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The Star Online: Lifestyle: Health

The case of the mysterious illness that causes too much pain

Posted: 07 May 2014 09:00 AM PDT

There's hope for teens afflicted by the mysterious and excruciating Amplified Musculoskeletal Pain Syndrome.

One moment, Albiera Camaiora – Albi – is a stellar free-diver, a 13-year-old from Lima, Peru, with the lung capacity to hold her breath for more than two minutes and the daring to plunge deep beneath the ocean surface, up to (21m) 70 feet, unaided by scuba gear.

She is a driven athlete. Then, in February, everything changed.

"One day, she falls down in the shower," says her mother, Claudia Calderon.

The injury is nothing, a trifling hip bruise.

But soon after, while on a skiing trip to Colorado, Albi is struck by crushing headaches from the back of her neck to her eyebrows. Her father and mother think it is the altitude, or maybe a sinus infection, until the pain spreads to her shoulders and back.

On the return flight to Peru, it grows worse.

"That's when we start a nightmare," her mother says.

Over the next weeks and months, Albi sees more than 40 physicians from around the world – neurologists, cardiologists, rheumatologists, psychologists, psychiatrists, orthopedists, endocrinologists, experts in tropical diseases. They draw blood and analyse urine. They give her X-rays and MRIs. They prescribe pain pills. They can find nothing medically wrong.

By the time she flies in August to a children's hospital in Miami, the pain has spread to her entire body.

Unable to walk without horrendous difficulty, she uses a wheelchair. She can't lift her arms. Her mother must spoon-feed her and brush her teeth. Touching her skin sends her into excruciating paroxysms.

Albi is incapacitated.

After six weeks, Albiera Camaiora, graduates from the RAPS (Rehabilitation for Amplified Pain Syndrome) program of Children's Mercy Hospital in Kansas City, Missouri. Albi's team of health professionals met with her parents Stefano Camaiora, left, and Claudia Calderon on October 23, 2013, to summarize Albi's progress and discuss what lies ahead for her as she returns to life in Lima, Peru. (Jill Toyoshiba/Kansas City Star/MCT)

According to Albiera's parents, Stefano Camaiora and Claudia Calderon, Albiera saw more than 40 physicians from around the world, and they couldn't find anything medically wrong.

"We are desperate. We are afraid," her mother says. "You stay awake at night. You don't know what's going on. It's horrible."

Then comes a call from a relative, a journalist in Los Angeles, who has heard of a handful of children's hospitals in the US that see kids with symptoms like Albi's.

Within a day, in mid-September, the seventh-grader and her parents are on a plane to Kansas City.

"As of today, we are done with the wheelchair," rheumatologist Cara Hoffart, 34, tells the family at Children's Mercy Hospital.

Get up. Put it aside. Walk.

"As of today, we are done with the medications."

Hoffart knows what is wrong.

In some medical circles, a diagnosis of Amplified Musculoskeletal Pain Syndrome is as controversial as its symptoms are odd.

At Stanford University's Lucile Packard Children's Hospital, Elliot J. Krane, a leading professor of anaesthesiology and paediatric pain, insists the best way to think of amplified pain syndrome is as a catch-all phrase, not a diagnosis.

"It's a term to describe kids with widespread pain when they can't figure out what's causing it," he said.

Other names include Widespread Pain Syndrome, Pain Associated Disability Syndrome and Diffuse Idiopathic Pain Syndrome, with "diffuse" referring to its body-wide nature and "idiopathic" going directly to its mystery, meaning "of unknown origin".

While some experts argue that amplified pain is just another name for a juvenile form of fibromyalgia – itself a chronic, all-encompassing pain with no set cause – others argue they are different in two ways.

First, while specific medications sometimes ease fibromyalgia's symptoms, they don't work with amplified pain. Second, there's longevity.

"I think when you read about fibromyalgia, there is a sense that it is something you will live with the rest of your life," Hoffart says. "I don't accept that (with amplified pain). I see our kids get better."

What no one argues is the reality or intensity of symptoms that physicians suspect may have plagued some teenagers for years, but may have been misdiagnosed.

It's unclear how many children are affected by this kind of body-wide chronic pain. But in the last decade, several hospitals have started programmes to treat them. Children's Mercy opened Rehabilitation for Amplified Pain Syndrome in March, basing it on a similar programme at the Children's Hospital of Philadelphia.

Other programmes exist at the Mayo Clinic in Rochester, Minnesota; Boston Children's Hospital; Stanford in California; the Cleveland Clinic Children's Hospital; and a few other hospitals.

Their patients, like Albi, tend to be driven, type-A, perfectionist teens. Girls typically are more affected than boys.

