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Points for Reflection

1. Discuss the issues raised for Simon’s parents

2. How could you support them as a healthcare professional?

Activities

1. What are Simon’s care needs?

2. How might these differ from other chronic conditions that you have come across in your practice? (Reflect on the experiences you have had with your patients).

3. Further reading: Alex: The life of a child by Frank Deford (1983) Viking Press, New York. In this detailed and moving account of his daughter, Alex, Frank Deford writes about how the family coped with her cystic fibrosis until her death at the age of eight.

Quotes

"The professionals who made a difference… were teachers, either by example or by intent, skilfully passing on their knowledge to those with whom they worked."

Further information

Support groups include the Niemann-Pick disease group UK(http://www.niemannpick.org.uk/) and the Niemann-Pick Disease Foundation, Inc (http://www.nnpdf.org/).

US National Library of Medicine, Genetics Home Reference: Niemann- Pick

National Institute of Neurological Disorders and Stroke: Lipid storage disease fact sheet and information on Niemann-Pick

GP notebook: lipid storage diseases, including information on Niemann-Pick.


Location: The Stories
Transcript

You can download the full transcript here

Consider the carer: what are they doing when they are not attending clinics?…”
A diary account of caring for Simon, a child with the autosomal recessive condition Niemann-Pick (Type C)

A Life in the Day of a carer

Our son died aged nine, on the 29th August 2000, from the rare degenerative disorder Niemann-Pick (Type C). Our daughter is now twelve. My wife was asked to keep a diary of a typical day. This is a typed copy of the original hand-written document. My son was eight years old when this account was written.

Activities over a 24hr period

04.05 - 05.15 Attending to Simon who was in distress.

06.30 - 06.40 As above.

07.00 - 07.40 Simon had tonic clonic. Also had bowel movement so required changing.

08.00 - 08.45 Flushed-off Simon, attended to gastrostomy pump and giving-set. Gave medicines. Washed, and dressed Simon.

09.30 - 09.40 Moved Simon downstairs. (From bed to castered chair on landing, to stair-lift, to wheeled chair in lounge, to sofa in care area. Adjusted pillows and made comfortable.)

09.40 - 09.50 Had breakfast; told our daughter to finish breakfast and get dressed.

09.50 - 09.55 Put ironing board away. Checked post.

09.55 - 10.15 Simon had bowel movement; cleaned and changed him.

10.15 - 10.30 Simon had coughing fit; cleaned and settled him. Cleaned and flushed gastrostomy tube (to avoid blockage).

10.30 - 10.35 Took 'photo of our daughter, who'd dressed up as a princess. Checked and refilled Simon changing/equipment bag. (Delayed twice by Simon choking/coughing).

10.35 - 10.40 Dried pots from yesterday. Put items away in cupboard.

10.40 - 10.45 Rang lady from sitting service.

10.45 - 11.00 Washed up (for today).

11.00 - 11.05 Tidied up. Attended to personal needs.

11.05 - 11.55 Played with my daughter (beads); supervised Simon.

11.55 - 12.15 Cleaned kitchen work surfaces; emptied toaster of crumbs; swept floor.

12.15 - 13.00 Made and had lunch. Set up Simon feed.

13.00 - 13.15 Washed up. Gave Simon his medication.

13.15 - 13.30 Flushed off Simon. Attended to pump and giving set.

13.30 - 14.00 Played with our daughter and Simon.

14.00 - 14.15 Supervised children.

14.15 - 14.30 Changed Simon and attended to his needs.

14.30 - 15.00 Supervised children. Gave Simon medication.

15.00 - 15.15 Dressed Simon and put him in buggy. Got our daughter ready

15.15 - 16.00 Went to Post Office for Invalid Care Allowance with children.

16.00 - 16.30 Removed Simon buggy, undressed, put on futon. Set up feed.

16.30 - 17.15 Made tea.

17.15 - 18.00 Had tea. Supervised children.

18.00 - 19.30 Sat with Simon and kept him occupied.

19.30 - 20.00 Took Simon upstairs (futon/wheeled chair/stairlift/castered chair/bed), laid him on bed. Took our daughter upstairs.

20.00 - 20.30 Bathed our daughter, got her changed and into bed

20.30 - 21.00 Occupied both.

21.00 - 22.20 Medications (x3). Set up feed. Changed, washed Simon, pyjamas on.

22.20 - 22.25 Washed up.

