Woman Left Bedbound By Tiny Hole In Ear Reveals How It's 'Impossible' To Lead An Ordinary Life | HuffPost Life

How A Pin-Sized Hole In Her Ear Crippled This Woman's Life

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Parents editor at HuffPost UK

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Beth Rye, 24, had always dreamed of becoming a nurse.

But her world was turned upside down after being diagnosed with a rare health condition that left her bedbound and unable to lead a normal life.

In a cruel twist of fate, the nurse is now the patient.

"I feel like I'm constantly on a boat," she tells HuffPost UK Lifestyle. "I can't walk in a straight line because my balance is so bad. Sounds are like torture as they make my head spin even more - car horns literally make my eyes bounce.

"I've also lost a lot of my independence and rely on my parents to help me with daily living."

In 2013, Rye was diagnosed with Superior Semicircular Canal Dehiscence (SSCD), an extremely rare condition which means she has a pin-sized hole in the temporal bone which sits in her inner ear.

This tiny hole creates a third "mobile window" into the inner ear, which causes sound or pressure to trigger abnormal activation of the balance canals in the ear.

According to the Vestibular Disorders Association there are usually only two "windows" in the inner ear: the oval window, through which sound energy is transmitted into the inner ear, and the round window, through which sound energy is dispersed from the inner ear after traveling around the cochlea.

With SSCD, a third window is created and, as a result, Rye experiences symptoms such as dizziness, nausea and vomiting, vertigo and tinnitus.

"The hole in this bone is tiny, but the side effects can be devastating," reveals her brother, Tom, who has been instrumental in raising awareness of Rye's condition.

He recently launched a campaign called 'Get Beth Better', to help raise money to fund treatment for his sister in the US.

Beth and her brother Tom before her diagnosis

Since she was diagnosed with SSCD in 2013, Rye's life has altered dramatically - to the point where she is now a prisoner in her own bedroom.

The nursing graduate once had to spend 14 weeks hooked up to a hospital drip in order to survive, because her dizziness made her so sick that she was unable to keep food down.

To put her condition into perspective, one of Rye's university friends - who is now a qualified nurse working in London - tells HuffPost UK Lifestyle that the condition is "arguably worse than cancer, because it's so debilitating".

"People with cancer can have days where they're well enough to leave the house and do things, and if they're unwell they can watch films or chat on the phone," she says. "But that isn't the case for Beth."

"Nowadays everyone is in some way affected by cancer, which increases the support family and friends can provide," she adds. "But because Beth can't tolerate noise, she's even more isolated. She can't sit and chat because even the noise of typing on a computer hurts her."

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Rye first noticed something was wrong during her nursing placement at university, when she would become increasingly dizzy and need to lie down several times a day.

Confused by what was happening, she drove back to her parents house in Somerset and collapsed as she walked through the front door.

"I had severe nausea and vomiting, vertigo attacks, pulsatile tinnitus and ‘bouncy’ eyes. So my symptoms came on within 24 hours," reveals Rye.

As if this wasn't bad enough, her suffering would worsen with sound or movement.

Following her visit to the doctors, Rye was left disheartened after being prescribed nasal spray: "It took me 10 months to get a diagnosis. The doctors in my local town just kept discharging me, telling me I would get better and that I just needed to try and get on with my life.

"This was one of the hardest times because I felt like I wasn’t being taken seriously."

Needless to say, Rye didn't recover. In fact, the "life-changing" condition has meant that Rye has had to put her nursing career on hold - despite managing to graduate last July.

The past 11 months of her life have been spent bedbound, with occasional hospital trips breaking up the long, monotonous periods of time confined to her home.

"It’s isolating and can be hard to deal with at times," she admits. "It’s impossible to live a normal life."

Because her condition is so rare - in fact, the first medical research surrounding it was presented in 1998 - Rye has been forced to conduct a lot of research herself, with the help of her family.

After seeing a private consultant in Cambridge, Rye underwent two failed operations to try and correct her ear.

Now, her only hope for treatment is to visit a specialist in the US, but it's a trip which will cost her family more than £50,000.

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"After discussing my case with a surgeon in Boston, I discovered that the SSCD is in a tricky place, which is even more rare," explains Rye.

"For people in this situation there's a different surgical technique that can be used, increasing the chances of the repair working," she adds.

"The downfall is that they don't do this in the UK because there's simply not enough experience of the condition over here. Also, because they treat hundreds more patients, the success rate is based on a higher number of people."

