Cystic Fibrosis South Australia Cystic Fibrosis Queensland Cystic Fibrosis ACT Cystic Fibrosis Tasmania Cystic Fibrosis Community Care (Vic/NSW) Cystic Fibrosis Australia

July 2020

Joint Standing Committee on the National Disability Insurance Scheme Access to NDIS PO Box 6100 Parliament House Canberra ACT 2600

Submission to the Parliamentary Joint

Standing Committee - Agency

Submission

Inquiry: General issues around the implementation and performance of the NDIS

Title: Inclusion of Cystic Fibrosis as a disability type for NDIS funding.

Recommendation: That the Parliamentary Joint Standing Committee:

A: Agree to include cystic fibrosis to the list of disability types eligible for NDIS funded support.

B: Note and discuss the gap between mainstream related supports and the eligibility for NDIS funded health related supports for people living with cystic fibrosis.

Introduction

This is a joint submission from Cystic Fibrosis South Australia, Cystic Fibrosis Queensland, Cystic Fibrosis Community Care (Vic & NSW), Cystic Fibrosis Tasmania, Cystic Fibrosis ACT and Cystic Fibrosis Australia. The CF Federation in Australia is connected through Cystic Fibrosis Australia to the Global CF network.

The national Cystic Fibrosis Federation as a collective share the vision of a future where lives are unaffected by cystic fibrosis (CF).

The CF Federation promote evidence based clinical care and social support structures which will maximise health outcomes and personal potential for people living with CF. This is being achieved via research and development in collaboration with the most successful CF centres in the world.

Cystic Fibrosis Federation has established programs over 60 years, which provide a range of support to people living with CF including airway clearance and respiratory equipment, assistance with essential pharmaceuticals, welfare payments for food or utilities, respite, education and information, in home and in hospital support and advocacy. However, it is impossible for charities to fill all of the gaps and the people most in need will continue to miss out on the daily support that provide them with choice and control and which is necessary for them to achieve their goals. The community of people living with cystic fibrosis represents over 3500 people across Australia.

Cystic Fibrosis Federation funding relies on submissions for government funding for specific time limited projects, staff support, fundraising efforts, donations and philanthropic grants to meet the needs of our community. There is acknowledgement in Western Australia a specific state government funding arrangement is in place.

The purpose of our submission is to seek agreement from the Joint Standing Committee on two issues.

That CF should be added to the list of disability types eligible for NDIS funded support, and;
To acknowledge that there are gaps between mainstream related supports and the eligibility for NDIS funded health related supports for people living with cystic fibrosis.

Cystic Fibrosis

Cystic fibrosis (CF) is the most common life limiting, recessive genetic condition affecting young Australians. Primarily affecting the lungs and the digestive system CF results in ongoing damage to lung tissue and can also cause malnutrition, bowel and liver disease, diabetes, and osteoporosis.

There is no cure for cystic fibrosis and median life expectancy for people with CF is currently 47 years with respiratory failure being the most common cause of death. There is approximately 3500 people living with CF in Australia. Just over 50% of them are Adults.

In October 2019 health related supports where included in the NDIS, which we applaud. However, people who live with CF and experience permanent functional impairment as a result of their condition, remain excluded from the list of disabilities recognised by the NDIS. People with CF experience gaps in support and inequity of health care due to this oversight.

Many people with CF answer yes to all the eligibility questions on the NDIS checklist.

NDIS Eligibility Check List for people with CF


Do you usually need support from a person because of a permanent and significant disability?	✓
Do you use special equipment because of a permanent and significant disability?	✓
Do you need some supports now to reduce your future needs?	✓
Do you have a permanent disability (lifelong impact)?	✓
Do you have a significant disability (substantial impact on daily activities)?	✓
Will early intervention reduce the impact of your disability & build skills and independence?	✓

The NDIS was designed to provide people with a disability access to support and services to help them in their daily life, to facilitate participation in the community, and to assist them to reach their goals.

Every person with CF has a permanent disability, the significance of their disability varies from person to person. Every person with CF will have a greater chance of

achieving their goals and living independently if they have access to appropriate funded supports, this could be achieved through an NDIS plan.

Mainstream Health System

The Medical model of care for CF begins with early intervention and proactive treatment as soon as diagnosis is made. This means that in most cases, from a few weeks of age, people with CF have undertaken a daily regimen of medication, nutrition supplementation and physiotherapy in order to breathe, grow and fight infection. They are highly motivated people who work hard every day to stay alive. However, the same early intervention model is not in place for other supports. A person with CF can rarely access disability supports unless they are in palliative care or waiting for a lung transplant. Early intervention could be the most valuable service the NDIS could provide to people with CF ensuring they remain in control of their health, manage the daily burden of CF, access employment, maintain independence and achieve their goals.

