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Curative treatment; physio- and psychotherapy for sarcoidosis patients


Prof. Dr. med. Wurm, Dr. med. K. Kögler: experiences at Höchenschwand


1. Introduction and history

At a seminar at Bad Buchau Prof. Dihlmann from Hamburg Barmbek said approximately:

Medicine is no exact science.

Exact sciences are based on the laws of mathematics, which, as the computer, know nothing but “zero” and “one”. However, in medicine all fractions between zero and one do exist. This means that medicine is an experience-based science and experience ensues from errors, both the own ones and those of others, which are recognized.

When Prof. Wurm took over in 1950 the internistic treatment of patients suffering from inflammatory eye affections, above all “eye tuberculoses”, he detected in the X-ray imaging that number of them presented a double-sided hilus cell hyperplasia.

Yet, the clinical report indicated neither tuberculosis nor a malignant affection, for example in the sense of “Morbus Hodgkin”. These were cases of sarcoidosis. At that time it was considered a special form of tuberculosis and the patients were sent to a sanatorium at the expense of the public pension insurance. This way numerous sarcoidosis patients came to Höchenschwand (meanwhile more than 18.000 patients have been treated, partly several times).

The analysis of the successive X-ray diagnoses and the clinical observation led to the introduction of phase subdivision scheme after WURM, REINDELL and HEILMEYER, set up according to dynamic aspects in the sense of evolutionary laws specific to sarcoidosis. This was contradictory to the static subdivision conceived by Dressler (1942) according to 4 types of X-ray diagnoses.

It ensues from the evolutionary laws with regard to pathogenesis and aetiology, that sarcoidosis – in contrast to all other infectious diseases – is not caused by pathogens, but that it is due to genetic factors. However, the triggering agent is still unknown.

The still often employed thoughtless expression “Sarcoidosis of unknown genesis” ought definitely to be abandoned.

The scientific work of Prof. Wurm led subsequently to an unprecedented afflux of sarcoidosis patients. This way, Höchenschwand, the little village in the Black Forest, became an internationally known health resort.
 

2. Description of the health resort:

The village of Höchenschwand obtained the label “climatic health resort” in 1939. This means that the climatic features play an important role at Höchenschwand. The health resort can boast a bracing climate of level 3, the highest level which exists in Germany.

The health resort is situated at a height of 1015 metres on a mountain crest from where the slopes fall by about 700 metres down to the upper Rhine valley at the Swiss border within a distance of about 10 kilometres. Thanks to the situation on the heights, Höchenschwand benefits annually from 1848 sunshine hours on average, being the sunniest village of Germany.

The frequency of sunshine combines with the intensity of the solar radiation, given the height and the pure air.

It was already in 1965, when a scientific study, conducted by the “Institut für Balneologie und Klimaphysiologie der Universität Freiburg” (institute of balneology and climatic physiology), revealed that the cortisol production of the organism is significantly elevated in the bioclimatic conditions of Höchenschwand in comparison with the upper Rhine valley (Bad Krozingen).

According to studies of the ophthalmological University Clinic of Munich an increased cortisol production can be observed subsequent to a cataract nucleation; this means when light reaches again the eye background on a vision nerve via the hypothalamus-hypophesis. This increased cortisol production has a wide variety of impacts on the functions of the organism and in particular a positive effect on the mood. This reminds us especially of the autumn and winter depressions in Scandinavia, which are meanwhile treated with a light therapy, given this knowledge.

The air cleanness meets a primary need of the patient, in particular for those who live in urban centres. The situation of Höchenschwand above the inversion layer and the prevailing slight breeze provide for a constant air exchange.

The air temperature remains relatively moderate throughout the year with an annual average of +6,4 °C, an average maximum of 26,5 °C an a monthly minimum of -13,5 °C in January.

The atmospheric inversion which occurs in high pressure periods, being characterized by warmth, sunshine and a clear view on the heights in contrast to the cold in the Rhine valley under the fog layer. This phenomenon is for many guests a great experience.