After a minor injury, or even a sudden perceived failure, they develop symptoms that doctors think have as much to do with psychology as physiology.

The condition appears to exist at the nexus of illness and culture, suggesting that today's stressful, high-expectation teen environment plays a noxious role.

"I'll tell you about the pain I see and the kids I see," says Barbara Bruce, director of the Mayo Clinic's paediatric chronic pain programme, which opened in 2010. "There's a lot of pressure on the kids we see. They're driven."

Even before they get ill, these kids tend to worry, Bruce said, about school and the future. Pressure can arise from themselves, parents, peers and culture.

Often it's a small hurt that temporarily hobbles them but causes high-functioning teens suddenly to feel imperfect or vulnerable.

Stress kicks in, triggering a cascade of events in the nervous system. Hormones flood the body and, figuratively, set the nerves on fire. What began as a small pain grows and spreads.

Soon, more doctors are called in. Tests show nothing physically wrong. That's often when parents and teens are told the pain is purely psychological.

"But the pain itself is real," Bruce said.

Hoffart likens it to bleeding ulcers. They, too, can be induced by stress, but that doesn't mean the sore eating away at the gut isn't real.

The more doctors these children see, the thinking goes, the more their stress mounts. They miss school and fall behind. They see parents worry and medical bills mount.

The nerves in the body, already on high alert, become even more sensitive. What started out as an insignificant hurt amplifies to incapacitate the entire body.

"I have kids who are in so much pain they can't wear clothes," Hoffart said.

(The hospital's programme treats children with less severe symptoms at an outpatient clinic, but it has three spots in its most concentrated day-long programme for severe cases like Albi's.

The cost is considerable, although Children's Mercy has been successful at getting most insurance companies to cover the bills, which easily run in to the tens of thousands of dollars.

"I tell our kids they have weird pain. We do weird things," Hoffart said.

Their methods are specific, slow and controlled. But the last thing they do is steer clear of the pain.

"If it hurts to do something, that's what we tell them you should do," Hoffart said, which is one of the prime reasons they tell kids on day one to get up from their wheelchairs.

Hoffart also takes kids off their pain medications. Nor does the programme use pain medications or nerve blocks during therapies, an aspect of the programme that makes it more controversial than others that do use medicines.

It also tends to reflect differences in philosophy in programmes run by rheumatologists, like Hoffart, and those run by anaesthesiologists.

At Stanford, the anaesthesiologist Krane, for example, thinks "there is a role for medications from time to time".

"For some kids, physical therapy is just too painful, and you can't do it," he said. "I think it is cruel to subject a child to painful physical therapy without a nerve block."

Hoffart maintains that for most kids, these medicines have already failed to work.

For Albi, just attempting to walk is excruciating. Her steps were mincing, like a child walking on shards of glass.

Most of the day's therapy takes place in the hospital's Don Chisholm Building, an annex about one-third of a mile from the main hospital. The kids are forced to walk there.

On her first day, it took the once-athletic Albi more than 70 minutes.

Using balls and baskets, cones and other equipment, the kids are asked to walk up and down stairs, do push-ups and knee bends, walk on treadmills.

"We don't coddle them," says Wilson, the occupational therapist.

Desensitisation therapies – massages, touching, rubbing, showers, hair brushing – help the kids develop a tolerance to pain that, gradually, causes the pain to subside.

"They make me do a million things a day," Albi says during a brief break. "When I get out of here, I'm so tired and exhausted and frustrated, I'm in a bad mood and a lot of pain. But inside of me, I know this is good for me."

Meanwhile, the yoga, music therapy, art therapy and personal counselling are geared to find ways to reveal and deal with pressures and psychological stress.

When many of the teens first arrive at Children's Mercy, they deny that they are feeling undue stress, said psychologist Dustin Wallace.

But it often doesn't take long before kids are talking about the high expectations they face.

In counselling, Wallace said, kids reveal their most fundamental fears: that they're not living up to their potential, that they're not worth the sacrifices their parents have made for them.

"They may get a bad grade on a test," Wallace said, "and then think, 'If I get a bad grade, I'm never going to pass this class. If I don't pass this class, I can't get into college. If I can't get into college, I won't be able to get the job I want'."

Less than six weeks after she entered the programme in a wheelchair, Albi left able to shower, dress, brush her teeth and even jog. The walk to the annex took eight minutes instead of 70.

The programme is so stressful that pain sometimes actually increases in the weeks they're there, Hoffart said. But the experience at other programmes and Children's Mercy's own follow-ups show that the pain tends to subside to manageable levels over the following months.

When pain flairs, the teens have the tools to reduce it without medications. Time will tell if it ever completely goes away.

At Albi's "graduation" in October, her mother told Hoffart and her team, "You made a miracle."

