Patients' Guide to ILDs

       Interstitial lung disease (ILD) comprises of a group of several diseases of different causes but similar features. It is classified as either Primary (or idiopathic) and Secondary (Secondary to some other disease). Secondary ILD commonly occurs in patients with pre-existing diseases such as rheumatoid arthritis, systemic sclerosis, sarcoidosis and occupational disorders. Hypersensitivity pneumonitis is a group of common ILDs which occur on exposure to organic dusts which happens during farming, keeping birds, manufacturing cheese, air-conditioning etc. ILD can also develop following viral infections, administration of certain drugs, high-dose radiation and radiotherapy.

       Primary or idiopathic ILD is the more serious type whose cause is not identifiable. Of various kinds of idiopathic ILDs, idiopathic pulmonary fibrosis (IPF) is most important. There are a few other types of idiopathic ILDs importantly, non-specific interstitial pneumonia (NSIP), organizing pneumonias (OP), desquamative interstitial pneumonia and acute interstitial pneumonia.

Common complaints  

1.       Breathlessness especially on exertion. Patient may feel completely fine at rest.

2.       Dry cough which is quite hacking and troublesome, often not relieved with routine cough suppressants.

3.       Generalized weakness, malaise, fatigue and tiredness.

4.       Loss of appetite.

5.       Weight loss.

6.       Blueness of fingers and nails during exercise.

7.       Symptoms of underlying disease such as joint pains, skin rashes or other manifestations.

Investigations required for confirmation of diagnosis

1.       Routine blood tests: hematological, biochemical and immunological as considered important

by the physician.

2.       Chest X-Ray

3.       Pulmonary function tests – spirometry. Sometimes, blood gases assessment.

4.       High resolution CT Chest

5.       ECG and Echocardiography

6.       Fiberoptic bronchoscopy and lung biopsy as decided by the physician.

7.       Occasionally, lung biopsy with thoracoscopy or open surgery is required to establish the diagnosis.

Other tests may be required for identification and exclusion of a secondary cause of ILD such as for rheumatoid arthritis, sarcoidosis, occupational disorder or hypersensitivity pneumonitis. Serological tests may be required for hypersensitivity pneumonias.

Treatment of ILD 

       There is no efficacious therapy for ILDs. Treatment of IPF remains elusive. Patients and clinicians are faced with four options: (i) no treatment, (ii) corticosteroids and cytotoxic agents, (iii) anti-fibrotic drugs, (iv) other miscellaneous agents. 

       Based on the evidence available immunosuppression with corticosteroids and cytotoxic agents is not helpful for IPF. These drugs are helpful in secondary ILDs such as CTD associated ILD, sarcoidosis and some other forms of IIP (NSIP, COP and DIP). Pirfenidone is the one agent which may provide some benefit in IPF. It is an anti-fibrotic drug which is shown to decrease the decline in lung function parameters. Several other drugs are also employed, but not very useful in improving the condition.

        Most patients with IPF continue to experience an inexorable progression to death, with lung transplantation being the only measure shown to prolong survival. Currently, lung transplantation has been associated with improved lung function, exercise capacity, quality of life, and survival in this group of patients. The treatment is available at very few centres in India and the cost is prohibitive. Most importantly, there is very limited availability of organs.  Lungs for transplantation can be retrieved only from a brain-dead individual with fully informed consent of the family following compliance of all the requisite legal and medical guidelines. 

       Thus, the primary objectives of treatment in IPF are essentially to provide symptomatic relief of symptoms, oxygen therapy for desaturation and pulmonary rehabilitation. One also needs to treat the complications which occur either secondary to the disease per se, or the toxicity of drugs.

Prognosis Natural history of ILD 

       Most forms of ILDs are progressive in nature. There is no permanent cure. Patient’s condition is likely to deteriorate with time. IPF is the worst form of ILD which carries a poor prognosis with an average survival of 3-5 years. Other ILDs have variable natural history which is modifiable with treatment. 

_____________________________________________

Dr Surinder K. Jindal, MD, FCCP, FAMS, FNCCP

(Ex-Professor & Head, Department of Pulmonary Medicine

Postgrad Instt of Med Edu & Res, Chandigarh, India)

Medical Director, Jindal Clinics, SCO 21, Dakshin Marg, Sector 20 D,

Near Guru Ravi Das Bhawan, Chandigarh, India 160020.

