Huntington speech music therapy. A therapy based on the principles

Huntington speech music therapy. A therapy based on the
principles of the speech music therapy for aphasia,
adjusted for patients with Huntington’s Disease
Marlies Brandt1, Maaike Nieuwkamp1, Eline Kerkdijk1, Els Verschuur1,2
1Huntington
2Faculty
Expert Center Atlant Care Group, Apeldoorn/Beekbergen, the Netherlands;
of Health and Social Studies HAN University of Applied Studies, Nijmegen, the Netherlands
Background
There are several explanations why music has a positive influence on
verbal communication. It is known that the brain systems underlying to
music are shared with other functions such as speech. Evidence
suggests that music may activate these systems in a different way than
speech or other stimuli and enhances the way these systems work
together.
Huntington Speech Music Therapy (HSMT), based on the principles of
Speech Music Therapy for Aphasia (a treatment program for people with
aphasia and/or verbal apraxia), focuses on improving articulation and
clarity of speaking, improving use of voice, volume of speaking,
breathing and tempo, improving the fluency of speaking and
stimulating/activating the ability to speak.
Case History
In a joint session, the speech therapist and music therapist offer patients with Huntington’s disease (HD) the HSMT
program. In- and exclusion criteria are shown in table 1. The speech therapist instructs patients to perform words and
sentences accompanied by structured, repeating short melodies. The music therapist plays these melodies on the
piano. By continuously using the same melodic lines the music provides structure, which facilitates production and
reproduction of language (Table 2). HSMT therapy turns out to be very valuable for our HD patients. One patient
stated: “When I participate in the therapy there is enough time and quietness and I feel at ease. I can practice and
prepare for difficult and complex conversations. It helps me to structure the information. In daily life I don’t speak much
anymore but during this therapy I feel stimulated to talk”.
Table 1 In- and exclusion criteria
Inclusion criteria
Table 2 Huntington Speech Music Therapy program
Exclusion criteria
Patient has impairment in verbal Patient is not able to speak
communication which leads to
lack of intelligibility and/or
spoken language production
Patient has sufficient
comprehension of language
Patient is motivated
Patient has an affinity for music
Patient can not follow-up
instructions and advice due to
cognitive decline, apraxia and/or
fatigue
Patient has a short attention
span, is quickly distracted and
has difficulties focusing on tasks
Patient is too much focused on
the music and is emotionally
touched, influencing
concentration
Structure of the HSMT program
Automatic sequences
Greeting, counting, days of the
week, months of the year,
alphabet, et cetera
Patient-related words and
sentences
Names of significant others or
persons who are important for
the patient, topics of interest
and/or hobbies, important (life)
events, patients’ opinion on
various topics, et cetera
Conversation with music
Conversation on current topics or
on topics from the news
Conclusions
HSMT improves speech and communication of HD patients during treatment.
Correspondence
Marlies Brandt
Music therapist
[email protected]
www.huntingtonexpertisecentrumatlant.nl
Delay of institutionalization by offering an overnight stay
to HD day care patients
Lia van Gelder1, Joke Westrik1, Karen Lammertsen1, Els Verschuur1,2
1Huntington
2Faculty
Expert Center Atlant Care Group, Apeldoorn/Beekbergen, the Netherlands;
of Health and Social Studies HAN University of Applied Studies, Nijmegen, the Netherlands
Background
Huntington Expertise Centre Atlant offers multidisciplinary care to 50 Huntington disease (HD) patients
institutionalized in 2 Skilled Nursing Facilities (SNF) and to 25 ambulatory patients. These ambulatory
patients visit our day care centre on a regularly basis.
Figure 1
Only a 5 min. drive from
over night stay to day care
Several HD patients suffer from fatigue due to traveling to
and from the day care centre. In addition, informal care givers
often feel overburdened being 24/7 responsible for the care
of their next of kin. This may lead to unnecessary early
admissions of HD patients. In general, admission to a SNF is
a major step for HD patients. In order to delay admission and
to relieve the burden of the informal caregivers, we
developed an overnight stay facility, only 5 minutes by car
away from day care (Figure 1). In this facility, HD patients can
stay 1-2 nights per week; 2 consecutive nights or 2 nights
distributed over the week as appropriate.
Case histories
In this case report, we describe 6 HD patients using the
overnight stay facility. The characteristics of these patients
are shown in table 1. Given the physical condition and the
emotional distress of their informal caregivers, these
patients faced immediate admission. By offering the
possibility of overnight stays, the patients were in a better
condition participating their day care program. In addition,
they had the opportunity to get used to the SNF, so that
eventually admission would be a logical next step. The
informal caregivers experienced less burden and they had
some more time for themselves. Patients and caregivers
were very satisfied with this intervention: it may have
delayed admission for 2-11 months.
