Chiari Malformation

It is my hope that the information below will help others gain awareness of Chiari Malformation. Although it is a rare condition more people are being diagnosed as a result of better technology. I was diagnosed several years ago, but as of late the condition has progressed and I am in the process of seeking a second opinion before proceeding with surgery.  Learning to live with this condition as well as with a spinal cord injury has made daily life a challenge.

What are Chiari Malformations

Chiari malformation is a condition in which the cerebellum and part of the brain stem push through the lower part of the skull. Normally the cerebellum, part of the brain that controls balance, and parts of the brain stem sit in a designated space at the base of the skull above the foramen magnum.

Chiari malformations may occur when the space where the cerebellum and part of the brain stem is smaller than normal, thus causing them to protrude downward into the upper spinal canal. As a result the flow of cerebrospinal fluid (CSF) may become blocked. Cerebrospinal fluid is the clear liquid that surrounds and cushions the brain and spinal cord. When the pathways are blocked is can cause a buildup of fluid on the brain called hydrocephalus or in the spinal cord called syringomyelia.

These malformations are uncommon, yet with improved modern imaging techniques and comprehensive exams physicians are beginning to make more accurate diagnoses.
Chiari Malformation type 1 is the most common type of the four possible types of the condition. Type 1 is also the only type that can be acquired. All other types are congenital.

Patients with type 1 normally do not have symptoms until late childhood or adulthood. This is because the malformation does not develop until the skull and brain have developed. On the other hand, patients with types 2-4 are often diagnosed in utero (during pregnancy), during birth, or during the first stages of infancy.

Symptoms

Patients that have Chiari malformation type I may complain of neck pain, headaches, balance problems, muscle weakness, vision problems, numbness or abnormal feelings in the legs and/or arms, ringing of the ears (tinnitus),hearing loss, dizziness, nausea, difficulty swallowing, facial pain, insomnia, and depression. Fine motor skills and hand coordination may also be affected.
Symptoms may vary from person to person and some may have no symptoms at all. The difference in symptoms depends on the buildup of CSF and the change in pressure on the tissues and nerves. For example, adolescent and adults may have no symptoms initially then later in life may develop signs of the disorder. Infants may show symptoms of any type of malformations. Their symptoms maybe difficulty swallowing, irritability during feedings, gagging or vomiting, excessive drooling, arm weakness, breathing problems, developmental delay and inability to gain weight.

If Chiari malformation type 1 if untreated may also result in the development of fluid filled cavities in the brain (hydrocephalus) or spinal cord (syrinx) which may eventually lead to syringomyelia. This can lead to irreversible damage to the brain or spinal cord.

How common are Chiari malformations?

In the past it has been estimated the condition happens one in every 1,000 to 2,00 births. However, the development of better technology and the increase use of diagnostic imaging has shown that the malformations may be more common. The estimates of actual cases is difficult as people born with the condition do not display symptoms at all or may not display them until they are in adolescence or adulthood.

Diagnosis

The most common method of diagnosis is the use of an MRI (Magnetic Resonance Imaging) in conjunction with the patient history, and a neurological examination. Sometimes a special MRI scan called CINE studies are needed to make the diagnosis. Additional imaging techniques may be used. Early diagnosis of Chiari malformation type 1 can lead to treatment before the formation of syrinx.

Treatment

The treatment of Chiari type 1 varies on several factors such as the severity of symptoms, whether or not syrinx (CSF fluid filled areas on the spinal cord) exists. For example, if a patient has no symptoms or neurological findings, the doctor ill often just observe the patient and schedule periodic visits to manage the condition. If the patient displays mild symptoms that are manageable, the physician may prescribe medications to help reduce the pain.

If the patient has symptoms that progress in severity, such as a decline in neurological function and/or they develop an enlarging syrinx, surgery may become necessary.

If surgery is recommended, it typically begins by making an incision is the middle of the back of the skull , and extends to about midway down the back of the neck. All the muscles are separated from the base of the skull to the fist cervical vertebra.

Next, a surgical removal of a portion of the cranium called a craniectomy is performed. This is done to enlarge the opening at the base of the skull and create additional space next to the brain to allow better flow of CSF. The neurosurgeon uses a specialized surgical instrument to carefully shave a small piece of bone about the size of a silver dollar. Next, the first cervical vertebra is removed. This called a laminectomy. At times, removal of additional cervical vertebra may be necessary.

Conclusion

Most patients with Chiari malformation type 1 often have no symptoms. The patient only finds out they have the condition after it is found during an evaluation for other non-related conditions.

