Celiac Disease: All In The Family

Aliases: celiac disease, coeliac disease, celiac sprue, non-tropical sprue, and gluten sensitive enteropathy

Being gluten free is in fashion, but while it’s optional for some, it’s obligatory for the many suffering from celiac disease (CD). CD is an autoimmune disorder that’s estimated to affect 1 out of every 100 people2. The actual count is unknown, because a staggering 85-90% of people with the condition are undiagnosed1. That means that in the US alone, 2.5 million people with CD are unaware that they have the disease2.

Although public awareness of CD has certainly increased recently (to the point where people are sick of hearing the phrase “gluten free”), improvement in diagnosis alone doesn’t explain the huge jump in case numbers. In fact, it doesn’t come close. Even controlling for better detection, the number of people with CD is growing rapidly; prevalence rates are doubling every 20 years. Environmental factors, like greater wheat consumption, are thought to have played a role in this astronomical leap in prevalence, but the importance and extent of their contribution remains unclear1.

While the environmental triggers and even the symptoms of this complex disease are difficult to disentangle (see below), its genetics have proved relatively straightforward. CD is the most common genetically-based food intolerance in the world, and it hinges on two genes. People with the HLA-DQ2 or DQ8 genes are at a greater risk for CD, these genes contribute about 40% of the disease risk. Its strong genetic links keep CD in the family; having an immediate family member with the disease gives you a 1 out of 10 chance of having it1.

celiac disease
Gluten free is the way to be.

Cause: Like most autoimmune diseases, CD results from a combination of genetic predisposition and environmental factors. Once the disease is active, it is expressed when the ill consume gluten, a protein found in several grains, including wheat, rye, and barley2. Several environmental factors increase the likelihood of developing CD, including early infections, infant gut microbiota, and the amount and timing of the first exposure to gluten1.

Consequence: When gluten is digested by someone with CD, their body misidentifies it as a pathogen and sets off an immune response that attacks the lining of the small intestine, damaging the villi. This causes inflammation and impairs nutrient absorption, which can lead to an array of about 300 possible symptoms, including abdominal bloating and pain, weight loss, fatigue, migraines, irritability, and depression2. Although it is a disease of the gut, gastrointestinal symptoms can be minor or even absent, while symptoms outside the digestive tract can be major1. And to heap injury upon injury, CD is also associated with a range of other conditions, including anemia, osteoporosis, infertility, epilepsy, Type I diabetes, multiple sclerosis, and intestinal cancers2. The older a person is when he or she is diagnosed, the greater his or her chance of developing another autoimmune disorder; one study found that people diagnosed with CD after age 20 had a 34% chance of developing another autoimmune disorder2.

Cure: Because of its ridiculous number of symptoms, definitively diagnosing CD is a tricky business. Typically, once the disease is suspected a blood test is used to look for celiac-specific antibodies and, if positive, damage to the intestinal lining is then assessed with a biopsy. The only treatment for celiac disease is avoidance: a strict gluten free diet1. Some ties bind for life.

References

  1. Guandalini, S. & A. Assiri. 2014. Celiac disease: a review. JAMA Pediatrics, 168: 272-278.
  1. What is Celiac Disease? Celiac Disease Foundation. Web. 9 June 2015.

Image source: Creative Commons, http://www.marksdailyapple.com/gluten-free-fad/#axzz3d6qrTOfc

Hospice Care: Where the Heart Is

Home is more than where you live. It is an idea that ties together the emotional, physical, and practical aspects of our lives. It is where we feel most centered, and often it is where we feel at our strongest and most loved (if those are, in fact, separate things). At times of great stress, many seek the solace of home. In fact, most people want to spend their final days at home, and doctors and loved ones agree2. Though the advent of hospitals shifted sickness and death out of the home, modern medicine is turning the tide. Increasingly, medicine allows us to anticipate death, with better screening and diagnosis, and gives the opportunity to plan for its arrival.

Hospice has revolutionized end-of-life care. Hospice is referred to as compassionate care, and it lives up to that moniker. Its mission is to provide services that maintain the comfort and control the symptoms of patients with terminal illnesses. This can include everything from medical to pastoral services for the patient, and bereavement services for family members4. Typically, hospice is provided where the patient lives (their ‘home’ may be a nursing home, etc.), with a family member acting as the primary care provider3. Hospice care allows people to meet death on their own terms.

hospice care
There’s no place like it.

