Friday, May 17, 2013
Monday, May 6, 2013
What is Ehlers-Danlos Syndrome
From the Ehlers Danlos National Foundation
Written by Mark C. Martino
More than 1.5 million people around the world have Ehlers-Danlos syndrome (EDS). Someone with EDS is born at least every half hour, and many won’t know what’s wrong with them until it's too late for them to be helped. EDS occurs in between one in 2,500 and one in 5,000 men and women, of every race and ethnicity. EDS was named less than 100 years ago, and it remains largely unknown. Recent evidence indicates it may be one of the most under-diagnosed disorders, because there is no cure and many doctors consider EDS too rare for anyone to have. They fail to see that diagnosis is important, not just in accurately assessing how many have EDS, but in getting help to their patients for the many problems known to be caused by EDS.
Ehlers-Danlos syndrome is a group of disorders. Each type of EDS is defined as a distinct genetic problem in making or using one of the types of collagen. Collagen is used by the body to provide strength and elasticity to tissue; normal collagen is a strong protein that allows tissue to be stretched, but not past its limit, and then is safely returned to normal. WIth badly built or processed collagen, some of the soft tissue can be pulled further than is safe, causing damage. Collagen is the body's most abundant protein and can be found almost anywhere, in skin, muscles, tendons and ligaments, blood vessels, organs, gums, eyes, and so on.
Vascular Type EDS is particularly destructive because of potential arterial or organ rupture; life expectancy is shortened with a majority of individuals living only into their forties. Arterial or intestinal rupture commonly presents as acute abdominal or flank pain that can be diffuse or localized. Spontaneous arterial rupture is most likely to occur in a person’s twenties or thirties, but can occur at any point in life. Cerebral arterial rupture may present with altered mental status and be mistaken for drug overdose. Mid-size arteries are commonly involved. Arterial, intestinal, or uterine fragility or rupture usually arise in EDS Vascular type, but should be investigated for any EDS type.
There is a very wide range of symptoms because of how widespread in the body collagen is, and it is still unclear why one collagen defect can be expressed so differently in people, even in the same family. EDS can cause systemic weakness and instability throughout the body, resulting in many and unexpected effects. Some can be seen, but not all; EDS is largely invisible. Pain, for instance, can be widespread—collagen makes up the fascia, the tissue sheet that holds everything together and wraps around your entire body—but the pain will not show up in MRIs or X-rays, and will be probably be out-of-proportion to any findings. While EDS is not curable, many symptoms can be prevented or treated as they arise: joint hypermobility (loose or unstable joints that slip or dislocate); early osteoarthritis; tissue fragility, skin that tears and bruises easily, blood vessels and internal organs such as intestines that can rupture easily; eye problems and gum disease; spine problems that may also involve the cerebellum and cerebrospinal fluid pressure.
Collagen is found throughout the body. Ehlers-Danlos syndromes come down to a structural problem. An analogy. If you build a house with bad materials, say weak wood or cheap nails, you know you'll have problems. Some problems are more likely to show up than others, but because the bad materials were used everywhere and aren't necessarily visible, you can be surprised by a problem. So the problems resulting from being built out of a protein that doesn't behave the way it should can be widespread, in a wide range of severities, and maybe even show up in places you wouldn't think are related until it occurs to you that collagen is used there, too.
Each doctor you see is going to look at things differently. Endocrinologists see hormones as the problem, or CFS. Rheumatologists in the US think fibromyalgia's the problem. Surgeons see weaknesses that need surgery. And if none of them are remembering that EDS is the root cause, and maybe their solution won't work in that environment of your body, they may be inappropriately treating you. There are some medical specialists, and more with each generation of doctors, but the best suggestion for the longterm health of someone with EDS is to learn everything you can about EDS and what/how it affects you, so you can educate those doctors and nurses who are willing to admit they don't know everything and are willing to learn.
More than a quarter century of experience, free information and resources from Ehlers-Danlos National Foundation can be found at www.ednf.org or reaching the foundation on Facebook. "So You Think You May Have EDS?" is an introduction to EDS written by EDNF members at http://bit.ly/Olw4L, where you can also discover many links to professional and patient publications and web pages through the "EDS Guides" and "EDS Knowledge" menus on the left side, throughout the web site. EDNF holds a national conference, usually each year.
