The Sultans Story

Written by Sham Sultan, Mum. This is the story of Adeel, Aseefa and Deen Sultan. Each of them has Osteopetrosis.

Adeel was born in 1982 and was in hospital for 3 months from the age of 3 weeks with whooping cough. When he was 3 years of age for some strange reason he developed a limp. Upon x-rays and a scan we were told he had an abscess on his hip and needed weights on legs to drain the abscess. It wasn’t until a month after that we went for a check up and were told he had Osteopetrosis.

Nobody really knew anything about Osteopetrosis in 1986 and we were told that his life expectancy was short and that there was no cure. I was not satisfied with that so I contacted some friends in New York asking for an address for a paediatric doctor. I then wrote to this doctor who in turn told me that there was a cure and that was a bone marrow transplant. The doctor looking after Adeel then referred him to the children’s hospital to see an Oncologist. The doctor was wonderful and still is. Dr Darbyshire explained everything about a BMT and we (Me, my husband and my (8 mnth old, at that time) daughter) were tested for a Bone marrow match. I was the one match.

Adeel had his transplant in 1991. Prior to that, when he was five years old he had optic nerve decompression surgery. This was to lessen the pressure on his optic nerves causing him to slowly lose his eyesight. First they operated on the right side as that was the worsening eye and 6 months later they operated on the second side. This actually stopped his sight from deteriorating although it couldn’t make it better.

Adeel went into hospital on the 18th of March 1991 to start treatment for his transplant. He died on May 21st 1991 while still on the transplant unit. Aseefa was born on March 16th 1993. She seemed like a healthy baby but caught a chest infection when she was 6 weeks old. As I took her to be checked I prayed and prayed that she’d be all right. Nothing could prepare me for the following day.

When Adeel was diagnosed with Osteopetrosis we were told that it was a one in a million thing that had happened to Adeel, but when the doctor looking after Aseefa came to talk to us he came straight out and admitted that her x-rays quizzed him.

That’s when I knew Aseefa had Osteopetrosis. I broke down in front of him and he was very apologetic, thinking he had said something wrong. When I managed to calm myself I found my husband explaining Osteopetrosis to him. He hadn’t heard of it as many before him. All we could do was tell him what we knew.

Aseefa was then referred back to the children’s hospital and Dr Darbyshire.

With the help of family and friends and the Internet I came across a doctor in America who told me about his research and the medication he had tried out. With the 2 doctors corresponding Aseefa was put on Gamma Interferon Injections and Calcitrol (vitamin d) tablets. For a while I thought the interferon was working but it was having an emotional effect on Aseefa and the family. Aseefa was afraid of the injections and when the time came for her treatment she would hide away or not talk to either of us. This went on for 2 years but the results couldn’t justify her going through that much emotional stress. At the moment she is on Calcitrol capsules and is fairly well. She also had optic nerve decompression surgery but only to her left side. Unfortunately she has lost the little vision she did have in that eye and we have decided not to go ahead with the second surgery. Aseefa’s legs were bending outwards from the knee, in 2002 she had staples inserted (under the skin) into her knees to prevent her legs from bending further. These staples will be taken out at a later date(when the doctor thinks they have corrected the growth)..

Deen was born on 2nd June 2002. His Osteopetrosis was diagnosed when he was a week old. He was admitted to hospital because he was having fits. Small jittery fits, which were put down to, low calcium level. Deen had blood taken and an x-ray but I knew he had Osteopetrosis before they could even recognise the family name.

I explained that Aseefa was under Dr D and then they pulled up Aseefa’s records and checked Deen over thoroughly. He had a hearing test, a CT scan, his heart monitored and his sight checked which all are at the moment fine.

They both go for regular check ups and at the moment Deen is fine. His growth is slow but he has put on weight.

I have two other children born in between Adeel and Aseefa, and Aseefa and Deen and they are fine. My eldest daughter had an x-ray when she was 6 years of age and Shabeena who was born in 1996 had an x-ray the day after she was born. They both have normal x-rays but no one in the family is a match for either Aseefa or Deen. Shabeena and Deens cord blood cells were taken and are stored if needed. Deen is an exact match for Aseefa but they both cannot help each other as they both have Osteopetrosis. We are at the moment awaiting a cord blood cell match or a bone marrow match.