Sue - Interview 31

Age at interview: 63

Age at diagnosis: 62

Brief Outline: Sue was diagnosed with bulbar onset motor neurone disease less than a year ago (2006). She is now unable to speak and uses a laptop with voice software. Her arms and legs are still relatively strong.

Background: Sue is a retired business administrator, married with 4 children aged 37, 33 and twins aged 22. Ethnic background/nationality' White British.

More about me...

(Sue was interviewed at first in writing via e-mail, and then using her computer to speak the text on video).

Sue had experienced muscle aches in her neck and arms for many years, which she put down to helping with heavy work in her husband's garden design business. Her sports therapist recommended some fit-ball classes to help, but she actually found it very hard work. On one occasion, early in 2005, she found her neck and jaw felt locked after the class, and her speech was slow and slurred. Looking back, she wonders if there is any connection with a severe throat reaction she experienced the previous year during crop-spraying opposite her house. She would like to see more research into causes of MND and possible links with agricultural chemicals.

Within a month, her speech was becoming so slurred that people began to joke about whether she had been drinking. Her GP immediately thought it was a neurological problem, perhaps a stroke, and referred her to a neurologist. It was a 3-month wait for an appointment. Sue had lots of tests, but it took a year of testing and excluding other conditions before a second neurologist in London diagnosed sudden bulbar onset MND. She and her husband were shocked when the consultant told her she had a life expectancy of 2-3 years, and said her local hospice would be contacted. Sue was horrified to hear that she might need hospice care, because to her it meant a place to die. Now she really appreciates the support from her hospice in all kinds of areas, including help with obtaining benefits, providing services such as aromatherapy and massage, and offering emotional support.

Sue was advised to have a RIG (radiologically inserted gastrostomy) when her swallowing became weaker. She had it done before a planned trip to New Zealand. In the event she found it very painful and her recovery was quite slow, so the trip to New Zealand was cancelled. She felt the ward staff were not very well informed about her condition or how to communicate with her, and it took a while for staff to realise how much pain she was in. Now the RIG has settled, and Sue uses it to take thin liquids and ground-up tablets. She can still eat many foods and thick liquids by mouth, although eating out is difficult because she gets quite messy and worries about choking. She also has some problems with excess saliva, especially later in the day and when tired.

Since the diagnosis less than a year ago (2006) Sue has completely lost her speech, and communicates using a laptop with voice software, which she finds much more flexible than a Lightwriter. Although she misses being able to laugh and talk, she can still enjoy conversations with her family, friends and neighbours using the English female voice on the software, known as 'Audrey'. So far her arms have not deteriorated much and she is still able to type very quickly, and her legs are still quite strong. She is still independent and able to do most daily activities apart from vacuuming and gardening. She can still drive, but chooses not to, and prefers not to shop alone. She is worried about what the future holds, for both herself and her husband who is now her main carer. One piece of advice to others with MND is to learn to type, in case this becomes an important means of communication. For her it has been crucial.

She went to a fit-ball class to help with aching in her neck and arms, and afterwards found she...

I first noticed symptoms in February 2005. I was actually partaking in a fit-ball class rather like pilates, in that it strengthens the core muscles. For years I had been suffering with muscle aches in neck and arms, mainly (I thought) due to my work. I had been part of my husband's garden design business and carried loads far too heavy for me and suffered tennis elbow. Because of all this I had frequent treatments from a sports therapist who suggested that the class would improve my neck mobility. It didn't really help; I found the exercises very difficult and would strain my neck to lift it off the ball. On one occasion, the last, I felt strange in my neck and jaw; it was slightly locked. When I went to speak, the words wouldn't come out right - very slow and slurred. It was an enormous effort. This symptom worried me; it was certainly not just tiredness and I feared I had damaged a nerve in my neck. 

By mid March 2005 speaking was an effort. I had to compensate all the time. Actually I was not aware that I was doing it - the brain clearly accommodated the disability; I realized this some while later. My slurred speech gave the impression I was drunk, and in fact my boss was the first to comment, when he rang into the office one day and I answered with the usual company name (with two ell's in the word). He said, “Sue, have you been on the bottle?” I was worried but tried to laugh it off by saying something had gone awry.

