LIVING WITH IDIOPATHIC HYPERSOMNIA
Tiffany first shared her story with us in 2016. It started with;
"I sleep a lot. I always have. 10+ hours a night. Every night.
I nap a lot, too. 2 hour naps. More than once a day. Everyday. This is my normal.
The theme of this year's (2016) Idiopathic Hypersomnia Awareness Week is "Improving Quality of Life".
I love this theme as it is something that I have been personally working towards this year." You can read the rest of her early story here.
Tiffany is in a very different place now (2022). The following is a letter Tiffany wrote to her local member, Mr Andrew Hastie MP, the WA State Health Minister and the Federal Health Minister in August 2022. Her letters were part of our IH Awareness Project 2022.
I was diagnosed with Idiopathic Hypersomnia (IH) in 2013 when I was 34 years old and have been living with this diagnosis for almost 9 years. IH affects my life in many ways and makes it impossible to live a normal life.
I am a Medical Scientist and had a very good career but I have always struggled with getting the amount of sleep my body needs due to IH. I sleep an average of 16 hours a day and it is not possible for me to accomplish all the activities of daily living and maintain employment. In 2017 I was medically retired and I am now reliant on the Disability Support Pension. I have lost all the benefits of my employment: the good income, the superannuation benefits, the access to employee benefits, and the career I worked so hard for.
When I was diagnosed I was not offered access to any supports and it is my experience that my medical practitioners do not have a good understanding of the impact that IH has on me and my family.
My cognitive abilities are greatly affected by my IH. It takes me time to wake up from sleep and in this time I experience symptoms of sleep drunkenness. I cannot think properly in this time and make poor decisions. My speech is affected. My balance and spatial awareness are affected and I will bump into walls, furniture, and doorways. This is why my sleep physician has said I am not allowed to drive when I first wake up. It may take me up to an hour to wake up, or I may fall back asleep again during this time. The symptoms of sleep drunkenness will then return after a couple of hours and I will need to sleep again. If I do not sleep I will experience symptoms of sleep deprivation including poor concentration, mood changes, increasing irritability, temper tantrums, emotional explosions and outbursts, overactivity and hyperactive behaviours. These symptoms affect my relationships and make it very difficult to form or maintain friendships. I do my best to manage these symptoms by having regular sleeps through the day. These sleeps will usually be 1-3 times daily and 1-3 hours in duration. This is in addition to 10 or more hours sleep at night.
Even with 16 hours sleep a day I still struggle daily with reduced alertness, shortened attention span, poor judgement, reduced decision-making skills, poor memory, reduced concentration, mentally ‘stalling’ or fixating on one thought, moodiness and bad temper, loss of motivation, making mistakes by forgetting to do something, making mistakes by choosing the wrong option, problems with impulse control leading to risk-taking behaviours.
Public Transport is difficult for me to use unaccompanied as I may fall asleep which places my safety at risk. I also forget to tag off my SmartRider as my cognitive abilities wane after being awake for more than two hours and I have received a number of fines from the WA Public Transport Authority as a result of my forgetting the simple process of tagging my SmartRider or paying for parking.
Recently, I had surgery and it was scheduled for 11am. However, due to an unexpected delay my surgery did not happen until hours later. I had been awake since 9am in order to wake, shower, and drive to the hospital. Hospitals are not quiet environments and so I was unable to sleep properly. I became increasingly sleep deprived and it took all my willpower to wait in my bed for my surgery and not walk out of the hospital. I was extremely irritable and felt like I was ready to explode.
Catching up with family or friends requires careful planning. I will either need my friend to pick me up from home or I need to very carefully manage my time so that I can wake up, drive to a local cafe and get home again before I get too sleepy. I cannot catch up with people spontaneously. I cannot see a movie and then go and have a coffee or dinner afterwards as I will be too sleepy to enjoy the experience.
I have to be careful when I attend medical appointments as these often run late. Recently, I attended an appointment that ran late and I was very close to having to call a family member to collect me as I was very nearly too tired to drive myself home from the appointment. My mother is often the person I will call to help me in this situation.