History and current extent

Hospice has grown rapidly since its founding by volunteers in 1974. There are now more than 5,500 hospice programs, covering a wide range in size and scope. In 2012, between 1.5 and 1.6 million patients received hospice care, and approximately 1.1 million deaths occurred while in hospice3. The program’s expansion has been greatly assisted by the Medicare hospice benefit, created in 1982, which allows Medicare to pay for hospice care4; Medicare paid for 84% of hospice care received in 20123.

There are four main types of hospice care: most (97%) hospice patients receive routine home care, where a hospice worker periodically visits the home, 2.7% receive continuous home care, 0.5% receive general inpatient care (care in an inpatient facility), and 0.3% receive inpatient respite care, which is short-term care at an inpatient facility to provide respite for the caregiver3. The average length of stay for hospice patients has been steadily increasing over the past few decades, while the median stay length has decreased. An increase in the number of very long stays is driving this trend; the longest stays are so much longer than a typical stay that they skew the average stay length, making the median stay length a better estimate3,4. In 2012, the median stay was about 3 weeks (18.7 days), meaning that 50% of patients stayed for a shorter period and 50% stayed longer, while the average stay was 69.1 days3.

Contrasts in care

Hospice care is not uniform. The drivers behind the patterns in hospice care remain unclear, nevertheless there are major differences in stay length between care providers and clear trends in patient demographics. Between 2000 and 2007, the average length of the top 10% of stays increased from 115 to 183 days, but this increase was not equal across all program types. Patients receiving care from for-profit hospice programs are more likely to stay longer than those in non-profit or state-run programs. The majority of hospice programs, 63%, are for-profit3, and the percentage of hospice patients in for-profit programs is rising; it has increased from 15.8% in 2000 to 31.2% in 2007. Patients in for-profit programs are less likely to stay for a week or less and more likely to receive care for a year or more4.

Hospice patients typically fit the same general profile as nursing home patients: elderly and white. In 2012, 83.4% of hospice patients were at least 65 years old, and more than one third were at least 85 years old. Young adult and pediatric patients made up less than 1% of the patient population. The vast majority of hospice patients are white; in 2012, less than 20% of hospice patients were minorities3. Cancer is the most common illness affecting hospice patients (36.9% of patients have cancer), followed by unspecified illnesses (14.2%), dementia (12.8%), heart disease (11.2%), and lung disease (8.2%)3.

Compassionate care

Hospice care is hard work. It is emotionally and physically demanding to care for the dying, and hospice workers are asked to tend to many patients at once. The average hospice nurse manager cares for 11 patients and the average hospice social worker cares for 273. Facing death every day can wear on caregivers; a recent study evaluated the dreams of hospice workers, and found that they were coping with work stress and fear of their own mortality1.

Despite the challenges, people not only work for hospice, they volunteer in large numbers. Volunteerism has been ingrained in hospice from its beginning, and has continued to be a critical part of the program. Medicare requires that at least 5% of patient care be done by volunteers, and in 2012, 5.4% of clinical staff hours were provided by volunteers. That is a staggering amount of work, shouldered by people giving their time out of kindness. It is humbling to consider the lengths these volunteers go to to ease the suffering of others, to help bring them home.

References

1. Hess, SA, S Knox, CE Hill, T Byers, & P Spangler. 2014. Exploring the dreams of hospice workers. American Journal of Hospice and Palliative Medicine, 31(4):374-379.

2. Kassam, A, J Skiadaresis, S Alexander, & J Wolfe. 2014. Parent and clinician preferences for location of end-of-life care: home, hospital or freestanding hospice? Pediatric Blood Cancer, 61:859-864.

3. NHPCO’s facts and figures: Hospice care in America, 2013 edition. National Hospice and Palliative Care Organization. Web. 27 June 2014. http://www.nhpco.org/sites/default/files/public/Statistics_Research/2013_Facts_Figures.pdf

4. Sengupta, M, E Park-Lee, R Valverde, C Caffrey, & A Jones. 2014. Trends in length of hospice care from 1996 to 2007 and the factors associated with length of hospice care in 2007: findings from the national home and hospice care surveys. American Journal of Hospice and Palliative Care, 31(4):356-364.

Image source: Creative Commons: http://www.agencyq.com/blog/q-corporate/a-new-home-for-q/attachment/white-picket-fence-home/