I went to my GP and she noticed immediately something was wrong. She guessed it to be neurological and possibly a stroke. I couldn't see that being a likely cause, since nothing in my health would back up that theory. I was recommended to a neurological consultant, but had to wait 3 months. At this time, my mouth was very sore on one side only. Eating was starting to be arduous; I noticed when I was tired, my tongue didn't have the mobility it should.

She uses cranial-sacral therapy, acupuncture, and aromatherapy. She takes tomato juice for excess saliva, and Echinacea and Bach Flower Remedies. [Voice software interview].

Six months on I have cranial-sacral therapy, acupuncture, and aromatherapy massage on my face. My energy is improved with these treatments. Also the circulation to my hands is better and my sinuses are clearer. This is to improve my wellbeing and to cope with the MND. When I over-exert myself, I produce excess saliva. This is very troublesome and drags me down. At night I suffer from a dry mouth which is equally distressing.

I have tried various medications to relieve the excessive saliva, but none are effective. I am currently experimenting with eye drops taken orally. There is a slight relief which wears off by the afternoon. It is not troublesome when I am sitting or lying, so I conclude it's nature's way of telling me to rest. It comes on distressingly when I am exerting myself. By late afternoon it is worse. Tomato juice and eating by mouth seems to pacify it. By the time I was diagnosed in mid 2006, my laugh had changed. Currently, my laugh is a hilarious sound and people are unsure if I am crying or laughing. It is an uncontrollable noise, very loud, strange, and most embarrassing [laughs]. Crying is very distressing because it increases the saliva and it is easy to choke on it. When the saliva is really bad I become emotional.

I wear magnets at night to help breathing. These were offered by the local hospice and are invaluable. Apparently they have an anti-inflammatory effect so I use them to target muscle aches. I take Echinacea daily to boost my immune system, and Bach flowers - Oak and Star of Bethlehem. These help mood. I am also taking Vitamin E and make sure I have a glass of orange juice daily through the [PEG] tube.

She has thought of many things which might have caused her MND including injury, stress, dental...

I thought back over the previous few months for an explanation - could I have trapped a nerve in an attempt to do the exercise that was expected of me? The car hatchback lid dropped on my head 2 weeks previously - did this cause a trauma to my skull? I was suffering a certain amount of stress, in that I was main carer for my difficult mother-in-law - could this be part of the problem? My mother died in her 50s with cancer of the throat and for 4 years she could not speak. This is an uncanny connection. That was another fear for me - could this be predisposition to cancer?

In the previous year I had a vile reaction in my throat when the farmer was crop spraying opposite our house - could this be connected? Crop spraying had been going on for 30 years, but this was the only time I felt the reaction directly in my throat. My mouth remained sore after the spraying incident and it left me with sensitivity to strong smells, tastes, certain fruits, perfumes, some plants, chemicals, etcetera. I couldn't identify changes to any other parts of my body, simply the mouth and throat area. However, it suddenly opened my eyes to possible damage to our family's health; my husband suffered from neck and head aches for as long as I can remember and my twin daughters also have unusually sensitive muscles. Now, looking back, perhaps growing vegetables in our allotment alongside the treated field might well have put us all at risk.

I had face pain a few years ago connected with over-large fillings. When one was ground off a little after my protestations, the face pain disappeared. Was this a TMJ [temporo-mandibular joint] problem? Was the amalgam in my teeth leeching into my system? In 1999 I caught a virus and was unwell for one year and was advised to stop physical work and change my life style. Now, looking back, I believe I had ME [myalgic encephalopathy or chronic fatigue syndrome] - can this be a pre-cursor to motor neurone disease? In the light of my new problem, I went back to my dentist for his advice and he was certain that it was neurological rather than physical, even though he had to admit I had unusual face and neck aches over the years.

Searching for a cause helps people cope with MND, but it can become an obsession. She would like...