I am reliant on my family to help with the activities of daily living as my hours of useful wakefulness are very limited. My mother helps with my cooking, cleaning, housework and laundry whilst I sleep as there are not enough wakeful hours in my day to get these things done. My dream and goal is to live independently especially as my mother is ageing and will be 70 this year. It is not fair that my disability is a burden to her.
Accessing support such as the NDIS seems so overwhelming mostly because IH is not a disability that is recognised by the NDIS and the medical community does not seem to understand how disabling this disorder is. I do not have the wakefulness or energy to try and obtain the reports needed to access the NDIS when it means I have to try and explain everything to my doctors about how IH affects me every day. If I had a well understood disorder the need for these supports would not need to be explained. Focussing on the difficulties of my disability is emotionally draining and having to explain time and again how it impacts me is exhausting. I had to do this for Centrelink and I cannot face doing this all over again for the NDIS.
I am anxious about the future. Being unable to continue my career as a Medical Scientist and reliant on the DSP is awful and is not at all how I imagined my life would be. I will have little superannuation for my “retirement years”. The DSP is a fraction of my previous income and does not keep pace with the cost of living.
My life is made harder by the lack of understanding of IH and my having to explain it time and time again. I need IH to be better understood by the medical community and by society. I need to not be explaining my disability every time I meet with a new doctor, or try to access a support service.
What is Idiopathic Hypersomnia (IH)?
Idiopathic Hypersomnia (sometimes referred to as Idiopathic Hypersomnolence in Australia) is a rare neurological sleep/wake disorder. Sufferers have an extraordinary need for excessive sleep yet, despite sleeping for 12 -16 hours (or more) in a 24 hour period, they wake up feeling like they have had no sleep at all.
This results in a range of extremely disabling symptoms including:
Excessive Daytime Sleepiness (EDS) and the overwhelming need to sleep.
Impaired vigilance or sustained attention.
Cognitive dysfunction, especially linked to poor memory.
Dependence on other people for awakening them.
Long (>1 hour) unrefreshing naps and profound sleep inertia, known as sleep drunkenness (elucidated as prolonged and extreme difficulty waking up accompanied by confusion, disorientation, irritability, and poor coordination with an uncontrollable desire to go back to sleep. It can also include automatic behaviour with amnesia, ie: performing tasks without consciously knowing it and not remembering you have done them, eg; turning off alarm clocks or answering the phone). This difficulty in transitioning from sleep to wake (often referred to as the time it takes to “get going” in the morning) can last at least an hour but may be as long as 2 to 3 hours when severe.
Despite extraordinary amounts of good quality sleep, people with IH never feel refreshed; they are in an almost constant state of sleepiness.
The pathogenesis of IH is unknown. An autosomal dominant mode of inheritance has been considered by researchers because around 50% of people with IH have at least one family member who also has symptoms. It appears IH may be more common in females. The prevalence of IH is thought to be 0.005%–0.3% but, due to lack of epidemiological studies, biological markers and misdiagnosis of the disease, the real prevalence is unclear.
Though the symptoms mainly begin in adolescence or young adulthood (typically between 15-30 years of age), it is not uncommon for there to be a delay of many years before an accurate diagnosis is made.
IH is a debilitating, lifelong condition often profoundly affecting work, education, relationships, and mental health. This is particularly the case for those without support.
The House of Representatives Standing Committee on Health, Aged Care and Sport inquiry into Sleep Health Awareness in Australia (2018) found that new drugs and novel medical technologies are urgently needed for people suffering from disorders of hypersomnolence. In the Chair’s foreword of the report, Bedtime Reading, Committee Chairman Mr Trent Zimmerman said “narcolepsy and associated conditions such as idiopathic hypersomnolence can have a debilitating impact on a person’s quality of life. There is a need to improve the awareness of these conditions both within the community and among medical practitioners. Further consideration should be given to emerging treatment options that may provide some people experiencing these conditions the opportunity to improve their quality of life.”
Despite this, nothing has been done. There is no research being done in Australia. There is no formalised education or training on Idiopathic Hypersomnia in the sleep specialty curriculum. The Royal Australasian College of Physicians acknowledges the limitations in their training in the ‘Sleep Medicine Advanced Training Curriculum’, “There are too few training posts in Australia and New Zealand that can provide broad exposure to and quality training in, the whole range of sleep disorders, particularly non-respiratory sleep disorders’. And there are currently no medications on the PBS for IH. There are medications (including Pitolisant, Solriamfetol and Xyway) being used to treat the symptoms of disorders of hypersomnolence, including IH, in other parts of the world that we do not have access to in Australia. This is a significant problem because the medications which are available here are not suitable for many people. Side effects prohibit a large number of people from using them, they don’t address many of the symptoms of IH and their long term inefficiency leaves some people without any options.
