People living with KD
Obtaining support for Kennedy’s Disease outside of the London Clinic at the National Hospital for Neurology and Neurosurgery can be challenging due to a very limited knowledge of the disease by GP’s and Neurologists. As there is no NHS adopted protocol for the management of the illness be prepared to fight your corner hard to secure the local support you need.
The following symptoms/medical issues are associated with Kennedy’s Disease:
Muscle weakness in the arms and legs
Muscle weakness is often the first symptom for many sufferers, and it is generally accompanied with muscle cramps.
As it stands there is no drug treatment to manage the impact of KD on muscle, but targeted Physiotherapy and Hydrotherapy can both help to slow down the progression of the muscle decline.
Hydrotherapy is particularly helpful for improving balance as falling is a major issue for KD patients. There are a limited number of hydrotherapy pools in the UK but if you don’t have access to one you can go to your local swimming pool, in a quiet period, armed with some exercises from your physiotherapist.
Most hospital groups have a specialist neuro physiotherapist, and you should ask your GP or Neurologist for a referral. There are also many excellent private physiotherapists who understand neurological diseases. The NHS has a habit of giving you a couple of sessions and then signing you off. You need to make it clear to your therapist that Kennedy Disease is a progressive illness and that you will need ongoing support.
Kennedy Disease patients also invariably have a marked hand tremor and fasciculations under the skin which will be very obvious to an experienced Neurologist.
Click above to download a protocol for managing the disease that has been prepared by the French neurologist Pierre Pradat. You may wish to give a copy of this to your GP, Neurologist, family members, or any health professionals you are working with to help inform them about the illness.
Kennedy Disease can manifest itself in many ways. As the disease progresses you may need support from a multi-disciplinary team. Please do note that very few patients develop all the symptoms that are associated with the illness and the speed of onset varies considerably from patient to patient.
If you can make it to the specialist Kennedy Disease Clinic in London, they will monitor you for many of the conditions below and recommend treatment/management paths.
If you cannot make it to the clinic please click here for other routes to access care.
KD-UK has a partnership with TGA Mobility. They will give people living with the disease a 10% discount on all their excellent products. Please simply mention you are a Kennedy’s Disease patient when ordering. Click Here to visit their website.
Muscle weakness in the bulbar region
As the disease progresses many patients have difficulty with clear speech, swallowing and chewing food. This is due to the impact of the illness on the bulbar muscles.
Engaging with a Speech and Language Therapist can help you to manage the symptoms. You can be referred to a local Speech and Language Therapist by either your GP or a Neurologist.
Another common Kennedy Disease symptom is dry drowning or laryngospasm. It is the result of a spasm in the adductor muscles – the muscles closing or bringing the vocal folds together. Laryngospasm happens when your voice box or the area of the windpipe below the voice box detect the entry of water or another substance. The vocal folds spasm and the airway shuts down. The vocal cords very immediately and very effectively close. Chewing foods longer, not trying to talk while eating, and not gulping liquids will all help. It is also found that daily throat exercises have helped minimize choking. It’s alarming when it happens but it is not dangerous. Please click here for more information. https://kennedysdisease.blogspot.com/2010/05/are-you-afraid-to-go-to-sleep.html
Peripheral Neuropathy
Another relatively common early symptom of the illness is Peripheral Neuropathy or nerve damage. This results in number fingers/feet, tingling and sometimes a sharp, jabbing, nerve pain. Excessive nerve pain can be treated with drugs and if you have peripheral neuropathy symptoms you should ask your GP to refer you to a Neurologist.
The Neurologist is likely to commission nerve conduction studies to determine the extent of your neuropathy.
Gynaecomastia
Gynaecomastia, or excessive breast growth, is another common early symptom of Kennedy Disease and often occurs before the onset of muscle weakness. It is often the first symptom patients present with and it occurs in about 75% of patients.
