My AS experience and Self-Management Plan

DIAGNOSIS AND DENIAL
In 2001, I was diagnosed with Ankylosing Spondylitis (AS). I had graduated from the University of Western Ontario only 2 years prior with 2 Bachelor degrees and was in the early years of my nursing career at Markham Stouffville Hospital. The world was my oyster and I was in denial--who had time to slow down? Not me. What was this thing...Ankylosing Spondylitis? Could it be what was causing my lower back pain since Grade 9? Nah. I feel fine so I must be fine. I'm just a bit tired...all the time. It must be because of these 12 hour shifts.

As you can tell, health care professionals may not always make the best patients. We're used to being the ones who help others...the ones who have it all under control. I was obviously (but unknowingly) suffering from the Invincibility Complex--the same complex we are convinced has taken over the world's teenage population.

MY EXPERIENCE
This has been a difficult disease to cope with, particularly given my young age, Type A personality, and my persistent Invincibility Complex. I never thought that in my 20's I would ever need assistance with dressing, toileting, managing stairs, or even getting out of bed--but I did. Not all the time. Because of the stiffness associated with AS, I would need assistance (thanks Sis) for 2-3 days a week during an inflammatory flare-up and then I would not need assistance for a few weeks to a few months afterwards. The stiffness can be so severe that I would not be able to stand erect, and yet I could not even bend forward. Sometimes the stoop was mild where I would look like a young Hunchback of Notre Dame, and other times I was literally bent at a 90 degree angle staring at my beautiful toes for several hours up to a full day until the inflammation subsided.

Although my stiffness tends to be worse upon awakening due to the prolonged level of inactivity and immobility, the evenings have also become a painful time of day for me. Fatigue is a common complaint of patients with AS, occurring in approximately 65% of patients--for me it was no exception. Because I try to be active, fatigue is my largest frustration because it limits my activity and wrecks havoc on my motivation. I consider my fatigue to be moderately severe. Increased levels of fatigue are associated with increased pain, stiffness, and decreased functional capacity.

One reason that makes it difficult to cope with is that no one can tell you have a disease. Most people with AS look healthy and may appear to function relatively well. No one can see the persistent pain you are experiencing throughout the day, and no one sees how stubborn your body can be in the mornings and evenings when all you want to do is get out of the car or straighten up from rinsing your mouth out in the sink. People may notice you changing positions in your seat frequently, or may hear your back crack so often they ask you to leave the room (it's happened to me). They may think you are lazy because you are frequently fatigued. Or they may think you are a loner because you are depressed. It's hard to admit that you have a disease that forces you to change the way you are used to doing things, and it's hard to predict how others will relate to you after that. Although I now have an administrative position in the healthcare industry, I will admit that my primary ongoing concern is that my employer (regardless of the company) may assume I cannot perform my role or I would be a health liability to the organization. It is a fear that most people with chronic illnesses face.

THE MEDS
My Rheumatologist originally prescribed Voltaren to reduce my lower back stiffness and ease the constant dull pervasive pain nagging at my sacroiliac joints. The medication was effective for several weeks, but eventually the pain intensity returned. I was then prescribed Piroxicam which offered no relief. Indomethacin eventually provided effective pain relief by reducing my joint inflammation; however, the ache once again returned. Even the recommended maximum dosage could not provide the relief I needed to participate in even the most basic activities of daily living.

My Rheumatologist decided to prescribe Bextra to replace my Indomethacin, but the international drug warnings that started with Vioxx and trickled down to other Cox-2 inhibitors, ensured that Bextra would not remain part of my treatment plan. I was fortunate enough to be approved for Enbrel--a biologic therapy--which I started November 2004. Enbrel is a type of protein called a tumor necrosis factor (TNF) blocker that blocks the action of TNF, a substance your the immune system makes. It requires the person to take an injection below the skin twice a week. The company has just released a new dosage requiring only one injection each week. The medication became effective three months after initiating the treatment (which is anticipated by the pharmaceutical manufacturers of the drug) and I once again found relief. I was able to participate in recreational and daily living activites again without much stiffness. My only side effect was a common site reaction (redness, the emergence of small bumps, and itchiness). It was a small price to pay for regaining my independence and functionality again. I described it as "feeling like a normal person...like everybody else."

Unfortunately, at present, I do not have health benefits resulting in my inability to afford the Enbrel since February, 2006. The medication costs approximately $1600 each month. Without this medication the inflammation will return related to AS being an auto-immune disease. In other words the immune system loses its ability to recognize some tissue or system within the body as "self" and targets and attacks it as if it were foreign. Inflammation results in stiffness and pain at the affected area, and eventually reduced functional ability. Until I am able to afford Enbrel, I have returned to taking my remaining capsules of Indomethacin.

