Case Studies

Hilda's Story

An inspirational lady

One of the key objectives of Action on Pain is to provide support and empathy to people with chronic pain and their carers. Knowing that you are not the only one who has a day to day battle with pain can be comforting. So Id like to briefly share the story of a lady who lived with pain for over thirty years and was still having a joke with nursing staff the day before she died.

Hilda was about forty years old when she was diagnosed with rheumatoid arthritis (RA). RA is a disease that not only affects joints throughout the body so that they deteriorate more rapidly but it can also present with general tiredness. It has been described as like waking up each day with low key flu like symptoms. Hilda's symptoms started with pain affecting one hand and much to her frustration, stopped her playing hockey. She was a very active and sporty lady so it was assumed at first that her joint pains were more likely to be due to osteoarthritis; that is general wear and tear. As the years progressed more of Hildas joints became affected but she continued to work full time as a teacher until she was about fifty seven; being persuaded only then by her three children to consider early retirement.
It was only in the later years of her condition that Hilda was to benefit from the more recent advances in medication to control RA: By then it was probably a bit too late and by the time she died she had received five different joint replacements with the likelihood that others would have needed to be replaced in the future.

Hilda enjoyed life to the full and was well known throughout her town, being regarded as the lady who always had a song and a smile.

She had a very supportive family but had lived alone for the previous fifteen years. But she rarely felt alone because her personality and interests had enabled her to create a large social network. Despite her physical limitations she continued to drive, was actively involved in a local church, provided entertainment for various social events, belonged to a very popular local entertainment group and held fund raising events for several charities that she supported. As the years progressed she had to delegate work at these charity events, recognising that she was unable to physically do it. So whilst others worked she socialised and provided the humour! When she was unable to achieve some of the simple dance steps required in the entertainment group, rather than give up they modified routines so that she could sing sitting down.

In the last two years of her life, Hilda's health deteriorated and following failed surgery, along with heart problems her walking was severely limited, becoming dependant upon others and a wheelchair to get out and about. As she became more confined to the house she still enjoyed herself; she was happy in her own company and was never without visitors. In fact on one occasion a friend called by because having been to visit someone else first she had left feeling depressed. Mm Thought this friend. Who shall I visit next? I know, I'll go and see Hilda she's bound to cheer me up!

No one enjoys the prospect of hospital and surgery but Hilda regarded each procedure positively and remained cheerful. She was always determined to get going after her operation and was frequently the life and soul of the ward. She was popular with the medical staff and rarely complained even when complications arose. As I said earlier she was having a joke the night before she died. Her funeral attended by over three hundred people was certainly a celebration of her life; recognising that throughout her 72 years, Hilda had inspired numerous people in so many different ways. She was determined that her pain and disability would never overcome her and it rarely did.

Hilda's story is one of inspiration-doing what she could rather than thinking about what she could not do. Instead of letting her pain run her life she challenged it in so many ways. As her disability crept up on her she was able to put all her skills and abilities to new uses when it would have been far easier to sit back and do nothing. It was an absolute joy to visit her walking into a house full of love; warmth and kindness.

So if you feel overwhelmed by your pain unsure what to do next perhaps you might like to think of Hilda's story and perhaps you will be inspired. Helping others even after her death-she would have liked that!!

Written by Judith Semmons- Hilda's daughter

From JOURNAL OF WOUNDCARE VOL13, NO. 10, NOVEMBER 2004

Life, epidermolysis bullosa and chasing tornadoes
Sam Hall was diagnosed with epidermolysis bullosa as a baby. Here she describes growing up with the condition and the continual dressing changes it brings, and how she has not let it prevent her from leading an active and exciting life.

I t 's probably a parent 's worst nightmare. There is something wrong with your newborn baby and the doctors say they've never seen such a problem before and have no idea what is wrong. That is what happened when I was born on 10 June 1983.

The nurses were as horrified as my mum and dad when they removed my name and date-of-birth label and the top layer of skin peeled off with it.

Fortunately, the next day I was examined by a specialist who diagnosed recessive dystrophic epidermolysis bullosa: a rare genetic skin condition that causes the skin to blister and tear off at the slightest touch. This explained the large blister covering my right leg and foot. Dad recalls how it looked transparent and how he could see the veins in my leg through the large balloon. The diagnosis of epidermolysis bullosa was sure to offer a reasonable answer to all the puzzling questions everyone in the special care baby unit was asking.

What is epidermolysis bullosa?

Epidermolysis bullosa has three main forms:

• Junctional —the worst of the three groups as children tend to die within two years from malnutrition and anaemia caused by extensive blistering in the pharynx and oesophagus along with the outer blistering.
• Dystrophic —either dominant or recessive. Mine is recessive as both my parents carry the faulty gene yet do not have epidermolysis bullosa themselves.
  The most annoying part of having dystrophic epidermolysis bullosa is the tendency for wounds to heal with scarring. This causes fingers to fuse together, toes to grow in abnormal ways due to skin tightening, and narrowing of the oesophagus.
• Simplex — tends only to affect the hands and feet.

The severity of dystrophic epidermolysis bullosa varies greatly from person to person, but generally the skin worsens with ageing due to the scarring of tissue, which in turn can lead to squamous cell carcinoma. This form of skin cancer is linked directly to epidermolysis bullosa and differs from other skin cancers. Scientists are currently researching to discover why it differs and to find a way of treating it more effectively. My skin has definitely worsened over the years due to scarring and numerous operations to free my fused fingers and toes.