Written by Mark C. Martino
More than 1.5 million people around the world have Ehlers-Danlos syndrome (EDS). Someone with EDS is born at least every half hour, and many won’t know what’s wrong with them until it's too late for them to be helped. EDS occurs in between one in 2,500 and one in 5,000 men and women, of every race and ethnicity. EDS was named less than 100 years ago, and it remains largely unknown. Recent evidence indicates it may be one of the most under-diagnosed disorders, because there is no cure and many doctors consider EDS too rare for anyone to have. They fail to see that diagnosis is important, not just in accurately assessing how many have EDS, but in getting help to their patients for the many problems known to be caused by EDS.
Ehlers-Danlos syndrome is a group of disorders. Each type of EDS is defined as a distinct genetic problem in making or using one of the types of collagen. Collagen is used by the body to provide strength and elasticity to tissue; normal collagen is a strong protein that allows tissue to be stretched, but not past its limit, and then is safely returned to normal. WIth badly built or processed collagen, some of the soft tissue can be pulled further than is safe, causing damage. Collagen is the body's most abundant protein and can be found almost anywhere, in skin, muscles, tendons and ligaments, blood vessels, organs, gums, eyes, and so on.
Vascular Type EDS is particularly destructive because of potential arterial or organ rupture; life expectancy is shortened with a majority of individuals living only into their forties. Arterial or intestinal rupture commonly presents as acute abdominal or flank pain that can be diffuse or localized. Spontaneous arterial rupture is most likely to occur in a person’s twenties or thirties, but can occur at any point in life. Cerebral arterial rupture may present with altered mental status and be mistaken for drug overdose. Mid-size arteries are commonly involved. Arterial, intestinal, or uterine fragility or rupture usually arise in EDS Vascular type, but should be investigated for any EDS type.
There is a very wide range of symptoms because of how widespread in the body collagen is, and it is still unclear why one collagen defect can be expressed so differently in people, even in the same family. EDS can cause systemic weakness and instability throughout the body, resulting in many and unexpected effects. Some can be seen, but not all; EDS is largely invisible. Pain, for instance, can be widespread—collagen makes up the fascia, the tissue sheet that holds everything together and wraps around your entire body—but the pain will not show up in MRIs or X-rays, and will be probably be out-of-proportion to any findings. While EDS is not curable, many symptoms can be prevented or treated as they arise: joint hypermobility (loose or unstable joints that slip or dislocate); early osteoarthritis; tissue fragility, skin that tears and bruises easily, blood vessels and internal organs such as intestines that can rupture easily; eye problems and gum disease; spine problems that may also involve the cerebellum and cerebrospinal fluid pressure.
Collagen is found throughout the body. Ehlers-Danlos syndromes come down to a structural problem. An analogy. If you build a house with bad materials, say weak wood or cheap nails, you know you'll have problems. Some problems are more likely to show up than others, but because the bad materials were used everywhere and aren't necessarily visible, you can be surprised by a problem. So the problems resulting from being built out of a protein that doesn't behave the way it should can be widespread, in a wide range of severities, and maybe even show up in places you wouldn't think are related until it occurs to you that collagen is used there, too.
Each doctor you see is going to look at things differently. Endocrinologists see hormones as the problem, or CFS. Rheumatologists in the US think fibromyalgia's the problem. Surgeons see weaknesses that need surgery. And if none of them are remembering that EDS is the root cause, and maybe their solution won't work in that environment of your body, they may be inappropriately treating you. There are some medical specialists, and more with each generation of doctors, but the best suggestion for the longterm health of someone with EDS is to learn everything you can about EDS and what/how it affects you, so you can educate those doctors and nurses who are willing to admit they don't know everything and are willing to learn.
More than a quarter century of experience, free information and resources from Ehlers-Danlos National Foundation can be found at www.ednf.org or reaching the foundation on Facebook. "So You Think You May Have EDS?" is an introduction to EDS written by EDNF members at http://bit.ly/Olw4L, where you can also discover many links to professional and patient publications and web pages through the "EDS Guides" and "EDS Knowledge" menus on the left side, throughout the web site. EDNF holds a national conference, usually each year.
Friday, May 3, 2013
Recovery and Setbacks
Please consider donating to my medical expense fund. Surgery is expensive. So is recovery! http://www.youcaring.com/medical-fundraiser/carolyn-s-surgery-fund/36820 Thank you so much to everyone that has donated! This wouldn't be possible without your help!!!
Every recovery has its setbacks, and this one is unfortunately no different. After a few weeks of relative peace, with my condition gradually improving, this week has been a setback.
I've had a change in my POTS symptoms. My blood pressure is no longer low. I don't need Florinef or Midodrine to keep it in the normal range. This is great news! Unfortunately, I'm not feeling any better, because I've developed constant tachycardia. Not just orthostatic tachycardia. Constant tachycardia. Perhaps the related autonomic condition called Inappropriate Sinus Tachycardia. This makes me feel very short of breath all the time. It really feels like I'm running, even when I'm laying down or sitting.