Once a Chiari malformation is diagnosed, it is important to get an evaluation by a neurologist who then can refer you to a neurosurgeon if further treatment is needed. Early diagnosis and treatment of this condition is critical as surgical intervention for symptomatic. Chiari malformation type 1 is most often successful when done early on. Many patients experience significant reduction of their symptoms after surgery and are able to return to daily life without activity restrictions.

Learning-to-deal

Each day is different. Some days are great in that the pain is down usually around a 3 to 5 on the pain scale. Other days no matter what I do the pain remains high at about 5 to 9. There is no reason for it as my medications remain the same. Why? This is the one of the questions that plague me. I am learning to deal with so many things, but the one big thing is letting go of the why and learning to live in the now. This is beyond difficult.

Some days it feels like God is gone and has left me. He hates me. He not only took the use of my legs, but has allowed me to continue to hurt at levels that are unbearable at times. I have become isolated from those around me as their lives cannot accommodate someone like me who is slow moving and unreliable because the pain. My only communication with “friends” is often via things like Facebook or Instagram. It is a lonely place to be. The reality is people don’t just want to sit around watching TV, playing a game or talking with someone who is hurting because we are not fun to be around. Besides their lives don’t have time for people like us who are in constant pain and disabled.

Learning to let all of that go and see the “bright side”  is not easy. Realizing that God is still there and He really does love us seems like a dream. For me, deep down I know that God is real and He loves me. The enemy loves to sneak in and make me doubt everything. So often questions like, can my family still love me the same? How can I still be whole? How can I take care of my family as I can barely take care of myself? Each day seems to bring new questions and doubt. It sucks. It doesn’t help when the people you love are constantly telling you things like- Pick up you feet, bend your knees, don’t bend over and pick things up. As for me, I already know my body doesn’t work right I really don’t need to be reminded constantly.

So how do I let is all go? I don’t know at this point. Each day all I can do is pray constantly and do my best to move forward. With God’s help it is my hope that I will find a purpose for my life and I can learn to let all of this go accepting what is happening to me.

 

Days that aren’t so good, can be little blessings

As I continued to hope that the pain would get better, I realized that wasn’t going to happen. Because of the pain I had to miss yet another outing. I did not get a chance to go out trick-or-treating with my 11 yr old daughter and husband, but instead I stayed home with our 15-year-old and his friend who were handing out candy.

My pain remained fairly high at about a 7 most of the evening regardless of how much medication I was able to take. Of course I have to be very careful, as the potential to over-dose is high so there are times that I have to just deal with the pain.  Even so, my heart ached to have to miss yet another outing, but at the same time I was thankful. Because I hurt I got to stay home and hear the laughter joy that was coming out of the other room.

Our son and his friend were playing video games, eating pizza, joking, and laughing. Sounds that I wish we heard more often. You see, our son often is isolated in his room programming his computer, playing computer games, or listening to/playing  music. He is the typical teenager. He does come out and join us for family game night and movies occasionally.

But in the midst of pain, God has blessed me yet again.  Instead of just sitting around watching TV and hurting,  I was able to be a part of this special moment of laughter and fun.  As they both ate pizza and came in for seconds they were pushing each other and making jokes. Listening to them bought smiles to my face. Rare moments like these are cherished even when physical pain is makes it difficult.  Actually listening to the joy coming out of the other room helped reduce my pain. I am so thankful for these little moments that God keeps giving me. Continue reading

Dance, Dance , Dance

Having lost the feeling in my legs and living in pain made the thought of dancing seem impossible. My husband and I attended a Halloween party over the weekend. I was just  excited and happy to be there as last year I was in the hospital recovering from one of the multiple surgeries.

As I sat next to my husband watching people come into the party dresses as various characters, I turned to find an old childhood friend. She recognized me immediately, we talked and  made me smile and laugh. She vowed to get me up to dance. My fear that I was going to be unable to do so set in. How could I dance being that I walk with a walker and I can only feel my left leg? At least my pain level was fairly low at a 5.

I stood next to my husband near the seating area and moved a bit to the beat of the music. Oh, how I missed dancing and moving. After all, I was a dancer most of my life performing in various places over the years. It has been one of my the biggest losses. Loosing the use of my legs, not being able to feel or move is one of the hardest things I have had to deal with. The thought of not being able to stand cheek to cheek with my husband and move slowly to the music help bring my spirit down.

Standing and moving felt amazing. It was a bit scary, but invigorating at the same time. My pain level remained low at about a 5. My friend came over and signaled, “Let’s go.” So I followed her with my walker out onto the dance floor. I began to move to the music. It felt great. I had to keep looking at my feet as without looking at them my brain has no idea where my feet are in space and I will fall over. I held on to my walker and began to move my feet and body. It was an amazing feeling. I never thought I was going to be able to do this again. But yet again, God blessed me with a special gift.