Email: dr.skjindal@gmail.com    Website: jindalchest.com

Ph.  Clincis: +91 172 4911000

"Lung dialysis"

Some things are beyond belief, though clearly plausible - as the following example illustrates.

     A male patient in the US suffering from cystic fibrosis underwent a double lung transplant followed by failure. He was posted for re-transplant but was told that his carbon dioxide levels were too high. However, his physician, who was a well read man and in contact with the bioengineering world decided to go in for something out of the ordinary. A sort of a mini ECMO machine had been developed a few years earlier, for removing carbon dioxide from the lung. Known as the Hemolung RAS, it functions by removing carbon dioxide from and adding oxygen to blood just like a dialysis machine. As it was not approved by the FDA, emergency approval was taken within 24 hrs and the patient put on the machine. Lo and behold, he improved, the carbon dioxide levels reduced to acceptable limits and he was taken up for retransplant!

Some salient points:

1. Cystic fibrosis is a sort of death sentence in general practice in India with only symptomatic treatment available
2. This patient underwent double lung transplant not once but twice
3. Emergency approval applied for and recieved within 24 hours!! (Seems like some sort of dream)
4. As the machine was not available in the US, the doctors incharge personally acquired it from across the border i.e. Canada
5. The patient remained for 20 days on the machine
6. The machine was not recommended for use by any professional society

Lessons:

1. Out of the box thinking is the key
2. Be uptodate in one's field
3. Persistence and perseverance are essential

The full article can be read here: http://medicalxpress.com/news/2014-07-patient-implanted-hemolung-lifesaving-lung.html

Thanks to Dr.Ajay Handa for updating me on this.

End of Life Care: The Hindu Viewpoint

The traditional Hindus believe in the continuity of life after death.  There is a great degree of sanctity attached to the pre-death worship, performance of last rites and to the rituals both before and after the death.  It is strongly believed that the type and mode of death is an important determinant of the peace for the immortal soul ever thereafter.  An easy and peaceful death is crucial to attain ‘nirvana’ or ‘mukti’ i.e. liberation from the sufferings and miseries of the life and the death cycle.

The terminal care therefore essentially focuses to achieve the ‘best possible’ quality of life without interfering with the attempt to prolong the life.  There is supreme importance of ‘care beyond cure’ and to ‘dying with dignity’.  Factually, the Hindu concept of life is centred around respiration and the length of life is measured by the ‘limited’ number of breaths which are fixed.  Many a life-prolonging treatments especially the artificial continuation of breathing with the help of ventilators are therefore in direct conflict with the traditional viewpoint.  This, I believe is applicable only for artificial continuation of life in an otherwise death (e.g. the brain-dead) individual than for assisted respiratory support as a mode for treatment.

Palliative treatment in Hinduism is quite in conformity with the existing concept of terminal care which involves the ‘care beyond cure’ philosophy.  One aims to prevent, relieve or soothe the symptoms of disease without affecting a cure.  The pre-terminal (and terminal) are designed to offer symptomatic relief from the pain and suffering of approaching death.  It is not a substitute or an alternative to curative treatment, but only an acceptance of the inevitability and of the limitations of life-prolonging treatments.

Rituals and Rites

                Both the individual and the family are generally concerned with the last wish acts, and rituals near the death.  Death in the bed is analogous to death of the sick which must be avoided.  A dying individual is preferably moved to the floor – not for cardiac massage but to lie in the lays of mother-earth.  Verses from the holy books such as the Gita or the Vedas should be sung and the water from the sacred rivers, especially the Ganges should be made available.  The dying does like to bless the children as much as hey like to be blessed.  There is no better death for a Hindu senior than to die with everyone of the progeny around.

                After-death handling of the body is both sacred and ceremonial.  It must be properly cleaned and bathed.  Nice and preferably new clothes, sometimes including the jewellary are worn before consigning the body to the flames.

