NKH Progression

The progression of NKH is tricky – because there are so many different mutations each child progresses differently. However, there are some anecdotal progression stages that parents have noticed.

These aren’t hard and fast progressions – there hasn’t been any research done into NKH progression and they won’t apply to every child. However, we know how as NKH parents the future and knowing what to expect can help as we come to terms with NKH.

So, we have a very loose NKH progression guideline. Please take it with a grain of salt.

Birth

The baby may be born incredibly lethargic, and hypotonic (floppy, like a rag doll). They may have difficulty breathing and be difficult to wake/feed.

Day 2 – 3

Severe babies will find it difficult to breathe and may be placed on a ventilator.

Some parents will make the very difficult, courageous decision to withdraw care at this point. If your child is extubated more towards the 10-day mark, there is a chance your child may survive.

Day 10 – 14

Babies will typically go one of two ways.

  1. They will begin to breathe over and above the ventilator, eventually being able to self-ventilate.
  2. They may not come off the ventilator.

This is very difficult to time, we send you love.

After Two Weeks

Babies will be the best they possibly can be. Typically you will have a diagnosis at this point and should have started Sodium Benzoate + Dextromethorphan.

Your baby will eventually learn to suck and may be able to take some feeds orally. They may even be able to have the NG tube removed.

Two month vaccinations

Vaccinations can have an odd effect on our children. They can also be a catalyst for the Two Month Curse. If you can, delay vaccinations until your child is a bit older and stronger. There is no harm in delaying vaccinations.

The Two Month Curse

Around two months (sometimes earlier, sometimes later) the seizures will start, otherwise known as hypsarrhythmia.

Often these kinds of seizures aren’t responsive to medication.  Please familiarise yourself with the no list (specifically: your child should not be given phenytoin or sodium valporate).

Often a child will go into a seizure coma, and be on seizure medication infusions. You will be told there is nothing they can do for your child, and you may be referred to hospice. It’s a horrible time. Stay strong.

Things to note:

  • If your child’s head isn’t repositioned regularly, they will develop a flat spot. This will become a problem later, so rotate your childs head every 15 – 20 minutes to stop plagiocephaly forming.

Two Month Curse Recovery

Despite all the doom and gloom of the doctors, there is a chance your child will recover from the Two Month Curse.

Seizures will be a regular thing, but can be managed. Hopefully, your child will recover from the seizure coma, wake up, and be able to go home.

At this point, if possible:

  • Get regular physio sessions in (ideally weekly)
  • Start neuroprotectant vitamins, if you haven’t already.
  • Invest in a pulse oximeter that has alarms, that you can use as a seizure monitor overnight.
  • Encourage your child to spend a lot of time on their belly – this will help prevent a flat spot from forming. Else, be sure to reposition their head regularly.

Five Months

If your child does have a flat spot, now is the time to get it checked out and sorted. Do not let your doctor tell you it is just cosmetic, as it’s quite simple to fix with a helmet if you need to.

Six Months

Teething will become a thing relatively soon. Pain often brings on seizures, so be prepared with paracetamol and bonjela.

Other things to start thinking about

  • Your child may not be fixing or following (visually). If possible start patterning high contrast vision activities and vision patterning. You may also want to get an opthamologist to confirm Cortical Vision Impairment.
  • You might want to start looking at scoliosis prevention. You could look at a sleep system, hipp helpers, a DMO Suit and/or happy straps to ensure proper hip positioning.
  • Now might be a good time to start introducing objects of reference – these are objects that make a noise or have a particular shape/texture and are used to indicate a specific activity is about to start (for example, placing an object in the childs hand to indicate a nappy change is coming). Helpful for transitions and to build anticipation.

One Year

At this point a neurotypical child is beginning to stand and cruise. If your child doesn’t start weight bearing their hip sockets will not form and they will be at risk of hip dysplasia (which can be quite painful).

So, invest in a stander and have your child spend a lot of time in it (working up in 5 minute intervals to at least an hour a day)

Around this time (in the UK, a lot earlier the US) your child might be fitted with a gastrostomy to help with feeding and meds. If you have the capacity, it might be helpful to start exploring a blended diet. Some parents have had positive results with the Failsafe Diet – some NKH kids have quite severe reactions to colourings, msg, preservatives and antioxidants, amongst other things and removing these ingredients can be beneficial.

Life expectancy

Because there are so many different mutations and the spectrum is wide – there is no clear answer as to how long our children might live. Here are some difficult, very not firm, estimates from the literature:

  • 80% of babies with NKH will not survive the neonatal phase.
  • Most children with severe NKH are not expected to live past their fifth birthday.
  • Most children with mild NKH are not expected to live to adulthood.
  • The eldest, currently living person with NKH is 38. She has very very mild NKH and her particular experience is very rare.

This is very grim to read, but have hope. The doctors are not always right and repeatedly our children surprise us. Additionally, we are much closer to a cure than we’ve ever been before. Please donate towards NKH Research – the current bottleneck to our children’s health is funding.