Unfortunately, there are few treatment choices for IH, there are no medications specific for the illness.

The treatment is centred around the stimulant/wake promoting drugs that are prescribed for Narcolepsy.  Lifestyle changes, around sleep hygiene, may make some little difference. However people with IH have little problem going to sleep anyway.

The key point here is that all of these drugs treatments aren’t approved for IH and must be prescribed “off label”.

Off label prescribing

The European Medicines Agency has not approved any treatments for central hypersomnias except Modafinil for Narcolepsy. The UK’s National Institute for Health and Care Excellence (NICE) and the Medicines & Healthcare Regulatory Agency (MHRA) will take on this role after Brexit.

As such any prescriptions are normally provided “off label” by a hospital consultant. “Off label” means a drug is prescribed in a way not approved by its EU (UK) product licence. However in no way does this means it is unsafe.

Your consultant will also have made a risk assessment in line with their Trust’s guidelines. This is to see whether it is suitable for you and your condition given your own circumstances.

Shared Care

Some GPs are happy to prescribe these medications once the consultant is satisfied with your treatment plan. This is known as Shared Care.

Shared Care is negotiated directly between the consultant and the GP on behalf of the patient. This may be done in conjunction with the Clinical Commissioning Group (CCG) who regulate local budgets. The local Formulary who medicate drug prescribing will also have a say.

In Scotland group shared care agreements can also be agreed for specific illness groups and patients.

Post-Brexit, it is unlikely that there will be a major shift in this stance without some form of advocacy by patients and medical professionals.

Medicinal treatments

The first-line treatment for IH involves wake-promoting (stimulating) drugs. The drug of choice seems to be Modafinil.

Modafinil is what is known as a “racemic” compound. It has 2 forms – enantiomers –  (R and S), or left and right handed versions. These are chemically identical, but mirror images of the chemical compound. In some countries (but not usually the UK) Armodafinil, is also prescribed. It is pure R enantiomer of Modafinil and is a longer-lasting, and stronger version, of Modafinil.

Other drugs include Methylphenidate (as used with ADHD under the name Ritalin), dextroamphetamine, and sometimes Pitolisant (Wakix). Methylphenidate can be prescribed in both instant and extended release forms.

A Consultant will often start a patient on a low dose to assess the effectiveness of the treatment. This gives room to increase the dose if the patient become acclimatized to the treatment regime.

Doctors also use this period to monitor their patients’ blood pressure, and check for heart issues with an ECG. These drugs have the capability to increase patients’ blood pressure which can cause problems in its own rights.

They can also assess how well the treatment works and which side-effects manifest, and if these are manageable by the patient or with other therapies.

Some patients may find the drug treatments ineffective, or hard to handle. However, as there are limited options available this leaves little room for manoeuvre  in developing an effective treatment strategy. As such patients often accept a treatment if it isn’t totally effective.

Other drugs are in clinical trials worldwide, but few or none in the UK. The UK is behind the USA and Europe in trials for existing and new drugs, and this is unlikely to change in the short term.

My belief is not to expect any changes to occur on this front in the near future short of NICE changing its attitude to new drugs for IH.

Note: Methylphenidate and dexamphetamine are Class B drugs according to the law.  Pharmacists may make patients provide more information and sign for them when dispensing such medicines.  As such you need to be careful in their use and when carrying them around.

This is based on my personal experience. You may wish to develop your own strategy.

I had to talk to my GPs and explain the illness to them. They had little or no knowledge of IH and I needed to educate them. However they knew my complete medical history and had the training and experience to help manage the changes required. This included:

• lifestyle changes
• side effects
• interaction with other medications
• the impact of the illness and drugs on my mood or anxiety levels.

The discussion about interactions and side effects is very important and this was a worthwhile conversation.

One thing not relevant to me, also came into the discussion:

It is very important for women on modafinil taking the contraceptive pill, or planning to get pregnant, to discuss this with their doctor. Modafinil both reduces the impact of the contraceptive pill and has an impact on pregnancy. (Canadian researchers think this is now reality).

Current advice is to NOT take ANY stimulant medication during pregnancy, but this is a discussion that needs to be had between the mother to be and her medical team.

Clinical Guidance on drug Interactions with hormonal contraceptives

I’m not sure how we got on to it, but I thought that was a useful piece of information.

The other main aspects of managing IH was to make certain lifestyle changes and establish a routine. However this is still not an easy task for many.