ALTERNATIVE TREATMENT
I purchased an EMPI Transcutaneous Electrical Muscle Stimulation (TENS) device for slightly under $800. I was fortunate enough to have employee health benefits that fully reimbursed the cost of the device. I had researched TENS machines as a non-medicinal treatment alternative for AS, and research was favourable for pain management. To achieve effective pain relief, I need to use a very high intensity (level 40-50) of electrical stimulation on the machine. Although this level can be intolerable for many, it is effective for me.
I have found massage and physiotherapy to be very effective in managing my pain and immobility. I went for a fabulous Swedish massage at a spa on April 2nd. The therapist was knowledgable about AS, and tailored the massage to provide me with the most therapeutic benefit. I am convincing myself to have a massage every 2 weeks--after all, who doesn't need pampering?

SELF MANAGEMENT
Exercise and stretching appear to be the only 2 non-pharmaceutical therapies that you can do independently to manage AS. I have always been physically active, and my physiotherapist has praised my active lifestyle for the current state of my mobility. I regularly attend the gym for weight training and aerobics classes, and I run several times a week for cardiorespiratory benefits.

Participating in the Arthritis Self Management Program has helped me understand my disease a lot more and how to manage the symptoms. Each week we had to create an action plan: during my first week I decided to start stretching. Experiencing a loss of flexibility made me feel older than I want to feel--it was a good motivator. I have always hated stretching. With respect to a physical activity, if it did not make me sweat, then I did not consider it beneficial. Watching my flexibility slowly slip away and the resulting disability was finally enough of a reason for me to engage in stretching. I even initiated an appointment with an Arthritis Society physiotherapist, Mary Aisen, at the St. Paul's L'Amoreaux Centre to educate me on stretching techniques. She took the time to thoroughly assess my health history, physical mobility and flexibility, and to educate me on stretching. I was very impressed by her comprehensiveness and patience, and the fact that she had previously been in my shoes as a Joints in Motion Training Team member.

My goal is to continue stretching every day, and to start participating in yoga and pilates classes at the gym. I plan to incorporate mountain biking as a way of cross training and strengthening different muscle groups that would benefit my running. This blog will allow me to communicate my training progress to you. I hope you have enjoyed reading about my personal experience with AS.

Arthritis Self-Management Program

I completed my first Arthritis Self Management Program (ASMP) on March 23rd, 2006. The program was hosted in Ajax at the Kinsman Club by Beth Jacobs and Joan Lambert. Although both suffer from different debilitating forms of arthritis that have significantly altered their lives, they continue to find the time and energy to volunteer with the Arthritis Society, providing education and support to enable us to be proactive managers of our disease.
The program ran for six weeks in two-hour sessions and covered information on nutrition, exercise, problem solving, developing action plans, the pain cycle, and getting the most out of time-limited physician visits, coping mechanisms for dealing with various symptoms (such as fatigue, depression, etc), non-traditional treatments, and medication managment.
For the cost of only $35 (tax deductible), I received an open forum to communicate with others in similar situtations, the ability to network with others suffering with arthritis, education and support from knowledgeable volunteers, printed resources about my disease and how to better utilize the Arthritis Society, and a research-based text on arthritis management.

If you have been diagnosed with ankylosing spondylitis, there are steps that you can take at home to help reduce pain and stiffness and allow you to continue daily activities. These steps include:

• Educating yourself, such as through The Arthritis Self-Management Program. Learn all you can about your condition and know what complications to watch for. This will help you control your symptoms and stay more active.
• Reducing pain by taking pain relievers such as nonsteroidal anti-inflammatory drugs, or using heat to decrease your pain and stiffness. Warm showers or baths or sleeping under a warm electric blanket may reduce stiffness.
• Exercising and stretching regularly. This reduces pain and stiffness and helps maintain fitness and mobility of the spine, chest, and joints. Your doctor may recommend physical therapy to get you started on an exercise program.
• Swimming as part of your exercise program helps to maintain chest expansion and movement of the spine without jarring the spine. Breast stroke is especially good for chest expansion.
• You should avoid contact sports, since joint fusion may make your spine more likely to fracture as the disease progresses, but your doctor may approve of other activities such as golf and tennis. Check with your doctor before you add any new activity.
• Maintaining proper posture and chest expansion. Good posture is important because it can help prevent abnormal bending of the spine. Maintaining chest expansion will help prevent problems such as lung infection (pneumonia). Deep breathing exercises can improve or maintain lung capacity. It's a good idea to lie on your stomach a few times each day to keep your spine and hips extended. For sleeping, choose a firm mattress and a small pillow that supports your neck.
• Using assistive devices such as canes or walkers. Your local chapter of the Arthritis Society, your physical therapist, or a medical supply company may be able to help you find assistive devices in your area. If your neck is becoming stiff, your doctor may recommend that you wear a soft neck brace when you ride in the car, to prevent injury in case of an accident.
• Avoiding smoking, to prevent serious breathing difficulties and lung scarring. Lung damage from smoking, combined with decreased chest expansion and the lung infections that sometimes go with ankylosing spondylitis, can seriously limit your ability to breathe freely.
• Seeing your doctor and/or rheumatologist at least once each year to check on your condition and watch for any complications. Catching complications early and treating them can prevent further problems.
• Having regular eye exams by an ophthalmologist to check for inflammation of the colored part of the eye (iritis).
• Joining a support group. For more information in Canada, call The Arthritis Society's Information Line at 1-800-321-1433, or visit their Web site at http://www.arthritis.ca

