|

Received: 22 September 2020    Accepted: 24 November 2020

DOI: 10.1111/hae.14229

ORIGINAL ARTICLE

Assessing the current knowledge, attitude and behaviour of

adolescents and young adults living with haemophilia

Richa Mohan1  | Nita Radhakrishnan2  | Meera Varadarajan3 | Sandip Anand4

1
Empowering Minds Society for Research
and Development, New Delhi, India
2
Department of Pediatric Hematology
Oncology, SSPH&PGTI, Noida, India
3
Department of Clinical Hematology,
Bangalore Medical College and Research
Institute, Bangalore, India
4
Xavier University Bhubaneswar,
Bhubaneswar, India
Correspondence
Richa Mohan, Member, Psychosocial
Committee, WFH and Director,
Empowering Minds Society for Research
and Development, New Delhi, India.
Email: richa.mohan07@gmail.com
Abstract
Introduction: There is significant incidence of Haemophilia in India, with second largest
number of persons with Haemophilia A. 20,778 patients registered with Haemophilia
Foundation of India in 2018. Research in India includes diagnostic studies, complica-
tions and co-morbidities, prenatal diagnosis, inhibitor development and gene therapy.
Limited is known about quality of life of these patients. Since Haemophilia leads to the
loss of ‘normal lifestyle’ in young people resulting in emotional distress and depres-
sion, it is important to analyse Knowledge, Attitude and Behaviour of persons with
Haemophilia.
Aim: The aim of the study is to focus on exploring the status of Haemophilia and
knowledge, attitude, behaviour of adolescents and youths with haemophilia with the
objectives to study 1) the current medical status of haemophilia amongst target popu-
lation; 2) the knowledge, attitude and behaviour of patients with haemophilia towards
their condition.
Methods: Respondents in the age group of 15–30 years, who were registered with
the Hemophilia Treatment Centers of Government Hospitals/Hemophilia Societies,
were interviewed. Data were collected using a structured questionnaire. The study
was conducted in two different states and with respondents of two different age
groups.
Findings: Most respondents suffered from severe haemophilia and co-morbidities
such as anxiety, stress, chronic pain and head-ache. All of them felt that haemophilia
interferes in leading a normal life and perceive a grim future.
Conclusion: Young people in India need technical, financial and psychological support
to prevent complications related to haemophilia. While most of them take responsibil-
ity for their health, more behavioural changes need to be inducted to improve quality
of life.

KEYWORDS
adolescent, attitude, behaviour, emotional health, hemophilia, knowledge, young adults

Haemophilia. 2020;00:1–7. wileyonlinelibrary.com/journal/hae© 2020 John Wiley & Sons Ltd     1 |