To raise the money needed to get their daughter better, Rye's family have rallied around to organise fundraising efforts for her including an open garden event, a horse racing night and a 5km run. And she's even been supported by complete strangers.

"Words cannot describe how amazing it is to see people fundraising or donating to help me. I've even had donations from people I don’t know.

"I quite often get sent pictures of peoples supporting the campaign which really makes my day. I didn’t ever expect this kind of response and I think it is fair to say this world is full of such generous, loving people!"

The next step for Rye is to visit the US in a month's time to have more tests, scans and consultations with two leading SSCD surgeons, who will then review her results and find a solution for fixing her ear.

But, of course, this all depends on her financial situation.

"I am lucky to live with the most supportive parents and brother," she adds.

"This illness has made me stronger as a person and I am much more grateful for the little things in life.

"I know I still have a long way to go, but I'm proud of how far I've come and can’t wait to be living a normal life again."

Visit Beth's 'You Caring' page to donate to help her reach her target.

21 Rare Diseases

Stiff Person Syndrome(01 of21)

People who suffer from this acquired neurological disorder experience repeated, often painful, muscle spasms as well as muscular rigidity and stiffness. According to the National Organization of Rare Diseases, spasms can occur at random or they can be caused by something as seemingly benign as light physical contact or an unexpected noise. The cause of Stiff Person Syndrome isn't yet known, but symptoms can be stabilized with medication. Left untreated, however, a person can lose the ability to walk.

Gigantism(02 of21)

This disease, which according to the NIH is most often caused by a begnin tumor in the pituitary gland, results in an excess of GH, or growth hormone. This causes sufferers to grow abnormally large, not just in terms of height, weight but also organ size. It results in complications like delayed puberty, increased sweating, and secretion of breast milk.

Pica(03 of21)

Characterized as an eating disorder, Pica causes people to eat what the National Organization for Rare Disorders describes as "non-nutritive" things. That umbrella term can include (but isn't limited to) dirt, clay, paper, and paint. Interestingly, it's not unusual for young kids to experience transient pica as a kind of phase, and pregnant women are also known to develop temporary pica cravings. The cause? Unknown. But in order to be diagnosed with full-blown Pica, a person's symptoms must last for more than a month.

Maple Syrup Urine Disease(04 of21)

MSUD, which is passed down through families, is a life-threatening metabolic disorder passed down through families that stems from the body's inability to process certain amino acids, leading to a build-up of them in the body. According to the NIH, symptoms usually surface in early infancy and can include vomiting, lack of energy, seizures, and developmental delays. MSUD takes its name from another symptom -- the urine in affected infants smells like caramel or maple syrup.

Situs Inversus(05 of21)

Situs Inversus is a congential condition in which internal organs of stomach and chest lie in mirror image of their normal body position -- something many sufferers aren't aware of until they seek medical help for an unrelated problem. People with Situs Inversus typically wear some form of identification to help doctors in the case of a medical emergency.

Trigger Thumb(06 of21)

Trigger thumb, or trigger finger as it's sometimes known, causes a person's finger or thumb to get caught in a locked position. According to the Mayo Clinic, it can then stay stuck or straighten with a painful "snap." The cause? It depends. Trigger Thumb is the result of a narrowing of the sheath around the tendon in the problem figure, but that can be caused by a lot of things, including any activity that requires people to grip things frequently. Trigger Thumb is also more frequent in women.

Scurvy(07 of21)

Once the disease of sailors and pirates, scurvy does still exist in the United States, though predominantly in older, malnourished adults. It usually stems from a Vitamin C deficiency, which can result in gum disease, skin leisons, and swelling of the joints. (credit:Getty )

Wilson's Disease(08 of21)

This progressive genetic disorder causes sufferers to store excess copper in tissues, including the brain and liver. Though the Mayo Clinic says that the body depends on copper in order to use iron and sugar, too much of it in the body can have real consequences. Which is why sufferers of Wilson's Disease are prone to liver failure.

Foreign Accent Syndrome(09 of21)

According to researchers at the University of Texas at Dallas, this speech disorder causes people to experience a sudden shift in their accents and though it is known to be caused by things like brain trauma, conversion disorder, or multiple sclerosis, an exact reason behind the syndrome is unknown. Sufferers of FAS dramatically shift their speech in terms of timing and intonation, which often causes them to sound foreign, but they remain totally comprehensible. Documented accent shifts include from American English to British and from British to French.