The “cooling figure”, which depends on the temperature and the air current speed, is a crucial factor for the “bracing” intensity of the climate. At Höchenschwand an absolute absence of wind occurs rarely. The almost permanent slight breeze impedes sultriness even in summer.

Given the geographic situation the precipitation rate is relatively low. Precipitation reaches a maximum in December and hits the bottom in February. Fog is a rare event. All this contributes to a wholesome dry air. Snow can fall already in October. Normally, the landscape is snow-covered from Christmas to the end of March, with the blanket reaching on average 80 centimetres.

Beside the described physical properties, other factors, though being partly still insufficiently studied, have to be taken into account, such as atmospheric electricity, the earth magnetism and natural radiation as well as the positive effect of radiation hormesis.

The physiological impact on the human being is always the result of different simultaneous influences. The effects depend moreover on the individual physical and mental sensitivity.

The benefits of the healthy climate consist above all in the stimulation of the defence mechanisms of the immune system. This results not only in an acceleration of the healing process, but also in a reduction of the vulnerability to illness, what is commonly called “reversal of predisposition”.

The landscape is the second most important specific healing factor at Höchenschwand after the climate. The health resort owes its leading position to the situation of the village with its still rural character amidst a charming landscape combined with the climatic assets. In this unobstructed countryside without any industry composed of regularly alternating meadows, fields and woods, the view to the far horizons and to the endless heaven has a deeply liberating effect.

Even the “early bird” may admire at Höchenschwand for the first time, particularly in winter and spring, the beauty of dusk, when the sun rises over the Alpine peaks. Anyone is deeply impressed by the view of the glaciers and snow fields of the seemingly so near Swiss Alps gleaming in the sunlight. In early summer and even more in autumn the guest can admire the “colour performances” of the sinking sun.

These permanent and varied incentives to stay in the open air push both the holiday guest and the patient softly to relaxation and contemplation. For others such a landscape is an invitation for hiking and sportive activities.

A wide variety of offers for physical exercise, sports, entertainment, dance, games and pleasure is a necessary supplementary infrastructure of a health resort.
 
3. Experiences in the treatment of sarcoidosis patients

One of the first medical measures is a critical examination of the patient in regard to the previous diagnosis
  • results of the precedent treatment
  • assessment of the physical ability
  • concomitant diseases not related to sarcoidosis
  • need of a therapy

This results in an individual therapy concept aiming at improving the health conditions of the patient:
  • enhancement of the physical ability
  • recovery of capacities for the private and professional life
  • improvement of the patient’s participation

In 1995 we elaborated the therapeutic concept of Höchenschwand containing the four major elements of the sarcoidosis therapy from A to D:
•    A) medicaments
•    B) therapeutic exercise 
•    C) active participation
•    D) diet


Concerning item A
Regardless to ulterior enunciations, some remarks ought to be made about the experiences at Höchenschwand concerning the immune suppressive therapy.


1. Acute sarcoidosis
  • use of corticoid only if the therapy with NSAR reveals insufficient
  • therapy of several weeks at maximum
  • longer therapy in case of considerable extrapulmonary manifestation
  • eye
  • heart
  • central nervous system
  • further observation is necessary


2. Chronic sarcoidosis

The indication in case of pulmonary sarcoidosis ensues almost always from the analysis of the clinical report and of the evolution the X-ray diagnosis and from the result of the functional controls, so that ulterior examinations are not necessary in case of an assured diagnosis.
  • in the clinically asymptomatic stadium I a cortisone therapy is not necessary; quarterly and later half-yearly or yearly controls are sufficient
  • no monthly radiography
  • in most of the cases a yearly radiography is sufficient
  • imaging technology
  • in stadium II with slight symptoms, an intact pulmonary function and absence of pain we would first control only half-yearly

A pharmaceutical treatment is only necessary when the disease evolves.