Bereavement

                Generally speaking, the degree of emotional attachment and inter-dependence is high in Hindu families.  A sudden, or even a slow exit of a member of the family is mourned and remembered for long.  The period required for resolution of grief is longer. It is possibly for this very reason that a number of sacred acts are undertaken afterwards.  For example, the body remains and ashes are collected on the 3^rd day or so and immersed in a sacred river.  Several other functions are undertaken in the next two weeks and on fixed intervals in the following year. 

References

1. Banerji SC.  Indian Society in the Mahabharata.  Varanasi: Bharata Manisha, 1976.
2. Basham AL.  Aspects of Ancient Indian Culture.  New York: Asia Publishing House, 1970.
3. Crawford SC (eds).  Hindu Bioethics for the twenty – first century.  State University of New York Press, New York 2003.
4. Humphry D (eds).  The Practicalities of Self-Deliverance and Assisted Suicide for the Dying.  Time Books International, New Delhi 1991.
5. Crawford SC (eds).  Dilemmas of Life and Death.  State University of New York Press, Albany 1995.

_____________________________________________

Dr Surinder K. Jindal, MD, FCCP, FAMS, FNCCP

(Ex-Professor & Head, Department of Pulmonary Medicine

Postgrad Instt of Med Edu & Res, Chandigarh, India)

Medical Director, Jindal Clinics, SCO 21, Dakshin Marg, Sector 20 D,

Near Guru Ravi Das Bhawan, Chandigarh, India 160020.

Email: dr.skjindal@gmail.com

Website: jindalchest.com

Ph.  Clincis: +91 172 4911000,  Res.  +91 172 2712030/ 31

Withdrawal of Life Supports?

Life Prolonging Treatment : The Right to Refuse?

(Legal and Ethical considerations)

            In medical parlance, a life prolonging treatment refers to all treatments which have the potential to postpone the death of patients suffering from incurable and terminal illnesses.  These conditions may include advanced cancers, failures of organs such as the lungs, heart, kidneys, liver or others and progressive, end stage neurological illnesses.  Advancements in modern technology have made it possible to sustain lives of such patients for several days, weeks or sometimes years with treatments involving cardiopulmonary resuscitation, artificial respiration, cancer chemotherapy, dialysis, artificial nutrition, hydration and other multiple drugs. 

The subject has raised a whole lot of new questions and dilemmas in not only the medical and legal  circles but among the general public all over the world.  Media has often extensively covered and discussed such examples.  Legal and medical literature is replete with opinions and judgments on such questions and cases involving legal interventions.  Most such examples have emanated from the West, although there have been a few instances in India.  With a rapid expansion of techno-medical scenario promising almost moon to every individual, the demands of people to live long have also increased. But the costs are tremendous and there are wider gaps in availability of resources, expertise and manpower, infrastructure, awareness and knowledge in providing life prolonged treatment.  This has posed a huge burden on the shoulders of medical practitioners, health professionals and policy makers. 

Even more basic than all other considerations is the core issue of medical judgement and its legal sanctity to provide life prolonging treatment.  It is a curious turn of events that medical practitioners tend to look to law for each of their action.  No citizen, much more so a medical doctor can afford to ignore law.  But medical judgements cannot always be surrogated to legal justifications especially since the law is other silent or ambiguous on most of the issues related to prolongation of life.

I give below two real case examples for you all to consider:

      

            

                The legal considerations as well as the medical opinions guiding the judgements and actions of doctors are yet in an evolving phase in this country on the issue under discussion.  It is therefore, worthwhile to look into examples available elsewhere.  Most of the Western medical associations have come up with more clear guidelines to help doctors to decide action.  The British Medical Association has laid quite comprehensive recommendations on several issues with respect to decision making on ‘withholding’ or ‘withdrawing’ life prolonging treatment.  Needless to say that the guidelines can only act as an aid in the process of decision-making rather than form a protocol of action.  A doctor is essentially guided in his action by the primary goal of medicine i.e. to benefit the patient by restoring or maintaining the patient’s health.

