SCID or Severe Combine Immune Deficiency is the most severe of the Primary Immune Deficiency diseases. The defining characteristic of SCID is the absence of T cells and, as a result, lack of B cell function as well. Unless these defects are corrected the child will die of opportunistic infections before their first or second birthday.  There are several different types of SCID, however no matter what the form of SCID, the end result is that critical immune defences are missing and treatment should be considered a paediatric emergency.

 

Liams’ Story

Liam was born with no complications on 19th February 2008 and was a happy little boy who loved food! 

Liam was about 6 months when we took him to Eurodisney with his older sister and just 2 days after our return he was taken by ambulance to the William Harvey Intensive Care.  Liam was unconscious and the doctors initially suspected meningitis and septicaemia, the doctors exact words where ’Liam is already in a critical condition, he needs to respond to the antibiotics in the next 30 minutes or this could become much more serious’.  Upon scanning Liam’s chest they discovered his heart and left lung was surrounded by infected fluid and was rushed to the Evelina Children’s hospital in London.  Liam had ‘Pericarditis’, this was unknowingly Liam’s first encounter of SCID!

Thankfully Liam made a good recovery from Pericarditius, however his immune system continued to let him down as he then deteriorated suffering from severe ear infections only curable with IV antibitotics, pneumonia and severe mouth ulcers. 

 

Eventually, after seeing many consultants Liam was referred to Great Ormond Street and after many complex blood tests he was diagnosed with SCID and failure to thrive.  We was told he would need a Bone Marrow transplant as soon as possible and was put on immunoglobulin therapy (human antibody treatment), profalactive antibitotics to give him protection against infection and steroids.  Liam could no longer go into busy public places such as Town Centres, Supermarkets etc and the few people that came into contact had to wash there hands for fear of an infection that he may be fatal.  In addition, he was given a nasal gastric tube for milk feeds day and night because Liam stopped eating when the severe mouth ulcers appeared.

 

Liam was admitted for his transplant on 12th October 2009.  His treatment started with intensive chemotherapy for 7 days to wipe out his own immune system and then his new stem cells received from a cord blood donor was infused.  Liam was quite poorly during the wait for his new immune system to grow.  At the beginning intravenous medicines where infused for approximately 20hrs a day.  Despite all of this Liam showed us amazing strength and courage as he coped with isolation, sickness, infections and Graft vs. Host disease all of which are side effects of chemotherapy and the new stem cells initially looking to attack his body. 

Liam had us worried a few times during the transplant, however compared to some of the children we met on the ward, Liam did extremely well to only be in hospital for 10 weeks and we owe that all to the wonderful Consultants, Doctors and Nurses on the Bone Marrow Transplant ward at Great Ormond Street.  They really do all they can to give children like Liam and others with similar life threatening conditions a chance of life and for this we are truly grateful.

Bone Marrow Transplant (BMT) offers the only long-term "cure" for SCID at present.  The aim is to replace the faulty immune system with an immune system from a healthy donor. Healthy bone marrow is rich in stem cells, from which all the cells of the immune system develop, and it is possible to take bone marrow from a healthy individual and give it by transfusion into the child with SCID. A BMT is not an operation like a heart or kidney transplant. Stem cells contained in the donor bone marrow are able to find their way from the bloodstream to the child's bone marrow where they start to produce healthy blood cells. A BMT does involve a number of risks, and complications can arise - some of which are temporary, others of which can be life-threatening.

 

Liam regularly visits Great Ormond Street for complex blood tests to see how far his new immune system is progressing. 

Liam is now 11 months post transplant and is small for his age but otherwise he is doing really well and is starting to thrive again.  He still receives immunoglobulin therapy, 2 profilactive antibitotics and steroids.  Hopefully in Spring/Summer 2011 his immune system will be ready to deal with childhood vaccines and we hope he will build good / normal responses. 

 

Great Ormond Street have invited the family to take part in a 10 mile sponsored walk to support the Blood and Marrow Transplant Fund.

 

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