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2      MOHAN et al.
1  |  I NTRO D U C TI O N
Improvements in haemophilia care over the last decade might lead
to expectations of a better quality of life for patients in India. This
changing scenario warrants research to control the disease and
its impact on population. The World Federation for Haemophilia
(WFH) survey 2018 identified total number of persons with hae-
mophilia in 125 countries was 2,10,454.1 80% of these live in de-
veloping countries. 2 India also experiences a significant burden of
the disease.
The number of cases prevalent in India is 0.9 per 1,00,000
population. 3 20,778 patients are registered with Haemophilia
Foundation of India as of 2018.1 These cases are expected to swell
near 85,000–1,00,000 when investigative techniques, that is, co-
agulation laboratories become available across 750 districts in the
2
country. India has the second largest number of patients with
haemophilia A. 3 80 per cent of Indians with the serious blood dis-
order are not diagnosed due to the absence of proper diagnostic
facilities in the remote area.4 Better knowledge about the disease
amongst clinicians and supporting staff shall help in more regis-
trations of the PWH and better collection of the data for better
5
planning and management of disease.
Haemophilia research in India is one of the examples of need-
based research.6 The genetic basis of this disorder has been studied;
however, social costs of this condition and opportunities for offering
3
genetic counselling have not been reported.
On micro level, the disability is most prevalent amongst the
economically weaker sections of society. The treatment being
expensive, it requires prevention services for haemophilia in
low-income countries to mitigate the suffering due to lack of af-
fordable treatment.7 Government run programs have proved to be
cost-beneficial. 8
2  |  TH EO R E TI C A L BAC KG RO U N D
2.1  |  Knowledge, attitude and behaviour (KAB) of
patients
Patient's perspective is significant for the assessment of efficacy of
treatment.9 A study (Nazarro et al.) of KAB of patients concluded
that behaviour change in terms of early treatment and exercis-
ing regime was important for preventing complications.10 Another
such study in Canada identified gaps in the patients' knowledge.11 A
qualitative study (Babington-Ashaye et al.) reported that due to lack
of knowledge, people of Senegal attributed haemophilia to various
religious beliefs.13
There is need to find out emotional health of affected persons
as haemophilia impacts patients not only physically but socially
14 15
and psychologically as well. Khair et al . suggested that ado-
lescents with severe haemophilia suffer emotionally as they have
limited contact with their peers, and opportunities for experiential
learning. Parents of a children with haemophilia report feelings of
guilt, disappointment and a desire to increase society's under-
standing of haemophilia, The Haemophilia Experiences, Results and
Opportunities (HERO).16
Haemophilia Experiences, Results and Opportunities study
surveyed 1,386 people in 11 countries,16 reported that majority of
people with haemophilia suffered with chronic pain, amongst other
co-morbidities. Lack of knowledge, lack of access to treatment cen-
tres etc. were responsible for dissatisfaction amongst patients. Age
was found to be an important criterion. Elderly patients who suffer
from Haemophilia are prone to emotional problems.17
Research shows that behaviour of affected persons and change
in lifestyle are important for improved quality of life.18 A study re-
ported that young boys preferred choosing their own schedule of
treatment which offered them maximum protection with minimal
interference in their day-to-day activities.19
2.2  |  Rationale
There is limited research study available on quality of life of person
with haemophilia in India. In order to improve their quality of life, it
is important to analyse KAB of persons with Haemophilia. Such a
study will help in identifying gaps in currently running programmes
to improvise the lives of affected population.
Our study aims to fill the gap by assessing KAB of in a quantitative
manner. The study is conducted in two Indian States which are at dif-
ferent stages of development, presenting a comparative picture.
2.3  |  Objectives of the study
The objective of the study is to examine;
1. The current medical status of haemophilia amongst target
population.
2. The knowledge, attitude and behaviour of haemophilia patients
towards their condition.
3  |  R E S E A RC H M E TH O D O LO G Y
3.1  |  Research instrument
Data were collected with the help of a questionnaire, developed with
the help of literature review and as focused group discussions with
persons with haemophilia. The questionnaire was tested for reliabil-
ity with strong Cronbach alpha score of 0.88. It consisted of four
sections, section A consisted of questions related to Haemophilia
type and co-morbidities. Section B was on education and employ-
ment status, section C related to severity of disease and section D
was about KAB towards haemophilia. The questionnaire was pilot
tested for clarity, relevance of questions and refinement. It was
translated into regional languages.
MOHAN et al. |
      3

TA B L E 1  Demographic profile
3.2  |  Target population
Uttar
Young boys with haemophilia in the age group of 15–40 years from Total Pradesh Karnataka

two different geographies namely, Uttar Pradesh and Karnataka, Number of respondents 169 79 90
were targeted for the study. The respondents were persons with Mean age 22.8 21 24.25
haemophilia enrolled with the Haemophilia Treatment Centres/ Education level
Haemophilia Societies in both States. The states were selected on No education 7.1 2.5 11.1
their levels of socio-economic development like state GDP, literacy Secondary 26.6 29.1 24.4
and availability of care.16,17 The incidence of Haemophilia patients is
Senior secondary 20.7 12.7 27.8
higher in Uttar Pradesh. Total number of Persons with Haemophilia
Graduation 35.5 43.0 28.9
in Karnataka as per the National Haemophilia Registry was 2,400
Post-graduation 10.1 12.7 7.8
and 3,100 in Uttar Pradesh.

3.3  |  Data collection procedure and ethical 6.5

considerations 15.4 People with HA
People with HB
Data collection was facilitated by field. Participation was voluntary,
and informed consent was obtained. If the participant was found H with inhibitors
77.5 N = 169
uncomfortable answering the questionnaire, he was allowed to
withdraw at any moment. The subjects’ information was recorded
F I G U R E 1  Type of haemophilia (%)
without name, and a code was allocated to each case.