Mainstream Health Care is not always accessible when it is needed, CF care teams in public hospitals do an exceptional job and are passionate about delivering the best medical care to their patients living with CF. The reality is that people with CF do not all live within a reasonable distance of the nearest CF care team. They often need help between routine (quarterly) clinic appointments, each of which can be up to three hours long. All would benefit from care being delivered local to them and especially in their home if they are very unwell. Access to local care will be particularly beneficial to people who live in rural, remote, and regional communities.

On the occasions a person with CF needs, for example, additional physiotherapy, nutrition or psychological support, access to timely care (early intervention) will make a significant difference to their health outcomes.

It is crucial that local care providers have the appropriate training to deliver care to people with CF and the delivery of training may need to be negotiated with the public health system in each state. Currently the vast majority of CF experts in each medical and allied health field are employed in the public health system.

Importantly, not every person with CF will choose to seek local or additional support but it is reasonable that they have the choice and means to do so when they need to.

CF is a permanent, incurable, degenerative condition. Physical Impairment attributed to CF is unlikely to be reversed although with targeted medication, physical therapy, and appropriate emotional support, a person with CF may live a fulfilling life even while experiencing significant impairment for many years. Some examples of physical impairment include:

Permanent and significant damage to lung tissue resulting in reduced lung capacity and permanent shortness of breath. Pathophysiology includes hemoptysis (coughing up blood) and chronic fatigue. Impairment increasing in severity over time to impact the person’s ability to:
- laugh, talk or sing
- exercise or participate in sport
- prepare meals or undertake routine household chores
- work or volunteer
- carry out personal hygiene tasks
- undertake medical treatment at home independently
- walking and accessing public transport
Postural deviations with associated back pain are caused by persistent coughing and shortness of breath, over time the condition is exacerbated by reduced thoracic capacity due to hunched back. Postural deviations and back pain impact a person’s ability to:
- Work or volunteer
- Study
- Sleep
- Carry out routine household chores
- Enjoy normal social activities
- Walking and accessing public transport
Pancreatic dysfunction resulting in non-production of enzymes affecting the absorption of nutrients impacting a person’s ability to:
- Consume the calories required for adequate nutrition, up to 2x the calories required by a person who does not have CF (many young people with CF will have a feeding tube (PEG) inserted in their stomach and receive additional nutrients overnight)
  - PEG feeding exacerbates social isolation for people with CF
- Maintain healthy weight
- Fight infection and respiratory exacerbations
- Participate in normal physical activity
- Pancreatic insufficiency results in early diagnosis of Osteoporosis in people with CF and limits their ability to participate in many physical activities due to the risk of bone fracture

Diabetes is 30% more likely to affect a person with CF than the general population and onset of diabetes is likely to occur in their 30’s. CF diabetes is particularly difficult to manage because of the dietary requirements of a person with CF. Additionally during routine glucose testing CF related diabetes does not behave like Type 1 diabetes and the true nature of the condition can be difficult to diagnose. Diagnoses of diabetes has a significant emotional impact for a person with CF because it is associated with hastening disease progression. Diabetes impacts the person’s ability to:
- Work
- View the future positively
- Play sport or exercise
- Participate fully in family and social activities
  - Complications over time may affect the feet, eyes, kidneys, and cardiovascular health
Peripheral muscle weakness is related to exercise intolerance and is common for adults with CF who have experienced the disabling effects of the above impairments. The impairments caused by peripheral muscle weakness include:
- Pelvic floor weakening resulting in permanent incontinence
- Poor posture resulting in back, neck, chest and torso pain
Due to the environment within a CF lung, people with CF are at high risk of contracting infection from another person with CF. Cross Infection Risk for people with CF impacts a person’s inability to:
- Use public transport
- Attend large gatherings
- Work
- Participate fully in study opportunities - i.e. camps or excursions
- Attend family events
- Participate in usual social activities their peers enjoy
Chronic fatigue impacts many people with CF, even those with relatively high or stable lung function and can impair their ability to perform daily routine tasks. Chronic fatigue can be associated with shortness of breath, reduced blood oxygen levels, poor sleep, anxiety, and the treatment burden of CF.

Additionally it is preferable that people with CF do not perform high risk tasks like vacuuming, dusting and cleaning the bathroom. Activities that substantially increase exposure to airborne bacteria and fungi (mold), increase their risk of lung infections and significant lung damage. During times of lower functional ability, it is particular important that people with CF have additional support in this area.

It is strongly recommended that people with CF minimise exposure to fumes, dust and disease-causing microorganisms in the workplace. This requires the person with CF to be able to negotiate changes to workplace practices where necessary. However, it may

make it very difficult for people with CF to enter or remain in the workforce at all. People with CF would benefit considerably from support to access appropriate employment and to negotiate with employers.