I don't want this condition to prevent me from working hard on my recovery. I still need to exercise on the recumbent bike twice a day. Unfortunately, I'm not able to walk around much right now, because my heart gets out of control.
So, we've booked an appointment with Dr. Grubb's PA, Beverly. We'll be driving out to Toledo for an appointment on May 20th. I've just got to survive until then. This is so freaking exhausting. It's one thing after the next with this recovery, as usual.
Every recovery has its setbacks, and this one is unfortunately no different. After a few weeks of relative peace, with my condition gradually improving, this week has been a setback.
I've had a change in my POTS symptoms. My blood pressure is no longer low. I don't need Florinef or Midodrine to keep it in the normal range. This is great news! Unfortunately, I'm not feeling any better, because I've developed constant tachycardia. Not just orthostatic tachycardia. Constant tachycardia. Perhaps the related autonomic condition called Inappropriate Sinus Tachycardia. This makes me feel very short of breath all the time. It really feels like I'm running, even when I'm laying down or sitting.
I don't want this condition to prevent me from working hard on my recovery. I still need to exercise on the recumbent bike twice a day. Unfortunately, I'm not able to walk around much right now, because my heart gets out of control.
So, we've booked an appointment with Dr. Grubb's PA, Beverly. We'll be driving out to Toledo for an appointment on May 20th. I've just got to survive until then. This is so freaking exhausting. It's one thing after the next with this recovery, as usual.
Thursday, April 25, 2013
Six Weeks Post-Op
Please consider donating to my medical expense fund. Surgery is expensive. So is recovery! http://www.youcaring.com/medical-fundraiser/carolyn-s-surgery-fund/36820 Thank you so much to everyone that has donated! This wouldn't be possible without your help!!!
Things are continuing to improve, just very gradually.
I've had two unexpected improvements from surgery:
1. My digestion and food sensitivities have improved a lot! I seem to tolerate gluten okay, at least small portions. I can also tolerate small amounts of dairy without a problem!
2. My hormone regulation has improved. (I formerly had amenorrhea for 1 1/2 years, but that has resolved.)
I don't know how to explain either of these things really, but I appreciate being a little more normal.
Post-surgical issues are still improving gradually. This includes my POTS symptoms, my muscle tightness, my surgical pain, my muscle weakness, my endurance, etc.
The POTS symptoms are the most challenging, functionally. These include:
-unstable blood pressure
-unstable heart rate
-lightheadedness
-palpitations
-shortness of breath
-tightness in chest
-extreme fatigue
-exercise intolerance
These symptoms affect my life a lot. The mornings and early afternoons are still when my POTS symptoms are worse. Also, if I overdo my exercise or activity level, it may cause an episode later that day, or not until the following day. I don't get any immediate feedback from my body that I am overdoing it. So I have to carefully measure and track the amount of activity I have each day, and the amount of time spent upright.
Surgical pain and muscle tightness are still daily (and nightly) issues, and will probably remain issues for some time...especially as I try to become gradually more active. The pain is worse when I sit up and when I move. I also have trouble with bumpy rides in the car. I don't get out much yet. So I also have to find a balance between increasing activity, and pushing my body too hard. Sometimes, I overdo it, and the pain becomes too much.
I finally got my bone growth stimulator today! It is an electromagnetic device that I wear over my collar. I am to wear it for four hours a day, for several months. I will continue to wear it until I have x-ray confirmation that there is a successful bony fusion.
Thanks for still caring, after all this time.
Things are continuing to improve, just very gradually.
I've had two unexpected improvements from surgery:
1. My digestion and food sensitivities have improved a lot! I seem to tolerate gluten okay, at least small portions. I can also tolerate small amounts of dairy without a problem!
2. My hormone regulation has improved. (I formerly had amenorrhea for 1 1/2 years, but that has resolved.)
I don't know how to explain either of these things really, but I appreciate being a little more normal.
Post-surgical issues are still improving gradually. This includes my POTS symptoms, my muscle tightness, my surgical pain, my muscle weakness, my endurance, etc.
The POTS symptoms are the most challenging, functionally. These include:
-unstable blood pressure
-unstable heart rate
-lightheadedness
-palpitations
-shortness of breath
-tightness in chest
-extreme fatigue
-exercise intolerance
These symptoms affect my life a lot. The mornings and early afternoons are still when my POTS symptoms are worse. Also, if I overdo my exercise or activity level, it may cause an episode later that day, or not until the following day. I don't get any immediate feedback from my body that I am overdoing it. So I have to carefully measure and track the amount of activity I have each day, and the amount of time spent upright.
