APPENDIX 3
Memorandum by the Progressive Supranuclear
Palsy [PSP-Europe] Association (CC 06)
1. The Progressive Supranuclear Palsy [PSP-Europe]
Association was established in April 1994. The three main objectives
of the PSP Association are to:
— promote and sponsor research worldwide
into the cause, effective treatment and eventual cure of PSP;
— provide information and support for
PSP afflicted families across Europe; and
— engender awareness, particularly
amongst relevant medical professionals, of PSP and of the Association,
mainly within the UK.
2. PSP is a little known neurodegenerative
disease, involving the progressive death of neurons (nerve endings)
in the basal ganglia and brain stem, just above the nuclei—hence
supranuclear. The cause is not known and there is, as yet, no
effective treatment and no known cure. PSP affects progressively
a person's vision, balance, mobility, speech and ability to swallow.
It can also cause personality changes, incontinence and behavioural
problems. Average life expectancy from onset is some seven years,
the last two of which are often spent wheelchair or bed-bound,
tube fed, on 24 hour care, unable to communicate with the world
around, although with intellect largely intact. It mainly affects
those aged over 50, but PSP patients still in their 30s have joined
the PSP Association.
3. Leading neurologists estimate that there
could be up to 10,000 cases of PSP in the UK today. However, correct
diagnosis, especially in the early stages of the condition, is
difficult and until recently many health professionals were unaware
of the disease. Misdiagnosis and non-diagnosis is still common.
PSP is at least as nasty as and recent research confirms at least
as common, in the UK, as its far better known "cousin"
Motor Neurone Disease. It is the disease from which the British
born actor and comedian Dudley Moore suffered.
4. At some stage during the progression
of the disease, and usually by the time a person is admitted to
a care home, he or she will have become helpless. They will have
to be put to bed and lifted from the bed, perhaps into a wheelchair,
dressed, fed, toileted, bathed, turned regularly in bed and wheelchair
to avoid sores and discomfort. As various bodily functions
close down, complex and seemingly random symptoms will appear
unpredictably. These often need to be dealt with by a NHS multi-disciplinary
team informed by, and often under the guidance of, neurological
expertise. Since there is no treatment and no cure for this disease,
the requirement is for informed palliative care in its later stages.
5. The PSP Association has been increasingly
concerned about Continuing Care criteria and their operation over
recent years. We have received a large number of requests for
help with understanding the situation with regard to NHS Continuing
Care. In addition our members have reported, on a number of occasions,
funding decisions that appeared unfair and inexplicable given
the nature of PSP and the needs of people with the condition (see
annex for two anonymised case studies). It is quite a common experience
that funding is eventually agreed after relatives have sought
an appeal and argued their case.
6. A particular problem for people with
PSP and their families is the rapid deterioration in physical
health that the condition usually causes. Decisions on funding
for continuing care need to be made speedily and without the bureaucracy
of appeals systems and reviews. Relatives are often left trying
to sort out the case even after he person with PSP has died.
7. The PSP Association would like to see
the following questions addressed by the Committee's Inquiry in
relation to the Minister's statement on 9 December 2004:
(a) How will the new proposed continuing
care framework ensure that decisions can be made speedily?
(b) How can the Minister ensure that consistent
criteria can be APPLIED consistently across the country?
(c) What plans are there to communicate clearly
to people involved in seeking continuing care funding what the
criteria are and how they are applied?
(d) What is the timescale for establishing
the new framework?
February 2005
Annex
ILLUSTRATIVE CASE STUDY OF WOMAN WITH PSP
WHO HAS NOT YET BEEN ACCEPTED FOR CONTINUING CARE FUNDING
1. About six years ago, my wife began to
experience a loss of balance and had several serious falls, two
of which led her into hospital. She was otherwise healthy and
young for her age, with no significant age-related problems. It
took about two years to get a diagnosis of PSP. By then, she used
a wheelchair but could walk short distances with my support. Generally,
I carried her from room to room. I had to turn her regularly at
night and she developed a disease-associated bladder problem,
so we rarely had more than three hours continuous sleep in over
two years. The muscles controlling her eye movement failed; she
was unable to scan and thus unable to read. She sometimes became
confused, forgetful, irritable, depressed or agitated—all
typical symptoms of the disease.
2. Towards the end of 2002 she suffered
a disease-related event which led her into our local General Hospital
and then two more hospitals. After three months she came home,
but the NHS insisted that she was their responsibility and would
release her into my care only when they were satisfied that an
adequate care package was in place. By then, I had developed a
spinal problem as a result of carrying her and, because I could
no longer lift her, after a few weeks she had to go into a nursing
home.
3. Her NHS neurological consultant said,
about 14 months ago, that he doubted she would be alive in two
years and not at all surprised if she were gone within one year.
