful for Dr. Neal Hermanowicz and Dr. Marvin Bergsneider. Not only patients and their families, but also practicing professionals should hear this story.

 There's more to tell. For instance, Dr. Bergsneider has performed thousands of surgeries and he is very knowledgeable and experienced.

 If you'd like more info on our father's road to healing, feel free to contact me.

--------------------------


Electroconvulsive Therapy for Catatonia in a Boy With Hydrocephalus and an Arachnoid Cyst
  • Our interest in hydrocephalus cases has grown since our father was diagnosed with hydrocephalus in 2003 and was eventually treated successfully. In his case, he received successful shut implantation and has followed up with periodic adjustments of his shunt. The following is a success report from three physicians treating a boy with hydrocephalus.

Electroconvulsive Therapy for Catatonia in a Boy With Hydrocephalus and an Arachnoid Cyst
Volume 43, Issue 1, Pages 73-75 (July 2010) Electroconvulsive Therapy for Catatonia in a Boy With Hydrocephalus and an Arachnoid Cyst

  • Lee E. Wachtel, MD
  • Kristin Baranano, MD, PhD
  • Irving M. Reti, MBBS
  • "We report on the successful use of electroconvulsive therapy in a 13-year-old boy with congenital hydrocephalus, a history of multiple shunt revisions, and a stable prepontine arachnoid cyst, who experienced profound catatonic deterioration. After initial shunt placement at age 20 months, the patient had followed normal motor, cognitive, and social developmental trajectories. Two uncomplicated shunt revisions were performed at ages 10 and 13 years. Three months after the last revision, the patient demonstrated multiple psychomotor signs, culminating in hospital admission for rigidity, posturing, waxy flexibility, stupor, mutism, and cessation of all oral intake. An extensive neurologic workup related to his preexisting conditions produced negative results, and a course of electroconvulsive therapy for acute catatonia was pursued, with outstanding improvement."
-----------------------
Related Links


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Permalink: http://www.thedailydecibel.com/p/success-story-hydrocephalus-hemophilia.html



A Word About Hydrocephalus

Adults can acquire hydrocephalus. My father first had two of the first three triage symptoms in his early 70's. It took about four (4) years from his symptoms to the date he was given a shunt implant. Since then, he's had several adjustments of his shunt.

And one thing was rare about his shunt. My father is a hemophiliac. That means he risks significant losses of blood. That means that if he bleeds, depending upon the bleeding, it could get serious. Up until the very last minute, the medical exams apparently could not positively confirm that he had hemophilia. At the very last minute, the physician team concluded that he did, positively, have Hemophilia B.

And that means that he had to be given infusions during and after the surgery. A concentration was pumped into his wrist area to make sure that he did not suffer significant loss of blood.

Hydrocephalus is not only diagnosed in adults, but also in children. We've had the opportunity to meet people who had hydrocephalus from birth. Treatment and operations are available for those of different ages, including young people.

For instance: CHOC, the Children's Hospital of Orange County, is staffed with neurosurgeons who have performed shunt implants on children. We've had the opportunity to meet with one of them.

For more information, you might want to contact the National Hydrocephalus Foundation. They began in Illinois and now they are a nationwide group. They accept donations, and donations are tax-deductible.

----------------------------



Electroconvulsive Therapy for Catatonia in a Boy With Hydrocephalus and an Arachnoid Cyst
Electroconvulsive Therapy for Catatonia in a Boy With Hydrocephalus and an Arachnoid Cyst
Volume 43, Issue 1, Pages 73-75 (July 2010)
Electroconvulsive Therapy for Catatonia in a Boy With Hydrocephalus and an Arachnoid Cyst
  • Lee E. Wachtel, MD
  • Kristin Baranano, MD, PhD
  • Irving M. Reti, MBBS
"We report on the successful use of electroconvulsive therapy in a 13-year-old boy with congenital hydrocephalus, a history of multiple shunt revisions, and a stable prepontine arachnoid cyst, who experienced profound catatonic deterioration. After initial shunt placement at age 20 months, the patient had followed normal motor, cognitive, and social developmental trajectories. Two uncomplicated shunt revisions were performed at ages 10 and 13 years. Three months after the last revision, the patient demonstrated multiple psychomotor signs, culminating in hospital admission for rigidity, posturing, waxy flexibility, stupor, mutism, and cessation of all oral intake. An extensive neurologic workup related to his preexisting conditions produced negative results, and a course of electroconvulsive therapy for acute catatonia was pursued, with outstanding improvement."
-------------------------
Related Links

Permalink: http://www.thedailydecibel.com/p/success-story-hydrocephalus-hemophilia.html


Normal Pressure Hydrocephalus Success
(NPH Success Story)

(Originally posted circa June 30, 2010)

We've posted previously regarding our father's success story for his NPH (Normal Pressure Hydrocephalus). He is a success story because the NPH shunt surgery -- and subsequent shunt adjustments -- have helped him live a longer, fuller life. Here's his story.