Co-morbidities such as anxiety, stress, chronic pain and head-

3.4  |  Sample size
A total of 200 questionnaires were obtained while only 169 were
found complete and suitable for analysis.
4  |   R E S U LT S A N D A N A LYS I S
The questionnaire was analysed using descriptive statistics, chi
square test and correlation to identify if there was any association
between knowledge, emotional state and behaviour of respondents
and categorical variables such as age and state of residence.
4.1  |  Demographic
ache were common. Depression and insomnia were also found. The
incidence of stress and chronic pain was found to be higher in Uttar
Pradesh. Younger population suffered more from chronic pain and
anxiety while adults were afflicted with depression, insomnia and
stress Figures 1–4.
When a bleeding occurs, 50% respondents said that they reach
the hospital within 6 h, reason being the hospital or the infusion cen-
tre was far while others wait for the symptoms to improve naturally.
Mostly the treatment is on demand.
More than 70% respondents feel that their condition interferes
with all of their daily chores. 68% respondents exercise at home.
Out of 32% not undertaking any exercise regime, 50% respondents
were not doing it either due to fear of bleeding or because they feel
lethargic.

The respondents were grouped into two age groups of 14–21 years
and 22–40  years. The median age was found to be 21  years.
Approximately 45% respondents were pursuing their studies in ei-
ther class 10 or in class 12 Table 1.
4.2  |  Status of haemophilia
77% of the respondents were having haemophilia type A and
were treated with factor VIII. Around 60% experienced spontane-
ous bleeding in the last 12 months. Most respondents had severe
bleeding episodes in urine, which occurred three or four times in
a year.
4.3  |  KAB of patients
More than 70% showed good knowledge about haemophilia. 64%
respondents were aware about the types of haemophilia. Around
60% were aware that airways can get obstructed. Only 58% re-
spondents were aware about home therapy.
Knowledge levels were found to be less in Uttar Pradesh and
amongst young. (see Table 2).
The results showed that emotional health was a major con-
cern. Approximately 66% feel uncomfortable about disclosure of
their condition to others. More than 50% respondents feel over-
whelmed with the financial burden. 75% respondents felt that the
Government is supporting the affected families with emergency
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4      MOHAN et al.

Karnataka UP

Anaemia 6.7
3.8
Depression 6.7
8.9 N for UP = 79
5.6 Karnataka - 90
insomnia 10.1
Headache 15.6
17.7
Stress 20.0
21.5
Anxiety 14.4
30.4
Chronic pain 17.8
30.4

F I G U R E 2  Co-morbidities experienced by respondents (%)

50.0 Respondents scored high on behaviour related indicators. More

40.0 than 85% respondents follow the regime as advised by their doctors.
29.6
30.0 46.7
More than 30% respondents did not contact the treatment centre
20.0 13.0
8.9 immediately upon experiencing symptoms.
10.0
The respondents in older age group did show mature behaviour
0.0
1 joint 2 or 3 joints more than 3 none in managing their condition. (see Table 4).
Only 78% felt that the treatment centre is accessible. There was
F I G U R E 3  Respondents suffering recurrent bleeding in joints a felt need for induction of more staff in hospitals for handling and
(%) management of their condition. More than 90% responded in affir-
mative that the Government should invest in prevention measures.
100.0 We tried to explore the relationship between behaviour with
Total
knowledge and attitude with the help of Table 5. There was positive
50.0 UP
correlation between the knowledge and attitude variables with be-
Karnataka
0.0
Less frequent Oral Medicines prophylaxis long acting haviour variables. Positive significant correlation indicates that more
IV factors knowledge warrants better action and response plan.

F I G U R E 4  Future management of haemophilia

care. Mean average score for this parameter—Government is un-
dertaking enough initiatives for patients with haemophilia—was
found to be higher for Karnataka than for UP. It is worthwhile to
note that respondents in elder age group were found to be under
more stress and less satisfied with Government initiatives. (see
Table 3).
5  |  D I S C U S S I O N
5.1  |  Status and Co-morbidities
The survey report of World Haemophilia Federation reported
about 82% of total respondents were suffering from haemophilia
type A in 2018.1 The present study reported 77% respondents had

TA B L E 2  Knowledge—state-wise and
Age group Age
age-wise variation in mean scores
Mean score Overall Karnataka UP A group B