The psychological impact of living with a chronic, life limiting condition manifests in elevated levels of depression and anxiety in people with CF. Psychological distress impacts a person’s ability to:
- Adhere to treatment and medication routines
- Exercise or participate in sport
- Study
- Work
- Sleep
- Undertake normal personal hygiene and household tasks
- Prepare nutritious meals
- Attend CF clinic appointments

People with CF (and parents of children with CF) report overwhelmingly their experience of anxiety related to hospital visits. CF Clinic appointments, routine procedures and allied health care is delivered in Public hospitals. Improvements in understanding the role of anxiety in overall wellbeing suggests that avoiding unnecessary hospital visits by delivering care locally or in the home will have a positive impact on health outcomes for people with CF.

Other Pathophysiology which impacts a person with CF can include:
- Sleep apnea
- Hearing loss from antibiotic use
- Peripheral neuropathy from antibiotic use

Most people with CF will experience some of these conditions some of the time but not every person will experience all of them. Respiratory failure is the most common cause of death for a person with CF and respiratory symptoms mentioned above impact almost every person with CF from birth. The progress of CF disease may be slowed with good medication, treatment and adherence to treatment regimens and nutrition, but it cannot be stopped, reversed or cured.

The Health Response to people living with CF

The Health System response to cystic fibrosis is based on global evidence for multidisciplinary team care within the state and territory health systems and delivered through major public hospitals. While this centralised model provides effective medical interventions and achieves very good health outcomes, it does not provide a system of support, nor does it subsidise or fund vital health equipment, now identified in the NDIS list of health-related supports.

Cystic Fibrosis Functional impairments

From the list of functional impairments, it becomes apparent that CF is a complex condition which becomes more difficult to manage as the person with CF ages. Throughout their life they will experience regular respiratory and gastroenterological exacerbations which may be life threatening. These episodes result in medical intervention and at times long term hospitalisations. After each episode, the person with CF expects to live with decreased lung function or an increase in digestive complications. People with CF who have experienced a normal disease progression may have spent as much as two years in hospital by the time they reach adulthood. The impact of a severely interrupted education can be inability to access tertiary education, trade training or employment.

Most people with CF spend up to two hours a day undergoing treatment and physiotherapy, just to be able to breathe. Daily treatment regimens serve to maintain existing function and delay disease progression.

CF daily treatment, exacerbations and hospitalisations place a huge burden of care on the person with CF, their parents, partners or carers and on the hospital system.

For many, managing cystic fibrosis is equivalent to a part-time job (20h per week) when not experiencing an exacerbation and a full-time job (50+h per week) when experiencing an exacerbation.

Living independently, working and Income:

The Federal Government’s Department of Social Service response to people living with cystic fibrosis is to include it in the list of recognised disabilities on its website (below). This means that carers of children with CF can access some Commonwealth payments to assist in the care of their child. Unfortunately, a significant number of parents are reporting a loss of support when their child turns 16. At an age when support is so vital to their success in education, transition to adulthood and access to employment, these people are left behind.

This reinforces the inclusion of cystic fibrosis in the list of disabilities eligible for funding via NDIS, when it is already recognised as such by the Federal Department of Social Services.

https://www.dss.gov.au/our-responsibilities/disability-and-carers/benefits-payments/carer-allowance/guide-to-the-list-of-recognised-disabilities

Access to an NDIS plan will have far reaching positive impacts on people with CF, including but not limited to:

Guaranteed access to airway clearance and other essential respiratory equipment
Improved Management of CF related Diabetes (less associated comorbidities)
Assistance with household chores and personal hygiene when needed
Assistance with transport to attend medical appointments when needed
Ability to live independently and achieve personal development goals
Ability to remain in education
Ability to remain in daily occupations such as paid and voluntary work
Access to personal fitness training programs which avoid cross infection and improve muscle strength, fitness, posture, and airway clearance
Reducing financial distress
Improve engagement in work, life, community and reduce social isolation
Reduced hospital stays because of decreased exacerbations
- The average cost of an overnight hospital stay in Australia is $4680 according to the Australian Institute of Health and Welfare

Conclusion

It is important to understand that while CF is a complex condition, it does not impact every person the same way at the same time. It is likely that many people with CF will access a small number of supports at the time they need them. Accessing reasonable NDIS funding when it is necessary will ensure people with CF have the support that they need to keep control of their health, to make choices about their health care and achieve their goals while living with a permanent and significant disability.

The purpose of this submission is to seek agreement from the Joint Standing Committee on two issues.

That CF should be added to the list of disability types eligible for NDIS funded support.
To acknowledge that there are gaps between mainstream related supports and the eligibility for NDIS funded health related supports for people living with cystic fibrosis.

Cystic fibrosis is recognised as a disability type by the Federal Department of Social services and we have demonstrated above that CF manifests in permanent and life shortening significant disability. It is reasonable to accept that people with CF have

access to NDIS funded support to provide them with choice and control over their supports and the tools they need to achieve their life goals.

Signatures

Cystic Fibrosis SA	Cystic Fibrosis Queensland
Cystic Fibrosis ACT	Cystic Fibrosis Tasmania
Cystic Fibrosis Community Care	Cystic Fibrosis Australia