Surgical pain and muscle tightness are still daily (and nightly) issues, and will probably remain issues for some time...especially as I try to become gradually more active. The pain is worse when I sit up and when I move. I also have trouble with bumpy rides in the car. I don't get out much yet. So I also have to find a balance between increasing activity, and pushing my body too hard. Sometimes, I overdo it, and the pain becomes too much.
I finally got my bone growth stimulator today! It is an electromagnetic device that I wear over my collar. I am to wear it for four hours a day, for several months. I will continue to wear it until I have x-ray confirmation that there is a successful bony fusion.
Thanks for still caring, after all this time.
Monday, April 15, 2013
I have Chiari; why should I be concerned over EDS or Tethered Cord?
Compiled by Robin Armstrong Griffin
When I hear a member in the support groups is diagnosed with Chiari the next question they often ask is why then should I concern over Ehlers-Danlos Syndrome (EDS) or Tethered Cord (TC)? Isn't chiari just caused by a small skull so the brain is forced down?
Chiari is a hind brain herniation of the cerebellar tonsils/lower brain down out of the skull and into the neck. It is caused by a malformation of bones within or near the skull or the pulling from below by Tethered Cord Syndrome, (explained below). In other words, chiari is a symptom caused by a malformation/underlying condition of something else. And a small skull is not the only condition that can be present to cause it, which many doctors feel is the case. So knowing the true underlying condition is key to the proper treatment of your Chiari.
Ehlers-Danlos Syndrome (EDS) is a connective tissue disorder. Connective tissue acts like "glue" in the human body, holding everything together. Collagen, a protein that adds strength to the connective tissues, is not produced correctly in an individual with EDS. The resulting weak connective tissues are too weak to hold the organs, ligaments, joints, etc.of the body in place. Ehlers-Danlos Syndrome is currently separated into 6 major types. Hypermobility, Classic, Vascular, Kyphoscoliosis, Arthrochalasis and Dermatosparaxis. More and more individuals with Chiari are being found to also have EDS and/or Tethered Cord (TC). (The EDS and/or TC being the CAUSE of the hind brain herniation/chiari.) Although at risk for any type, Hypermobility or Classic are seen the most, some the more rare Vascular type.
Individuals with Ehlers-Danlos Syndrome (EDS) are at risk for cranio-spinal instability (CSI), which often results in cranial settling. In cranial settling the tissues in the neck are so weak (due to EDS) that the cervical bones are not held in place. Slumping or collapsing onto itself occurs. These usually leads to abnormal angles/malformations of various bones and this can lead to both anterior and posterior brain stem compression. Many chiari surgeries (Decompression) fail because only the posterior brainstem compression was addressed. And since most have an MRI laying down the true picture isn't seen because the patient wasn't upright for gravity to reflect to true image. Hence the importance of upright MRI's. These malformations force the cerebellar tonsils out of the skull into the spinal column through the small hole at the base of the skull called the foramen magnum, a phenomena called cerebellar herniation, AKA Chiari. When the cerebellar tonsils are out of place, they take up space normally allotted for the cerebral spinal fluid (CSF), the brainstem, and all the arteries and veins leading through this area to the brain. Blockage can be both posterior and anterior. The cerebellar tonsils are the lowest part of the cerebellum, which controls the sensory perception and motor control systems of the body. The brainstem controls the autonomic nervous system, or everything your body does without you having to think about it, heart rate, respiration, blood pressure control, etc. Damage to these systems is very serious. CSI may also allow the Odontoid bone at the front of the neck to angle into the brainstem (Retroflexed Odontoid), causing even more concerns, such as Basilar Invagination or Basilar Impression. (Brain Stem Compression).
Postural Orthostatic Tachycardia Syndrome (POTS) is a type of dysautonomia. It is also commonly seen with individuals who have EDS and EDS with Chiari as a result of brainstem concerns. In addition to this, an individual with EDS and instability is at risk of subluxation (partial dislocation of bones) of the neck or abnormal sliding of the skull in flex/extension, further compromising the brainstem, tonsils and vertebral arteries. Making this life threatening. When you flex and extend your head, your skull should shift up to 1-2mm, with instability your skull can exceed that, (Stephens' is 8mm) further endangering the individual and for some is even life threatening.