Many of us newly diagnosed patients search our memories for any clues as to what could have caused this neurological problem, such as genetics, lifestyle, environment, diet, harmful chemicals. Reading the website called 'Patients Like Me' it strikes me that MND patients never stop hoping for a cure and it's this that keeps them positive and able to cope with their worsening condition. We search for a cause from day one when our odd symptoms occur. Many people who suffer loss of limb function first of all focus on MS, ME, CFS etcetera, because these are the well-known disorders. This searching occupies their minds full-time and can become an obsession. Once diagnosed with MND (and probably this is the case with other neurological diseases as well) they continue the rest of their life searching for answers. Could old symptoms be a precursor to the disease, etcetera? In many cases they continue to wonder if they have been misdiagnosed. 

I want to see more public awareness of this disease, which is by no means a minority illness if, as I am told, seven in 100,000 at any one time have it, or whilst people are at work today, three new patients will be diagnosed. We need the media to focus on it as they did with cancer to remove the misconceptions and to forge ahead with more research. I would like to see more attention paid to the iniquitous use of chemicals in our daily life - they are a hazard to our health and we are only just beginning to see what damage they can do. More research is needed urgently. The Agro-industry and the Government are shutting their eyes to a link between crop spraying and illnesses and they simply declare there is no evidence. But how do we know there is no evidence if there is no research into the effects of chemicals on our health? There are health and safety regulations to protect farm workers, but no means of protecting the public who live alongside the areas of spraying. The spray chemicals are designed to kill the insect's neurons. Chemicals used in the farming industry surely progress through the food chain from crops to livestock to milk and meat etcetera. This is one aspect of the environmental issue that does not seem to be of concern to politicians, but is a growing concern to the public.

She couldn't tell staff how much pain she was in after the RIG procedure. She could still eat but...

I have only had one hospital stay so far, for gastrostomy, and I suffered a bad reaction - the recovery time was 3 months. The gastrostomy was thought to be necessary because we were planning to have a holiday of a lifetime to New Zealand and the hospital considered it would be sensible to have the stomach tube in place beforehand. The procedure was horrendous. I know everyone is different, and of course had I known too much beforehand I might have refused it.'Months further on, I believe improvements need to be made to prepare patients for the gastrostomy procedure. I am saying this for the benefit of improving care for future patients.

The ward nursing staff seemed to be unpractised with speech impaired patients, possibly due to temporary 'bank' nurses moving from ward to ward. But considering the ward is experienced at RIG and PEG procedures for MND patients, I was surprised they weren't more aware of how to handle a patient with speech loss.

I was in severe pain for 24 hours following the insertion of my stomach tube and, with my inability to talk, I was not given pain management until a young doctor witnessed my distress. Imagine being in severe pain and not being able to tell anyone about it. In fact I was ignored pretty much. On the sixth day the consultant wanted me gone, saying I would recover quicker at home and clearly MRSA was a real worry on the ward, but my husband refused, saying I was too ill, and the nursing staff agreed. So I stayed one more day and I was given an enema before I left. They insisted it was bad luck and probably due to the gas and constipation. If constipation has a major effect on the patient's recovery, could it not be organised through the GP and district nurse that prospective patients have an enema at home a day or so before surgery?

No one asked if I could eat or not, and presumably had no information about my pre-op condition, since I was not offered food, nor was I asked by nurses if I could eat. Early on they twice attempted food by a slow drip into the tube but my painful stomach rebelled. I was not offered food by mouth, but towards the end of my stay I requested some, because I was hungry. It would have been helpful if soup had been available at any time if I felt I could eat it, but it was not an option. I would have liked the staff generally to have been more aware of my condition and considered my difficulties. There were not enough skilled nurses.

The speech therapist and dietitian were caring and helpful and monitored my progress on the ward frequently, but the release letter from the ward did not arrive with the GP until almost a week later. We had to chase up the district nurse. There was no system in place for me.

No one seemed particularly concerned about trying to get me up and moving ahead of my departure from the ward. A physiotherapist should have been involved, because seven days of pain lying in bed made me extremely groggy and my diaphragm was weak. Perhaps I am expecting too much from the NHS.

I was told the stomach tube inserted was a RIG. It is done by a doctor in the Radiology Unit with nurses to assist. Gas is pumped into a tube via the nose to inflate the stomach to make for accurate placing of the tube. All the while I was able to follow the procedure on the monitor. Four injections of local anesthetic are given around the site, and the contraption itself is pushed through the numbed skin straight into the stomach. The connection cap is fitted on the surface of the skin just below the rib cage.

