Crying is part of normal behaviour and there can often be a simple straight-forward reason that can be easily addressed. However, crying can signify pain or some other cause of distress. In a normal child who can communicate their feelings it can be possible to determine the cause. However, just as in babies or infants who cannot yet talk, individuals with a CDKL5 disorder are not usually able to express themselves or communicate sufficiently. In this situation it can be very difficult to understand and identify a cause and this can lead to considerable distress to parents or carers.
Parents will usually develop a checklist of the common things they know to address if their child starts to cry. It might be something simple like they need a drink or they are hungry. They may need a nappy change or just be tired and need to rest. Sometimes they may need a change of position or be too hot or too cold or perhaps just be in a grumpy mood and need cheering up – just like us! However, if their crying persists despite checking the simple things then it is worth thinking of other causes.
There are potentially a whole range of problems that will cause a child with a CDKL5 disorder to cry and become distressed. Clearly, there will be some uncommon causes and there may even be particular issues that are specific to CDKL5 that we have yet to learn about. It would be impossible to cover all the known potential problems here, but we have tried to cover a few of the common causes.
There are a number of causes of pain depending on your child’s age. Children are prone to infections and may have an earache or a sore throat. Sometimes a child will have developed a urinary infection. In these situations they will usually have a high temperature. They may be teething either in early life with milk teeth or later with their adult and wisdom teeth. A more unusual cause is when a hair becomes wrapped around a toe or a finger creating a tourniquet effect that can cause pain and swelling.
Colicky pain is very common particular in children who swallow a lot of air. There may be a pattern to the crying. For instance, is crying related to eating food or when your child is lying flat? Gastro-oesophageal reflux (GOR) can be a particularly painful condition that might affect you child at any time but particularly when lying down such as in bed at night and therefore be a cause of night-time distress. Has your child been constipated, which itself might be a cause of abdominal pain? Constipation in a child is generally defined as not passing stool for 4 days (3 days in an adult) and with dry, hard stools that are difficult to pass. This is something that parents and carers should be particularly vigilant about as very occasionally surgical complications occur which can be serious. Furthermore, the effort of passing stool might cause small tears (anal fissures) that can be very painful. Other related causes of pain may be skin sores or rashes.
It is possible that your child has accidentally injured themselves and you should check for lumps, bumps and bruises. Does your child wear splints and could these be causing pressure problems.
Little is known about the hormonal aspects of CDKL5. Around the age of puberty, some girls can become tearful due the associated hormonal changes. Girls with a CDKL5 Disorder may be no different.
There is obviously still a great deal to understand about CDKL5-related seizures. Whilst many children continue to have tonic-clonic seizures, others may be having seizures which are much more subtle and possibly difficult to recognise. In these situations your child may be alert enough for the seizure activity to be a source of distress. Again, there may be a pattern in terms of a precipitating factor or event that can be identified.
In conclusion, children with a CDKL5 Disorder do cry and can become quite distressed. In many cases there will be a straight-forward explanation that you can address. However, there will occasionally be important causes that may need medical attention. The understanding of your child’s distress is confounded by their inability to communicate effectively. Obviously, if you go through your checklist and your child remains distressed then you need to contact your family doctor as soon as possible. Although there have been many studies into the causes of crying in normal children, there is very little research about the causes in children with a Neurodevelopmental Disorder. This link is to a good review of causes in normal children. It is fairly detailed but you may find it helpful.
You may also like to take a look at the FLACC Pain Scale. The FLACC scale is a measurement used to assess pain for children between the ages of 2 months and 7 years or individuals such as those living with CDKL5 Disorder that are unable to communicate their pain. Click on the thumbnail for the PDF.
Written by Dr Martyn Newey, Trustee and Medical Adviser CDKL5 UK