As shared from Dr. Clair Francomano at Harvey Institute of Genetics. "The term "Chiari Malformation" is used to describe the situation where the cerebellum, a structure at the base of the brain, is extending through the opening at the base of the skull, which is called the foramen magnum. The extension through the foramen magnum is called "herniation." This can result from cranial settling, which "pushes" the cerebellum through the opening, or tethered cord, which pulls the cerebellum from below. The conventional definition of a Chiari Malformation states the cerebellum tonsils must protrude at least 5mm below the foramen magnum. However, patients with less extreme herniations can also experience significant neurological symptoms involving the brain stem and cerebellar functions.
A wide variety of neurologic symptoms include headaches, neck pain, tinnitus, swallowing difficulties, visual disturbances, a sensation of pressure behind the eyes, autonomic nervous system dysfunction (POTS, neurally mediated hypotension), sleep disturbances, brain fog and memory issues may result from Chiari Malformations.
Another complication of hereditary connective tissue disorders is called occult tethered cord. In this condition, a band of connective tissue called the filum is wrapped around the base of the spinal cord and exerting pressure on the nerves that go to the legs, pelvis, bladder and bowel. This condition may cause numbness in the legs and pelvis area as well as severe incontinence as well. Because of the hyperextensibility of the connective tissue in patients with this disorder, the filum may become stretched out over the years and is not visible on an MRI. This is why it is called "occult", it cannot be seen using current imaging techniques. Diagnosis is usually made based on clinical findings and also the consequences of tethering which may be seen on a cervical and brain MRI, affecting the angles between specific landmarks in the spine and skull (Milhorat TM et al. Association of Chiari Malformation type 1 and tethered cord syndrome: preliminary results of sectioning filum terminale. Surgical Neurology 72: 20-25, 2009). At this time, it is not known why this condition occurs more frequently in patients with hereditary disorders of connective tissue."
The problem is if you have EDS and it isn't known and you have decompression, ESP with laminectomy, it adds instability. This can greatly effect the success of the decompression because you've added more instability and only addressed the posterior issues and not the anterior issues often seen with cranial settling. Often times those with EDS & Chiari need fusion done at the same time or known to be needed at a later time or tethered cord release done first or soon after. Many also chose to wear cervical collars to help with the instability symptoms and pain. The decompression alone often treats only a part of the issue, not all and even if CSF flow is restored posterior the pain and neuropathy issues still remain due to cranio-instability and continued brainstem compression in other areas or tethered cord still pulling the tonsils from below. Thank you for reading!
Resources: Dr. Clair Francomano, Harvey Institute of Genetics and www.MedicalZebras.com
When I hear a member in the support groups is diagnosed with Chiari the next question they often ask is why then should I concern over Ehlers-Danlos Syndrome (EDS) or Tethered Cord (TC)? Isn't chiari just caused by a small skull so the brain is forced down?
Chiari is a hind brain herniation of the cerebellar tonsils/lower brain down out of the skull and into the neck. It is caused by a malformation of bones within or near the skull or the pulling from below by Tethered Cord Syndrome, (explained below). In other words, chiari is a symptom caused by a malformation/underlying condition of something else. And a small skull is not the only condition that can be present to cause it, which many doctors feel is the case. So knowing the true underlying condition is key to the proper treatment of your Chiari.
Ehlers-Danlos Syndrome (EDS) is a connective tissue disorder. Connective tissue acts like "glue" in the human body, holding everything together. Collagen, a protein that adds strength to the connective tissues, is not produced correctly in an individual with EDS. The resulting weak connective tissues are too weak to hold the organs, ligaments, joints, etc.of the body in place. Ehlers-Danlos Syndrome is currently separated into 6 major types. Hypermobility, Classic, Vascular, Kyphoscoliosis, Arthrochalasis and Dermatosparaxis. More and more individuals with Chiari are being found to also have EDS and/or Tethered Cord (TC). (The EDS and/or TC being the CAUSE of the hind brain herniation/chiari.) Although at risk for any type, Hypermobility or Classic are seen the most, some the more rare Vascular type.