It is not a serious health issue but if it results in pain, or causes significant anxiety, it can be treated through surgery.
Respiratory Issues
In the later stages of the illness some, but by no means all, patients have respiratory issues and suffer from sleep apnoea. Some also suffer from a weak cough. If you believe that you are having these issues, then you need to ask year GP to refer you to a respiratory specialist who can arrange for the appropriate tests to be carried out.
There are many treatments for diagnosed respiratory issues that include CPAP, BIPAP and cough assist machines. Ventilation is very rarely needed for Kennedy Disease patients.
Osteoporosis and Osteopenia
Kennedy Disease patients are susceptible to Osteopenia and Osteoporosis as the disease progresses. With the frequent falls that are inevitably part of the life of someone living with Kennedy Disease this can result in fractures.
It is advisable for a diagnosed Kennedy Disease patient to have regular bone density (DEXA) scans to monitor their bone health as a broken hip can be quite devastating. Osteopenia, and Osteoporosis, can be effectively treated with drugs and calcium supplements. Your GP can refer you for the scan and the scan is quick and painless.
Genito Urinary Issues
In some cases, patients may have a reduced sex drive and difficulty getting an erection. A few people with severe illness may also have fertility problems. Hormone replacement therapy is not suitable for Kennedy Disease patients, but your GP should be able to advise you about your other medications that might help.
A minority of patients also suffer from a weak bladder, especially if they develop diabetes. If you feel that you fall into this category, ask your GP to refer you to a Neurologist.
Diabetes
People living with Kennedy Disease have an increased risk of glucose intolerance and developing Type 2 Diabetes. You should ask your GP to regularly monitor the level of glucose in your blood. If you show signs of diabetes, you can be referred to a specialist clinic that can help you manage the disease.
Impact on the Heart
Rarely Kennedy Disease can have an impact on the heart and cause Brugada Syndrome. Brugada syndrome is a rare condition that affects the electrical messages that control your heart rate. This causes an abnormal, very fast heartbeat. Treatment can control the risks and help you manage the condition. Your GP can refer you to a Cardiologist if you have any symptoms.
Recent research would also indicate that Kennedy Disease results in a constant raised level of Troponin T which is a protein that many hospitals measure to determine if you have had a heart attack following chest pain. If you end up in hospital you need to let the A&E Consultant know about this research or you could be incorrectly diagnosed. Some hospitals measure the Troponin I protein and the research indicates that this is not affected by Kennedys Disease making it a better indicator for Kennedy Disease patients.
Anaesthetics
People with neuromuscular disorders must take care if they are to have a local or general anaesthetic. Even someone with very mild, or non-existent symptoms, or someone who has a family history of a disorder, needs to let the anaesthetist know well in advance so that tests can be carried out and proper care after the operation can be arranged.
Psychological Issues
Being diagnosed with KD undoubtedly comes a shock to many and, as the disease progresses, it does significantly impact on thee way that you lead your life. This can lead to health anxiety and depression.
Do not hesitate to raise any psychological concerns with your GP. There are many therapies, that the GP can refer you for, which will help you manage the psychological impact of the illness.
Further information from our partners
HOME ADAPTIONS
Key Messages:
• Make your home adaptations ahead of when you need them. It will increase quality of life and take away a lot of stress as your illness progresses. Some items can have quite a long lead time and Kennedy Disease is a progressive disease. It will only go one way!
• Thoughtful adaptations can significantly help you to reduce your dependence on your Carer. Carers need to be nurtured. We should be under no illusion – most Carers did not choose to be one.
Some Ideas on
Adaptations:
Note: This list is far from exhaustive and what is right for one person living with KD is not right for another. That said, this list has been prepared by someone living with the disease.
Emergency Call System
An emergency call system can be invaluable if you fall over and are unable to get back on your feet. It will also give your family members peace of mind when they leave you on your own. Some watches, including Apple Watch, have an emergency call capability and are more discrete than something around your neck.