PiNSALiNK

PiNSA Newsletter January 2003-01-08

At last some time to concentrate on PiNSA! 2002 was a busy year for me as I presented a paper at a conference in July, moved back to KwaZulu Natal in August, presented another paper in September, went to the IPOPI conference in Weimar in October then presented another paper in Cornwall later that month. Anyway, things have settled in my new job can now work on PiNSA.

PiNSA

PiNSA, Hans Van Rensburg, our Treasurer and fund-raising officer has received a promotion post in the Eastern Cape and has thus resigned. Hans and Engela have been very supportive over the years and this website is indeed dedicated to Morne, their little boy who died of SCID in 1998. We wish you well, both of you, please keep in touch…

Our charity number has again been promised – how slowly the wheels of bureaucracy turn - and our lawyer is going to Pretoria to expedite it. Without it, we can do nothing, as it needs to go on all the letterheads for fund-raising/launches etc. Anyway, shortly thereafter the committee is going to have a strategic planning meeting in order to plan our way forward. Two of the staff from the Natal Bioproducts Institute, Carolyn Rochat and Andrea Smith were in Weimar as was Sheryl Shapiro our secretary. This gave us an opportunity for informal discussion regarding our association and we all came back renewed with commitment. One of the suggestions was to consider employing a part-time fund-raiser as the job really necessitates someone with full focus. Dr Loubser and I had rewritten the Jeffery Modell booklet for South Africa but having seen a similar one produced by IPOPI feel that we should rework it into a simpler format. Alternatively, we could ask for copies of the IPOPI booklet and redirect the finances elsewhere – perhaps to a single day conference/launch in Johannesburg. Speaking of finances, we are now registered with http://www.givengain.com. Should you wish to donate money either as a one off donation per credit card or on a monthly debit order you can simply register at the website. It is absolutely secure and is an easy way to make a donation.

We have some new people on board PiNSA, two of whom are desperate for help.

“Adriana van der Merwe is a 6 year old girl, living in Johannesburg, South Africa. She was recently diagnosed as having SCID - severe combined immunodeficiency caused by purine nucleoside phosphorylase deficiency (PNP). (SCID) Purine Nucleoside Phosphorylase (PNP) deficiency is a very rare genetic disease, initially described in 1975; since then 32 other patients with the disorder have been documented. Since this disease is very rare, not much is known about it.

Adriana's doctor, Dr. Michael Loubser, stated the following in his assessment: "Long-term outlook and management. This is a very rare disease, less than 40 cases have been reported in the literature. Adriana is the 3rd patient with PNP deficiency that I have treated in the past 10 years. Unfortunately this disease carries a poor prognosis. Twenty-nine of the 34 informative cases have died. Sixteen deaths were due to infections including three to chickenpox. Five were due to tumours (four lymphoma and one lymphosarcoma) and three died of GvHD following bone marrow transplantation."

This from her mom Colette Edeling, Systems Analyst co -coordinator
Project Manager - ARIES Payroll Standardisation Project, 011 374-7349/7519
083 377 6161

The website is www.adrianasfund.co.za

and

HI, WE JEETESH & SAMEERA SEECHARAN, HAVE A SON WHO HAS BEEN DIAGNOSED WITH “WISKOTT ALDRICH SYNDROME”. PLEASE MEET OUR LITTLE ANGEL, SHAMEER SEECHARAN.

HE SUFFERS FROM A RARE GENETIC, IMMUNO DEFICIENCY SYNDROME WHICH CAUSES RECURRING VIRAL AND BACTERIAL INFECTIONS, STUNTED GROWTH, VASCULITIS, ECZEMA AND EVENTUALLY LEADS TO LYMPHOMA (AN INCURABLE CANCER IN THE LYMPH GLANDS) IF NOT CURED IN TIME.

Since the age of 3 weeks, he has been frequently hospitalised for Excessive Blood Loss, Recurring Bronchol Pneumonia, Dr Johnson’s syndrome (A severe skin disease), Diarrhoea and Excessive Weight Loss.

After various tests his Syndrome was diagnosed by our Immunologist Dr Michael Loubser at the Olivedale Clinic in Randburg and supported by Professor Beatty at the Red Cross Hospital in Cape Town. Since then Shameer has been on Daily Oral Medication and Monthly Polygam Infusions, which are not enough for him to lead a Normal, Healthy Life. He is 2 Years and 4 Months old but he cannot walk, as his Bones are too weak & he does not sleep much as his inflamed skin itches a lot and his lips are always cut and sore.

The only cure for his Syndrome is a Bone Marrow Transplant failing which he would not live longer than 8 to 10 Years of Age. After extensive Research by Dr Michael Loubser & our numerous visits to prospective facilities in South Africa we have come to the conclusion that South Africa does not have the Specialised Transplant Units or the Skilled Staff to do this Transplant. Dr Paul Veys at the Hospital for sick children at the Great Ormond Street in London has agreed to do the Transplant for Shameer. However it would cost us approximately 70 000 Great British Pounds (Approx. 1.1 million rands) which we are trying to raise.

We have set up a Trust for Shameer, the Trust Details are as follows:
Name of Trust: Shameer Seecharan Trust Bank: Nedbank Branch:East Rand Mall Account Number:1136 009329 Branch Code:113642 Account Type:Cheque Contact People:
Jeetesh Seecharan (083 789 9511) Sameera Seecharan (011) 421-9990
Lux Seecharan (011) 923-1373

and

Jeandre Tinhof

Thank you very much for the information gathered and posted on the Internet. It really helped me after I discovered that my son, Jeandre, suffers from hypo-gamma-immunoglobulineamia. He has low IGA, IGG1, IGG3 and IGG4 levels.

He is 5 and a half years old and started his first POLYGAM treatment six weeks ago. This past Saturday he received his second treatment. I would appreciate it if you could give me some tips with regards to the administration of the drip. Everytime that my son needs a drip, there seems to be some problem with the numbing of the site and the flow of the drip. I
read that your daughter often receives treatment in hospital - well done to the two of you for the positive manner in which you approach her illness. I find it hard to come to grips with the seriousness of Jeandre's condition and the impact that it already has on our lives and definitely will have in future.

Are you aware of any other members of your Association that lives in or close by East London? I need to get more information regarding the POLYGAM treatments, the risk thereof and the progress of research with regards to immunodeficiencies.

All of the best to you and your daughter,

Madre Tinhof Desktop and LAN Specialist T – Systems Daimler Chrysler plant, 7 Settlers Way, East London PO Box 671, East London, 5200 Tel (W): (043) 706 2933
Mobile: 083 415 4101 Fax :(043) 706 2085
E-mail: madre.tinhof@t-systems.co.za

If anyone can help, in any way possible, please do so.

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