About the Joints in Motion Training Team, 2006

Joints in Motion is the international athletic training program for the Arthritis Society. The program has been designed to guide people of all ages and fitness levels through an extensive training program to complete a marathon. On behalf of the Arthritis Society, participants raise funds and awareness to fight arthritis.
I became interested in joining the Ontario division of the Canadian Team several years ago after completing my first half-marathon. However, competing demands of my time practically eliminated the time I would need to dedicate to properly training for a full marathon. Realizing that I am nearing the end of earning my Master in Health Administration from the University of Toronto, I began planning my return to running. I knew that I wanted to complete a full marathon this year, so my plans started to focus on selecting one. It was during my participation in the Arthritis Self Management Program that I made the commitment to run for a cause.

I will be racing 42K in Honolulu, Hawaii on December 10th, 2006.

What is Ankylosing Spondylitis

WHAT IS ANKYLOSING SPONDYLITIS

Ankylosing spondylitis (AS; also known as Bechterew's disease; Bechterew syndrome; Marie Strümpell disease / Marie Struempell disease / Spondyloarthritis) is a chronic, painful, progressive rheumatic disease that causes chronic inflammatory arthritis of the spine and sacroiliac (SI) joints--resulting in eventual fusion--and can cause inflammation of the eyes, lungs, and heart valves. The person exhibits intermittent episodes of back pain that occur throughout life. AS does not necessarily starts bilaterally. Research clearly indicates that the iliac, not the sacral side and the dorsocaudal part of the SI joints are affected early on. Complete fusion results in a complete rigidity of the spine, a condition known as bamboo spine

TESTS:
Tests may include:

• HLA-B27 antigen test is positive.
• A spine X-ray or pelvis X-ray shows characteristic findings. Routine lumbar spine and sacroiliac joint X-rays are only recommended for HLAB27-positive patients.
• ESR may or may not be elevated. Elevation may be mild.
• CBC may show mild anemia.
• Magnetic resonance imaging has proved useful for visualising inflammation in the SI joints in adults and children.

This is a challenging diagnosis to make correctly. Diagnosing a person with AS prior to the development of signs seen on Xray or MRI will continue to be very difficult. Genetic testing can improve diagnosis by confirming specificity.

GENETICS:

Ankylosing spondylitis is relatively rare. It is considered a multifactorial disorder, or one that is the result of both genetic and environmental factors interacting. Two genes have been identified that confer susceptibility to AS, both of which are forms of an HLA gene on chromosome 6 (detected by a blood test).

Some HLA types have been implicated in various autoimmune diseases, meaning diseases in which the immune system attacks the body's own cells and tissues. Researchers have determined, however, that a particular version of the HLA-B gene (called HLA-B27) increases the risk of developing this disorder. The association of HLA B-27 and AS has been clearly established. Ninety-five percent of individuals with AS are B-27 positive, and since AS appears to be a dominant trait, the presence of at least one B-27 allele (a form of the gene) confers a greatly increased chance of developing symptoms. While this population risk may seem relatively high, it is important to realize that only about 9% of the population carries the B-27 allele. Of these individuals who are B-27 positive, only 2–8% will develop AS. It is not known how HLA-B27 increases the risk of developing ankylosing spondylitis. HLA-B27 is a Class 1 HLA molecule found in 95 per cent of patients with AS, compared with a prevalence of 9 per cent in the normal population in the UK.

What is clear, is that the prevalence of AS parallels that of HLA-B27 and AS occurs with a greater prevalence in ethnic groups with a high population prevalence of B27, for example, the Haida Indians of North America.