"I can't explain the emotion to come home with Albiera again as a normal person," she said. "I don't have the words. Thank you from my heart."

Albi said that when she got home, she had a plan.

"I'm going diving again," she said. — The Kansas City Star/McClatchy-Tribune Information Services

World Ovarian Cancer Day: One voice for every woman

Posted: 07 May 2014 09:00 AM PDT

Today is World Ovarian Cancer Day, and on this day, ovarian cancer organisations from around the world unite to educate their communities about ovarian cancer and its symptoms.

Each year, nearly a quarter of a million women around the world are diagnosed with ovarian cancer, and the disease is responsible for 140,000 deaths annually.

Statistics show that just 45% of women with ovarian cancer are likely to survive for five years, compared with 89% of women with breast cancer.

Recognising the severity of the disease and the need to raise awareness about ovarian cancer, the first World Ovarian Cancer Day was organised on May 8, 2013.

On this day, ovarian cancer organisations from around the world unite to educate their communities about ovarian cancer and its symptoms.

Today marks the second annual World Ovarian Cancer Day, and for women living with the disease, and their families and friends, this day has built, and will continue to build, a sense of solidarity in the fight against the disease.

The theme for 2014 is "One Voice for Every Woman".

Ovarian cancer is a cancer of the female reproductive system, and is the fifth most common cancer among Malaysian women.

Approximately 500 women are diagnosed with ovarian cancer every year in our country; of these, 90% are classified as epithelial ovarian cancer.

The remaining 10% consists of germ cell tumours and sex cord stromal tumours, which are less common and generally have a better outlook compared to epithelial ovarian cancer.

Family history is a significant factor as approximately 5-10% of epithelial ovarian cancer cases are due to genetic factors. This means having a close blood relation on either the mother or father's side of the family who has had breast cancer before the age of 50, or ovarian cancer at any age.

The ovaries, which are two small organs located on both sides of a woman's uterus, are responsible for releasing eggs every month during a woman's reproductive years.

They also produce hormones which trigger the secondary sex changes which occur at puberty and maintain reproductive organ function.

When a woman reaches menopause, the ovaries stop releasing the eggs and hormone production decreases.

Epithelial ovarian cancer (hereafter referred to as "ovarian cancer") in its early stages produces few or non-specific symptoms.

Symptoms and screening

Due to its non-specific symptoms, one of the most significant challenges of ovarian cancer is the absence of a test for early detection which offers a better chance for successful treatment and improved survival rates.

Lack of awareness of symptoms and late stage diagnosis results in many instances of the disease progressing undetected until it reaches an advanced stage – approximately 70% of women are diagnosed at Stage 3 or Stage 4 of the disease, with an overall five-year survival rate of only 30%.

To aid in earlier detection, even vague symptoms should be carefully evaluated. These symptoms include abdominal bloating or fullness, anorexia (poor appetite and loss of weight), nausea, vomiting and urinary symptoms.

However, due to the non-specific nature of these symptoms, most of the women who present themselves with these symptoms would not necessarily be suffering from ovarian cancer.

Unlike the Pap smear which detects pre-cancerous changes in the cervix, or mammograms which detect breast cancer at an early stage, there is no definitive screening test for ovarian cancer.

There is, however, ongoing research evaluating a combination of blood tests for tumour markers together with ultrasound scans with the hope of developing a screening test for the future.

Unfortunately, at the present time, there is not enough scientific evidence to recommend this approach for screening for ovarian cancer.

Screening using the tumour marker CA125 has not been proven to be useful because only 50% of patients with early stages of ovarian cancer have raised levels of CA125. Among patients at an advanced stage of ovarian cancer, 90% of patients have raised CA125 levels, but at such an advanced stage, other signs and symptoms are usually already evident.

Testing for CA125 is also associated with a high false positive rate – i.e. patients may not have cancer, but still exhibit high levels of CA125. In such cases, the patient may needlessly undergo further tests and investigations, which can be costly and invasive.

One percent of healthy women, women in early pregnancy, as well as women with common conditions such as fibroids, endometriosis, kidney disease, lupus, gastrointestinal and liver conditions as well as cancers of the liver, lung, breast and pancreas may have raised levels of CA125.

Hence, this test is not specific enough to detect ovarian cancer and is an ineffective tool.

Diagnosis and treatment

To ensure an accurate diagnosis, a doctor requires the patient's full medical history to perform a clinical examination, including an abdominal and pelvic examination.

The doctor then arranges other tests such as ultrasound scans, blood tests, including tests for tumour markers, and CT scans. While the number and type of examinations may vary depending on the individual patient, a careful and thorough assessment is essential before embarking on surgery and treatment.