Haemophilia is incurable 4.1 4.3 3.8 3.8 4.8

Haemophilia can turn 3.9 4.0 3.7 3.4 4.4
into a life-threatening
disorder
I am aware about the 3.9 3.3 4.4 3.5 4.3
types of haemophilia
Home treatment is 3.6 4.1 2.9 3.5 4.6
possible
Care for haemophilia 4.2 4.5 3.9 4.0 4.4
requires constant
flexibility and
continuing resources
MOHAN et al. |
      5

TA B L E 3  Attitude—state-wise and age-wise variation in mean scores

Mean score Overall Karnataka UP Age group A Age group B

I would be able to overcome all the upcoming sporadic, unpredictable 3.9 3.8 4.1 4 4.1
times of crisis
My family and I am receiving appropriate guidance and counselling. 4.2 4.3 4.1 4.4 3.6
I am responsible for my own care 4.6 4.6 4.6 4.5 2.9
I feel the Government is doing enough to relieve the affected person of 4.1 4.3 3.9 4.3 3.7
the financial burden caused due to disease.

TA B L E 4  Behaviour—state-wise and age-wise variation in mean scores

Mean score Overall Karnataka UP Age group A Age group B

I keep myself psychologically healthy by talking to people 4.4 4.4 4.4 4.4 4.1
I share with people about my disorder so that they are aware and 4.1 4 4.2 4.1 4.2
create a safe environment
I have a response plan to my bleeding episodes 4.2 4.4 4 4 4.7
I take utmost physical care of my body since I have bleeding disorder 4.4 4.7 4.2 4.3 3.8

haemophilia A and 60% of them suffered spontaneous bleeds in the
last 12 months implying that they suffered from severe haemophilia
and often experience spontaneous bleeding.10
Chronic pain was the most common co-morbidity found in the
study. This supported the HERO study where over a third of PWH
18
experienced chronic pain.
5.2  |  Knowledge about haemophilia
Knowledge of haemophilia can be categorized under three sub factors—
fundamental knowledge, technical knowledge and knowledge about
management of haemophilia. Fundamental knowledge will include basic
tenets of haemophilia such as genetic nature of haemophilia or that it is a
life-long condition or that it is manageable. Technical knowledge relates to
the different types of haemophilia, factors associated or its various symp-
toms. Knowledge about management of haemophilia relates to aware-
ness about home management or maintaining a treatment diary etc.
Variation in knowledge of respondents across the two age groups
is understandable and it is supported by Hosseini et al12 study that
it is important to consider the developmental stage of target popu-
lation while designing knowledge materials for the patients. Further,
variation in knowledge in the two states too needs to be addressed.
5.3  |  Attitude towards haemophilia
Attitude towards Haemophilia can be grouped into broad sub
heads—grievances, financial burden and feeling of insecurity.
Findings support prior research that patients suffer from feelings of
insecurity, feelings of not fitting in with their peers, lack of confi-
dence, uncertainty with respect to career, anxiety about future, fam-
ily and spouse etc.9,17
Also, most of them felt that they are under constant supervision
which they take as interference in their privacy. This aspect of emo-
tional health is very important and needs to be addressed through
counselling and other stress management techniques.
Almost all respondents feel that their condition is financially bur-
densome and it is in sync with previous studies.3,7
Younger respondents did not show extreme emotional issues.
The respondents in older age group will need repeated counselling
and workshops for changing their inherent feelings of uneasiness
vis-à-vis their condition. Previous studies brought out the issues like
marriage and forming intimate relationships,14 planning of special
interventions and preventive strategies for early age group, better
management of physical and psychological well-being for older age
groups.17 Disease-specific social networking enhances compliance
with treatment and improves quality of life.15
5.4  |  Behaviour of people suffering from
haemophilia
Behaviour of haemophilia patients can be grouped into—immediate
response, preventive care and Self-care. Most responded that they
take care of themselves. Neilson et al11 indicated that patients with
mild haemophilia rarely disclosed their haemophilia or took the ad-
vice of doctors seriously as they wanted to be the same and not dif-
ferent from their peers.
Findings indicate that patients wait for their symptoms to im-
prove with time and do not approach for medical help immediately.
Adherence to treatment 21 and accessibility of the treatment cen-
tre16 worsens the disease morbidity.
Respondents stay away from outdoor sports or activities which
can increase the chances of a bleeding episode; however, they need
to be educated about significance of daily physical exercise, sports and
 |
6      MOHAN et al.