Table 1 Characteristics of 6 HD day care patient using the
overnight stay facility
Age in years; mean ± sd (range)
Gender; no. of patients (%)
- male
- female
Overnight stay in days until admission;
mean ± sd (range)*
Nights per week; no. of patients (%)
- 1 night
- 2 nights
n=6
53.7 ± 7.7
(42.7-62.6)
3 (50)
3 (50)
225 ± 113
(70-328)
1 (17)
5 (83)
* 2 patients are still living at home
Conclusions
The overnight stay facility may delay admission in a SNF by a mean of 7 months. Our HD patients were
more rested and better able to undergo their treatment, and institutionalization seemed to be a smaller
step for these patients. In addition, informal caregivers were able to care for their next of kin for a longer
period.
Correspondence:
Lia van Gelder
Unit manager
[email protected]
www.huntingtonexpertisecentrumatlant.nl
Antipsychotics and metabolic syndrome in advanced
Huntington’s Disease
Tom Stor1, Agaath Bruin1, Els Verschuur1,2
1Huntington
2Faculty
Expert Center Atlant Care Group, Apeldoorn/Beekbergen, the Netherlands;
of Health and Social Studies HAN University of Applied Studies, Nijmegen, the Netherlands
Background
Metabolic syndrome may occur as an adverse effect of both typical and atypical antipsychotics (AP). It may
reduce life expectancy and quality of life of patients. In mental health institutions, protocols describe to
perform blood tests and somatic screening in patients who are taking antipsychotics. When complications
occur, psychiatrists switch antipsychotic or adjust doses. These protocols are rarely used in nursing homes,
due to limited life expectancy of the majority of patients. AP are often prescribed to patients with Huntington’s
Disease (HD) to reduce chorea, aggressive behavior or psychotic symptoms. Since diagnoses, HD patients
have a life expectancy of up to 20 years. Little is known on prevalence of metabolic syndrome in HD.
Aims
Table 1 Criteria metabolic syndrome of the USA NCEP
To determine the prevalence of metabolic syndrome in
patients with Huntington’s Disease on antipsychotics.
1. Abdominal obesity: male > 102 cm / female > 88 cm
2. Triglycerides ≥ 1.7 mmol/l
3. HDL cholesterol: male < 1.0 mmol/l / female < 1.3 mmol/l
4. Glucose ≥ 6.1
5. Blood pressure ≥ 130/85
Methods
We performed blood tests in 28 institutionalized HD patients. In addition, we assessed blood pressure, BMI
and abdominal circumference, risk factors such as smoking and family history for diabetes and cardiovascular
disease.
Results
Table 2 Characteristics and outcomes of 28 HD patients on
antipsychotics
Male
Female
n=15
n=13
Age in years;
mean ± sd (range)
51.1 ± 9.0
(31.9 – 67.1)
57.6 ± 9.1
(35.3 – 67.4)
Metabolic syndrome;
no. of patients (%)
4 (27)
6 (46)
New diabetes;
no. of patients (%)
0 (0)
2 (15)
Raised Prolactine levels;
no. of patients (%)
5 (2)
0 (0)
Twenty-eight HD inpatients were on AP drugs and
expected to live at least 5 years. Of these patients,
10 (36%) patients met the criteria of metabolic
syndrome of the US National Cholesterol
Education Program (NCEP) (Table 1). Seven
(25%) patients had the combination of elevated
triglycerides levels and reduced HDL levels (Table
2). Five (18%) patients (all male) on atypical AP
turned out to have slightly raised prolactin levels.
Four (14%) patients met the criteria of type 2
diabetes, and started a diet and/or medication.
Finally, 2 (7%) patients had mild gynecomastia.
Conclusions
We found a high prevalence of metabolic syndrome in HD patients with AP. The type 2 diabetes was
presented as atypical, without the usual symptoms such as polydipsia, polyuria and weight loss. We expected
to find high cholesterol levels due to the diet that is enriched with kcal and fat many HD patients have to
prevent weight loss.