For many patients in the advanced stages of the disease, the tumour often spreads beyond the ovaries to neighbouring organs such as the uterus, bowel and other areas within the abdominal cavity.

Surgery is recommended if cancer is diagnosed; this procedure is called a laparotomy, whereby doctors will remove all or as much of the tumour as possible together with the ovaries, the uterus and omentum (the fat which hangs from the stomach and large intestine).

Removal of the tumour is called "debulking", and plays an important role in determining the patient's survival as there is a very close relationship between the amount of tumour left behind after surgery (residual tumour) and the patient's survival rate.

Simply put, the lesser the amount of residual tumour, the better the chances of survival. In selected patients where the cancer is still in its early stages and confined to only one ovary, it may be possible to perform conservative surgery, which means removing only the cancerous ovary and preserving the uterus and remaining unaffected ovary so that the patient may still have children in the future.

Surgery should be performed by a specialist with the necessary skill, training and experience in treating and managing ovarian cancer. A gynaecologist who specialises in the treatment of women with cancer of the reproductive organs (uterus, ovaries, fallopian tubes, vagina and vulva) is called a gynaecological oncologist.

Several studies have confirmed that better cure rates are obtained when this complex surgery is carried out by a gynaecological oncologist and both the US National Cancer Institute as well as the National Health Service in the United Kingdom have endorsed this recommendation.

Surgery also helps doctors to determine the stage of the cancer; it's essential for doctors to understand this so they can create an effective treatment plan for the individual patient.


Chemotherapy is the use of drugs to stop the growth of cancer cells, either by killing the cancer cells or preventing the cells from dividing, multiplying and spreading.

Patients with very early cancer (stage 1A Grade 1) don't require chemotherapy. Those with more advanced cancer will benefit from chemotherapy. Most patients will be treated with six courses of chemotherapy. Each course lasts for five to six hours and a course is repeated every three to four weeks.

Some patients, especially those in poor health, may be advised to have neo-adjuvant chemotherapy. This involves giving three courses of chemotherapy first, followed by surgery, and then a further three courses of chemotherapy.

In conventional chemotherapy for ovarian cancer, the drugs are given intravenously. Known as systemic chemotherapy, this treatment decreases the risk of recurrence, prolongs survival and increases the chances of cure.

The most common chemotherapy drugs used for ovarian cancer are carboplatin and paclitaxel, a combination which is well tolerated.

Common side effects of treatment include hair loss (which grows back after the treatment is over), low blood counts and decreased immunity.

Monitoring for side-effects will be carried out at specific intervals by clinical examination and blood tests.

New treatment approach with anti-angiogenesis therapy

Anti-angiogenesis therapy is a form of targeted therapy that has been found to be effective for patients with ovarian cancer.

Angiogenesis or new vessel formation is a pre-requisite for the progression and metastasis (spread) of cancer cells.

A pro-angiogenetic factor in blood, known as vascular endothelial growth factor (VEGF), is related to the progression of the disease and corresponding poor prognosis for ovarian cancer patients.

In 1971, a scientist named Judah Folkman came up with the concept of using drugs called angiogenesis inhibitors to block the growth of tumours by preventing the formation of new blood vessels.

Since then, this form of therapy has been further refined, and subsequent research with anti-VGEF therapy shows that it slows tumour progression, causes resolution of ascites (fluid in the abdominal cavity related to cancer) and makes chemotherapy work better.

In a nutshell

Ovarian cancer is a challenging disease to treat. The cornerstone of treatment is surgery by a trained and experienced surgeon.

Following surgery, most patients will benefit from chemotherapy.

A relatively recent form of targeted therapy, known as anti-angiogenesis inhibitors, has been found to be effective in reducing the progression and metastasis (spread) of cancer cells.

The best hope for successful treatment, however, lies in early detection. At present, ovarian cancer is difficult to detect in its early stages as its symptoms are few and non-specific and there is lack of an effective and proven screening test, hence most patients are only diagnosed at advanced stages of the disease.

Women should be aware of changes in their body and be alert for unusual symptoms. It is said that "ovarian cancer whispers" – some of the signs and symptoms may be mild or subtle.

Evaluation of women with unusual or suspicious signs and symptoms should include assessment by an experienced gynaecologist.

Women should make it a point to have regular check-ups. At these check-ups, unusual symptoms should be discussed and assessed. A pelvic examination should be carried out during routine Pap smears as this may result in the detection of gynaecological problems, including ovarian cancer.

> Dr Suresh Kumarasamy is an obstetrician & gynaecologist/gynaecological oncologist in private practice based in Penang. He is immediate past president of the Obstetrical & Gynaecological Society of Malaysia, a council member of the Asian Society of Gynaecological Oncology and an adjunct associate professor at Penang Medical College.


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