In 2000, our father began a shuffling-of-the-feet and slowness of gait (walking slowly). Through the course of the next couple of years, he also evidenced hand tremors -- a quivering of his joints.

When his symptoms intensified (increased), he agreed to see a neurologist who diagnosed him in November 2000 with Parkinson's Disease. The doctor prescribed the gold standard Parkinson's medication, called Sinemet. The dosage was gradually increased for a year and a half -- but our father never showed signs of improvement. When the neurologist then told him that his Parkinson's was progressing faster than average, our mother sought a second opinion.

In 2003, our father was examined by Dr. Neal Hermanowicz at the University of California, Irvine. Dr. Hermanowicz actually suspected NPH (Normal Pressure Hydrocephalus) and ordered an MRI blood flow (also called an MRI flow test) at one of the few facilities in the area with a specialized machine that could perform that particular test. (See here and here for articles on Magnetic Resonance Imaging.)

The test was inconclusive. Our father displayed two of the triad symptoms of NPH, poor balance and incontinence -- but no noticeable dementia except for normal forgetfulness that a 72-year-old might have. He spent an additional two years of treatments, medication and physical therapy ordered by Dr. Hermanowicz.

We enrolled him in a wonderful Balance and Mobility class directed by Dr. Debra Rose at one of the California State University Centers for Successful Aging. This kept his muscles strong and taught him how to prevent falls. In late 2003 he began a series of weekly six-hour gamma globulin infusions, a treatment akin to chemotherapy, at the UCI Medical Center in Orange, CA for what Dr. Hermanowicz then thought might be an autoimmune disorder. At the conclusion of the twelve-week series there was no improvement in his condition and he was losing hope and becoming depressed. Finally, in 2004 Dr. Hermanowicz referred us to neurosurgeon Dr. Marvin Bergsneider at UCLA.

After his first appointment at UCLA, he spent five days at the UCLA Medical Center in July 2004 for a procedure called the lumbar drain, a precursor to shunt surgery. His doctor was Dr. Paul Vespa, MD, at UCLA. Cerebral spinal fluid (CSF) was drained from his spine and measured hourly. The procedure did not bring noticeable improvement in his balance, as was hoped, so it was decided to postpone shunt surgery. Several months later when he realized that his gait disturbance was advancing rapidly, he envisioned he would soon be wheelchair-bound and decided to call Dr. Bergsneider to request shunt surgery.

In 2005, he finally had an adjustable shunt implanted in his brain at UCLA (see here, here and here and here for information on adjustable shunts.) And the shunt implantation, along with periodic adjustments over a year, brought about significant improvement in our father's gait, joint motion, mood, confidence, and memory.

 We thank God for blessing us with this life-changing surgery and are so very grateful that we were led to Dr. Bergsneider. And we are grateful that there are medical devices that can make our father's life extension possible. There are just a handful of manufacturers of the shunts. Our father was given a programmable shunt. This means that when difficulties arise in the patient, such as urinary incontinence, you can go to the doctor and ask that the shunt be adjusted. The doctor may "tighten" the setting on the shunt, and the patient can be given relief of the symptom, such as urinary incontinence.



A Word About Hydrocephalus
  • Adults can acquire hydrocephalus. Our father first had the symptoms in his 70's. It took about four (4) years from his symptoms to the date he was given a shunt implant. Since then, he's had several adjustments of his shunt.
  • And one thing was rare about his shunt. Our father is a hemophiliac. That means he risks significant losses of blood, making operations more risky. Up until the very last minute, the medical exams apparently could not positively confirm that he had hemophilia. At the very last minute, the physician team concluded that he did, positively, have hemophilia.
  • Thus, he had to be given infusions during and after the surgery. A blood concentration -- Blood Clotting Factor IX -- was pumped into his wrist area to make sure that he did not suffer significant loss of blood.
  • Hydrocephalus is not only diagnosed in adults, but also in children. We've had the opportunity to meet people who had hydrocephalus from birth. Treatment and operations are available for those of different ages, including young people.
  • For instance: CHOC, the Children's Hospital of Orange County, is staffed with neurosurgeons who have performed shunt implants on children. We've had the opportunity to meet with one of them.
Related Links

    --------------------------
    Permalink: http://www.thedailydecibel.com/p/success-story-hydrocephalus-hemophilia.html


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