Individuals with Ehlers-Danlos Syndrome (EDS) are at risk for cranio-spinal instability (CSI), which often results in cranial settling. In cranial settling the tissues in the neck are so weak (due to EDS) that the cervical bones are not held in place. Slumping or collapsing onto itself occurs. These usually leads to abnormal angles/malformations of various bones and this can lead to both anterior and posterior brain stem compression. Many chiari surgeries (Decompression) fail because only the posterior brainstem compression was addressed. And since most have an MRI laying down the true picture isn't seen because the patient wasn't upright for gravity to reflect to true image. Hence the importance of upright MRI's. These malformations force the cerebellar tonsils out of the skull into the spinal column through the small hole at the base of the skull called the foramen magnum, a phenomena called cerebellar herniation, AKA Chiari. When the cerebellar tonsils are out of place, they take up space normally allotted for the cerebral spinal fluid (CSF), the brainstem, and all the arteries and veins leading through this area to the brain. Blockage can be both posterior and anterior. The cerebellar tonsils are the lowest part of the cerebellum, which controls the sensory perception and motor control systems of the body. The brainstem controls the autonomic nervous system, or everything your body does without you having to think about it, heart rate, respiration, blood pressure control, etc. Damage to these systems is very serious. CSI may also allow the Odontoid bone at the front of the neck to angle into the brainstem (Retroflexed Odontoid), causing even more concerns, such as Basilar Invagination or Basilar Impression. (Brain Stem Compression).
Postural Orthostatic Tachycardia Syndrome (POTS) is a type of dysautonomia. It is also commonly seen with individuals who have EDS and EDS with Chiari as a result of brainstem concerns. In addition to this, an individual with EDS and instability is at risk of subluxation (partial dislocation of bones) of the neck or abnormal sliding of the skull in flex/extension, further compromising the brainstem, tonsils and vertebral arteries. Making this life threatening. When you flex and extend your head, your skull should shift up to 1-2mm, with instability your skull can exceed that, (Stephens' is 8mm) further endangering the individual and for some is even life threatening.
As shared from Dr. Clair Francomano at Harvey Institute of Genetics. "The term "Chiari Malformation" is used to describe the situation where the cerebellum, a structure at the base of the brain, is extending through the opening at the base of the skull, which is called the foramen magnum. The extension through the foramen magnum is called "herniation." This can result from cranial settling, which "pushes" the cerebellum through the opening, or tethered cord, which pulls the cerebellum from below. The conventional definition of a Chiari Malformation states the cerebellum tonsils must protrude at least 5mm below the foramen magnum. However, patients with less extreme herniations can also experience significant neurological symptoms involving the brain stem and cerebellar functions.
A wide variety of neurologic symptoms include headaches, neck pain, tinnitus, swallowing difficulties, visual disturbances, a sensation of pressure behind the eyes, autonomic nervous system dysfunction (POTS, neurally mediated hypotension), sleep disturbances, brain fog and memory issues may result from Chiari Malformations.
Another complication of hereditary connective tissue disorders is called occult tethered cord. In this condition, a band of connective tissue called the filum is wrapped around the base of the spinal cord and exerting pressure on the nerves that go to the legs, pelvis, bladder and bowel. This condition may cause numbness in the legs and pelvis area as well as severe incontinence as well. Because of the hyperextensibility of the connective tissue in patients with this disorder, the filum may become stretched out over the years and is not visible on an MRI. This is why it is called "occult", it cannot be seen using current imaging techniques. Diagnosis is usually made based on clinical findings and also the consequences of tethering which may be seen on a cervical and brain MRI, affecting the angles between specific landmarks in the spine and skull (Milhorat TM et al. Association of Chiari Malformation type 1 and tethered cord syndrome: preliminary results of sectioning filum terminale. Surgical Neurology 72: 20-25, 2009). At this time, it is not known why this condition occurs more frequently in patients with hereditary disorders of connective tissue."
The problem is if you have EDS and it isn't known and you have decompression, ESP with laminectomy, it adds instability. This can greatly effect the success of the decompression because you've added more instability and only addressed the posterior issues and not the anterior issues often seen with cranial settling. Often times those with EDS & Chiari need fusion done at the same time or known to be needed at a later time or tethered cord release done first or soon after. Many also chose to wear cervical collars to help with the instability symptoms and pain. The decompression alone often treats only a part of the issue, not all and even if CSF flow is restored posterior the pain and neuropathy issues still remain due to cranio-instability and continued brainstem compression in other areas or tethered cord still pulling the tonsils from below. Thank you for reading!
Resources: Dr. Clair Francomano, Harvey Institute of Genetics and www.MedicalZebras.com
Sunday, April 14, 2013
1 Month Post-Op
Please consider donating to my medical expense fund. Surgery is expensive. So is recovery! http://www.youcaring.com/medical-fundraiser/carolyn-s-surgery-fund/36820 Thank you so much to everyone that has donated! This wouldn't be possible without your help!!!
