Haemophilia: scope for rehabilitation in India.JS Kale
Occupational Therapy School and Centre, Seth G. S. Medical College and K.E.M.Hospital, Parel, Mumbai, India., India
Correspondence Address: Source of Support: None, Conflict of Interest: None PMID: 0010734354
Source of Support: None, Conflict of Interest: None
Keywords: Hemophilia A, rehabilitation,Human, India,
The most prevalent of the hereditary disorders of coagulation are the haemophilias A and B i.e. deficiency of coagulation factors VIII and IX respectively. They have a sex linked recessive inheritance pattern; males are usually affected and females are carriers. Classic haemophilic is characterized by extensive spontaneous bleeding in various parts of the body e.g. soft tissue haematomas and haemarthrosis. Of the various bleeding manifestations of haemophilia, haemarthrosis is the most common, most physically, economically and psychologically debilitating manifestation, occurring in 90 % of affected patients.
In fact most of physical, psychological, social and financial problems in haemophilia are caused by the effects of recurrent haemarthrosis, chronic arthropathy, joint deformities and restriction of mobility. The aim of management in haemophilia lies in the prevention of joint deformities and preservation of joint function,.
Optimal treatment involves a combination of adequate factor replacement, rest, ice application, slight compression of the affected part using compression bandages or orthotic devices and a supervised rehabilitation program.
Haemophilic patients in developing countries pose a considerable challenge to the haematologist and the therapist in the day-to-day management.
There is a perennial shortage of blood and blood components required for their treatment, especially in the smaller cities, towns and villages of India. Even to afford their cost. Thus, management of joint bleeds and deformities in our country often taxes the ingenuity of the treating haematologist and the therapist.
The Haemophilia Federation of India (HFI) was established in the year 1982 by haemophilia patients and their families to help and support patients of haemophilia, all over the country. Today it has 16 chapters in cities and towns of India in various stages of development.
The HFI, provides factor concentrates at subsidised rates to all its members and also arranges for their treatment and rehabilitation where feasible.
The haemophilia society Mumbai chapter in conjunction with the haematology department of King Edward Memorial hospital runs a comprehensive care centre for its 450 members.
The centre offers the following facilities;
1. Facilities for an accurate diagnosis of haemophilia-
a. Various factor assays.
b. Inhibitor screening.
c. Inhibitor assay.
2. Screening for HIV/Hbs Ag /HCV.
3. Indoor admission facilities for the treatment of acute and chronic bleeds.
4. Rehabilitation of chronic deformities.
5. Surgical procedures
6. Carrier detection and diagnosis.
As our centre is an advanced one, it caters not only to patients from Mumbai and Maharashtra, but also to haemophilics from other towns and villages of India.
Most of the haemophiliacs in India do not have access to the services of a haematologist or to factor therapy and therefore come with severe, acute and chronic joint deformities, pain and restriction of movement. These lead to decreased mobility, increased dependence on the family, difficulties in completing education or finding employment and severe psychological problems for the patient and his family.
Thus rehabilitation at our centre stresses on the following two aspects.;
1. Prevention of the deformity.
2. Treatment of the existing deformity.
Prevention of the deformity:
Prompt treatment of acute haemarthrosis with adequate factor replacement analgesics, ice application, application of compression bandages and early careful mobilisation care is instituted.
The patient is taught a series of exercises to facilitate absorption of the haematoma prevent muscle wasting, improve joint range and strengthen muscles. Exercises for strengthening muscles could be used in one of the following forms or in combination:
a) Isometric Exercises. These exercises involve muscle contractions without movement of the joint. They are helpful in preventing muscle wasting and in early absorption of haematoma.
b) Active assistive exercises. These are exercises, which are assisted by the therapist, their aim is to prevent restriction of movement at the joint and maintain the available range.
c) Active exercises. These are simple exercises carried out by the patient, under the supervision of the therapist. However, the therapist neither assists nor offers resistance. These help in achieving greater range of movement, maintain the achieved ranges, also prevent muscle wasting and improve muscle strength.
d) Active resistive exercises. These are exercises prescribed or performed against progressive resistance to build further muscle strength,.
Besides these, isokinetic exercises can be incorporated later in the patients exercise regime.
These exercises and supportive devices are also incorporated into a home management programme for the patients with weekly follow -ups at the clinic. The patients are also given a special training in correcting abnormal postures and gait pattern. Due to recurrent bleeds, patients tend to compensate by using abnormal postures, which in turn leads to further stress and strain on the affected joints and hence aggravates bleeding episodes in future. These measures go a long way in reducing the frequency of bleeding and helping patients deal with their problems.
Treatment of existing deformities:
Haemarthrosis occurs in 90% of patients of haemophilia; the weight bearing joints are more prone to bleed. Repeated bleeds lead to a chronic haemophilic arthropathy with complete destruction of the joint, fibrous ankylosis and contractures. A large number of our patients come with these deformities. The knee joint is the most commonly involved and most often permanently crippled. Contractures of the knee joint may be classified depending on the duration:
1. Neoflexum - days to weeks old.
2. Paleoflexum- weeks to months old.
3. Archiflexum.- years old., leading to fibrous osseous ankylosis.
Patients presenting in the first stage are treated with a programme of intensive rehabilitation and orthosis, under adequate factor cover. Most patients respond well to these measures. Stages 2 and 3 are more difficult to treat conservatively. After a period of vigorous rehabilitation training, these patients may be taken up for corrective surgeries, such as soft tissue release and external fixation. These procedures are also done under factor cover. The ultimate goal is to give the patient maximum functional independence.
Patients of haemophilia with chronic joint deformities are often required to produce disability certificates for travel education and employment. This is done for each patient on an individual basis after taking into consideration his existing deformities and functional limitations, jointly by the rehabilitative therapist, orthopaedic surgeon and the haematologist.
Limitations in prophylactic and preventive care can be attributed to the high cost of factor and factor concentrates, low financial status of the patients in third world countries.
Inability of the chapters to cope up with the surgical expenses too has limited the operative care in India. Lack of machinery and infrastructure is also a contributory factor, limiting the scope for surgical intervention.
Regular exercise regimes may not be followed by all patients on a day-to-day basis at the hospital clinic due to difficulties of travelling. Training patients and parents for home exercise schedules is important. Many of such patients may not follow the exercise regime at home and often continue to have recurrent bleeding episodes. It may be necessary to have more of community centres offering services to patients staying far off. This indicates a greater number of medical, paramedical and scientific staff to be trained in the field of haemophilia.
With the present system of disability certification many haemophilics may not fall under the 40% disability criteria and may be deprived of the benefits for handicapped though they are actually handicapped in areas of function, mobility, job placement etc.
Disability certification thus needs to be modified in future for these patients.
In the developed countries, management of haemophilia has steadily improved over the years. Today, grossly deformed joints are a novelty in the west where factor concentrates are readily and easily available. Pools milestone discovery of making cryoprecipitate with surprising ease has made the treatment of bleeding in a haemophiliac possible in the developing world. However, most of our patients do not have an access to prompt treatment or the finances to afford the treatment. A large majority of our patients come with neglected joints and gross deformities. These patients benefit largely with rehabilitative therapy at a more affordable cost. This awareness amongst haemophiliacs has increased over the past few years and a many of our patients are on regular exercise and rehabilitation programme. These patients show a decrease in the frequency of bleeds and improved quality of life.
I thank the Dean, Seth GS Medical College for allowing me to publish this article. I would specially like to thank Dr. Farah Jijina for her co-operation and guidance and also Professor Kenkre for her co-operation.