                One of the most contentious subject is related to the decision of patients to refuse a life prolonging treatment.  The law generally gives the right to an individual to decide and choose an option.  One is however faced with two different scenarios i.e. in case of a dying patient who may have the capacity to make and communicate decision vis a vis a patient who does not have this capacity (for example because of altered or impaired consciousness).  In other words, a patient may be either competent or may have lost the competence to decide.  Our discussion here refers to only the adults since babies, children and several other groups may not legally possess this competence at all.

                Legally speaking, an adult has the full competence to make decisions unless there are doubts on grounds of mental incapability or misconception of reality.  In a famous trial in U.K. (reg. MB (Medical Treatment), in 1997), the Court has rejected the decision of a patient since he believed that his blood was poisoned because it was red.  The mere observation that individual’s decision appears irrational or unjustified to others cannot be taken as evidence of lack of mental capacity.  But doubts may arise if the decision is contrary to the previously expressed wishes. 

                The right to refuse treatment is firmly established in British Law.  This had been reasserted in other cases including St. George’s Health Care, National Health Service Trust vs S (etc.).  Interestingly, the right of refusal was upheld even in a psychotic patient who held erroneous views on several matters but was considered as correct with respect to refuse amputation of his gangrenous foot.  In the United States too, the Supreme Court in the Cruzan case as well as several other cases had cleared expressed the principle that an individual has the constitutional right to refuse treatment even if this may result in his/her death.  This right has been reiterated in several other judgements even where the patients did not have life threatening illnesses.

                Another important issue which has emerged in terminal care is the concept of ‘advanced directives’.  Several sick patient like to leave written (or even verbal) directives expressing their wishes and desires regarding resuscitation and terminal care.  A ‘Do Not Resuscitate (DNR)’ directive by a terminally ill patient is not an uncommon practice in Western medicine. 

                Most ‘advanced directives’ have got legal sanctions and several judgements of the courts are available on the issue.  In U.S.A., the courts and legislatures have recognized this  legal tool of “advance care planning”.  Following the patient self-determination Act (1990), the hospitals are required to inform patients of their right to refuse medical treatment and to make advance directives.  These directives can be considered at par with the expressed desires of people for example the will regarding inheritance of their properties, wishes to donate eyes and body organs after death, or the method of their funerals and last rites. 

The real dilemma is for the doctor looking after a patient who has left a DNR or ‘refusal to get treatment’ order.  The competence of such an advance directive is always challengeable.  On the other hand, providing treatment against the expressed wishes of a patient may also land the doctor in trouble.  The Medical Associations would therefore, recommend that wherever genuine doubts exist about the validity of an advance refusal, the doctor should act in favour of giving at least emergency treatment and buy more time to decide in consultation with the family and the colleagues.

                Undoubtedly, the debate on the issue and the dilemma faced by the caring doctors are bound to continue.  No final protocol can be made for the doctors to act.  They need to follow the existing cultural and medical practices of the land.  Nonetheless, more individuals are likely to assert their rights in refusing life prolonging treatments.  The core philosophy in terminal care remains – Exit with Dignity.

Resource References

1. British Medical Association.  Withholding and withdrawing life-prolonging medical treatment. London, BMJ Books 1999.
2. Emanuel LL, von Gunten CF, Ferris FD.  The Education for Physicians on End-of-Life Care Curriculum, EPEC Project.  The Robert Wood Johnson Foundation, 1999.
3. British Medical Association and the Law Society.  Assessment of Mental Capacity: Guidance for Doctors and Lawyers. London: BMA, 1995.
4. Cassel CK, Foley KM.  Principles for care of patients at the end of life: an emerging consensus among the specialities of medicine. New York Milbank Memorial Fund, 1999.
5. Meisel A.  The right to die, 2^nd ed. New York: John Wiley and Sons, 1995.

_____________________________________________

Dr Surinder K. Jindal, MD, FCCP, FAMS, FNCCP

(Ex-Professor & Head, Department of Pulmonary Medicine

Postgrad Instt of Med Edu & Res, Chandigarh, India)

Medical Director, Jindal Clinics, SCO 21, Dakshin Marg, Sector 20 D,

Near Guru Ravi Das Bhawan, Chandigarh, India 160020.

Email: dr.skjindal@gmail.com

Website: jindalchest.com

Ph.  Clincis: +91 172 4911000,  Res.  +91 172 2712030/ 31