Four months on from the gastrostomy, the fog lifted and I felt more in control of myself again. I got back into dog walking every day, albeit only short distances, and also cooking meals.

We had to cancel the trip of a lifetime because I felt unwell after the gastrostomy and we considered it would have been a huge risk. However we have just had a very successful five-day driving trip to Germany. Because the roads are so smooth, it was not a tiring way to travel.
 

She misses being able to talk, laugh and shout, but she loves her voice software and the freedom...

Within a few weeks of the diagnosis, friends had provided me with a lightweight laptop with a text-to-voice programme using American software. I now have an English voice, 'Audrey', and she is speaking now. Because I have been a typist for years, it was second nature to convert to this way of communicating. Within 6 months I had forgotten what my own voice sounded like, and while typing I could forget my disability more or less. The amazing thing is that a young friend of mine (now 19), who as a child was unable to communicate effectively, heard of my diagnosis and felt a kinship with my new disability. It was he who found the software programme which enables me to speak via the computer. Trial voices can be downloaded from www.NextUp.com and tried for a short period free of charge. The software including the English voice costs under '100. The company is very helpful and prompt with their replies to queries. I have experienced mixed reactions from friends. Some consider Audrey is not unlike my old voice. But some friends prefer to sit alongside me and watch the typing on the screen, and have a conversation that way. I really do miss not being able to laugh or call or shout, but whilst I have the computer in front of me it does help me to forget that I cannot speak, and I feel less frustrated. Obviously it is not suitable for all occasions because of its size and the need to be sitting. For example, I cannot take the laptop into shops or out walking. But it is so useful for hospital and doctor's appointments. I can tell them in my own words rather than scribbling my questions and answers. I was offered a 'light-writer' at the Hospital which is free to patients and is funded by the MND Association, but it is old technology now and wasn't right for me because I am a typist [laughs]. The next generation are growing up with computers so future patients will need to be offered laptops and not 'light-writers'. I hope the MNDA will help fund them.

My message to other people living with MND is to learn to type because it is likely that as your illness progresses, you may lose the ability to speak. For people caring for a family member diagnosed with MND, try to be patient and sit down with them for conversations each day even if they can't communicate through a computer like me. Not being able to talk is so isolating. I really miss not being able to talk on the phone to my grown-up children and join in the family discussions over the dinner table. My 6-year-old granddaughter said just recently she misses the old Nanny, and followed it up with a lovely letter saying that she knows I may not get better and she is finding it difficult to talk to my computer. She is an excellent reader and she prefers to watch the screen. We have little games with the various American voices, listening to how words and numbers sound. This helps break down the barrier.

More needs to be done to raise awareness of the needs of speech-impaired people, and ways need to...

I have been incredibly lucky to have a friend who bought me a very good laptop and another friend provided the software voice. However that is not the end of it. Everywhere there are enormous difficulties. No one seems to realise there are people who can't talk. Deafness is so well catered for and recognised but there are no options for speech impaired people. The internet is my lifeline - my way of communicating with my friends, family and businesses day-to-day. But the businesses always insist on telephone numbers. How can we get them to be more aware of the speech impaired? We still have bank accounts and need all the things that we had before. There seems to be no hands-free telephone system ideally compatible for use with a laptop and voice. BT used to market one but it is not in production anymore. People like me need a handset with a good microphone that can sit or hook on to the laptop. It is crucial that the receiver hears the tapping on the laptop so they wait for the speech. I have a pre-defined phrase set up so that I introduce myself and my synthesised voice when I answer the call. But without the right handset, it is fraught with difficulties.

She has several tips to help with eating and swallowing. [Voice software interview].

Types of food and drink
Prior to gastrostomy, my local speech therapist only offered basic advice such as sipping iced water prior to having a meal. Swallowing liquids involves a double swallow, with the tongue in the roof of the mouth. Thickened juices rather like an unset jelly were provided by the GP, and I was not sorry to dispense with those. I was no longer able to kiss, blow, whistle or suck through a straw.
 