After diagnosis the hardest part was accepting the limitations on my life and lifestyle. However I felt that I should still maintain goals and aims in life.

IH does not define who we are or what we can achieve.

Knowing these limitations exist has allowed me to develop some plans for the future. These take into account some of the issues,  and actually add more purpose and direction. When you know your free time is limited, you enjoy it that bit more.

These routines can include:

• Managing when to sleep and wake on a daily basis (very popular with doctors)
• Taking medication at the same time every day
• Drinking lots of fluid and having regular meals
• Planing out the day. (This last one can often be the hardest as problems still occur and throw plans to the wind).

Regular exercise is meant to help especially as time in the sun can help improve mood and general health. This can be something simple as just walking the dog.

Unfortunately, these drugs can also affect one’s appetite and diet, increasing or decreasing weight. You may need to look at amending the prescription, or changing what you eat and when.

For many, carbs are one solution to trying to address the inertia or brain fog. However if that is the case, you may wish to reduce this and improve the amount of protein and veg in your diet instead.

There is also the issue of getting a “slump” in the afternoon, prior to a pick up in the evening. This may be corrected by amending when you take your medication, as often the evening is the best time for people with IH.

It appears many people with IH are categorised as night owls too.

Even when medicated there may still be a need to nap, though nap is not the best description. IH sufferers have to put up with long deep naps unlike the short narcolepsy power naps. In addition having to endure the same period with sleep drunkeness is often still part of your life.

From current planned naps also have no impact on IH, unlike with Narcolepsy. Issues wakening is particularly strong in sufferers of the illness.

Sleep Hygeine

Good sleep hygiene is suggested on all NHS and sleep websites, but its impact is not fully known, as it is more relevant to people who cannot sleep.

The main rules are:

• Optimise where you sleep and make it comfortable and relaxing.
• A bedroom should not be a TV room; sleeping and sex are the only purposes here.
• Make sure the room is not too warm, and that any external light is excluded, and establish a routine for going to sleep.

Other issues around sleep

People with IH should not have any issue both getting to sleep or staying asleep. If you have issues there go and talk to your sleep doctor as a priority as you may have a different hypersomnia.

As mentioned before, the big issue for IH patients is waking up from sleep. If you work, you will need to establish a waking strategy that may involve alarm clocks and other people. Engage as many senses as possible when trying to wake, such as touch and smell as alarms can be ignored.

This is individual to each IH patient, but is crucial is managing a routine and having a purposeful day.

Waking up is an issue for many as it often interferes with the circadian rhythm set by IH. This is often out from the standard 24-hour cycle. Waking up when your body dictates is easier, but this may not be possible due to work or family commitments. As such there is often trial and error to find the right solution for you.

Alcohol is also known to have a disrupting influence on sleep, and can be a sedating influence for people with IH. You may wish to avoid it in the late afternoon or evening to maximize the time awake at night. However, now and then, a glass of wine or beer with friends is just what is needed to stay social and happy.

Whilst receiving a diagnosis and treatment plan may be a relief to suffers, it is not the answer that many seek. Adjustments have to be made to manage the disease. As stated before, not all patients find that the drug therapies work as well, or as long, as expected.

One way of adjusting to the illness is to pace one’s day. Understand that trying to achieve too much may have consequences later in the day or on following days. In addition, there will be days when it is still hard to awaken even with medication. Also the EDS feeling and in-opportune naps will have an impact despite taking regular medication.

Another thing to do includes getting those around you to understand the impact IH has on your life. This includes how sometimes things can become beyond ones reach.

Mood disorders and depression can go hand in hand with the illness too. A question exists of whether depression causes hypersomnolence or does hypersomnolence cause depression? For many, managing such a debilitating disease is very hard, without factoring in family life, studying, holding down a job, or claiming benefits.

As such maintaining good mental health is key. Few observations have been made on how this can be satisfactorily achieved. Talk to friends and family about how you’re feeling and use online resources such as the 2 main IH support charities, and other resources to stay informed.

Try not to let others “shame” you for your illness, or try to put you down; put people like that out of your life.

Work at staying in contact with friends and society. This may involve you explaining the impact IH has on you and your life and that is is an “invisible” illness, and incurable.

Finally, loneliness and isolation can be one of the greatest un-factored issues around IH. If you’re single look at getting some form of pet if possible or adjusting your social time around IH. Look at accessing some of the free counselling services, or talk to your doctor about further support.