For the brief moment in time I felt great. My pain was low, my legs held me up and moved to the beat. I smiled and felt alive. I will cherish this moment as I don’t get many of them. So thankful for my blessing. Lord, thank you for all you give me. I still struggle from day to day as my pain fluctuates. I only hope that as I continue to go to physical therapy that my legs with get stronger and work better. I hope that I will move from a walker to a cane. That I may  regain some mobility and self-worth.  I hope that my pain level will drop so that I can enjoy more of my life. For now all I know for sure is that God continues to be with me and watch over me. He continues to help heal me. For this I am blessed.

 

Another day

So thankful to be alive another day. I only wish I didn’t have to live in pain all the time. Even with pain meds I can’t control the pain. Living with a spinal cord injury is not what I planned for. Nobody plans for this. Who would want to wake up everyday with a pain level of 5+? Who would want to find it difficult to even stand or walk? I realize that there are those who can’t even walk. I am thankful that I at least am able to walk with a walker and I am working on improving my ability by going to physical therapy three times a week.

Even so the questions keep coming. When is it my turn to wake up not hurting? Will I ever get a break from the pain? Will my legs ever get better? Will I ever get feeling back in my right leg? Will my proprioception ever get better? When do I get to run and play with my grandchildren or will I ever get to kick a ball with them? These are just a few of the questions that run through my head daily and sometimes several times a day.

Some days it is hard to deal with it at all. Just getting up and taking a shower takes all I have sometimes. First, just getting undressed is extremely painful. Taking my shirt off feels like the skin on my back is being sliced open by sharp pieces of foil. Next, the water that should be refreshing feels like spiked fireballs hitting my skin.Then drying off feels like being my skin being dried off by sandpaper. So you can only imagine how hard it is for me to put clothes on. I put on my Lidocaine pain patches first which is a not an easy task. I have to balance myself and use both hands to apply the patches to my back in the areas where the burning and pain come from.  Often it takes several tries to get them on correctly, on days when my husband is home he helps me. This task alone causes extra pain, but eventually the patches help to reduce the pain some by numbing the area some.

I hate having to take so many medications. I take Baclofen 20 mg three times a day to help reduce the spasms in my back and legs, Gabapectin/Lyrica ( changing  meds slowly), OxyContin 30 mg every 8 hrs., and Oxycodone 15 mg every 4 hrs as needed for break-through pain.  I also have to take Senna 2 tabs every morning along with Miralax and Metamucil to help keep myself from getting constipated as taking all the narcotics slows my bowls done. Even so, my pain is often not controlled well. This makes life  difficult and causes me not to be able to do many of the things I like to do.

Getting out with family and friends is very difficult. Most people don’t understand the amount of discomfort I am in nor do they understand how to be around me. Yes, I need help with some things but not everything. People tend to want to help by treating like I am incapable of doing anything. For example,  I can walk with a walker slowly, but I don’t need to be held like I am going to fall over. I can also walk up and down stairs fairly well and don’t have be held up. But when I need to stop and rest, I need to stop and rest not be treated like I am falling over or I am about to die. What people forget is that doing as much as I can for myself is important. It helps me recover and feel a sense of accomplishment especially since I have lost the ability to do so many things. Feeling like I have value is important to recovery. I only wish those around me understood that.

My hope is that people who read this will understand that if they or someone they know is going through anything similar that they are not alone and that are others that understand what they are going through. Whether it be pain or loss of function or both, we still have value. We still want to be treated like we count not like we are fragile and incapable of contributing. Yes, we may need more time to complete a task, but give us the chance and if we need help we will ask for it.

 

 

Pain Is it real or just in my head

As each day passes my physical pain never seems to go away. The question I ask myself is, Is my pain real or just in my head? I ask myself this because I have been told over and over again that, your brain controls and you have the ability to control your brain. Well, when you have a physical condition that creates pain how does that constitute that your pain is all in your head?

I have been to so many doctors over the past few years that deal with my physical conditions of spinal cord injury, arachnoid cysts in my thoracic spine, and nerve damage related to the 8 surgeries to decompress my spinal cord. Some of my doctors recognize that my pain is real and others like to pretend that my pain is going to go away. Regardless I am still left with the limitations that my pain creates.

Some days I am able to move around with less pain than others. Often I am asked, why is your pain better? Why aren’t you hurting as bad? Why are you moving better? The response of ” I don’t know” is never acceptable.  Instead I often hear, Well, you’re the only one that tell us why Or What did you do different? When I explain that I have done nothing different, that I have taken all the same medications in the same order as I do everyday it is still not acceptable. The frustration is beyond what one person should have to deal with. Why don’t others get that if I could figure it out I would duplicate it so that everyday would be better?

