“Non-ketotic hyperglycinaemia (NKH) is a rare inborn error of glycine metabolism. [F] has presented with features consistent with classic NKH, i.e. epileptic encephalopathy and hypoventilation during the neonatal period. His genetic testing showed he is homozygous for the severe mutation p.Arg515Ser. All children with classical NKH have a profound neurodisability and seizure disorder. The seizures typically worsen in infancy and continue to progress and become intractable. The seizures are often resistant to the use of multiple anti-epileptic medications. Two other medications used to try to aid seizure management are sodium benzoate and dextromethorphan. The former is used to reduce glycine levels and the latter to block the glutamate binding site of the excitatory NMDA receptor. The ketogenic diet can also be used as an adjunct to seizure management. At this time there are no curative treatment options available so treatment if symptomatic. Gastrostomy feeding is required due to the unsafe swallow seen and other complications can include cortical blindness and orthopaedic problems such as scoliosis. Severe NKH is considered to be a life-shortening condition, but it is not possible to put a definitive timeframe on survival. It is appropriate for an advanced care plan and do not attempt resuscitation order to be in place given the severe neurodisability and seizure disorder absence of any curative treatment options.”
“In the case of [F], the Trust’s position is that given his diagnosis and prognosis, the following treatments will NOT be offered to him in any event, because they would be clinically inappropriate: a. Chest compressions b. Defibrillation c. Cardiac/ALS drugs (usually in conjunction with chest compressions) d. Intensive care admission. The consequence of this, is that this Court should not make any determination as to whether or not such treatment might be in [F]’s best interests – see for example Burke v General Medical Council[2005] EWCA Civ 1003 [2006] QB 273 at paragraphs 50 and 55; AVS v. A NHS Foundation Trust & ors.[2011] EWCA Civ 7 at paragraph 35 and Aintree[2014] AC 591 , para 18. With respect to the two remaining treatments to which those with parental responsibility do not consent on [F]’s behalf (namely non invasive ventilation and intraosseous access), the Trust can see some circumstances in which these treatments could be offered to [F], but take the view that given his condition and diagnosis, it is unlikely to ever be in his best interests for him to be provided with them. The Trust accepts however that in respect of those treatments, the Court could make a determination of [F]’s best interests. It is important to note however that the CYCAP and RESPECT form are not legally binding and should circumstances change, and the Trust were to take the view on some future date that any of these treatments were not only clinically indicated (i.e. would be offered to [F]) but were also in his best interests, the Trust is not bound by the record of agreement in the CYCAP. In such circumstances: a. The Trust would consult with those with parental responsibility explaining their changed view in the usual way, seeking their consent to deliver the treatment that has been assessed as being in [F]’s best interests. b. In the event that agreement could not be reached, the Trust could make an application to the Court for determination of [F]’s best interests.” a. The Trust would consult with those with parental responsibility explaining their changed view in the usual way, seeking their consent to deliver the treatment that has been assessed as being in [F]’s best interests. b. In the event that agreement could not be reached, the Trust could make an application to the Court for determination of [F]’s best interests.”
“Ultimately, however, a patient cannot demand that a doctor administer a treatment which the doctor considers is adverse to the patient's clinical needs.”
“This person is less than 18 years old and those holding parental responsibility have been fully involved in discussing and making this plan.”
“The RESPECT form is a clinical record of agreed recommendations. It is not a legally binding document.”
“End of life care for infants, children and young people with life-limiting conditions: planning and management.”