Correspondence:
Tom Stor
Elderly Care Physician
[email protected]
www.huntingtonexpertisecentrumatlant.nl
THE USE OF A BALL BLANKET FOR PATIENTS WITH
HUNTINGTON’S DISEASE
Manon van Kampen1, Yvonne Pas1, Mariska van Maanen1, Els Verschuur1,2
1Huntington
2Faculty
Expert Center Atlant Care Group, Apeldoorn/Beekbergen, the Netherlands;
of Health and Social Studies HAN University of Applied Studies, Nijmegen, the Netherlands
Background
In the Netherlands it is no longer allowed to use freedom restricting
measures such as fixation of patients in bed. However, risk of falls,
mental and physical restlessness in bed are common problems in
patients with Huntington’s Disease (HD). The occupational therapists of
Atlant Care Group are, together with the nursing staff, looking for safe
alternatives.
A “ball blanket” seems to be a good alternative for maintaining the
safety of HD patients. This blanket is filled with plastic balls and/or
polystyrene granules (Figure 1). It stimulates the sensoric system and
improves physical wellness in HD patients. The weight of the balls
provides pressure on the pressure points of the body allowing different
senses to be stimulated, such as stimulation of the sense of touch,
muscles and joints. This stimulation has a suppressive and regulatory
effect on impulses to the central nerve centre which increases body
awareness.
Figure 1
and/or
Ball blanket with plastic balls and
polystyrene granules
Case History
We offered the ball blanket to 7 HD patients (4 males; mean age 51 years [range: 33-66]) with nocturnal
restlessness due to physical and mental limitations.
These limitations were: difficulty to relax, severe chorea, increased muscle
tone and increased alertness, frequently getting out of bed and problems with
staying asleep.
All HD patients experienced a benefit of the ball blanket. They felt safe and
more relaxed (Figure 2). The chorea decreased and breathing became
calmer and deeper. Due to the ball blanket, patients became drowsy which
made it easier for them to fall asleep for a few hours or even the whole night.
Figure 2
Henk, very relaxed due to
his blanket
The nursing staff is also very positive using the ball blanket. One nurse
indicated: “It is so good to see that our HD clients are comfortable and
relaxed by the sense of security the ball blanket offers. It is remarkable to see
the results, …. almost immediately, …. even before I am leaving the
apartment”
Conclusions
Considering our experiences, the ball blanket is likely to be of benefit for HD patients. The blanket may have a
positive effect on quality of life. However, further research is needed to establish whether the blanket is beneficial
for all HD patients.
Correspondence:
Manon van Kampen
Occupational Therapist
[email protected]
www.huntingtonexpertisecentrumatlant.nl
Vitamin D deficiency in institutionalized patients with
Huntington’s Disease
Marco Loffredo1, Tom Stor1, Agaath Bruin1, Els Verschuur1,2
1Huntington
2Faculty
Expert Center Atlant Care Group, Apeldoorn/Beekbergen, the Netherlands;
of Health and Social Studies HAN University of Applied Studies, Nijmegen, the Netherlands
Background
Elderly dementia patients are prescribed cholecalciferol (vitamin D) when institutionalized in one of our wards.
For this, we follow guidelines from the Dutch Health Council which are evidence-based. In the Netherlands,
no large-scale research or guidelines are available on Vitamin D deficiency and supplementation in
institutionalized patients with Huntington’s Disease (HD), Korsakov’s syndrome or Acquired Brain Injury. Chel
et al. (Dermatoendocrinol, 2013 5(3):348-51) found high prevalence of vitamin D deficiency and insufficiency
among 28 HD patients admitted in a Dutch Skilled Nursing Facility.
Aims
To determine the prevalence and level of vitamin D deficiency in institutionalized HD patients.
Methods
In 21 institutionalized HD patients we performed blood tests to determine the level of Vitamin D (serum
25(OH)D level). In these patients, blood samples had to be drawn for other indications, and the vitamin D
level was assessed at the same time.
Results
All HD patients (mean age 55 yrs.; male n= 11
[52%]) had advanced disease with a life
expectancy of at least 5 years. We found
deficiency in all patients included (Figure 1).
Mean vitamin D level was 25 nmol/L; with a
range of 3-51 nmol/L (reference values 50-150
nmol/l). Some of these patients are on a high
kcal diet, adding drinks and/ or meal
replacements. Despite extra vitamins (including
vitamin D) added to their daily intake, the vitamin
D levels were low.
Vitamin D serum level in nmol/l
80
Target level
70
60
Minimum level
50
40
30
20
10
0
1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
18
19
20
21
Figure 1 Vitamin D serum levels of 21 HD patients
Conclusions
Vitamin D deficiency is common in advanced HD. We recommend to perform lab tests at admission in a
home care facility, and to prescribe vitamin D to all HD patients with deficiency on a monthly basis.
Correspondence:
Marco Loffredo
Elderly Care Physician
[email protected]
www.huntingtonexpertisecentrumatlant.nl

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