In case of a serious X-ray diagnosis and a deterioration of functions a pharmaceutical interventions is compulsory
  • in stadium III a cortisone treatment is in any case indicated and even in an apparently obvious fibrosis stadium, a therapy of several weeks has first to be conducted in order to decide, whether an improvement can still be achieved by a further treatment
  • the treatment of extrapulmonary manifestations is generally executed within the indication concerning the lung affection; this is in particular true in case of an affection of the eyes and the skin.

In case of a non-progressive pulmonary sarcoidosis or if there is no need for treatment of the disease any intervention has to be based exclusively on the clinical report concerning the organ.

Functional tests are insignificant in this regard.

The myocardial sarcoidosis and the less frequent involvement of the central nervous system are much dreaded.

In both cases the suspicion alone justifies (for example: because of heart rhythm disorders or persistent headache) the immediate treatment with high-dose corticosteroids.

The sarcoidosis including extrapulmonary manifestation requires often a long time pharmaceutical therapy.

Treatment period of chronic sarcoidosis:
  • initial treatment: 1 year
  • recurrence: at least 2 years
  • involvement of the myocardial muscle or of the central nervous system: at least several years
  • involvement of the eyes: according to the diagnosis
  • dose not less than 7.5 to 8 mg!
  • the inhalation therapy is not sufficient
  • the pharmaceutical treatment of patients suffering from chronic or chronic-obstructive bronchitis, which can always develop along with sarcoidosis, depends on special recommendations and shall not be discussed

Concerning item B

The therapeutic exercise is the second pillar after the pharmaceutical treatment.

The aims are:
  • the enhancement of the residual capacities and reactivation after a frequently inadequate phase of inactivity
  • weight reduction
  • avoidance of collateral effects of the cortisone treatment

Based on the assessment of the residual capacities, the following activities may be programmed
  • morning gymnastics (about 25 to 30 minutes)
  • morning sports (about 25 to 30 minutes)
  • individual and guided hikes of different length and effort
  • hiking group A: 1.5 to 2 hours, 7.5 to 9 kilometres
  • hiking group B: 1 hour, 5 kilometres
  • hiking group C: 45 to 60 minutes, 2.5 to 3 kilometres
  • hiking with the mountain rescue service, about 3 to 4 hours, 10 to 15 kilometres
  • outdoor training trails
  • trail 1: length 1.7 kilometres, elevation difference 15 metres
  • trail 2: length 3.2 kilometres, elevation difference 22 metres
  • trail 3: length 3.54 kilometres, elevation difference 28 metres
  • trail 4: length 5.78 kilometres, elevation difference 110 metres
  • trail 5: length 15 kilometres, elevation difference 184 metres
  • trail 6: length 16.5 kilometres, elevation difference 280 metres
  • swimming and swimming courses

An indoor swimming pool and in summer the highest heated outdoor swimming pool of Germany are at your disposal
  • training therapy (effort of two thirds of the maximum performance; training doesn’t mean to strain!)
  • bicycle ergometer
  • running belt
  • rowing box
  • stepper
  • dance!
  • tennis (open air courts, tennis hall)
  • in winter: cross country skiing on prepared trails (50 kilometres)
  • except in case of serious liver involvement with elevated “liver function values” and in case of considerable subjective pain
  • only moderate therapeutic exercise consisting of walks, swimming, respiration exercise in the group and osteoporosis gymnastics
  • beside the therapeutic exercise a “controlled polypragmasia” is carried out within the physiotherapy
  • local balneological and physical treatment of the mucous membranes of the respiratory tract
  • inhalations
  • Japanese medical herb oil
  • camomile
  • brine steam
  • bepanthen
  • bubbling baths with balm essence
  • arm baths after Hauffe with essences
  • general balneological-phyiscal treatment for the resilience enhancement and the elimination of disorders of basic vegetative functions
  • sauna
  • alternating hot and cold shower
  • alternating hot and cold baths
  • applications after Kneipp
  • treading baths
  • showers etc.
  • rehabilitation treatment of the respiratory system
  • pneumological treatment (similar to the mechano-therapy)
  • respiration therapy
  • single treatment, possibly with the hot roll
  • relaxation and stretching exercises
  • massages
  • additional therapy
  • vertebral column gymnastics, aquatic gymnastics as well
  • therapeutic exercise for osteoporosis patients
  • autonomous continuation of the learned exercises
  • hot air treatment
  • mud applications
  • lymphatic drainage
  • lymph-related gymnastics
  • psychological assistance
  • autogenic training
  • progressive muscle relaxation after Jacobson
  • nicotine withdrawal training
  • singing
  • handicrafts