Carcinoid Syndrome(10 of21)

According to the National Organization of Rare Diseases, about 10 percent of people with carcinoid tumors -- which the Mayo Clinic says are slow growing tumors that produce excess serotonin and usually appear in the gastrointestinal tract or lungs -- get this syndrome. It occurs only in patients whose tumors have metastasized to the liver. The symptoms? Wheezing, hotness, and extreme facial blushing.

Cyclic Vomiting Syndrome(11 of21)

Children and adults afflicted with CVS experience recurrent episodes of severe vomiting, which can last for days, followed by sudden periods of no vomiting. While kids are likely to experience more frequent attacks, adults's often last longer. To date, the cause of CVS is unknown.

Peeling Skin Syndrome(12 of21)

This obscure, genetic skin disorder does exactly what its name implies: causes sufferers to experience constant shedding of their skin. (In some patients, peeling is limited to the feet and hands.) Along with that, sufferers often feel itching and redness -- symptoms that can appear from birth or develop later in life. Although the exact cause is unknown, a mutation in the TGM5 gene has been identified in many sufferers.

Platelet Storage Pool Deficiency(13 of21)

This is a blanket designation for several rare platelet abnormalities, most of which lead to mild or moderate bleeding disorders. According to the NIH, the problem stems having limited granules -- the parts of platelets that, among other things, store ADP -- the energy released when a molecular bond is broken. That reduction in storage space inhibits the platelets' ability to secrete ADP in a speedy fashion, which is thought to be what causes the bleeding. Classic symptoms of a Platelet Storage Pool Deficiency are nosebleeds, excessive bruising, and profuse bleeding in surgery.·

ACDC(14 of21)

Only nine people in the United States are known to have this disease, which was just given a name in a recent study in the New England Journal of Medicine. ACDC, or arterial calcification due to CD73 deficiency, results in calcium build-ups in the arteries below the waist of sufferers and in the joints of their hands and feet. According to the NIH, the breakthrough discovery found that the disease is related to a variant in the NT5E gene.3

Conversion Disorder(15 of21)

This condition, which typically occurs after an extremely stressful emotional event, causes a person to experience sudden blindness or paralysis that can't be otherwise explained. People with mental illlnesses are at particularly high risk and psychological treatment can help lessen the symptoms.·

Ochronosis(16 of21)

Ochronosis results in black or blue external tissues, often the ear cartilage or eye, though it can occur throughout the body. It affects people who suffer from certain metabolic disorders, but it can also be caused by exposure, though scientists aren't exactly sure to what. Not just a cosmetic issue, the affected areas can become brittle and degenerate over time.

VLCAD Deficiency(17 of21)

This condition, which is caused by genetic mutations in the so-called ACADVL gene, keeps people from converting certain fats to energy. According to the NIH, the deficiency often presents during infancy in symptoms including low blood sugar, weakness, and lethargy.

Hairy Tongue(18 of21)

As the name suggests, Hairy Tongue is a condition in which the tongue develops a black, hairy texture. According to the Mayo Clinic, the disease is "harmless" and is caused by an overgrowth of bacteria in the mouth, which can be treated with antibiotics. The NIH reports that hairy Tongue can also present as brown, yellow, or green discoloration. ·

Ochoa (Urofacial) Syndrome, or, Peculiar Facial Expression(19 of21)

This obscure, inherited disorder presents at birth and causes infants to grimace when, in fact, they are attempting to smile. The disorder also includes an extreme urinary abnormality: an obstruction that interrupts the connection between nerve signals in the spinal cord and bladder, leading to incontinence. Though treatment does exist in the form of antibiotics and bladder re-education, some patients can develop renal failure in their teens and 20's, which can be life-threatening.

Eagle Syndrome(20 of21)

This syndrome, caused by a particular calcified ligament or elongated bone, frequently causes sufferers to feel that something is stuck in their throat. (Scientists don't know the reason behind said elongation/calcification.) Other symptoms can include ear and throat pain as well as difficulty swallowing. According to the NIH, it can be treated surgically or non-surgically with steroid injections and other anti-inflammatories.

Progeria(21 of21)

According to the Mayo Clinic, only around 130 cases of this devastating genetic disorder have been documented since it was discovered in 1886. Affected babies normally appear normal at birth, but within 12 months begin to have symptoms like hair loss and wrinkles. According to the Mayo Clinic, progeria is caused by a genetic mutation, but not one that's passed down through families; it's a chance event that affects only one egg or sperm. The average life expectancy for sufferers is 13.

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