____________________________________________________________
This has been a really interesting time. Last Monday, we had to let go of our sweet dog, Jango. He lived a very happy life, but was in a lot of pain at the end. I am glad that we could at least relieve his pain. He was a wonderful spirit, full of joy, and we miss him dearly.
As part of the healing process from this loss, we decided to get a new puppy. He is a delight, and helps to fill the emptiness that we are feeling. His name is Dexter. He is an 8-week old Coton, and he loves to cuddle!
I got my stitches out on Thursday. What a relief! The stitches were very embedded after a month in my head, so it took quite a while to work them out, but it feels so much better now.
My dysautonomia is my biggest problem at this point. My body is still very unstable and unpredictable. My heart rate jumps up, my blood pressure drops, or I become suddenly short of breath or have heart palpitations. I also experience reduced consciousness at times...particularly in the early hours of the afternoon. I am on several medications for this, but they don't always help.
I am exercising daily to try to improve the symptoms of dysautonomia. I have to exercise sitting down, so the recumbent bike is perfect. But I have to be very careful. If I overdo it one day, the next day may be a very sick day with low blood pressure and reduced consciousness. So, I have to be very careful about exercising the same amount every day, and barely ever increasing. At this point, I can do 11 minutes, twice a day. This will probably take months to improve, with a lot of hard work and persistence.
The pain is still being managed with Tylenol. I'm taking 1500 mg of acetaminophen a day, only 3 tablets (when 8 is the daily maximum). This keeps most pain manageable. I've still had a few migraines that require separate migraine medicine.
The muscle spasms are still the biggest cause of pain. I still cannot tolerate any muscle relaxants. So, I'm using heating pads, topical creams (China-gel), and massage to relieve the pain. The pain still feels very similar to migraine pain, but in a slightly different location than a normal migraine. I can usually tell them apart now.
My eyes were injured during my 16 hours under anesthesia. I got large corneal abrasions across each eye. The intense pain only lasted a few days. But I still get burning, dry eyes many times a day. I'm not ready to wear my contact lenses yet. I believe they are healing, but not healed yet.
I also developed large areas of numbness on each thigh from surgical positioning. The numbness was complete at first--no sensation for temperature, sharp/dull, or pressure, besides very deep pressure. Gradually, the feeling returned, but these areas became hyper-sensitive to touch and quite painful. These areas are still healing. The pain is decreasing.
Ready for the good news? My digestion seems to be improved since surgery. I am having less food sensitivities and less digestive disturbance and pain. This is an unexpected improvement!
My swallowing is still improved, but no longer perfect. I still have aerophagia at times with large amounts of water. It is usually mild, currently.
So, given the complexities of this recovery, here is what daily life is like for me right now:
I need to sleep about 11 hours each night. That's about 11:00 pm to 10:00 am. I wake up once each night. As long as I can get back to sleep, I'm fine. If something disturbs my sleep, my dysautonomia symptoms will flare the whole day.
My mornings are a bit better recently. If I take my morning meds right away, including my salt and extra fluids, my blood pressure is usually pretty good, and my heart rate is mildly elevated. I need to spend the first few hours of the day mostly horizontally.
After lunch, I seem to have a flare of autonomic symptoms. My heart starts racing, my blood pressure drops, I become short of breath, and I experience reduced consciousness, or even temporary loss of consciousness. We manage this as well as we can with medication, salt, and elevating my legs above my heart.
On a good day, these symptoms will improve by dinner time. I can sometimes eat dinner at the table, something I could never do before surgery. Sitting up in a chair has always worsened my symptoms. So, I think this is a really good sign for my long-term recovery. I also get my exercise on the recumbent bike at this time of day. I still sometimes get shortness of breath or an elevated heart rate, but I usually stay fully conscious. I can spend some time upright during this window. I still have to rest frequently.
I still get frustrated by my symptoms, especially on bad days, where I feel like I'm not getting better at all, or when I spend a lot of time really out of it. But overall, I am satisfied with my progress so far. I just have to keep reminding myself that recovery takes a very long time, and this is just the beginning. I know there are good times ahead for me. A new puppy helps! I really look forward to being able to move back in with my husband, even though my mom and step-dad are taking such good care of me!
Thanks to everyone for caring and supporting me.
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This has been a really interesting time. Last Monday, we had to let go of our sweet dog, Jango. He lived a very happy life, but was in a lot of pain at the end. I am glad that we could at least relieve his pain. He was a wonderful spirit, full of joy, and we miss him dearly.
As part of the healing process from this loss, we decided to get a new puppy. He is a delight, and helps to fill the emptiness that we are feeling. His name is Dexter. He is an 8-week old Coton, and he loves to cuddle!