The pathogenetic role of B27 in AS is uncertain, but it may be associated with a number of genes that confer susceptibility to the disease. The HLA-B gene provides instructions for making a protein that plays an important role in the immune system. HLA-B is part of a family of genes called the human leukocyte antigen (HLA) complex. The HLA complex helps the immune system distinguish the body's own proteins from proteins made by foreign invaders (such as viruses and bacteria). The HLA-B gene has many different normal variations, allowing each person's immune system to react to a wide range of foreign invaders. Other genes are believed to affect the chances of developing ankylosing spondylitis and influence the progression of the disorder. Some of these genes likely play a role in the immune system, while others may have different functions. Researchers are working to identify these genes and clarify their role in ankylosing spondylitis.

The cause of AS is not known and there is no cure. Delayed diagnosis is common because symptoms are often attributed to more common back problems.

Ankylosing spondylitis is 2-3 times more common in males than in females, which is why I was stunned when I was diagnosed with AS in 2001. Male gender and detection of the HLAB27 antigen were strongly associated with a diagnosis of ankylosing spondylitis (p<0.001).>

Other environmental and genetic factors most certainly contribute to development of the disease. This becomes more evident when considering that B-27 positive individuals with an affected first-degree relative have a significantly higher chance of developing AS than a B-27 positive individual with no family history. In families with multiple affected members, studies estimate that no more than half of AS recurrence is explained by HLA type. Additionally, there are several B-27 subtypes that have been studied; some confer susceptibility and some do not. Importantly, about 5% of people with AS are B-27 negative. Other environmental and/or genetic factors must certainly be associated with disease in these individuals. Another HLA type—B-60—has also been shown to confer susceptibility, although the association appears to be much weaker and is not seen in all studies. Certain infections are suspected as being necessary for triggering AS in some individuals. In the future, additional susceptibility genes and environmental factors can be expected to be identified.

DEMOGRAPHICS

Ankylosing spondylitis affects about 0.5 percent of people of Western European descent. In the US: AS affects 0.1-0.2% of the US population. Internationally: AS affects 0.1-1.0% of the world population.

Native North Americans, Alaskan Eskimos, and Norwegian Lapps all have relatively high levels of B-27 and AS. Low levels of B-27 and AS occur among individuals of most types of African ancestry, Australian aborigenes, and Native South Americans.

SIGNS AND SYMPTOMS

The signs of AS vary, but a typical case involves slow or progressive onset of lower back pain and stiffness (typically in the morning) over weeks or months, rather than hours or days. Other symptoms include fatigue, feeling feverish and experiencing night sweats, early onset weight loss, and feeling better after exercise and worse after rest. The earliest symptoms of this disorder result from inflammation of the joints between the base of the spine (the sacrum) and the hipbones (the ilia). These joints are called sacroiliac joints, and inflammation in this region is known as sacroiliitis. The disorder also causes inflammation of the joints between vertebrae, which is called spondylitis.

Ankylosing spondylitis can involve other peripheral joints and other body organs as well, including the shoulders, hips, and, less often, joints in the limbs, resulting in severe joint and back stiffness, loss of motion and deformity as life progresses. Over time, this disorder can affect the joints between the spine and ribs, restricting movement of the chest and making it difficult to breathe. Breathing exercises can help to maintain lung capacity.

Crohn's disease and ulcerative colitis are found in 10-15 per cent of patients with AS.

COMPLICATIONS:

• heart valve disease, typically aortic valve stenosis
• aortitis
• eye inflammation (uveitis/iritis)
• pulmonary fibrosis
• kidney failure (rare complication)
• osteoporosis can cause spinal fractures

Mild or early ankylosing spondylitis

• Ankylosing spondylitis usually starts with dull pain in the low back and back stiffness. Some people with ankylosing spondylitis have "flares" of increased pain and stiffness that may last for several weeks before decreasing again.
• Affected bones of the low back, middle back, hips, or neck may become painful, stiff, and limited in motion. Pain tends to increase slowly over a period of weeks or months, and it is often hard to point to exactly where the pain is. Stiffness is usually worse in the morning. Physical activity often helps decrease pain and stiffness.
• A feeling of tiredness is common as the disease progresses. This tiredness comes from the body fighting the inflammatory process that is part of ankylosing spondylitis, and from ongoing stiffness and pain.
• The colored part of the eye (iris) may become inflamed. This inflammation, called iritis, occurs in about 25% to 30% of people with ankylosing spondylitis. Symptoms of iritis include redness and pain in the eye and sensitivity to light.

Severe or advanced ankylosing spondylitis
If, over time, the inflammation continues, it will lead to scarring and permanent damage.