As a baby, after I had been allowed home, the district nurse visited daily to change my dressings and assist mum and dad in my general care. Before too long the toes on my right foot began to fuse together and contract backwards.

To my mum and dad 's horror they were asked to saw through the skin daily with gauze to try to keep my toes open. Eventually, dad refused to do this. It was causing everyone pain for no good reason, since after mum and dad had re-opened the toes and dressed the area, the skin would grow and push the gauze out. They were fighting a losing battle.

Choosing the right dressings
Dressing my skin has been a problem in the past. As a baby, I had gel applied directly on my skin, covered by gauze and then padded for comfort and absorption. I was then laid on a cotton wool layer topped with a soft cotton sheet. Dressings covered my right leg and foot, along with my left foot, both hands and back. I looked like I was wearing boxing gloves, ready to give anyone a swift left hook! These ‘mittens 'helped prevent further skin damage by protecting my skin when I scratched. The dressings were bulky but provided enough protection for a wriggling, active baby. My skin did not seem to worsen until my early teenage years. In my quest for an alternative to this, I have even tried cling film. As a child growing up in an active environment —doing tap-dancing and making up daring games at school —it was important to have the right dressings not only for safety but also for general happiness. I remember being on holiday with my mum and dad, playing table tennis, only to have to return to our apartment several times to change my dressings: they would slide down my legs, uncovering my wounds and exposing them to outside germs as well as sticking to my clothes. It was frustrating and upsetting having to stop doing something that I enjoyed purely because my dressings were not right.

If this were to happen continually without . finding better dressings, someone with epidermolysis bullosa might simply give up and not participate in activities or games. This would make me miserable.

Thus, I have changed the kind of dressings I use many times throughout my life and only now can I really say I have found something that is not only extremely comfortable to wear but also fits in with my active lifestyle: Mepitel and Mepilex Transfer (Mölnlycke).Some dressings suit people differently, but I have found what works for me. Dressings are a major part of life for a person with epidermolysis bullosa: they are life.

These dressings are ‘sticky 'but do not glue to my skin as a plaster would. There is enough stickiness to hold my dressings in place until my next bath. Now I can go out and enjoy an evening with friends knowing that I can rely on my dressings to stay in place and provide comfort and protection. Before, with gauzes and padding, this was always an uneasy, unreliable situation.

I also need the dressing's flexibility for tricky areas, such as joints, to enable me to move freely — the bulky dressings made me walk stiffly and uncomfortably. The new dressing is sufficiently thin not to show through my tights, so I can wear skirts with confidence. My legs do not look lumpy any more!

Having lumpy legs was not a feature I desired at 13, when all my school friends were wearing short skirts and tights. Although the tights hid the colour of the dressings, you could still see their shape through the tights. When I was young this did not bother me but, of course, being a teenager changes everything.

 

Dressing changes

In addition to these initial problems, dressing changes were never fun. The gauze dressings stuck like superglue to my wounds, which caused the skin to pull when I removed the dressing. Soaking in the bath did not help much as the water gave me a stinging shock every time the dressing fell away from my skin. This made dressing changes a terrifying prospect. All I wanted to do was to leave them on and play.

Unfortunately, I only started using Mepitel optimally after I became seriously ill when I was 15 with an infection caused by not changing the gauze dressings often enough. Dressing changes were so painful that I avoided them. At the time, I had a lot of GCSE work to do and, regrettably, I didn't put my health first. The pain was so severe that I was unable to walk, and the infection seemed to eat away my skin. To this day, I have holes in my legs to remind me. Never again will I put my health second.

That said, I believe everything happens for a reason. Initially, after being admitted to hospital to treat the infection, I used Aquacel (ConvaTec) directly on the wounds, covered with Mepitel and then Mepilex. Miraculously, after the first dressing change I was able to walk quite well. I had to have strong drugs to relieve the pain and I still take these now. These help me to cope a lot better with dressing changes and offer adequate pain relief. I hope that by keeping to this regimen I will never be struck down with a similar infection again.

Many people with epidermolysis bullosa are nervous about trying these ‘sticky 'dressings. All through your life you are almost trained not to put anything sticky on your skin as it will cause more damage. Yet Mepitel is not so sticky that it will cause further damage. If I do have a particularly dry wound and the dressings stick more than I would like, I simply sit in the bath for a few minutes and the dressing eventually . oats off the skin without any pain. Applying the dressings is virtually pain-free too. Because they are very flexible, they do not sting, irritate or reduce mobility. As a teenager, bulky dressings limited the number of shoes I could wear because not many would fit my feet comfortably. But with the dressings I wear now, I can wear almost any type.

Freedom
Going on holiday has become a lot easier as well. I have been to New York with a friend, a trip that wouldn't have been possible had I not been confident about my dressings. I knew I would be comfortable and protected, allowing me to enjoy an active city break and having the freedom to be a ‘normal ' teenage girl..

After New York I feel even more confident about going to new places, either on my own or with friends. I 'm hoping my next trip will be tornado chasing in America —and if the tornado wants to suck up my dressings, it'll have to take me with them as once they are in place, they do not move until the next dressing change.

A fighting spirit
I cannot thank my mum and dad enough for all they did for me, putting me in mainstream school, letting me do ‘normal 'things as much as my condition permitted. Many parents tend to wrap their disabled children in cotton wool and assume that they cannot do anything that would have the tag as being the norm, but every child, whether disabled or not, should have the opportunity to discover their own limits. Only then can they grow up to be fighters, not letting anything stand in the way of what they want to do.

 

Sam Hall can be contacted direct on