I got my stitches out on Thursday. What a relief! The stitches were very embedded after a month in my head, so it took quite a while to work them out, but it feels so much better now.
My dysautonomia is my biggest problem at this point. My body is still very unstable and unpredictable. My heart rate jumps up, my blood pressure drops, or I become suddenly short of breath or have heart palpitations. I also experience reduced consciousness at times...particularly in the early hours of the afternoon. I am on several medications for this, but they don't always help.
I am exercising daily to try to improve the symptoms of dysautonomia. I have to exercise sitting down, so the recumbent bike is perfect. But I have to be very careful. If I overdo it one day, the next day may be a very sick day with low blood pressure and reduced consciousness. So, I have to be very careful about exercising the same amount every day, and barely ever increasing. At this point, I can do 11 minutes, twice a day. This will probably take months to improve, with a lot of hard work and persistence.
The pain is still being managed with Tylenol. I'm taking 1500 mg of acetaminophen a day, only 3 tablets (when 8 is the daily maximum). This keeps most pain manageable. I've still had a few migraines that require separate migraine medicine.
The muscle spasms are still the biggest cause of pain. I still cannot tolerate any muscle relaxants. So, I'm using heating pads, topical creams (China-gel), and massage to relieve the pain. The pain still feels very similar to migraine pain, but in a slightly different location than a normal migraine. I can usually tell them apart now.
My eyes were injured during my 16 hours under anesthesia. I got large corneal abrasions across each eye. The intense pain only lasted a few days. But I still get burning, dry eyes many times a day. I'm not ready to wear my contact lenses yet. I believe they are healing, but not healed yet.
I also developed large areas of numbness on each thigh from surgical positioning. The numbness was complete at first--no sensation for temperature, sharp/dull, or pressure, besides very deep pressure. Gradually, the feeling returned, but these areas became hyper-sensitive to touch and quite painful. These areas are still healing. The pain is decreasing.
Ready for the good news? My digestion seems to be improved since surgery. I am having less food sensitivities and less digestive disturbance and pain. This is an unexpected improvement!
My swallowing is still improved, but no longer perfect. I still have aerophagia at times with large amounts of water. It is usually mild, currently.
So, given the complexities of this recovery, here is what daily life is like for me right now:
I need to sleep about 11 hours each night. That's about 11:00 pm to 10:00 am. I wake up once each night. As long as I can get back to sleep, I'm fine. If something disturbs my sleep, my dysautonomia symptoms will flare the whole day.
My mornings are a bit better recently. If I take my morning meds right away, including my salt and extra fluids, my blood pressure is usually pretty good, and my heart rate is mildly elevated. I need to spend the first few hours of the day mostly horizontally.
After lunch, I seem to have a flare of autonomic symptoms. My heart starts racing, my blood pressure drops, I become short of breath, and I experience reduced consciousness, or even temporary loss of consciousness. We manage this as well as we can with medication, salt, and elevating my legs above my heart.
On a good day, these symptoms will improve by dinner time. I can sometimes eat dinner at the table, something I could never do before surgery. Sitting up in a chair has always worsened my symptoms. So, I think this is a really good sign for my long-term recovery. I also get my exercise on the recumbent bike at this time of day. I still sometimes get shortness of breath or an elevated heart rate, but I usually stay fully conscious. I can spend some time upright during this window. I still have to rest frequently.
I still get frustrated by my symptoms, especially on bad days, where I feel like I'm not getting better at all, or when I spend a lot of time really out of it. But overall, I am satisfied with my progress so far. I just have to keep reminding myself that recovery takes a very long time, and this is just the beginning. I know there are good times ahead for me. A new puppy helps! I really look forward to being able to move back in with my husband, even though my mom and step-dad are taking such good care of me!
Thanks to everyone for caring and supporting me.
Wednesday, April 10, 2013
Pics from surgery
Please consider donating to my medical expense fund. Surgery is expensive. So is recovery! http://www.youcaring.com/medical-fundraiser/carolyn-s-surgery-fund/36820 Thank you so much to everyone that has donated! This wouldn't be possible without your help!!!
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Here's some photos of hospital and home recovery. As always, I can't seem to control the order of the photos. So it's more of a collage. Recovery is slow, but I do see some tiny improvements overall. I'll update more tomorrow, after my stitches finally come out!!!
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Here's some photos of hospital and home recovery. As always, I can't seem to control the order of the photos. So it's more of a collage. Recovery is slow, but I do see some tiny improvements overall. I'll update more tomorrow, after my stitches finally come out!!!
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