• Scarring in the spine causes the joints of the spine to grow together (fuse, or "ankylose"). As the bones fuse, back pain will gradually go away, but the spine will remain very stiff and unable to bend. The fused spine is more likely to fracture if injured, especially the neck (cervical spine).
• Changes in the spine can cause problems with balance, safety, and mobility. The upper spine can curve forward until eventually the person has a hard time looking straight ahead. In addition, as the spine loses its natural curves it becomes hard to balance for standing and walking, especially if the hips are also affected.
• Breathing can become difficult as the upper body curves forward and the chest wall stiffens. Severe ankylosing spondylitis can also cause scarring of the lungs (pulmonary fibrosis) and an increased risk of lung infection. This can cause even greater problems in smokers because their lungs are already more prone to lung infection and scarring.
• Scarring in the eye can lead to permanent visual impairment and glaucoma.
• In rare cases, the heart muscle can become scarred and the heart valves may become inflamed. The heart may be unable to pump properly (heart failure). The main artery leading from the heart (aorta) can also be affected by becoming inflamed and enlarged near where it leaves the heart.

Note that the stiffening of the chest can feel like the discomfort or "heaviness" of a heart attack. Ankylosing spondylitis can also cause the heart to function less efficiently. Finally, lung infection is common in advanced ankylosing spondylitis

TREATMENT:

The optimal treatment of ankylosing spondylitis involves medications that reduce inflammation or suppress immunity, physical therapy and exercise to maintain mobility and flexibility.

MEDICATIONS:
Nonsteroidal anti-inflammatory medications (NSAIDs) such as aspirin and indomethacin are used to reduce inflammation and pain associated with the condition. They allow patients to exercise, which improves posture and breathing.

Corticosteroid therapy or medications to suppress the immune system may be prescribed to control various symptoms. Some health care professionals use cytotoxic drugs (drugs that block cell growth) in people who do not respond well to corticosteroids or who are dependent on high doses of corticosteroids.

TNF-inhibitors, such as Enbrel, have been shown to improve the symptoms of ankylosing spondylitis.

SURGERY
Surgery is done if pain or joint damage is severe.

LIFESTYLE CHANGES
Exercises can help improve posture and breathing. Lying flat on the back at night can help maintain normal posture. Use devices to help with activities of daily living.

PROGNOSIS:
The course of the disease is unpredictable. The disease activity in AS usually fluctuates; remissions and relapses may occur at any stage. The majority of patients with mild disease are able to maintain a reasonable functional and employment capacity unless the hips are severely involved, but a significant minority of patients with severe disease develop progressive functional impairment. One study identified seven variables that correlated with disease severity and hence poorer prognosis:

* Hip arthritis
* Dactylitis (a sausage-shaped swelling of the fingers and toes which can be painful)
* Poor NSAID response
* High ESR (>30mm/h)
* Reduced range of lumbar spine movement
* Peripheral joint disease
* Disease onset age <16 years.

Only 1 per cent of patients enter long-term remission. Prognosis is adversely affected by smoking, recurrent extra-articular complications and peripheral joint disease. Recent research suggests that AS patients are also at increased risk of cardiovascular complications because of their chronic, low-grade spinal inflammation.

References:

Amor B, Santos RS, Nahal R et al. Predictive factors for the long- term outcome of spondyloarthropathies. J Rheumatol 1994;21:1883-7

Chee, M.M. "MUSCULOSKELETAL: Putting the spotlight on ankylosing spondylitis." The Practitioner (Nov 27, 2006): 19.

Kennedy LG, Edmunds L, Calin A. The natural history of ankylosing spondylitis. Does it burn out? J Rheumatol 1993;20(4):688-92

Khan MA. HLA-B27 and its subtypes in world populations. Curr Opin Rheumatol 1995;7:263-9

McLauchlan GJ, Gardner DL. Sacral and iliac articular cartilage thickness and cellularity: relationship to subchondral bone end-plate thickness and cancellous bone density. Rheumatology (Oxford) 2002;41:375–80.

Muche B, Bollow M, Francois RJ, Sieper J, Hamm B, Braun J. Anatomic structures involved in early- and late-stage sacroiliitis in spondylarthritis: a detailed analysis by contrast-enhanced magnetic resonance imaging. Arthritis Rheum 2003;48:1374–84.

Ostensen M, Ostensen H. Ankylosing spondylitis - the female aspect. Rheumatol 1998;25(1):120-4

Zochling J, Braun J. Management and treatment of ankylosing spondylitis. Curr Opin Rheumatol 2005;17(4):418-25