physiotherapy which will help their joints remain active and enhance

I keep myself psychologically

healthy by talking to people
immunity. People with haemophilia experience the same benefits of ex-
ercise as the general population, being physically healthier and enjoy a
higher quality of life through social inclusion and higher self-esteem.18
The study indicated that the treatment was mostly done on
demand. Most individuals with haemophilia treat bleeding epi-
sodes when they occur.10 Khair et al. reported that the goal of

0.27*
0.14
zero bleed was achievable for 32% of patients who received pro-
-
phylaxis treatment. 20 Prophylaxis treatment which involves tak-
I share with people about my disorder so that they

ing precautionary factor level check will need to be undertaken

for better management. Most of our respondents were not aware
about home treatment. In countries like India, where the popu-
are aware and create a safe environment

lated density is high and resources are limited, focus needs to be

laid on home treatment.
While there was a felt need to induct more staff for providing
care for haemophilia, enhancement of knowledge of paramedi-
cal and nursing staff through awareness lectures is a step towards
changing the levels of attitude and developing effective national
haemophilia programme.5
−0.09

0.058

6  |  CO N C LU S I O N
-
I have a response plan to my

The study points out that while knowledge for haemophilia was found
to be satisfactory, its conversion into behaviour specifically was not
up-to mark. There was a gap in term of technical awareness as well as
bleeding episodes

knowledge about management of haemophilia. Counselling should help

in boosting confidence and managing emotional health of patients.
Small sample size was the limitation of the study. However, our study
0.198*

sheds light on a few unanswered questions and opens up way to more

0.25*
0.24*

studies which need to be undertaken. A large study of KAB of Haemophilia

patients is imminent. It will help in charting out effective policies.
I take utmost physical care of my body

AC K N OW L E D G E M E N T S
We would like to thank the contributions Ms. Pratigya Kwatra, for
since I have bleeding disorder

her assistance in analysis and Mr. Rushil Gupta for the desk review.

D I S C LO S U R E
"The authors stated that they had no interests which might be per-
ceived as posing a conflict or bias".
*p value is significant at 95% confidence interval
−0.112
0.25*
0.17*
0.04

DATA AVA I L A B I L I T Y S TAT E M E N T

The data that support the findings of this study are available from
the corresponding author upon reasonable request.
Treatment can be done at home.