Concerning item C

These activities aim at informing the patient so as to achieve a better active participation in the management of his disease.

Regular lectures concerning the following themes:
  • sarcoidosis
  • immune system
  • cortisone (dissipation of fears!)
  • regular lectures on general health issues
  • individual patient training in case of concomitant obstructive bronchitis
  • sporadic discussion forums of the “German sarcoidosis association” (Deutsche Sarkoidosevereinigung)
  • information about the “self-help” group and distribution of information material
  • permanent notice board concerning sarcoidosis
  • the involvement of the family doctor’s shall also be improved by appropriate therapy reports and suggestions


Concerning item D


A special sarcoidosis diet doesn’t exist.

The main problem of our therapies is unfortunately the prevailingly iatrogenous obesity due to the cortisone treatment.

It can be attenuated by:
  • calorie-reduced food combined with physical exercise
  • patient training
  • diet instruction
  • training kitchen
possibly change of the cortisone preparation

The nutrition during a long time cortisone treatment shall always be rich in proteins and carbohydrates, in order to compensate the catabolic effect.

At the end of my enunciations I would like to indicate to some of the most frequent errors committed in the treatment of sarcoidosis patients:
  • useless cortisone therapy in stadium I without important involvement of organs
  • cortisone therapy only for the reason of elevated virulence parameters                  (ACE, LZM, IL- 2), while the X-ray and functional diagnosis remains stable
  • too long cortisone treatment in case of acute sarcoidosis
  • too short cortisone treatment in case of chronic sarcoidosis often combined with a
  • premature dose reduction
  • or
  • too low permanent dose
  • too short or no immune suppression in case of the involvement of the heart or the central nervous system
  • pacemaker: anti-arythmic or anti-convulsive therapies are not sufficient!
  • too frequent X-ray controls, partly including computer-assisted tomography and magnetic resonance imaging
  • omitted controls or omitted observation; it should be continued for life-time, since a reactivation or recrudescence is possible even decades later (current example: recrudescence after 31 years of stability of diagnosis without immune suppressive therapy).
  • insufficient control of the collateral effects of the therapy
  • eye pressure
  • clouded lenses
  • bone density
  • blood pressure
  • blood sugar level
  • weight
  • striae
  • insufficient observation of the evolution of X-ray diagnoses; comparison only with the precedent radiography
  • unfounded physical inactivity, in particular after the end of the cortisone therapy

The so called “cortisone withdrawal syndrome” consisting in pain in various parts can be relieved by physical activity.

* unfounded long time medical certificate, which may cause
- the loss of the job and
- social decline

Literature:
Wurm, K., author, “Sarkoidoseleitfaden” (Sarcoidsis Manual), Thieme, 1997
Wurm, K., K. Kögler, “Die vier wichtigen Elemente der Sarkoidose-Therapie, Ärztl. Praxis” (The Four Important Elements of Sarcoidosis Treatment, Medical Practice) 21,
March 14th 1995, 18-19
Rathmer, B., “Vergleichende Gruppenuntersuchungen über die physiologischen Wirkungen des Mittelgebirgsklimas, Inaug.-Diss.” (Comparative Group Study Concerning the Physiological Effects of the Low Mountain Range Climate, Dissertation), Freiburg, 1966