All I can do is keep moving forward.  Continue to deal with my pain with the use of medications, natural remedies,  and daily exercises to help strengthen my brain’ s communication with my legs and back. Right now my brain doesn’t fully realize I even have legs. With the loss of proprioception in both legs, my brain forgets things like I am standing, which causes me to fall if attempt to do “normal things” like try to stand and take a picture or walk without look down at my feet or legs.

My brain is also hyper over sending signals which make my nerves overactive. This causes my back to feel like my skin is being stretched and it is on fire. Even the slightest touch feels intense and painful. Putting on clothing hurts to the point of giving me the chills at times. It also causes my right leg that is almost completely numb and heavy to feel like there are a million spiders with sharp needles on theirs legs to keep poking me repeatedly.  I am prescribed Gabapectin 700 mg three times a day for my neuropathic pain in conjunction with Oxycodone 15 mg every 3.5 hours to control pain. None of this works well. I find that the Oxy takes about an hour to start working than only works at its full capacity for about two hours then decreases quickly. This leaves me in pain most of the day. It is often hard to get motivated to do much or to really want to be around people.

So what is left? Self help and prayer. It is easy for me to understand how depression can set in for quickly. This is where the power of prayer and hope come in. Continuing to have hope that I will get better and that new medications and/or treatments will be discovered to help me an others like me are what keep me moving forward. God has promised that if we have faith and ask Him to heal us that it will be done. ” And the prayer offered in faith will make the sick person well; the Lord will raise them up. If they have sinned, the will be forgiven” James 5:15. Having hope is what helps me continue each day even though I know I will wake up in pain and end my day in pain. My prayers continue to be answered as each day I improve even if it is only a small bit. I went from being mostly in a wheelchair to walking with a walker in just two months after my 8th surgery. I refuse to give up as I know God will keep His promise.

Still Recovering 2months After Surgery

It is now just over two months after my 8th surgery in less than a year decompress my spinal cord from Arachnoid Cysts which have been compressing spinal cord. I continue to have almost no feeling in my right leg other than it feeling heavy. I have lost the ability to feel hot and cold on my right leg from the waist down with one exception of a small space on my inner calf where I feel touch and slight temperature. My left leg is numb in the thigh area but mostly has feeling. I continue to have proprioception loss in both legs. What all this means is that standing and walking are extremely difficult even with assistive devices such as a Swedish Knee Cage, ankle braces, and walkers. The most frustrating part is that after all of this the amount I pain I have is unexplainable at times. I live at a pain level of 4 to 5 most days with it increasing several times during the day to about an 8 sometimes more.

Proprioception loss means I have difficulty knowing where my legs are in space which affects my ability to know the position of my legs. This affects my ability of motion and equilibrium. For example, if a person is blindfolded they can tell if the leg/ arm is moved up or down or to the right or left. They also know if their toe is up or down. With the loss of proprioception I cannot do this. Through therapy proprioception can be improved. I am currently working on this yet again. Some of the things I do is wear weights around my ankles when I walk at home. This does two things. One it helps build the muscles in my legs and hips which became very weak after my surgeries. Two it gives sensory input into my pain by on my leg and hip as well as giving it a different feeling. Another thing I do is the spell out the alphabet in the air with each foot several times a day. This also helps make connections between my legs/feet and brain. I also do many other exercises at home and at therapy to improve my muscle strength and coordination.

Along with the proprioception problems I continue to have lots of pain. My pain now appears to be more nerve pain with less spasms. My nerve pain feels like burning. My back often feels like it is on fire. So often when I walk, each step sends signals of intense burning running up the back of my leg, through my buttocks and up my back mainly on my left side with it radiating to the right side at times. There are times that even while sitting do nothing or sleeping that the intense burning sensation happens. By the end of the day I am exhausted because of the pain. I am currently taking several medications to help combat the nerve pain as well as the pain caused by my spasms. Currently I take Neurotin 700 mg three times a day for the nerve  pain as well as Oxycodone 15 mg every 3 1/2 hrs.  with acetaminophen 325 mg. Yet, the pain continues to be intense and out of control at times. I have already tried Lyrica, Cymbalta and amitriptyline with no real impact. The side effects of the medications were awful. The worst one was Cymbalta.

I am not sure what to do next or if there is anything out there that can help. It is frustrating and at times debilitating. All I can do is pray and ask God to protect and continue to heal me. I have gotten through each day by God’s grace even the days when I have a huge set back such as a fall.  God has prevented me from having any new injuries and kept me from having to have another surgery. His love is what keeps me going each day. I am thankful He is with me. May He continue to love me and help me. My faith is sometimes weak and I want to give up, but He doesn’t let me as His presence is felt and I get a renewed push forward.