Hypersomnolence Australia (HA) has participated in The House of Representatives Standing Committee on Health, Aged Care and Sport Inquiry into Sleep Health Awareness in Australia and the more recent (2020) Inquiry into the Approval Processes for New Drugs and Novel Medical Technologies in Australia, with a particular focus on those for the treatment of rare diseases and conditions where there is high and unmet clinical need. HA has also approached the Australasian Sleep Association (ASA) on numerous occasions about the aforementioned issues, dating back to at least 2014. However, apart from having various ASA members agree that something should be done, nothing has been achieved. People with disorders of hypersomnolence understand that things take time however it is not acceptable for Australia to be so far behind the rest of the world.
People with IH desperately need more awareness, access to better treatment options and they need more support to manage their day to day lives.
Hypersomnolence Australia (HA) does its best to provide support, information, and advocacy for people with IH. However, they are extremely limited due to a lack of resources. HA does not receive funding from any source and they do not charge membership; they provide their services completely free. HA hosted an IH and Narcolepsy Education Day in Brisbane on 10th April, 2022. This was a very worthwhile event, attended by both medical professionals and members of the IH and Narcolepsy patient communities. The event was funded primarily by people in our small community. The general consensus on the Education Day was that patients often receive little or no information when they are initially diagnosed with IH. They are left feeling confused and wondering how they are going to manage the rest of their lives. HA would like to meet this need for information by producing an educational resource for doctors to give to patients when they are first diagnosed. This would be vetted by medical advisors and answer the many questions patients have from the outset. We would also like to curate more events like the Education Day but we can’t do any of this without financial support.
Who is Hypersomnolence Australia?
Hypersomnolence Australia (HA) is the only not for profit organisation in Australia, and was the first in the world, to specifically represent and support the neurological sleep/wake disorder Idiopathic Hypersomnia (IH). Its primary purpose is to support the medical community work toward a better understanding of IH, effective treatment options, support for people affected by IH and raising awareness and educating others about hypersomnolence disorders. HA's goal is to change not just the process to diagnosis but also the level of care and services available to patients post diagnosis. However, HA relies solely on donations to survive. This means that the resources and support it can provide is limited. Please add your voice to our plight by engaging with our social media and consider making a donation to support our work. Learn more at https://www.hypersomnolenceaustralia.org.au.
1. Dauvilliers Y, Bassetti CL. Idiopathic Hypersomnia, Principles and Practice of Sleep Medicine (Sixth Edition) 2017, Chapter 91, pp 883-891.e4.
2. Trotti LM. Waking up is the hardest thing I do all day: sleep inertia and sleep drunkenness. Sleep Med Rev, 35, 2017, pp. 76-84.
3. Vernet C, Arnulf I, Idiopathic hypersomnia with and without long sleep time: a controlled series of 75 patients, Sleep 2009 Jun; 32(6): pp.753-9.
4. Lammers G, Bassetti CL, Dolenc-Groselje L, Jennum PJ, Kallweit U, Khatami R, Lecendreuxi M, Manconi M, Mayer G, Partinen M, Giuseppe P, Reading PJ, Santamaria J, Karel S, Dauvilliers Y. Diagnosis of central disorders of hypersomnolence: A reappraisal by European experts. Sleep Medicine, 2020.
5. The House of Representatives Standing Committee on Health, Aged Care and Sport, inquiry into Sleep Health Awareness in Australia, Bedtime Reading, 2019.
The IH Awareness Project has resulted in over 50 letters going out to politicians around Australia. The aim of the project is to create awareness of IH and the impact it has on the lives of those living with IH and their family. By sharing our stories and relevant information about IH with the politicians entrusted with representing us we hope to influence policy making in a meaningful way.
If you would like help writing to your local member or other politicians please reach out to Michelle Chadwick at firstname.lastname@example.org