22.25 - 22.35 Did Milton. Gave Simon his inhaler.

22.35 - 22.40 Sorted airing cupboard and tidied our daughter's room.

22.40 - 23.00 Read the paper.

23.00 - 23.30 Sorted and prepared Simon school bag.

23.30 - 23.50 Bath.

23.50 - 24.00 Wrote card.

24.00 - 24.20 Two sewing jobs.

24.40 - 06.45 Bed. (Up again at 6.45 for Simon).

This is not an exceptional day of unusual, extra-ordinary demands. It is an ordinary day of the USUAL exceptional demands

How has the illness of your child changed your life?

We have been married for 16 years and have had two children: Simon (who died aged nine, on the 29th August 2000, from the rare degenerative disorder Niemann- Pick Type C) and our daughter, who is now twelve.

Simon's father was asked to do a presentation (to a group of health professionals) which would highlight the difficulties carers face. I wrote this poem as an introduction. It expresses my feelings of isolation and desperation. It was used with a series of questions to stimulate a discussion on care issues.

The Prisoner.

From my childhood

I remember a scene in a history book.

A man in chains sits in the centre of a cold flagged floor, and as if on stage,

is encircled by a pool of light,

which streams into the gloomy cell from a small window, high above.

The thin, tapering cone of brilliant light

is all that links him with the outside world.

Its soothing warmth thrusts aside the cold darkness of the room

and reminds him of the other world that once was his;

a world of freedom, enjoyment and friendship.

But now I am there, for I am that person in the room,

imprisoned not because I have erred,

but because I choose to care.

The picture in my mind is now my reality:

For I am a carer.

Discussion points raised by the poem

• What are the “chains” that bind the carer?

• To what extent are these restraints invisible to others?

• Why might the carer feel invisible?

• Why does the carer believe the room to be “gloomy” and “cold”?

• What might “The thin, tapering cone of brilliant light” represent both in abstract and concrete terms?

• Why is there only a memory of:- “the other world that once was his; a world of freedom, enjoyment and friendshp.”

• Do carers have a choice as to whether or not they become prisoners?

Telling Stories, Understanding Real-Life Genetics

Our son died aged nine, on the 29th August 2000, from the genetic, degenerative disorder Niemann-Pick (Type C). He was diagnosed at the age of four, after he had started to display developmental delays. Initially, we had great difficulty in obtaining accurate information about the condition, possibly because of the low number of sufferers. Eventually we discovered that the condition is rare and can affect adults but occurs most frequently in children of school age. Life expectancy varies considerably.

Families with a disabled and ill child are far less resilient than other families. They are, in effect, “disabled” by the rigid timetable of care routines. This unfortunately results in varying degrees of depersonalisation, brought about by:-

Lack of sleep.*

Loss of privacy and personal space.*

Loss of the opportunity to be creative and reflective.*

Loss of freedom of choice in personal and family matters.*

Loss of leisure activities, including holidays.*

Social isolation.*

* (2004 Blackwell Publishing:)

We found that the professionals who made a difference to our son’s quality of care exhibited certain characteristics. These were as follows:-

A true professional was a person who was empathetic and well organised. They found their work intrinsically rewarding and were therefore motivated to provide a high quality service. They made a positive and even memorable contribution to the lives of the patient, the siblings and the parent-carers.

They were individuals who were prepared "to go the extra mile" and do things which were beyond their basic job description.

We found they had initiative, were usually well qualified and were experienced and skilful in working with children. They were also reliable, trustworthy and consistent, recognising that when on duty, patient care should come before personal convenience.

In order to work successfully within the domestic setting, they recognised the need to be present but not conspicuous. Being polite, patient, resourceful, conscientious, creative, supportive, friendly and helpful facilitated this. The fact that they were, in addition, modest, gentle, caring, interested in and clearly concerned about the patient was reflected in their bedside manner and the way they treated the parent carers. They were careful to keep to a minimum any disturbance to the patient and avoid distress and pain.

Generally, their positive and optimistic outlook, together with a quiet humour, had a positive effect on the moral of the patient and the carers.

They were inspirational, in that they made others aware of just how much could be done and even did what at first sight seemed to be impossible.

They were teachers, either by example or by intent, skilfully passing on their knowledge to those with whom they worked. They valued the patient and the carers, recognising, encouraging and developing the carer's knowledge, skills and personal qualities.

Often the people who made the greatest contribution were those who did not realise how skilful they were.