Her condition has deteriorated dramatically recently. She is skeletal
thin and has gone from a ladies' dress size 16 to a Size 8-10,
decreasing. She is totally helpless and unable to do anything
for herself. She has to be put to bed and lifted from bed into
a wheelchair, dressed, fed, toileted, bed-bathed and have her
nose blown when necessary. Because she cannot move from one buttock
to the other unaided, she has to be turned regularly in bed and
wheelchair. Although her intellect remains active and intelligent,
she can neither read nor operate a radio or television set. She
cannot see or speak very well and finds it difficult to complete
a sentence.
4. When she was first diagnosed, her NHS
neurological consultant told me that there were no drugs that
would save her or ameliorate her condition. He said that her treatment
would be palliative and reactive. That is to say, the treatment
would be managed to ease her discomfort and shield her from unavoidable
harm. Since the progress of the disease is unpredictable and complex,
symptoms of further functional failure would be treated as they
arose by NHS multi-disciplinary specialists. That is what has
since happened.
5. When not in bed, she is always in a wheelchair.
She must be correctly positioned in the chair and repositioned
frequently to avoid sores and discomfort. The chairs are produced
by the local NHS Wheelchair Service; their specialists brief the
care staff at the Home on the precise and essential procedures
for positioning and restraining her.
6. The muscles in her throat are failing
so she has progressive difficulty in swallowing and there is a
danger of choking. This will probably soon lead to peg-feeding.
She is hand fed at present; solids are cut up, liquids are thickened
and fed through a straw. This deterioration in swallowing is monitored
regularly by the NHS Speech Therapist from a local clinic, who
advises staff at the Home on the correct food and drink and how
it is to be given.
7. She developed a severe dermatological
problem and was referred by her NHS neurological consultant to
an NHS dermatology consultant at a local hospital. That lady prescribed
certain medical and moisturising creams and unguents which are
administered by the care staff.
8. To deal with her agitation, depression,
mood swings, and the tremors caused by the palsy, her NHS neurological
consultant recommended certain sedative drugs which were then
prescribed by her NHS GP.
9. The local NHS Incontinence Unit treated
her bladder problem.
10. She developed Type 2 Diabetes, which
was treated initially by the local Diabetic Clinic and is now
controlled by diet on the advice of her NHS GP.
11. She suffers progressive numbness and
loss of use in her left arm, an effect which is moving into her
left leg and will eventually involve both arms and legs. It is
a typical symptom of the Cortico Basal Degeneration (CBD) variety
of the PSP from which she suffers. She has, from the beginning,
been treated by NHS physiotherapists, both hospital and practice,
and is now visited regularly by the NHS Community Physiotherapist
from a local health centre.
12. As her eye problems developed, she was
referred to an NHS consultant ophthalmologist.
13. My wife's primary need is a health need.
She is in a nursing home because she has a disease. Her treatment
needs are medical and palliative. She has virtually no social
or cosmetic needs. Her accommodation needs are trivial: a small
room and a bed and a little food, the cost of which could probably
be covered by her State Pension.
NOTES ON A WOMAN'S ACCOUNT OF HER HUSBAND'S
CASE (AS REPORTED) TO PSP ASSOCIATION)
14. In 2000 her husband, then aged 72, was
diagnosed with Parkinson's disease (PD). In 2003, after many often
horrific falls, usually backwards and with other PSP type symptoms,
her husband took an overdose to commit suicide as he had had enough.
He was admitted as a PD patient to a local hospital and remained
there for nine months. His wife wanted to nurse him at home, but
Social Services, she said, were unhelpful and pressured her into
accepting that he should be transferred into a Care Home.
15. He suffered there, because his symptoms
(typical of PSP); rigidity, staring eyes, falls, difficulty in
swallowing etc were not symptoms with which they were familiar.
His condition worsened. He did not want to be tube fed. His wife
took him home.
16. A short while later, when he had recovered
a bit, he cut his wrists in another attempt to "end it"
and was then readmitted to the hospital. He was seen by the Registrar
there who suspected he had PSP and not PD. He was then taken to
a hospital with more neurological expertise and examined by a
neurologist, who put on his notes that he also felt the man had
PSP; but referred him to an eminent neurologist (who is about
to retire) to make a deciding diagnosis (not yet made).
17. Meanwhile, the original hospital assessed
him and turned him down for Continuing Care and wishes to discharge
him back home or into care. The wife is appealing against this
decision, because she felt the criteria were not interpreted fairly
(they still have not confirmed that he has PSP, although all the
evidence points to this). The fact that the hospital is some one
million pounds overspent, makes her feel that he will be rejected
on appeal, although there are the strongest grounds of complex
neurological need.
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