unpredictable times of crisis

TA B L E 5  Correlation matrix

Care for haemophilia requires

all the upcoming sporadic,

I would be able to overcome

ORCID
constant flexibility and
Haemophilia is incurable

continuing resources

Richa Mohan  https://orcid.org/0000-0003-0225-527X

Nita Radhakrishnan  https://orcid.org/0000-0002-7941-5641

REFERENCES
1. World Federation of Haemophilia. Global annual survey 2018.
http://www1.wfh.org/publi​c atio​ns/files/​pdf-1731.pdf. Accessed
July 12, 2020.
MOHAN et al.
2. Ghosh K, Shukla R. Future of haemophilia research in India. Indian
J Hematol Blood Transfus. 2017;36:1-2: https://doi.org/10.1007/
s12288-017-0872-2
3. Kar A, Phadnis S, Dharmarajan S, et al. Epidemiology & social costs
of haemophilia in India. Indian J Med Res. 2014;140(1):19-31.
4. World Haemophilia Day 2019: 80 percent haemophilia cases un-
diagnosed in India according to doctors, publishd April 17, 2019
on https://www.hindu​stant​imes.com/healt​h/world-haemo​philia-
day-2019-80-perce​n t-haemo​p hilia-cases-undia​g nosed-in-india-
accor​d ing-to-docto​r s/story-RoguP​3 LSSK​Y1BeE​N bEFZ​V L.html.
Accessed July 12, 2020.
5. Shrivastava N, Shah N, Seema N. Assessment of knowledge about
hemophilia, a genetic bleeding disorder amongst paramedical and
nursing students. Int J Multidiscip Res Dev. 2016;3:168-171.
6. Ghosh K, Shetty S, Sahu D. Haemophilia care in India: innova-
tions and integrations by various chapters of haemophilia feder-
ation of India (HFI). Haemophilia. 2010;16(1):61-65. https://doi.
org/10.1111/j.1365-2516.2009.02097.x
7. Dharmarajan S, Phadnis S, Gund P, et al. Out-of-pocket and cata-
strophic expenditure on treatment of haemophilia by Indian fami-
lies. Haemophilia. 2014;20:382-387.
8. Singh P, Mukherjee L. Cost-benefit analysis and assessment of
quality of care inpatients with hemophilia undergoing treat-
ment at national rural health mission in Maharashtra, India. Value
Health Reg Issues. 2017;12C:101-106. https://doi.org/10.1016/j.
vhri.2016.11.003
9. Aledort L, Bullinger M, Von Mackenson S. Why should we care about
quality of life in persons with haemophilia? Haemophilia. 2012;18(3):1-
4. https://doi.org/10.1111/j.1365-2516.2012.02771.x
10. Nazzaro AM, Owens S, Hoots K, Larson KL. Knowledge, attitudes,
and behaviors of youths in the US haemophilia population: results
of a National survey. Am J Public Health. 2006;96:1618-1622.
11. Nilson J, Schacher C, Mulder K. A qualitative study identify-
ing the knowledge, attitudes and behaviours of young men
with mild haemophilia. Haemophilia. 2011;18(3):1-6. https://doi.
org/10.1111/j.1365-2516.2011.02714.x
12. Hoseini FA, Valizadeh L, Zamanzadh V, et al. Knowledge, atti-
tudes and practices of preventing complications among adoles-
cents with severe hemophilia. Thrita. 2014;3(2):e8758. https://doi.
org/10.5812/thrita.8758
|
      7
13. Babington-Ashaye A, Diop S, Geissbuhler A, de Moerloose P.
Unravelling the knowledge, beliefs, behaviours and concerns of
Persons with Haemophilia and their carriers in Senegal. Haemophilia.
2020;26(5):1-7. https://doi.org/10.1111/hae.14040
14. Blamey G, Buranahirun C, Buzzi A, et al. Hemophilia and sexual
health: results from the HERO and B-HERO-S studies. Patient Relat
Outcome Meas. 2019;10:243-255. https://www.dovep​ress.com/
by178.171.108.96.
15. Khair K, Holland M, Carrignton S. Social networking for adolescents
with severe haemophilia. Haemophilia. 2011;18(3):1-7. https://doi.
org/10.1111/j.1365-2516.2011.02689.x
16. HERO study. http://www.heros​tudy.org/hero-insig​hts.html.
Accessed June 20, 2020.
17. vonMackensenS,GringeriA,SiboniSM,etal.Health-relatedqualityoflifeand
psychological well-being in elderly patients with haemophilia. Haemophilia.
2011;18(3):1-8. https://doi.org/10.1111/j.1365-2516.2011.02643.x
18. Negrier C, Seuser A, Forsyth A, et al. The benefits of exercise for
patients with haemophilia and recommendations for safe and ef-
fective physical activity. Haemophilia. 2013;19:487-498. https://
doi.org/10.1111/hae.12118
19. Khair K, Gibson F, Meerabeau L. The benefits of prophylaxis: views
of adolescents with severe haemophilia. Haemophilia. 2011;18(3):1-
4. https://doi.org/10.1111/j.1365-2516.2011.02644.x
20. Uttar Pradesh state budget analysis. https://www.prsin​
dia.org/
sites/​d efau​l t/files/​b udget_files/​S tate​% 20Bud​g et%20Ana​l ysis​
%20-%20UP%202019-20%20fin​al.pdf. Accessed August 15, 2020.
21. Challenges in the management of haemophilia A, 2019, https://
medic ​ a lly.roche.com/conte ​ n t/dam/pdmah ​ u b/non-restr ​ i cted/​
haema​t olog​y/haemo​p hili​a /Chall​e nges​% 20in%20the​% 20man​
ageme​nt%20of%20Hae​mophi​lia%20A.pdf. Accessed July 2, 2020.
How to cite this article: Mohan R, Radhakrishnan N,
Varadarajan M, Anand S. Assessing the current knowledge,
attitude and behaviour of adolescents and young adults living
with haemophilia. Haemophilia. 2020;00:1–7. https://doi.
org/10.1111/hae.14229