Roseanne Cash: Comes Out About Having Chiari Malformation

The daughter of the late and great man in black, Johnny Cash was diagnosed a while ago with the debilitating disease of Chiari Malformation. Roseanne Carter Cash has lived with the symptoms for most of her adult life. She recently discussed having the disease due in part to canceling her upcoming Black Cadillac Tour.

When Roseanne came out to the public about having brain surgery, she had to explain to fans as well as the media that she has Chiari -- a benign brain condition, and that she plans to make a full recovery.

I've gathered some information that explains the symptoms and lifestyle effects that Roseanne has had to deal with.

Arnold- Chiari (Kee-AR-ee) Malformation can lie dormant for many years until physical symptoms begin to appear.

Arnold Chiari Malformation or ACM, is a rare and sometimes deadly neurological disorder that effects less than 200,000 people in the United States.

ACM is a congenital malformation, which means a person can be born with it. It is a serious condition which debilitates the body in such a way that it can eventually lead to paralyzing pain an neurological discomfort.

ACM was first discovered in the 1890's by German pathologist, Hans Chiari and his colleague Dr. Julius Arnold. Together, they categorized the disease into four different categories by order of severity --with IV or 4, being the most advanced.

The symptoms of ACM do not always appear at birth or in early childhood. Sometimes adults show signs of a neurological disorder but the ACM will go undiagnosed. Therefore, many people do not know they have this disease until it is in its latter stages. The grueling surgeries and day-to-day struggles often leave ACM patients and their families devastated, both physically and financially.

But what exactly is Arnold- Chiari Malformation? Technically, there is a compartment in the back of the skull that holds the cerebellar tonsils that extend to the bottom of the Skull. These tonsils are normally round but in a case of ACM they become elongated and then protrude down the spinal canal, where they can become herniated. This blocks the flow of spinal fluid to the brain -- the cranial nerves and the brain stem. This results in the lower portion of the brain becoming compressed or in some cases, crushed. This in turn affects brain function in certain areas of the brain. Usually once this has happened the disease is in it's advanced stages.

What are the different types of Arnold- Chiari Malformation? Type I which is what Roseanne Cash has been diagnosed with. Type 1 goes unnoticed until certain neurological problems arise in the person's teens and twenties. The base of the skull and upper area of the spine at this time are not forming properly. Type II is the most common type of ACM. In this type, the back of the brain literally shifts down through the bottom of the skull. Infants who are born with hydrocephalus may also be associated with ACM. Hydrocephalus is a birth defect where there is an overproduction of cerebral spinal fluid inside of the brain. Fluid increases and can cause death if untreated. A shunt is typically placed in the brain and drained throughout the body. Type III is more advanced than type II. The back of the brain actually protrudes out of an opening near the back of the human skull. Type IV is where the entire back of the brain does not develop normally at all -- resulting in severe mental dysfunction.

What are the symptoms of ACM? Typically, symptoms do not arise until people are in their teens or early adult years. In very small infants, slow movements and difficulty in swallowing are the onset of symptoms. Most patients complain of dizziness, vertigo or a spinning sensation, severe head and neck pain, headaches that are made worse by coughing or sneezing, muscle weakness and balance problems. Vision and hearing problems can also occur in severe cases. Difficulty swallowing is also a stage four advancement into the disease and can only be relieved by surgery.

How is ACM diagnosed? If symptoms persist and your doctor suspects you may have ACM -- further testing is recommended. This can be done by having X-Rays. An X-Ray machine takes a picture of the internal tissues bones and organs. A CT scan may also be done and be able to help your doctor get a better look at what is going on inside your neurological system. An MRI, is an advanced diagnostic procedure that uses magnets, radio frequencies and a computer to produce fine detailed images of your brain and spinal cord area. All of the above diagnostic tests are essential in making a definitive diagnosis.

Is there any kind of treatment for ACM? Some form of surgery is typically the only option for patients who want to help relieve their symptoms. Relieving the pressure on the brain will most likely result in successful but temporary treatment, although surgery is very risky. A neurosurgeon specializing in ACM is a wise choice.

The only other option is constant pain and surgery has a good chance of providing some form of relief to the patient. Palliative care may also be instituted to make the patient feel comfortable and pain-free.

With proper physician care and follow up, persons living with ACM can live the rest of their life as pain free as possible. Finding a good ACM support group is essential in continuing education and disease maintenance.

ACM isn't always death sentence, if you feel you are misdiagnosed or know someone who may have the disease, don't hesitate to contact a specialist who has dealt with the disease. The sooner you are treated, the better your long-term prognosis.

Scientists and specialists are working around the clock to find cutting edge treatment options and hoping to one day find a cure.

ACM doesn't have to be debilitating. Learning ways to cope and deal with the disability and pain is the first step in wellness. I am proud that Roseanne has decided to go public and share her illness with others -- this raises ACM awareness and helps her fans better understand how she copes with the disease.