Currently all my meals are still by mouth. I am choosy of course. Boneless fish is very easy to eat, cauliflower cheese, thick homemade soups, jacket potatoes with tuna mayo, casseroles with finely cut meat. Now I take all thin liquids by the tube [PEG], along with ground up pills. I aim to have two pints of water daily. Liquids by mouth are bought vegetable juices, milkshakes and an occasional coffee or tea with sugar. I had instructions from the dietician to eat as much fattening foods as I like, full cream milk, ice cream, etc. To supplement my meals I also now take 200ml a day of a balanced nutritious drink through the tube. I prefer not to eat out, since it is embarrassing if I choke and I am now a messy eater.
 
Exercises to help strengthen weak mouth muscles (advice from a Speech & Language Consultant at a major MND Clinic)

• Blow cheeks out and hold for a few seconds. But I can’t do this one now without holding a towel to my nose and mouth to prevent the air escaping.
• This technique is handy for bowel movements!
• Jaw stretch. Open wide and shut jaw, repeat 10 to 20 times a day.
• Purse lips and then wide grin, repeat often (I can’t do this one either now)
• Cheek stretch. Put thumb inside mouth and stretch the cheek while pressing thumb against other fingers on the outside of cheek and pulling it forwards. I don’t like doing this one, so I neglect it but it does improve the stretchability of the cheeks.
• Be vigilant with teeth cleaning (Dentist’s advice)
• Carers, friends and family should learn the ‘assisted cough’ and mucus shifting routine
• Take in plenty of water

 
Posture and Position for Eating - to help chew food

• Sit in soft chair with head and neck supported on pillow. Adjust to a comfortable position to make swallowing easier
• Put finger in mouth to move food around and assist chewing. Believe me, you will get used to doing this. Best not when dining out!
• Request hydrotherapy from the local hospital. It is very helpful to strengthen muscles and improve diaphragm breathing.
• To keep sinus passages clear at night, sniff salt water or by dropper. This works just as well as Sudafed but not damaging long term. (Advice from the Hospice). Or Vaseline on a cotton-bud helps the dry nose.
• Use Glandsosane spray to moisten mouth as needed at night. Not the answer to the problem but does help. Actually I find dipping fingers in water and wiping them over tongue. I have used foam swabs but the texture is off-putting.

 
Drinking

• Take small sip & swallow twice or more with tongue in roof of mouth
• Or try holding cup to bottom lip and take lots of small sips without putting cup down. (This sometimes seem to work for me).
• Tomato or V8 vegetable juice to assist with saliva

 
Equipment to help

• Obtain a sucking machine for clearing saliva or food particles (community Physiotherapist provided mine free of charge as it’s on loan).
• Electric toothbrush
• Hand held electric mixer for reducing food to easy consistency. I find it is wonderful for mashing up food for smoothies

Her hospice is a place of kindness, giving practical and emotional support, including home visits...

The local Hospice has been marvellously supportive; within a matter of weeks after diagnosis I had a call from the specialist nurse for palliative care at the hospice. She described herself as a 'cage rattler'. She came to visit, and has done so every three weeks to give medical and moral support. I very quickly learned about the ethos behind the hospice and why I had been put under their care. It is NOT a place to die, as I had thought. It is a place where there is immense kindness and support for anyone with a life-threatening condition. They offer emotional support to the patient, their carer and families, and respite care and, ultimately, palliative nursing care. It was a revelation to me that they work in conjunction with the whole patient team - the specialists, the GP and community nurses. The nurse suggested I have a weekly visit at home from the hospice's aromatherapist to help my weak face muscles. Then she arranged for a representative from their Welfare Department to talk to us about finance and a Living Will (ADRT), which is something I was keen to set up. The Living Will (ADRT), is put into the Emergency Services database, so that if they are called out they will be informed of my wishes regarding resuscitation. These are all areas of the patient support network at the hospice. The welfare assistant was able to 'spearhead' the benefit system through the maze of forms and jargon, which would have defeated us. Because the applications are made on our behalf by the hospice, the Pensions Department hurry the decisions through. All the benefits have kicked in and were backdated some months.